GASTROSCHISISJon Ryckman, MD

SDPA

OBJECTIVES

• Define gastroschisis

• Discuss timing and technique of closure

• Discuss different techniques for abdominal wall closure

EMBRYOLOGY

• At 3 weeks’ gestation, lateral folds join to form pleuroperitoneal canals

• Gut tube has formed along length of embryo with communication at umbilicus at yolk sac

• Gut elongates within the umbilical coelom until 10 weeks when it returns to the abdominal cavity

GASTROSCHISIS

• Abdominal wall defect

• Failure of umbilical coelom to develop

• Usually occurs to the right of the umbilicus

• Related to involution of the right umbilical vein

• Occurs around week 4-5 of gestation

PATHOPHYSIOLOGY

• Linked to young mothers, nulliparous mothers

• Sympathomimetic drugs

• Maternal smoking

• No significant genetic predisposition identified

• Incidence increasing

FERTILIZERS

• Recent article from Washington state

• Gastroschisis occurred more frequently in those residing <25km from sites of high surface water atrazine concentration

• Especially important in spring conceptions

•Environmental exposures important in South Dakota as incidence seems higher in this state

•Average 9-15 gastroschisis babies at Sanford every year.

TYPES OF CLOSURE

• Primary Closure

• Silo with Planned Secondary Closure

PRIMARY CLOSURE

• Advantages

• Immediate closure

• Cosmetic advantage

• Faster time to feeding

• Less time on ventilator

• Disadvantages

• Possibility of abdominal compartment syndrome

• Higher incidence of necrotizing enterocolitis

PLANNED SILO

• Advantages

• No possibility of abdominal compartment syndrome

• No need for emergent surgery

• Could avoid GA

• Disadvantages

• Larger defect

• Longer time to feeding

• Fluid problems

• Unsightly

EVIDENCE

• Reviewed multiple studies over past 5 years

• Main outcomes are survival, time to feeding, LOS, time on vent

• Not many prospective studies comparing the two techniques

• Are some prospective studies looking at one technique

• Bias involved as centers tend to do what they are comfortable with

Closing arguments for gastroschisis: management with silo reduction. J Perinat Med. 2006;34(3):243-5. Chiu B, Lopoo J, Hoover JD, Almond PS, Arensman R, Madonna MB.

• Retrospective review

• Data back to 1994

• More complications seen in primary closure group

• NEC, Compartment syndrome, ventral hernia

• Recommend planned silo be the treatment of choice.

Gastroschisis closure--does method really matter? J Pediatr Surg. 2008 May;43(5):874-8. Weinsheimer RL, Yanchar NL, Bouchard SB, Kim PK, Laberge JM, Skarsgard ED, Lee SK, McMillan D, von Dadelszen P; Canadian Pediatric Surgery Network.

• CAPSNET data - prospectively generated, retrospectively reviewed

• No difference in silo or primary closure groups on all variables

• Successful primary closure did decrease the risk of prolonged time to enteral feeds and the risk of extended LOS

• Only failure of primary closure was associated with any impaired outcomes

• Babies undergoing sutureless closure had delays in initiating feeding

Routine use of a SILASTIC spring-loaded silo for infants with gastroschisis: a multicenter randomized controlled trial. J Pediatr Surg. 2008 Oct;43(10):1807-12. Pastor AC, Phillips JD, Fenton SJ, Meyers RL, Lamm AW, Raval MV, Lehman E, Karp TB, Wales PW, Langer JC.

• Prospective study in Toronto, UNC, Salt Lake City

• 27 patients in each group

• No significant difference in LOS, days on TPN, incidence of sepsis or NEC

Intestinal venous congestion as a complication of elective silo placement for gastroschisis. Semin Pediatr Surg. 2009 May;18(2):109-12. Ryckman J, Aspirot A, Laberge JM, Shaw K.

• Silo was treatment of choice at MCH

• Two patients suffered venous congestion and ischemia while in a silo due to “accordion” or “funnel” effect

• Second patient suffered bowel perforation, resection of all small bowel except 27cm

Method to our madness: an 18-year retrospective analysis on gastroschisis closure. J Pediatr Surg. 2010 Mar;45(3):579-84. Banyard D, Ramones T, Phillips SE, Leys CM, Rauth T, Yang EY

• Retrospective, Vanderbilt, based on intended closure technique

• 235 patients - 188 attempted primary, 139 successful

• Attempted primary associated with significant improvements in ventilator days, LOS, TPN days, and total charges

• No difference in complications or survival

• Trend towards higher rate of compartment syndrome in primary closure

No free ride? The hidden costs of delayed operative management using a spring-loaded silo for gastroschisis. J Pediatr Surg. 2010 Jul;45(7):1426-32. Lobo JD, Kim AC, Davis RP, Segura BJ, Alpert H, Teitelbaum DH, Geiger JD, Mychaliska GB.

