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Basal cell nevus syndrome (aka Gorlin syndrome, Gorlin-Goltz syndrome, nevoid basal cell carcinoma syndrome, and fifth phacomatosis) is a rare but well-described autosomal dominant condition with variable penetrance. We present a female patient who has been successfully treated using local surgical excision and diligent skin surveillance for more than 4 decades, demonstrating that simple local incision is an efficacious and reasonable surgical alternative that may circumvent the specialization and expense of Mohs technique.

The treatment of multiple basal cell carcinomas in patients with Gorlin's syndrome presents a therapeutic challenge. The carbon dioxide laser presents a unique treatment option due to increased surgical speed, a bloodless operating field, reduced postoperative pain and discomfort, and acceptable scars. Six patients with Gorlin's syndrome have been treated with the carbon dioxide laser. Between 14 and 110 lesions were treated in one session under local anesthesia. Pre- and postlaser biopsies of the lesions confirm complete eradication of the tumors. Mean follow-up is 20 months. No local recurrence has been observed.

Gorlin's syndrome is a complex hamartomatous/neoplastic syndrome with multisystemic manifestations involving the skin, central nervous system, and bony skeleton. The purpose of this article is to describe how a patient with Gorlin's syndrome was managed with a multipronged approach that included exposure osteotomies for keratocyst removal combined with orthognathic surgery. This case demonstrates the advantages of standard maxillofacial osteotomies to gain access for the removal of odontogenic cysts and benign tumors.