Adult Presentations of Congenital Midgut Malrotation: A Systematic Review

Abstract

Background Adult midgut malrotation is a rare cause of an acute abdomen requiring urgent intervention. It may also present in the non-acute setting with chronic, non-specific symptoms. The objective of this study is to identify the clinical features, appropriate investigations and current surgical management associated with adult malrotation. Methods A systematic review was conducted according to PRISMA guidelines, identifying confirmed cases of adult malrotation. Patient demographics, clinical features, investigation findings and operative details were analysed. Results Forty-five reports met the inclusion criteria, totalling 194 cases. Mean age was 38.9 years (n = 92), and 52.3% were male (n = 130). The commonest presenting complaints were abdominal pain (76.8%), vomiting (35.1%) and food intolerance (21.6%). At least one chronic symptom was reported in 87.6% and included intermittent abdominal pain (41.2%), vomiting (12.4%) and obstipation (11.9%). Computerised tomography scanning was the most frequent imaging modality (81.4%), with a sensitivity of 97.5%. The whirlpool sign was observed in 30.9%; abnormalities of the superior mesenteric axis were the commonest finding (58.0%). Ladd's procedure was the most common surgical intervention (74.5%). There was no significant difference in resolution rates between emergency and elective procedures (p = 0.46), but length of stay was significantly shorter for elective cases. (p = 0.009). There was no significant difference in risk of mortality, or symptom resolution, between operative and conservative management (p = 0.14 and p = 0.44, respectively). Conclusion Malrotation in the adult manifests with chronic symptoms and should be considered as a differential diagnosis in patients with abdominal pain, vomiting and food intolerance. Abbreviations CTComputerised tomography MRI Magnetic resonance imaging SMA Superior mesenteric axis

Intestinal malrotation in adults: incidental diagnosis.

“…Su incidencia es de 1/500 recién nacidos vivos (0,2% de la población) 1,3-7 coincidiendo con 1% identificado en autopsias 2 . No hay diferencias entre sexos y la edad media de presentación de los síntomas va de los 25 a los 45 años 4 . Malrotación intestinal en el adulto: diagnóstico incidental.…”

“…Característicamente el dolor aparece a nivel epigástrico o en el cuadrante inferior izquierdo del abdomen, pudiendo ser intermitente o fijo y de intensidad leve o moderada; suele aparecer postprandialmente con una duración de entre 30-60 minutos 1 . Aunque con menor frecuencia también pueden existir diarrea, dispepsia, pérdida de peso, intolerancia alimentaria, sensación de saciedad, déficits nutritivos e incluso trastornos del crecimiento 1,2,4,5 Habitualmente el diagnóstico de malrotación intestinal en los adultos se retrasa por la amplia constelación de síntomas que se pueden observar en otras muchas patologías 1 debiendo formar parte del diagnóstico diferencial de adultos que presenten dolor abdominal no explicado por otras causas 5 . La radiología convencional no presenta sensibilidad ni especificidad para el diagnóstico, aunque la presencia de pliegues yeyunales derechos o la ausencia de heces en colon a nivel de cuadrante inferior derecho pueden ser sugestivos 7 .…”

“…En el tránsito intestinal con contraste la unión duodeno-yeyunal no sobrepasa la línea media y descansa por debajo del nivel del bulbo duodenal 7 . El TAC es la prueba de imagen que presenta mejores resultados en el adulto 4,5 ya que no sólo muestra la malposición intestinal sino que informa de hallazgos extraintestinales que ayudan al diagnóstico 1,7 como: apariencia arremolinada de la vascularización que entra en una zona intestinal volvulada (signo del remolino), asas de intestino delgado en el cuadrante superior derecho del abdomen, falta de visualización del ciego en la fosa iliaca derecha, dilatación de asas duodenales y flexura duodeno-yeyunal hacia la derecha (signo del sacacorchos) [2][3][4] , asociado a la alteración de la relación anatómica entre la arteria y la vena mesentéricas superiores que muestra una relación vertical o incluso una inversión izquierda 1,7 .…”

Perforated Meckel’s Diverticulitis in a Patient with Unknown Intestinal Malrotation: Clinical Pitfall

“…Intestinal malrotation is a congenital malformation due to the incomplete rotation of the fetal intestine around the superior mesenteric vessels. With an incidence of 0.2%, it is diagnosed during the first year of life in 90% of cases [6]. Incidental diagnosis in adult patients is rare.…”

Combination of ectopic pancreas and intestinal malrotation presenting as non-specific right iliac fossa pain in a SARS-CoV-2 positive patient

“…Non-contrast radiography is of little diagnostic benefit, but the position of the small and large bowel may be suggestive. Contrast studies, especially CT scans, are used most commonly with a sensitivity of 97.5% 4 and may contain additional signs such as the vertical positioning of the superior mesenteric vein and artery. 5 Although controversial there is growing evidence on the benefits of surgical correction of malrotation in the adult population.…”