Gingival Fibromatosis Combined With Cherubism and Psychomotor Retardation: A Rare Syndrome

Abstract

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.

Cherubism Combined with Epilepsy, Mental Retardation and Gingival Fibromatosis (Ramon Syndrome): A Case Report

“…In the present case, features such as epilepsy, GF, mental retardation in the setting OMIM online mendelian inheritance in man of cherubism may well relate to Ramon syndrome though hypertrichosis was not a feature [8][9][10]. Review of literature revealed that cherubism have been reported in association with gingival fibromatosis [5], psychomotor retardation and gingival fibromatosis [24], neurofibromatosis and multiple non-ossifying fibromas of the femoral bone [25,26], and Fragile X syndrome [27]. Others have reported diabetes mellitus, vascular skin lesions and ocular abnormalities as part of Ramon syndrome [12,21,22].…”

Hereditary Gingival Fibromatosis: Report of Two Cases

“…1,4,19,20 Bu ol gu çalış-masında, ai le sel ge çi şi gös te ren soy ağa cı in ce lendi ğin de, ay nı an ne den do ğan üç ço cuk ta da HGF gö rül me si ve ba ba la rın da bu lun ma ma sı, bu has ta lı -ğın oto zo mal do mi nant ge çiş li ol du ğu nu des tek lemek te dir.…”

“…HGF'nin men tal re tar das yon, epi lep si, hi per tri koz, Zim mermann-La band, Ra mon, Cross sen dro mu gi bi durum lar la bir lik te gö rü le bil di ği bil di ril miş tir. 1,3,7,19 Fa kat bu ol gu çalışmasında in ce le nen hiç bir ai le bire yin de her han gi bir ek bul gu ya rast lan ma dı. Tüm ai le bi rey le ri men tal açı dan nor mal ve sis te mik olarak sağ lık lı idi.…”

“…1,19,20 Ge nel lik le sert kı vam lı, so luk pem be renk li olan diş e ti bü yü me le ri ne, yapı şık diş e tin de be lir gin "stipp ling" ya pı eş lik eder. 1 Bu çalışmada, er kek has ta da da ha şid det li ola cak şekil de her iki has ta nın her iki çe ne sin de yay gın ola rak gö rü len HGF'nin kli nik bul gu la rı lite ra tür ile uyum lu dur.…”

“…His to pa to lo jik ça lış ma lar da HGF'nin uza mış "re te peg"le re sa hip hi perp la zik diş e ti epi te li al tın da, art mış kol la jen ve azal mış hüc re sel içe ri ğe sa hip fib röz bağ do ku suy la ka rakte ri ze ol du ğu gös te ril miş tir. 1,14,19,21 Bu iki ol gu da, he ma tok si len eo zin ile bo ya nan ke sit le rin ışık mik ros ko bun da in ce len me si so nu cun da uza mış "re te peg ler", epi tel de pro li fe ras yon ve ka lın de met ler oluş tu ran bağ do ku su kol la jen fib ril le ri iz len di. Diş e ti bağ do ku sun da inf la ma tu ar hüc-re le rin az ol ma sı, il ti ha bi be lir ti ler gös ter me yen diş e ti nin mev cut kli nik gö rü nü mü nü des tek lemek te dir.…”