Case Report
Copyright ©2012 Baishideng Publishing Group Co., Limited. All rights reserved.
World J Gastroenterol. Nov 21, 2012; 18(43): 6333-6337
Published online Nov 21, 2012. doi: 10.3748/wjg.v18.i43.6333
Octreotide in Hennekam syndrome-associated intestinal lymphangiectasia
Siham Al Sinani, Yusria Al Rawahi, Hamed Abdoon
Siham Al Sinani, Yusria Al Rawahi, Hamed Abdoon, Child Health Department, Sultan Qaboos University Hospital, Muscat 123, Sultanate of Oman
Author contributions: Al Sinani S participated in study concept, analysis of data, preparing the initial and final draft of the manuscript, critical revision of the manuscript for intellectual content and manuscript submission; Al Rawahi Y collected data and participated in the first draft of the case reports; Abdoon H cared for and started the first patient on octreotide.
Correspondence to: Siham Al Sinani, MD, FAAP, FRCPC, Child Health Department, Sultan Qaboos University Hospital, Al Khod, Muscat 123, Sultanate of Oman. siham_ss@hotmail.com
Telephone: +968-241-43403 Fax: +968-241-43009
Received: September 6, 2011
Revised: April 23, 2012
Accepted: August 15, 2012
Published online: November 21, 2012
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.

Keywords: Hennekam syndrome; Lymphangiectasia; Octreotide; Protein losing enteropathy