Dental Management of a Child With CLOVES Syndrome

El Chehouri H, Weiss J
Jamaica Hospital Medical Center, Jamaica, NY

Introduction:
CLOVES syndrome is a rare congenital disorder characterized by Congenital Lipomatous Overgrowth, Vascular malformations, Epidermal nevi, and Skeletal anomalies resulting from somatic, mosaic PIK3CA mutations. The condition is associated with progressive overgrowth and multisystem involvement that can significantly increase anesthetic and surgical risk and complicate access to routine dental care. As a result, affected patients are at increased risk for untreated dental disease, pain, and infection, necessitating individualized and multidisciplinary dental management.

Case Report:
This presentation describes a 5-year, 3-month-old African American female with CLOVES syndrome who presented for dental evaluation. Her medical history was significant for immunocompromise, anemia, coagulopathy, multiple vascular malformations, lung collapse, gastrostomy tube dependence, and a history of thrombotic events. She had previously undergone debulking surgeries for lymphatic malformations and was scheduled for right lower extremity amputation due to progressive overgrowth. Due to high anesthetic risk and medical instability, the patient was not a candidate for dental treatment under general anesthesia. Dental examination revealed extensive caries, poor oral hygiene, high caries risk, silver diamine fluoride application, and dental anxiety. A staged outpatient treatment plan was implemented using nitrous oxide for anxiolysis, protective stabilization, and minimally invasive restorative and preventive approaches. Medical clearance was obtained through coordination with the patient’s multidisciplinary care team. This case highlights the importance of conservative outpatient dental strategies and multidisciplinary collaboration to manage medically complex pediatric patients when surgical treatment is contraindicated.

Identify Supporting Agency and Grant Number: Research supported by departmental resources; no external grant funding was received.