A case of Basal Cell Nevus Syndrome with Acute Maxillary Sinusitis.
- Author:
Kwang Hoon KIM
1
;
Young Ho SONG
;
Kyung Seob RHO
;
Hoe Young AHN
Author Information
1. Department of Otolaryngology, School of Medicine, Kyung-Hee University, Seoul, Korea.
- Publication Type:Original Article
- Keywords:
Basal cell nevus syndrome;
Maxillary sinusitis
- MeSH:
Basal Cell Nevus Syndrome*;
Carcinoma, Basal Cell;
Child;
Fibroma;
Follow-Up Studies;
Humans;
Jaw Cysts;
Maxillary Sinus*;
Maxillary Sinusitis*;
Medulloblastoma;
Nevus;
Ribs;
Skin;
Skull;
Spine
- From:Korean Journal of Otolaryngology - Head and Neck Surgery
2001;44(8):881-885
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
A basal cell nevus syndrome, known as the Gorlin syndrome, is a rare autosomal dominant disorder, occuring especially in the oriental population. It is a complex hamartomatous/neoplastic syndrome with multisystemic manifestations involving the five major features: (1) multiple basal cell nevi, occasionally basal cell carcinoma usually seen at an early age; (2) multiple jaw cysts; (3) skeletal abnormalities of ribs, skull, and spine; (4) ectopic calcification; (5) palmar and plantar pits. Because patients are predisposed to this disorder prior to basal cell carcinoma of the skin, ovarian fibroma, and medulloblastoma, the most important aspects of management is frequent examination and early treatment of small tumors. We report here about a 16-year- child with odontogenic maxillary sinusitis, who has developmental multisystemic disorders which coincided with the basal cell nevus syndrome. The patient underwent a sinus operation and now is under a cautious follow-up.