• Retrospective Review - University of Michigan

• Primary closure attempted in all, but groups split on final procedure

• Survival 100% primary closure (10), 89% silo (27)

• Time on vent 3.6d for primary, 16d for silo

• No difference in time to feeding

• Silo complications: Replacement, enlargement, mesh

• 3 silo patients with ischemia at level of abdominal wall

Clinical features of newborns with gastroschisis and outcomes of different initial interventions: primary closure versus staged repair. Pediatr Neonatol. 2010 Dec;51(6):320-5. Tsai MH, et al.

• Retrospective review from Taiwan

• 32 primary, 12 silo

• No difference in survival, time on ventilator, days to reach full feedings.

• Mortality 16% (delayed surgical care, operative complication)

• Severity of complications higher in primary closure group

Review of the evidence on the closure of abdominal wall defects. Pediatr Surg Int. 2010 Dec 14. Mortellaro VE, Peter SD, Fike FB, Islam S.

Infants with congenital abdominal wall defects pose an interesting and challenging management issue for surgeons. We attempt to review the literature to define the current treatment modalities and their application in practice. In gastroschisis, the overall strategies for repair include immediate closure or delayed operative repair. The best level of data for gastroschisis is grade C and appears to support that there is no major difference in survival between immediate closure or delayed repair. In patients with omphalocele, the management techniques are more varied consisting of immediate closure, staged closure or delayed closure after epithelialization. The literature is less clear on when to use one technique over the other, consisting of mostly grade D and E data. In patients with omphalocele, a registry to collect information on patients with larger defects may help determine which of the management strategies is optimal.

TYPES OF CLOSURE

• Sutured closure (Casas-Melley technique)

• Mobilize skin off of fascia surrounding the defect

• Mobilize umbilical cord

• Close fascia, leaving cord in center of defect

• Reapproximate skin to umbilical cord

DELAYED REDUCTION FOR GASTROSCHISIS 1339

Fig 1. Child with gastroschisis demonstrates typical bowel fea- tures.

and umbilical port closure with the umbilical cord and without anesthesia or sedation. There was no other form of selection. Gesta- tional age was 31 to 40 weeks, and birth weight was 1.5 to 2.5 kg. DeIivery was vaginal in 13, and one had an emergency cesarean section for fetal distress. The umbilical port, always to the right of the cord attachment, had a diameter of 1.5 to 2.5 cm. Thirteen children had a full-length bowel, but one also had a membranous ileal atresia. A further child. with a segmental ileal atresia, mitially had a proximal end stoma placed at the umbilical port. Bowel continuity, umbihcal port closure with a purse string suture, and umbilicoplasty were undertaken 3 weeks later, Small bowel dilatation led to longitudinal intestinal lengthening and tailoring CLILT)5 before enteral feeding was established.

RESULTS

During and after bowel reduction, all children were fully conscious and alert, retaining all vital reflexes. Meconium was usually evacuated spontaneously. Mild to moderate venous congestion in the lower body cleared rapidly within 30 to 120 minutes of completion. The abdomen, initially full and firm, softened within hours. Twelve appeared and behaved no differently than normal neonates with no respiratory embarrassment, stable car- diovascular parameters and peripheral perfusion, and normal urine output. In particular, there was no abdomi- nal pain. A mild localized periumbilical inflammation developed in two children (Table 1). Primary circumfer- ential contracture of the umbilical port occurred in all

Fig 2. Child left undisturbed for an average of greater than 4 hours, in a right lateral position with bowel flowing into a clean plastic bag.

Table ‘7. Morbidity, Mortality, and Long-Term Outcome

Morbidity outcome

9 Uncomplicated Alive and well 1 lleal atresia - Stoma + LILT* Alive and well 2 Mild periumbllical inflammation Alive and well 2 Abdominal wall cellulitis

Midgut voIvuIus* Died at 22 mo lleal atresia, perforation* Died at 7 mo

*Additional transverse right hypochondnal laparotomyscar

cases, leaving an aesthetically “normal” umbilicus. All survivors, except for one child who underwent an LILT procedure, have a scarless abdomen (Fig 3). Umbilical herniorrhaphy or umbilicoplasty have not been required. Eleven established full enteral nutrition by the 1 lth day to the 32nd day with eight establishing by the 18th day. All 12 survivors have normal absorption and show physical and neurological development along expected centiles.

