Annals of Pediatric Surgery, Vol 4, No 3&4 July-October, 2008 PP 83-88

Original Article

Management and Outcome of Neonatal Bowel Perforation

Almoutaz A. Eltayeb, Mostafa Hashem
Pediatric Surgical Unit & Radiology Department, Assiut University Children's Hospital, Faculty of Medicine, Assiut University, Egypt

Abstract
Background/Purpose: Despite the recently improved neonatal management, gastrointestinal perforation during the neonatal period is still a major challenge for pediatric surgeons. Neonatal bowel perforation may be spontaneous (idiopathic) or due to necrotizing enterocolitis (NEC) or mechanical obstruction. The aim of this study was to analyze the possible etiology, clinical findings, operative procedures and the outcome of neonatal bowel perforation in Assiut University Children's' Hospital (AUCH). Patients and methods: Thirty neonates presented with bowel perforation were admitted to AUCH from March 2005 to February 2008. Eighteen were males and twelve were females. Thirteen were full term and seventeen were premature. Their ages at presentation ranged from 1 day to 15 days. Ten cases undergone closed abdominal drainage in the neonatal intensive care unit; while twenty were candidates for surgery in addition to two cases failed the conservative measures. Results: Abdominal radiograph showed free gas under diaphragm in 24 cases while in the remaining 6 cases the diagnosis of bowel perforation was made intra operatively. The peritoneal cavity was clean in two cases only. The cause of perforation was NEC in 10 cases, idiopathic in 9 cases, Hirschsprung's disease (HD) in 4 cases, Anorectal Malformations (ARM) in 2 cases, meconium ileus in 2 cases, and intestinal atresia in 3 cases. Conclusions: Early recognition of perforation contributes to successful management. Primary laparotomy should remain the standard method for initial surgical management of neonatal bowel perforation, as it provides safe and timely definitive management. However closed abdominal drainage has a very important life saving role in critically ill patients. Key words: Neonatal bowel perforation.

INTRODUCTION
espite the recently improved neonatal management, gastrointestinal perforation during the neonatal period is still a major problem for paediatric surgeons.1 Neonatal bowel perforation may be spontaneous (idiopathic) or due to necrotizing enterocolitis (NEC) or mechanical obstruction. Spontaneous bowel perforation in neonates was first described in 1825 but the first successful operation for neonatal ileal perforation was only in 1943.2Lloyd in

1969(3) reviewed 400 cases of spontaneous bowel perforation in an extensive manner. He discovered that stomach perforation accounted for more than half of the cases. Perforations due to mechanical intestinal obstruction as in imperforate anus, Hirschsprungs disease (HD), intestinal atresia, and meconium ileus are infrequently encountered at the present time because of earlier diagnosis and treatment.4,5 However

Correspondence to: Al Moutaz A. El Tayeb, MD. Pediatric Surgery Unit, Department of Surgery, Faculty of Medicine, Assuit University, E mail almoutazeltayeb@hotmail.com

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(NEC) has become the major cause of intestinal perforation especially in premature babies.(6,7) The aim of this study is to analyze the possible aetiology, clinical findings, operative procedures and the outcome of neonatal bowel perforation admitted to Assiut University Children Hospitals (AUCH).

then Clostridium Welchei and Klebsiella. leucocytosis > 10.000 /mm was encountered in 20 cases, thrombocytopenia < 140.000 /mm in 15 cases and prothrombin time > 13 sec. in 18 cases. Abdominal radiograph showed free gas under diaphragm in twenty four cases while in the remaining 6 cases the diagnosis of bowel perforation was made intra operatively. Twenty two cases were explored after correction of dehydration, electrolyte disturbance, acidosis and coagulopathy including the two cases failed to improve on closed abdominal drainage. Operative findings: The peritoneal cavity was clean only in 2 cases. The size of perforation ranged from 1 mm to 1.5 cm. sixteen cases had single perforation, while six had multiple perforations. Neonatal bowel perforation due to NEC occurred in10 cases; 7 of them were candidates for surgery, one had single perforation in the terminal ileum and six had multiple perforations in the ascending and transverse colon. All cases had the clinical manifestations and radiological signs of NEC with bowel necrosis on exploration. Resection of the necrotic bowel with primary anastomosis was done in two cases with smooth post operative recovery. Resection and stoma was done in the remaining five cases. Three of them died on the first two postoperative days because of septicaemia and bleeding tendency. They were premature and of low birth weight. Stomal closure for the survived two cases was done 3 months later.

