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Supraglottic cyst in adult patient with Shabbir syndrome

A 29-year-old male patient presented with a history of voice change and feeling of a lump in throat for the last 20 years. He was tracheostomised at the age of 10 years. Endolaryngeal examination showed a mucosal bulge in supraglottic region arising predominantly from right side. He had multiple healed scar marks on neck, arms and elbows, and had dystrophic nail changes. MRI of the neck showed a supraglottic cyst covering glottis. The patient was diagnosed as a case of laryngo-onycho-cutaneous syndrome which is also known as Shabbir syndrome. He underwent supraglottic cyst excision via transoral ultrasonic surgery. Glottic scarring along with subglottic stenosis was found intraoperatively, which was released using carbon dioxide laser and finally Montgomery (MT) tube was inserted. The patient got his tracheostomy tube removed for the first time in 20 years. He tolerated corking of MT tube. He was able to breathe through nose and phonate using false cords.