Neurologic Variant Laryngomalacia Associated with Chiari Malformation and Cervicomedullary Compression: Case Reports

• Published in: Annals of otology, rhinology & laryngology Vol. 120; no. 2; pp. 99 - 103
• Main Authors: Petersson, Rajanya S., Wetjen, Nicholas M., Thompson, Dana M.
• Format: Journal Article
• Language: English
• Published: Los Angeles, CA SAGE Publications 01.02.2011; Annals Publishing Compagny; SAGE PUBLICATIONS, INC
• Subjects: Arnold-Chiari Malformation - complications; Biological and medical sciences; Decompression, Surgical; DiGeorge Syndrome - complications; Female; Humans; Infant; Infant, Newborn; Laryngomalacia - complications; Laryngomalacia - surgery; Male; Malformations of the nervous system; Medical sciences; Neurology; Otorhinolaryngology. Stomatology; Upper respiratory tract, upper alimentary tract, paranasal sinuses, salivary glands: diseases, semeiology; laryngomalacia; stridor; brain stem compression; Chiari malformation; Human; Stridor; Nervous system diseases; Compression; Brain stem; ENT; Congenital disease; Cerebral disorder; Laryngomalacia; Case study; Arnold-Chiari malformation; Malformation; Central nervous system disease; ENT disease; Larynx disease
• ISSN: 0003-4894; 1943-572X
• DOI: 10.1177/000348941112000205

Two infants presented with intermittent stridor and evidence of laryngomalacia on flexible laryngoscopy. The first was a 10-month-old girl who had undergone 3 supraglottoplasty surgeries at an outside institution, without long-term resolution of symptoms. She was found during our evaluation to have a Chiari malformation. Laryngomalacia symptoms resolved after suboccipital decompression and C1 laminectomy, and the patient remained symptom-free at 6-month follow-up. The second infant was a 24-day-old boy with velocardiofacial syndrome who was found to have posterior cervicomedullary junction compression at the level of C1. He underwent C1 laminectomy for decompression of the brain stem, which resulted in immediate resolution of symptoms, and he remained symptom-free at 12-month follow-up. Neurologic abnormalities have been reported in up to 50% of infants with laryngomalacia. As such, brain stem dysfunction should be considered among the causes of laryngomalacia during evaluation, especially in patients with failure of supraglottoplasty. Both of these infants had resolution of symptoms after their neurosurgical procedures.