Superior mesenteric vein absence with intestinal malrotation: a case report

Area of Science:

• Vascular Surgery
• Pediatric Surgery
• Gastroenterology

Background:

• Congenital abnormalities, especially combined malformations, are rare.
• Congenital malformation of the superior mesenteric vein (SMV) can range from asymptomatic to severe.
• When combined with intestinal malrotation, SMV abnormalities may necessitate early surgical intervention.

Observation:

• A 22-year-old patient presented with severe iron deficiency anemia since infancy due to the absence of the proximal superior mesenteric vein.
• Chronic blood loss from duodenal collaterals, formed as a compensatory mechanism, led to persistent anemia requiring transfusions.
• This case highlights a rare congenital absence of the SMV with a severe clinical course.

Findings:

• Surgical intervention was planned to address duodenal collaterals and chronic blood loss.
• A successful bypass surgery was performed, creating an anastomosis between a collateral vein and the anterior inferior pancreaticoduodenal vein.
• The procedure aimed to reduce duodenal vascularity and resolve anemia.

Implications:

• This case report emphasizes the potential clinical severity of rare SMV malformations.
• Understanding these anomalies is crucial for surgical planning in patients with combined congenital defects.
• Effective surgical strategies can manage chronic blood loss and associated anemia in complex vascular malformations.