Situs inversus totalis associated with a tumor in the cecum.

Abstract

Dear Editor: Situs inversus totalis (SIT) is a rare congenital condition characterized by a mirror-image transposition of both the abdominal and thoracic viscera with an incidence of 1/8,000 to 1/25,000 in the normal population. First described in 1600 by Fabricius [1], SIT may complicate the diagnosis and therapeutic management of abdominal pathology. This congenital condition involves inverted positions of all of the thoracic and abdominal organs that represent mirror images of their normal locations. In the abdomen, the stomach, spleen, and pancreas are on the right side of the body; the liver and gallbladder are on the left side; and the colonic flexures are reversed [2]. Here, we report the case of a 32-year-old man with SIT, colon cancer in the cecum, and malformation of the inferior vena cava (IVC) who underwent a right hemicolectomy. A patient was examined in our hospital for SITand a tumor in the cecum. His only significant medical history was fatigue and weakness. His family history was essentially negative for SIT or familial and hereditary diseases. The patient’s clinical features at admission were as follows: blood pressure, 130/ 70 mmHg; pulse, 60 beats/min; and temperature, 36.5 °C. The palpebral conjunctivae were anemic. Breath sounds were normal. The abdomen was flat, soft, and non-tender. A decreased red blood cell count of 3.8 million (range, 4.5–6 million) and a hemoglobin level of 12.2 mg/dL (range, 13–18 mg/dL) were noted with no hepatic or renal dysfunction or electrolyte abnormalities. Computed tomography (CT) revealed dextrocardia, situs inversus in the colon in which a splenic flexure of the colon was on the right side of the abdomen with the ileocecal region on the left side. A 3.0×2.5-cm elevated lesion was present in the cecum that was associated with regional adenopathies. The remainder of the colon and upper gastrointestinal tract were unremarkable. CT revealed abnormalities including an anomaly of the IVC. The area from the origin of the IVC to the level of the renal vein was normal. At this level, an interruption and extensive vena azygos localized on the left side due to situs inversus was seen. CT also showed dilatation of the vena azygos in the upper part of the posterior mediastinum in the thoracic cavity where the diameter was the same as that of the thoracic aorta (2.5 cm). A big development shows too, and venous hemiazygos were also seen. The hemiazygos vein was less dilated than the azygos vein. A short segment of the IVC was seen prior to the entrance of the right atrium. Based on these findings, the patient was diagnosed with a tumor of the cecum, and, hence, resection of the cecum and ascending colon were performed. An end-to-end functional anastomosis between the ileum and the transverse colon was performed. Histopathologically, the primary lesion was cecum colon cancer with moderately differentiated adenocarcinoma. Situs inversus itself has no pathophysiological significance but poses diagnostic and surgical difficulties that arise from the inversion. SIT is frequently associated with other disorders including Kartagener’s syndrome, primary ciliary dyskinesia, and asplenia or polysplenia. Other authors have reported additional concurrent anomalies of the digestive system including a short pancreas, symmetric lobulation of the liver, S. Bilali (*) General Surgery Department, University Hospital Center “Mother Teresa”, Tirana, Albania e-mail: sbilali@hotmail.com