Neurologic Evaluation in Children With Laryngeal Cleft | Congenital Defects | JAMA Otolaryngology–Head & Neck Surgery | JAMA Network
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Original Investigation
July 2017

Neurologic Evaluation in Children With Laryngeal Cleft

Author Affiliations
  • 1Department of Otolaryngology and Communication Enhancement, Boston Children’s Hospital, Boston, Massachusetts
  • 2Department of Otolaryngology, Harvard Medical School, Boston, Massachusetts
  • 3Department of Neurology, Boston Children’s Hospital, Boston, Massachusetts
JAMA Otolaryngol Head Neck Surg. 2017;143(7):651-655. doi:10.1001/jamaoto.2016.4735
Key Points

Questions  When and why should patients with laryngeal cleft undergo neurologic evaluation and what imaging should be performed?

Findings  In this medical record review of 242 patients with laryngeal cleft, 36% were referred to a neurologist; of these, 38% had examination findings indicative of neuromuscular dysfunction or dyscoordination. Abnormalities were found in 32 of 50 patients who underwent brain imaging.

Meaning  Referral to a neurologist and magnetic resonance imaging of the brain are recommended when (1) the degree of aspiration noted on a modified barium swallow study cannot be explained by the anatomical defect, (2) significant oral and/or oropharyngeal dysphagia is present, (3) a patient fails to improve, or (4) any neurologic symptoms or signs are present.

Abstract

Importance  Referral to a neurologist and imaging play important roles in the management of laryngeal cleft. Swallowing involves a complex series of neuromuscular interactions, and aspiration can result from anatomical causes (eg, laryngeal cleft), neuromuscular disorders, or some combination thereof. To date, no protocols or guidelines exist to identify which patients with laryngeal cleft should undergo neuroimaging studies and/or consultation with a neurologist.

Objective  To establish guidelines for neurologic evaluation and imaging techniques to identify or rule out neuromuscular dysfunction in children with laryngeal cleft.

Design  Retrospective review of the medical records of 242 patients who were diagnosed with laryngeal cleft at a tertiary children’s hospital between March 1, 1998, and July 6, 2015. Based on this review, an algorithm to guide management of laryngeal cleft is proposed.

Main Outcomes and Measures  Data extracted from patient medical records included the type of laryngeal cleft, details of neurologic referral, results of neuroimaging studies, and objective swallow study outcomes.

Results  Of the 242 patients, 142 were male and 100 were female. Mean age at the time of data analysis was 8.7 years (range, 10 months to 25 years), and there were 164 type I clefts, 64 type II, 13 type III, and 1 type IV. In all, 86 patients (35.5%) were referred to a neurologist; among these, 33 (38.4%) had examination findings indicative of neuromuscular dysfunction or dyscoordination (eg, hypotonia, spasticity, or weakness). Abnormal findings were identified in 32 of 50 patients (64.0%) who underwent brain imaging. Neurosurgical intervention was necessary in 3 patients diagnosed with Chiari malformation and in 1 patient with an intraventricular tumor detected on neuroimaging.

Conclusions and Relevance  A substantial proportion of patients with laryngeal cleft have coexistent neuromuscular dysfunction as a likely contributing factor to dysphagia and aspiration. Collaboration with a neurologist and appropriate neuroimaging may provide diagnostic and prognostic information in this subset of patients. At times, imaging will identify critical congenital malformations that require surgical treatment.

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