Literature Review and case report of two CLOVES Syndrome patients treated with sclerotherapy

SAIMA AHMAD, Adeel Riaz, Ahmed Imran, Abid Ali Qureshi



CLOVES syndrome is a rare congenital overgrowth disorder caused by mutations in the phosphatidylinositol 3-kinase catalytic subunit alpha (PIK3CA) gene. It is part of the PIK3CA-related overgrowth syndrome (PROS) spectrum and its treatment is challenging. The truncal masses characteristic of CLOVES syndrome are described as vascular malformations or lipomatous lesions with variable vascular components PROS malformations have traditionally been treated by surgery, but research into other treatments has increased over the past decade. 

Herein, we describe our institution's experience with scleroembolization of CLOVES-related vascular malformation and discuss future directions in the treatment of these complex anomalies.



A single-institution retrospective review was performed for patients diagnosed with CLOVES syndrome. One patient who underwent sclerotherapy for vascular malformation was included and another patient diagnosed with CLOVES was included. The outcome of sclerotherapy was determined by clinical examination, post-sclerotherapy ultrasonography and subjective feelings of the patient. Complications and follow-up duration (1.5 years) were also recorded.



Two pediatric patients were reviewed, accounting for 2 sclerotherapy sessions. One patient underwent two sclerotherapy sessions for large truncal vascular malformation with mass. The other case was only diagnosed with CLOVES syndrome. Both lesions were located on the upper chest, thorax and back or flank with various degrees of muscular involvement. We gave bleomycin foam according to the weight of the baby in the range of 3-5mg per session. After two sessions of bleomycin foam sclerotherapy, the lesions were completely gone and the complete resolution of symptoms. 



Sclerotherapy with bleomycin foam is a non-surgical procedure that can help reduce the size of the vascular anomalies associated with CLOVES, as well as the pain they may cause. 


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