2/2/14

GlobalCast RN

Abdominal Wall Defects: Abdominal Wall Defects Include:

– Gastroschisis – – Bladder & Cloacal Exstrophy – Prune-belly syndrome – Urachal Remnants – Umbilical cord

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 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 mainly upon severity bowel, renal failure of associated problems • Infection – Prematurity • Necrotizing enterocolitis – Intestinal atresia (10-15%) • Short gut syndrome with TPN dependence – Short Gut-loss of bowel due to ischemia, infarction – NEC (5-10%) • Prolonged ileus/dysmotility – Intestinal inflammatory dysfunction ? Dysmotility?- • GERD affects almost all of these patients • Inguinal • Improved since the advancements in IV nutrition- better overall at meeting nutritional needs, lipid sparing protocols

NEC following Gastroschisis Gastroschisis or Omphalocele?

• NEC occurs in 5-10% of gastroschisis patients • Current approach is same as for isolated NEC, with standard indications for laparotomy • Tends to occur later in clinical course • Can often be successfully treated non-operatively • No correlation between NEC and primary vs. staged abdominal wall repair for gastroschisis

continued Case presentation No history of birth defects on either side of family – Infant female DOL 0 – Pre-natal dx of gastroschisis Social history – Delivered vaginally after spontaneous rupture of membranes – Maternal age 20 years th at OSH, 1 week prior to planned induction – Mother smoked ½ ppd cigarettes until 6 week of pregnancy – Occasional alcohol use prior to 6th week of pregnancy – Gestational age at birth 36 2/7 weeks – Father 32 years, 1 previous child, no problems – Birth weight 2550 grams – No complications at time of delivery Medications: – Spontaneously breathing in room air, IV access obtained, – Ampicillin (Omnipen) 100mg/kg every 12 hours intestines and lower body placed in bowel bag and infant – Gentamicin 4mg/kg every 24 hours transported to neonatal intensive care unit for surgical – D10W with heparin 0.25 Units/mL IV solution evaluation and treatment

6 2/2/14

continued Laboratory Results: WBC 12.1 (9.0-30.0 K/mcL) Physical Exam: RBC 3.9 (3.9-5.5) – Eyes: PERRL, extraocular movements intact HGB 15.5 (13.5-19.5 gm/dL) – ENT: Exam normal, mmm, neck supple HCT 44.6 (42-60) – Skin: no rashes – Lungs: respiratory effort normal, CTA with normal BS bilaterally PLT 262 (135-466 K/mcL) – Heart: RRR, no murmur, normal S1 S2 Differential was unremarkable – Abdomen: scaphoid abdomen with 4cm defect to the right of the POCT Blood gas/Lytes/GLUC, ISTAT Results: umbilicus. Small bowel and part of stomach noted to be outside of – Na 137 132-142 mmol/L abdomen. Bowel with mild matting, no definite atresia. – K 4.0 3.4-6.2 mmol/L – GU: Normal female genitalia. Patent anus. – CA ionized 1.08 0.72-1.2 mmol/L – Musculoskeletal/Extremities: WWP, no cyanosis, clubbing or edema – Glucose 109 41-110 mg/dl – Neurological: awake, alert, movement equal in all extremities – pH 7.47, pCO2 29, pO2 38, HCO3 21.4

– O2 sat 77% (50-80%)

• No radiology studies performed Post Silo Placement • Plan: Bowel to be reduced into silo for serial reduction over next 5-7 days. Plan for fascial closure in OR once bowel reduced. • Procedure: placement of 4cm silo for reduction of gastroschisis at bedside. No bowel was trapped between silo ring and the fascia. Silo was suspended to the bar above the infant. • Anesthesia with morphine and versed • No additional concerns were noted during the procedure

Post procedure orders: Hospital Course Include which of the following?: • Bowel gradually reduced daily over the next 4 days. A. Start TPN, daily abdominal x-ray, foley to • Patient remained on TPN with NG to low continuous gravity drainage, strict I&O. suction with average output of 40mL/kg/day, replaced B. Start TPN, NG tube to LWS, foley to gravity drainage, 0.5mL:1mL with LR strict I&O, replace NG output 0.5mL/mL with LR every 4 • Urine output remained >3mL/kg/hr hours • Required vent support overnight following silo C.HOB elevated 450, continue TPN, daily abdominal x- placement due to sedation. Easily weaned to RA ray, foley to gravity drainage, strict I&O • Patient was taken to the OR for closure on HD 5. D. NPO, NG to LWS, foley to gravity drainage, D5 ½ NS at ½ maintenance rate.

7 2/2/14

Post-op Closure:

– monitor for increased intra-abdominal pressure • Poor perfusion to lower extremities • Decreased urine output • Increased edema • Increased oxygen requirement, respiratory difficulty

• Maintain temperature >36.5 C

• Naso or orogastric decompression: close attention to assure working properly- irrigate every 4 hours and prn with air via air port and NS via drainage port • Strict I&O, replacement of gastric output 0.5mL:mL with LR

Summary of Clinical Course Continued • Bladder pressures were initially elevated post-op into the mid to – Maintain urinary catheter- monitor urine output q1hr (minimum 1mL/ upper 20’s with UOP maintained >2.5mL/kg/hr abdomen kg/hr) distended, full but not tense on exam. – Bladder pressure every 4 hours call for pressure >20mm Hg • Bladder pressures gradually decreased over next 12-16 hours. – Fluid management Attempts to wean vent settings on POD1 were unsuccessful • PICC line was in place, continued TPN and gastric replacement with fluid intake of 140mL/kg/day with worsening of CBG results. • Monitor glucose, electrolytes, LFT’s , replace, adjust as needed • Remained on vent until POD3 and weaned to RA by next day. • Bladder pressures continued to be monitored with intermittent – Respiratory/Pain increases to mid 20’s, but with continued good UO. Abdomen • Vecuronium drip, as well as morphine and versed drips remained distended, but softer on exam. NG output relatively • Intubated from OR on vent high until POD4, did have stool later that day. • A chest film was obtained to confirm placement of the ETT. • POD6, NG discontinued with output <20mL/kg/day. Remained • Blood gas was obtained after return to NICU with stable results NPO on TPN. • Occupational therapy 3x/week

