Multimodality treatment of a massive cervicothoracic lymphatic-venous malformation in a 13-year-old boy

• Main Authors: Cynthia Reyes, MD, FACS, FAAP (Author), Taylor Parnall, BS (Author), Tania Kraai, MD (Author), Linda Butros, MD (Author), Anna Fabre, MD (Author), Jimmy Windsor, MD (Author), Jason McKee, MD (Author), Gresham Richter, MD (Author)
• Format: Book
• Published: Elsevier, 2017-06-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.
• Notes: 2213-5766; 10.1016/j.epsc.2017.03.011
• Keywords: Lymphatic-venous malformation; Kassabach-Merritt syndrome; Treatment; Pediatrics; RJ1-570; Surgery; RD1-811
• Source: Journal of Pediatric Surgery Case Reports, Vol 21, Iss C, Pp 15-18 (2017)

We report the multi-modality treatment of a massive cervicothoracic lymphatic-venous malformation (LVM) in a 13-year-old boy that was present at birth as a small cervical mass. At four years of age, sclerotherapy significantly reduced the size of the malformation but over subsequent years the mass increased dramatically in size and resulted in a consumptive coagulopathy and limited left shoulder function. Treatment with sildenafil was without effect. The consumptive coagulopathy improved with subcutaneous enoxaparin. A multi-disciplinary team excised part of the mass when he was 11 years old. After surgery, gradual enlargement of the mass and rise in d-dimer prompted treatment with sirolimus. Two sessions of sclerotherapy arrested enlargement of the mass. Multi-modality treatment resulted in a marked reduction of the LVM in the child. However, enlargement of the mass and further treatment is anticipated as a cure has not yet been found.