Introduction ! Congenital segmental dilatation (CSD) of the small bowel is a rare cause of neonatal intestinal obstruction [1, 7, 8] and is associated with various degrees of intestinal obstruction [3]. CSD most frequently involves the small bowel [3], mainly the ileum and exceptionally the jejunum, duode- num or Meckel’s diverticulum [3,5,12]. To date, about 40 patients with small bowel segmental dilatation have been reported in the English-lan- guage literature [3], and about 60 cases have been reported worldwide [9]. In this article, we describe a particular case of CSD of the small bowel with its clinical presentation. Case Report ! A 9-year-old boy was admitted to hospital with severe malnutrition. He was born at term by ce- sarean section with a birth weight of 4200 g and without delayed passage of meconium. Since the age of seven, he had undergone multiple opera- tions in another hospital. According to their re- port, a dilated bowel loop had been found and not resected; instead, an intestinal anastomosis was confected together with an appendectomy. The patient was referred to us for complaints of repetitive intestinal pseudo-occlusions, bilious vomiting, severe cachexia, and weight loss up to – 2 SD. His physical examination revealed an epi- gastric distension with a sensitive left hypochon- drium and an absence of hepatosplenomegaly. Results of a sweat test and a PCR to search for cystic fibrosis were found to be normal. Plain ab- dominal X-ray showed a single, dilated, jejunal loop (l " Fig. 1); an upper gastrointestinal study with barium sulfate demonstrated a dilated stomach and a much more dilated single jejunal loop with barium stagnation. A diagnosis of seg- mental dilatation was proposed and a laparotomy was subsequently done, revealing a dilated jeju- nal loop with a length of 40 cm (l " Fig. 2), and a previously created diversion consisting of “gas- troileostomy and jejunoileostomy” (l " Fig. 3). The dilated loop was resected with an end-to-end anastomosis; the gastroileostomy and jejunoi- leostomy were disconnected, thus establishing normal digestive continuity. Histological exami- Abstract ! Congenital segmental dilatation (CSD) is a rare pathology of unknown etiology, usually with early-onset presentation in the neonatal age. We present a case of CSD of the jejunum of a 9-year- old boy with severe malnutrition. Its clinical, ra- diological and histological features are similar to those described in the literature but the remark- able aspect of this case lies in its late presentation. Fig. 1 Plain ab- dominal X-ray showing a single dilated jejunal loop. Congenital Segmental Dilatation of the Small Bowel (CSD) Authors P. Daher, J. Ghanimeh, E. Riachy, S. Zeidan, B. Eid Affiliation Department of Pediatric Surgery, Hôtel Dieu de France Hospital, Beirut, Lebanon Key words l " congenital segmental dilatation l " chronic small bowel obstruction l " abdominal distention l " jejunal obstruction Mots-clés l " dilatation segmentaire congénitale l " obstruction intestinale chronique l " distension abdominale l " obstruction jéjunale Palabras clave l " dilatación segmentaria congénita l " obstrucción intestinal crónica l " distensión abdominal l " obstrucción yeyunal Schlüsselwörter l " segmentale Dilatation l " Dünndarm l " chronischer Darmverschluss received April 30, 2007 accepted after revision May 29, 2007 Bibliography DOI 10.1055/s-2007-965535 Eur J Pediatr Surg 2007; 17: 289 – 291 © Georg Thieme Verlag KG Stuttgart • New York • ISSN 0939-7248 Correspondence E. Riachy Department of Pediatric Surgery Hôtel Dieu de France Hospital Boulevard Alfred Naccache, Achrafieh PO Box 16-6830 Beirut Lebanon eddy_riachy@msn.com 289 Daher P et al. Congenital Segmental Dilatation… Eur J Pediatr Surg 2007; 17: 289 – 291 Case Report