Unusual presentation of midgut malrotation with incidental nutcracker syndrome in adulthood: case report and literature review

Abstract

SummaryMalrotation of the midgut is generally regarded as paediatric pathology with the majority of patients presenting in childhood. The diagnosis is rare in adults, which sometimes results in delayed diagnosis and treatment. We present the case of a 28-year-old woman who presented with vomiting and mild acute pain in the abdomen. CT scan showed abnormal location of the midgut and abnormal relation of the superior mesenteric artery (SMA) and superior mesenteric vein (SMV) and duodenal jejunal flexure, confirming midgut malrotation. Peroperative findings showed the duodenum, small bowel loops located on the right side of the abdomen. The caecum and appendix were located at the midline in the epigastrium with the presence of Ladd's bands. SMA right and anterior to SMV whirled around SMA. Incidentally, the intraoperatively dilated left renal vein was compressed between the SMA and the aorta, which was confirmed retrospectively on CT scan with no symptoms related to the condition. BACKGROUND

Superior mesenteric artery syndrome: A review of the literature

“…8,16 Rarely, Nutcracker syndrome also can be related to a normal variant retroaortic left renal vein that gets compressed by the aorta anteriorly and the vertebral body posteriorly, but this version is much less common than the classic anterior form. 17,18 Nutcracker syndrome can be associated with other anatomical pathologies, such as gut malrotation or, as in our patient's case, SMA syndrome. 8 This possible association of SMA and Nutcracker syndrome with Ehlers-Danlos syndrome (EDS) has also been described, 19 so this triad should be considered in other similarly presenting tall, overly flexible patients.…”

Nutcracker syndrome complicated with intestinal malrotation

“…To the best of our knowledge, however, there have been no reports regarding paediatric cases of preoperatively diagnosed NCS associated with intestinal malrotation. In contrast, there has been an adult case of intraoperatively and incidentally identified asymptomatic NCS complicated with intestinal malrotation 7. The present report describes the first paediatric case of NCS, complicated with (and possibly induced by) intestinal malrotation, wherein haematuria improved after surgical intervention for intestinal malrotation.…”

Right Paraduodenal Hernia as a Cause of Acute Abdominal Pain in the Emergency Department: A Case Report and Review of the Literature

“…Additionally, abdominal CT can exclude other causes of abdominal pain or depict concomitant conditions. A rare association that has been recently highlighted by a few case reports is that between gut rotation abnormalities and the nutcracker phenomenon [ 14 , 15 , 16 ]. The nutcracker phenomenon involves the entrapment and compression of the left renal vein between the aorta and SMA when the angle they form is steeper than usual.…”