slusher abdominalwalldefects
TRANSCRIPT
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Abdominal Wall Defects: Gastroschisis
GlobalCast RN
Abdominal Wall Defects Include:
– Gastroschisis – Omphalocele – Bladder & Cloacal Exstrophy – Prune-belly syndrome – Urachal Remnants – Umbilical cord hernia
– Abdominal wall forms during 4th week of gestation – During 6th week of gestation, rapid growth of
intestines causes herniation of the midgut into the umbilical cord
– Week 10, the midgut is returned to the abdominal cavity and the small bowel and colon assumes a fixed position
– Any disruption in process may result in an abdominal wall defect
Embryology: Week 6 Physiological Umbilical Herniation
• As a result of rapid growth and expansion of the liver, the abdominal cavity temporarily becomes too small to contain all the intestinal loops. • The intestinal loops enter the extraembyronic cavity within the umbilical cord during the sixth week of development.
Source: Langman’s Medical Embryology. Ninth Edition.
Week 10 Return to Abdominal Cavity
During 10th week of development, herniated intestinal loops begin to return to the abdominal cavity.
Factors responsible for this return are not precisely known.
Anterior abd wall progressively closes leaving only an opening at the umbilical ring
Source: Langman’s Medical Embryology. Ninth Edition.
Gastroschisis vs Omphalocele Gastroschisis
Omphalocele
Incidence 2-‐4.5:10,000 1:10,000-‐20,000
Defect Size 2-‐5 cm 1-‐15 cm
Umbilical Cord To left of defect In center of membrane
Contents Open, exposed Covered
Bowel Inflamed, edematous, matted
Normal
Associated Anomalies
10% 60%-‐75%
Mortality < 5% 25%
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Gastroschisis
• Herniation of intestinal loops through full-thickness defect in anterior abdominal wall • Defect lateral to the umbilicus (right>left), usually less than 4cm in size • No sac covers the extruded viscera
Etiology:
Unknown: – Thrombosis of the right umbilical vein causes
necrosis – Right omphalomesenteric artery prematurely
involutes Other theories: – In-utero rupture of omphalocele – Rupture of abdominal wall due to rapidly
increasing volume – Abnormal development of the ventral abdominal
wall-failure of midline fusion of the lateral folds.
Gastroschisis
• Incidence of gastroschisis noted increased past 20 years – Incidence: 2-5 per 10,000 live births
• Young maternal age (21 years or less) • Prematurity and low birth weights, secondary
to intrauterine growth retardation • 10% incidence of associated anomalies
• As compared to 60-75% in omphalocele
Risk Factors: • 4X more common in women < 20 years of
age • Smoking • Stressed and undernourished mothers • Over the counter meds with vasoactive
properties: pseudoephedrine, aspirin, ephedrine
• Multifactorial
Diagnosis:
• Often pre-natal • Typically on routine US • Slightly elevated AFP
• Helpful for postnatal planning • Pediatric surgeon availability • Counseling • Mode of delivery is controversial, but no data in
literature to support either c-section or vaginal
Neonatal Management- pre-op • Initial assessment of airway, breathing and circulation
• Sedation and/or intubation as indicated • Assess bowel viability
• Place eviscerated bowel into sterile bowel bag and position midline to protect from kinking
• May have a tight defect causing vascular compromise to the bowel • Minimize heat loss - >36.5 C
• Radiant warmer • Incubator temperature control • Warm IV fluids if indicated
• Obtain IV access and begin fluid resuscitation • Significant fluid losses from exposed bowel • Bolus 20mL/kg NS or LR • Infusion of D10 ¼ NS at 2-3 times maintenance • Strict Intake and Output • Monitor vital signs, including blood pressure
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• Gastric Decompression • Naso or orogastric, use low continuous suction for dual-lumen
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
Gastroschisis & Intestinal Atresia • May be difficult to identify atresia in acute setting • Bowel reduced back into abdomen and plan for reoperation after
4-12 weeks if atresia suspected by feeding intolerance and/or imaging study
Surgical Management of Abdominal Wall Defects
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 Goal: return the viscera to the abdominal cavity and close the defect while minimizing risk of damage to the intestine from trauma or increased intra-abdominal pressure. Options:
– Primary Surgical Closure: Success dependent on size of the defect and size of the abdominal and thoracic cavities.
