Surgical therapy of primary intestinal lymphangiectasia in adults.

Primary intestinal lymphangiectasia (PIL) is a rare disorder, especially in adults. It causes a local disruption of chylus transport and is part of the exudative gastroenteropathies. Conservative therapy includes dietary measures or somatostatin medication. Taking the differential diagnosis of PIL into consideration is a major challenge, since patients suffering from PIL may present with diarrhoea and lymphedema or chylous ascites. This can be explained by the chronic lymphedema of the bowel leading to dilation of the vessels (intraluminal loss) and sometimes even to a rupture (peritoneal loss). Push-pull enteroscopy and capsule endoscopy are the proper interventional diagnostic tools to discover PIL. Exploratory laparoscopy may be useful in unclear cases. Surgical resection of the altered intestine has been described with positive results. Exploratory laparoscopy may even be a diagnostic tool in unclear cases. Resection of the altered intestine is a treatment option in symptomatic and treatment-refractory cases.