Abstract

The lysosomal storage disease mucolipidosis presents formidable challenges for the pediatric anesthetist, not only due to progressive multi-system involvement but also because of intricate airway abnormalities. A two-year-old boy with mucolipidosis type II (GNPTAB mutation) presented with history of T.B under treatment and posted for flexible bronchoscopy with bronchoalveolar lavage (BAL). This report demonstrates the complexity of perioperative airway management in such children as guided by modern difficult airway algorithms, and the pivotal role of the I-gel® supraglottic airway device as both primary and rescue tool.

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Funding