Intrapulmonary ectopic liver associated with scimitar syndrome

We report a case of intrapulmonary ectopic liver associated with Scimitar syndrome. A two month old male child who presented with features of congestive cardiac failure underwent extensive cardiac and radiological investigations and was diagnosed with Scimitar syndrome. He was also found to have a mass in the lower lobe of the right lung, the possibilities being either a pulmonary sequestration or a diaphragmatic hernia. As his dyspnea progressively worsened, a right lower lobectomy was performed at the age of two years. Intraoperatively, no connecting pedicle or hernia sac was seen. The resected lung specimen showed a light brown mass enclosed by the lung parenchyma and microscopy showed cords of normal hepatocytes with portal tracts. The hepatocytes were positive for Hep Par1, and bile ducts were CK 7 positive. To the best of our knowledge, this is the first reported case of an intrapulmonary ectopic liver associated with Scimitar syndrome.