Hereditary gingival fibromatosis: aggressive 2-stage surgical resection in lieu of traditional therapy.
BACKGROUND
Hereditary gingival fibromatosis (HGF) is a rare condition characterized by progressive enlargement of the gingiva. Most cases follow autosomal dominant genetics, with a reported incidence of 1 in 750,000. In addition to cosmetic concerns, the compromised oral cavity may cause difficulty with eating, speech, hygiene, and oral competence. In addition, social consequences can be dramatic, forcing patients to lead isolated, reclusive lives. Traditional therapy uses serial gingival resections ("quadrantectomies") with primary closure. However, this method is associated with frequent recurrence and additional resections. We report on a family of 12 siblings, 8 of whom, including monozygotic twins, are affected with HGF. We describe corrective surgery for 3 siblings, as well as their long-term follow-up.


METHODS
Three sisters suffered from severe maxillary and mandibular gingival hyperplasia. All complained similarly of weight loss, difficulty with articulation, and social isolation. Physical examination revealed massive overgrowth of both maxillary and mandibular gingiva, as well as multiple ectopic teeth. Following diagnostic biopsy, all 3 patients underwent staged resections. The first stage involved resection of the maxillary component, followed by a planned second-stage resection of the mandibular component. All resections included gingivectomies, odontectomies, and alveolar ridge ostectomies. The oral cavity was allowed to heal by secondary intention.


RESULTS
All 3 patients were female, and ages ranged from 34-48 years (mean: 43). Follow-up on the 3 patients ranged from 3-18 months (mean: 11 months). There has been no evidence of recurrence to date. The gingiva remucosalized uneventfully within several weeks, and all patients are tolerating solid food. All patients report dramatically improved speech, oral hygiene, and self-confidence.


CONCLUSIONS
In an effort to address the high recurrence rate, we describe our aggressive surgical approach, including resection of the hypertrophied gingiva and alveolar processes. Two-staged gingival resection, as opposed to the more traditional 4-stage quadrantectomy approach, has resulted in no recurrence to date, suggesting an improved outcome over the traditional techniques.
Odessey
Eric A
EA
University of Chicago Hospitals, Section of Plastic Surgery, Chicago, IL, USA.
Cohn
Alvin B
AB
Casper
Francis
F
Schechter
Loren S
LS
D000328:Adult; D005260:Female; D005351:Fibromatosis, Gingival / Q000235:genetics* / Q000473:pathology / Q000601:surgery*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D019651:Plastic Surgery Procedures / Q000379:methods*