Acute Umbilical Complaints in the Pediatric ER

Or

“my babies navel looks/smells funny”

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Issues Umbilical - Case 1• 9 week girl infant. Presents to PLC-ER• Swelling of the umbilicus for ~5 hours• Erythema and a central Umbilical “lump”

noted• No fever• Some poor feeding with no vomiting for less

than a day• ~6 wet diapers past 24 hours

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Issues Umbilical - Case 1• 5.11 kg, Cap refill <3 sec• T 36.4, R28, P 145, BP 78/49• Alert, no distress• N H&N• N chest and HS• Soft benign abdomen with no masses• Central, red umbilical bulge within skin cuff

(cushion)• Small volume thin purulent drainage?• Slight erythema 4-7o’clock? No induration or

demarcation

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Issues Umbilical• Referred to ACH-ER with:

“? Umbilical hernia, R/O Omphalitis”

ACH ER exam similar overall• C&S of Umbilical “discharge”• CBC, Lytes• Felt likely to be Omphalitis• Referral to General Surgery• Ancef 25 mg/kg commenced

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Issues Umbilical• CBC• WBC 9.7, Neuts 2.6• Hb 106, Platlets 522

• Na 138, Cl 103, K 4.7, HCO3 23

• Cr 17, Urea 2.2• U/A neg

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Issues Umbilical• General Surgical Opinion (in the am!)• Likely omphalitis• Consider infected urachal remnant

• Admitted• Change to IV clindamycin• U/S booked

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Issues umbilical• In Hospital course• Always remained afebrile• C&S umbilical discharge “scant skin flora

only”• U/S abdomen:• Swollen protruding umbilicus noted to be filled

with echogenic material. A sinus tract is identified which extends form the lower umbilicus and connects to the superior and anterior wall of the bladder in the midline. The appearance is consistent with a patent urachus.

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Issues Umbilical• Day 4 • Discharged home for urgent elective repair

to be booked• Clindamycin oral course

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Objectives of Naval Mission• Discuss omphalitis • Discuss common cord care• Understand the non-infectious

abnormalities that can occur in the umbilicus, notably in the infant

• Not to discuss • Umbilical hernia management• Case room cord examination and

implications

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Normal Cord care• Policies vary greatly in developing vs

developed countries• Marked decrease in incidence of Omphalitis in

developed countries • ~0.7% vs up to 6 %

• In developed countries:• Cochrane review shows no form of cord

cleaning/antiseptic is better than dry cord care

• In developing countries antiseptics in cord care markedly decrease death and omphalitis (chlorhexidine, AgSulfadiazine, Triple dye…)

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Cord Separation• Normal timing of ~1 week or less for

separation• Prolonged by certain agents• 70% alcohol: ~17 days• Triple dye: 3-8 weeks

• True “delayed” separation (without agent application) is in excess of 3 weeks

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Umbilical infection• All cords are nearly immediately

colonized • Staph and other gm+ves within hours• Enteric organisms shortly thereafter

• Devitalized tissue is a good bacterial growth medium

• Mild discharge and absent inflammatory change, even with some odor is usually still a normal occurrence.• No proof for or against Rx with Alcohol, Bacitracin

or Mupirocin…but many choose this.

• When does this constitute early Omphalitis?

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Omphalitis• Predominately Neonatal• Mean age of onset in term infants is 3.5

days• Infection of umbilicus and/or surrounding

tissues• Purulent (+/-bloody) drainage from stump• Surrounding induration, erythema,

tenderness

• BUT• Lethargy, fever, Irritability, poor feeding

suggest more severe infection/impending sepsis

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Omphalitis

• Complications:• Sepsis / death• Septic umbilical arteritis/portal vein thrombosis• Peritonitis/liver abscess/intestinal gangrene• Small bowel evisceration• Necrotizing fasciitis

• Present-day Mortality: 7-15%

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Case 2• 14 day infant girl transferred to ACH-ICU

for umbilical infection• 41 weeks GA• C/S for fetal distress• APGARs 81 & 95

• GBS+ve • Passed N mec. At 24 hours • No jaundice• Breast fed/BM 8x/day• Cord loss ~1 week of age

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Case 2• Day 11• Some peri-umbilical redness, afebrile• Poor evening feeding

• Day 12• Worsening erythema, wider area• Abdomen appeared “puffy”• T = 38.50C• To local community hospital; blood-streaked stool in

ED, and with all serial later BMs• Much worse feeding and lethargy• Sepsis workup/LP/Ampicillin and Cefotaxime and

admitted

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Case 2• Day 13• General progression of anorexia, and

increasing abdo wall abnormalities. • U/S abdomen, and transferred to ACH

overnight

• Day 14 ACH - PICU• Change to Flagyl, Meropenum, Clindamycin.

