Pediatric SurgeryHOUSESTAFF MANUAL

UNIVERSITY OF CALIFORNIA, SAN FRANCISCO

Last revised: January 2013

In Pediatric Surgery, always remember:“Call Early, Call Often”

“Children are NOT little adults”

“You don’t know what you don’t know”

“Bilious vomiting is a surgical emergency until proven otherwise”

“A baby has no language but a cry”

“Primum non nocere”

“Before anything else, do no harm”

“But also, do some GOOD”

“To cure sometimes, to CARE always”

476-2538 - 24/7/365

Prayer of the Newborn Undergoing Surgery:Let them keep me warm,

Let them keep my airway clear,

Let them maintain my blood volume,

And please LORD, let them get me right the first time.

IntroductionThis manual is intended to serve as an orientation to the Pediatric Surgical Service at Parnassus. Wesee and treat a wide breadth of problems on this service. Management of pediatric surgical patientsrequires constant attention to detail with little margin for error. The tempo of disease processes inchildren can be quite rapid. Be careful when ordering medications and intravenous solutions—dosages for pediatric patients are based on mg/kg. There are always plenty of resources available,particularly if the care of children is new to you. For any problem that arises, always err on the sideof too much communication rather than too little communication with the attending. You may not knowwhat you don’t know when it comes to the care of children. Remember, children are NOT littleadults!

FTC/Pediatric Surgery OfficeThe FTC/Pediatric Surgery Office is located at 400 Parnassus, 1st floor, Room A-123 (next to theclinical lab). Our office staff and nurses are located in this area. There may be times when you arepaged by the Nurse Practitioner to see a patient here. There is one exam room with limited supplies.This area is only used for drop-in or urgent patients. Sometimes the attending surgeon may alsoschedule a patient to be seen here during non-clinic hours.

Important Phone NumbersAll numbers are area code 415 unless stated otherwise.

24/7 PedSurg Attending: 476-2538

Pediatric Surgery

Michael Harrison, MDCell: 235-5812Ranch: 707-847-3430Home: 665-0297Office: 476-4086

Lan VuCell: 283-8837Pager: 443-2897Office: 476-0447

Hanmin LeeCell: 235-7702Pager: 443-8700Home: 285-1323Office: 502-7392

Doug MiniatiCell: 816-6025Pager: 443-1929Home: 564-6615Office: 476-3642

Shin HiroseCell: 519-2417Pager: 443-2443Office: 476-0447

MacKenzieCell: 238-6934Pager: 443-9930Office: 476-2479

Maura O'DayCell: 508-272-7066Pager: 443-4088Office: 476-3641

Barbara BrattonCell: 699-0716Pager: 443-1565Home: 452-2804Office: 476-9717

Jody FarrellCell: 650-218-6147Pager: 443-3402Home: 650-692-8147Office: 476-0445

Appt Scheduler, Olga MartinOffice: 502-6740Fax: 476-2929

OR Scheduler, Brian FormanOffice: 476-8080Office: 476-2539

Surgery AssistantOffice: 476-8580

Important UCSF NumbersGeneral

Academic Offices HSW 1601 476-4086

ACC Fax 353-2279

ACC Front desk Pediatric Surgery 353-2586

ACC Pediatric Primary Care 353-2287

ACC Pediatric Urgent Care 353-2025

ACC Team Room 353-2852

Access Center/Pediatric admissions1-877-UC-CHILD1-877-822-4453353 1611

Chaplain Services 443-2773/353-1941

Child Life Services 353-1203

Teresa McDonald(outpt) 443-4159

Consult Pagers

Adolescent Medicine 443-1469

Cardiology 443-4547

Child Life 443-4914

CT Surgery Fellow 443-7834/443-0338

Dentistry 443-2243

Dermatology 443-7792

Derm Fellow 443-6221

Discharge Coordinators

ICN: Irma China 353-1825

ICN: Amanda O’Byrne 353-1824

Pedi: Anne-Marie Philpott 353-8410

Pedi: Anne-Marie Pager 443-1519

Endocrine/Diabetes 443-9086

Feeding Therapy:

Nora Chun-Uba 443-4081

Miriam Crennan 443-8981

Helen Sang 443-0668

Genetics 443-8075

GI-Academic office 476-5892

Hematology/Oncology 443-6966

Infectious Disease Fellow 443-2384

Neurology 443-9204

Neurosurgery 443-4323

Ophthalmology 443-9654After hours 443-9652

Orthopedic Surgery 443-5621

Otolaryngology 443-8724

PICC line RN: Anna Liang 443-7492/3-1143

Plastic Surgery 443-7307

Psychiatry 6-7231After hours 476-8905

Pulmonary 443-0631

Renal Academic office 476-2423

Rheumatology/Immunology Academic office 476-2491

Sedation team 443-1121

Urology Academic office 353-2200

Urology Fellow Pager 443-0372

CPMC Radiology dictation line 1-866-803-3838

Directions to campus 476-2999

Emergency Room-Main number 353-1238

Pedi Urgent Care in ER 353-1818

Family House 476-8321

Family Resource Coordinator 353-2016

Fetal Treatment Office 476-0445

Interpreter 353-2690

ATT (UCSF ID# 295007) 1-800-874-9426

Lab services 353-1667

Blood gas lab 15 353-1755

Blood gas lab PICU 353-1195

Chemistry 353-1501

Hematology 353-1747

FNA Service 443-1845

Microbiology 353-1268

Pathology 353-1613

Blood Bank 353-1313Medical Records Chart request 353-2473

Nutrition

Lisa Boice (ICN) 443-9460

Carrie MacFarland (Peds) 443-2299

Pain Service 443-6100

Pathology Hot Seat 353-1296

Grace Kim 443-2763

Pharmacy

TPN 353-4710

On call pharmacist 443-1779

IV Adds 353-1216

Main 353-1028

Pediatric ORs

Room 21 353-8521

Room 22 353-8522

PACU 353-1965

Surgical Waiting Room 353-1626

Pedi OR Scheduling 353-1591

Pediatric Surgery Office 476-2538

Pediatric Surgery Social workers

Caroline Casey (pager) 443-3988

Eunice Flores-Uselman (pager) 443-8663

Sharon Nomberg LIFE Clinic (pager) 443-9603

Pediatric Urgent Care

Phlebotomy: Tatyana Mendes 443-0451

PreOp Fax 353-1303

Prepare Clinic 353-1150

Radiology

CT 353-1189

CT body 353-1354

Fluoro 353-8585

Reading room 353-1384

On call reading room 353-9027

Interventional 353-8694

IR scheduling 353-1300

IR on call Fellow 443-9417

Nuclear Med 353-1521

US Moffitt 353-1963

Moffitt Reading room 353-1353

US ACC 353-2572

ACC Reading room 353-2038

Radiology Scheduling 353-2573

Registration 353-1724,1037

Surgery Center 353-2384

ER Main 353-1238

Urgent Care Pedi (in ER) 353-1818

6 Long 353-1921

6 Long Doctors Room 353-1047,1053,1054,1064

6 Long Senior Peds Resident 443-MDUC6 PCRC 353-8744

6 PICU 353-1352

6 North 353-1005

7 Long 353-1631

7 Long Doctors Room 353-1159,1161,1162

7 North 353-9140

7 BMT 353-1616

7 PICU 353-1955

14 Long 353-1321

15 ICN 353-1565

15 East Doctors Room 353-1525,1527

15 West Doctors Room 353-1450,1451,1453

15 WBN 353-1978

15 L&D 353-1787

Surgery Scheduling (ask Brian) Transfers See Pediatric Access Center) 353-16111-877-UC-CHILD

Pediatric bed control 353-1937

Resident ResponsibilitiesPlease meet with Dr. Hirose 3 times during your rotation (start, middle, end)

The residents on the Pediatric Surgery team are responsible for the following activities:

Patient ListPlease confirm the patient lists on APEX daily: short list and active list.

Keep inactive surgical patients on short list until discharge.

Do not sign off on surgical patients without consulting with attending.

HoursResident work hours must be logged on a weekly basis.

Hours will be reviewed at Wednesday am conference.

No full weekend call is permitted.

Submit schedule to Dr. Hirose before starting.

RoundsPatient rounds and notes should be completed prior to AM cases starting in the OR. Attending walkrounds will be done on a daily basis. Page the surgical nurse (443-4088) to “15” at the beginning ofdaily rounds.

Please notify the attending surgeon of all significant changes in patient condition, regardless of thetime of day.

AdmissionsPromptly inform attending surgeon of any new admissions & consults. The majority of outsidereferrals are received directly by the attending surgeon, via the answering service (476-2538), or bythe hospitalist service. If you receive a call directly from an outside pediatrician/hospital, please taketheir name, hospital and contact number and promptly report to attending. Do not accept any patientsto the pediatric surgical service without approval of the attending surgeon.

All accepted inter-facility intensive care transports are to be referred to the fellow of the neonatalICU (3-1565) or the pediatric ICU (3-1352), as appropriate, for coordination of admission. Aneonatal transport is considered for any patient under 30 days of age. Emergent admissions to thepediatric acute care floor should be coordinated with the pediatric transfer center (1-877-UC

CHILD).

Attending PhysicianThe schedule for the attending physician changes weekly and may also include differentnight/weekend coverage. Please check with the office or attending on service regarding the on callschedule.

Attendings: Phone CallsCall attending directly or positively ascertain attending has been notified upon the followingsituations:

Death (even if expected)Cardiac arrestRespiratory failure either requiring intubation or significantly increased O2 demands.Severe respiratory distressAirway issuesTransfer to ICU or higher level of careConcern that patient needs a procedure or operationA new need for acute dialysisBleeding requiring transfusionHypotension/hemodynamic instabilitySymptomatic and severe hypertensionSignificant new arrhythmiaSuspected MISuspected PENew onset severe chest painNew onset severe abdominal painAbrupt deterioration in neurologic exam or profound decreased mental statusSignificant change in neurovascular exam of extremityPatient or family wishes to speak to the attendingPatient wishes to be discharged AMA

Plus

Any other significant change in clinical status of patient that is of major concernAny new admissionThe arrival of a patient accepted in transfer from another institution

ConsultationsPerform consultations requested by other services including the Pediatric and Neonatal ICUs. The

consultation template must include the name of the requesting attending physician (even if a residentrequests the consult).

ICN ConsultationsWhen rounding in the ICN, remove lab coat and jewelry (watches, bracelets, and rings). Roll upsleeves. Scrub hands prior to patient contact once in the AM. Check with nurse before examiningpatients.

Operating RoomThe pediatric surgery service has block time in the main OR on the following days: Monday all day,Tuesday AM, Thursday all day, Friday all day. Residents should check the OR schedule ahead oftime and review the relevant imaging, learn the patient history, and read about operative managementprior to each case.

Provide operating room coverage as assigned. Residents must be in the OR by 7:15 am for first cases.A clinic letter (in APEX for patients evaluated at UCSF) will suffice as the H&P, provided that thephysical exam has been updated. Our service is not to be the reason for any delays into the OR (i.e.no H&P or consent).

Cases may be scheduled through our office (Brian at 6-2539 or 6-8080) up to 11 am the day prior.After this time, cases are placed as add-ons at the front desk after 3 pm the day prior (353-1545).Some prenatally diagnosed cases may be scheduled under the mother’s medical record number. Thisinformation will need to be updated after the infant is born.

All operative forms and operative dictations are to be completed by the attending surgeon.

Patient ManagementThe surgical team works closely with the PICU and ICN teams for any surgical patient in these units.Management plans are discussed with these primary services on a daily basis. If a surgical procedureis performed on a patient and we are NOT the primary team covering this patient, a transfer orderform is required in the PACU to return the patient to the floor.

Following communication from our team regarding the surgical procedure and care, the primary teamwill complete the remaining orders. It is very important to call the primary team after all operationsso that they can prepare to accept the patient back on the ward. In general, if there are any questions,consult the attending surgeon regarding to which team a patient belongs.

All patients less than 40 kg require PEDIATRIC order forms. This is a home call service; however,verbal orders are RARELY accepted. The SOD and/or pediatric on call resident may also be willingto write routine orders for you if you are on home call. If there is any question, it is probably mostprudent to try to clarify orders prior to leaving the hospital.

This may be the first “home call” experience for some residents. New consults require return to thehospital for evaluation. It is less clear when to return to evaluate inpatients and it is difficult to create

specific guidelines for each clinical scenario. Certainly, any significant change in vital signs wouldprompt a return, as would airway, breathing, or bleeding issues. When in doubt, come in. If an hourviolation would result, call attending and inform him/her of problem.

ClinicAttend outpatient clinic Wednesday at 1:00 pm. The clinic is located on the second floor of theAmbulatory Care Center. Patients are seen in consultation with the attending surgeon. All clinicdictations are performed by the attending surgeon.

Discharge plansDiscuss anticipated discharge plan with the family on rounds early. The goal of the pediatric floors isto have discharges by 11 am. Notify the discharge planner and pharmacist on call 48 hours prior toplanned discharge. The discharge planner will help to set up follow-up clinic appointments, medicalsupplies, and home nursing. The on-call pharmacist will help complete the prescription forms, callthe prescriptions into the pharmacy, and resolve any pharmacy-related issues (i.e. insurance).

Note: There are no pediatric pharmacists here on the weekend. Please call and notify the pharmaciston Friday for anticipated weekend discharges. Discharge orders should be written early in themorning on the day of discharge. Patients must be discharged by 11 am, or the family will be chargedfor an additional day of hospital stay.

Most patients do not need to return to the office for a follow-up visit and are discharged to the care oftheir primary provider. Those patients who require a specific postoperative intervention need toreturn for a clinic visit (such as patients who undergo anorectal operations and require anal dilation).When in doubt about the need for a clinic visit, ask the attending or surgical nurse.

For inpatients, the discharge coordinator will schedule follow-up clinic appointments. Foroutpatients and 23 hour stays, the parents are asked to call our office at 476-2538 to schedule anappointment or contact the surgery team for postoperative questions and/or phone follow-up in 2weeks. Please give each family our business card.

Patients who only need phone follow-up/no clinic visit are:

Umbilical hernia, epigastric hernia, inguinal hernia (lap and open), simple non-perf’d appy, pyloricstenosis, lap chole, lumps/bumps, urachal remnants, hydrocele.

No phone follow-up/no clinic visit: lines.

LIFE ClinicLong-term Infant-to-adult Follow-up and Evaluation

LIFE clinic is available for patients with CDH, Hirschsprung’s disease, imperforate anus, esophagealatresia, sacrococcygeal teratomas, choledochal cysts/biliary atresia and CCAM/CPAM andpulmonary sequestrations. These clinics are offered monthly to annually. Contact the pediatric surgery

NP to determine if a patient is eligible for a LIFE clinic appointment at the time of discharge.

Social WorkerPediatric Surgery has an assigned social worker to assist families during their hospitalization. Shecan assist our families with questions about housing, insurance and community resources. Our socialworkers can be reached at 443-3988 and 443-8663 (pager). In the ICN: 443-9603 (pager).

Specific duties of each resident

Senior ResidentOrganize weekly M & M conference, including topics for discussion. The inpatient list will berun during the Wednesday am conference. Update number of work hours spent in the hospital foreach surgical resident in pediatric surgery.Participate in education of junior resident and medical students.Supervise dictation of timely discharge summaries.Generate call schedule for the month on service, give to surgical nurse.Run the twice daily work rounds.Assign cases for the following day. Prepare for major cases—see the patient, review x-rays,discuss operative plan with attending, read about the disease process and surgery.Pertinent scans, etc. should be available in the operating room for all major cases.Read prior operative reports for all re-do cases.

Junior ResidentCollect admission, discharge, consultation, operative cases and hours worked by housestaff forweekly M & M conference. Directly supervise medical students and make certain that notes areco-signed.Coordinate radiology list for Wed conference: compile list of patients to discuss for weekly M &M and email list of patients names with pt history and clinical question to pedi radiologists(Pierre Cohen, Robert Brasch, John MacKenzie, Jesse Courtier, and Andrew Phelps) by Mondayprior to conference.Check all lab results and other diagnostic procedures obtained on patients that day.Responsible for all discharge summaries from the service.Preop inpatients the night prior. Not all patients require type and cross, coags.Communicate daily with inpatient pharmacy, TPN staff.

Conferences

Tuesday1:00 PM - Fetal Treatment Meeting - M 1486 Page Library

Wednesday7:00 AM - Surgery Grand Rounds - U142 Toland Hall8:00AM-10:00AM - Basic Science/Skills lab/Journal club - Toland Hall, Mount Zion8:30 AM - Lab Meeting (optional) - HSW 160110:00 AM - Morbidity and Mortality - HSW 1601 Conference room - Work hours must beturned in!!1:00 PM - Clinic - ACC 2nd floor

Thursday11:00 AM - Tumor Board - L-34

Perioperative Management

NPO Guidelines (UCSF only)No solid food, formula or milk after midnight.Patients may continue clear liquids for up to 4 hours prior to procedure.All inpatient patients should be NPO at midnight prior to procedure to allow for schedulingflexibility

Apnea and Bradycardia monitoringA 23 hr admission of any ambulatory surgery patients if:Less than 37 wk ga @ birth and less than 50 wk total post gestational age

or

Term (37wk or greater) and less than 6wk old

All neonates undergoing major procedures should have routine, continuous pulse oximeter monitoringpostoperatively.

Fluid and Electrolytes

Maintenance FluidD10 1/4 NS + 20 meq KCl/L if < 30 days of ageD5-D10 1/2 NS + 20 meq KCl/L if > 30 days of ageAnd < 6 monthsD5 1/2 NS + 20 meq KCl/L if > 6 months “Normal” Neonates (Day 1-5) 65-70 ml/kg/day “Normal” Neonates (Day 6-7) 80 ml/kg/day “Normal” Neonates (Day 8-30) 100-150 ml/kg/dayOlder infants and children

0-10 kg 100 ml/kg/day11-20 kg 1000 ml + 50 ml/each kg over 10 kg> 20 kg 1500 ml + 20 ml/each kg over 20 kg

ElectrolytesNa+ 2-4 meq/kg/dayK+ 2-3 meq/kg/dayCl 2-3 meq/kg/day

Ca 0.5-2.5 meq/kg/dayMg 0.25-0.5 meq/kg/dayP04 1-2 mM/kg/day

Gastric drainage replacement:Bilious: LR (ml/ml)Clear: D5 1/2 NS + 20 meq KCl/l (ml/ml)Enterostomy: LR (1/2 ml/ml)

Nutrition

Nutrient needs of children per day<</th>

Age Energy needs (kcal/kg) Protein needs (gm/kg)

Neonate 100-150 2-3.5

0-6 months 108 2.2

6-12 months 98 1.6

1-3 years 102 1.2

4-6 years 90 1.1

7-10 years 70 1.0

11-14 years 50-55 1.0

15-18 years 40-45 0.8

Pediatric Nutritionists are available to advise you regarding enteral and parenteral feeding. Seeimportant phone #’s.

