Heterotopic supradiaphragmatic livers are rare. A total of 23 cases of primary supradiaphragmatic livers have been reported in the literature. The clinical presentations of heterotopic supradiaphragmatic liver are variable. The simultaneous detection of intrathoracic accessory liver and pulmonary sequestration is extremely rare, and only one case has previously been reported. It is difficult to make a correct diagnosis preoperatively. We presented a 53-year-old woman with complaints of an intermittent, productive cough and dyspnea for two months that was refractory to medical treatment. She had no previous history of trauma or surgery. A chest radiograph only showed a widening of the mediastinum. Contrast-enhanced computed tomography of the chest revealed a well-circumscribed homogenous soft-tissue mass, approximately 4.35 cm × 2.5 cm × 6.14 cm in size, protruding through the right diaphragmatic crura to the right pleural cavity, attached to the inferior vena cava, esophagus and liver. There was no conclusive diagnosis before surgery. After the operation, we discovered that this patient was the first case of a supradiaphragmatic heterotopic liver, which passed through the inferior vena cava foramen and was coincidentally combined with an intralobar pulmonary sequestration that was found intraoperatively. We discussed its successful management with surgical resection via a thoracic approach and reviewed the published literature.
Keywords: Intrathoracic liver; Pulmonary sequestration; Supradiaphragmatic ectopic liver.