And Gentamycin• Surgery/Plastics consult

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Case 2• Physical• 88/60, 153,100%RA, 37.5, 40, 4.0Kg• AF flat, no jaundice• CVS N save CRT “2-5 seconds”• No increased WOB• Mottled extremities• Distended abdomen. Black umbilicus,

surrounded by an inner purple and outer white halo, both non-blanching. Rt > Lt, ~30% of abdo wall

• Whole remainder of abdomen wall is erythematous

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Case 2• Lab • WBC of 33.7• CRP 72.8• Hb 148, Platlets 501

• To ACH-OR for debridement, and bowel inspection for R/O NEC• Abdo wall biopsy and C&S• Bowel observed to be vital without NEC• Umbilicus and surrounding tissues resected

including necrotic skin and abdo. wall to healthy fascia

• Frozen section biopsy consistent with Nec Faciitis

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Case 2: Intra-operative, Post Umbilical Resection

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Case 2: Intra-operative, Post Umbilical Resection

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Case 2• OR visits on PICU-days 1,2,4,6 and 8 for

serial lesser debridements and bowel inspection

• Wound closure PICU day 8 but subsequent dehisence day 19

• Change to tazocin/vancomycin day 7• Wound grew • Enterococcus faecalis• Coag neg Staph• Actinomyces

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Case 2

• Day 12 - extubated• Day 13 - to the ward• Day 19 - Wound dehisced• Day 30 - discharged home• All Abx discontinued • planned delayed closure abdo. wall ~2

weeks later

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Omphalitis• Risk factors• LBW• Prolonged labor• PROM• Non-sterile delivery• Umb.A. cathetrization• Home birth• Improper cord care

• (cow dung, bentonite clay)

• Immune abnormalities

• Poorer Prognosis• Male• Premature• “Septic delivery”

• (including un-planned home delivery)

• Temperature instability• Necrotizing fasciitis

• (up to 85% mortality)

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Omphalitis/Any Soft Tissue Infection

• There is a continuum of severity:

CellulitisInfection of skin and S.C. fat

Necrotizing fasciitisInfection of skin, S.C. fat and superficial and deep fasciae

Myositis/myonecrosis

Deep muscle infection with muscle death

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Omphalitis/Any Aggressive Soft Tissue Infection

• Should be presumed to be poly-microbial at outset• “the usual suspects” in Omphalitis:• Staph Aureus• Gp A Strep• Coag Neg Staph• Enterococci• Gm Negs: E Coli, Klebsiella P., Proteus Mirabilis…• Anerobes: Bacteroides, Clostridium

perfingens/tetani

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Omphalitis• Pathology of infection is presumed to be

polymicrobial from the outset• Abx must cover for this, and include:

• Anti-stahpylococcal penicilin or vancomycin• Aminoglycoside• Probable Clinamycin or Metronidazole

Esp. if maternal chorioamnionitis and/or foul discharge, for anaerobic coverage

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Omphalitis

• Necrotizing Fasciitis• Rare complication of omphalitis• Polymicrobial• Involves skin, subcutis, superficial and deep

fasciae• Rapid spread is typical• Bacteremia, systemic toxicity, and shock in

high proportion. Death 60-85%• Early aggressive surgical intervention, broad

spectrum antibiotics, and supportive ICU care

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Case 3• 38 2/7 week boy• 30 yr G1P1 mother, N Vtx Vag delivery• APGARs 81 and 85

• Short ACH transfer Day 1-3 for ?ileal atresia…final Dx Meconium plug

• Day 13• Peri-umbilical redness noted by family

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Case 3• Day 14• Admitted to local hospital• Dx Omphalitis• Ampicillin and Gentamycin

• Day 15• Increasing redness in abrupt fashion: 5cm

above and 3cm below umbilicus• Transfer to ACH ICU• Dx Omphalitis, R/O Necrotizing Fasciitis

