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Gastroschisis Abdominal Wall Defects Include: Embryology

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Gastroschisis Abdominal Wall Defects Include: Embryology 2/2/14 GlobalCast RN Abdominal Wall Defects: Abdominal Wall Defects Include: Gastroschisis – Gastroschisis – Omphalocele – Bladder & Cloacal Exstrophy – Prune-belly syndrome – Urachal Remnants – Umbilical cord hernia Week 6 Physiological Umbilical Herniation Embryology: • As a result of rapid growth and – Abdominal wall forms during 4th week of gestation expansion of the liver, the abdominal – During 6th week of gestation, rapid growth of cavity temporarily becomes too intestines causes herniation of the midgut into the small to contain all the intestinal loops. umbilical cord – Week 10, the midgut is returned to the abdominal • The intestinal loops enter the cavity and the small bowel and colon assumes a extraembyronic cavity within the fixed position umbilical cord during the sixth week of development. – Any disruption in process may result in an abdominal wall defect Source: Langman’s Medical Embryology. Ninth Edition. Week 10 Gastroschisis vs Omphalocele Return to Abdominal Cavity Gastroschisis Omphalocele During 10th week of development, Incidence 2-4.5:10,000 1:10,000-20,000 herniated intestinal loops begin to Defect Size 2-5 cm 1-15 cm return to the abdominal cavity. Umbilical Cord To left of defect In center of membrane Factors responsible for this return are Contents Open, exposed Covered not precisely known. Bowel Inflamed, edematous, Normal Anterior abd wall progressively closes matted leaving only an opening at the umbilical Associated 10% 60%-75% ring Anomalies Mortality < 5% 25% Source: Langman’s Medical Embryology. Ninth Edition. 1 2/2/14 Gastroschisis Etiology: • Herniation of intestinal loops Unknown: through full-thickness defect in – Thrombosis of the right umbilical vein causes anterior abdominal wall necrosis – Right omphalomesenteric artery prematurely • Defect lateral to the involutes umbilicus (right>left), usually less than 4cm in size Other theories: – In-utero rupture of omphalocele • No sac covers the extruded – Rupture of abdominal wall due to rapidly viscera increasing volume – Abnormal development of the ventral abdominal wall-failure of midline fusion of the lateral folds. Gastroschisis Risk Factors: • Incidence of gastroschisis noted increased • 4X more common in women < 20 years of past 20 years age – Incidence: 2-5 per 10,000 live births • Smoking • Young maternal age (21 years or less) • Stressed and undernourished mothers • Prematurity and low birth weights, secondary • Over the counter meds with vasoactive to intrauterine growth retardation properties: pseudoephedrine, aspirin, ephedrine • 10% incidence of associated anomalies • As compared to 60-75% in omphalocele • Multifactorial Neonatal Management- pre-op Diagnosis: • Initial assessment of airway, breathing and circulation • Sedation and/or intubation as indicated • Assess bowel viability • Often pre-natal • Place eviscerated bowel into sterile bowel bag and position midline to • Typically on routine US protect from kinking • May have a tight defect causing vascular compromise to the bowel • Slightly elevated AFP • Minimize heat loss - >36.5 C • Radiant warmer • Helpful for postnatal planning • Incubator temperature control • Pediatric surgeon availability • Warm IV fluids if indicated • Obtain IV access and begin fluid resuscitation • Counseling • Significant fluid losses from exposed bowel • Mode of delivery is controversial, but no data in • Bolus 20mL/kg NS or LR literature to support either c-section or vaginal • Infusion of D10 ¼ NS at 2-3 times maintenance • Strict Intake and Output • Monitor vital signs, including blood pressure 2 2/2/14 • Gastric Decompression • Naso or orogastric, use low continuous suction for dual-lumen Gastroschisis tubes • Infection Control • Administer antibiotics as ordered • Sterile gloves and barriers • Thorough Examination of Infant • Exclude co-existing congenital anomalies • Very careful examination of intestine looking for intestinal atresia, necrosis or perforation • Pain Management • Assess using neonatal pain scale • Assess physical responses to care, procedures • If giving benzodiazepines or narcotics careful monitoring for apnea Gastroschisis & Intestinal Atresia Surgical Management of • May be difficult to identify atresia in acute setting • Bowel reduced back into abdomen and plan for reoperation after Abdominal Wall Defects 4-12 weeks if atresia suspected by feeding intolerance and/or imaging study Key Considerations – Reduce evisceration safely – Close defect with a cosmetically acceptable outcome – Identify and treat associated anomalies – Focus on nutritional support – Recognize and treat abdominal, wound, or bowel complications Surgical Treatment Gastroschisis Goal: return the viscera to the