Case Series
Volume 3 Issue 11 - 2020
Gorlin-Goltz Syndrome: Report of 4 Cases
Amine Oualime1*, Ouassime Kerdoud1 and Faical Slimani1,2
1Faculty of Medicine and Pharmacy, Hassan 2 University of Casablanca, Morocco
2Department of Stomatology, Oral and Maxillofacial Surgery, Hospital of August 20th, University Hospital-CHU Ibn Rochd, Casablanca, Morocco
*Corresponding Author: Amine Oualime, Faculty of Medicine and Pharmacy, Hassan 2 University of Casablanca, Morocco.
Received: August 08, 2020; Published: October 31, 2020




Abstract

Introduction: Gorlin-Goltz syndrome, also known as basal cell nevomatosis (CBN), is a rare inherited disorder belonging to the family of neurocristopathies or diseases caused by abnormalities of the neural ridges.

The mode of transmission is autosomal dominant with full penetrance and variable expressivity.

The diagnosis of disease is clinico-radiological, which can be confirmed by genetic study. The risk of malignant transformation of the skin lesions requires ad vitum monitoring of patients.

We report the case of 4 patients, suffering from this syndrome, followed up in the stomatology and maxillofacial surgery service in Casablanca, including a familial form in two cases: a mother and her son and 2 isolated cases, followed and treated in our service of maxillofacial surgery of the August 20 hospital in Casablanca.

A clinical, biological and radiological assessment was made followed by surgical treatment for the 3 patients, an anatomo-pathological examination and the establishment of a strict monitoring plan.

Case Report: We report the case of a 60-year-old woman and her 39-year-old son as well as a 55-year-old man, and another 36-year-old. 3 of the patients presented with multiple maxillo-mandibular cysts.

Observation 1: A 55-year-old man, with no pathological history followed in our department since November 2018, Conservative surgical treatment was performed in the patient consisting of enucleation of the cysts. Anatomical pathological examination concluded with odontogenic keratocysts.

Observation 2: A 60-year-old woman with a history of: High blood pressure under hypertensive treatment, excision of multiple maxillo-mandibular cysts in 2007, in whom clinical examination shows: a budding lesion with raised edges at the level of the lower right eyelid with extension to the free edge of the lower eyelid, a lesion in the upper left eyelid, frontal bump, diffuse café-au-lait spots on the trunk and upper limbs, surgical excision under general anesthesia with reconstruction of the lower eyelid was performed, the postoperative follow-up were favorable.

Observation 3: A 39-year-old man (the son), having as an antecedent: 1st degree consanguinity with the above-mentioned patient, on clinical examination presents: a large frontal bump, macrocephaly, diffuse pigmentary lesions at the level of the face and hypertelorism.

Observation 4: A 36-year-old man, operated at the age of seventen for a mandibular cyst (no documents), on clinical examination presented: two swelling in the mandibular level; angular right and at the level of the left horizontal branch, an enlarged frontal bump, and two pigmentary lesions at the level of the two lower eyelids

The diagnosis of Gorlin Goltz syndrome was made in all four patients based on various clinical and radiological criteria.

The monitoring plan is made up of regular clinical and radiological monitoring.

Discussion: Gorlin Goltz syndrome is a rare, autosomal dominant genetic disorder with variable penetrance. Its manifestations are dominated by basal cell carcinomas and multiple maxillo-mandibular odontogenic keratocysts. The course is marked by a risk of aggressive extension or recurrence of keratocysts after surgical treatment. The prognosis is conditioned by the risk of malignant transformation of the skin lesions requiring regular monitoring and systematic genetic counseling.

Keywords: Gorlin-Goltz Syndrome; Keratocystic Odontogenic; Basocellular Nævomatosis

Citation: Amine Oualime., et al. “Gorlin-Goltz Syndrome: Report of 4 Cases”.EC Clinical and Medical Case Reports 3.11 (2020): 76-84.

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