Gingival fibromatosis combined with cherubism and psychomotor retardation: a rare syndrome.
Gingival fibromatosis is frequently an isolated condition, but rarely associated with some uncommon syndromes. This paper describes an 11-year-old patient with pronounced gingival enlargement, cherubic facial appearance, and psychomotor retardation and discusses the major aspects of the case. The most striking finding orally was the presence of grossly hyperplastic gingiva, which completely covered all teeth except the occlusal surfaces of some teeth. The swelling in the lower part of the face and the appearance of sclera beneath the iris suggest cherubism. The diagnosis was confirmed by the detection of giant cell regenerative granuloma and perivascular eosinophilic particles and osteoclasts after biopsy of the mandible. In this case, surgery was the only effective way to treat the patient. A full-mouth gingivectomy procedure was performed under general anesthesia in 2 stages. The case was followed for 12 months and no recurrence was seen. An appropriate oral hygiene regimen was established.
Yalçin
S
S
Department of Oral Surgery, School of Dentistry, University of Istanbul, Capa-Istanbul, Turkey.
Yalçin
F
F
Soydinç
M
M
Palandüz
S
S
Günhan
O
O
D001706:Biopsy; D002636:Cherubism / Q000150:complications* / Q000473:pathology; D002648:Child; D005260:Female; D005351:Fibromatosis, Gingival / Q000150:complications* / Q000473:pathology / Q000601:surgery; D005500:Follow-Up Studies; D005886:Gingival Hypertrophy / Q000150:complications / Q000473:pathology; D005890:Gingivectomy; D006101:Granuloma, Giant Cell / Q000150:complications / Q000473:pathology; D006801:Humans; D008334:Mandible / Q000473:pathology; D009069:Movement Disorders / Q000150:complications* / Q000473:pathology; D009910:Oral Hygiene; D010010:Osteoclasts / Q000473:pathology; D011597:Psychomotor Performance / Q000502:physiology*; D015422:Scleral Diseases / Q000150:complications / Q000473:pathology