Congenital agenesis of the right lobe of the liver in situs ambiguous revealed by a colonic obstruction due to a wandering spleen torsion: report of a case

摘要

Congenital agenesis of the right lobe of the liver in situs ambiguous is rare. This abnormality revealed by a colonic obstruction due to a wandering spleen torsion has never been described. We report the case of a young man admitted for acute colonic obstruction associated with a painful hypogastric mass. Plain abdominal X-ray showed an enormous colonic dilatation with a soft-tissue density imaging in the lower middle quadrant. Laparotomy found a colonic obstruction due to pedicle torsion of a wandering spleen around the sigmoid. The liver was located at the left hypochondrium and presented a congenital agenesis of the right lobe with a retrohepatic gallbladder. The patient underwent splenectomy and sigmoidectomy. Liver and gallbladder were respected. The post-operative course was uneventful. This case presentation highlights the necessity to diagnose liver and other organ abnormalities in situs ambiguous earlier in order to prevent acute complications and to survey hepato-biliary patterns. Introduction Situs ambiguous or heterotaxia is a very rare situation characterized by an abnormal arrangement of organs and vessels without any mirror-image location of the viscera, relative to situs solitus (normal position of the heart and abdominal viscera) [1]. It is commonly asymptomatic and associated with abnormalities of location and morphology of liver and spleen. However, congenital agenesis of the right lobe of a left-sided liver associated with a wandering spleen revealed by an acute abdomen has never been described in situs ambiguous. The objective of this case presentation is to report an exceptional case of congenital agenesis of the right lobe of the liver in situs ambiguous revealed by a colonic obstruction induced by a pedicle torsion of a wandering spleen. Observation A 21-year-old man was referred to our emergency department because of diffuse and continuous abdominal pain, vomiting and constipation related to a 48-hour bowel obstruction. His past medical history was not significant except presence of a very mobile and indolent mass in the lower part of his abdomen. The patient had no history of previous abdominal surgery or trauma. On physical examination, he did not present jaundice or fever. The peripheral arterial pulses and blood pressure were normal and all vital signs were stable. The patient’s abdomen was much distended; moderate tenderness and diffuse tympanic percussion were noted. Hypogastric palpation found a large and firm painful mass. The rest of the physical examination was normal. There was a moderate thrombocytopenia while other laboratory data were normal. Plain abdominal X-ray showed evidence of colon obstruction, with enormous bowel dilatation which reached the diaphragm and two air-fluid levels in left and right flanks (figure 1). A normal liver silhouette was missing in the right upper quadrant. A spleen-like mass silhouette was seen as a soft-tissue density imaging in the lower middle quadrant.