Sleep apnea in hurler syndrome: Looking beyond the upper airway

Sumera Shaikh Solaiman, Daniel Rifkin, Harish Rao

Research output: Contribution to journalArticle

Abstract

This case involves a 13-month-old male with Hurler syndrome. Due to oxygen desaturations during sleep, this patient was referred for polysomnography, which revealed severe mixed sleep apnea (apnea-hypopnea index [AHI] 72 events/h). Because sleep apnea in patients with Hurler syndrome is frequently attributed to upper airway obstruction, he was referred to otolaryngology. Prior to his evaluation by otolaryngology, he underwent ventriculoperitoneal (VP) shunt placement, which had been scheduled due to hydrocephalus on brain magnetic resonance imaging (MRI). After VP shunt placement, his oxygen desaturations during sleep resolved. Repeat polysomnogram revealed mild sleep apnea (AHI 1.9). The etiology of his sleep apnea was likely his hydrocephalus. This is the first report of a patient with Hurler syndrome with sleep apnea which markedly improved with ventriculostomy and VP shunt placement. This highlights the importance of considering neurological etiologies for sleep apnea in Hurler's patients, despite their predisposition for airway obstruction.

Original languageEnglish (US)
Pages (from-to)1423-1424
Number of pages2
JournalJournal of Clinical Sleep Medicine
Volume12
Issue number10
DOIs
StatePublished - Jan 1 2016

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Mucopolysaccharidosis I
Sleep Apnea Syndromes
Ventriculoperitoneal Shunt
Otolaryngology
Apnea
Airway Obstruction
Hydrocephalus
Sleep
Ventriculostomy
Oxygen
Polysomnography
Magnetic Resonance Imaging
Brain

All Science Journal Classification (ASJC) codes

  • Pulmonary and Respiratory Medicine
  • Neurology
  • Clinical Neurology

Cite this

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Sleep apnea in hurler syndrome : Looking beyond the upper airway. / Solaiman, Sumera Shaikh; Rifkin, Daniel; Rao, Harish.

In: Journal of Clinical Sleep Medicine, Vol. 12, No. 10, 01.01.2016, p. 1423-1424.

Research output: Contribution to journalArticle

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