See TPN guidelines [FIX]See pediatric formulas table

SedationAvoid painful procedures in children. We use the OR and general anesthesia liberally in children.Conscious sedation can also be performed by the sedation team. There are pediatric guidelines forpainful procedures like blood drawing and tube removal/dressing changes. Please ask the nurse fordetails.

Procedural SupportFor any anticipated invasive, painful, or anxiety-provoking procedures at the bedside, please callChild Life and the bedside nurse before the procedure to prepare the child and family. Please use the

treatment room for patients on the pediatric floor.

Family SupportChild life services and social workers are available to support patients and families through thehospital experience. Please make referrals as needed.

Bowel PreparationPlease check with the attending of the week before starting the prep.

In House:Infants may be admitted the day prior for bowel preps.

IV hydration is required for this age group during the prep.

Begin at noon or sooner:

1. Start IV: D5 1/2 NS with 20 meq KCl/L at maintenance2. Place NG tube3. Begin golytely 25 ml/kg/hr via NG tube for 4-6 hours, or until stool is clear4. Pedialyte or clear liquids ad lib until patient NPO for OR

Ask attending for maximum fluid limit for golytely prep and additional instructions re: stoma/rectalirrigations.

Outpatient: Bowel preps can be done at home for older patients, however we may need to admit them if they arenot tolerating the prep:

Miralax Cleanout Instructions (5 days)5 days prior – Miralax 1 capful in 8 ounces of water or juice Q am and pm daily2 days prior – clear liquid diet1 day prior – 14 capsuls of Miralax in 64 ounces of Gatorade. Finish by early afternoon.Dulcolax 5 mg pill at 3pm and 9 pm. If unable to take Dulcolax pill, can take Senna ex-lax 7.5 mgat 3pm and 9pm. Clear liquid diet until NPO after midnight.

Rectal IrrigationRectal irrigation volumes should be 10-15 ml/kg of normal saline. Infants can become severelyhyponatremic if tap water enemas are performed!

Composition of fluids

Na K Cl HCO3

Saliva 10 26 10 30

Stomach 60 10 130

Duo 140 5 80

Ileum 140 5 104

Colon 60 30 40

Panc 140 5 75 115

Bile 145 5 100 35

Guidelines for RBC transfusionDo not order any blood product transfusion without consulting with the attending surgeon first.Anemia is well-tolerated by otherwise healthy children. Blood consent will be required.

All patients: Hemorrhagic shock not responding to 2 crystalloid fluid boluses (20 cc/kg x 2).Infants and children: Hct < 21%Neonates:

Hct < 30% if receiving < 35% Fi02, if on CPAP or mechanical ventilation, if having A&Bspells, if P > 180 or RR > 80, or if undergoing surgeryHct < 35% if receiving > 35% Fi02 or on mechanical ventilation with MAP 6-8 cm H20Hct < 40% for critically ill neonatesHct < 45% for critically ill hypoxic (pa02 < 60) neonates or neonates with cyanotic congenitalheart disease

Note: no absolute indications for transfusion based on Hct. If patient is stable and no further bloodloss is expected, transfusions may be held.

Table for amount of transfusion based on current hct, target hct, and body weight is in Harriet Lane.

Generally, 10cc/kg is roughly equivalent to tranfusion of 1 unit in an adult.

Central LinesPlease clarify with the referring service to determine what the line is for and what type of line isneeded. The requesting attending should document the type of line needed in APEX prior to the OR.Not all patients require a CXR in the PACU following line placement, please consult with theattending surgeon.

Broviac (1 or 2 lumens, power or silastic)Portacath

Vascath for dialysis/pheresisPermacath for dialysis/pheresis Look at fever curveCheck CBC and platelet count especially if the patient is on chemotherapy

Central line problemsClotted: A hospital policy is in place, based on tPA administration. Available from thepharmacy.Broken: Repair broken broviac catheters with appropriate repair kit. Each type of broviaccatheter has its own specific type of repair kit based on size and number of lumens. They arelocated in the 6Long Treatment room Pyxis.Infected: Treat the patient with systemic antibiotics (through the line) to “sterilize” the line ifpossible. Remove the line, after discussion with the chief resident or attending, if the patient is inseptic shock, the infection persists for > 3 days, a tunnel infection is present, or if the gram stainis positive for yeast, fungi, or AFB.

Central Lines/Broviacs/PortsIn general, we place lines in the operating room with ultrasound and fluoroscopy. Occasionally, wewill place them at the bedside in a NICU or PICU patient.

Central lines are percutaneously placed, using seldinger technique and are held in place withsutures. Removal does not need a trip to the OR.Broviacs are tunneled catheters that can be left in place long term. Initially, a nylon suture is usedto keep them in place, but ultimately, they are held in place by tissue ingrowth around asubcutaneous cuff. Removal usually requires a trip to the OR or at least sedation in the NICU orPICU.Ports are completely subcutaneous devices that are connected to a subcutaneously tunneledcentral catheter. These are all placed in the OR and removal requires a trip to the OR.

Appendix

Colonic irrigation with normal salineFor treatment of Hirschsprung’s Enterocololitis

ManagementFECES, solid or liquid, cannot be sterilized. Abnormalities of the colon, such as Hirschsprung’sDisease (HD) can lead to chronic constipation, megacolon, fecal stasis and eventually bacterialovergrowth and enterocolitis. If not treated promptly, and adequately, the infant/child withenterocolitis can rapidly deteriorate into septic shock. This may occur preoperatively in the proximalmegacolon or postoperatively in the pulled-through colon.

Mainstays of treatment of Hirschsprung’s enterocolitis are NPO, NGT, IVFs, and Metronidazole.

Always remember TUBES:

Nasogastric TUBE for gastric decompressionUrethral TUBE for monitoring urine outputCentral venous TUBE for rapid IV Fluid and Electrolyte resuscitationTrans-anal Colonic TUBE for Colonic Decompression/Irrigation

Colonic irrigation may be used in the hospital as part of the treatment of HD enterocolitis. It may alsobe used at home for patients with abdominal distention in the following circumstances:

Abdominal distension occurring 4-6 weeks post-pull through, AFTER BOWEL MOVEMENTSHAVE BEEN WELL ESTABLISED.

Colonic irrigation with normal saline solution Irrigations should only be initiated and continued under the direct supervision of the attendingsurgeon. Indications are abdominal distention due to retained liquid stool and air.

PreparationPrior to starting, evaluate for fecal impaction by digital rectal exam. If present, disimpaction undergeneral anesthesia may be necessary.

SuppliesSilicone Foley catheter the size of the child’s thumb (16 Fr. for children under one year of age toa maximum of 24 Fr. for children over one year of age). It may be necessary to put additionalholes on the side of the catheter. The Foley balloon is NEVER inflated. DO NOT USE REDRUBBER CATHETERS. They are too stiff and lumen is too small vis a vis the outside diameter.

Use a 60ml catheter tip syringe.Normal Saline (NS).2 basins (or equivalent) and a large plastic bucket.Water soluble lubricant

Standard Procedure1. Lubricate catheter and gently insert 10-12cm into the rectum (10cm for children less than 1 yr of

age and 12cm for children more than 1 yr of age).2. The tip of the catheter is in the right place when there is a gush of air/liquid stool through the

catheter.3. DO NOT INFLATE THE FOLEY BALLOON.4. Test the catheter position by pushing/pulling with the plunger of the syringe. Air should freely

move in and out of the syringe. If this does not occur, pull back on the catheter and test again untilair moves freely.

5. If air does not move freely, discontinue the procedure and consult with the attending physician.6. Fill the clean basin with NS.7. Aspirate 20 ml NS from the clean basin with the syringe and irrigate the catheter.8. Pull back on the plunger to allow the stool and saline to return. If there is no return flow,

disconnect the syringe from the catheter and allow fluid to drip into the dirty basin. If return flowis slow, remove the catheter, clear the holes of stool and reinsert the catheter.

9. If drainage is unsatisfactory, discontinue the procedure and consult with the attending physician. 10. If drainage is satisfactory, you may proceed.11. Repeat the process making sure the volume of fluid drained is greater than or equal to the volume

of fluid introduced. By the time the clean basin is empty of NS, the soiled basin should be filledwith an equivalent volume of effluent.

12. Refill clean basin with NS and empty dirty basin into the large plastic bucket. 13. Continue irrigation until return flow is clear.

This treatment may be repeated as ordered by the attending physician.

Anal DilationsOrder anal dilator set to send home with family. Instruct family to bring dilator set to first clinicappointment, where they will be taught how to start the dilations

Anal Dilation scheduleDilators given to family at first post op clinic appt:

See in clinic q2wk as increasing dilations

1. Continue the dilations, at home, with a size_______ dilator. 2. Use twice daily, for one week.

3. Every week, change to a dilator that is one size larger. 4. Use twice daily for one week.5. Stop increasing the size once you get to a _______ dilator.

Tapering scheduleStart tapering 1 month after goal dilation; See in clinic Q month while tapering

1. Continue twice daily dilations with a size_______ dilator for 2 weeks.2. Decrease frequency to once daily for 2 weeks.3. Decrease to every Monday, Wednesday and Friday for 2 weeks.4. Decease to once a week for 2 weeks.5. Rectal exam in clinic prior to stopping dilations6. Stop dilations.*

*Regular follow up is recommended for bowel management of children with Imperforate Anus andHirschsprung’s Disease in our LIFE clinic.

Guidelines for sizing1-4 Months #12 4-8 Months #13 8-12 Months #14 1-3 Years #15 3-12 Years #16 > 12 years #17

Nasogastric tubesSmallest sump tube is 6 Fr Replogle

6- 10 Fr for neonatesReplogle tube preferred in infants because the two holes are near the tip of the tubeThe air vent on the sump tube will reduce the likelihood of occlusion of the tube—commonproblem in small suction tubes

The approximate size of an NGT is the size of the child’s forefinger.

If a bigger tube is needed, insert an OGT The approximate size of an OGT is the size of the child’sthumb.

Gastrostomy TubesThere are many types of g-tubes

Low profileLie flush with the abdominal wall, need to be attached to a tube for feeds and drainageBard button and microvasive—no balloon Mic-key, Ross, AMT minibutton--inflates with a balloon

G tubes with outer tubesFoley catheter (cut off the rounded tip)Peg tube—flanged tubeMic tubes-inflatable balloon (5, 20 ml)Malecot - flanged tube (you will need a butterfly introducer; an anal fistula probe can also beused)

Guidelines for Replacing a G-tubeThe most important fact to know before replacing a G-tube is how long ago it was placed and ifplaced open, laparoscopically, or PEG (by Internist – possibility of bowel between the stomach andabdominal wall)

1. If the g-tube was placed 2 weeks to 2 months ago, tube can be replaced and a g tube study may benecessary.

2. If the g-tube was placed less than 2 weeks ago, notify senior resident/attending, tube may need tobe replaced operatively.

3. If there is any question after replacing a G-tube, obtain a G-tube study (use water soluble radio-opaque dye)

Leaking G-tubesLeaking G-tubes may be addressed as follows:

1. Place more fluid in the balloon.2. Secure G-tube in place with nipple or tape under a little tension. If these do not work call the

surgical nurse before doing #3.3. Replace with larger G-tube.4. Remove G-tube for 24 hrs, let tract close down, and then replace

The surgical nurse or attending should be consulted before attempting these maneuvers.

Infant FormulasBreast milk: 20 kcal/ozInfant Formulas: 20 kcal/oz

Cow’s milk-based: Enfamil, Similac, Carnation Good Start, lactofree, similacSoy based: Prosobee, Isomil, Alsoy

Special Needs formulas:Hydrolyzed casein: Nutramigen (vegetable oil)

Hydrolyzed casein: Alimentum, Pregestimil (MCT oil)MCT oil based: PortagenSevere protein allergy: Neocate (free amino acid)GERD: Enfamil AR (rice starch thickener)

Pediatric formulas:Pediasure and Pediasure with FiberKindercalResource Just for kidsCompleat Pediatric

Elemental and Semi-elemental: For children > 1 year of age with malabsorption, SBS, multiplefood allergies, Crohn’s

Pediatric VivonexNeocate 1+Peptamen Jr.

Muscle biopsy protocolMuscle biopsies are frequently performed by our service. Preferred sample size is 0.5 x 0.5 x 2 cm.Biopsies must be received by the pathology department before 4:30 pm on weekdays. Specimensshould be left on gauze which is dampened with saline and wrung out, then placed in a plastic screw-top specimen container with a label.

If muscle biopsy is needed for a neuro-muscular problem, biopsy should be done under localanaesthesia

Page neuropathology fellow with questions: 443-2693

Skin biopsy protocolRequests made by genetics for skin biopsy for analysis should include: transport media (DME),clinical lab slip, and chromosome analysis form filled out by primary team. Skin biopsy is sent to the5th floor Moffitt clinical lab.

Hemophilia patientsPreoperative medications are usually ordered by the hematology service. Replacement guidelines areas follows:

Target plasma level factor VIII/IX

80-100% for major procedures50% for minor procedures/postop maintenanceInitial dose for life-threatening bleeding 45-50 units/kg IV FVIIIEach unit Factor VIII concentrate raises level approximately 2%

Vital SignsAge Weight Heart rate Resp rate BP (sys)

Premie 1 kg 145 bpm <40 42 ± 10

Newborn 2-3 125 60 ± 10

1 month 4 120 24-35 80 ± 15

6 months 7 130 89 ± 29

1 year 10 130 20-30 96 ± 30

2-3 years 12-14 120 99 ± 25

4-5 years 16-18 100 99 ± 20

6-8 years 20-26 100 12-25 105 ± 13

10-12 years 32-42 75 112 ± 19

> 14 years >50 70 12-18 120 ± 20

Conditions & Procedures

Ovarian Cysts A. Etiology

1. Neonatal: Maternal hormonal stimulation in-utero can result in a cyst on the fetal ovary2. Prepubertal: enlargement of cystic follicle; rare because gonadotropin stimulation of the

ovary decreases in infancy and early childhood and increases with the onset of puberty3. Postpubertal: failure of the maturing follicle to ovulate and involute

B. Epidemiology 1. Uncommon between the neonatal period and puberty2. Common between menarche and 18 years of age

C. Clinical presentation1. Prenatal: prenatal ultrasound2. Neonatal: Pelvic mass 3. Prepubertal- can be hormonally active and result in precocious pseudopuberty (McCune-

Albright syndrome)- premature vaginal bleeding, abdominal mass or increasing abd girth,feeling of bloating or abd fullness, urinary retention or frequency

4. Postpubertal – can by asymptomatic or cause menstrual irregularities, pelvic pain, or if large,can cause constipation, urinary frequency, and pelvic heaviness

5. Rupture: intraabdominal pain and bleeding (range from minor to severe)D. Workup

1. Pelvic ultrasound2. Detailed menstrual and sexual history3. Pregnancy test4. CBC

E. Treatment1. If simple, small and detected as incidental finding can be observantly managed. 2. If they are symptomatic, greater than 3-5 cm in diameter, or have solid components or

septations, resection with ovarian sparing is initial surgical therapyF. Outcome

1. Pelvic ultrasound in 3 months with clinic appt2. No further follow-up needed

Ovarian TorsionA. Etiology

1. Can occur with ovarian cyst of any size and normal ovaries 2. since ovary descends to the true pelvis by puberty, the ovary is an abdominal organ

susceptible to torsion during the prepubertal periodB. Clinical presentation

1. Lower abd pain of sudden onset, nausea, vomiting, low grade fever, pallor, leukocytosis(with left shift), followed by less severe localized pain

2. several presentations of abd pain:3. chronic aching abdominal pain (periumbilical or lower quadrant) 4. acute severe pain simulating appendicitis or peritonitis 5. intermittent pain from intermittent torsion

C. Workup1. Pelvic ultrasound- size discrepancy in ovarian volumes with classic peripheralization of

follicles2. Doppler ultrasound – to eval blood flow to ovary 3. CT or MRI – have been used but value of these have not been established

D. Treatment1. Surgery at time of diagnosis to salvage ovary by untwisting vascular pedicle

E. Outcome/follow-up1. Ultrasound in 3 months with clinic appt2. No further follow up needed

CCAM/CPAM/SequestrationA. Type

1. Pulmonary sequestration – a non-communicating mass comprised of lung parenchyma, withinthe lung (intralobar) or outside of the lung (extralobar). May be found above, below or rarelywithin diaphragm. Distinguished by an abnormal arterial blood supply.

2. Congenital cystic adenomatoid malformation/congenital pulmonary airway malformation – asolid or cystic lung mass comprised of an overgrowth of bronchiolar tissue, immature alveoliand abnormal development of connective tissue and smooth muscle that does not functionnormally. Stratified into types I-IV.

B. Etiology - Uncertain but thought to be a congenital, not acquired, malformation of the foregutduring the first weeks of fetal development.

C. Epidemiology - Rare, approximately 1:30,000 live births.D. Associated Anomalies - May be found in patients with other anomalies such as Congenital

Diaphragmatic Hernia. E. Clinical presentation

1. Fetal diagnosis by ultrasound2. Patients are often asymptomatic3. Respiratory distress in the newborn period is possible but rare4. Pneumonia5. Asthma-like symptoms6. CHF for patients with large sequestration

F. Work up – CT scan of the chest with IV contrast performed between 3-6 months of life.G. Treatment

1. No intervention2. Surgical resection of mass - open or thoracoscopic

3. Hospital stay – approximately 72 hoursH. Outcome

1. Pathology results2. Annual follow up in LIFE clinic3. Non surgical outcomes may include: Pneumonia, asthma, malignant degeneration, CHF

possible with sequestration. 4. Postsurgical outcomes may include: Pneumonia, asthma, scoliosis, pectus deformities.

AppendicitisAppendicitis is by far the most common abdominal surgical problem in childhood. The diagnosis ofappendicitis can be quite difficult in children.