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Case 3• ICU:• Not toxic• Abdo wall is only abnormality of serious note• WBC 16.5, N diff, INR N, Lytes N and Neg AG• Urgent tissue biopsy• No Nec Fasciitis; consistent with cellulitis• Neg gram stain

• Neg blood and urine C&S.• Surface Umb C&S from Primary hospital• Coag neg staph, and enterococcus faecalis

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Case 3• I.D. Service: Antibiotics changed to • Meropenum, Clindamycin, and Gentamycin

• Day 16• Child improves sufficiently that ward transfer

is in process…..then oliguria unresponsive to fluids arises

• Scrotal swelling and severe progressive abdominal wall edema

• ICU stay maintained

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Case 3• Day 17• 03:00 Resp failure/ETT• 05:00 dobutamine infusion• 05-10:00 progressive metabolic acidosis• 10:00 to OR• Abdominal exploration. Healthy bowel. • Abdo wall : Excision of navel and surrounding

tissue. Biopsy now positive for Necrotizing fasciitis

• Deterioration: • with coagulopathy, WBC up to 49.5, INR elevated,

ARDS / pulmonary hemorrhage

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Case 3

• Day 17• Progressive deterioration and difficulty

ventilating. Rising Cr up to 180• 13:30 back to OR• Abdominal compartment syndrome• Bowel “eviscerates” under pressure and

ventilatability markedly improves…bowel seems healthy; Abdo Wall Margins still look healthy, and back to ICU with bowel encased in a “silo bag”

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Case 3• Severe oliguria• Lines placed and dialysis commenced• Poor tolerance with repeated hypotension and need

for fluid bolusing

• Day 18• Several bradycardic arrests• Progressive instablilty and dialysis

discontinued• Family agree to discontinue all supportive Rx• 04:20 child pronounced

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Case 3• C&S from initial umbilical ACH biopsy• Coag neg staph• Enterococcus faecalis• Clostridium sordellii

• Autopsy conclusion• Necrotizing faciitis of poly-microbial nature• Sepsis

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Conclusion

Respect Omphalitis

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Something is wrong with my babies Navel

• Umbilical Granuloma

• Omphalo-mesenteric duct remnants

• Urachal remnants

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Case 4• 12 day infant girl

• 41 3/7 weeks, vacuum assisted SVD• GBS -ve• Thriving• Cord dehisced day 7• Umbilicus raw, oozing with sero-sanguinous

discharge since

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Case 4• Looks well• P 165, R 26, T 37.1, BP 76/42• General Exam Normal• No peri-umbilical redness• Moist “nodule” of pinkish-red tissue over

stump site. Bleeds easily

• ?Umbilical Granuloma (vs some other developmental lesion)…Referred to Surgery Clinic DDR

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Case 4• In clinic 1 month later• Major lump had “fallen off” and moist base

was cauterized with AgNO3

• Re seen 3 weeks later:• Area dry and fully healed

• Diagnosis:

Umbilical Granuloma

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Umbilical Granuloma• Most common cause of umbilical mass

and umbilical drainage• Usually post cord separation• Persistent drainage of serous or sero-

sanguinous fluid around the umbilicus• A mass of pink granulation tissue at

umbilical base• Moist• Pink• Friable• Soft• Often pedunculated • Usually 3-10 mm

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Umbilical Granuloma• Treatment:

• AgNO3 local Rx 1-2 x per week

• If it persists post 3-4 Rx sessions • Can be ligated (be sure its not a polyp!) or

referred to general surgery for formal excision

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Omphalo-mesenteric Duct Remnants

• Omphalo-mesenteric duct (Viteline duct):

• Connects the developing GI tract to yolk sack

• Regresses by ~9th week GA• Disruption of this regression causes the list

of abnormalities:

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Vitelline, or Omphalomesenteric Duct Embryology

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OMD Remnants• Umbilical fistula• Complete patency of OMD with stoma-like

connection to the terminal ileum

• Partial persistence of OMD• Fibrous band umbilicus to ileum• “Distal” remnant - OMD-enteric cyst• “Proximal” remnant - Meckel’s diverticulum• Umbilical polyp - a mucosal remnant in the

umbilical stump

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OMD remnants• Fibrous band • can cause volvulus; obstruction and/or volvulus

are most common infant presentation

• Umbilical Polyp• Usually enteric, but occasionally urachal origin.

