Ampullectomy for an unexpected ampullary hamartoma in a heterotaxic patient

Heterotaxy designates rare congenital disorders of organ positioning in the thoracic and abdominal cavities, which can be associated with numerous anomalies, complicating the surgical management because of the loss of conventional anatomic landmarks.

A 72-year-old man was found to have asymptomatic cholestasis. Further workup included computed tomography and magnetic resonance cholangiopancreatography that revealed anomalies of lateralization of digestive organs associated with intestinal malrotation and polysplenia, and a stone-like element in the main bile duct. Endoscopic retrograde cholangiopancreatography failed to extract the lesion. Laparotomy found no stone, but a polypoid tumor with ampullary implantation. Pancreaticoduodenectomy was judged unreasonable due to the presence of macroscopic cirrhosis and a complete ampullectomy was performed. Histopathological examination revealed a hamartomatous polyp.

The unusual angle of the duodenoscope in a left-sided duodenum may have contributed to the improper pre-operative diagnosis. Endosonography could have recognized the tissular origin of the lesion and prompted a more detailed preoperative planning. It was fortunate that the patient ended up receiving the appropriate treatment despite the absence of an adequate pre-operative diagnosis, as the option of performing an extended resection was ruled out due to the presence of cirrhosis.

Although heterotaxy leads to increased technical difficulties in performing usual endoscopic and surgical procedures, it can be safely managed by experienced surgeons as illustrated by the present case. Imaging modalities have limited sensitivity in the diagnosis of small ampullary tumors. As false-negatives are likely to occur, this possibility should guide the choice of the best operation.