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Pediatric Abdominal Wall Defects
L Yuko Shimotake Richmond University Medical Center September 24, 2015
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Case Presentation
• HPI: 37.6 week gestational age female born via C-section
• Maternal Hx: 21yo G0110, GBS+, no prior medical problems
• Prenatal care: diagnosed with Gastroschisis at 12 weeks gestation, monitored via serial US
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Case Presentation • Birth: 2410g, APGAR 9/9
• Defect - 4.5cm abdominal wall defect involving stomach, small bowel, rectosigmoid colon, bladder, internal genitalia
• No other abnormalities appreciated - patent anus
• Vitals 98.0F 69/35 172 40 100%
16.2 14.4
45.9 396
137
4.0
105 7 367
19 0.8
31
3.2 16 0.6
39 5.4
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Case Presentation
• Isolation bag with saline, NPO, IV hydration, Ampicillin/Gentamycin
• Echocardiogram 8/12 - patent foramen ovale, slight mitral regurgitation
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Day of Life 1 • Intubation, OG tube
placement
• Extension of abdominal wall defect and reduction of contents
• Respiratory depression – O2 sat to 40%
• 7.5cm silo application
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Day of Life 6
• Indication: ill-fitting of silo
• Lysis of adhesions, re-application of silo
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Day of Life 9 • Indication: Open
abdomen, inability to reduce bowel
• Skin flap creation to flanks
• 8x5 inch Goretex mesh application
• Skin closed over
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Day of Life 22 • Indication: Central skin
necrosis
• Excision of necrotic skin and Goretex graft
• Attempted primary closure with noted mottling of lower extremities
• Application of 6.4cm Ventralex mesh
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Day of Life 29
• Indication: Abdominal wall closure, skin dehiscence
• Mesh removal
• Approximation of fascia and abdominal wall
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Today - doing well!
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Questions?
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Pediatric Abdominal Wall Defects
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Outline • Embryologic development of abdominal wall
• Gastroschisis
• Omphalocele
• Prenatal care
• Neonatal care
• Surgical options
• Post-operative concerns
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Weeks 3-5 Abdominal Wall Development
• Folding of embryo in 4 planes ventral ring (future umbilicus)
• cranial
• caudal
• left and right lateral
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Week 6 Physiologic Midgut Hernia
• Rapid growth of intra-abdominal contents protrusion of midgut
• via ventral ring
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Week 10 Reduction of Hernia
• 270 degree counterclockwise midgut rotation around SMA
• Return of abdominal contents into abdominal cavity
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Gastroschisis
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Gastroschisis • Incidence – 1-2/10,000
• Etiology - vascular accident
• involution of umbilical vein full thickness defect of abdominal wall
• premature involution of right vitalline artery
• Risk Factors
• smoking
• young maternal age <20yo
• maternal medications - vasoactive medications - pseudoephedrine, aspirin
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Gastroschisis - Hernia
• 2-5cm defect
• right>left of umbilicus
• Contents - small and large bowel, rarely liver
• NO SAC - freely protruding contents
• Appears thickened, dry and matted intestines
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Gastroschisis • Associations
• prematurity
• cryptorchidism 30%
• GERD 16%
• intestinal atresia 10%
• Mortality <5%
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Omphalocele
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Omphalocele
• Incidence – 1/10,000
• Etiology
• failure of midgut regression
• incomplete lateral body wall fold - larger defect involving liver
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Omphalocele - hernia
• 1-15cm defect
• Umbilical defect
• Contents - normal appearing small bowel, spleen, liver
• SAC - covered by peritoneal/amniotic sac - can rupture
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Omphalocele • Associated malformations 60%
• pulmonary hypoplasia
• cardiac - VSD is most common
• renal, limb, facial abnormalities
• Pentalogy of Cantrell
• Beckwith-Wiedemann Syndrome
• trisomy 13,14,15,18
• smaller defect without liver has higher risk of chromosomal abnormalities
• Mortality 25% - due to chromosomal abnormalities
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Pentalogy of Cantrell • Omphalocele
• Anterior diaphragmatic hernia defects
• Sternal cleft
• Ectopia cordis
• Intracardiac defect - VSD
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Beckwith-Wiedemann Syndrome
• Macroglossia
• Macrosomia
• Ear creases or ear pits
• Neonatal hypoglycemia
• Midline abdominal wall defects - omphalocele, umbilical hernia, rectus diastasis
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High Yield Gastroschisis Omphalocele
size small small or large
location periumbilical (right>left) umbilical
contents midgut, bladder midgut, stomach, spleen, liver
sac none 2 layers of amniotic peritoneal membrane
bowel appearance matted, edematous normal
associated conditions
intestinal atresia, prematurity, GERD,
cryptorchidism
cardiac anomalies, renal defects, limb defects, pentalogy of cantrell,
BWS, trisomies
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Prenatal Testing • Ultrasound
• Elevated maternal and amniotic alpha-fetoprotein levels
• Polyhydramnios