Rarely pancreas, liver• Firm masses. No response to AgNO3,and must be

surgically excised

• OMD cyst • often asymptomatic, or may be an umbilical or

abdominal mass; occasionally infected

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Urachal Remnants• Urachus is the embryologic descendant

of the allantois.

• Allantois is the most distal projection of the primitive gut, projecting into the extra-embryonic cord. Of it’s Intra-embryonic portions:• The bladder = proximal portion.• The urachus = more distal portion.

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The Allantois

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Urachal Remnants• Urachal fistula - complete patency of the

urachus• Urachal cyst - remnant along tract

(usually lower 1/3)

• Urachal sinus Blind umbilical tract, unconnected to the bladder

• Vesico-urachal diverticulumAntero-superior midline bladder dome

• Umbilical (urachal) polyp

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Urachal Remnants• Ultrasound is the ideal investigation for

initial definition

• Sinogram for patent urachus (“fistula”) or urachal sinus are other options

• Renal U/S and VCUG have also been recommended

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Urachal Remnants• Presentations:• May be subtle with erythema +/- drainage

• Umbilical discharge or Omphalitis spectrum

• Umbilical pain or retraction on micturition

• Umbilical mass or cyst

• Peri-umbilical pain

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Urachal Remnants• All need to be excised

• In adults, 50% have malignant (adneocarcinoma) changes at the time of excision (nil in children)

• Cuff of normal bladder mucosa is excised during resection

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Questions?

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References1) Vane D.W. et al “Viteline Duct Abnormalities:

Experience with 217 Childhood Cases Arch surg122:542, 1987

2) Pomeranz A. “Anomalies, Abnormalities and Care of the Umbilicus” Pediatric Clinics of N.A. 51:819, 2004

3) Rescorla F. J. “Hernias and Umbilicus” in Principles and Practice of Pediatric Surgery, volume 2, 2005

4) Cilento B. G. et al “Urachal Anomalies: Defining the Best Diagnostic Modalitiy” Urology52:120, 1998.

5) Ashley R.A. et al “Urachal Anomalies: a Longitudinal Study of Urachal Remnants in Children and Adults” J Urol 178:1615, 2007

6) Cushing A.H. “Omphalitis: A Review”Pediatr Infect Dis 2:282, 1985

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References7) Sawardekar K.P. “changing Spectrum of Neonatal

Omphalitis” Pediatr Infect Dis J 23:22, 20048) Mason W.H.et al “Omphalitis in the Newborn

Infant”Pediatr Inf Dis J 8:521, 19899) Kosloske A.M. “Cellulitis and Necrotizing Fasciitis of the

Abdominal Wall in Pediatric Patients”. J Pediatric Surg 16:246-251, 1981

10)Simon N.P. “Changes in Newborn Bathing Practices may Increase the Risk for Omphalitis” Clin Pediatr 43>763-767, 2004

11) Louie J.P. “Essential Diagnosis of Abdominal Emergencies in the First Year of Life”Emer Med. Clinics of N A 25:1009-1040

12) Zupan J. et al “Topical Umbilical Cord Care at Birth(Review)”Cochrane Library 2008, Issue 3

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References13) Mullany L.C et al “Development of a Clinical Sign Based

Algorithm for Community Based Assessment of Omphalitis” Arch Dis. Child. Fetal Neonatal Ed. 91:F91-F104, 2006

14) Mullany, L.C. “Topical Applications of Chlorhexidine to the Umbilical Cord for Prevention of Omphalitis and Neonatal Mortality n Southern Nepal: a Community-based, Cluster-randomized Trial” Lancet 367:910, 2006

15) Hseih, W.S. et al “Neonatal Necrotizing Fasciitis: A report of Three Cases abd Review of the Literature” Pediatrics103:e53, 1999

16) Iacono, G. “Red Umbilicus”:a Diagnostic Sign of Cow’s Milk Protein Intolerance. J. Ped.Gastro. And Nutr. 42:531-534, 2006

17) Burd R.S. et al “Evaluation and Initial Management of Miscellaneous Pediatric Surgical Problems”Pediatric Annals30:752-759, 2001