Two children appeared immediately and obviously unwell with abdominal pain and tenderness, pallor, tachycardia, and a metabolic acidosis. Marked cellulitis of the abdominal wall developed rapidly in both. At laparotomy, one had midgut volvulus with necrosis and died at 22 months of short bowel-related hepatic failure, and the other, with a perforation proximal to a membra- nous ileal atresia, died at 7 months of Enterobacter cloacae septicemia.

al Fig 3. Long-term follow-up shows a normal unscarred abdom

Id an aesthetically acceptable umbilicus. len

MINIMAL INTERVENTION

• Bianchi et al. 1998

• Stitches umbilical cord to rectus sheath after reduction at bedside

CLOSURE

• Sutureless closure

• Reduce abdominal viscera

• Flap umbilical cord over defect without suturing it

• Secure cord in place with Steri-strips and Tegaderm

• Keep dressing in place 5-7 days

mechanics. The consequence is prolonged high pressureventilation, prolonged narcotic analgesia, and the occasionalneed for medical paralysis to prevent closure breakdown andenable ventilation. Secondly, closure of larger defects hasvariable cosmetic outcomes: an unsightly scar, an abnormallyplaced umbilicus, and/or a tubular appearance to the abdomencan result. Finally, the aggressive manipulation of the bowelalong with narcotic effects may contribute to an ileus inalready dysmotile bowel, prolonging time to full feeds.

In 2004, Sandler et al [1] proposed a “sutureless”technique in which they covered the defect with theremnant umbilical cord and a Tegaderm dressing. The useof this approach dates back to one of the first reportedrepairs of gastroschisis in 1878 by Fear who reduced theviscera and covered the defect with the umbilicus, heldin place by a suture [2]. A recent article recommendedbuttressing the sutureless technique of Sandler et al withthe addition of reinforcing synthetic mesh [3]. These newtechniques offer excellent cosmetic results, but preliminarydata based on small sample sizes suggest an associationwith longer hospital stays and increased rates of umbilicalhernias. However, given its simplicity and safety, suturelessclosure of gastroschisis is gaining popularity, representing9% of all cases in a Canadian series [4], and deservesfurther study.

Since 2006, we adopted the sutureless approach tothe surgical care of gastroschisis at our institution. Thisstudy reviews our experience with 26 consecutive cases ofsutureless gastroschisis closures over a 2.5-year period—thelargest reported to date. We compare outcomes to a historicalcontrol group of patients undergoing sutured closure.

1. Methods

1.1. Patients

All patients from the Stanford affiliated hospitals (LucillePackard Children's Hospital, Santa Clara Valley MedicalCenter and Good Samaritan Hospital) treated for gastro-schisis between 2004 and 2008 were initially included in

this study. We excluded all complicated cases defined ashaving severe bowel pathology (atresia or perforation) orneonatal death.

1.2. Study design and statistical analysis

Variables recorded included patient descriptors (birthweight, gestational age, and type of delivery), managementvariables (size of abdominal defect, primary vs. secondaryrepair and sutured vs sutureless repair), and functionaloutcome variables (time to extubation, time to initiation oforal feeds, and time to discharge).

A retrospective case control study was performed toassess the effect of closure method on functional outcomes.All patients admitted between 2004 and 2006 were treated inthe operating room with sutured fascial closure. Thosepatients admitted between 2006 and 2008 were all treatedwith the sutureless technique under investigation. To ourknowledge, the primary neonatal intensive care unit (NICU)teams did not change their gastroschisis protocols between2004 and 2008.

Demographic data from the treatment and control groupswere compared using analysis of variance for continuousvariables and the chi-square test for categorical variables toexclude baseline differences.

Associations between patient descriptors, managementvariables and functional outcomes were then derived by Coxmultifactorial regression to control for covariates. Beforerunning the regression, all variables were assessed forcollinearity using the Pearson correlation factor. In cases ofsignificant correlation between 2 variables, only the one withthe greatest effect on the outcome variables was included inthe model. Odds ratios (OR) were derived with 95%confidence intervals (CIs) and corresponding P values. Allstatistical analysis was performed with SPSS 16 (SPSS,Chicago, Ill).

1.3. Surgical technique

All patients were initially managed in the NICU withconventional supportive therapy including a plastic bag over

Fig. 1 Sutureless technique for gastroschisis closure. (A) Day 1. Upon admission to the NICU, the patient's exposed bowel is covered with asterile plastic bag. When the bowel cannot be completely reduced (as shown here), the bag is tied off and tied to the top of the incubator,forming a silo. (B) Day 5. The bowel is reduced further in the silo with umbilical ties. (C) Day 7. Once all bowel is reduced, the abdominaldefect is covered with a Mepilex pad and a Tegaderm dressing. (D) Six weeks. Full healing of the abdominal defect is seen with a smallumbilical hernia.