Intermittent abdominal pain turning to persistent; increasing in intensity; and epigastric painshifting to a specific quadrant, should make you suspect appendicitis.Localized and reproducible tenderness in a specific quadrant is the cardinal reliable sign ofappendicitis. Due to the rotation of the intestines in the fetus, the cecum and appendix may be inany of the 4 quadrants. Appendicitis may be accompanied by anorexia, nausea, and vomiting, but these are notdiscriminating signs. Conversely, a hungry child may mean it is not appendicitis. Diarrhea may bepresent with retrocecal appendicitis.The younger the patient, the more likely that rupture and peritonitis will be present on admission.Additional imaging by US or fine cut CT scan may be necessary.IV Zosyn should be initiated once the diagnosis is confirmed (use IV Gentamicin/Clindamycin ifallergic).(If allergic use Cipro/Flagyl)Be aware of appendicitis in any in patient admitted for another diagnosis who developsabdominal pain

Perforated Appendicitis Rare non-operative management dictated by surgeons

Post-operative managementNPO, IVF (D51/2 w/ 20 meq KCL/L)IV Zosyn until tolerating POif suspect post op ileus, consider TPN/PPNDiet advanced per surgeonobtain WBC when pt is afebrile x 24 hrs

Discharge criteriaAfebrile >24 hrs, normal WBC with no left shift, tolerating PO, pain managed with oral painmeds, ambulating with minimal pain

Discharge PlansOral Pain management

Augmentin x 7 daysReturn to clinic in 2 weeks

Inguinal herniaThe incarcerated inguinal hernia is a common emergency room/inpatient consultation. Incarceratedinguinal hernias occur most often in infants during the first year of life. Most, if not all, can bereduced manually, which obviates the need for emergency surgery.

Reduction techniquesOccasionally, holding the baby in very steep Trendelenburg reduces the hernia, due to the pull ofthe mesentery. The hernia will be nearly impossible to reduce in a crying child. If sedation is necessary, obtainassistance from the ER staff.Fingers of one hand should fix the internal ring while the other should put gentle ‘downwardtraction’ on mass and squeeze the contents (imagine squeezing out the contents of a sausage) untilit squirts back through the ring.In females, when the ovary is palpated in the sac, minimal manipulation can reduce it even if ithas been present for several days. It can be exceedingly difficult to distinguish between an ovaryand a lymph node unless the lymph node is lateral to the external ring.Emergency surgical intervention is required if the hernia cannot be reduced, or if there is post-reduction evidence of persistent intestinal obstruction, or nonviable bowel/testicle/ovary.It can be exceedingly difficult to distinguish between an incarcerated inguinal hernia and a cordhydrocele. Transillumination may help. A rectal exam will allow palpation of the inside of theabdominal wall at the level of the internal ring.

Foreign body in airway/esophagusGet a PA and lateral x-ray of the neck and chest

Position of coin on CXray/neck film tells location-esophagus or trachea

GI foreign bodiesCoins are the most common esophageal foreign body.If the coin is in the proximal to mid esophagus, it is removed in OR with esophagoscopy.If the coin is in stomach or intestine, generally, the FB will pass on its own.Frequently, patients with esophageal foreign bodies get discharged home from PACU postremoval.Patients with long-standing airway foreign bodies or copious airway secretions will stay in thehospital for observation.Difficult esophageal foreign body removals or esophageal dilations may require a bariumswallow- this is done at the discretion of the attending surgeon.

Check with attending about getting a CXR postoperatively on esophageal foreign bodies.

Airway foreign bodiesPeanuts are the most common airway foreign body.If foreign body in trachea and pt is talking and stable, pt goes to OR, NPO period will bediscussed with anesthesia team.If patient is not talking or in acute distress, perform Heimlich maneuver.Airway foreign bodies are generally admitted for observation.All airway foreign bodies should get CXR postoperatively.

Discharge parents should be instructed to bring the child back for the following:

High spiking feversS.O.B.Refusal to swallowSevere irritability

GERDGastroesophageal reflux disease is one of the most common consults. Patients need a UGI as the bareminimum for a workup of fundoplication. The UGI is not to determine reflux but to look for anatomicanomalies that may be contributing to reflux (e.g. pyloric stenosis, duodenal web, etc.).

Additionally, a pH probe is usually needed to assess the degree of reflux. This should be done off ofGI meds. Occasionally, in lieu of a pH probe, other tests may determine significant reflux. Abronchoalveolar lavage that shows lipid-laden macrophages is nearly pathognomonic for GERD.Tube feeds that have been marked with dye that shows up in tracheal aspirates may also be sufficientcause for a fundoplication. Additionally, a neurologically impaired child who needs a G-tube willfrequently require a fundoplication.

Laparoscopic fundoplication and G-tube A. Pre Op: obtain UGI with limited SBFT B. If the patient is in house: Check NPO guidelines C. Post-op orders laparoscopic nissen + G-tube

1. NPO, G-tube to straight drain overnightD. Start clears via GT bolus/drip feeds

1. POD #1 if patient is not distended 2. Begin tube feeds later on POD #1 with advancement to full feeds by next AM

E. Post-op orders, laparoscopic nissen1. Clears postoperatively if not distended2. Post-nissen diet POD # 2

Post fundoplication Diet (first 3 months post-op)A. 1st 2 weeks: liquids only

1. avoid carbonated beverages or gum 2. (pt may not be able to burp or vomit- do not allow carbonated beverages or gum until pt able

to burp) 3. ex: water, milk, milkshakes, soup, jello, pudding, pureed baby food, yogurt

B. 3rd and 4th weeks: soft foods1. ex: mashed potatoes, pasta, rice, bread, fish, crackers, cereal, ground beef, cheese, peanut

butterC. Next 2 months: can resume his or her normal diet with some exceptions

1. Avoid choking hazard foods: hotdogs, steak, chicken, pizza, nuts

Medscan stop reglan or ranitidine right after operationProton-pump inhibitor – continue for 1 week at ½ dose, then 1 more week at ¼ orig dose, thenstop

Other ordersNutrition consult G-tube teaching per surgical nurseGI referral for outpatient appointmentG-tube supplies for homeEmergency supplies need to be placed at bedside post-op: foleys (one same size fr as GT and onesmaller), lubricant, extra GT of same size, 5ml syringe, tape, blue Kelly clamp)MIC-Key GT and Malecot GT will be first changed 2 months post-op Hollister Drain tube Holder device PMM# 2078 or bottle nipple with tip cut off is used to holdmalecot GT in place.

Sickle Cell PatientsAdmit day before surgery (these patients will be admitted to the hematology service)

hydrate 1.5x maintenanceCBCtransfuse slowly to reach a goal Hct to 30 (do not want too much higher as a much higher Hctincreases blood viscosity)post op

pain controlpulmonary toiletincentive spirometrychest physiotherapy

Pre-Op Splenectomy14 days prior administer vaccines: (if unable, administer vaccines after POD 14)

pneumovaxmeningo vax- depending upon agehflu vax- depending upon age

S/p splenectomy OPSS prophylaxis

125 mg pcn V bid for children < 6 years old250 mg pcn V bid 6 years or older

Scrotal painDifferential dx

Testicular torsionEpididimytisTorsion appendix testis

Ways to differentiate

cremasteric reflex present- unlikely to be testicular torsion

Torsion, high lying in scrotum

<12 y/o unlikely testicular torsion (except newborn, in which case, it is bilateral; bell-clappertype of testes)<12 y/o unlikely epididymitis

Epididymitis, low lying in scrotum

relief of pain when testicle is liftedblue dot = torsion appendix testisvomiting is more common with testicular torsiontesticular torsion is usually< 12hrs duration

Treatment

Testicular torsion is a surgical emergency, the other two are not.Epididymitis is treated with antibiotics and analgesics.Torsion of the appendix testis is treated with NSAIDS +/- antibiotics.If the diagnosis is unclear then order a Doppler ultrasound for vascular compromise or go to theOR for scrotal exploration.

Hypertrophic Pyloric Stenosis

Medical Emergency – Not Surgical Emergency

A. Etiology - unknown1. Lack of NO synthetase

B. Epidemiology1. Genetic predisposition (esp. male offspring of affected females)2. Male:Female = 2.5 - 5:13. 1-4 per 1000 live births in white (less in African Americans and Asians)

C. Associated anomalies - noneD. Clinical presentation

1. 3-6 wks of age; rare at birth2. “Projectile”, non-bilious emesis

E. EvaluationF. Palpable “olive” in RUQ

1. How to examine:a. Place an ogt 10-12fr anderson tube, empty stomachb. Put sugar (sweeties) on pacifierc. Bend baby’s kneesd. Examine with warm handse. Be patient!

2. Hypochloremic, hypokalemic metabolic alkalosis3. US (> 4 mm thick and > 16 mm long) for term infants4. UGI (when US negative or questionable)

G. Treatment1. NPO preop/No NG tube2. Correct alkalosis and dehydration3. Decreases postop apneic spells4. Bolus with 20 ml/kg of NS if Cl < 100 or HCO3 > 305. Start D5 1/2 NS with 20 meq KCl/L at 1.5 x maintenance6. To OR only when HCO3 < 307. Pyloromyotomy (Fredet-Ramstedt)

a. laparoscopic vs. open 8. IV Cefazolin x 1 in OR

H. Outcome1. Complications

a. Perforation of mucosa - 1° repair with omental patch; delay feeds 24 hoursb. Incomplete myotomy - rarec. Post-op emesis - common (dysmotility); resolves in a few days

2. Survival - 100% Pyloric Stenosis

Pyloric Stenosis Post-op feeding schedulesContinous pulse oximetry postop for floor patients!!

NPO until 6 hours post op.Feed pedialyte 1 oz initially, patients may feed ad lib if tolerates pedialyte May substitute breastfeeding/breastmilk otherwise no change in formulaNo NPO after emesis but repeat same feed/volumeIVF D5 1/4 NS +2meqKCL/100cc @maint.Narcotics are not required for postop pain, use tylenol Tylenol 10-15mg/kg/dose po/pr q 4h prn d/c IVF when tolerating 60cc po q 3hMust tolerate two full feeds without emesis prior to discharge

IntussusceptionA. Etiology

1. “Idiopathic”: 90% (Peyer’s patches) Age Incidence: 6-9 months2. Specific lead point: 10% Usually in small bowel

a. Age incidence: <2mos, >2yrsb. Inverted Meckel’s diverticulumc. Polyp (Peutz-Jegher’s syndrome)d. Duplication cyste. Hemorrhage: Henoch-Schonlein purpura, hemophilia, coagulopathyf. Lymphoma

B. Epidemiology - Most common site- ileocolic 1. Age: 6 mos–3 yrs; rare neonatal unless with specific lead point2. Incidence: 1-2 per 1000 live births; 3. Seasonal incidence: spring and summer- peak of enteritis; mid winter- peak of respiratory

tract infections.4. Male:female = 3:2

C. Associated conditions1. Lymphoma in > 4 year age group2. Post-operative intussusception from a variety of surgical procedures

D. Clinical Presentation: Ileo-cecal Intussusception:1. Colicky abdominal pain (may be completely pain free between episodes)2. Nonbilious (initially, as ileum enters cecum)3. Bilious emesis (as ileum progresses through colon)4. Passage of blood per rectum/hemoccult (+) stools “currant jelly stools”5. Palpable RUQ abdominal mass6. Profound lethargy

E. Clinical Presentation: Small Bowel Intussusception1. Colicky abdominal pain2. Early bilious vomiting3. May or may not have currant jelly stools4. May or may not have palpable abdominal mass

5. Profound lethargy; early necrosis of intussusceptumF. Work-up

1. KUB: May demonstrate RUQ mass2. US: target sign3. Contrast enema4. Barium5. Air

G. Treatment: Ileo-cecal Intussusception1. Enema reduction (ONLY if NO peritoneal signs)

a. Air: <120 cm H20 pressure (air pump with pressure gauge)b. Barium: Enema can 3 feet above tablec. Must see filling of several loops of small bowel

2. Surgicala. For failed enema reduction or presence of peritonitisb. Manual reduction: “Push don’t pull” (laparoscopic – pull)c. Resection with primary anastomosis

H. Outcome1. Complications

a. Recurrence: with enema reduction - 5-7%b. Recurrence: with surgical reduction < 5%c. Perforation during attempted reduction: 1-2%

2. Survival: 99%a. Death from delay in diagnosis

Pectus Excavatum Sunken chestA. Incidence

1. The most common congenital chest wall deformity occurring in 1:300 live births with a 3:1male predominance

2. Familial associationB. Associated anomalies

1. Musculosketal disorders including Marfan’s and Poland Syndrome,2. Cardiovasular disease

C. Clinical presentation1. Unbalanced posterior growth of costal cartilage displacing and often rotating sternum.2. Inferior portion of sternum most severely affected. 3. Severity varies, may be present at birth but commonly develops during rapid growth in

adolescence.4. Subjects may report chest pain, SOB with exertion

D. Work up1. CT scan or chest radiographs for Pectus Severity Index (also known as Haller index; usually

>3.25 is an indication for surgery).2. Cardiac echo for anomalies, as indicated

3. R/o Marfan’s disease, if suspected4. Pulmonary function tests, as indicated.

E. Treatment: Treatment is elective 1. Post puberty

a. Open cartilage resection (Ravitch- rare)b. Pectus bar placement (Nuss- most common)

2. Prepuberty - 3MP clinical trial using magnetsF. Outcome

1. Post operatively monitor for entrained air, seroma, or very rarely pericarditis 2. Remove pectus bar at 2-3 years3. Post surgical recurrence possible following both standard repairs.

Pectus Excavatum Post-Operative Pain Protocol

POD1 2 3 4 5 6

x x Epidural, wean POD 3, painservice

x x x Toradol IV, painservice

x x x Morphine IV, painservice

x x x x x Lortab/Vicodin/Norco/Percocet

x x Ibuprofen

x x x x Miralax (also at d/c)

x x x x Senna

D/C on oral analgesic

Obtain PA and lateral CXR prior to d/c

Pectus Carinatum Pidgeon chest A. Incidence

1. Less common than pectus excavatum.2. Male predominance3. Familial association

B. Associated anomalies1. Musculosketal disorders such as Marfan’s and Poland Syndrome,

C. Clinical presentation1. Unbalanced anterior growth of costal cartilage displacing and often rotating sternum.2. Severity varies, may be present in childhood but commonly develops during rapid growth in

adolescence.D. Treatment

1. Elective surgical resection of malformed cartilage – generally offered post puberty

2. Bracing, may begin early in childhood E. Outcome

1. Post operatively recurrence is rare2. Bracing may be unsuccessful, if offered after puberty or wear time is minimal.

Childhood Tumors - General Considerations

IncidenceA. Cancer is 2nd leading cause of death in children older than 1 year of age. (Accidents 1st)B. Cancer affects 12,500 children per year in US. (170 new cases/106 children/year in US)

OverviewA. Most common pediatric malignancy: Leukemia (ALL > AML)B. Most common solid pediatric malignancy: Brain tumor (astrocytoma most common)C. Most common solid extra cranial (para-vertebral - sympathetic ganglia) pediatric malignancy:

NeuroblastomaD. Most common abdominal pediatric malignancy: Wilms’ tumor (< 6mos—congenital mesoblastic

nephroma)E. Most common newborn malignancy: Neuroblastoma (adrenal medulla)F. Most common pediatric soft tissue malignancy: RhabdomyosarcomaG. Most common pediatric liver malignancy: HepatoblastomaH. Most common pediatric mediastinal malignancy: LymphomaI. Most common site for abdominal Burkitt’s lymphoma: ileocecal areaJ. Most common pediatric germ cell tumor: Sacrococcygeal teratoma

K. Incidence of Neuroblastoma: 1 in 8,000 - 10,000; 700 new cases/yearL. Incidence of Wilms’ tumor: 1 in 10,000; 500 new cases/year

M. Most common testicular tumor of childhood: Yolk sac tumor (endodermal sinus tumor)

NeuroblastomaA. Cell of origin/Histology

1. Neural crest cells (sympathetic nervous system; adrenal medulla)2. Ganglioneuroma, ganglioneuroblastoma, neuroblastoma3. Genetics: deletion at 1p36 or 11q14-23; ALK mutation4. Homer-Wright pseudorosettes (neuropil surrounded by neuroblasts)

B. Signs, symptoms, associated syndromes1. Hard, irregular, fixed lateral or midline abdominal mass (adrenal medulla or sympathetic

chain)2. Neurological dysfunction (extension through vertebral foramina; “dumb-bell” tumor)3. Opsomyoclonus (“dancing eyes and prancing feet”) - ataxia and random eye movements

4. “Panda eyes” - retro-orbital mets5. Intractable secretory diarrhea (VIP)6. Hypertension - renovascular or catecholamine mediated7. Constitutional symptoms: weight loss, malaise, fever8. Bone pain, limp (bony cortical mets)9. Neurocristopathy: NBL, Hirschsprung’s disease, central hypoventilation syndrome (Ondine’s

curse)10. Horner’s syndrome (stellate ganglion)11. BWS (Beckwith-Wiedemann Syndrome; EMG- Exomphalus, Macroglossia, Gigantism

Syndrome)C. Work-up

1. Urinary catecholamines (VMA, HVA, etc.)2. KUB: stippled calcifications; CXR: posterior mediastinal mass3. CT: abdominal, chest, pelvic, neck depending upon location4. Spinal MRI: R/O intra-spinal extension (“dumb-bell tumor”)5. Skeletal survey (bony cortical mets)6. Bone scan: R/O mets (bony cortical mets)7. Bone marrow aspiration and biopsy: R/O mets (BM mets not seen on skeletal survey nor

bone scan)8. MIBG: taken up by mets and primary

D. Markers1. Urinary and serum catecholamines (VMA, HVA, dopamine, epinephrine, norepinephrine,

etc.)2. Ferritin (increased = bad)3. Neuron-specific enolase (NSE) (increased = bad)4. LDH (increased = bad)

E. Stages1. International Neuroblastoma Staging System (INSS)

1: Complete gross excision; nodes neg.2a: Unilateral tumor with incomplete gross excision; nodes neg.2b: Unilateral tumor with complete or incomplete gross excision with pos. ipsilateral andneg. contralateral nodes3: Unilateral tumor with pos. contralateral nodes; tumor infiltrating across midline (toopposite side of spine) with or without pos. nodes4: Metastatic4S: Stage 1 or 2 with remote disease confined to liver, skin, bone marrow (NOT cortex—cortex makes it stage 4); less than 18 months old.

2. Staging done on exploratory laparotomy except 4SF. Treatment

1. Surgery; In stage 4S, primary tumor (usually adrenal gland) is resected; no need to debulkliver mets.a. Resect if possible, otherwise biopsy and stageb. Debulking not beneficial initially (maybe considered after chemotherapy).

c. Definite role for “2nd look” resection after chemotherapy (need over 95% gross totalresection).

2. Chemotherapya. Mainstay of treating advanced disease (or disease after 18 months of age)b. Main agents are carboplatin, cyclophosphamide, doxorubicin, etoposide +/- ifosfamide,

vincristine, cisplatinc. NO CHEMO for completely excised, low-risk stage 1, 2, or 4S

3. Radiotherapya. NBL variably radiosensitiveb. Used for local control, bone painc. IORT (intra-operative radiation therapy) may have a role in select cases.

