gastroschisis final presentation

GOOD MORNING!

General Objective

To present a case of an anterior abdominal wall defect

Specific Objectives To discuss the presentation, causes and prognosis of

gastroschisis

To discuss the medical and surgical management of gastroschisis

To compare the two most common anterior abdominal wall defects: omphalocoele and gastroschisis

General Data

• Baby Boy V

• newborn

• Trancoville, Baguio City

• born and admitted:

February 17,2010 at 1:53pm

Chief

Complaint

Pre-natal History

• Mother: 17 y/o, G1P0

• housekeeper

• high school graduate

• denied family history of congenital anomalies and heredofamilial diseases

• cognizant: 3rd month AOG• 1st PNCU- 4th month AOG*• regular intake of Multivitamins

Pre-natal History

• URTI : 5month AOG• (-) exposure

viral exanthematous diseases, radiation, alcohol beverages and cigarette

Pre-natal History

• LMP- May 29,2009

• EDC by LMP – March 5, 2010

• AOG by LMP- 38-39wks

12 days prior to delivery……

Single, live intrauterine fetus in cephalic presentation, 32weeks and 3 days AOG.

Extra-abdominal tubular structures probably bowel loops due to an anterior wall defect (gastroschisis)

Perinatal History 13 hours prior to delivery

↓

labor pains

↓

8 hours prior to delivery

↓ BGHMC-ER

• single, live intrauterine pregnancy in cephalic presentation, 33weeks AOG by fetal biometry

• posterior placenta Grade II-III maturity, modified BPPS of 8/8, normohydramnios

• consider fetal abdominal defect probably gastroschisis

• expected date of delivery: 3/31/10• expected fetal weight: 2285g

Natal HistoryAPGAR SCORE

0 1 2 SCORE

1min 5min

Heart rate Absent <100 >100 2 2Resp. effort

Absent Weak cry Loud cry 2 2Muscle tone Flaccid Some

flexionActive 2 2

Reflex No response

Grimace Cough / sneeze 1 2

Color Blue, pale

Body pink ext. blue

Pink 1 1TOTAL SCORE 8 9

BALLARD SCORING

19

Ballard Scoring

19

PHYSICAL EXAMINATION

• General Survey: active, hypothermic with good and loud cry, in mild respiratory distress

• Vital Signs– respiratory rate: 48/min

– cardiac rate: 147/min

– temperature: 36.1ºC

ANTHROPOMETRIC MEASUREMENTS

• Birthweight:1.9 kg (below 10th percentile)

• Birth length: 42 cm (below 10th percentile)

• Head circumference: 31cm

(below 10th percentile)

• Chest circumference: 27cm

• Abdominal circumference: 26cm

• Arm:10cm

PHYSICAL EXAMINATION

• Skin: pink body, blue extremities, with cracking, pale areas, rare veins and bald areas of lanugo

• HEENT: normocephalic, non-bulging anterior and posterior fontanelles, no facial asymmetry, anicteric sclerae, ears are formed and firm with instant recoil, (+) alar flaring, no cleft lip, no cleft palate

PHYSICAL EXAMINATION

• Chest and lungs: symmetrical chest wall expansion, (+) grunting respiration, no tachypnea, no retractions, good and equal air entry

• Heart: adynamic precordium, normal rate, regular rhythm, PMI at 4th ICS, LMCL,no murmurs

Abdomen

Scaphoid,

(+) evisceration of

edematous intestines,

no sac, noted at the

paraumbilical area,

right, abdominal wall defect measures 2.5 cms by 2.5 cms, with intact umbilicus,

• Ano-genital:grossly male, testes down, good rugae, patent anus

• Extremities: no gross deformities, pink nail beds, creases over all over, equal and full peripheral pulse, good capillary refill

PHYSICALEXAMINATION

• term, newborn, male

• scaphoid abdomen,

• (+) evisceration of edematous intestines, no sac, noted at the paraumbilical area, right, abdominal defect measures 2.5 by 2.5 cms, with intact umbilicus

