Superior Mesenteric Artery Syndrome Associated with a Malrotated Ectopic Left Kidney: A Rare Case Report and Surgical Considerations

Abstract

Background: Superior mesenteric artery (SMA) syndrome, also referred to as Wilkie syndrome, represents an uncommon aetiology of proximal intestinal obstruction, arising from extrinsic compression of the third portion of the duodenum between the abdominal aorta and the superior mesenteric artery. The syndrome is frequently precipitated by rapid or significant weight loss, which results in a reduction of the mesenteric fat pad and consequent narrowing of the aortomesenteric angle. While most cases are acquired, congenital anatomical variations contributing to SMA syndrome are relatively rare. Furthermore, the simultaneous occurrence of SMA syndrome with renal ectopia and intestinal malrotation is exceedingly uncommon, with only a limited number of cases documented in the literature. Recognition of these atypical associations is crucial for accurate diagnosis and appropriate management. Aim: To report a rare case of SMA syndrome associated with a malrotated ectopic left kidney and to emphasize the importance of detailed preoperative imaging and anatomical understanding in surgical planning. Case Presentation: An 18-year-old female presented with chronic postprandial epigastric pain, recurrent bilious vomiting, early satiety, and significant weight loss. Symptoms were exacerbated during menstruation and partially relieved in the supine position. Contrast-enhanced computed tomography (CT) with angiography demonstrated a markedly reduced aortomesenteric angle and distance, confirming SMA syndrome. Additionally, a malrotated ectopic left kidney was identified in the pelvis with anomalous renal vasculature. Conservative management failed, following which the patient underwent an elective retrocolic side-to-side duodenojejunostomy, resulting in complete symptomatic resolution. Conclusion: This case highlights a rare association of SMA syndrome with a malrotated ectopic kidney. Awareness of such anatomical anomalies and meticulous preoperative imaging are crucial to prevent iatrogenic vascular injury during surgery due to distorted retroperitoneal architecture. This case report highlights the presence of a rotational vascular anomaly and a condition that does not require prior structural anatomical alteration, but rather specific phenotypic characteristics, which could potentially confuse the physician in their diagnostic approach