8 2/2/14

Continued: Summary con’t • Patient remained NPO, with bilious output from NG tube on TPN • Breast milk at 1mL/hr started, discontinued several hours later and lipids for additional week. following bilious emesis. NG returned to suction, continued TPN/ • Mother continued to save breast milk for future use. lipids and patient remained NPO for several additional days. • Occupational therapy continues 2-3x/week. Infant has good • Another attempt to start enteral feedings was unsuccessful, suck on pacifier, improved positioning and tone. continued on TPN, required PICC replacement when original • Patient with weight gain of 40+gm/day on TPN/lipids PICC no longer central. Surgical incision healed well. Abdomen remained soft once past the initial few days post-op. • TPN labs followed 2x/week, remained stable • Patient failed enteral attempt again several days later and a • Glycerin suppository bid. contrast study was obtained: • Next attempt to start enteral feeds was successful with slow progression. • UGI/SBFT to exclude anatomic obstruction showed delayed transit, with no contrast in colon in first 4h; subsequently • Decision made to place g-tube due to slow progress. contrast in colon 24h later thus no atresia. • Patient was subsequently d/c’d on DOL 60 with combination of g-tube and PO feedings.

Our typical feeding protocol Follow-up

– Start enteral feedings 24hr after repogle tube removed if Dysmotility is significant longterm factor for patients with patient is stable. gastroschisis. – Feedings are initiated at 1mL/hr and increased by 1mL/hr If patient discharged on TPN- followed in Intestinal Care Center each day until day 5. (ICC) – If tolerating advancement, increase rate bid and then – Multi discipline team – Increase every 8 hours day 9 if continue to tolerate – PO feedings are introduced depending on gestational age – 2 GI physicians, 1 surgeon, 1 neonatologist and skill level and advanced as tolerated. – 3 RN’s (GI department, follow both inpatients & outpatients) – TPN is weaned when enteral volume and caloric content is – 2 dieticians adequate. – Social worker – Caloric concentration and volumes are adjusted to meet – Speech pathologist weight gain goals. – If there is a problem with tolerance we return to previously – Round 1x/week on all inpatients and see patients in clinic tol amount and transition more slowly. with appointments available on 2 separate days/week

continued Long Term – Patients on TPN also followed by HHC nurse, labs every week, daily I&O recorded by family • Growth and Development – Patients discharged on tube feeds or oral feedings – South et al (2008) prospective study, 17 children @16-24 are followed in the High Risk Clinic months- 1/3 <10th percentile for wt at 1 year, overall no 2010-present 26 patients with gastroschisis who neurodevelopmental delay at time of evaluation required long term TPN – Minutillo et al (2013), Western Australia, retrospective study, th – 22 simple gastroschisis 112 infant at 1 year, 30%<10 percentile for wt, without • Average 35 days on TPN, 3 patients still on tpn at 1 year significant neurodevelopmental delay. Within this article, cited Gorra et al with similar developmental outcomes @ 2yr – 4 with associated atresis • Average 146 days on TPN, all off TPN within 10 months, – Manen et al (2013), Canada, echoed similar findings, also longest 222 days noted SNHL as most common disability in their cohort of children from 2005-2008 evaluated at 18mo. 15% remained on tube feedings, 2 with SBS, 1 required multi organ tx

9 2/2/14

continued continued • Readmission – Fallon et al (2012) retrospective review 2000-2007, reported – South et al (2011) Cincinnati, OH, reported on patients from 2006 that gestational age<37wk and development of through 2008. cholestasis were independently linked to poor growth in • 58 infants with initial discharge following repair for gastroschisis. 21% their patients, but that low birth wt <2500g was not. primary closure, all other silo. • 40% of the patients were readmitted at least 1 time (5 multi admits) within 1st year, more than ¼ directly r/t gastroschisis complications. – 2010, our group described outcomes for 71 infants (simple • Most common reason for re-admit was bowel obstruction and abd and 6 pt with complex) using standard nutritional protocol distention/pain. from 2006-2009. Enteral feedings initiated on DOL16 • They found no difference in re-admit related to place of birth, bowel (median), median LOS was 42 days, 6pts discharged on PN, resection during initial hospitalization, complex vs simple, SGA at 24% d/c’d on tube feedings birth, mode of delivery, timing of initiation of enteral feeds, PN duration, initial LOS, gender, maternal age and prenatal dx. • 8/12 (67%) patients primary closure were re-admitted; 9/46 (20%) silo were re-admitted. Admission for bowel obst occurred in 17% of primary closure and 7% of silo. (did not reach statistical significance)

Summary • Holland et al (2010) Gastroschisis: an update Summarized the overall picture well

– Increased frequency of occurrence 4.7/10,000 – Major congenital malformation, occurs early in gestation – Despite early studies, the association of risk factors attributed to tobacco use, illicit drug use appear low – Does affect young, teenage mothers – Readily dx on US, utility and frequency of subsequent monitoring is unclear – No strong evidence to support either routine early delivery or c- section – Delivery should occur at a center with pediatric surgical facilities – Need for a multi-center prospective RCT to address advantages and complications of closure methods. – The focus should also move to improved evaluation of long-term nutritional and neurodevelopmental outcomes.

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