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Related Experiment Videos

Gorlin's syndrome with associated odontogenic cysts.

J D Lovin1, C L Talarico, S L Wegert

  • 1Department of Radiology, Womack Army Hospital, Fort Bragg, North Carolina.

Pediatric Radiology
|January 1, 1991
PubMed
Summary

Basal cell nevus syndrome, also known as Gorlin-Goltz syndrome, commonly presents with multiple cysts in the jaw. Magnetic resonance imaging (MRI) aided in evaluating these lesions in an affected family, revealing a potential new associated middle ear anomaly.

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Area of Science:

  • Medical Genetics
  • Radiology
  • Otolaryngology

Background:

  • Basal cell nevus syndrome (Gorlin-Goltz syndrome) is an autosomal dominant disorder.
  • Key features include multiple jaw cysts (keratocystic odontomas) and basal cell carcinomas.
  • Other associated anomalies can affect various organ systems.

Observation:

  • This study presents cases from a family affected by Gorlin-Goltz syndrome.
  • Magnetic resonance imaging (MRI) was utilized for the evaluation of cystic lesions in the jaws.
  • A previously unreported middle ear anomaly was identified in affected individuals.

Findings:

  • MRI proved valuable in assessing the extent and characteristics of maxillary and mandibular cysts.
  • The identified middle ear anomaly may represent a novel association with Gorlin-Goltz syndrome.

Related Experiment Videos

  • This highlights the importance of comprehensive imaging in syndromic conditions.

    • Implications:

    • The findings suggest MRI is a key imaging modality for evaluating Gorlin-Goltz syndrome.
    • The potential new association warrants further investigation and could expand diagnostic criteria.
    • This research contributes to a better understanding of the phenotypic variability of BCNS.