– Staged Closure: Gradual reduction of the contents into the abdominal cavity using an extra-abdominal extension of the peritoneal cavity (silo) and using gentle pressure. Usually requires 1-14 days, after which the defect is then closed.
• 41 patients over past 3 years- 1 death • All silo reduction, except for patients with small defects
Gastroschisis Surgical Options for Treatment Primary Closure +/- Prosthetic Mesh Staged closure with
– Spring loaded Silo – Silastic Sheet- Sutured to medial aspect of rectus
fascia (not used as much any more) Sutureless closure with Tegaderm after Silo reduction- may require ventral hernia repair later
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Gastroschisis Complications of Primary Fascial Closure: • Deceased Venous Return • Abdominal Compartment Syndrome • Pulmonary Compliance • Renal Failure • Necrotizing Enterocolitis
Gastroschisis • Important to Measure Bladder Pressures
– < 20 mm Hg – Monitor Ventilatory Pressures During and After
Closure • Clinical diagnosis
– abdominal exam (rigid), – poor perfusion (urine output) – worsening ventilation (increased ventilator settings)
Surgically created Silastic Silo Silo
Spring-loaded Silo
To prevent ischemia with reduction; the defect is enlarged if needed Proper orientation of bowel
Spring loaded Silo suspended for proper orientation of bowel
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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 – Post-silo support silo/bowel to avoid twisting or kinking – monitor for increased intra-abdominal pressure
• Poor perfusion to lower extremities • Decreased urine output • Increased edema • Increased oxygen requirement, respiratory difficulty • For silo- frequently assess bowel; should be pink, fluid in bag
serous and free of stool
• 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
Nursing Care – Strict I&O, replacement of gastric output as ordered
– Maintain urinary catheter- monitor urine output q1hr (minimum 1mL/kg/hr)
• Bladder pressure <20mm Hg – Fluid management
• Central venous access obtained • Increased fluid and albumin needs –fluids 120-140mL/kg/day, albumin
as needed • TPN, replacement of gastric output • Strict I&O
– Respiratory • Monitor for distress (especially after closure) • Monitor pulse oximetry and ABG’s • Maintain sat >95 • Intubated, sedated if required
Nursing Care – Infection Prevention
• Sterile technique during dressing changes • Antibiotics as ordered • Dressing around silo per your institutions routine- betadine soaked
gauze, changed bid • Monitor for separation, redness or drainage at base of silo or suture line
post closure • CVC care per policy • Temperature, vital signs, labs
– Pain Management • Assess using neonatal pain scale hourly during initially post-op period • Monitor physical parameters • Use behavioral interventions and medications as appropriate
Nursing Care – Skin Care
• Monitor pulses, temperature, color of extremities • Consider positioning, especially if using silo • Consider gestational age • Report drainage or dehiscence
– Discharge Planning • Teaching regarding feeding method (PO, NG, GT, TPN if
necessary) HHC arrangements • Teach CPR prior to discharge, Car seat is appropriate • Alert family to call MD office/ED for s/s of bowel obstruction/
volvulus-(bilious emesis, abd distention, no stool output, diarrhea)
• Assure follow-up is arranged
Refer to: Nursing Care of the Pediatric Surgical Patient (2013) pp 287-289
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Post-op complications:
• Abdominal compartment syndrome – ischemic, infarcted bowel, renal failure
• Infection • Necrotizing enterocolitis • Short gut syndrome with TPN dependence • Prolonged ileus/dysmotility • GERD • Inguinal hernias
Gastroschisis • Prognosis is dependent mainly upon severity
of associated problems – Prematurity – Intestinal atresia (10-15%) – Short Gut-loss of bowel due to ischemia, infarction – NEC (5-10%) – Intestinal inflammatory dysfunction ? Dysmotility?-
affects almost all of these patients • Improved since the advancements in IV nutrition-
better overall at meeting nutritional needs, lipid sparing protocols
NEC following Gastroschisis
• 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
Gastroschisis or Omphalocele?