1948 J. Riboh et al.

mechanics. The consequence is prolonged high pressureventilation, prolonged narcotic analgesia, and the occasionalneed for medical paralysis to prevent closure breakdown andenable ventilation. Secondly, closure of larger defects hasvariable cosmetic outcomes: an unsightly scar, an abnormallyplaced umbilicus, and/or a tubular appearance to the abdomencan result. Finally, the aggressive manipulation of the bowelalong with narcotic effects may contribute to an ileus inalready dysmotile bowel, prolonging time to full feeds.

In 2004, Sandler et al [1] proposed a “sutureless”technique in which they covered the defect with theremnant umbilical cord and a Tegaderm dressing. The useof this approach dates back to one of the first reportedrepairs of gastroschisis in 1878 by Fear who reduced theviscera and covered the defect with the umbilicus, heldin place by a suture [2]. A recent article recommendedbuttressing the sutureless technique of Sandler et al withthe addition of reinforcing synthetic mesh [3]. These newtechniques offer excellent cosmetic results, but preliminarydata based on small sample sizes suggest an associationwith longer hospital stays and increased rates of umbilicalhernias. However, given its simplicity and safety, suturelessclosure of gastroschisis is gaining popularity, representing9% of all cases in a Canadian series [4], and deservesfurther study.

Since 2006, we adopted the sutureless approach tothe surgical care of gastroschisis at our institution. Thisstudy reviews our experience with 26 consecutive cases ofsutureless gastroschisis closures over a 2.5-year period—thelargest reported to date. We compare outcomes to a historicalcontrol group of patients undergoing sutured closure.

1. Methods

1.1. Patients

All patients from the Stanford affiliated hospitals (LucillePackard Children's Hospital, Santa Clara Valley MedicalCenter and Good Samaritan Hospital) treated for gastro-schisis between 2004 and 2008 were initially included in

this study. We excluded all complicated cases defined ashaving severe bowel pathology (atresia or perforation) orneonatal death.

1.2. Study design and statistical analysis

Variables recorded included patient descriptors (birthweight, gestational age, and type of delivery), managementvariables (size of abdominal defect, primary vs. secondaryrepair and sutured vs sutureless repair), and functionaloutcome variables (time to extubation, time to initiation oforal feeds, and time to discharge).

A retrospective case control study was performed toassess the effect of closure method on functional outcomes.All patients admitted between 2004 and 2006 were treated inthe operating room with sutured fascial closure. Thosepatients admitted between 2006 and 2008 were all treatedwith the sutureless technique under investigation. To ourknowledge, the primary neonatal intensive care unit (NICU)teams did not change their gastroschisis protocols between2004 and 2008.

Demographic data from the treatment and control groupswere compared using analysis of variance for continuousvariables and the chi-square test for categorical variables toexclude baseline differences.

Associations between patient descriptors, managementvariables and functional outcomes were then derived by Coxmultifactorial regression to control for covariates. Beforerunning the regression, all variables were assessed forcollinearity using the Pearson correlation factor. In cases ofsignificant correlation between 2 variables, only the one withthe greatest effect on the outcome variables was included inthe model. Odds ratios (OR) were derived with 95%confidence intervals (CIs) and corresponding P values. Allstatistical analysis was performed with SPSS 16 (SPSS,Chicago, Ill).

1.3. Surgical technique

All patients were initially managed in the NICU withconventional supportive therapy including a plastic bag over

Fig. 1 Sutureless technique for gastroschisis closure. (A) Day 1. Upon admission to the NICU, the patient's exposed bowel is covered with asterile plastic bag. When the bowel cannot be completely reduced (as shown here), the bag is tied off and tied to the top of the incubator,forming a silo. (B) Day 5. The bowel is reduced further in the silo with umbilical ties. (C) Day 7. Once all bowel is reduced, the abdominaldefect is covered with a Mepilex pad and a Tegaderm dressing. (D) Six weeks. Full healing of the abdominal defect is seen with a smallumbilical hernia.

1948 J. Riboh et al.

SUTURELESS CLOSURE

• First described in 2004

• Study from Stanford Pediatric Surgery (J Pediatr Surg. 2009 Oct;44(10):1947-51)

• No difference in time to feeding or LOS

• Prospective comparison between sutured and sutureless closure started and recruiting patients.

ANY QUESTIONS?