Octreotide in Hennekam syndrome-associated intestinal lymphangiectasia.

Nov 2012

Al Sinani S, Rawahi YA, Abdoon H.

Source

Siham Al Sinani, Yusria Al Rawahi, Hamed Abdoon, Child Health Department, Sultan Qaboos University Hospital, Muscat 123, Sultanate of Oman.

Abstract

A number of disorders have been described to cause protein losing enteropathy (PLE) in children. Primary intestinal lymphangiectasia (PIL) is one mechanism leading to PLE. Few syndromes are associated with PIL; Hennekam syndrome (HS) is one of them. The principal treatment for PIL is a high protein, low fat diet with medium chain triglycerides supplementation. Supportive therapy includes albumin infusion. Few publications have supported the use of octreotide to diminish protein loss and minimize hypoalbuminemia seen in PIL. There are no publications on the treatment of PIL with octreotide in patients with HS. We report two children with HS and PLE in which we used octreotide to decrease intestinal protein loss. In one patient, octreotide increased serum albumin to an acceptable level without further need for albumin infusions. The other patient responded more dramatically with near normal serum albumin levels and cessation of albumin infusions. In achieving a good response to octreotide in both patients, we add to the publications supporting the use of octreotide in PIL and suggest that octreotide should be tried in patients with PIL secondary to HS. To the best of our knowledge, this is the first case report on the use of octreotide in HS-associated PIL.

World Journal of Gastroenterology

A case of Hennekam syndrome presenting with massive pericardial effusion.]

A case of Hennekam syndrome (lymphangiectasia) presenting with massive pericardial effusion.
Turk Kardiyol Dern Ars. 2008 Jul
Nişli K, Oner N, Kayserili H, Ertuğrul T.
Department of Pediatrics, Istanbul Medicine Faculty of Istanbul University, Istanbul, Turkey. kemalnisli@yahoo.com.

Hennekam syndrome is an autosomal recessive disease characterized by intestinal lymphangiectasia accompanied by severe lymphedema of the limbs, genitalia, and face, and learning difficulties. A 38-month-old boy was admitted with breathing difficulty. He had facial abnormalities and preputial hyperplasia consistent with Hennekam syndrome. Lymphangiography showed lymphedema in the left eye and right foot. Teleradiography showed cardiomegaly and echocardiography showed massive pericardial effusion. He first underwent pericardiocentesis for the removal of pericardial effusion, but pericardial tube drainage was required upon recurrence of effusion. On the fifth day, the drain was removed because of significant decrease in the drainage.

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