Case presentation – Infant female DOL 0 – Pre-natal dx of gastroschisis – Delivered vaginally after spontaneous rupture of membranes
at OSH, 1 week prior to planned induction – Gestational age at birth 36 2/7 weeks – Birth weight 2550 grams – No complications at time of delivery – Spontaneously breathing in room air, IV access obtained,
intestines and lower body placed in bowel bag and infant transported to neonatal intensive care unit for surgical evaluation and treatment
continued No history of birth defects on either side of family Social history
– Maternal age 20 years – Mother smoked ½ ppd cigarettes until 6th week of pregnancy – Occasional alcohol use prior to 6th week of pregnancy – Father 32 years, 1 previous child, no problems
Medications:
– Ampicillin (Omnipen) 100mg/kg every 12 hours – Gentamicin 4mg/kg every 24 hours – D10W with heparin 0.25 Units/mL IV solution
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continued Physical Exam:
– Eyes: PERRL, extraocular movements intact – ENT: Exam normal, mmm, neck supple – Skin: no rashes – Lungs: respiratory effort normal, CTA with normal BS bilaterally – Heart: RRR, no murmur, normal S1 S2 – Abdomen: scaphoid abdomen with 4cm defect to the right of the
umbilicus. Small bowel and part of stomach noted to be outside of abdomen. Bowel with mild matting, no definite atresia.
– GU: Normal female genitalia. Patent anus. – Musculoskeletal/Extremities: WWP, no cyanosis, clubbing or edema – Neurological: awake, alert, movement equal in all extremities
Laboratory Results: WBC 12.1 (9.0-30.0 K/mcL) RBC 3.9 (3.9-5.5) HGB 15.5 (13.5-19.5 gm/dL) HCT 44.6 (42-60) PLT 262 (135-466 K/mcL) Differential was unremarkable POCT Blood gas/Lytes/GLUC, ISTAT Results:
– Na 137 132-142 mmol/L – K 4.0 3.4-6.2 mmol/L – CA ionized 1.08 0.72-1.2 mmol/L – Glucose 109 41-110 mg/dl – pH 7.47, pCO2 29, pO2 38, HCO3 21.4 – O2 sat 77% (50-80%)
• No radiology studies performed • 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 Silo Placement
Post procedure orders:
Include which of the following?: A. Start TPN, daily abdominal x-ray, foley to
gravity drainage, strict I&O. B. Start TPN, NG tube to LWS, foley to gravity drainage, strict I&O, replace NG output 0.5mL/mL with LR every 4 hours C. HOB elevated 450, continue TPN, daily abdominal x-ray, foley to gravity drainage, strict I&O D. NPO, NG to LWS, foley to gravity drainage, D5 ½ NS at ½ maintenance rate.
Hospital Course
• Bowel gradually reduced daily over the next 4 days. • Patient remained on TPN with NG to low continuous
suction with average output of 40mL/kg/day, replaced 0.5mL:1mL with LR
• Urine output remained >3mL/kg/hr • Required vent support overnight following silo
placement due to sedation. Easily weaned to RA • Patient was taken to the OR for closure on HD 5.
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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
Continued – Maintain urinary catheter- monitor urine output q1hr (minimum 1mL/
kg/hr) – Bladder pressure every 4 hours call for pressure >20mm Hg – Fluid management
• PICC line was in place, continued TPN and gastric replacement with fluid intake of 140mL/kg/day
• Monitor glucose, electrolytes, LFT’s , replace, adjust as needed
– Respiratory/Pain • Vecuronium drip, as well as morphine and versed drips • Intubated from OR on vent • A chest film was obtained to confirm placement of the ETT. • Blood gas was obtained after return to NICU with stable results
Summary of Clinical Course • Bladder pressures were initially elevated post-op into the mid to
upper 20’s with UOP maintained >2.5mL/kg/hr abdomen distended, full but not tense on exam.
• Bladder pressures gradually decreased over next 12-16 hours. Attempts to wean vent settings on POD1 were unsuccessful with worsening of CBG results.
• Remained on vent until POD3 and weaned to RA by next day. • Bladder pressures continued to be monitored with intermittent
increases to mid 20’s, but with continued good UO. Abdomen remained distended, but softer on exam. NG output relatively high until POD4, did have stool later that day.
