Abstract

Congenital intestinal malrotation is mainly detected in childhood and caused by incomplete rotation and fixation of the intestines providing the prerequisites for life-threatening volvulus of the midgut. The objective of this study was to evaluate a large cohort of adult patients with intestinal malrotation. Thirty-nine patients, 15–67 years, were diagnosed and admitted to a university setting with congenital intestinal malrotation 2002–2013. The patients were divided into three age groups for stratified evaluation. Medical charts were scrutinized, and clinical outcome of surgery was reviewed. Twelve patients presented as emergency cases, whereas 27 were admitted as elective cases. Diagnosis was established in 33 patients who underwent radiological investigation and in the remaining 6 during surgery. A Ladd’s operation was performed in 31 symptomatic patients; a conservative strategy was chosen in eight cases. Volvulus was more common in the younger age group. Twenty-six surgically treated patients were available for telephone interview, 1–12 years after surgery. All patients, except one, regarded their general condition improved to a high degree (n = 18) or with some reservation (n = 7). Twelve patients suffered remaining abdominal pain of a chronic and diffuse character. Due to recurrence of malrotation six patients were reoperated. Symptomatic malrotation occurs in both children and the adult population. Improved awareness and an accurately performed CT scan can reveal the malformation and enable surgical treatment. A Ladd’s procedure relieved most patients from their severe complaints even when a history of several years of suffering existed.

Highlights

  • In congenital intestinal malrotation an impaired embryological development of the gut causes incomplete rotation and fixation of the intestines to the abdominal wall (Dott 1923)

  • The aim of this study was to increase knowledge concerning this diagnosis by describing symptoms, treatment and clinical outcome in our cohort of adolescent and adult patients with intestinal malrotation

  • Intestinal congenital malrotation should be recognized as a reason for abdominal pain in adults which has been emphasized in a recent population based study by Coe et al (2015)

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Summary

Introduction

In congenital intestinal malrotation an impaired embryological development of the gut causes incomplete rotation and fixation of the intestines to the abdominal wall (Dott 1923). The fixation of the full-length bowel is complete during the twelfth week (Penco et al 2007). Congenital malformations such as diaphragmatic hernia, omphalocele or gastroschisis are associated with. In the worst case scenario, malrotation may develop into a midgut volvulus with torsion causing high risk of ischemia and necrosis of the parts of the intestine supplied by the superior mesenteric artery. This life-threatening condition is well known among pediatric surgeons and is always considered when physicians treat critically ill infants with abdominal symptoms and unknown diagnoses

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