• Amniotic karytotyping if omphalocele
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Delivery • Involve obstetrics, NICU, pediatric surgery teams
• Vaginal delivery versus C-section - no difference
• Spontaneous labor versus induction
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Neonatal Care • Fluid management
• Nutritional support
• Identify and treat associated anomalies
• Bowel status
• Cosmetic and safe closure of abdominal wall defect
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Neonatal Care • Fluid management
• Nutritional support
• Identify and treat associated anomalies
• Bowel status
• Cosmetic and safe closure of abdominal wall defect
• Saline gauze dressing and bowel bag - minimize heat loss
• IV fluids - resuscitative versus maintenance
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Neonatal Care • Fluid management
• Nutritional support
• Identify and treat associated anomalies
• Bowel status
• Cosmetic and safe closure of abdominal wall defect
• IV fluids
• albumin
• TPN
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Neonatal Care • Fluid management
• Nutritional support
• Identify and treat associated anomalies
• Bowel status
• Cosmetic and safe closure of abdominal wall defect
• Non-ruptured Omphalocele
• Chest X-ray
• Echocardiogram
• Renal US
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Neonatal Care • Fluid management
• Nutritional support
• Identify and treat associated anomalies
• Bowel status
• Cosmetic and safe closure of abdominal wall defect
• 10-15% intestinal injury
• Consider resection with primary anastomosis
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Neonatal Care • Fluid management
• Nutritional support
• Identify and treat associated anomalies
• Bowel status
• Cosmetic and safe closure of abdominal wall defect
• Primary closure
• Staged closure
• Delayed closure
• Sutureless closure
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Primary Closure • Reduce abdominal contents
• Circumferential dissection
• Excision and ligation of sac
• Fascial closure with mattress sutures
• Recreation of umbilicus with purse string
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Primary Closure
• For larger defects
• Flap creation
• Component separation
• Mesh - bioabsorbable
• Tissue expanders
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Staged Closure • Excision of sac
• Application of Silo bag
• Slow reduction of abdominal contents - 7-10 days
• Return to OR for fascial closure
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Delayed Closure • Indication: severe
congenital anomalies
• Allow for epithelialization of omphalocele sac - Silvadene or Povidone-Iodine
• Delayed ventral hernia repair
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Sutureless Closure • Leave umbilical cord long
• Reduction of hernia contents
• Umbilical cord plug with overlying adhesive dressing
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• 10 patients - 6 primary reduction with simple “plastic” closure; 4 “plastic” closure after silo placement
• Results - 2 required bowel resection, 6 umbilical hernias managed conservatively, 1 required repair at 13mo
• Conclusion - limits abdominal compartment syndrome and sequelae, more cosmetic
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Post-operative Concerns • Short-term
• Monitor for compartment syndrome - ventilatory pressures, urine output, limb perfusion
• Wound care
• Infection/Sepsis
• Necrotizing enterocolitis
• Nutritional support - TPN
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Post-operative Concerns • Short-term
• Monitor for compartment syndrome - ventilatory pressures, urine output, limb perfusion
• Wound care
• Infection/Sepsis
• Necrotizing enterocolitis
• Nutritional support - TPN
• Long-term
• Need for umbilicoplasty/ventral hernia repair
• Adhesive small bowel obstruction
• Sequelae of associated anomalies
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Summary
• Embryology is complex
• Gastroschisis versus Omphalocele
• Neonatal care is important
• Multitude of surgical options
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Quick Quiz • Which of the following is NOT part of the Pentalogy
of Cantrell?
• A. Omphalocele
• B. Ectopic cordis
• C. Posterolateral diaphragmatic hernia
• D. Sternal cleft
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Quick Quiz • Which of the following is NOT part of the Pentalogy
of Cantrell?
• A. Omphalocele
• B. Ectopic cordis
• C. Posterolateral diaphragmatic hernia
• D. Sternal cleft
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Quick Quiz • Which is more commonly seen with Gastroschisis
than Omphalocele?
• A. Pyloric stenosis
• B. Chromosomal abnormalities
• C. Prompt bowel function
• D. Young maternal age
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Quick Quiz • Which is more commonly seen with Gastroschisis
than Omphalocele?
• A. Pyloric stenosis
• B. Chromosomal abnormalities
• C. Prompt bowel function
• D. Young maternal age
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Thank You!
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References • Ledbetter, DJ Congenital Abdominal Wall Defects and Reconstruction in Pediatric
Surgery: Gastroschisis and Omphalocele. Surg Clin N Am 92 (2012) 713-27.
• Rubens Figueroa, J et al. Rev Esp Cardiol, 2011; 64: 615-8, Vol 64, Num 07
• Abramowicz, JS. First-Trimester Ultrasound: A Comprehensive Guide. Springer, 2015
• Swanson, J et al. Gastroschisis Versus Omphalocele/Exomphalos. CME credits
• Grosfeld, Pediatric Surgery 6th edition
• Pansky, B Embryonic Folding and Flexing of the Embryo. Review of Medical Embryology.
• Sandler, A et al A “Plastic” Sutureless Abdominal Wall Closure in Gastroschisis, 2004
• Brant, et al. Schwartz’s Principles of Surgery, 9th edition