SALIENT FEATURES

ASSESSMENTTerm, male, 38-39 weeks Age of

Gestation by Ballard Scoring, born via “E” Low Segment Cesarian Section, for Fetal Abdominal

defect, and Non-reassuring Fetal Status with a Birthweight of 1.9 kg, Low Birth Weight, Small for gestational Age, Abdominal

wall defect, Gastroschisis

GASTROSCHISIS OMPHALOCELE

O

Gastroschisis

• covering is absent• located

paraumbilical,right• 2-4cm• IUGR is common • associated anomalies

less common• herniated organs are

intestines

Omphalocele

• covering is present• located midline

• 2-15cm• IUGR is not common• associated anomalies

more common• herniated organs are

intestine, stomach, liver, spleen

nelson's 16th edition

On admission…..Patient immediately brought in the Nursery

Diagnostics:– CBC, Platelet Count, Typing, Blood

C&S,

Hgb- 151

Hct- 0.49

WBC- 33.9 (neu-.22,lymp-.71,mid cells-.07)

Plt.ct-535– Hemogluco test: 80 mg/dl

– CXR

Therapeutics:

– Routine Newborn care was done– D10W with TFR-60cc/kg as maintenance

line, and PNSS at 10 cc/kg/hour until OR– FFP*– Ampicillin IV at 50mg/kg/dose– Amikacin IV at 15mg kg/dose– Metronidazole at 15mg/kg as loading dose,

7.5mg/kg as maintenance dose

– immediately referred to Pediatric surgery

– scheduled for “E” primary abdominal wall repair

postoperatively……..

patient was immediately brought Neonatal Intensive Care Unit for post-op care

• occurs in approximately 1 in 5,000 births

• sporadic

• few familial cases

• occurrence in twins has been reported

GASTROSCHISIS

textbook of surgery- Schwartz

• herniation of abdominal contents through a paramedian full-thickness abdominal fusion defect

• the abdominal herniation is usually to the right of the umbilical cord

• no genetic association exists

GASTROSCHISIS

• usually contains small bowel and has no surrounding membrane

• the herniated bowel is non-rotated and devoid of secondary fixation to the posterior abdominal wall

GASTROSCHISIS

• intestine maybe normal in appearance

• thick, edematous, discolored and covered with exudates, implying a more long standing process

GASTROSCHISIS

textbook of surgery- Schwartz

• controversy exists regarding the cause of gastroschisis

• defect is caused by abnormal involution of the right umbilical vein*

textbook of surgery- Schwartz

PATHOPHYSIOLOGY

• antenatal detection rates are 70-72%

• prenatal sonography: primary imaging modality

• fetal AFP level: 200-300 times as high as the concentration in amniotic fluid

DIAGNOSIS

• Primary gastroschisis repair– the baby undergoes surgical

repair immediately after birth

– this is the preferred method of repair because it is associated with a reduced risk of infection and fluid loss

TREATMENT

textbook of surgery- Schwartz

• Staged gastroschisis repair

– the bowel is covered by a sheet of protective material and allows graduated reduction on a daily basis as the edema in the bowel decreases.

textbook of surgery- Schwartz

• A Silastic (silicone plastic) pouch is first placed over the baby's exposed bowel and anchored to the surrounding muscle. Each day, the pouch is tightened to push the intestine back into the abdominal cavity.

textbook of surgery-schwartz

• maintenance fluids + deficit computed at 10-20cc/kg per number of hours herniated organs exposed

• run PNSS in between transfusion of FFP and albumin

• run maintenance fluid on a separate line

FLUID MANAGEMENT

nelson's 16th edition

MORTALITY AND MORBIDITY

• postoperative hospital stay is often lengthy • complications occur frequently, especially

related to the gastrointestinal tract. • survival rates after surgery are 87-100%. • mortality rate of 17%

textbook of surgery- Schwartz

MORTALITY AND MORBIDITY• most deaths occur as a result of premature

delivery, sepsis, and bowel infarction

• poor prognosis:

– evidence of bowel damage, such as atresia

– necrosis, or severe dilatation or thickening of the bowel, or the inability to close the abdominal defect

textbook of surgery- schwartz

Thank youThank you