4. Bone marrow transplantation (or stem cell)a. For “rescue” following marrow-ablative, intensified chemotherapyb. Questionable benefit in improving long-term survival

G. Prognostic Factors1. Age: Younger better (less than 1y)2. Stage: 1, 2, 4S, 3, 4; prognosis of stage 4S is the same as Stage 1, 23. Bone cortex mets: Worst (seen on skeletal survey or bone scan); BM mets seen on aspiration

or biopsy not prognosticated as stage 4.4. Location: chest, neck better than abdomen5. HVA/VMA ratio > 1: better6. Ferritin, NSE, LDH: elevated = bad7. N-myc amplification: defined as 4-fold increase; BAD; significant prognostic factor in NBL8. DNA index (DI)

a. DI = 1 (diploid): badb. DI > 1 (hyperdiploid): good

H. Results1. “Low risk”: > 90% cure

a. INSS 1, 2, 4S b. Age < 1 yearc. No N-myc amplficationd. DI > 1.25

2. “Intermediate risk”: 70-90% curea. INSS 3 or 4b. Age > 1 yearc. No N-myc amplificationd. Low serum ferritin and NSEe. Near diploid (DI 1.0)

3. “High risk”: 10-40% cure a. INSS 3 or 4 Age > 18 monthsb. N-myc amplifiedc. Near diploid

d. High serum ferritin

Wilms TumorA. Cell of origin/Histology

1. Blastema (fetal renal tissue)2. Triphasic histology

a. Blastemab. Epithelial elements (tubular)c. Stroma (muscle, fat, cartilage)

3. Geneticsa. WT1 gene: short arm of chromosome 11 (11p13); associated with WAGR syndrome

(Wilms, Aniridia,Genital anomalies, Retardation)b. WT2 gene: short arm of chromosome 11 (11p15.5); associated with Beckwith-

Wiedemann syndrome [BWS: EMG – Exomphalus, Macroglossia, Gigantism (organhypertrophy) omphalocele; renal, pancreatic, liver hypertrophy; macroglossia; hemi-hypertrophy)

c. 16q and 1p: worse relapse and overall survival.d. Others: CACNA1E, 7p21, SKCG-1, p53, FBXW7, MYCN.

B. Signs, symptoms, associated syndromes1. Smooth, firm, movable, flank to abdominal mass; may cross midline2. Hypertension (renovascular; renin): 25-50%3. Aniridia (sporadic): 1%4. BWS (EMG): 10-20% develop Wilms tumor5. Hemi-hypertrophy: 3%6. GU anomalies: 4-5%7. WAGR syndrome: up to 50% will develop Wilms

C. Work-up1. NWTS protocol (National Wilms Tumor Study)2. Renal US (R/O IVC extension)3. Abdominal, chest CT scans4. CXR (staging based on CXR, not CT)5. If cytology is “clear cell sarcoma”: bone scan and skeletal survey and brain MRI6. If cytology is “rhabdoid”: brain MRI

D. Markers - none other than geneticE. Stages

I (43%): Limited to kidney and completely excisedII (20%): Extends through capsule or into extra-parenchymal renal vessels but completelyexcisedIII (21%): Residual nonhematogenous tumor in the abdomen (pos. nodes, spillage, peritonealimplants, incomplete excision, biopsy only, intrathoracic IVC extension)IV (11%): Hematogenous mets to lung, liver, bones, brain, distant lymph nodesV (5%): Bilateral when diagnosed

F. Treatment1. Surgery

a. Transperitoneal exploration, radical nephrectomy, node bx, visualize and palpatecontralateral tumor, biopsy suspicious areas

b. Bilateral Wilms: Biopsy only initiallyc. Unresectable: Biopsy; stage

2. Chemotherapy (NWTS)a. Primary agents: Actinomycin D and vincristineb. ALL patients require chemotx, based on Stages and histology of the tumor (“Favorable”

vs. “Unfavorable”)c. Additional agents for advanced stage or unfavorable histology: doxorubicin,

cyclophosphamide, etoposide (VP-16).3. Radiotherapy

a. None for Stage I or II with favorable histology or focal anaplasiab. RT for Stage II with diffuse anaplasia, Stage III, Stage IV, and all clear-cell sarcoma

patients (total abdominal (III) or total abdominal plus total lung (IV))G. Prognostic factors

1. Histology (Favorable vs. Unfavorable (anaplasia))2. Stage (I, II, V, III, IV)

H. Survival1. Stage I, II, III FH: 90-98%2. Stage IV FH: 90%3. Unfavorable histology: 73%

RhabdomyosarcomaA. Cell of origin/Histology

1. Arises from embryonic mesenchyme2. Major subtypes

a. Embryonal (1) Botryoid (“cluster of grapes”); gross appearance when tumor is in thelumen of a tube; ie, vagina/uterus, bile ducts/gall bladder, urinary bladder

b. Alveolar; histologic appearance looks like lung alveoli c. Pleomorphicd. Spindle-celle. Undifferentiated

B. Signs, symptoms, associated syndromes1. Variable, depending upon site of origin2. Head and neck (orbit, parameningeal, nonparameningeal):

a. masses or interruption of normal function3. Genitourinary

a. Bladder/prostate (B/P): urinary obstructionb. Non-bladder/prostate: paratesticular, perineum, vagina, uterus (mass, vaginal bleeding)

4. Extremity: painless mass or adenopathy

C. Work-up1. CT and/or MRI of primary tumorsite2. Chest CT, bone marrow aspiration and biopsy, skeletal survey,bone scan: R/O mets. (bony

cortical mets)3. LP for parameningeal 1° 4. Open incisional biopsy

D. Markers - none specificE. Stages and Groups (IRS)

1. Clinical Grouping based on pretreatment and operative outcomeGroup I: Localized disease, completely removed; nodes neg.Group II: Gross excision with microscopic residual or local nodes pos.Group III: Incomplete resection or bxGroup IV: Distant metastases

2. Staging takes into account location of tumor, tumor size, nodal status, and metastases.F. Treatment (IRS)

1. Surgery - General principlesa. Wide local excision and surrounding tissuesb. Preserve cosmesis/functional statusc. Mutilating excisions not indicatedd. Incomplete excision, debulking not indicatede. Incisional biopsy for unresectable lesionsf. Primary re-excision for incomplete resection (positive edges) on pathological

examinationg. Second-look operation following chemotherapy

2. Chemotherapya. VAC - vincristine, actinomycin D, cytoxanb. VAI - vincristine, actinomycin D, ifosfamidec. VIE - vincristine, ifosfamine, etoposide (VP-16)d. VadriaC - Vincristine, adriamycin, cytoxan

3. Radiation therapya. For local control of residual tumor

G. Treatment for Specific sites1. Head and neck

a. Orbit: chemotherapy +/- RTb. Parameningeal: RT + intrathecal and systemic chemotxc. Nonparameningeal: Excision

2. Bladder/prostate: Neoadjuvant chemotx +/- RT, then surgery3. Non-bladder/prostate (vagina,uterus, etc.): Neo-adjuvant chemotx +/- RT, then surgery4. Paratesticular: Inguinal orchiectomy with high ligation of cord

a. Routine retroperitoneal node bx or excision not indicated; indicated for clinical suspicion5. Extremities: Complete excision or primary re-excision with limb sparing plus node bx,

followed by chemo-tx according to stage

6. Trunk (chest wall abdominal wall, etc.): Complete wide local excision plus chemotx and/orRT as indicated by stage

H. Prognostic factors1. Histology

a. Favorable: Botryoid, spindle-cellb. Intermediate: Embryonal, pleomorphicc. Poor: Alveolar, undifferentiatedd. Ploidy: Diploid worse than hyperdiploid (same as for NBL)

2. Sitea. Favorable: Orbit, nonparameningeal head and neck; bladder/prostate, GU non-

bladder/prostateb. Unfavorable: Parameningeal, extremity, other

3. Clinical group I. Results (IRS: 5 year survival)

1. Clinical Group (Overall: 71%)I: 93%III: 73%II: 81%IV: 30%

2. Sitesa. Orbital: 95%b. Non-bladder/prostate: 89%c. Bladder/prostate: 81%d. Non-parameningeal: 78%e. Parameningeal: 74%f. Extremity: 74%g. Other: 67%

HepatoblastomaA. Cell of origin/Histology

1. Fetal liver cell2. Histological types

a. Pure fetal – best prognosisb. Embryonalc. Undifferentiated

3. Arises in normal liver (vs. abnormal liver for hepatocellular CA)B. Signs, symptoms, associated syndromes

1. Asymptomatic large abdominal mass in child < 3 years old; liver in NBT (IVS) bigger (olderfor hepatocellular CA)

2. BWS (EMG)3. Constitutional symptoms: weight loss, anorexia, pain

C. Work-up

1. CT, MRI, and/or US for primary (MRI with Eovist preferred) 2. CXR, chest CT: R/O mets3. Exploration for bx and staging or excision

D. Markers1. Alpha-fetoprotein (AFP): elevated in 90% (also increased in 50% of patients with

hepatocellular CA)E. Stages (determined following exploration)

I: Confined to liver; totally excisedII: Confined to liver; microscopic residualIII: Unresectable, partially resectable, nodes pos., rupture of capsuleIV: Distant mets (lung, brain)

F. Treatment1. Only chance for cure is TOTAL excision. Liver transplantation has been reported for

involvement of both lobes w/o distant mets.2. Cisplatin-based chemotherapy for all stages3. Other agents: vincristine, 5-FU, adriamycin4. Little role for RT

G. Prognostic factors1. Histology (fetal best prognosis)2. Stage3. Rising AFP indicates recurrent disease

H. ResultsStage I, II: 90%Stage III, IV, resected: 60-70%Stage III, IV, not resected: 0%Chemotherapy can convert nonresectable tumors into resectable ones in 80% of patients

Germ-Cell Tumors A. Cell of origin/Histology

1. Primordial germ cells (allantois region of embryonic yolk sac)2. Classification3. Gonadal

a. Seminoma, dysgerminoma4. Extragonadal

a. Teratoma - most common pediatric germ-cell tumor (sacro-coccygeal, most commonlocation)

b. Embryonal carcinomac. Chorio-carcinomad. Endodermal sinus tumor (yolk sac tumor) – most malignant

B. Signs, symptoms, associated syndromes1. Intersex disorders: Gonadoblastomas in 50%

2. Gonadal dysgenesis3. Testicular feminization syndrome (androgen insensitivity)4. Undescended testes: 30-50 x risk; seminoma most common; orchidopexy at any age does not

change the risk of malignancy)5. Mass at site of origin

C. Markers1. Alpha-fetoprotein (AFP): marker for endodermal sinus tumor (yolk sac)2. Beta-HCG: marker for chorio-carcinoma

D. Specific sites

Ovarian germ-cell tumorsA. Epidemiology

1. 2nd most common location for germ-cell tumor (sacrococcygeal 1st)2. Mature teratomas most common3. Malignancy more common in younger child (vs. teenager)

B. Stages (CCG-POG)I: Limited to ovary; markers return to normalII: Microscopic residual or pos. lymph node < 2 cmIII: Gross residual or bx only; pos. nodes > 2 cm; omentum or washings pos.; elevatedmarkersIV: Distant mets, including liver

C. Treatment1. Surgery

a. Collect ascites or peritoneal washingsb. Bx suspicious areas on peritoneumc. Unilateral salpingo-oophorectomyd. Wedge bx contralateral ovary if suspiciouse. Omentectomyf. Bilateral retroperitoneal node bx for suspicious nodes; not routineg. follow up will entail ultrasounds Q 3, 6, 9, 12 months x 2years then yearly. Beta HcG and

AFP levels with each U/S. 2. Chemotherapy: PEB

a. Agents: Cisplatin, Etopside (VP-16), Bleomycin

Testicular germ-cell tumorsA. Epidemiology

1. Endodermal sinus (yolk sac) tumors (most common testicular tumor of childhood) orteratomas

B. Stages (CCG - POG)I: Limited to testis; markers return to normalII: Transcrotal orchiectomy; microscopic residual; pos. nodes < 2 cm; increased markers

III: Nodes > 2 cm pos.IV: Distant mets, including liver

C. Treatment1. Inguinal orchiectomy2. Hemi-scrotectomy for Stage II because of scrotal bx3. Retroperitoneal node bx and debulking Stage III and IV (not formal retroperitoneal node

dissection)4. PEB chemotherapy for Stages II, III, IV, and I if markers remain elevated

Sacrococygeal TeratomaA. Epidemiology

1. Most common type of teratomaa. 40% of all germ cell tumors in childrenb. 80% of all extragonadal germ cell tumors

2. 1 in 35,000 live births3. Most benign in neonatal period; malignant if noted later (17% overall malignancy rate)

a. 50% mortality when diagnosed antenatally; 10% mortality when diagnosed postnatally4. Female:male = 3-4 : 1

B. Types (Altman)I: External (perineum) 47%II: External with intrapelvic extension (35%)III: Visible externally but predominantly internal including abdominal (supra-pubic) (9%);(“dumb-bell” type)IV: Entire internal (pelvic) (presacral) (9%)

C. Histological types1. Mature: Contains only mature “adult” tissue, usually from all three embryonic tissues

(endoderm, mesoderm, ectoderm)2. Immature: Contains some “fetal” tissue, most frequently neuro-ectodermal3. Malignant: Contains malignant tissue, usually of germ-cell origin (e.g., yolk sac/endodermal

sinus, etc.)D. Prognostic factors

1. Age: > 2 months, higher malignancy rate2. Type: III, IV, higher malignancy rate3. Sex: Males, higher malignancy rate

E. Workup1. Labs: AFP, bHCG2. Imaging: Abdominopelvic MRI with IV contrast

F. Treatment1. Complete resection including coccyx 2. PEB chemotherapy for malignancy

G. Complications1. Tumor rupture, bleeding

2. Intra-operative bleeding, coagulopathy3. Secure vessels transabdominally (internal iliacs and mid sacral artery) for giant tumors4. Apply tourniquet around base of tumor, wrap/compress tumor with gauze roll

H. Follow-up 1. Weekly AFP and bHCG levels until normalization, then every 3 months for 3 years, then

yearly until adulthood (co-followed by oncology Dr. Goldsby)2. Abd and pelvic MRI with and without contrast at 1 year post resection3. Yearly clinic visits with rectal exam, surveillance for symptoms of recurrence (vague

back/pelvic pain, urinary or stooling complaints) and cosmetic concerns related to surgicalincision

Hodgkin’s LymphomaA. Cell of origin/Histology

1. Hodgkin's (40%): Reed-Sternberg cell2. Lymphocyte predominance (3%); best prognosis3. Nodular sclerosis (67%)4. Mixed cellularity (25%)5. Lymphocyte depleted (5%); worst prognosis

B. Signs, symptoms, associated syndromes1. Asymptomatic cervical/mediastinal lymphadenopathy2. “B” symptoms3. Weight loss > 10% body weight4. Fever5. Night sweats6. Associated with Epstein-Barr virus in 30% of cases

C. Work-up1. CXR, chest and abdominal CT2. Lymphangiogram3. Staging laparotomy

a. Total splenectomyb. Liver bx, needle and wedge, right and left lobesc. Lymph node bx (celiac, porta hepatis, perisplenic, periaortic, mesenteric, right and left

iliac)d. Bone marrow bx

D. Markers - none specific1. Immunophenotypes used for classification

E. Stages: Hodgkin’s (Ann Arbor)I: Single node region or single extranodal site (IE)II: Two or more nodal regions on same side of diaphragm or single extranodal site plus oneor more lymphatic areas on same side of diaphragm (IIE)III: Both sides of diaphragm which could be accompanied by splenic involvement (IIIS),extranodal site (IIIE), or both (IIIES)

IV: Diffuse dissemination of one or more extranodal sites +/- node involvementA: No symptomsB: Fever, weight loss, night sweats

F. Treatmenta. Radiotherapy less frequently used in children as sole therapyb. Chemotherapy mainstay of therapy in children

1. Agents: MOPP (Mustard, vincristine (Oncovin), Prednisone, Procarbazine) ABVD(Adriamycin, Bleomycin, Vinblastine, Dacarbazine (DTIC))

c. Combination therapy used to decrease toxicity of single modality therapy (less RT)G. Prognostic Factors

a. Histologic type (see A)H. Results

1. Complications of therapy (RT and chemotx)a. Second malignancies (leukemia and solid malignancies -thyroid, breast)b. Cardiopulmonary damage

1. Pulmonary fibrosis, radiation pneumonitis (RT, bleomycin)2. Cardiomyopathy (adria)3. Coronary artery disease (RT)

c. Diminished height (RT)d. Infertility (MOPP in males, azoospermia; MOPP in females, ovarian failure)e. Complications of staging laparotomyf. Post-splenectomy sepsis (7% @ 15 years)g. SBO (10%)

I. Survivala. Localized: 90-95%b. Disseminated or recurrence: 75%

Hodgkin’s and Non-Hodgkin’s LymphomaA. Cell of origin/Histology:

1. Non-Hodgkin’s Lymphoma (NHL) (60%): B- and T- cells2. Large cell lymphoma (B- or T- cell): 15%3. Lymphoblastic lymphoma (T-cell): 30-40%4. Diffuse undifferentiated “DUL”; non-cleaved; “Burkitt’s” (B-cell): 50%

B. Signs, symptoms, associated syndromes1. Rapidly enlarging mediastinal mass with respiratory compromise, SVC syndrome, pleural

effusions, etc.2. Rapidly growing abdominal mass with bowel obstruction,ascites, ureteral obstruction, acute

renal failure, etc.3. Intussusception in child > 4 years old4. Incidental finding at laparotomy for “acute abdomen” (e.g. appendicitis)5. Mass in other areas of presentation (naso-pharynx,oropharynx, neck, pelvis, testis/ovary,

bones, breast)

6. Burkitt’s lymphoma frequently associated with EB virusC. Work-up

1. Appropriate imaging studies2. LDH, uric acid3. Biopsy

a. Pleural, acidic fluid may sufficeb. Node bx under local anesthesiac. FNA, core bx may sufficed. Bx primary masse. Do NOT delay treatmentf. Debulking NOT indicatedg. Bone marrow aspiration and biopsy

D. Markers - none specific1. Immunophenotypes used for classification

E. Stages: NHL (St. Jude (Murphy’s) system (POG))I: Single site, NOT in mediastinum or abdomenII: Regional involvement on one side of diaphragm; or 1° GI tumor which is completelyresectedIII: Regional involvement on both sides of diaphragm; any 1°intrathoracic tumor; extensive 1°intra-abdominal diseaseIV: Any of above with initial involvement of CNS, bone marrow, or both

