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Jejunal atresia associated with idiopathic ileal perforation

P. C. Das, Rakesh Rai1, Grover J. Lobo1 Departments of and 1General Surgery, Fr. Muller Medical College and Hospital, Mangalore, Karnataka, India

Correspondence: P. C. Das, Department of Pediatric surgery, Fr Muller Medical College and Hospital, Mangalore, Karnataka - 575 002, India. E-mail: [email protected]

ABSTRACT

Jejunoileal atresia is one of the common causes of neonatal intestinal obstruction. Intestinal perforation with peritonitis in the neonatal period, which carries a high mortality rate, is also common. The association of jejunal atresia with idiopathic ileal perforation is very rare.

KEY WORDS: Intestinal perforation, idiopathic ileal perforation, jejunoileal atresia,

INTRODUCTION the patient underwent on the second day. During laparotomy, type-1 jejunal atresia was found in the newborn is one of the at approximately 10 cm distal to the D-J flexure, most common surgical problems, and its successful and a single distal ileal perforation was observed on management depends upon both timely diagnosis and the antimesenteric side with evidence of meconium prompt therapy. Intestinal atresia is one of the three peritonitis [Figure 1]. The entire part of the gut distal common causes of neonatal intestinal obstruction, the to the atresia was unused and collapsed. Further, there other two being Hirschsprung’s disease and anorectal was no evidence of gut inflammation. Jejunal atresia was malformation. Among the types of intestinal atresias, treated by tapering jejunoplasty and end-to-side jejuno- jejunoileal atresia is the most common one. Unlike jejunostomy by using a transgastric jejunostomy infant , it is less commonly associated feeding tube. Ileal perforation was treated with resection with Down’s syndrome, malrotation and congenital and anastomosis. Feeding was started through the cardiac defects.[1] We report a case of jejunal atresia transgastric jejunostomy tube on the 4th post-operative associated with distal ileal perforation. On search of day. Postoperative recovery was uneventful. The baby English literature, we find only one case of atresia with perforation in distal atretic segment.[2]

CASE REPORT

A full-term male neonate weighing 2.8 kg was born by spontaneous vaginal delivery. The child cried immediately after birth. Antenatal sonogram showed dilated bowel loops suggestive of intestinal obstruction. The mother did not had during pregnancy. The child was admitted to the neonatal intensive care unit. He was active, pink and stable. Cardiovascular system was normal. He had upper abdominal distension without any mass. An abdominal erect x-ray showed three air-fluid levels in the upper half of abdomen with the distal half being gasless. No free air was found in the abdomen. Renal function Figure 1: Proximal jejunal atresia (type-1). The tip of the forceps test was normal. With the diagnosis of jejunal atresia, indicates the site of distal ileal perforation

J Indian Assoc Pediatr Surg / Apr-Jun 2008 / Vol 13 / Issue 2 88 Das, et al.: Jejunal atresia with idiopathic distal perforation was discharged after two weeks. Histopathological not properly treated during preoperative, operative and examination of the resected perforated showed postoperative period. Today, mortality and morbidity single perforation with congestion and hemorrhage in of these cases can definitely be reduced with good ICU the submucosa. There was no evidence of necrotizing care, advanced anesthesia, proper antibiotics, early enterocolitis (NEC). Ganglion cells were present in the surgery and total parenteral/enteral nutrition. resected segments. ACKNOWLEDGEMENT DISCUSSION We are grateful to the Dean of Fr. Muller Medical College Jejunoileal atresia is the most common type of intestinal and the Medical Superintendent of Fr. Muller Hospital, atresia. There are several reports of non-NEC-related Mangalore for allowing us to publish this paper. neonatal intestinal perforation in the literature.[3-5] [2] Sajja et al. reported the first case of duodenal atresia REFERENCES associated with idiopathic small bowel perforations. [2] Possibly, our case is the second a similar case report 1. Grosfeld JL. Jejunoileal atresia and . In: Welth KJ, (perforation distal to the site of atresia). It is not yet Randolph JG, Ravitch MM, et al, editors. Pediatric surgery. 4th ed. proved whether idiopathic (non-NEC-related) bowel Chicago: Year Book Medical Publishers, Inc; 1986. p. 838-48. 2. Sajja SB, Middlesworth W, Niazi M, Schein M, Gerst PH. Duodenal perforation with intestinal atresia proximal to the site atresia associated with proximal jejunal perforations: A case report of perforation share a common etiology or it is just a and review of the literature. J Pediatr Surg 2003;38:1396-8. coincidence. Jejunoileal atresia is caused by mesenteric 3. Litwin A, Avidor I, Schujman E, Grunebaum M, Wilunsky E, vascular accident during the fetal life, whereas Wolloch Y, et al. Neonatal intestinal perforation caused by congenital defects of the intestinal musculature. Am J Clin Pathol duodenal atresia is due to the failure of vacuolization 1984;81:77-80. [1,6,7] from the solid-cord stage of development. The 4. Weinberg G, Kleinhaus S, Boley SJ. Idiopathic intestinal etiology of idiopathic intestinal perforation is not perforations in the newborn: An increasingly common entity. J completely known. Various causes suggested for the Pediatr Surg 1989;24:1007-8. 5. Mintz AC, Applebaum H. Focal gastrointestinal perforations not idiopathic intestinal perforation are the absence or associated with necrotizing enterocolitis in very thinning of muscularis propria, absence of muscular neonates. J Pediatr Surg 1993;28:857-60. layer, excessive resorption of muscularis as a part of 6. Koga Y, Hayashida Y. Intestinal atresia in fetal dogs produced regression of the omphalomesenteric duct and localized by localized ligation of mesenteric vessels. J Pediatr Surg [2,8] 1975;10:949-53. ischemic process. Intrauterine cocaine exposure has 7. Puri P, Fijimoto T. New observations on the pathogenesis of been reported as a cause of non-NEC-related intestinal multiple intestinal atresias. J Pediatr Surg 1988;23:221-5. perforation.[9] The site of perforation of idiopathic 8. Tatekawa Y, Muraji T, Imay Y, Nishijima E, Tsugawa C. The intestinal perforation has been reportedly found in the mechanism of focal intestinal perforations in the neonates with low birth weight. Pediatr Surg Int 199915:549-52. stomach, , ileum and colon – ileum being the 9. The TG, Young M, Rosser S. In-utero cocaine exposure and [2] most common site. Diagnosis of intestinal perforation neonatal intestinal perforation: A case report. J Natl Med Assoc with proximal intestinal atresia is only possible during 1995;87:889-91. laparotomy. The classical finding of 10. Raghuveer G, Speidel B, Marlow N, Porter H. Focal intestinal perforations in preterm infants are an emerging disease. Acta will never be observed in these types of cases by using Pediatr 1996;85:237-9. the following techniques: x-ray ultrasound, and CT 11. Zamir O, Goldberg M, Udassin R, Peleg O, Nissan S, Eyal F. abdomen. This is because of the presence of proximal Idiopathic intestinal perforation in the neonate. J Pediatr Surg atresia. Mortality and morbidity of idiopathic intestinal 1988;23:335-7. perforation with meconium peritonitis remains high. [10,11] Prognosis of patients with intestinal atresia with distal Source of Support: Nil, Confl ict of Interest: None declared. idiopathic intestinal perforation will be worse if it is

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