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2.
J Craniofac Surg ; 21(3): 833-6, 2010 May.
Article in English | MEDLINE | ID: mdl-20485063

ABSTRACT

Gorlin-Goltz (GG) syndrome is an inherited autosomal dominant condition. Its diagnosis may be clinically confirmed by checking either major or minor signs that define the diagnostic criteria. It may occur that, although GG syndrome is a well-known condition, only the specific symptom could be observed by different specialists. Therefore, the patient cannot be placed into an always complex clinical panel. We introduce an example in this report. Throughout a 20-year clinical history characterized by the lack of proper diagnosis and missed follow-up operations, a patient with GG syndrome underwent partial amputation of the jaw after severe complications. A 52-year-old man required an implant-prosthetic rehabilitation since becoming edentulous after a partial resection of the jaw due to a keratocyst, which was later reconstructed through a free fibula flap. The observation of a typical phenotype and various symptoms that succeeded for longer than 20 years, with anamnestic evaluation and clinical examination, led us to suspect a complex pathologic condition such as GG syndrome, which was not previously considered, although the patient had undergone several polyspecialistic evaluations. Diagnosis has been eventually confirmed by a genetic study, which was always mandatory. The simultaneous presence of muscular and skeletal malformations, basocellular nevi, and multiple cysts of the jaw can represent signs linking to a condition such as GG syndrome. There are many syndromes involving the head and neck region, and specialists are supposed to be alerted when faced with similar typical expressions associated with a characteristic soma so as to avoid delays in diagnosing the syndrome.


Subject(s)
Basal Cell Nevus Syndrome/pathology , Follicular Cyst/pathology , Follicular Cyst/surgery , Jaw Cysts/pathology , Jaw Cysts/surgery , Mandible/pathology , Mandible/surgery , Odontogenic Cysts/pathology , Odontogenic Cysts/surgery , Amputation, Surgical , Basal Cell Nevus Syndrome/surgery , Diagnosis, Differential , Fibula/transplantation , Humans , Male , Middle Aged , Plastic Surgery Procedures , Surgical Flaps
3.
J Craniofac Surg ; 18(5): 1012-7, 2007 Sep.
Article in English | MEDLINE | ID: mdl-17912074

ABSTRACT

OBJECTIVE: Surgical therapy to improve the symptoms and the lesions in osteonecrosis (ON) of the jaws in patients in therapy with bisphosphonates. DESIGN: to evaluate the patient's therapeutic protocols, performance status, and factors promoting ON to prevent surgical failure. MATERIALS AND METHODS: 18 patients affected by osteonecrotic lesions of the jaws associated to BF, were treated by surgery. RESULTS: The results were recorded after 6 months. All the patients showed improvement of symptoms, in particular the pain. In addition, all the patients referred to a sensation of fresh and clean mouth, the disappearance of fetor ex ore, and a healthy mouth. CONCLUSIONS: The management and the resolution of BF osteonecrotic lesions is arguable and complex because in most cases, the patients are affected by oncologic disease when the better approach is prevention, but when the ON lesion is clear, surgery can improve the symptoms and in some cases, it can be resolute. To prevent surgical failure, it can be useful to evaluate the patient's therapeutic protocols, performance status, and factors promoting ON.


Subject(s)
Bone Density Conservation Agents/adverse effects , Diphosphonates/adverse effects , Jaw Diseases/surgery , Osteonecrosis/surgery , Aged , Female , Humans , Jaw Diseases/chemically induced , Male , Middle Aged , Osteonecrosis/chemically induced
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