Extensive cervical-mediastinal cystic lymphatic malformation treated with sclerotherapy in a child with Klippel-Trenaunay syndrome Levent Duman a ,I ˙ brahim Karnak a , Devrim Akinci b , F. Cahit Tanyel a, * a Department of Pediatric Surgery, Hacettepe University Faculty of Medicine, 06100 Ankara, Turkey b Department of Radiology, Hacettepe University Faculty of Medicine, 06100 Ankara, Turkey Abstract Klippel-Trenaunay syndrome is a rare congenital disorder characterized by combined vascular malformations, varicosities, and limb enlargement. A child case of Klippel-Trenaunay syndrome with a large cervicomediastinal cystic lymphatic malformation and a vascular mass on the chest wall has been presented to demonstrate combined use of conservative and surgical approaches in this syndrome. Cystic lymphatic malformation was treated by sclerotherapies including ultrasound-guided transthoracic bleomycin injection into mediastinal cyst, which was tried for the first time in the literature, and vascular chest wall tumor necessitated surgical excision. D 2006 Elsevier Inc. All rights reserved. Klippel-Trenaunay syndrome (KTS) is a rare congenital abnormality characterized by combined capillary, venous and lymphatic vascular malformations, varicosities, and limb enlargement. It has been described by French physicians Klippel and Trenaunay in 1900. Males and females are frequently affected and the lower limb is the most affected part of the body. The treatment is usually conservative and surgery is preserved for specific conditions such as ulcerations, discrepancy of limb lengths, digital deformities causing functional disabilities, and bulky of mass distorting body configuration [1- 4]. Herein, the authors describe a case of KTS with a large cervicomediastinal cystic lymphatic malformation and a chest wall mass of combined vascular malformation that has been treated with conservative sclerotherapies and surgical approach such as excision of vascular tumor. 1. Case report A 14-month-old male infant was admitted to our unit with complaints of slowly enlarging masses located on the left side of the neck and on the left anterior thoracic wall in addition to abnormally developed left upper limb since birth. He was born to a healthy woman after an uneventful twin pregnancy. His sibling was healthy without any similar lesions. His body temperature, pulse rate, and blood pressure were in normal limits at physical examination. There was a soft, lobulated, painless cystic mass 5 Â 6 cm in size containing many small and large fluid-filled cysts on the left side of the neck. The mass was extending inferiorly into the thoracic cavity and the inferior margin of the mass could not be palpated. It was typical cystic lymphatic malformation. 0022-3468/$ – see front matter D 2006 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2005.10.067 * Corresponding author. Department of Pediatric Surgery, Hacettepe University, Children’s Hospital, 06100 Ankara, Turkey. Tel.: +90 312 3115541; fax: +90 312 3115541. E-mail address: ctanyel@hacettepe.edu.tr (F.C. Tanyel). Index words: Klippel-Trenaunay syndrome; Cystic lymphatic malformation; Mediastinum; Sclerotherapy Journal of Pediatric Surgery (2006) 41, E21–E24 www.elsevier.com/locate/jpedsurg