Abstract
ABSTRACTGorlin and Goltz syndrome are a very complex syndrome and a multisystemic process that is characterized by the presence of multiple pigmented basocellular carcinomas, keratocysts in the jaws, palmar and/or plantar pits and calcification of the falx cerebri. Along with these major features a great number minor features have also been described which involves numerous skeletical, dermatology related, neurological, ophthalmological and reproductive anomalies. It exhibits high penetrance and variable expressivity. Presented here is the case of Gorlin-Goltz in a 12 years old male patient which was diagnosed through its oral and maxillofacial manifestations. Treatment of odontogenic keratocyst was done by enucleation without primary suturing. Iodoform dressing was kept to enhance the healing and to reduce the recurrence of the lesion.It is important to provide the early diagnosis for detection of clinical and radiological manifestations in young patients and for provision of advice concerning preventive treatment like protection of the skin from the sunlight and genetic sensitivity testing so that possible complications associated with this syndrome can be prevented.How to cite this article: Bahadure RN, Jain ES, Badole GP. Gorlin and Goltz Syndrome: A Case Report with Surgical Review. Int J Clin Pediatr Dent 2013;6(2):104-108.
Highlights
Jaw cyst-basal cell nevus-bifid rib syndrome known as a Basal cell nevus syndrome or Gorlin and Goltz syndrome
This syndrome was first described by Jarish and White in 1894, noticed the presence of multiple basocellular carcinoma
In 1960, Robert J Gorlin and Robert W Goltz[1] established a classical triad that characterizes the diagnosis of this syndrome involving multiple basocellular epitheliomas, keratocysts in the jaws and bifid ribs. This triad was later modified by Rayner in 1977 who established that for giving the diagnosis at least cysts had to appear in combination with calcification of the falx cerebri or palmar and plantar pits
Summary
Jaw cyst-basal cell nevus-bifid rib syndrome known as a Basal cell nevus syndrome or Gorlin and Goltz syndrome This syndrome was first described by Jarish and White in 1894, noticed the presence of multiple basocellular carcinoma. In 1960, Robert J Gorlin and Robert W Goltz[1] established a classical triad that characterizes the diagnosis of this syndrome involving multiple basocellular epitheliomas, keratocysts in the jaws and bifid ribs This triad was later modified by Rayner in 1977 who established that for giving the diagnosis at least cysts had to appear in combination with calcification of the falx cerebri or palmar and plantar pits. The growth of swelling was slow in nature and noticed when it was approximately 1 cm in size When patient reported it was approximately 6 × 7 cm in diameter, extending from the canine to distal to the first permanent molar on right side of cheek (Fig. 1). Skeletal Anomalies[11,13]
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