Klipplel–Trénaunay–Weber Syndrome: A Case Report

The Klippel-Trénaunay-Weber (KTWS) syndrome is a triad of congenital anomalies characterized by hemangiomas, varicosities and unilateral bone and soft tissue hypertrophy. Hypertrophy usually affects one distal limb, but the trunk or face may be affected. Cutaneous hemangiomas (naevus flammeus) of varying extent and irregular contours are often present in the hypertrophic regions. Varicosities may also be part of the vascular lesions of this syndrome [1-5]. The pattern of inheritance for KTWS is unknown and its aetiology has not yet been determined, but it may be caused by mesodermal abnormalities during foetal development. Males and females are affected equally, and no racial predominance exists [6]. Although all oral tissues may be affected, only 5% of patients with KTWS show manifestations in the head and neck region [3]. The oral findings include an enlarged maxilla [7, 8], gingival enlargement [6], gingival fibromatosis [3, 9], displacement of teeth and malocclusion [8], premature eruption of teeth on the affected side [4] and hemifacial hypertrophy [10]. Angiomatous lesions on the lips, oral mucosa, tongue, palate and/or oropharynx have also been reported [7, 8]. The treatment is conservative in most cases. Surgery is reserved for patients with disabling morphological and functional alterations [5]. This report describes a case of gingival enlargement in an 8-year-old male child with KTWS. SUMMARY Klippel-Trénaunay-Weber Syndrome (KTWS) is a rare congenital malformation that may include port-wine stain, soft tissue and bony hypertrophy, and venous malformations and lymphatic abnormalities. The oral findings include an enlarged maxilla, gingival enlargement, malocclusion and premature tooth eruption. This report describes a case of gingival enlargement in an 8-year-old male child with KTWS. The extraoral clinical examination revealed discrete hemifacial hypertrophy on the left side and hyperpigmented spots on the anterior region of the neck, left arm and left leg. The intraoral clinical examination showed a deep palate and enlarged buccal and palatal gingiva in the anterior maxilla. Complete blood count and all general medical examinations showed values within normal range while the psychological evaluation indicated a mild learning delay. Although it rarely involves the orofacial region, KTWS should be included in the differential diagnosis of severe gingival enlargement. In view of the potentially dangerous complications that can arise, it is imperative the dental surgeon has a thorough knowledge of this condition and its management.