Objective: Patients with malrotation, or an intestinal rotation abnormality (IRA), can experience serious adverse events. Increasingly, asymptomatic patients are being diagnosed with malrotation incidentally. Patients with symptomatic malrotation require surgery in an urgent or semiurgent manner to address their symptoms. The treatment of asymptomatic or incidentally discovered malrotation remains controversial.
Methods: Data were compiled from a broad search of Medline, Cochrane, Embase and Web of Science from January 1980 through January 2013 for five questions regarding asymptomatic malrotation.
Results: There is minimal evidence to support screening asymptomatic patients. Consideration may be given to operate on asymptomatic patients who are younger in age, while observation may be appropriate in the older patient. If reliably diagnosed, atypical malrotation with a broad-based mesentery and malposition of the duodenum can be observed. Regarding diagnostic imaging, the standard of care for diagnosis remains the upper gastrointestinal contrast study (UGI), ultrasound may be useful for screening. A laparoscopic approach is safe for diagnosis and treatment of rotational abnormalities. Laparoscopy can aid in determining whether a patient has true malrotation with a narrow mesenteric stalk, has nonrotation and minimal risk for volvulus, or has atypical anatomy with malposition of the duodenum. It is reasonable to delay Ladd procedures until after palliation on patients with severe congenital heart disease. Observation can be considered with extensive education for family and caregivers and close clinical follow-up.
Conclusions: There is a lack of quality data to guide the management of patients with asymptomatic malrotation. Multicenter and prospective data should be collected to better assess the risk profile for this complex group of patients. A multidisciplinary approach involving surgery, cardiology, critical care and the patient's caregivers can help guide a watchful waiting management plan in individual cases.
Keywords: Asymptomatic malrotation; Atypical malrotation; Diagnosis and surgical management of asymptomatic malrotation; Heterotaxy syndrome; Intestinal rotation abnormalities; Malrotation.
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