Complex Truncal Masses in the Setting of CLOVES Syndrome: Aesthetic and Functional Implications.

BACKGROUND

Congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and skeletal abnormalities (CLOVES) is a complex overgrowth syndrome with dramatic aesthetic and functional implications. The truncal masses characteristic of CLOVES syndrome are described as vascular malformations or lipomatous lesions with variable vascular components. Herein, we describe our single-institution experience with surgical excision of CLOVES-related truncal masses and discuss future directions in treatment of these complex anomalies.

METHODS

A single-institution retrospective review was performed for patients diagnosed with CLOVES syndrome. Patients undergoing excision of truncal vascular malformations were included. Outcome measures included perioperative characteristics [estimated blood loss (EBL), specimen size/anatomic location, blood-product requirement], as well as length-of-stay [LOS], and complication profile. Mean follow-up was 23.4 months (range 4.2-44).

RESULTS

Three consecutive patients were reviewed, accounting for 4 surgical operations. One patient underwent two operations for two distinct masses. All lesions were located on the upper back or flank with various degrees of muscular involvement. One patient required no transfusions with an uneventful 2-day hospitalization. The remaining three patients had an EBL ranging from 1500 to 6450 mL, requiring 9-13 units of packed red blood cells and 5-8 units of fresh frozen plasma during LOS (averaging 5 days). Mean weight of resected masses was 6.26 lbs (range 2.04-12 lbs) and mass dimensions ranged between 1778.9 and 15,680 cm. One patient with recurrence was subsequently treated with a combination of sclerotherapy and rapamycin, leading to significant mass reduction.

CONCLUSIONS

Management of CLOVES syndrome requires a collaborative and multimodal approach. Although surgical debulking is one treatment option, non-invasive medical modalities and sclerotherapy should be considered prior to surgical resection.

LEVEL OF EVIDENCE IV

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