Clinical Spectrum of Malrotation in Children

Abstract

The aim of the study. Retrospective analysis of diagnosis, surgical management and final outcome of malrotation in children. Material and methods. Clinical charts, operative notes and imaging studies of all children operated on for malrotation in years 1985-2005 were studied. Children in whom malrotation was diagnosed incidentally during an operation performed because of other indications were excluded. A detailed analysis of the presentation, signs and symptoms, clinical course, and diagnostic modalities used in affected patients was carried out. Anatomical variants of malrotation and their operative management were evaluated as well. Results. In the period from 1985 to 2005 forty children with malrotation were treated. There were 25 neonates, 10 infants and 5 children older than 1 year of age. Thirty-five children were referred directly as inpatients from other hospitals. An acute or urgent clinical presentation was noted in 32 children, while in 8 patients as the presentation was subacute or chronic. In all children bile-stained vomiting was the predominant sign. In 21 children, ileus was the referral diagnosis. Only 5 children were admitted with an established diagnosis of malrotation. In six cases duodenal atresia was suspected. Delay in diagnosis of congential intestinal anomaly was identified in 15 children. In five newborns, the initial diagnosis was enterocolitis. Four other patients, including one infant and three older children, were admitted to the pediatric departments several times before receiving the final diagnosis. In 18 children, the only pre-operative imaging was abdominal plain X-ray. In 22 patients, contrast studies were performed as well. All children underwent operative management. Ladd's syndrome was diagnosed in 16 patients, and in another 7 children was associated with small bowel torsion. Isolated small bowel torsion was noted in 7 patients. In 6 patients, midgut volvulus with strangulation was found intraoperatively. Two children with typical anatomical features of non-rotation presented with recurrent episodes of ileus. In single cases, mesocolic internal hernia and sigmoid torsion were noted. 5 children died postoperatively, four of whom had midgut volvulus with massive small bowel necrosis. Conclusions. Malrotation is a rare congenital anomaly that can present with a wide anatomical and clinical spectrum but should be considered in the differential diagnosis for all children, regardless of age, manifesting with bile-stained vomiting. Suspicion of malrotation seems to be an obvious indication for referral to a tertiary pediatric surgical department where necessary imaging should be undertaken with the participation of an experienced radiologist. Regardless of its clinical presentation, malrotation should be considered an unequivocal indication for surgical treatment due to risk of midgut volvulus with its serious sequelae.