Anomalies of intestinal rotation and fixation: consequences of late diagnosis beyond two years of age

Abstract

The malrotation and no well fixation anomalies of the digestive tract is also frequent in older child, young and adult, with characteristic and specific clinical presentation. Actually, the diagnostic and treatment seem to be late, after suffering prolonged symptoms and/or in emergency. We present nine cases of anomalies in the embryonic development of the digestive tract which were diagnosed and treated in infants or young, all above 2 years old. Eight cases were of more or less complete intestinal malrotation; one of them was a complete malrotation with an intrinsic duodenal stenosis associated (no bands of Ladd) and another one was a right paramesocolic hernia, always accompanied by malrotation. The association with other extra-digestive anomalies, especially urological, was 70%. The predominant symptom was intermittent abdominal pain (IAP)-80%-sometimes accompanied by vomiting (35%) and episodes of diarrhoea (25%). In all the cases, while the clinical background was early, diagnosis was late. Indeed, in 60% of the cases diagnosis was made intra-operatively in emergency surgical interventions. The imaging procedures employed were scanning and Doppler ultrasound, CT scan, and contrast gastrointestinal series (GIS). Up to 30% of errors in interpretation occurred, although they were eventually corrected with other tests. The most reliable diagnostic procedures were GIS and CT scan with contrast, although partial interpretation errors occurred with the latter procedure. Surgery was essential in 80% of the pre-operative cases, and in another two it was required as a preventative measure. Post-operatively, there was notable persistence of SBS in the cases of intestinal necrosis, and of other lesser symptoms in the rest. We conclude that: intestinal malrotations and malfixations are still being diagnosed very late, with serious systemic consequences such as intestinal obstructions or necroses, and prolonged clinical suffering. This could all be avoided if more attention were paid to the digestive symptoms associated with IAP, and to subocclusion or other abdominal phenomena (distension,...), together with, in the case of doubt about the findings with the previous procedures, the opportune imaging tests (e.g., abdominal Doppler ultrasound, CT scan with contrast, and barium GIS). Unlike other authors, we consider that the morbidity/mortality associated with cases of late diagnosis of these anomalies is high, and calls for earlier surgical treatment.