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
small to contain all the intestinal
intestines causes herniation of the midgut into the
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
umbilical cord during the sixth week
fixed position 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.

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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,
•  10% incidence of associated anomalies ephedrine
•  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
protect from kinking
•  Typically on routine US
•  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

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•  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 Sutureless closure with Tegaderm after Silo
which the defect is then closed.
•  41 patients over past 3 years- 1 death reduction- may require ventral hernia repair later
•  All silo reduction, except for patients with small defects

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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),
–  poor perfusion (urine output)
•  Necrotizing Enterocolitis
–  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

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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 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
•  CVC care per policy
•  Teaching regarding feeding method (PO, NG, GT, TPN if
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

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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 hernias •  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

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 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.

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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
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
•  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.
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.
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
2/2/14
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.
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
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
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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