Gorlin and goltz syndrome: a case report with surgical review.

Int J Clin Pediatr Dent

Lecturer, Department of Conservative Dentistry, VSPM Dental College, Nagpur, Maharashtra, India.

Published: May 2013

AI Article Synopsis

Article Abstract

Gorlin 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.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4086581PMC
http://dx.doi.org/10.5005/jp-journals-10005-1199DOI Listing

Publication Analysis

Top Keywords

gorlin goltz
12
goltz syndrome
12
syndrome case
8
case report
8
report surgical
8
surgical review
8
syndrome
5
review gorlin
4
syndrome complex
4
complex syndrome
4

Similar Publications

Article Synopsis
  • - The study examines the rare skeletal anomaly known as bifid rib, which appears in less than 1.5% of the population and faces challenges in identification due to postmortem damage and similarity to other conditions.
  • - It presents five cases of rib bifurcation from four individuals across different populations in the Kujawy region of Poland, analyzed through morphological and paleoradiological methods.
  • - The research also critiques existing evidence linking bifurcated ribs to naevoid basal cell carcinoma syndrome and proposes a new way to classify this anomaly in osteological studies.
View Article and Find Full Text PDF

Nevoid basal cell carcinoma syndrome is a rare autosomal dominant disorder characterized by a diverse clinical presentation, which includes developmental abnormalities and tumorigenesis that can impact multiple organ systems. Basal cell carcinoma is the most common and characteristic clinical presentation in patients with NBCCS. There are three identified causative genes for this disease, the PTCH1 gene located at 9q22-31, the PTCH2 gene at 1p32-34, and the SUFU gene at 10q24.

View Article and Find Full Text PDF
Article Synopsis
  • Gorlin-Goltz syndrome (GGS) is an autosomal dominant genetic condition linked to an increased risk of multiple basal cell carcinomas (BCCs) and associated with mutations in the PTCH1 or SUFU genes, though diagnosis can occur without these markers.
  • The main symptom of GGS is the development of multiple BCCs, which usually grow slowly and stay localized, but can sometimes be more aggressive in affected individuals.
  • A recent case demonstrates that radiation therapy (RT), typically avoided due to the heightened risk of BCCs in GGS patients, can be effective and safe in treating an inoperable BCC that had metastasized, suggesting the need for personalized treatment approaches.
View Article and Find Full Text PDF
Article Synopsis
  • Focal dermal hypoplasia (FDH), also known as Goltz syndrome, is a rare genetic condition affecting primarily women, linked to mutations in the PORCN gene, that impacts the skin, bones, and eyes.
  • A study at Aarhus University Hospital examined four confirmed cases of FDH, revealing typical symptoms like skin atrophy, limb abnormalities, and eye issues, as well as identifying new genetic variants.
  • The prevalence of FDH in Western Denmark was estimated at 1.6 cases per million, highlighting the rarity and complexity of the disorder, which necessitates comprehensive medical collaboration for effective diagnosis and care.
View Article and Find Full Text PDF

Nevoid basal cell carcinoma (Gorlin-Goltz) syndrome: an incidental finding.

BMJ Case Rep

August 2024

Oral Medicine and Radiology, JSS Academy of Higher Education and Research, Mysore, Karnataka, India.

Article Synopsis
  • Gorlin-Goltz syndrome, also referred to as basal cell nevus syndrome, is a rare genetic disorder that features skeletal issues, specific cysts in the mouth, and skin growths known as basal cell nevi.
  • Diagnosis involves assessing both major and minor clinical signs as well as imaging tests, with oral medicine and radiology specialists playing a key role due to the condition's oral manifestations.
  • A case study of a male in his late 20s reveals that using advanced digital imaging techniques is essential, as traditional X-rays could miss important details about his multiple cystic lesions, ultimately leading to the diagnosis of Gorlin-Goltz syndrome.
View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!