F. Treatment1. Limited role for surgical resection: only if complete resection can be done (e.g. incidental

finding, intussusception mass, etc.)2. Radiation has no role in treatment 3. Chemotherapy mainstay of treatment4. Lymphoblastic lymphoma

a. Same agent as for ALLb. 8-10 drugs; intrathecal chemotx; 12-32 mos

5. Diffuse undifferentiated (“Burkitt’s”) lymphomas (DUL)a. Short-term, intensive therapy emphasizing alkylating agents (cytoxan); intrathecal

6. Large cell lymphomaa. Based on histological subtypes

G. Prognostic Factors1. LDH and uric acid levels indicate disease burden2. Cell type (DUL worst)

H. Results1. Complications of therapy (RT and chemotx)

a. Second malignancies (leukemia and solid malignancies -thyroid, breast)b. Cardiopulmonary damage

1. Pulmonary fibrosis, radiation pneumonitis (RT, bleomycin)2. Cardiomyopathy (adria)3. Coronary artery disease (RT)

c. Diminished height (RT)d. Infertility (MOPP in males, azoospermia; MOPP in females, ovarian failure)e. Complications of staging laparotomyf. Post-splenectomy sepsis (7% @ 15 years)g. SBO (10%)

I. Survival1. Lymphoblastic: 80-90% limited disease 65-80% advanced disease2. Diffuse undifferentiated (DUL; “Burkitt’s”) 90-100% limited disease; 75-85% advanced

disease3. Large cell: 50-70%

Esophageal AnomaliesA. Types

1. Isolated TEF (Gross E) - 4%2. Isolated EA (Gross A) - 10%3. EA with TEF (Gross C) - 85%

B. Etiology1. Faulty division of foregut2. Inadequate mesenchymal, epithelial growth

C. Epidemiology1. 1:4000 live births2. Slight male predominance

D. Associated AnomaliesV - VertebralA - AnorectalC - CardiovascularT - Tracheo-esophagealER - RenalL - Limb (radial hypoplasia)

E. Clinical Presentation1. Isolated TEF - recurrent PNA2. Isolated EA -

a. Maternal polyhydramniosb. Drooling; “mucosy baby”

3. EA with TEFa. Maternal polyhydramniosb. Drooling; “mucousy baby”c. Cough, gag with feedingsd. Pneumonia

F. Work-up: Complete physical exam emphasizing VACTERL BABYGRAM

1. Isolated EA and EA with TEFa. CXR/KUB with oro-pharyngeal tube in proximal esophagusb. ECHO to assess side of aortic arch (must happen pre-op)c. Renal USd. MRI (r/o tethered cord)e. r/o imperforate anus

G. Treatment: Isolated EA - frequently “long-gap”1. Feeding gastrostomy2. Delayed primary repair3. Esophageal substitution (colon or stomach)4. Gastric pull-up with esophago-esophagostomy

H. Treatment: EA with TEF – Healthy infant, no respiratory compromise1. Primary repair

a. Thoracotomy/ thoracoscopy; side opposite the aortic archb. Extra-pleural approachc. One layer, end-to-endd. Separate suture lines (fistula closure and Esophageal anastomosis)

I. Treatment: EA with TEF – “Sick” infant, respiratory compromise1. Delayed primary repair

a. Gastrostomy (decompression)b. Central line (TPN)c. Correct underlying condition before repair (PNA, long-gap, CHF, etc.)

2. Staged repaira. Gastrostomy (decompress/feeding)b. Divide TEFc. Correct underlying condition before repair (HMD, PNA, long-gap, severe prematurity,

CHD, etc.)J. Outcome: Complications

1. Anastomotic leak (obtain esophagram 1 week post-op in all cases; keep chest tube in untilthis shows no leak)

2. Esophageal stricture3. Recurrent TEF4. GE Reflux – all get treated for it post-op to prevent stricture5. Tracheomalacia – may be part of original anomaly6. Esophageal dysmotility – may be part of original anomaly

K. Outcome: Survival1. 85-95%2. High risk infants3. Birth weight < 1500 gm4. Major CHD5. Ventilator dependency6. Long-gap atresia

Duodenal Atresia, Web, Stenosis, Annular PancreasA. Types

1. Atresia - complete obstructionType I - Intact membrane; muscularis intactType II - 2 blind ends connected by cordType III - 2 blind ends with V-shaped mesenteric defect (vascular compromise)

2. Web - incomplete obstruction (wind-sock; Ampulla of Vater usually at edge of web)3. Stenosis - incomplete obstruction (rare)4. Annular pancreas - incomplete or complete obstruction

B. Etiology1. Failure of recanalization of duodenal lumen after solid phase of embryological development2. Aberrant rotation of ventral pancreatic bud and fusion with dorsal pancreatic bud. (Annular

pancreas)C. Epidemiology

1. 1:7,000 - 10,000 live births2. Associated anomalies 3. Down’s syndrome in 1/3 of cases (usually pre-ampullary)4. Rotational anomalies5. CHD (in 1/2 of patients with Down syndrome)

D. Clinical presentation1. Maternal polyhydramnios2. Bilious emesis 3. Non-bilious emesis - 15% are pre-ampullary, so, vomiting is NON-bilious; common in

Down’s syndromeE. Work-up: BABYGRAM

1. KUB - “Double bubble”; better seen in upright film (AP and Lat)2. If Down’s, ECHOcardiogram3. Karyotype

F. Treatment1. Gastric decompression, F&E correction2. Duodenoduodenostomy (diamond shape anastomosis)3. Rule out additional distal obstructions (esp if intra-luminal webs)4. +/- Tapering duodenoplasty

G. Outcome: Complications1. Delayed gastroduodenal emptying (esp if with delayed diagnosis)2. Duodenomegaly

H. Outcome: Survival - 90-95%1. Risk factors: Down’s and CHD

MalrotationBilious emesis in children is a surgical emergency until proven otherwise

A. Etiology: Normal rotation1. 270° Counterclockwise duodenojejunal rotation behind SMA2. 270° Counterclockwise ileo-cecal rotation over SMA3. Fixation: Duodenum to R gutter; duodeno-jejunal junction to L gutter (Ligament of Treitz);

Base of mesentery, from the Ligament of Treitz in LUQ to RLQ; R colon to R gutter; L colonto L gutter; Cecum and sigmoid colon not fixed.

B. Etiology: Malrotation1. Nonrotation: (a), (b), (c) do not occur2. Incomplete rotation: (b), (c) do not occur3. Incomplete fixation: (c) does not occur; (cecal volvulus, para-duodenal hernia, internal

hernia – behind R or L mesocolon)4. Reverse rotation – 270° clockwise duodeno-jejunal rotation in front of SMA; pylorus and 1st

part of duodenum to the L of vertebral column; no Ligament of Treitz.C. Epidemiology

1. 1% of newborn intestinal obstruction2. MOST COMMON cause of bilious emesis within 1st 6 weeks of life (excluding 1st 2 wk

period – webs and atresias)3. Age of onset

a. Newborn - 30%b. Within 1st month - 65%c. Within 1st year - 85%

D. Associated anomalies1. Gastroschisis; omphalocele: always associated with CDH2. Duodenal atresia, annular pancreas (cause or effect?): frequently

E. Clinical presentation1. Duodenal obstruction (Ladd’s bands)

a. Bilious emesis similar to duodenal atresiab. May be intermittent; Ladd’s bands tighten when cecum pulls to the L (twist-untwist)

2. Midgut volvulus, acutea. Bilious emesis within 1st month of lifeb. Cardiovascular collapse

3. Midgut volvulus, chronic or intermittenta. Recurrent abdominal painb. Malnutrition; malabsorption

4. Internal hernias (“paraduodenal”), cecal volvulusa. SBO

5. Asymptomatic, incidental findingF. Work-up

1. UGI. This should be done emergently if there is any suspicion of malrotation/midgut volvulus;caution if with bilious emesis and risk of aspiration

2. BE. For suspicion of malfixation (intermittent bilious emesis; intermittent double-bubble signesp if with some gas in the small bowel)

G. Treatment

1. Ladd’s procedure2. Correct volvulus (counterclockwise rotation)3. Lyse Ladd’s bands;4. straighten duodenum5. jejunal loop in R gutter if w/o Ligament of Treitz6. Separate duodeno-jejunal loop from ileo-cecal segment – and place small bowel on the right,

large bowel on the left7. Appendectomy8. “2nd look” procedures in case of ischemia

H. Outcome: Complications1. Recurrent 360 degree volvulus - very rare2. Short-gut syndrome - extensive resection3. Chronic abdominal complaints – dysmotility;

I. Outcome: Survival1. Related to degree of intestinal necrosis - 65% mortality with more than 75% necrotic bowel

Necrotizing EnterocolitisA. Etiology

1. Known risk factors: severe prematurity, low birth weight, low APGAR, PROM, RDS, UAC,maternal drug use, hyper-osmolar feeds

2. 10% occur in term infants3. 10x more common in infants who have been enterally fed (hyper-osmolar feeds)

B. Clinical presentation1. Ileum, cecum, right colon most common sites of involvement (end of SMA – vascular

compromise in a low flow state)C. Evaluation

1. KUB: dilated intestinal loops, “fixed” intestinal loops (necrotic loop), pneumatosis (in crossand longitudinal section), portal venous gas, pneumoperitoneum (“foot-ball” sign)

D. Treatment1. NPO, NG decompression, broad spectrum antibiotics2. KUB and lateral decubitus or cross table films (AP and Lat) q 6-8 hours to define acute

progression of NEC3. CBC, platelet count, blood pH, electrolytes, ABGs

E. Surgical Indications1. Pneumoperitoneum2. Abdominal wall cellulitis or RLQ mass (for drainage)3. Worsening clinical course on medical therapy, dropping blood pH4. Intraperitoneal drain placement/enrollment into NEC study for patients < 1000 gm.

Biliary atresiaA. Types

1. Intrahepatic (non-correctible except for Kasai)2. Extrahepatic

a. Entire extra-hepatic biliary tree (90%)b. Patent GB and CBD; atretic common hepatic duct (intra-hepatic) (10%)c. Cyst at the porta (rare)

B. Etiology - unknown1. Progressive inflammatory cholangiohepatitis (Landing)2. Viral infection, vascular, congenital malformation, etc.3. Auto-immune (maternal?)

C. Epidemiology1. 1 in 10,000 - 12,000 live births2. Female predominance (1:0.6)

D. Associated anomalies1. Polysplenia syndromes2. Preduodenal portal vein3. Alagille’s syndrome: biliary hypoplasia, butterfly-like vertebral defects, pulmonary artery

hypoplasia or stenosis.E. Clinical presentation

1. Progressive jaundice during 1st month of life (dirty yellow or bronze rather than brightyellow of unconjugated hyper-bilirubinemia)

2. Clay-colored stools (neg for stool bilirubin)3. Hepatosplenomegaly (biliary cirrhosis; portal hypertension)

F. Work-up1. Rule out other causes of jaundice (TORCH titer, ABO or Rh incompatibility, sepsis, breast

milk, etc.)2. Specific W/U (when conjugated bilirubin >30% of TB)

a. Alpha-1-antitrypsin levelb. GGT (gamma-glutamyl transpeptidase) levelc. Duodenal aspiration for biled. US (R/O choledochal cyst; absent GB)e. HIDA scan (before conjugated bilirubin becomes too high)f. Open liver biopsy and cholangiogram (early, before conjugated bilirubin becomes > 50%

of TB)G. Treatment

1. Kasai procedure (portoenterostomy), with or without modifications - “noncorrectable”(intra-hepatic) type; Roux n Y (60-90cm) limb

2. Cyst (porta hepatis) excision and hepaticoenterostomy - “correctable” type (rare); Roux n Y(60-90) limb

3. Post-op managementa. Zosyn while NPO per BARC protocol (peds GI)b. if not on BARC protocol, d/c with Bactrim for a year

4. Liver transplantationa. Failed “Kasai”

b. Very late diagnosis (> 3-4 mos. old)H. Outcome: Complications

1. Cholangitis2. Portal hypertension and3. esophageal varices4. Cirrhosis

I. Outcome: Survival1. If jaundice clears: 80% at 10 years2. If jaundice persists: 10% at 10 years3. Overall: 65% at 10 years4. MOST patients ultimately require liver transplant5. Patients usually discharged on Actigall, ADEK (parenteral), Bactrim +/- steroids

Biliary Atresia Research Consortium (BARC) StudiesPI: Philip Rosenthal, MD Study Coordinator: Shannon

If pt not on BARC protocol: Bactrim x 1 year

PROBE P003 Database/Repository***This is the observational data and biospecimen collecting database/repository study. This studyenrolls ALL cholestatic babies (meeting inclusion/exclusion criteria listed below), not just those withBA. Parents must consent their child into this database study to be eligible for the steroid trial.

Inclusion Criteria

Infant’s age less than or equal to 180 days at initial presentation at UCSFCholestasis defined as serum direct bili of 2.0 or greater

Exclusion Criteria

Acute liver failurePrevious hepatobiliary surgery with dissection or excision of biliary tissue outside of UCSFDiagnoses of bacterial or fungal sepsis, hypoxia, shock, ischemic hepatopathy, any malignancy,any primary hemolytic disease, or ECMO or TPN-associated cholestasis

Specimens to be collected at time of planned Kasai portoenterostomy or cholangiogram

Liver biopsy tissue Gall bladder Bile (if present)Biliary remnant (to be oriented by surgeon for pathologist/coordinator)

Study coordinator, Shannon Fleck, is usually present in OR to collect specimens. Grace Kim is thestudy pathologist if she is unavailable.

START P004 Steroid Trial***This is the randomized, double-blinded, placebo-controlled steroid trial. Child must be in P003 tobe eligible.

Inclusion Criteria

Portoenterostomy or gall bladder Kasai within the past 72 hours

Exclusion Criteria

Known sensitivity to corticosteroidsPresence of significant systemic hypertension for ageDocumented bacteremia or other tissue infection, known congenital infection or disease withHSV, toxoplasmosis or CMV inclusion disease of the liver

Study Protocol

Patient must be randomized to steroid vs. placebo and have study drug administration initiated within72 hours of returning from Kasai procedure. All study drugs are dispensed by the investigationalpharmacy. Scott Fields is the study pharmacist.

Days 1-3: Study drug (methylprednisolone/placebo ) IV 4 mg/kg/day, divided BIDDays 4-7: Study drug (prednisolone/placebo) PO 4mg/kg/day, divided BIDWeek 2: Study drug 4 mg/kg/day, divided BIDWeek 3-4: Study drug 2 mg/kg/day, divided BIDWeek 5-6: Study drug 1 mg/kg/day, once a dayWeek 7: Study drug 0.8 mg/kg/day, once a dayWeek 8: Study drug 0.6 mg/kg/day, once a dayWeek 9: Study drug 0.4 mg/kg/day, once a dayWeek 10: Study drug 0.2 mg/kg/day, once a dayWeek 11: Study drug 0.1 mg/kg/day, once a dayWeek 12-13: Study drug 0.1 mg/kg/day, every other day

Additional Study Meds

The study (investigational pharmacy to dispense) also provides the following meds and Pregestimilfree of charge for up to two years for enrolled infants:

Zantac efferdose tablets 12.5 mg PO twice daily Septra 4 mg TMP/kg/day AquADEKs 1 ml PO twice dailyVitamin K 2.5 mg daily three times a week (Mon/Wed/Fri) Ursodeoxycholic acid 20 mg/kg/day

Dose Reduction

In the event that a subject has a potential expected or unanticipated side effect of the study

drug/placebo (such as irritability) but there is no indication to stop the study drug/placebo, theclinical site PI will have the option to reduce the dose of the study drug/placebo by 50%. The subjectwill then be monitored for improvement in these symptoms. If the symptoms improve within 48 hours,the subject will be maintained at the reduced dose for the remainder of the days planned for theoriginal dose, as well as for the duration of treatment with this dose according to the normal taperschedule. If symptoms do not improve/resolve, a further reduction by 50% of the new dose will beconsidered by the clinical site PI. If the symptoms persist beyond 48 hours and the clinical site PIjudges it necessary that the study drug/placebo be discontinued, the tapering and discontinuationprotocol will be followed.

