A giant cystic lymphangioma of the abdominal body wall in a child

An 8-year-old boy was admitted to our vascular center with painless mass on the right abdominal wall which had been gradually increasing since his birth. He did not have any work-up of the mass in the prior 8 years and he had no fever or any other associated symptom. On physical examination, a giant, soft, well-defined lump was found in the right side of abdominal wall. (Figure A ) He underwent a computed tomography (CT) scan which showed a giant mass on the right abdominal wall. (Figure B) Contrast-enhanced magnetic resonance imaging (MRI) examination confirmed a 390mm × 230mm mass, with altered signal intensity involving the soft tissues of the anterior, lateral and posterior abdominal wall on the right side. There were multiple hypointense intralesional septations, but with no intra-abdominal extension. (Figure C) This mass was consistent with a diagnosis of cystic lymphangioma. Surgical resection was recommended, but the patient's guardians refused further surgical treatment and chose conservative treatment with observation for financial difficulties. The cystic lymphangioma (CL) is a benign congenital malformation of the lymphatic system, affecting mostly the craniofacial region, neck and chest. It is usually discovered during childhood accidentally and the location in the abdominal body wall is uncommon.1Mulliken J.B. Fishman S.J. Burrows P.E. Vascular anomalies.Curr Probl Surg. 2000; 37: 517-584Crossref PubMed Google Scholar, 2Abernethy L.J. Classification and imaging of vascular malformations in children.Eur Radiol. 2003; 13: 2483-2497Crossref PubMed Scopus (74) Google Scholar, 3De Perrot M. Rostan O. Morel P. Le Coultre C. Abdominal lymphangioma in adults and children.Br J Surg. 1998; 85: 395-397Crossref PubMed Google Scholar The natural course of the CL is slow progression in size. It is usually asymptomatic but may be symptomatic depending on the size, which can also cause abdominal discomfort because of its mass effect and sometime complications such as peritonitis by rupture, hemorrhage, or infection.3De Perrot M. Rostan O. Morel P. Le Coultre C. Abdominal lymphangioma in adults and children.Br J Surg. 1998; 85: 395-397Crossref PubMed Google Scholar Contrast-enhanced MRI is the most important diagnostic tool as it can show the extent of the lesion and differentiate it from nonvascular and other vascular lesions.2Abernethy L.J. Classification and imaging of vascular malformations in children.Eur Radiol. 2003; 13: 2483-2497Crossref PubMed Scopus (74) Google Scholar Complete surgical excision is recommended if there is no contraindication for surgical treatment.4Makni A. Chebbi F. Fetirich F. Ksantini R. Bedioui H. Jouini M. et al.Surgical management of intra-abdominal cystic lymphangioma. Report of 20 cases.World J Surg. 2012; 36: 1037-1043Crossref Scopus (62) Google Scholar The author received no financial support for the research, authorship, and/or publication of this article.