Swallowing Dysfunction in Children
Care Coordination is Key to Treating Swallowing Dysfunction, Laryngeal Cleft
Maria Koenigs, MD, is a fellowship-trained pediatric otolaryngologist (ear, nose, and throat specialist) at Hasbro Children’s Hospital.
Swallowing dysfunction is common in children, affecting as many as one in 100, with increased prevalence in at-risk populations. Despite the high prevalence of dysphagia, its diagnosis and management are often challenging.
Swallowing is one of the most complex physiologic actions in the body. To swallow requires intricate coordination of more than 30 muscles and nerves. This can make it difficult for a single practitioner to discover the exact etiology of dysphagia. At Hasbro Children’s Hospital, there is a close collaboration between the otolaryngology, pulmonology, gastroenterology, and speech-language pathology departments to help diagnose and treat a child’s swallowing dysfunction.
An increasingly recognized cause of dysphagia and aspiration in children is a laryngeal cleft. A laryngeal cleft is a rare malformation arising from incomplete fusion of the posterior larynx (interarytenoid muscle, posterior cricoid, and/or posterior tracheoesophageal septum) which affects 1:10,000-20,000 children.1 This malformation can lead to laryngeal penetration and aspiration during swallow.
The laryngeal cleft is a spectrum disorder, with a significant difference in presentation based on cleft severity. Benjamin and Inglis devised a grading schema for diagnosis of laryngeal cleft.2 A type I cleft extends just below the level of the vocal cords; type II extends into the cricoid cartilage; type III goes past the cricoid into the cervical trachea/esophagus; and type IV extends down into the thoracic trachea/esophagus.
Children with type III and type IV laryngeal cleft often present early in life with frank aspiration and poor pulmonary status, generally requiring intubation in the first days. The presentation for children with type I and type II laryngeal cleft is, unsurprisingly, milder, with most presenting with respiratory symptoms (cough, aspiration, cyanosis, history of hospitalization for pulmonary infection).
Because of the common nature of many of these symptoms, it is thought that laryngeal cleft may be underdiagnosed. Type I or II laryngeal cleft may be missed in children who are otherwise healthy, because of the non-specific nature of their symptoms.
Additionally, many children with laryngeal cleft may have comorbidities that present with swallowing dysfunction, including neurologic and cardiac malformation, which further delay diagnosis. Laryngeal cleft has been described in children with tracheoesophageal fistula, Pallister Hall syndrome, VACTERL syndrome, and CHARGE syndrome. In these populations, early investigation is critical.
In the pediatric otolaryngology department at Hasbro Children’s, our approach to the workup for a suspected laryngeal cleft often includes flexible laryngoscopy and an instrumental swallow evaluation. Although a laryngeal cleft, in general, cannot be easily identified on bedside examination, this examination is important to rule out anatomic causes of aspiration (such as vocal cord paralysis).
Because of the concern for aspiration in this population, a modified barium swallow (MBS) test is the preferred instrumental evaluation, though for breast-feeding infants a functional endoscopic evaluation of swallow can be useful. The gold standard for diagnosis of a laryngeal cleft is operative palpation of the posterior larynx during direct laryngoscopy with bronchoscopy.
Once a laryngeal cleft has been identified, Hasbro Children’s specialists take an individualized approach to treatment, based on a child’s symptoms and the severity of the cleft. Most type III and all type IV laryngeal clefts are managed through open surgical repair. For type II laryngeal cleft and select type III laryngeal clefts, the preferred approach is endoscopic repair using both a CO2 laser and suture repair.
For type I laryngeal clefts, the management strategy is complex, and we tailor it to the needs of the child and family. We often begin with non-surgical interventions such as dietary modification with thickened feeds and swallowing rehabilitation as performed by our speech-language pathologists.
As a percentage of children have improved swallowing as they age, temporary thickening may be sufficient. If there is no improvement with thickened feeds, we discuss with parents the options regarding repair, including temporary injection into the posterior larynx versus endoscopic surgical suture repair (similar to what is performed for a type II laryngeal cleft).
Children with laryngeal clefts are followed closely with repeat swallow evaluations to make sure they have symptom improvement and resolution. Because most children with a laryngeal cleft present with pulmonary complaints, we work closely with the pulmonology department to ensure that we optimize their status.
The team of specialists at Hasbro Children’s Hospital is committed to caring for children who have complex swallowing dysfunction with close interdisciplinary coordination. To further strengthen these collaborations and to help better care for our patients, a formal aerodigestive program including pediatric otolaryngology, pediatric pulmonology, pediatric gastroenterology, and speech-language pathology is being developed to provide optimal treatment for these patients in the near future.
1. Johnston DR, Watters K, Ferrari LR, et al. Laryngeal cleft: evaluation and management. Int J Pediatr Otorhinolaryngol 2014; 78:905-11.
2. Benjamin B, Inglis A. Minor congenital laryngeal clefts: diagnosis and classification. Ann Otol Rhinol Laryngol 1989; 98:417-20.