Please contact Shannon Fleck with any questions, potential patients or protocol deviations. Email:flecks@peds.ucsf.edu Phone: 476-1539 Pager: 443-3217

Choledochal CystA. Types

Type I – most common 85-95% of cases- cystic dilation of common bile ductType II- diverticulum of the common bile ductType III-aka choledochocele- intraduodenal . rarely intrapancreatic, dilation of the commonbile ductType IV- variant of Type I, intrahepatic and extrahepatic multiple cystic dilationsType V- aka Caroli’s disease- single or multiple intrahepatic cysts with a normalextrahepatic biliary tree, leading to hepatic fibrosis

B. Etiology – unclear1. Distal obstruction combined with pancreaticobiliary reflux in utero leads to degenerative

dilation of the extrahepatic ductal systemC. Epidemiology

1. rare, incidence 1:15,000 in the U.S. 2. female-to-male incidence 4:13. more frequent in Asians

D. Associated Anomolies1. anomalous junction of the pancreatic and common bile duct, distal bile duct stenosis,

intrahepatic ductal dilation, abnormal histologic findings of common bile duct, normal orcirrhotic hepatic histology

E. Clinical presentation1. 25% diagnosed early, prenatal and <6mo-1year, 2. prenatal diagnosis by maternal ultrasounds3. postnatal: presents with signs of complete extrahepatic biliary obstruction: jaundice,

hepatomegaly, acholic stools; similar to presentation of biliary atresia 4. remainder diagnosed later in life, usually by age 105. Present with subtle, episodic abdominal pain, minimal jaundice that may be unnoticed, or

pancreatitis sx from cholangitisa. 15% present with classic triad: intermittent pain, jaundice, abdominal mass

F. Workup

1. elevated LFTs: conjugated hyperbilirubinemia, increased alk phos, ALT, AST, GGT2. diagnosed by abdominal ultrasound or abdominal CT3. MRCP4. ERCP

G. Treatment1. total choldedochal cyst excision and Roux-en-Y hepaticojejunostomy (anastomosis of normal

proximal bile ducts to a limb of jejunum2. for Type V, lobectomy of involved liver or liver transplant if both liver lobes affected

H. Outcome1. if left untreated, can lead to chronic liver failure or biliary carcinoma due to chronic

cholestasis and biliary obstruction2. Improved outcomes if patient is <5 yo when resected

I. Outcome: Risks1. ascending cholangitis- infection of bile duct from ascending bacteria from small bowel

a. sx. charcot’s triad: jaundice, fever, abd pain… can lead to septic shockb. high WBC, high LFTs, c. w/up: blood culture, abd ultrasound to see duct dilationd. treatment: IVF and antibiotics

2. anastamotic stricture3. intrahepatic stone4. pancreatitis 5. portal hypertension6. small bowel obstruction7. adenocarcinoma of gallbladder and biliary ducts- from prolonged inflammation of bile ducts,

10-15% of patients can develop carcinoma if not excisedJ. Yearly follow-up

1. abdominal ultrasound2. LFTs

Jejuno-Ileal Atresia, StenosisA. Types

I: Complete web; bowel and mesentery intactII: Solid cord between ends; mesentery intactIII:

2 ends separated, V-shaped mesenteric defect “apple-peel” or “Chistmas tree” deformity jejunal atresia; absent dorsal mesenterybetween jejunum and ileum; distal ileum supplied by retrograde flow from middle colicartery through ileocolic artery

IV: Multiple atresias (20%) (sausage links)B. Etiology

1. Intrauterine vascular accident; intra-uterine ileo-cecal intussusception; Meckel’s D volvulusC. Epidemiology

1. 1 in 1000 live births2. Atresia 95%; Stenosis 5%3. Jejuno-ileal atresia: duodenal atresia = 2:1 (J-I Atresia: vascular accident; Duodenal

Atresia: failure to recanalize)D. Associated anomalies

1. Gastroschisis: 15%2. Malrotation: 15%3. CF: 10%4. Meconium ileus, meconium peritonitis: 10%5. Rare extra-intestinal anomalies

E. Clinical presentation1. Bilious emesis; the higher the atresia, the earlier the vomiting, the less likely abdominal

distention will be a prominent physical finding; Exception – Pre Ampullary Web (NON-bilious emesis)

2. Abdominal distention after birth; the lower the atresia, the more prominent the abdominaldistention, the later the vomiting

3. Failure to pass meconium; the lower the atresia, the later the passage of abnormal meconium;the higher the atresia, the more likely normal meconium will be passed; atresias form after theinitial passage of meconium from the CBD to the duodenum

4. Jaundice; the higher the atresia, the more prominent the Jaundice retrograde pressureimpeding passage of bile to the duodenum

F. Work-up BABYGRAM1. KUB: Multiple dilated loops with fluid levels; the higher the atresia, the less dilated loops

(“double-bubble” in Doudenal Atresia)2. BE: Micro-colon (the smaller the micro-colon, the lower the atresia)

G. Treatment1. Resect proximal end (transversely) (generous resection of bulbous end if ileal atresia) and

distal end (obliquely or transversely with anti-mesenteric extension) with end-to-endanastomosis

2. Tapering jejunoplasty for proximal jejunal atresias with end-to-end anastomosis (or taperingduodeno-plasty for jejunal atresia at Ligament of Treitz)

H. Outcome: Complications1. Functional anastomotic obstruction (sometimes, due to inadequate resection of bulbous

proximal end); resolves (STEP operation)2. Anastomotic leak

I. Outcome: Survival1. Overall 90%2. Adverse risk factors: proximal vs. distal atresias; multiple atresia, apple-peel atresias;

association with meconium ileus, meconium peritonitis or gastroschisis

Meconium SyndromesA. Types

1. Meconium ileus (= CF)2. Meconium peritonitis (= CF; Intrauterine NEC)3. Meconium plug syndrome (= CF; Hirschsprung’s Disease)4. Meconium ileus

B. Etiology1. CF: 80% of infants with meconium ileus have CF;2. 20% of infants with CF have meconium ileus3. Hyperviscous mucus secreted by abnormal intestinal glands + pancreatic exocrine

insufficiencyC. Types

1. Uncomplicated: Simple mechanical obstruction2. Complicated: Associated with meconium peritonitis, volvulus, atresia, meconium cyst

D. Epidemiology1. CF in 1:2500 live births2. Unusual in African-Americans and Asians3. Increased incidence of meconium ileus in siblings if index case had meconium ileus (0% vs

20% expected)E. Clinical presentation

1. Bilious emesis2. Abdominal distention after birth3. Visible and palpable loops of bowel; doughy4. Failure to pass meconium; passage of goat-dung like meconium

F. Work-up1. KUB: Soap-bubble appearance RLQ (Neuhauser’s sign)2. Gastrograffin enema: Microcolon with meconium pellets in terminal ileum3. Sweat chloride test or buccal swab4. discuss case with attending surgeon to determine if rectal biopsy necessary

G. Treatment: Non-operative (Uncomplicated cases)1. Gastrograffin enemas2. Mucomyst (acetyl cysteine) (5%) per OG tube

H. Treatment: Operative1. For uncomplicated cases where enemas have failed2. For all complicated cases3. Tube enterostomy with Mucomyst (acetyl cysteine) distal irrigations4. Resection, irrigation, 1° anastomosis5. Resection, irrigation, enterostomy6. Mikulicz7. Bishop-Koop8. Santulli

I. Outcome: Long-term complications (related to CF)1. Meconium ileus equivalent (= distal intestinal obstruction syndrome DIOS)2. Intussusception (1%)

3. Rectal prolapse (10-30%)4. Colon strictures (high-dose enzymes)5. Cholelithiasis (12-25%)6. Inguinal hernia (absent vas)

J. Outcome: Survival1. 90-100% for uncomplicated meconium ileus2. 75-90% for complicated meconium ileus

Meconium peritonitisA. Etiology

1. Intrauterine bowel perforationB. Associated anomalies

1. Atresias, volvulus, etc.2. Meconium ileus (CF)3. Intra-uterine perforated4. NEC

C. Clinical presentation1. Bilious emesis2. Abdominal distention at birth3. Giant cystic meconium peritonitis

D. Work-up1. KUB: Calcifications; fluid levels; giant meconium cyst (omental encystment)2. BE: Microcolon +/- meconium pellets

E. Treatment1. Lysis of adhesions2. Correct underlying problem (atresia, volvulus, meconium ileus, etc.)3. Drain giant meconium cyst

F. Outcome: 70% survival

Meconium plug syndromeA. Etiology

1. Meconium plug obstruction of colonB. Associated anomalies

1. Idiopathic; prematurity2. CF: 25%3. Hirschsprung’s disease

C. Clinical presentation1. Failure to pass meconium2. Abdominal distention after birth3. Bilious emesis

D. Work-up

1. KUB: Dilated loops; distal obstruction2. BE: Radiolucent plug in transverse or descending colon

E. Treatment1. BE evacuation of plug2. If still obstructed, R/O CF and Hirschsprung’s disease; ROUTINE RECTAL BIOPSY unless

CF+F. Outcome: cured with BE

Small Left Colon SyndromeA. Etiology

1. Associated with maternal diabetesB. Clinical presentation

1. Abdominal distention after birth2. Failure to pass meconium

C. Work-up1. KUB: Multiple dilated loops; distal obstruction2. BE: Small left colon with meconium plug at splenic flexure

D. Treatment1. Contrast enema is usually curative2. If obstruction persists, R/O Hirschsprung’s disease (routine rectal biopsy) and CF 3. May need colostomy (rare)

E. Outcome: Complications 1. Cecal perforation

F. Results1. Most are cured by BE

Hirschsprung’s DiseaseA. Etiology

1. Failure of complete cranio-caudad migration of enteric neuroblasts (neural crest origin)2. Hostile local enteric environment leading to destruction of migrated neuroblasts

(immunologic, vascular, infectious)3. Genetic aberrations

a. Familial Hirschsprung’s (long segment HD or TCA): 4-8% of casesb. Locus on RET protooncogene (Chromosome 10)c. Locus on endothelin-B receptor gene (EDNRB) Chrom 13

4. Pathophysiologya. Lack of ganglion cells in Meissner’s (sub-mucosal) and Auerbach’s (myenteric or

“sandwich” layer) plexib. Lack of non-adrenergic, noncholinergic inhibitory fibers (NO)c. Failure of relaxation of involved segment down to the internal anal sphincter

(condensation of most distal circular muscle fibers of rectum)

B. Epidemiology1. Incidence: 1:4000 - 1:7000 live births2. Male:female = 4:1 (Long-segment M:F = 1:1)3. Siblings of female index cases have 360X more risk than general population4. Siblings of male index cases have 120X risk5. Risk to son or brother of affected female with long-segment disease = 29% and 24%

respectivelyC. Associated anomalies

1. Down syndrome: 5-16%2. MEN IIA, neurocristopathies (RET)3. Waardenburg syndrome (mottling of periphery or retina, defective night vision, deafness)

D. Clinical presentation1. Failure to pass meconium within 1st day of life2. Abdominal distention3. Poor feeding, emesis4. “Constipation” (once a day is constipation in infants); “obstipation” (obstinate stools) in

older children5. Enterocolitis

a. Acute abdominal distention with feculent emesisb. Fever and malaise c. Explosive, foul-smelling, green, watery diarrhea, spontaneous and following rectal exam

E. Work-up1. KUB: Multiple dilated loops; dilated (mega) proximal colon (proximal to aganglionosis);

Distal obstruction (aganglionosis)2. BE

a. “Transition” (funnel shaped) zone between dilated ganglionic bowel and spastic(collapsed) aganglionic distal bowel

b. Absent in 20-30% of short segment HD in newborns; may be absent in long segment (Lcolon or total colonic) aganglionosis

c. Normal caliber colon in patient with distal obstruction (think total colon Hirschsprung’s -TCA)

d. Failure to evacuate barium on 24 hour delayed film – presumptive evidence of HD innewborns; in older children, may be due to psychogenic constipation

3. Anorectal manometrya. Failure of relaxation of internal sphincter with rectal distensionb. Not diagnostic in young infants

4. Rectal biopsya. Suction biopsy is a bedside procedure

1. Submucosal (Meissner’s plexus – H&E stain; acetylcholinesterase staining)2. Biopsy at least 2 cm above dentate line (“physiologic” aganglionosis <2cm)

b. Full-thickness biopsy is an operative procedure; Gold Standard1. For older infants and children

c. Evaluate for absence of ganglion cells (Auerbach’s plexus), hypertrophic nerves,and/or

increased acetylcholinesterase staining5. The absence of proof of ganglion cells is proof of the presence of Hirschsprung’s Disease.

F. Treatment1. Decompression after histologic diagnosis of HD

a. Diverting “leveling colostomy”: placed proximal to a biopsy (Frozen Section) confirming(+)ganglion cells in the transition zone;

b. Rectal irrigation program with NS; may be started before colostomy2. Definitive pullthrough procedure

a. Swenson’s pullthrough: Abdomino-perineal Proctosigmoidectomy with transanal colo-anal end-to-end anastomosis

b. Soave: Endorectal transanal pullthroughc. Duhamel’s pullthrough: Abdomino-perineal sigmoidectomy with Retrorectal transanal

colo-rectal side-to-side anastomosis d. Martin modification: Extended Duhamel: For long-segment diseasee. Kimura procedure: Right colon patch with Swenson pullthrough for total colon

Hirschsprung’sf. Georgenson procedure: Laparoscopic “Swenson” and transanal “Soave” either in

newborns or following rectal irrigation program3. Ultrashort-segment Hirschsprung’s

a. Definition: No transition zone on BE, absence of relaxation of internal sphincter onmanometry, ganglion cells present 3 cm above dentate line

b. Treatment: Anorectal posterior myomectomy4. Post-operative anal dilations – start with 12fr anal dilator and increase size weekly to 14 fr

and taper size weekly. G. Outcome

1. Complicationsa. Enterocolitis: 20-30%b. Abdominal distention, fecal retention, orc. Explosive watery diarrhea

2. Treatment: a. Diarrhea requiring constipation therapyb. Constipation requiring enema therapyc. Anastomotic leak: 1-5%d. Treatment – Consider diverting colostomy if peritonitis

H. Results1. “Normal” bowel habits: 85%2. Survival: 95%3. Leading cause of death is enterocolitis

Anorectal Malformations (“Imperforate Anus”)A. Types

1. “High” (Supralevator) - Males

a. Recto-urethral fistula (most common)b. Rectovesical fistula (male cloaca)c. Rectal atresia (old classification Type IV)

2. “High” (Supralevator) - Femalesa. Rectovaginal fistula (rare)b. Persistent cloaca (common)c. Rectal atresia (old classification Type IV)

3. “Low” (Infralevator) - Malesa. Perineal fistula (median raphe, “bucket handle”, meconium “pearls”, anterior, perineal)

4. “Low” (Infralevator) - Femalesa. Perineal fistula (anterior perineal fistula)b. Vestibular fistula (most common) behind vagina, outside hymen

B. Etiology1. Faulty division of the cloaca

C. Epidemiology1. Incidence: 1 in 5000 live births2. Males predominate (55-65% of cases)3. “High” lesions predominate in males; “low” lesions in females

D. Associated anomalies1. VACTERL syndrome (see esophageal atresia)2. “High” lesions have higher incidence of and more severe associated anomalies

E. Clinical presentation1. Diagnosis made by Physical Exam!!

a. The only acceptable reasons for not doing digital rectal exam are: No rectum; and 2. Nofinger

2. “High”, malea. No anal opening; “flat bottom”b. Meconium in urine

3. “High”, femalea. Single perineal orifice (cloaca) usually posterior to the clitorisb. No anal opening; “flat bottom”; “no cleavage”

4. “Low”, malea. Perineal fistula in median rapheb. “Bucket-handle”, meconium “pearls”

5. “Low”, femalea. Vestibular opening (outside of hymen)b. Perineal fistula (anterior to anal dimple)

F. Work-up1. Complete physical exam emphasizing VACTERL especially in cases of suspected down

syndrome2. Detailed perineal inspection 3. BABYGRAM

a. Prone cross-table lateral film at 24 hours of age with rolled towel under pelvis

b. Gas shadow < 1 cm from perineal (anal dimple) marker = “low” lesionc. renal US, possible VCUGd. R/O esophageal atresia/fistulae. Echocardiogramf. AP thoracic and lumbosacral spine filmsg. Spine ultrasound to r/o tetherd cord < 3 months h. Total spine MRI to r/o tethered cord > 6 monthsi. High pressure distal colostogram prior to PSARP.

G. Treatment1. “High” anomalies, or unsure of level2. Colostomy3. Divided - totally diverting4. Located in descending colon5. Posterior saggital anorectoplasty (Pena Procedure)6. Additional laparotomy/laparoscopy in 10% of males; and laparotomy in 40-50% of females

with cloaca (for mobilization)7. Posterior saggital rectovaginourethroplasty (Pena) for cloaca8. Additional laparotomy for uro-genital mobilization to facilitate urethro-vaginal

reconstruction9. Vestibular fistula in female

10. Either colostomy followed by limited Pena procedure or primary pullthrough (Pena, Pott’sperineal transfer, Mollard anterior perineal pullthrough, etc.)

11. Primary posterior pull through followed by colostomy only if vagina is violated12. “Low” (perineal fistula) anomalies 13. Primary perineal anoplasty without colostomy

H. Post-operative management1. anal dilations- starting 2 weeks post2. PSARP3. send family home with anal dilators upon discharge to bring to post-op clinic visit4. will need anal dilations during first year of life.5. see anal dilator table and schedule:6. s/p stoma takedown. Order skin protectant and educate family re: diaper rash prevention

I. Outcome: Complications1. Anal stenosis: 30%2. Mucosal prolapse3. Intractable constipation: 25%

a. More common with “low” anomalies 4. Incontinence

a. “Low”: 10%b. “High”: 50% (Variable depending upon type of anomaly and number of re-operations)

J. Outcome: Survival1. 95% for “low” anomalies

2. 85% for “high” anomalies3. Death from cardiac or renal anomalies; infection

Abdominal wall defectsOmphalocele: Membranous covering; often with other anomalies

Gastroschisis: No covering with cord intact

A. Hypothermia most immediate life-threatening problem.B. Plastic “turkey bag” dressing to reduce evaporative losses from exposed intestine.C. All infants require NG tube decompression.D. Initiate systemic IV antibiotics immediately.E. With gastroschisis in particular, intestine should be supported to avoid mesenteric angulation and

bowel ischemia.F. For omphalocele patients, obtain echo, renal US, chromosomal analysis. If with Macroglossia,

Hemi-Hypertrophy, monitor blood sugar.

Congenital Diaphragmatic HerniaA. Type

1. Bockdalek – unilateral posterior-lateral absence of a portion or all of the diaphragm.Bilateral disease possible but rare. 85% left-sided. 5% have hernia sac.

2. Morgagni – central/anterior, often detected later in life.3. Eventration – diaphragmatic paralysis.

B. Etiology1. Failure of fusion of transverse septum and pleuroperitoneal folds in 8th week of gestation 2. leading to incomplete formation of the diaphragm.

C. Epidemiology1. Sporadic, usually non-genetic (<2% familial). Possible relation to vitamin A/retinol

metabolism. Affects 1 in 2000 live births, equally affects males and females.D. Associated anomalies

1. Congenital heart disease (significantly increases morbidity/mortality)2. Pulmonary anomalies (e.g., pulmonary sequestration in 5%)3. Chromosomal abnormalities (up to 30%)4. Hepatopulmonary fusion – rare5. Intestinal malrotation

E. Clinical presentation1. Most detected with prenatal ultrasound.

a. LHR < 1.0 severe, may be candidate for fetal tracheal occlusion.b. LHR >1.4 better prognosis.

2. Scapoid abdomen and mild to severe respiratory distress from visceral contents herniatedinto chest, associated pulmonary hypoplasia, mediastinal shift and pulmonary hypertension.

3. Chest on affected side dull to percussion, bowel sounds may be heard in chest, heart soundsmore pronounced on right side if left sided defect.

F. Workup1. Chest radiograph2. Karyotype3. Echocardiogram4. Head ultrasound5. DHREAMS (CDH genetics) study enrollment—consent to obtain parental and patient blood,

patient skin and diaphragm tissue for analysisG. Treatment

1. Intubation and ventilator management as required by ICN teama. Permissive hypercapnia/”gentilation” strategy—avoid PIP>25

2. iNO, high-frequency oscillatory ventilation (HFOV) if needed 3. Paralysis or sedation4. Replogle to suction5. ECMO if unable to manage ventilation6. Surgical repair when deemed stable by attending surgeon and neonatologist.

a. In ICN or OR (depending on stability, generally between day-of-life 3 and 7)b. Thoracosopic vs openc. Primary vs patch/muscle flapd. Chest tube per surgeon

H. Outcomes1. Feeding difficulties may require NG tube vs. Gtube2. Gastroesophageal reflux – all patients discharged on Reglan and Ranitidine. May require

antireflux procedure.3. Nasal cannula oxygen as needed4. Persistent PPHN requiring pulmonary vasodilators5. Formal hearing study in ICN and post discharge for sensorineural hearing loss6. Developmental follow-up for hjgh-risk status7. Synagis RSV prophylaxis for first 1-2 winters8. Long-term follow-up in CDH clinic to monitor for recurrence and manage co-morbidities

Commonly Used MedicationsNever guess the dose of medication for infants and children. Making an estimate based on adultdosages is dangerous.

