Management of Gastroschisis with Limited Resources

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Annals of Pediatric Surgery Vol 5, No 3, July 2009, PP 166-171 Original Article Management of Gastroschisis with Limited Resources Almoutaz A. ElTayeb, Ashraf A Helmy Pediatric Surgical Unit, Assiut University Children Hospital, Assiut, Egypt Background/ Purpose: Gastroschisis (GS) management remains controversial. Most surgeons have advocated emergency reduction and operative fascial closure (OFC) under general anesthesia (GA). Others advised staged reduction (SR) using silo bags and delayed closure to avoid the sudden rise of intra-abdominal pressure (IAP) which may be fatal. The aim of this study is to assess the management and outcome of GS treated by primary reduction and (OFC) compared to staged reduction (SR). Materials & Methods: This study included 26 neonates presented with GS during the period from January 2006 to December 2008. Twelve of them were candidates for primary OFC (group A) and fourteen underwent staged reduction using sterile blood collecting bag as a silo (group B). Results: There was no significant statistical difference between the two groups as regard gestational age, birth weight, mode of delivery or in the period necessary for ventilation between the 2 groups, although in group A it was essential in 5 out of 12 cases while in group B only 2 cases out of 14 needed ventilation. There were ten mortalities encountered in this series, six among group A and four in group B. Conclusion: Treatment of GS should be individualized, staged reduction is recommended for edematous thickened bowel with evidence of visceroabdominal disproportion. The use of the sterile blood collecting bag proved to be a safe and effective alternative for the classic silo when the later is not available. Index Word: Gastroschisis, staged reduction. G INTRODUCTION astroschisis (GS) is a congenital abdominal wall defect, typically located to the right of the umbilicus with herniation of the midgut. It was first described by Calder in 1733 1.The reported incidence of GS is about 1 per 4000 live births, but there is strong evidence that its incidence has increased five folds during the last 30 years 2-9. GS is rarely associated with other anomalies, but the major morbidity and mortality are because of the intestinal damage which occurs during fetal life due to prolonged exposure of the gut to the amniotic fluid resulting in dysfunction of the mucosa and poor motility of the gut 10. The mortality from GS has decreased to 10-20% during the last two decades in many centers and this is mainly due to the advances in neonatal intensive care (NICU) and the development of parenteral nutrition 6. There is still much controversy about the management of GS. The main surgical challenge over the past two decades has been whether to perform a primary operative fascial closure (OFC) under general anesthesia after forcible stretching of the abdominal wall soon after birth 11-15, or performing staged reduction using a temporary surgically placed prosthetic silo followed by closure 16-20. Recently some authors recommended bedside reduction in the neonatal ICU i.e. Ward reduction (WR) without general anesthesia 21-22. Others used non Correspondence to: Almoutaz Ahmed ElTayeb, Pediatric Surgery Unit, Assiut University Children Hospital, Assiut, Egypt, Tel. +(20-)010-1657863, Fax +(2088)-2353322. e-mail: almoutazeltayeb@hotmail.com.