abdominal cavity and close the defect while minimizing risk of damage to the Surgical Options for Treatment intestine from trauma or increased intra-abdominal pressure. Primary Closure Options: +/- Prosthetic Mesh – Primary Surgical Closure: Success dependent on size of the defect and size of the abdominal and Staged closure with thoracic cavities. – Spring loaded Silo – Staged Closure: Gradual reduction of the contents into the abdominal cavity using an extra-abdominal – Silastic Sheet- Sutured to medial aspect of rectus extension of the peritoneal cavity (silo) and using fascia (not used as much any more) gentle pressure. Usually requires 1-14 days, after which the defect is then closed. Sutureless closure with Tegaderm after Silo • 41 patients over past 3 years- 1 death reduction- may require ventral hernia repair later • All silo reduction, except for patients with small defects 3 2/2/14 Gastroschisis Gastroschisis Complications of Primary Fascial • Important to Measure Bladder Pressures Closure: – < 20 mm Hg • Deceased Venous Return – Monitor Ventilatory Pressures During and After • Abdominal Compartment Syndrome Closure • Pulmonary Compliance • Clinical diagnosis • Renal Failure – abdominal exam (rigid), • Necrotizing Enterocolitis – poor perfusion (urine output) – worsening ventilation (increased ventilator settings) Surgically created Silastic Silo Silo Spring loaded Silo suspended for proper orientation of bowel Spring-loaded Silo To prevent ischemia with reduction; the defect is enlarged if needed Proper orientation of bowel 4 2/2/14 Silo reduction Debate about Closure • Mortellaro et al (2011) review of the literature shows no survival difference between primary closure and delayed closure with silo. Overall survival rate with either method ranged from 90-95% • McNamara et al (2011) in a retrospective review of their patients from 2002-2008 reported >95% survival for all patients with initiation of enteral feeding earlier, decreased TPN and shorter LOS for primary closure. Also discussed the subjectiveness of criteria used for determining method of closure. • Banyard et al (2010) reported on their outcomes from 1990-2008 with similar results as reported by McNamara. • Christison-Lagay et al (2011) reported results from their prospective study that use of a spring-loaded silo was assoc with shorter time on vent, shorter LOS, lower cost and lower risk of complications when compared to historical controls. • Owen, A. et al (2010) reported in a national observation study in the UK no clear benefit of one technique over the other. Post-op/post silo placement Nursing Care Nursing Care – Strict I&O, replacement of gastric output as ordered – Post-silo support silo/bowel to avoid twisting or kinking – monitor for increased intra-abdominal pressure – Maintain urinary catheter- monitor urine output q1hr • Poor perfusion to lower extremities (minimum 1mL/kg/hr) • Decreased urine output • Bladder pressure <20mm Hg • Increased edema – Fluid management • Increased oxygen requirement, respiratory difficulty • Central venous access obtained • For silo- frequently assess bowel; should be pink, fluid in bag • Increased fluid and albumin needs –fluids 120-140mL/kg/day, albumin serous and free of stool as needed • TPN, replacement of gastric output • Maintain temperature >36.5 C • Strict I&O • Naso or orogastric decompression: close attention to assure – Respiratory working properly- irrigate every 4 hours and prn with air via air • Monitor for distress (especially after closure) port and NS via drainage port • Monitor pulse oximetry and ABG’s • Maintain sat >95 • Intubated, sedated if required Nursing Care Nursing Care – Skin Care – Infection Prevention • Monitor pulses, temperature, color of extremities • Sterile technique during dressing changes • Consider positioning, especially if using silo • Antibiotics as ordered • Consider gestational age • Dressing around silo per your institutions routine- betadine soaked gauze, changed bid • Report drainage or dehiscence • Monitor for separation, redness or drainage at base of silo or suture line – Discharge Planning post closure • Teaching regarding feeding method (PO, NG, GT, TPN if • CVC care per policy necessary) HHC arrangements • Temperature, vital signs, labs • Teach CPR prior to discharge, Car seat is appropriate – Pain Management • Alert family to call MD office/ED for s/s of bowel obstruction/ • Assess using neonatal pain scale hourly during initially post-op period volvulus-(bilious emesis, abd distention, no stool output, • Monitor physical parameters diarrhea) • Use behavioral interventions and medications as appropriate • Assure follow-up is arranged Refer to: Nursing Care of the Pediatric Surgical Patient (2013) pp 287-289 5 2/2/14 Post-op complications: Gastroschisis • Abdominal compartment syndrome – ischemic, infarcted • Prognosis is dependent
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