A child’s dose is NOT half the adult dose.

Please refer to the Harriet Lane Handbook, the inpatient pharmacist or UCSF website (http://meds) toconfirm dosages. All medications are based on the weight of the patient; no ranges are to be utilizedfor pain medications. Medications must be ordered on a mg/kg basis with proper avoidance ofabbreviations (i.e. when in doubt spell it out).

Be very careful about decimal points.Check if mg/kg dose is per 24 hrs or per dose (and bid or q12hrs; tid or q8hrs; qid or q6hrs)Check if dose is PO or IV (sometimes oral meds are put in syringes to facilitate giving smallvolumes)

Emergency DrugsApproximate size of a child’s trachea is the size of the child’s small (5th) finger

Atropine 0.02 mg/kg/dose IVP

Min single dose = 0.1mgMax single dose children = 0.5mg, adolescents = 1mg)May repeat x 1 doseEndotracheal administration: 0.03 mg/kg

Flumazenil 0.01mg/kg/dose IVP

Max single dose = 0.2mg, Max TOTAL dose = 1mgMay repeat every 1 minDextrose 2-4ml/kg (1-2 grams/kg) D25W IVP over 5 minutesMax adult dose: 50-100ml (25-50 grams)

Naloxone 0.1mg/kg IVP (up to 20kg)

> 5 years or > 20kg give 2mg rapid IVPMay repeat every 2-3 minutesEndotracheal administration: double dose

Analgesics/Narcotics

Guide to Initial Management of New-Onset PainNot intended for use in neonates 0-28 days

Codeine is NOT a recommended pain medication.

Metabolism to the active form is inefficient, producing many side-effect-inducing metabolites.10% of patients lack the enzyme to metabolize codeine and thus get no analgesia1-2% of patients are ultra-rapid metabolizers, and may suffer severe opiate intoxication

Methadone and Fentanyl should only be used with attending guidance.

Dosing & Precautions

Step 1 (Mild Pain) OptionsAcetaminophen (Tylenol®)

15mg/kg/dose PO/PR q6h ATC or q4h PRN painMax PO acetaminophen: 75mg/kg/DAY (5 doses/day),1 gram/dose, 4 grams/day

Acetaminophen (Ofirmev™)15mg/kg/dose IV q 6hr ATCReserve for patients unable to take PO medsMax IV acetaminophen: 1gram/dose, 4 grams/day

Ibuprofen (Motrin®)10mg/kg/dose PO q6h ATC or q 6h PRN painMax ibuprofen = 800 mg/dose

Step 2 (Moderate Pain) OptionsTramadol (Ultram®)

1-2 mg/kg/dose PO q6h ATC or q4h PRNMax 100mg/dose, 4 doses/day

Acetaminophen with Hydrocodone(dose in mg hydrocodone) 0.2 mg hydrocodone/kg/dose PO q6h ATC or q 4h PRNMax 5 doses/dayMax initial hydrocodone dose = 10mg/dose;Max acetaminophen – see above

Ketorolac (Toradol®)≤ 5 days duration only)=Multiple dose use: 0.5mg/kg/dose IV q6h ATC or q6h PRNOr - Single dose use: 0.5 - 1mg/kg IV x 1; Max = 30mg/dose

Step 3 (Severe Pain) Options - initial dosing guidelines

Oxycodone 0.1-0.15mg oxycodone/kg/dose po q6h ATC or q 4h PRNMax initial oxycodone = 5mg/dose

Acetaminophen with Oxycodone (dose in mg oxycodone) 0.1-0.15mg oxycodone/kg/dose po q6h ATC or q4h PRN painMax initial oxycodone = 5mg/dose, max 5 doses/dayMax acetaminophen - see above

Morphine Sulfate 0.05-0.1mg/kg/dose IV q4h ATC or q2h PRN painMax initial dose for patients <30kg = 2mg/dose IVMax initial dose for patients 30-50kg = 3mg/dose IVMax initial dose for patients >50kg = 4mg/dose IVThese max doses are for treatment of severe pain, and may not be appropriate for othercircumstances (e.g. prep for procedure)

Hydromorphone (Dilaudid®) 0.015mg/kg/dose IV q4h ATC or q3h PRNMax initial dose = 0.6 mg/dose IV

Acetaminophen PrecautionsMax doses should account for acetaminophen from all sources including combination products(Lortab, Percocet, etc)Consult attending before using acetaminophen in oncology or BMT patients

Ibuprofen PrecautionsConsult attending before using ibuprofen in oncology or BMT patientsConsult attending before using ibuprofen or other NSAIDS in surgical patientsUse Ibuprofen cautiously in patients with volume depletion or renal impairment

Tramadol PrecautionsMay cause nervousness, tremor, or somnolenceSeek guidance before ordering for patients takingSSRIs or TCAsEffect may be antagonized by ondansetron or granisetron

Ketorolac PrecautionsAlways use an H2-blocker or PPI with KetorolacUse caution in oncology or other patients at riskfor bleeding.Consult attending before using in surgical patients

Do not use in patients with volume depletion or renal impairmentDo not combine with other oral or IV NSAIDs

Acetaminophen/Hydrocodone Dosage Forms

Name Hydrocodone Acetaminophen

Vicodin® Tabs 5mg 325mg

Norco® Tabs 10mg 325mg

Lortab® Elixir 2.5mg/5mL 167 mg/5mL

Acetaminophen/Oxycodone Dosage Forms

Product Oxycodone Acetaminophen

Tablet (Percocet®) 5mg 325mg

Liquid (Roxicet®) 5mg/5mL 325mg/5mL

Pain Management Guide Authored by: Stephen Wilson, MD, PhD & Karen Sun, MD; Approved by:UCSF BCH Medication Committee; Revised: July, 2011; Approved: August, 2011

Key Principles of Pain ManagementFor OPIATE NAÏVE PATIENTS – Start with low initial doses and re-assess and adjustfrequentlyCarefully consider monitoring options and frequency of vital signsBe wary of OVERSEDATION when combining opiates with benzodiazepines, Benadryl®, orother opiatesUse MORE THAN ONE class of pain med for moderate to severe pain

Acetaminophen or NSAIDS can greatly reduce the amount of opiate neededFor patients with sustained pain, provide BOTH background pain control AND PRN forbreakthrough

Around-the-clock Tylenol or Ibuprofen may be adequateConsider scheduled ketorolac, tramadol or a long-acting opiates (non-opiate-naïve patients) forpatients with moderate to severe pain

When ordering multiple PRN medications, specify sequence of drugs (i.e. for mild pain vs.moderate pain) ALWAYS include non-pharmacological adjunctive treatments when possible

Child lifeExercise/physical activityWarm packs, massage, etc.Biofeedback (HeartMath®)Acupuncture and/or Acupressure

Address Sleep Disturbances, Anxiety, and Nausea

Patients who have these symptoms will experience pain more severely and require larger dosesof pain medicationsA large percentage of hospitalized children suffer major sleep disturbance. Consider:Limiting unnecessary nighttime interventions (vital signs, etc)Emphasizing environmental factors such as “lights out”, “TV off”, establishing bedtime and wakeup scheduleMelatonin (starting dose 0.5 – 3 mg enterally at bedtime, depending on age of patient)Anxiety and sleep disturbances are not the same. Benzodiazepines are useful for anxiety but NOTas sleep agents (they induce non-restful sleep).

When using opiates, proactively address opiate Side EffectsConstipation – Start a bowel regimen before constipation sets in; pay attention to stool pattern andescalate as needed. Start with Miralax® daily, increase to BID dosing as needed. If constipationpersists, add Senna. If refractory to other treatments, consider oral naloxone (see dosing below)

Itching – Benadryl is not the treatment for most opiate-induced itching. The itching associated withmorphine and dilaudid is not histamine-mediated. Consider switching opiates or starting a low dosenaloxone infusion (consult pain service and/or pharmacy).

Nausea – Common, particularly with morphine, dilaudid, and hydrocodone. Give oral opiates withfood if possible.

Sedation – This is a precursor to more severe problems. Consult attending and/or pain serviceimmediately and adjust medication regimen.

Hypoventilation – Pay attention to resting respiratory rate and quality of respirations. Lowrespiratory rate, even in awake patients, is a sign of opiate intoxication. Immediately consultattending and/or pain service.

Resources for Pain Management ConsultationIntegrated Pediatric Pain & Palliative Care Service (“IP-3”): 443-6100

Pediatric Clinical Pharmacists (PharmD on team or call 353-1265)

Rapid Response Team: 353-1611 (via Access Center)

Indications for Pain Service ConsultationNeed for PCAPatient with complex pre-existing pain historyMultiple symptoms (pain, nausea, anxiety)Neuropathic painNeed to convert between oral opiatesAnticipated use of fentanyl patch or methadoneWeaning off long-term opiates or signs of withdrawal

Consideration of interventional modality (nerve blocks, epidurals)Indications for acupuncture, acupressure, biofeedbackLack of response to conventional pain medications

Naloxone (Narcan®) DosingOpiate intoxication (oversedation)

1 – 5 mcg/kg/dose (0.001-0.005mg/kg) IV, repeat q2-3 min as needed. Doses at the high end of this range may lead to reversal of analgesia.

Respiratory Arrest (code blue)0.1mg/kg (max 2mg) IV or IM

NOTIFY ATTENDING IF NARCAN® IS GIVEN

Low dose naloxone infusion for itching0.25 – 2 mcg/kg/hr (start at 0.25mcg and titrate by0.5 mcg/kg/hr to max dose of 2 mcg/kg/hour).

Oral naloxone for severe constipationStart at 0.5-1 mg/dose PO q 6hr (not weight based) Titrate up as needed to max of 4mg PO q6hr.Seek assistance before dosing young infantsMonitor for reversal of analgesia

Antiemetics

Ondansetron (Zofran®)Children ≥ 2 years < 40kg: 0.1mg/kg/dose IV q8h prn Children > 40kg: 4mg IV q8h prn nausea

Granisetron (Kytril®) 10-40micrograms/kg/dose IV q12h or q24h prn post-op nauseaMax single dose= 1mg IV, Max TOTAL dose = 2mg IV q24h Metoclopramide (Reglan®)0.1-0.2 mg/kg/dose IV/PO q6h prn nausea/GERD Max= 10mg/doseSyrup: 5mg/5mlTablet: 5mg, 10mg

Perphenazine (Triafon)0.015 – 0.025 mg/kg/dose IV q 6 hrs prn (max 1 mg)

Procloroperazine (Compazine®)For use in Children > 2 years > 10kg ONLY

Route: PO/PRWeight: 10-14kg – Dose: 2.5mg q12h prn nauseaWeight: 15-39kg – Dose: 2.5mg q8h prn nauseaWeight: > 40 kg – Dose: 5-10mg q8h prn nausea

Route: IV0.1-0.15mg/kg/dose q8-12h prn nauseaMax=10mg/dose

Antimicrobials For Infants and Children > 1 Month ofAgeApproved by the Antibiotic Advisory Subcommittee and the Pharmacy and Therapeutics Committee(11/98) Rev 5/2012

For assistance in antimicrobial dosing and selection, contact Infectious Diseases Pharmacy (443-9421). For diagnosis and management recommendations or formal consultation, contact Pediatric IDfellow (443-2384).

Some Antimicrobial drugs are ID-Restricted agents (ID-R) and are indicated as such in the followingdrug guid. Call ID Pharmacy (443-9421) for prior approval 8am-10pm. Orders will NOT beprocessed without approval (exceptions for certain agents noted in APEX). 10pm to 8am a singledose will be released; further doses require approval

Antimicrobial Drug GuideAll drugs in the following guide list three main dosage catagories depending on Creatinine Clearanceand Renal Function.

1. Creatinine Clearance ≥ 50ml/min/1.73m2 Or… Renal Function ≥ 50% of Normal2. Creatinine Clearance 10–50 ml/min/1.73m2 (ESRD not on dialysis) Or… Renal Function 10–

50% of Normal3. Creatinine Clearance ≤ 10ml/min/1.73m2 Or… Renal Function ≤ 10% of Normal

Acyclovir IV — Non-CNS HSV infection1. 5-10mg/kg/dose q8h2. 5-10mg/kg/dose q12h (25-50% of normal clearance) 5-10mg/kg/dose q24h (10-25% of normal

clearance)3. 2.5-5mg/kg/dose q24h

Acyclovir IV — Herpes Zoster1. 10-20mg/kg/dose q8h2. 10-20mg/kg/dose q12h (25-50% of normal clearance) 10-20mg/kg/dose q24h (10-25% of normal

clearance)

3. 5-10mg/kg/dose q24h

Acyclovir IV — Herpes Zoster (Immunocompromised Host)1. 500mg/m2/dose q8h2. 500mg/m2/dose q12h (25-50% of normal clearance) 500mg/m2/dose q24h (10-25% of normal

clearance)3. 250mg/m2/dose q24h

Acyclovir IV — HSV encephalitis, all neonatal infections1. 20mg/kg/dose q8h2. 20mg/kg/dose q12h (25-50% of normal clearance) 20mg/kg/dose q24h (10-25% of normal

clearance)3. 10mg/kg/dose q24h

Acyclovir IV — HSV encephalitis, all neonatal infections (ImmunocompromisedHost)1. 500mg/m2/dose q8h2. 500mg/m2/dose q12h (25-50% of normal clearance) 500mg/m2/dose q24h (10-25% of normal

clearance)3. 250mg/m2/dose q24h

Amphotericin B Liposomal (AmBisome®) (ID-R except for Heme-Onc)1. 3mg/kg/dose IV q24h (Invasive yeast infections) 5mg/kg/dose IV q24h (Invasive mold infections)2. No change*3. No change*

* Dosage reductions in renal disease are not necessary. However, due to the nephrotoxic potential ofthe drug, reducing the dose or holding the drug in the setting of a rising serum creatinine may bewarranted.

Ampicillin IV1. 50mg/kg/dose q6h2. 25mg/kg/dose q6h3. 25mg/kg/dose q8-12h

Max Daily Dosage: 2gm q4h

Ampicillin IV — Meningitis1. 100mg/kg/dose q6h2. 50mg/kg/dose q6h3. 25mg/kg/dose q8-12h

Max Daily Dosage: 2gm q4h

Caspofungin (ID-R)1. LD 70mg/m2 x1; Then 50mg/m2 q24h2. No Change3. No Change

Max Daily Dosage: 70mg

Cefazolin1. 25mg/kg/dose q8h2. 25mg/kg/dose q12h3. 25mg/kg/dose q24h

Max Daily Dosage: 2gm q8h

Cephalexin PO1. 10mg/kg/dose PO q6-8h 20-25mg/kg/dose PO q6h (Otitis Media) 25-30mg/kg/dose PO q6h

(Osteomyelitis)2. 10mg/kg/dose PO q8h-q12h3. 10mg/kg/dose PO q12-24h

Max Daily Dosage: 4gm/day

Cefepime1. 50mg/kg/dose q12h2. 50mg/kg/dose q12h (>30% of normal clearance) 25mg/kg/dose q24h (10-30% of normal

clearance)3. 12.5mg/kg/dose q24h

Max Daily Dosage: 2gm q12h

Cefepime — Febrile Neutropenia1. 50mg/kg/dose q8h2. 50mg/kg/dose q12h (>30% of normal clearance) 50mg/kg/dose q24h (10-30% of normal

clearance)3. 24mg/kg/dose q24h

Max Daily Dosage: 2gm q8h

Cefepime — Cystic Fibrosis1. 50mg/kg/dose q8h

2. Not documented3. Not documented

Max Daily Dosage: 2gm q8h

Cefotaxime1. 50mg/kg/dose q8h2. 50mg/kg/dose q8h-12h3. 25mg/kg/dose q12h

Max Daily Dosage: 2gm q6h

Cefotaxime — Meningitis1. 50mg/kg/dose q6h2. 50mg/kg/dose q8h3. 50mg/kg/dose q12h

Max Daily Dosage: 2gm q6h

Ceftazidime1. 50mg/kg/dose q8h2. 50mg/kg/dose q12h3. 50mg/kg/dose q24-48h

Max Daily Dosage: 2gm q8h

Ceftazidime — Cystic fibrosis1. 50mg/kg/dose q8h2. Not documented3. Not documented

Max Daily Dosage: 2gm q8h

Ceftriaxone1. 50mg/kg/dose q24h2. No change3. No change

Max Daily Dosage: 1gm q24h

Ceftriaxone — Meningitis1. 50mg/kg/dose q12h2. No change

3. No change

Max Daily Dosage: 2gm q12h

Cefuroxime IV1. 50mg/kg/dose q8h2. 50mg/kg/dose q12h3. 50mg/kg/day q24h

Max Daily Dosage: 1.5gm q8h

Ciprofloxacin IV1. 15mg/kg/dose q12h2. 15mg/kg/dose q12h (30-50% of normal clearance) 7.5mg/kg/dose q12h (10-30% of normal

clearance)3. 7.5mg/kg/dose q12h

Max Daily Dosage: 400mg q12h

Ciprofloxacin IV — Meningitis1. 15mg/kg/dose q12h2. Not documented3. Not documented

Max Daily Dosage: 600mg q12h

Clindamycin IV1. 10mg/kg/dose IV q8h2. No change3. No change

Max Daily Dosage: 900mg q8h

Clindamycin PO1. 2-8mg/kg/dose q6-8h2. No change3. No change

Max Daily Dosage: 450mg q6h

Clindamycin PO — Osteomyelitis1. 10-12mg/kg/dose PO q6-q8h2. No change

3. No change

Max Daily Dosage: 450mg q6h

Erythromycin IV1. 5-10mg/kg/dose q6h2. No change3. No change

Max Daily Dosage: 1gm q6h

Ertapenem1. 15mg/kg/dose q12h 1 gram daily (≥ 13 years old)2. < 30 ml/min/1.73m2 Decrease dose 50%3. < 30 ml/min/1.73m2 Decrease dose 50%

Fluconazole IV/PO1. 6-12mg/kg/dose q24h2. 3-6mg/kg/dose q24h3. 3-6mg/kg/dose q24h

Max Daily Dosage: 400mg q24h

Fluconazole IV/PO — Fungal prophylaxis1. 3mg/kg/dose q24h2. No change3. No change

Max Daily Dosage: 400mg q24h

Ganciclovir IV1. 5mg/kg/dose q12h (>80% of normal clearance) 2.5mg/kg/dose q12h (79-50% of normal

clearance)2. 2.5mg/kg/dose q24h (25-50% of normal clearance) 1.25mg/kg/dose q24h (10-25% of normal

clearance)3. 1.25mg/kg/dose q24h

Gentamicin1. 2.5mg/kg/dose q8h **consult with pharmacist for dose adjustment/level assessment**2. 2.5mg/kg/dose q12h3. 2.5mg/kg/dose q24h