operative placement of spring loaded silo for gradual reduction followed by delayed OFC under general anesthesia 23-25. The degree of the visceroabdominal disproportion and the condition of the herniated viscera plays an important role in making the surgical decision 6,26. Aim of the work: The aim of this work is to assess the management and outcome of GS treated by primary reduction and OFC compared to staged reduction SR using the sterile blood collecting bag as a silo when the later is not available.. PATIENTS AND METHODS Twenty six neonates with GS during the period January 2006 to December 2008 were included in this study. They were 14 males and 12 females. The delivery was vaginal in 11 and by cesarean section in 15 cases. Their age at presentation ranged from 6 to 48 hours. Twelve neonates were presented with hypothermia. Neonatal sepsis (clinical and laboratory) was evident in ten cases. The necessity for ventilation, commencement of full enteral feeding, duration of parenteral nutrition, duration of hospital stay, complications and mortality all were recorded. Cases associated with preoperative gut ischemia, atresia or perforation were excluded from the study. Also two cases with small sized GS were easily reduced and closed in the ICU (WR) and were also excluded from this study. There were no medical indications for ventilation before the operation. Making the surgical decision whether to perform primary OFC or to perform staged reduction was dependant on the degree of the visceroabdominal disproportion and the condition of the herniated viscera. Primary OFC (group A) was possible in 12 cases with mean gestational age 37 ± 1.79 weeks and mean birth weight 2.84 ± 0.31 kg; while SR and delayed closure (group B) was performed in 14 cases with mean gestational age 37± 1.78 weeks and mean birth weight 2.82± 0.32 kg. Preoperative preparation of all patients started by washing the herniated intestine with warm saline and diluted povidone iodine. The washed intestine was inspected to exclude atresia, perforation, ischemia or adhesions in between the loops or the abdominal wall which should be lysed and the viscera was wrapped in warm sterile towel. The size of the defect in both groups A&B ranged from 2.5 to 4 cm. Then nasogastric tube was inserted to keep the stomach empty. IV antibiotics (third generation cephalosporins 100mgm/kg/day and metronidazole 7.5mgm/kg/8hr) were given. OFC was done for cases in which the herniated viscera was relatively small in size and easily reduced without milking of the intestinal contents or increase in the intra-abdominal pressure. OFC was performed under GA while the patient is connected to the monitor for assessment of O2 saturation, heart rate and respiration. The abdominal wall was stretched to allow easy reduction without increase in the intra-abdominal pressure. During reduction the anesthesiologist ensured that there was no difficulty in ventilation. The fascia and skin were closed with preservation of the umbilical cord. In SR cases the herniated viscera was introduced into a blood collecting bag without twisting, under GA and complete aseptic conditions. The bag was sutured to the edge of the defect. The transparent nature of the bag allowed daily inspection of the bowel. The bowel was gradually reduced into the abdominal cavity using umbilical tape to tighten the bag over the viscera on daily bases till complete reduction over 9 to14 days and closure of the defect under GA was done one to two days after complete reduction. Oral feeding was started gradually in both groups when the gastric aspirate had diminished. The survived patients were discharged from the hospital when they were on full oral feeding, satisfactory weight gaining and followed up for 6 months after discharge. Statistical analysis of data was carried out by using SPSS (Chicago, Illinois). For continuous data, student t test was used to compare means, while Chi square was used to compare means of discrete data. p<0.05 was considered significant. RESULTS (Tables 1& Fig 1-3): There was no significant statistical difference between the two groups as regards gestational age, birth weight, days to start full oral feeding and duration of parenteral nutrition. There was a clear difference between the two groups in the need for post operative ventilation, where it was necessary in 5 cases from group A for 5 to 14 days. On the other hand, it was necessary only in 2 cases from group B for 3 days and 5 days. However this difference between the two groups was statistically insignificant. There was 167 Vol.5, No 3, July 2009

