Eur J Pediatr (2003) 162: 812–813 DOI 10.1007/s00431-003-1308-6
SHO RT REPOR T
Wael El-Matary Æ Mathew Jones Æ Neville Wright Mark Dalzell
Intestinal malrotation: an unusual presentation
Received: 15 May 2003 / Accepted: 5 August 2003 / Published online: 20 September 2003 Ó Springer-Verlag 2003
Intestinal malrotation is a common cause of upper gastro-intestinal obstruction and presents with duodenal obstruction caused by volvulus of the midgut loop [6]. Malrotation syndromes typically present during the first few months of life, but may sometimes present later, causing diagnostic difficulties [7]. We report an unusual presentation of malrotation. A 5-year-old boy initially presented, at the age of 18 months, with short history of anorexia, followed by nonbilious vomiting and fever, which was diagnosed as a non-specific viral infection with gastritis. Six months later, he presented with weight loss, occasional diarrhoea, vague recurrent abdominal pain but the most significant feature was his extremely poor appetite and suboptimal caloric intake. On both occasions, physical examination was unremarkable, but in view of his significant loss weight, he had a range of investigations including liver function tests, serum albumin level, coeliac disease screen, auto-antibody screen, immunoglobulin (Ig) profile, sweat test and urine and stool analyses. All investigations proved negative apart from an elevated C-reactive protein and the presence of fat globules in stool. Abdominal ultrasound and upper gastrointestinal endoscopy with biopsies were normal. Caloric supplements were prescribed. A few months later, he presented again with a similar picture. On physical examination, his abdomen was slightly distended without tenderness or rigidity and routine investigations showed a low lymphocyte count, hypoalbuminaemia (23 g/l), low serum IgG levels (total and subclasses), low but normal levels of the other immunoglobulins, raised faecal alpha-1-antitrypsin (9.75 mg/g stool, reference range 0.13–2.38 mg/g stool).
W. El-Matary (&) Æ M. Jones Æ N. Wright Æ M. Dalzell Gastroenterology Unit, The Royal Liverpool Children’s NHS Trust Alder Hey, Eaton Road, L12 2AP Liverpool, UK E-mail:
[email protected] Tel.: +44-151-2525373 Fax: +44-151-2525928
Fig. 1 A frontal view of the abdomen during barium meal followthrough examination shows a low-lying duodeno-jejunal junction and small bowel loops lying in the right flank consistent with malrotation
He was recognised as having an unexplained proteinlosing enteropathy and, in view of poor caloric intake, was treated with nasogastric supplementary nutrition. However, he developed persistent diarrhoea and abdominal pain and was initially managed with total parenteral nutrition and subsequently with enteral elemental feed. Over the next 3 months, his nutritional state improved and his serum albumin returned to normal (41 g/l). Normal diet was introduced. At the age of 3 years he deteriorated again and was re-investigated. Colonoscopy revealed colonic inversus
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and this was confirmed by an upper gastrointestinal contrast study which demonstrated malrotation (Fig. 1). A laparotomy with Ladd’s procedure was performed. Since then, the patient has made a full recovery with normalisation of his lymphocyte count, serum albumin, total IgG and subclasses and faecal alpha-1-antitrypsin levels. He is now eating a normal diet and his weight has returned to the 50th percentile. Although there have been a few case reports of malrotation presenting with diarrhoea and growth failure secondary to protein losing enteropathy, it is still not a well-recognised cause of feeding difficulties. The site of protein loss is not well characterised. Postulated mechanisms of malabsorption and protein loss include diffusion between mucosal cells, ruptures of lymphatics through the mucosal surface, and the leakage of protein across ulcerated mucosa [7]. Maxson et al. [4] reported 2 of 22 cases with malrotation (aged above 2 years) presenting with diarrhoea as a major symptom but he did not identify protein-losing enteropathy. Powell et al. [5] reported a series of patients, over the age of 2 months, with malrotation with an equal incidence of bilious and non-bilious vomiting, as well as a high incidence of vague non-specific complaints such as diarrhoea, constipation, failure to thrive, chronic intermittent abdominal pain, malabsorption and melaena. The concept that malrotation should be considered in the differential diagnosis of growth failure was emphasised in a paper published in 1986 by Yanez and Spitz [8]. In a review of 37 cases of malrotation which occurred in previously healthy children during a 12-year period [1], bilious vomiting was the commonest symptom followed
by constipation. Nevertheless, the majority of these children was younger than 8 weeks of age [1]. In general, older children have a longer course of vague antecedent symptoms such as intermittent, nonbilious vomiting and chronic abdominal pain [2, 3] which might contribute to feeding difficulties and nutritional debility. The combination of feeding disorder, growth failure and unexplained protein-losing enteropathy should prompt the consideration of malrotation as a possible cause.
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