An Unusual Congenital In Situ Malrotation of the Liver

Congenital anomaly of the liver is an uncommon and usually incidental finding. This report describes a case of in situ liver malrotation that has never been reported in the literature. Published literature relevant to the finding are briefly discussed. 1. Introduction Congenital deviations from the usual anatomy in the liver are uncommon. Some of such anomalies may be associated with severe genetic alterations or complex developmental defects and may eventually jeopardize liver function. Anatomical anomalies of the liver, for example, situs anomalies, or abnormal lobation including accessary lobe, in particular those causing little functional problem, can be detected in pediatric and adult patients during imaging exams [1], surgical procedures [2], or autopsies [3]. Recognizing various anatomical abnormalities of the liver will benefit clinical practice, especially valuable for radiologists and surgeons. Herein, a case of unusual anatomic anomaly of the liver is reported in an adult as an incidental finding during autopsy. 2. Case Report A 39-year-old African American female with no abdominal surgical history passed away due to catastrophic antiphospholipid syndrome secondary to active systemic lupus erythematosus with systemic thrombosis involving multiple organs including the lungs. The cause of death was supported by clinical presentation and laboratory studies which was confirmed by authorized autopsy. In addition, an unusual anatomical anomaly of the liver was demonstrated. The liver appeared to be malrotated in situ, with the inferior aspect and the gallbladder facing anteriorly (Figure 1(a)). The right lobe was unusually prominent, with the largest dimension of 23？cm from superior to inferior. The left lobe appeared to be atrophic (Figures 1(b) and 1(c)). The liver weighed 1,300 grams. The falciform ligament and the round ligament were thickened, whereas the bilateral triangular ligaments, the hepatogastric ligament, and the coronary ligament had become atrophic. The hepatogastric ligament was shifted to the left edge of the left lobe. The ligamentum venosum was completely obliterated. The hepatoduodenal ligament was identified and contained the common bile duct and the hepatic vessels. However, the ligament appeared to abnormally be away from the gallbladder neck region. Consecutive sectioning revealed uniform red-brown parenchyma with no gross fibrosis, nodules, tumor masses, or intravascular thrombi. The anteriorly located gallbladder was flattened and small, with a whitish glistening surface and thickened wall (Figure 1(a)). The gallbladder