Surgical Therapy of Segmental Jejunal, Primary Intestinal Lymphangiectasia.

Dec 2012

[Article in German]

Kneist W, Drescher DG, Hansen T, Kreitner KF, Lang H.

Source

Klinik und Poliklinik für Allgemein- und Abdominalchirurgie, Universitätsmedizin der Johannes-Gutenberg-Universität, Mainz.

Abstract

 

Key Words: primary intestinal lymphangiectasia - chylaskos - protein-losing gastroenteropathy - small bowel resection

Primary intestinal lymphangiectasia (PIL) is a protein-losing, exsudative gastroenteropathy causing lymphatic obstruction. Diagnosis depends on clinical examination and histological findings. Conservative treatment modalities include a low-fat diet and enteral nutritional therapy in order to reduce enteric protein loss and to improve fat metabolism. Other treatment options consist of administration of antiplasmin or octreotide to lower lymph flow and secretion. We report on a 58-year-old patient who underwent exploratory laparotomy due to a worsening physical status, recurrent chylaskos and leg oedema under conservative dietary therapy. Intraoperative findings showed a typical PIL of the jejunum about 20 cm distal to the Treitz's ligament. Histological examinations confirmed this diagnosis. One year after segmental small bowel resection (105 cm) with end-to-end anastomosis the patient is healthy, free of symptoms, has gained weight and his serum protein level has increased. Intraabdominal ascites and leg oedema (lymphedema) have not reoccurred since.

Thieme Journal