Metatropic dysplasia—a skeletal dysplasia with challenging airway and other anesthetic concerns

Theroux, Mary C.; López, M. L. Vidal; Olszewsky, Patricia J.; DiCindio, Sabina; Arai, Lynda; Ditro, Colleen; Bober, Michael B.; Olla, Olubukola Opeyemi; Uejima, Tetsu; West, David W.; Mackenzie, William G.

doi:10.1111/pan.13101

Cited by 7 publications

(1 citation statement)

References 16 publications

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“…Skeletal dysplasias (SD) including SMD commonly have anatomical features such as narrow nasal passages, large tongue, midfacial hypoplasia, micrognathia, and tracheomalacia posing airway challenges. 4–6 Tracheobronchial pathology needing airway reconstructive surgery has yet to be described in SMD. SD patients with advanced Morquio A syndrome (MPS IVA) are known to have progressive tracheal narrowing requiring reconstructive surgery 7 but this has not been described in any other forms of SD.…”

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confidence: 99%

Severe Multilevel Tracheal Stenosis with Significant Twisting in a Patient with Spondylometaphyseal Dysplasia: A Case Report

Stefko,

Oommen,

Jones

et al. 2024

A&A Practice

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Spondylometaphyseal dysplasia (SMD) is a rare genetic disorder affecting skeletal growth and development presenting anesthesiologists with many perioperative challenges. We present a case of a patient found to have multilevel tracheal stenosis due to twisting and folding of his trachea. This was discovered on imaging during a research review of SMD cases at our institution. Structural and functional abnormalities of the trachea have not been reported in SMD. This is the first description of a patient with SMD with severe multilevel tracheal disease requiring tracheal reconstructive surgery.

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confidence: 99%