KLIPPEL-TRENAUNAY AND STURGE-WEBER OVERLAPPING SYNDROME WITH CRANIOFACIAL IMPAIRMENT: A CASE REPORT

Abstract

Klippel-Trenaunay syndrome (KTS) is characterized by a triad of port wine stains, venous malformations, and unilateral bone and/or tissue hypertrophy. Sturge-Weber syndrome (SWS) is a neurocutaneous syndrome of a classical triad of capillary malformations, ipsilateral leptomeningeal angiomatosis, and ocular involvement. The coexistence of KTS and SWS is rare, and only a few cases have been reported. Here, we describe a combination of KTS and SWS and report a case of a 27-year-old male patient, diagnosed with interictal dysphoric disorder, epilepsy, and mild intellectual disability. He presents with port wine stains involving the right side of his face and the right upper limb and skeletal hypertrophy. We also observed angiomatosis involving both lips on the right side causing macrocheilia, resulting in hypertrophy of buccal mucosa, malocclusion, and enlargement of the tongue. These characteristics add an extra challenge to the dental treatment and must be known by dental practitioners. Klippel-Trenaunay syndrome (KTS) is characterized by a triad of port wine stains, venous malformations, and unilateral bone and/or tissue hypertrophy. Sturge-Weber syndrome (SWS) is a neurocutaneous syndrome of a classical triad of capillary malformations, ipsilateral leptomeningeal angiomatosis, and ocular involvement. The coexistence of KTS and SWS is rare, and only a few cases have been reported. Here, we describe a combination of KTS and SWS and report a case of a 27-year-old male patient, diagnosed with interictal dysphoric disorder, epilepsy, and mild intellectual disability. He presents with port wine stains involving the right side of his face and the right upper limb and skeletal hypertrophy. We also observed angiomatosis involving both lips on the right side causing macrocheilia, resulting in hypertrophy of buccal mucosa, malocclusion, and enlargement of the tongue. These characteristics add an extra challenge to the dental treatment and must be known by dental practitioners.