PATIENTS AND METHODS

Thirty neonates presented with bowel perforation admitted to AUCH from March 2005 to February 2008. Eighteen were males and twelve were females. Thirteen were full term with mean gestational age 37 weeks + 0.81 and birth weight 2.930 + 0.22. Seventeen were premature with mean gestational age 34 weeks + 0.56 and birth weight 2.490 + -0.08. Their ages at presentation ranged from 1 day to 15 days. The most common presenting symptoms were poor oral feeding, progressive abdominal distension with bilious vomiting, tachypnea and failure or delayed passage of meconium or bleeding per rectum. Complete blood cell count, serum electrolytes, prothrombin time and blood culture were done to all patients as a preoperative preparation. Preoperative contrast enema was done in one case suspected to have obstructed Hirschsprungs disease. Ten cases had closed abdominal drainage in the neonatal intensive care unit (NICU) as a primary life saving procedure because of their poor general condition. Four of them had the clinical and radiological manifestations of NEC; the remaining six did not have these manifestations or any possible mechanical obstruction classified as idiopathic. Of these ten cases 6 improved on this conservative measures (2 with NEC and 4 with idiopathic). 2 died (1 with NEC and 1 with idiopathic) because of sepsis and were premature and of low birth weight. The remaining 2 underwent surgical exploration due to failure of conservative measures. IV antibiotics was given to all patients in the form of third generation cefalosporins (100 ml/kg/d), ampicillen + salbactum (150 ml/kg/d) and metronidazol (7.5 ml/kg/d). The follow up period varied from 6 months to 1 year after definitive surgical management.

RESULTS
Positive blood culture was encountered in 18 cases.The commonest organisms isolated were E Coli

Fig 1. Distribution of the thirty neonates according to the etiology of perforation.

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Table I. Showing various procedures done and mortality among the thirty neonates.
Aetiology NEC (10 cases) Closed drainage only Exploration Site of perforation Mortality 3 7 6 colon+1 small intestine 4 Idiopathic (9 cases) 5 4 3 small intestine+ 1 stomach 1 Hirschsprung's Disease (4 cases) 4 4 caecum ARM (2 casesa) 2 2 rectum Meconium Ileus (2 cases) 2 2 small intestine 1 Intestinal Atresia (3 cases) 3 2 small intestine+ 1 caecum -

Neonatal bowel perforation without evident mechanical cause or clinical or radiological signs of NEC (idiopathic) occurred in 9 cases; only four were candidate for surgery. Three had small localized single perforation in the ileum and one had single perforation in the posterior wall of the stomach without bowel necrosis. Simple closure to all cases was done with smooth post operative recovery. Bowel perforation due to HD occurred in four patients all of them had positive history and came with marked abdominal distension, bilious vomiting and failure or delayed passage of meconium. Preoperative contrast enema was done only in one case where the transitional zone was evident in the rectosigmoid junction so simple closure of the caecal perforation and levelling colostomy was done then abdominal pull-through (PT) was done 3 months later. The patient had good post operative recovery. The remaining 3 cases did not have preoperative contrast study because of their poor general condition and because of indefinite transitional zone on exploration the perforation was taken out as a stoma with multiple seromuscular biopsies from rectum, sigmoid, descending and transverse colon, where the transitional zone was determined in two cases (one in the rectosigmoid junction and other at the lower end of descending colon). Abdominal pull-through was done accordingly and followed by stomal closure. Both patients had smooth post operative recovery a part from minimal wound infection in one case responded to antibiotics and dressings. One case came with equivocal biopsy report so radio opaque capsule was introduced through the distal loop of the caecostomy and followed under screen to indicate the presence of peristalsis till it was arrested in the
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rectosigmoid junction and this determined the extent of resection. So abdominal pull-through (PT) was done and stomal closure was done 3 months later. All cases had positive rectal suction biopsy. Perforation due to anorectal malformations (ARM) occurred in two cases. Both were males presented at the age of 4 and 5 days. One of them had low imperforate anus (LIA) while the other had high type (HIA) without fistula. The site of perforation was in the rectum in both. Colostomy in the lower part of the descending colon with closure of perforation was done as a salvage procedure followed by formal correction with posterior sagittal anorectoplasty (PSARP) six months later for the HIA and anoplasty 3 months later for the LIA. Both patients had good post operative recovery and stoma was closed 3 months later. Bowel perforation due to meconium ileus occurred in the terminal ileum in two cases. One of them had very dense adhesions so stoma and washouts was done but he died on the second postoperative day from sepsis and DIC. The survived case had bishop-koop stoma and was discharged on regular stoma washouts. The stoma was closed three months later. Perforation due to intestinal atresia occurred in three cases (two cases with ileal atresia and one with colonic). For the ileal atresia resection of the terminal dilated part of the ileum and primary anastomosis was done. Both cases did well with smooth post operative recovery. For the case with colonic atresia the perforated proximal part of the colon was exteriorized as a stoma. Gastrografin enema to visualize the rectum and distal colon was done 3

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months later before final reconstruction and stoma closure. The overall mortality was 6 cases out of 30 all of them were premature and of low birth weight.

