Managing Airway Complexities in Gorlin–Goltz Syndrome: A Comprehensive Approach to a Rare Genetic Disorder

Abstract Gorlin–Goltz syndrome (GGS) is a rare disorder with diverse clinical manifestations affecting multiple organ systems. Airway management during general anaesthesia in GGS patients poses unique challenges due to facial asymmetry from cystic enlargement of the mandible and maxilla. In addition, laryngeal papillomas may further complicate mask ventilation and intubation. In our series, the first GGS case presented with an anticipated difficult airway and was successfully managed using a video laryngoscope. The second case involved a paediatric patient, while the third had limited interincisor distance, and she was intubated awake via fibreoptic bronchoscopy. All three cases were effectively managed using tailored airway strategies.

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Publication Details

Journal
Airway
Published
2025-05-01
DOI
https://doi.org/10.4103/arwy.arwy_31_25
Primary Topic
Hedgehog Signaling Pathway Studies
Type
article
Field-Weighted Citation Impact
0.00
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article

Managing Airway Complexities in Gorlin–Goltz Syndrome: A Comprehensive Approach to a Rare Genetic Disorder

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Airway
Hedgehog Signaling Pathway Studies
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Managing Airway Complexities in Gorlin–Goltz Syndrome: A Comprehensive Approach to a Rare Genetic Disorder

Abid Majeed Rather, Subhasree Das, Sangeeta Sahoo, Alok Kumar Sethi, Sanjana Pandey
article en

Abstract

Abstract Gorlin–Goltz syndrome (GGS) is a rare disorder with diverse clinical manifestations affecting multiple organ systems. Airway management during general anaesthesia in GGS patients poses unique challenges due to facial asymmetry from cystic enlargement of the mandible and maxilla. In addition, laryngeal papillomas may further complicate mask ventilation and intubation. In our series, the first GGS case presented with an anticipated difficult airway and was successfully managed using a video laryngoscope. The second case involved a paediatric patient, while the third had limited interincisor distance, and she was intubated awake via fibreoptic bronchoscopy. All three cases were effectively managed using tailored airway strategies.

AirwayVol. 8(2)
All India Institute of Medical Sciences Bhubaneswar (IN)
Openalex Percentile: Top 80%
Hedgehog Signaling Pathway Studies
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