Successful treatment of giant lymphatic malformation with symptomatic cutaneous vesicles by surgical excision and covera
- PDF / 788,683 Bytes
- 6 Pages / 595.276 x 790.866 pts Page_size
- 51 Downloads / 156 Views
CASE REPORT
Successful treatment of giant lymphatic malformation with symptomatic cutaneous vesicles by surgical excision and coverage with Integra® Beatriz Berenguer 1 Carolina Agra 5
&
Concepción Lorca-García 1 & Maria San-Basilio 2 & Minia Campos 3 & Angel Lancharro 4 &
Received: 27 May 2020 / Accepted: 21 September 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Lymphatic malformations (LMs) are the consequence of errors during embryological development of the lymphatic system. Large diffuse lymphatic malformations are troublesome. They can affect different tissue planes, and depending on the anatomic location, they can cause significant esthetic disturbance and interfere with normal function. LMs with skin involvement often manifest as multiple vesicles that ooze, bleed, itch, and get easily infected causing cellulitis with intense pain and fever, resulting in frequent hospital visits and impaired quality of life. We report one such case of a giant mixed type LM in the right lower extremity of an 11-year-old boy. The goal of this article is to discuss the currently available treatment modalities for these complex cases and to describe the successful outcome after an initial treatment with combined sclerotherapy and lasertherapy followed by a definitive surgical excision and coverage with Integra® and skin grafts. Level of evidence: Level V, therapeutic study. Keywords Vascular anomalies . Lymphatic malformations . Sclerotherapy . Laser . Surgery
Introduction Lymphatic malformations (LMs) comprise a wide range of pathologic conditions of the lymphatic system, collected in the ISSVA (International Society for the Study of Vascular Anomalies) classification [1]. Common lymphatic malformations are further classified into macrocystic, microcystic, or mixed type, according to ultrasound and MRI findings [2]. They consist of dilated lymphatic channels
* Beatriz Berenguer [email protected] 1
Division of Pediatric Plastic Surgery, Hospital General Universitario Gregorio Marañón, Madrid, Spain
2
Hospital General Universitario Gregorio Marañón, Madrid, Spain
3
Division of Pediatric Dermatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain
4
Division of Pediatric Radiology, Hospital General Universitario Gregorio Marañón, Madrid, Spain
5
Department of Pathology, Hospital General Universitario Gregorio Marañón, Madrid, Spain
lined by endothelial cells with lymphatic phenotype that stain with the immunohistochemical markers Prox-1 and podoplanin D2-40 [3] (Figs. 1 and 2). The etiology of LMs remains poorly understood. It has been recently suggested that LMs may be caused by somatic activating mutations in the PIK3CA gene, which leads to lymphatic hyperplasia [4]. They are usually present at birth, but they may become apparent months or years later. LMs can occur in any region with a lymphatic network, but most of them appear in the head and neck (48%), followed by trunk and extremities (42%) [5]. The clinical presentation ranges from small focal cysts to lar
Data Loading...
