Gorlin-Goltz Syndrome: A Case Series.
Ameera Salahudheen1, Naqoosh Haidry1, Ejaz A Mokhtar1
1Oral and Maxillofacial Surgery, All India Institute of Medical Sciences, Patna, Patna, IND.
Cureus
|October 23, 2023
Summary
Gorlin-Goltz syndrome (GGS) is rare in India. Early diagnosis is vital due to potential malignant links. This case series highlights key features and conservative management strategies for GGS patients.
Area of Science:
- Oral and Maxillofacial Surgery
- Dermatology
- Genetics
Background:
- Gorlin-Goltz syndrome (GGS) is a rare genetic disorder.
- It is characterized by multiple basal cell carcinomas, keratocystic odontogenic tumors, and skeletal abnormalities.
- GGS diagnosis in the Indian population is infrequently reported.
Observation:
- This case series details four GGS patients treated between 2019 and 2023.
- The average age was 20 years, with common symptoms including jaw swelling and tooth displacement.
- Prevalent features included odontogenic keratocysts (100%), palmar pits (100%), plantar pits (50%), falx cerebri calcification (50%), and rib anomalies (50%).
Findings:
- Odontogenic keratocysts were universally present, with multiple cysts in 75% of cases.
- Conservative management, including marsupialization for larger cysts and enucleation for smaller ones, was employed.
- Recurrence or new cyst formation was observed in 50% of the patients.
Implications:
- Early and systemic evaluation is crucial for diagnosing GGS, especially in patients with histopathology-diagnosed odontogenic keratocysts.
- Conservative treatment approaches are recommended to avoid complications.
- This study emphasizes the importance of recognizing GGS features for timely intervention and management.
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