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Gorlin-Goltz Syndrome – A Concatenation of Six Case Reports

RATIONALE: Gorlin-Goltz syndrome (GGS) has a wide range of expressivity, with a majority of cases being first diagnosed from the oral findings. Early intervention can reduce its severity. PATIENT CONCERNS: The primary complaints of all the patients were pain and swelling. Clinical examination with r...

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Detalles Bibliográficos
Autores principales: Marimallappa, T. R., Rao, Keerthana, Kumar, KR Ashok, Kumar, R. Mahesh, Pal, Supriyo, Jha, Manisha
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer - Medknow 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10499285/
https://www.ncbi.nlm.nih.gov/pubmed/37711515
http://dx.doi.org/10.4103/ams.ams_153_22
Descripción
Sumario:RATIONALE: Gorlin-Goltz syndrome (GGS) has a wide range of expressivity, with a majority of cases being first diagnosed from the oral findings. Early intervention can reduce its severity. PATIENT CONCERNS: The primary complaints of all the patients were pain and swelling. Clinical examination with radiological and histopathological evaluation confirmed the diagnosis. DIAGNOSIS: This series presents the six cases of GGS treated over a time frame of five years (2018–2022). TREATMENT: The treatments range from enucleation, chemical cauterisation and peripheral ostectomy to aggressive modalities such as resection and reconstruction. OUTCOMES: This series comprises of six patients with ages ranging from 12 to 42 years, four of which were female and two were male presenting minimal expression to highly aggressive forms and its unpredictable frequent manifestation. TAKE-AWAY LESSONS: This emphasises the significance of long-term periodic follow-up and genetic screening for early detection, thereby reducing the intensity and aggressiveness of the disease.