• POD6, NG discontinued with output <20mL/kg/day. Remained NPO on TPN.
• Occupational therapy 3x/week
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Summary con’t • Breast milk at 1mL/hr started, discontinued several hours later
following bilious emesis. NG returned to suction, continued TPN/lipids and patient remained NPO for several additional days.
• Another attempt to start enteral feedings was unsuccessful, continued on TPN, required PICC replacement when original PICC no longer central. Surgical incision healed well. Abdomen remained soft once past the initial few days post-op.
• Patient failed enteral attempt again several days later and a contrast study was obtained:
• UGI/SBFT to exclude anatomic obstruction showed delayed transit, with no contrast in colon in first 4h; subsequently contrast in colon 24h later thus no atresia.
Continued: • Patient remained NPO, with bilious output from NG tube on TPN
and lipids for additional week. • Mother continued to save breast milk for future use. • Occupational therapy continues 2-3x/week. Infant has good
suck on pacifier, improved positioning and tone. • Patient with weight gain of 40+gm/day on TPN/lipids • TPN labs followed 2x/week, remained stable • Glycerin suppository bid. • Next attempt to start enteral feeds was successful with slow
progression. • Decision made to place g-tube due to slow progress. • Patient was subsequently d/c’d on DOL 60 with combination of
g-tube and PO feedings.
Our typical feeding protocol – Start enteral feedings 24hr after repogle tube removed if
patient is stable. – Feedings are initiated at 1mL/hr and increased by 1mL/hr
each day until day 5. – If tolerating advancement, increase rate bid and then – Increase every 8 hours day 9 if continue to tolerate – PO feedings are introduced depending on gestational age
and skill level and advanced as tolerated. – TPN is weaned when enteral volume and caloric content is
adequate. – Caloric concentration and volumes are adjusted to meet
weight gain goals. – If there is a problem with tolerance we return to previously
tol amount and transition more slowly.
Follow-up Dysmotility is significant longterm factor for patients with gastroschisis. If patient discharged on TPN- followed in Intestinal Care Center (ICC)
– Multi discipline team – 2 GI physicians, 1 surgeon, 1 neonatologist – 3 RN’s (GI department, follow both inpatients & outpatients) – 2 dieticians – Social worker – Speech pathologist – Round 1x/week on all inpatients and see patients in clinic
with appointments available on 2 separate days/week
continued – Patients on TPN also followed by HHC nurse, labs
every week, daily I&O recorded by family – Patients discharged on tube feeds or oral feedings
are followed in the High Risk Clinic 2010-present 26 patients with gastroschisis who
required long term TPN – 22 simple gastroschisis
• Average 35 days on TPN, 3 patients still on tpn at 1 year – 4 with associated atresis
• Average 146 days on TPN, all off TPN within 10 months, longest 222 days
Long Term
• Growth and Development – South et al (2008) prospective study, 17 children @16-24
months- 1/3 <10th percentile for wt at 1 year, overall no neurodevelopmental delay at time of evaluation
– Minutillo et al (2013), Western Australia, retrospective study, 112 infant at 1 year, 30%<10th percentile for wt, without significant neurodevelopmental delay. Within this article, cited Gorra et al with similar developmental outcomes @ 2yr
– Manen et al (2013), Canada, echoed similar findings, also 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
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continued – Fallon et al (2012) retrospective review 2000-2007, reported
that gestational age<37wk and development of cholestasis were independently linked to poor growth in their patients, but that low birth wt <2500g was not.
– 2010, our group described outcomes for 71 infants (simple and 6 pt with complex) using standard nutritional protocol from 2006-2009. Enteral feedings initiated on DOL16 (median), median LOS was 42 days, 6pts discharged on PN, 24% d/c’d on tube feedings
continued • Readmission
– South et al (2011) Cincinnati, OH, reported on patients from 2006 through 2008.
• 58 infants with initial discharge following repair for gastroschisis. 21% 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.
• Most common reason for re-admit was bowel obstruction and abd distention/pain.
• They found no difference in re-admit related to place of birth, bowel resection during initial hospitalization, complex vs simple, SGA at 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.