DISSCUSION
The aetiology of bowel perforation in the newborn has been significantly changed in the recent years. NEC that was hardly mentioned as a possible cause has become the leading cause of perforation especially in premature babies.(6) It was reported to affects 0.5% of all live births and 6% of premature infants with birth weight below than 1000 gram.(7) Although the most important risk factor for NEC is prematurity, NEC in fullterm infants had recently been increased.(8) There are certain risk factors in full terms including congenital heart diseases, birth asphyxia, intrauterine growth retardation and congenital hypothyroidism, immaturity of the gastrointestinal tract immune mechanism, ischemia and infection. All these factors have been suggested as etiological factors for NEC.(9) The commonest site of perforation due to NEC in our series was in the ascending and transverse colon and this correlates with that reported in the literature.(2) Pneumoperitonium is accepted as the absolute indication for surgical intervention.(10) Frey et al, 1987(11) reported the incidence of pneumoperitonium to be 50% to70% of cases with perforation. We had a higher rate of 80% in our series. However it is important to keep in mind that pneumoperitonium in the ventilated neonates may result from pulmonary air leak rather than intestinal perforation. Pneumatosis intestinalis is considered pathognomonic for NEC diagnosis. However it does not always correlates with the severity of the disease.(11) On the other hand portal venous gas not only correlates with the disease severity but also with high mortality rates reaching 100% in extremely LBW and 25% in LBW neonates.(12-13) In our series all cases had dilated fixed loopes and intestinal pneumatosis but only one case had portal venous gas. Souza at al.(14) had put relative indications for surgical exploration such as; fixed and tender abdominal mass, persistent fixed and dilated loops on serial x-rays, presence of gas in portal vein and cellulites of the abdominal wall. Cikrit et al(15) had reported high mortality rates among cases with NEC that were associated with bowel perforation especially those with the pathognomonic portal venous gas sign. However Bahaauldin et al, in his study does not show the presence of perforation to be associated with high
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mortality rates more than cases with NEC without bowel perforation.(16) We had a mortality of 40% with NEC presented by bowel perforation. All were premature and of low birth weight. However because of relative small number of this group we cannot compare them with the reported mortality in the literature. Also long term follow up is needed to record the late complications of NEC such as intestinal stricture, malabsorption, cholestatic liver disease and neurodevelopmental disorders. Idiopathic bowel perforation represents the second most common cause of perforation in neonates.17 The incidence has been estimated between 1.1% and 7.4% of all neonatal intensive care unit admissions of infants less than 1500 g and less than 1000 g respectively.(18) Deficiency of the enteric musculature in association with spontaneous intestinal perforation was first reported 1930.(19) A number of hypotheses have been suggested such as: local ischaemia, impaired collagen synthesis in smooth muscle secondary to exogenous steroids and trauma during pregnancy or delivery. Spontaneous gastric perforation was considered a congenital anomaly by Herbut20 in 1943. However Shaw et al 21 disprove this theory by suggesting that gastric perforation was caused by mechanical rupture secondary to increase gastric pressure rather than a congenital agenesis of gastric muscle. Rapid development of abdominal distension due to free gas and subsequently meconium discolouration seen through the thin anterior abdominal wall in an otherwise relatively stable premature neonate and without features of NEC is pathognomonic for spontaneous perforation.18,19 In comparison with prognosis of intestinal perforation due to NEC, idiopathic perforation had favourable outcome with mortality rates of 20%.(2) We had mortality in only one case out of nine with idiopathic perforation and four out of ten with NEC. However the mortality rates were reported to be much higher in prematures. Holland et al(19) reported a mortality rate of 26%. They referred this mortality to extreme prematurity and association of multiple medical comorbididty including hyaline membrane disease, pleural effusion and intraventricular haemorrhage. Many authors suggested closed peritoneal drainage as a primary or definitive procedure for cases with intestinal perforation whether due to NEC or idiopathic. 22,23 Bowel perforation occurs in 3.2% to 4.4% of patients with HD.(1) In our cases the site of perforation was in
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the caecum and this is in consistent with other authors.(24) It is crucial to confirm the diagnosis of HD and also to define the exact site of transitional zone before or even during the operation because it may be difficult to localize the transitional zone in the unused colon thereafter. Any attempt to close the stoma without reaching definite diagnosis about the possibility of HD will lead to a disastrous result. Perforations of the rectum in patients with ARM were reported to be rare.25 We had two cases both were presented late. Accordingly the possibility of perforation should be kept in mind in cases with delayed presentation.

5.