statistical significant difference between both groups regarding the duration of hospital stay where it was longer among group A (mean 34.7 vs. 27.5 days). The incidence of post reduction attacks of necrotizing enterocolitis (NEC) was evident in group A (three cases out of twelve). While only two cases out of fourteen developed NEC in group B. Wound infection occurred in four patients from group A and two from group B. All responded to conservative measures except one case from group A where complete burst occurred. Closure was done but the patient died later from septicemia and DIC. Chest infection was encountered in both groups (2 cases from group A& 1 from group B). All were controlled by antibiotics except in one case from group B which died from severe pneumonia after 20 days from reduction and closure of the defect. Other post operative complications included intestinal perforation occurred in two cases in group A and one in group B. There was no statistical difference between both groups regarding post operative morbidity. The overall mortality was ten cases out of twenty six (six from group A and four from group B). The main cause of death was NEC and intestinal perforation followed by severe chest infection. There was no statistical difference between both groups regarding post operative mortality. The follow up period was done for six months postoperatively for the survived cases and their weight gaining was satisfactory Table 1: Patients characteristics and postoperative complications of the twenty six neonates with GS Group A Group B OFC SR P-value Days to full enteral feeding Days on TPN Days on ventilator Duration of hospital stay (d) (n= 12) 24.58 ± 6.65 22.33 ± 6.37 n(5 cases) 9.80 ± 3.49 34.75 ± 10.52 (n= 14) 22.07 ± 6.00 19.92 ± 5.90 n( 2 cases) 4.00 ± 1.41 27.5 ± 6.07 0.32 0.32 0.082 0.038 NEC 3 2 0.63 Intestinal perforation 2 1 0.58 Wound infection 4 3 0.66 Chest infection 2 1 0.58 Mortality 6 4 0.42 Annals of Pediatric Surgery 168

Congenital anomalies including GS have been increased in frequency in upper Egypt during the last two decades 2,3. The cause of this increase is still unclear, however, young maternal age, positive consanguinity, plant fertilizers and the use of pesticides may be a predisposing factors. Fig 1: Fig 1: Gastroschisis with thickened oedematous bowel. Fig 3: Gradual reduction of the herniated viscera Fig 2: blood collecting bag after being applied DISCUSSION Watkins in 1943 9 was the first to report successful primary closure of a small GS. Different procedures are used for management of GS. Several authors reported that the advantages of primary OFC are decreased morbidity, mortality, TPN duration, short hospital stay and only one operative intervention 6,15,17,28. However, primary OFC is a procedure that involves vigorous stretching of the abdominal wall and milking of the intestinal contents. Also its potential danger is the ventilatory dysfunction and vascular compromise because of the increased intra abdominal pressure and additional injury to the already unhealthy bowel. This necessitates post operative mechanical ventilation with the use of muscle relaxants in large number of cases and accordingly increased morbidity and mortality in these cases 18,19. In this study, 12 cases were candidates for primary OFC (group A). In the remaining 14 cases, the evident visceroabdominal disproportion and the condition of the herniated bowel made emergency OFC unsafe and accordingly SR (group B) was decided. There was no statistical significant difference between the two groups as regard: gestational age, mode of delivery, birth weight, days to full enteral feeding and days on TPN. However, there is statistical significant difference in the duration of hospital stay between the two groups where it was longer in group A. Also the need for post operative mechanical ventilation and the use of muscle relaxants was more frequent and for longer duration among group A but the difference was not statistically significant. This may be due to preoperative intestinal injury caused by hypothermia, hypovolaemia and acidosis in addition to increased intra abdominal pressure among group A. In the presence of marked edema, swelling and dilatation of the bowel, emergency OFC may cause unacceptable rise in the intra-abdominal pressure with development of abdominal compartment syndrome and its dangerous sequelae. In addition, the prolonged intestinal dysfunction in GS cases is an important factor for increased postoperative morbidity and mortality 11. This confirms our results where the period necessary for post operative mechanical ventilation, duration of hospital stays and both morbidity and mortality rates were higher among group A. our results are in agreement with other authors 16,19,25. 169 Vol.5, No 3, July 2009

