Adult intestinal malrotation presenting as caecal volvulus with incidental findings of duplicate inferior vena cava and other rare abnormalities: case report

Abstract

Intestinal malrotation and duplication of the inferior vena cava are rarely diagnosed in adult patients; however, incidence is likely underestimated as they are usually asymptomatic. These congenital malformations have been previously reported in the same patient twice but never with colonic obstruction or ischaemia. A 25-year-old female presented with nausea, vomiting, obstipation, and abdominal pain, and on computed tomography of the abdomen and pelvis was diagnosed with a caecal volvulus and pneumatosis coli associated with intestinal malrotation requiring emergency right hemicolectomy. Incidentally, the patient was noted to have duplication of the inferior vena cava, azygos continuation of the inferior vena cava, and splenic fragmentation. This constellation of symptoms has not been reported in the literature previously. The pattern of malformations follows that of polysplenia syndrome. Although rare, awareness of these malformations can be useful to clinicians.

Keywords: caecal volvulus; duplicate inferior vena cava; intestinal malrotation; polysplenia syndrome.

Figure 1

Computed tomography of the abdomen and pelvis of 25-year-old female presenting with caecal volvulus, intestinal malrotation, duplicate inferior vena cava, azygos continuation of the IVC, and fragmentation of the spleen. RIVC, right inferior vena cava; LIVC, left inferior vena cava; RRV, right renal vein; LRV, left renal vein; AA, abdominal aorta; SP, splenic fragment. (A) Arrow indicates mesenteric whirl sign. (B) Arrow indicates suprahepatic IVC draining from right, middle, and left hepatic veins into the right atrium. (C) and (D) Arrow indicates communicating vein from right IVC to left IVC.

Figure 2

Computed tomography of the abdomen and pelvis of 25-year-old female patient. (A) Coronal slice demonstrating caecal volvulus with distended loop of colon. (B) Axial slice demonstrating 97 mm diameter distended caecum, multiple splenic fragments, arrow indicates pneumatosis coli, indicating colonic ischaemia. SP, spleen fragments.