Bisquera JA, Cooper TR, Berseth CL. Impact of NEC on length of stay and hospital charges in very low birth weight infants. Pediatrics. 109:423-428,2002. Bell MJ. Perforation in the gastrointestinal tract and peritonitis in the neonate. Surg. Gynecol. Obstet. 160:20-26,1985. Uauy RD, Fanaroff AA, Korones SB, et al. Necrotizing enterocolitis in very low birth weight infants: biodemographic and clinical correlates. National institute of child health and human development, Neonatal research network. J Pediatr. 119:630-8,1991. Andrews DA, Sawin RS, Ledbetter DJ, et al. Necrotizing enterocolitis in term neonates. Am J Surg. 159:507-9,1990. Kliegman RM, Ganaroff AA. Necrotizing enterocolitis. N Eng J Med. 310:1093-1103,1984.

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CONCLUSION
Early recognition of perforation contributes to successful management. Sudden refusal of feeding or increased gastric residuals, unexplained tachypnoea and abdominal distension may point to gastrointestinal perforation and radiological studies of the abdomen are mandatory. Primary laparotomy should remain the standard method for initial surgical management of neonatal bowel perforation, as it provides safe and timely definitive management. However when the general condition of the baby cannot allow surgical exploration closed abdominal drainage is considered a very important life saving measure. Also it is mandatory to look for HD as a common cause of mechanical perforation before and during laparotomy. Missing such diagnosis may be disastrous.

10. Bell MJ, Ternberg JL, Feigin RD, et al. Necrotizing enterocolitis: therapeutic decisions based on clinical staging. Ann Surg. 187:1-7,1978. 11. Frey EE, Smith W, et al: Analysis of bowel perforation in necrotizing enterocolitis. Pediatr Radiol. 17:380-382,1987. 12. Kliegman RM, Fanaroff AA. Neonatal necrotizing enterocolitis in the absence of pneumatosis intestinalis. Am J Dis Child. 136:618-620,1982. 13. Rowe MI, Reblock KK, Kurkchubasch AG, et al. Necrotizing enterocolitis in the exteremly low birth weight infant. J Pediatr Surg. 29:987-991,1994. 14. De Souza JC, Da Motta UI, Ketzer CR. Prognostic factors of mortality in newborn with necrotizing enterocolitis submitted to exploratory laparotomy. J. Pediatr. Surg. 36:482-486,2001 15. Cikrit D, Mastandrea J, West KW, et al. Necrotizing enterocolitis: factors affecting mortality in 101 surgical cases. Surgery. 96:648-653,1984. 16. Bahaauldin KH, Hassib A, et al. Factors affecting survival in surgically treated neonates with necrotizing enterocolitis. Annals Pediatr. Surg. 3:11-18, 2007. 17. Grossfeld JL, Molinari F, Chaet M, et al. Gastrointetinal perforation and peritonitis in infants and children: experience with 179 cases over ten years. Surgery. 120:650-656,1996. 18. Resch B, Mayr J, Kuttnig-Haim M, et al. Spontaneous gastrointestinal perforation in very low birth weight infants- a rare complication in a neonatal intensive care unit. Pediatr Surg Int. 13:165-167,1998. 19. Holland A, Shun A, et al. Small bowel perforation in the premature neonate: congenital or acquired? Pediatr Surg Int. 19:489-494,2003.

REFRENCES
1. 2. komura H, Urita Y, Hori T, et al. Perforation of the colon in neonates. J Pediatr surg. 40:1916-1919, 2005. Zamir O, Goldberg M, Udassin R, et al. Idiopathic gastrointestinal perforation in the neonate. J pediatr surg. 23:335-337,1988. Lloyd JR. The etiology of gastrointestinal perforation in the newborn. J Pediatr. Surg. 4:77-85,1969. Camberos A, Kaushal P, Applebaum H. Laparotomy in very small premature infants with necrotizing enterocolitis or focal intestinal perforation: postoperative outcome. J Pediatr. Surg. 37:16921695,2002.

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El Tayeb A. et al 20. Herbut PA. Congenital defect in the musculature of the stomach with rupture in a newborn infant. Arch. Pathol. 36:191-194,1943. 21. Shaw A, Blanc WA, Santulli TV, et al. Spontaneous rupture of the stomach in the newborn: A clinical and experimental study. Surgery. 58:561-571,1965. 22. Cass DL, Brandt ML, Patel DL, et al. peritoneal drainage as definitive treatment for neonates with isolated intestinal perforation. J Pediatr. Surg. 35:1531-1536,2000. 23. Demestre X, Ginovart G, Figueras-Aloy J, et al. Peritoneal drainage as primary management in necrotizing enterocolitis: A prospective study. J Pediatr. Surg. 37:963-967,2002. 24. Swenson O, Sherman JO, Fisher JH. Diagnosis of congenital megacolon: an analysis of 501 patients. J Pediatr.Surg. 8:587-94,1973. 25. Sharma SB, Gupta V, Sharma V. Gastrointestinal perforations in neonates with anorectal malformations. Indian. J. gastroenterol. 23:107-8,2004.

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