The rationale for using staged reduction is to reduce the edema of the bowel and increasing the abdominal domain gradually nullifying the visceroabdominal disproportion and to allow time to regain function of the gut. CONCLUSION The line of treatment of GS should be individualized and selective according to the general condition of the baby, the condition of the herniated viscera and the degree of visceroabdominal disproportion. Emergency OFC can be reserved for patients in whom the amount of the herniated viscera is relatively small without much visceroabdominal disproportion and without edema or thickening of the bowel. Staged reduction followed by delayed closure is recommended for GS with edematous thickened bowel with evident visceroabdominal disproportion. It avoids compartment syndrome with its complications and make fascial closure easier and safer. Using sterile blood collecting bag as a silo, when the latter is not available, proved to be safe and effective procedure for gradual reduction of the herniated swollen gut and can be applied once the neonate is stable. Similar findings was obtained by other authors using haemo derivatives bags in the treatment of GS 27. REFERENCE Calder J.: Two examples of children with pretermatural conformation of the guts. Med Essay abs.1:203-206, 1733. (Cited by Pastor AC, et al., 2008). 2-Wael H Samy: The increased neonatal surgical emergencies and the results of treatment. MS Thesis, Assiut University press, 2007. 3-Omaima A Mohamed: Pattern of clinically detectable congenital anomalies in newborns and infants attending Assiut university Hospital. MD thesis, Assiut University press, 2003. 4-Pastor AC, Philips JD, Fenton SJ, et al.: Routine use of a silastic spring-loaded silo for infants with gastroschisis: a multicenter randomized controlled trial. J Pediatr Surg. 43: 1807-1812, 2008. 5-Owen A, Marven S, Jakson L, et al.: Experience of bedside preformed silo staged reduction and closure for gastroschisis. J Pediatr Surg. 41: 1830-1835, 2006. 6-Di Tanna GL, Rasans A, Mastroiacous P: Prevalence of gastroschisis at birth: Retrospective study. BMJ. 325: 1389-90, 2002. 7-Coughlin JP, Drucker DE, Jewell MR, et al: Delivery room repair of gastroschisis. Surg. 114:822-826, 1993. 8-Aspelund G, Langer JC: Abdominal wall defects. Curr Paediatr. 16: 192-8, 2006. 9-Watkins D.: Gastroschisis Va Med Mon 70:1943. (Cited by Pastor et al., 2008). 10-Langer JC, Bell JG, Castillo RO, et al.: Etiology of intestinal damage in gastrtoschisis, II. Timing and reversibility of histological changes, mucosal function, and contractility. J Pediatr Surg. 25: 1122-1225, 1990. 11-Quirk JG Jr, Fortney J, Colins HB, et al.: Outcomes of newborns with gastroschisis: The effects of mode of delivery, site of delivery, and interval from birth to surgery. Am J Obstet Gynecol. 174: 1134-1138, 1996. 12-Moore TC, Collins DL, Catranzarite V, et al.: Pre-term and particularly pre-labor Cesarean section to avoid complications of gastroschisis. Pediatr Surg Int. 15: 97-104, 1999. 13-Fasching G, Mayr J, Sauer H: The effect of mode of delivery on outcome in fetuses with gastroschisis. Pediatr Surg Int. 11: 100-102 1996. 14-Stoodley N, Sharma A, Noblett H, et al.: Influence of place of delivery on outcome of babies with gastroschisis. Arch Dis Child. 68: 321-323, 1993. 15-Caniano DA, Brokaw B, Ginn-Pease ME: An individualized approach to the management of gastroschisis. J Pediatr Surg. 25: 297-300, 1990. 16-Komuro H, Imaizumi S, Hirata A, et al.: Staged silo repair of gastroschisis with preservation of the umbilical cord. J Pediatr Surg. 33: 485-488, 1998. 17-Canty TG, Collins DL: Primary fascial closure in infants with gastroschisis and omphalocele : A superior approach. J Pediatr. Surg.18:707-712, 1983. 18-Fonkalsrud EW, Smith MD, Shaw KS, et al.: Selective management of gastroschisis according to the degree of visceroabdominal disproportion. Ann Surg. 218: 742-747, 1993. 19-Sauter ER, Falterman KW, Arensman RM: Is primary repair of gastroschisis and omphalocele always the best operation? Am Surg 57: 142-144, 1991. 20-Bianchi A, Dickson AP: Elective delayed reduction and non anesthesia: "Minimal intervention management" for gastroschisis. J Pediatr Surg., 33: 1338-1340, 1998. 21-Kimbie RM, Singh SJ, Bourke C, et al.: Gastroschisis reduction under analgesia in the neonatal unit. J Pediatr Surg. 36: 1672-1674, 2001. 22-Fischer JD, Chun K, Moores DC, et al.: Gastroschisis: A simple technique for staged silo closure. J Pediatr Surg. 30: 1169-1171, 1995. Annals of Pediatric Surgery 170

23-Minkes RK, Langer JC, Mazziotti MV, et al.: Routine insertion of a silastic spring-loaded silo for infants with gastroschisis. J Pediatr Surg. 35: 843-846, 2000. 24-Bianchi A, Dickson AP, Alizai NK: Elective delayed midgut reduction no anaesthesia for gastroschisis: Selective and conversion criteria. J Pediatr Surg. 37: 1334-6, 2002. 25-Davies MW, Kimble RM, Cartwright DW: Gastroschisis; ward reduction compared with traditional reduction under general anaesthesia. J Pediatr Surg. 40: 523-7, 2005. 26-Schlatter M, Norris K, Uitvlugt N, et al.: Improved outcomes in the treatment of gastroschisis using a preformed silo and delayed repair approach. J Pediatr Surg. 38: 459-64, 2003. 27-Miranda ME, Tatsuo ES, Guimaraes JT, et al.: The use of a plastic haemoderivative bag in the treatment of gastroschisis. Pediatr Surg Int. 15: 442-4, 1999. 28- Olsen L, Ewald U, Meurling S: Gastroschisis: Primary closure is possible in most newborns. Pediatr. Surg. Int. 7:431-434, 1992 171 Vol.5, No 3, July 2009