Objectives. Gorlin-Goltz syndrome (GGS) is a rare autosomal dominant disorder. The disease shows multiple organ involvement with variable clinical presentation. A multidisciplinary approach is required for its prompt clinical diagnosis and management. The diagnosis of the syndrome was based on its clinical presentation, radiological features and histopathological findings. Methods. This paper highlights a surgical and medical treatment of a case of GGS. A 42-years-old Italian female, affected by GGS, was referred to our surgical department for evaluation of multiple naevoid basal cell epithelioma and recurrent radiolucent jaw lesions. The syndrome was diagnosed based on its clinical presentation, radiological features and histopathological findings. Results. Removal of basal cell carcinomas and enucleation of the jaw cysts along with chemical cauterization with Carnoy’s solution were performed under general anesthesia. In the left side a mandibular resection BRC according to Urken classification was necessary, and a free fibula flap through a minimally invasive technique was harvested for mandibular reconstruction. During follow-up no sign of recurrence was detected, ongoing implant oral rehabilitation. Conclusion. Gorlin-Goltz syndrome (GGS) is a rare disorder. As we describe on the paper the disease shows multiple organ involvement with variable clinical presentation. We suggest a multidisciplinary approach for its prompt clinical diagnosis and management.

New medical and surgical findings in multidisciplinary approach of gorlin-goltz syndrome / A. Russillo, G.A. Beltramini, M. Romano, C. Baserga, A.R. Bolzoni, D.S. Rossi. - In: ORAL & IMPLANTOLOGY. - ISSN 1974-5648. - 11:2(2018), pp. 121-128.

New medical and surgical findings in multidisciplinary approach of gorlin-goltz syndrome

A. Russillo
;
G.A. Beltramini;M. Romano;C. Baserga;A.R. Bolzoni;
2018

Abstract

Objectives. Gorlin-Goltz syndrome (GGS) is a rare autosomal dominant disorder. The disease shows multiple organ involvement with variable clinical presentation. A multidisciplinary approach is required for its prompt clinical diagnosis and management. The diagnosis of the syndrome was based on its clinical presentation, radiological features and histopathological findings. Methods. This paper highlights a surgical and medical treatment of a case of GGS. A 42-years-old Italian female, affected by GGS, was referred to our surgical department for evaluation of multiple naevoid basal cell epithelioma and recurrent radiolucent jaw lesions. The syndrome was diagnosed based on its clinical presentation, radiological features and histopathological findings. Results. Removal of basal cell carcinomas and enucleation of the jaw cysts along with chemical cauterization with Carnoy’s solution were performed under general anesthesia. In the left side a mandibular resection BRC according to Urken classification was necessary, and a free fibula flap through a minimally invasive technique was harvested for mandibular reconstruction. During follow-up no sign of recurrence was detected, ongoing implant oral rehabilitation. Conclusion. Gorlin-Goltz syndrome (GGS) is a rare disorder. As we describe on the paper the disease shows multiple organ involvement with variable clinical presentation. We suggest a multidisciplinary approach for its prompt clinical diagnosis and management.
epithelioma; fibula; Gorlin-Goltz syndrome (GGS); vismodegib
Settore MED/29 - Chirurgia Maxillofacciale
https://www.oimplantology.org/common/php/portiere.php?ID=1527a801a21d9da6fdb234eec1cd762e
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/2434/661977
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