Imipenem (ID-R)1. 20mg/kg/dose q6h2. 10mg/kg/dose q6-8h3. 10mg/kg/dose q12h

Max Daily Dosage: 1gm q6h

Linezolid IV/PO (ID-R)1. 10mg/kg/dose q8h (< 5 years old) 10mg/kg/dose q12h (> 5 years old)2. No change3. No change

Max Daily Dosage: 600mg q 12h

Meropenem1. 20mg/kg/dose q8h2. 20mg/kg/dose q12h (25-50% of normal clearance) 10mg/kg/dose q12h (10-25% of normal

clearance)3. 10mg/kg/dose q24h

Max Daily Dosage: 1gm q8h

Meropenem — Meningitis1. 40mg/kg/dose q8h2. 40mg/kg/dose q12h (25-50% of normal clearance) 20mg/kg/dose q12h (10-25% of normal

clearance)3. 20mg/kg/dose q24h

Max Daily Dosage: 2gm q8h

Metronidazole IV/PO1. 10mg/kg/dose q8h2. No change3. 10mg/kg/dose q12h

Max Daily Dosage: 500mg q6h

Nafcillin1. 25-50mg/kg/dose q6h2. No change3. No change

Max Daily Dosage: 2gm q4h

Penicillin G IV1. 100,000 - 250,000 units/kg/dayq4-6h2. 70,000-160,000 units/kg/dayq8h3. 50,000-125,000 units/kg/dayq12h

Max Daily Dosage: 4 million units q4h

Penicillin G IV — Severe Infection250,000-400,000 units/kg/dayq4-6h160,000-260,000 units/kg/dayq8h125,000-200,000 units/kg/dayq8h

Max Daily Dosage: 4 million units q4h

Piperacillin/Tazobactam (Zosyn®)80mg piperacillin/kg/dose q6h-8h80mg piperacillin/kg/dose q8h80mg piperacillin/kg/dose q12h

Max Daily Dosage: 4.5gm q6h

Piperacillin/Tazobactam (Zosyn®) — Cystic fibrosis100mg piperacillin/kg/dose q6hNot documentedNot documented

Max Daily Dosage: 4.5gm q6h

Rifampin IV/PO10-20mg/kg/day in 1-3 divided dosesNo changeNo change

Max Daily Dosage: 600mg qday

Tobramycin2.5mg/kg/dose q8h **consult with pharmacist for dose adjustment/level assessment**2.5mg/kg/dose q12h2.5mg/kg/dose q24h

Tobramycin — Cystic fibrosis10mg/kg/dose q24h

Not documentedNot documented

TMP/SMX (Septra) — Mild to moderate systemic bacterial infectionSS Tablet: 80 mg TMP; DS Tablet: 160 mg TMP; Oral Suspension: 40 mg TMP / 5 mL

1. 5mg/kg/dose TMP q12h2. 2.5mg/kg/dose TMP q12h3. 2.5-5mg/kg/dose TMP q24h

TMP/SMX (Septra) — Serious systemic bacterial infectionSS Tablet: 80 mg TMP; DS Tablet: 160 mg TMP; Oral Suspension: 40 mg TMP / 5 mL

1. 5mg/kg/dose TMP q6-8h2. 5mg/kg/dose TMP q8-12h3. 5mg/kg/dose TMP q12-24h

TMP/SMX (Septra) — Pneumocystis carinii pneumonia prophylaxisSS Tablet: 80 mg TMP; DS Tablet: 160 mg TMP; Oral Suspension: 40 mg TMP / 5 mL

1. 2.5mg/kg/dose TMP q12h three days per week2. 2.5mg/kg/dose TMP q12h three days per week3. 2.5mg/kg/dose TMP q24h three days per week

Max Daily Dosage: 160mg TMP bid

VancomycinPeak levels are not recommended. Trough levels (30 min before next dose) should be 10-20 mg/Ldepending on the severity of infection. Specifically, 15-20 for meningitis, sepsis and pneumonia.

1. 15mg/kg/dose q6h (with Cardiac Dysfunction/CICU consider q8-12h)2. 15mg/kg/dose q8-12h3. 15mg/kg/dose q12-24h

Max Daily Dosage: 1 gram IV q8h

Vancomycin — CNS/Osteo/Serious InfectionsPeak levels are not recommended. Trough levels ( 30 min before next dose) should be 10-20 mg/Ldepending on the severity of infection. Specifically, 15-20 for meningitis, sepsis and pneumonia.

1. 20mg/kg/dose q6h2. 20mg/kg/dose q8h3. 20 mg/kg/dose q12h

Max Daily Dosage: 1 gram IV q6h

Voriconazole IV/PO (ID-R except for Heme-Onc) — Documented infectionsPO should be used when possible, as oral bioavailability > 95%. The use of IV should be avoided ifpossible in patients with CrCl<50 mL/min due to the accumulation of the IV vehicle

1. LD = 7mg/kg q12h x 2 dosesMD = 5-7mg/kg q12hHigher doses may be required based on therapeutic drug monitoring (consultation with Pedi-IDrecommended)

2. No change3. No change

Voriconazole IV/PO (ID-R except for Heme-Onc) — Presumed/Empiric infectionPO should be used when possible, as oral bioavailability > 95%. The use of IV should be avoided ifpossible in patients with CrCl<50 mL/min due to the accumulation of the IV vehicle

1. LD = 6mg/kg q12h x 2 dosesMD = 4mg/kg q12hHigher doses may be required based on therapeutic drug monitoring (consultation with Pedi-IDrecommended)

2. No change3. No change

Estimate of Creatinine Clearance using Schwartz’s equationCLcr = K x L/Scr (Creatinine clearance in ml/minute/1.73m2) L = Length or height in cm

K = Constant of proportionality that is age specific Scr = Serum creatinine concentration in mg/dL

Age K

Preterm infants up to 1 year 0.33

Full-term infants up to 1 year 0.45

1-12 years 0.55

13-21 years female 0.55

13-21 years male 0.7

1-16 years with Chronic Kidney Disease 0.413

Bronchodilators

Nebulizers

Albuterol2.5-5mg nebs q4h prn

Ipratropium0.25-0.5mg nebs q6h prn

Racemic epinephrine2.25% 0.25-0.5ml nebs q3h prn

Sedatives

Lorazepam0.05-0.1mg/kg/dose IV q6h prn anxiety/sedationMax=4mg/dose

Midazolam0.05-0.1mg/kg IV q1h prn anxiety/sedationMax IV/IN = 5mg/doseNasal (IN): 0.2mg/kg/dose, may repeat in 5-15 minutesOral: 0.25-0.5mg/dose, Max PO = 20mg/dose

Chloral Hydrate25-50mg/kg/dose PO/PR prior to procedure, May repeat x 1 doseMax = 1000mg/dose

Diphenhydramine (Benadryl®)0.5-1mg/kg/dose IV / PO divided q6h prnMax = 50mg/dose

Laxatives

Docusate (Colace®)<3 yr: 20mg/dose q12h3-6 yr: 30mg/dose q12h7-12 yr: 50mg/dose q12h>12 yr: 100-250mg/dose q12h

Miralax®8.5 – 17 grams ( ½ -1 capful) PO q12hOr… q24h in 4-8 ounces in clear liquid of choice

Little Tummies® laxative oral drops 1 mL = 8.8 mg sennosides.Dosing range ½ to 2 ml/day PO to begin therapy for children 6 months of age or older.

Ex·Lax Regular strength15 mg sennosides per square.Dosing range ½ to 2 squares PO daily for to begin therapy for children taking table foods.

Suppositories

Liquid glycerin 4 mL per rectum 1-3 times daily for children with severe constipation.

Other common medications

Caffeine citrate20 mg/kg/dose PO (Loading)Then 5 mg/kg/dose PO q 24 hrs (maintenance)

Diuril1-4 mg/kg/dose IV q 12 hours10-20 mg/kg/dose PO q 12 hours

Furosemide0.5-1mg/kg/dose IV q12h

Lansoprazole (Prevacid®)1 mg/kg/dose PO q 24 hrs

Lansoprazole (Prevacid®)

Weight Dosage forms Dose Frequencya

< 5kg 3mg/mlb suspension 1mg/kg/dose q24h

5-10kg Solu-Tab 7.5mg q24h

10-20kg Cap (preferred) or Solu-Tab 15mg q24h

> 20kg Cap (preferred) or Solu-Tab 30mg q24h

a Frequency may be increased to q12h depending on specific patient goalsb needs to be compounded by pharmacy

Ranitidine (Zantac®) Preferred H2 BlockerIV: 1mg/kg/dose IV q8h, Max = 15mg/dosePO: 2mg/kg/dose PO q12h, Max = 150mg/doseSyrup: 15mg/mlTablet: 75mg [OTC], 150mg, 300mg

Neonatal and Pediatric TPNInitial Orders: Order 24-hour TPN solution on pre-printed order form or in TPN Calculator forpatients <40kg. Orders must be received in the Pharmacy by 1pm.

Pediatric Nutritionists are available to advise you regarding enteral and parenteral feeding. Seeimportant phone numbers below.

1. Obtain baseline labs: Pediatric TPN panel, glucose or Chemstrip. Monitor per back of TPN form.2. Determine euvolemic weight.3. Determine energy, protein, and fluid requirements.4. See ICN guidelines for unit specific details.5. Use adult TPN form for patients > 40kg.

Important Phone NumbersNutritionists 353-1814, 353-1461Pediatric GI/Nutrition 476-5892TPN Pharmacy 353-4710

PARENTERAL NUTRIENT REQUIREMENTS*

Age Kcal/kg/day* Gm Protein/kg/day* Fluid Requirements

Preterm 80 – 110 3 – 3.5

100 – 125 ml/kg (first 10kg)

20 ml/kg (> 20 kg)

20 ml/kg (> 20 kg)

< 1yr 90 – 110 2.5 – 3

1 – 3yrs 75 – 105 1.5 – 2.5

4 – 6yrs 65 – 95 1.5 – 2

7 – 10yrs 55 – 75 1.5 – 2

11 – 18yrs 40 – 60 1 – 1.5

*see Dietitian’s Assessment for individualized requirements

Fat20% emulsion yields 2 kcl/mlTo determine volume of Intralipid (mL): g fat desired per day divided per day divided by 0.2Run lipids over 18 – 24 hours. Enter rate at bottom of formInclude lipid volume in total fluid calculation in CICU/ICN only

FAT

Neonate Older Infant/Child

Initiate 0.5 g/kg/d 1.0 g/kg/d

Advance* 0.5 g/kg/d 0.5 – 1.0 g/kg/d

Max 3 g/kg/d ≤ 0.12 g/kg/hr 4.0 g/kg/d or ≤ 60% of total calories from fat ≤ 0.15 g/kg/hr

Goal Usual intake from fat provides ~20 – 30% of total kcal

ProteinAmino acids yield 4 kcal/gTo determine % AA: g Pro desired divided by Volume of TPN (ml) x 100Peripheral line max 3.5%Trophamine or equivalent solution should be used for all infants < 1 year, patients expected toreceive PN > 2 wk or if cholestatic

PROTEIN REQUIREMENTS

Neonate Older Infant/Child

Initiate 1 – 3 g/kg/d 1 g/kg/d

Advance 0.5 g/kg/d 0.5 – 1 g/kg/d

Goal/Max* 2.5 – 3.5 g/kg/d ≤ 2.5 g/kg/d

CarbohydrateCarbohydrate yields 3.4 kcal/gPeripheral line max is 12.5% dextrose < 40kg or 10% dextrose > 40kgTo determine % Dextrose from mg CHO/kg/min:mg CHO/kg/min x wt(kg) x 1.44 = g CHOg CHO divided by Vol. of TPN (ml) x 100To determine mg CHO/kg/min from current solution: % dextrose x rate divided by wt (kg)divided by 6

CARBOHYDRATE

Neonate Older Infant/Child

Initiate 4–6 mg CHO/kg/min or D10 4–6 mg CHO/kg/min or D10–D12.5

Advance 1–3 mg CHO/kg/min or 2.5% 2–3 mg CHO/kg/min or 2.5%

Goal/Max* 12–15 mg/kg/min 8–12 mg/kg/min

*Goal depends on total caloric goal

Pediatric TPN Contact Info

Dietitian: 353-1814/353-1461 (weekdays) ; 443-4822 (weekends)Pediatric GI476-5892TPN Pharmacy 353-4710

Blood ProductsChoose size based on patient wt, consider special needs (CMV-;irrad;leukored;etc.) (S. Tasian-Oct2008)

Product Indications Usual dose Transfusion time Expected result

PRBCs Anemia 10-15 mL/kg 2-4 hours ↑ Hb 2-3 g/dL

Pedi PRBCs “pedi” or“quad” pack Anemia in smaller children 10-15 mL/kg 2-4 hours ↑ Hb 2-3 g/dL

Pedi aliquot “mini RBCs” Anemia in infants 10-15 mL/kg 2-4 hours ↑ Hb 2-3 g/dL

Apheresis single donorplatelets-eq to 6 reg units

Thrombocytopenia (usuallywith bleeding)

1 adult unit for >20-25kg child 30-60 minutes ↑ Platelets 50-

100,000

Pedi apheresis platelets Thrombocytopenia (usuallywith bleeding)

1 pedi unit for 10-15 kgchild 30-60 minutes ↑ Platelets 50-

100,000

“Quad pack” platelets Thrombocytopenia (usuallywith bleeding)

1 quad platelet for <10kg child 30-60 minutes ↑ Platelets 50-

100,000

FFP Bleeding due to longPT/PTT 10-15 mL/kg Clotting factors ↑ 10-

20% pts↑ Clotting factors10-20%

Pediatric FFP Bleeding due to longPT/PTT 10-15 mL/kg Clotting factors ↑ 10-

20% pts↑ Clotting factors10-20%

Cryoprecipitate Bleeding due to low fibrino-gen, FVIII, vWF, FXIII

1 unit/2-5 kg; 1 adultdose for child >20 kg

As quickly as possible(can be pushed slowly)

↑ Certain clottingfactors 40-100%

Product Volume Contents Type-specific

Cross-match

PRBCs 240-320 mL RBCs & plasma Yes Yes

Pedi PRBCs “pedi” or “quad” pack 80 mL RBCs & plasma Yes Yes

Pedi aliquot “mini RBCs” 30 mL RBCs & plasma Yes Yes

Apheresis single donor platelets-eq to6 reg units 250 mL Platelets & plasma Yes No

Pedi apheresis platelets 125 mL (1/2 of adult unit) Platelets & plasma Yes No

“Quad pack” platelets 50-75 mL Platelets & plasma Yes No

FFP 250 mL Clotting factors, plasma proteins Yes No

Pediatric FFP 50-7 mL Clotting factors, plasma proteins Yes No

Cryoprecipitate 1 unit = 15-20 mL; adult dose= 10 units

Fibribo-gen, vWF, factor VIII &factor XIII Yes No

Hospital Epidemiology & Infection ControlDept

Phone: 353-4343 | Fax: 353-4348 | Needlestick Hotline: 719-3898

24-hr On-call Infection Control Practitioner Pager: 443-2644

Disease or Condition* Type of Precaution Private Negative

Acinetobacter baumanii Standard

C. difficile Contact X

Cellulitis, Abscess or Wound with uncontrolled drainage Contact – duration of illness X

Chickenpox (Varicella) Airborne X X

Conjunctivitis: bacterial Standard

Conjunctivitis: viral Contact

Cytomegalovirus infection (CMV), neonatal orimmunosuppressed Standard

Diarrhea/Gastroenteritis Contact X

External parasites e.g. lice (pediculosis), scabies Contact x24 hrs afterRx

Febrile, rash, URI with travel history Airborne / Contact / Droplet X X

Group A Streptococcus (call infection control – infants& young kids)

Standard (Droplet for pharyngitis, pneumonia,scarlet fever)

Hepatitis Standard

Hepatitis A – for diapered patients Contact X

Herpes simplex Standard (call infection control for neonates)

Herpes zoster (varicella-zoster, Shingles) localized innormal patient Standard

*See infection control manual for full listing

Standard PrecautionsAll Patients Clean hands before & after pt contact, after removing gloves & upon exiting roomClean hands after contact with patient environment & equipmentClean equipment with disinfectant between patient useUse gloves for contact with blood or other body fluids, excretions, secretionsAnticipate need for protective barriers (gowns, gloves, mask, eye protection)

Place patient in appropriate roomReport all blood & body fluid exposures

Transmission-based PrecautionsRN or MD may implement based upon disease or condition

ContactPrivate roomGlove & gown upon entering roomClean hands between tasksRemove gown 1st, then gloves, then clean hands upon exiting room

DropletPrivate roomSurgical mask + eye protection w/in 3 ft of patientGloves for contact with patient environment & equipment; remove gloves & clean hands uponexiting

Airborne AFBNegative Pressure roomN-95 respiratory mask before entering room; remove & clean hands upon exitingNotify Infection Control when initiating & upon receipt of MD order to D/C

AirborneImmunity requiredNegative Pressure roomNotify Infection Control when initiating & upon receipt of MD order to D/C

Guide to Cohorting Patients with Resistant OrganismColonization or InfectionConsult with Infection Control before Cohorting Patients

Guidelines for Infected/Colonized PatientIf the answer to any of the following is “YES” for the Patient infected or colonized with a resistantorganism, a roommate is NOT ADVISED to be placed with the infected/colonized patient:

1. Patient has non-intact skin, open wounds, stasis ulcers, decubiti, burns or indwelling devices?

2. Patient has diarrhea?3. Patient has long-term fecal or bladder incontinence (i.e. body wastes not fully contained in stoma,

catheter bag or incontinence diaper)?4. Patient has other drainage that is not contained?5. Patient unwilling or unable to cooperate in strategies to contain his/her body secretions?6. Patient cognitively impaired in ways that may promote resistant organism transmission?

Roommate has non-intact skin, open wounds, stasis ulcers, decubiti, burns or indwelling devices?

Guidelines for Potential Roommate of Infected/Colonized PatientIf the answer to any of the following is “YES” for a potential Roommate of a patient infected orcolonized with a resistant organism, it is NOT ADVISED that the potential roommate be placed witha colonized/infected patient:

1. Roommate has renal failure?2. Roommate significantly immunocompromised (i.e. neutropenic or on oral steroids or

chemotherapy)?3. Roommate on antibiotics or has the roommate been given antibiotics within last 3 months?4. Roommate known to be colonized with Methicillin-resistant Staphylococcus aureus?5. Roommate unable to cooperate in the proposed infection control measures?6. Roommate cognitively impaired in ways that may prohibit compliance with precautions?