Congenital Bilateral Vocal Fold Paralysis Research Articles

Bilateral Vocal Fold Paralysis in a Patient With Congenital MYOD1 Myopathy.

Congenital bilateral vocal fold paralysis (BVFP) is a rare but significant cause of morbidity in pediatric otolaryngology. The differential diagnosis is expansive, with common etiologies including birth trauma, brainstem neoplasms, and neurologic disorders. There are few known genetic causes of the condition. This report details the first known case of BVFP secondary to a genetic deficiency in MYOD1, a master transcriptional regulator of skeletal muscle cell specification. Genetics consultation and testing may be a useful adjunct in the workup of congenital BVFP and may help guide prognostication, additional workup, counseling, and clinical decision-making.

Bilateral Selective Laryngeal Reinnervation for Bilateral Vocal Fold Paralysis in Children

Bilateral vocal fold paralysis (BVFP) in pediatric patients is a challenging entity with multiple causes. Traditional approaches to managing BVFP include tracheostomy, arytenoidectomy, suture lateralization, cordotomy, and posterior cricoid enlargement. These interventions are used to create a stable airway but risk compromising voice quality. To assess the use of bilateral selective laryngeal reinnervation (SLR) surgery to manage BVFP and restore dynamic function to the larynx in pediatric patients. In this case series performed at 2 tertiary care academic institutions, 8 pediatric patients underwent bilateral SLR to treat BVFP (5 patients with iatrogenic BVFP and 3 with congenital BVFP) from November 2004 to August 2018 with follow-up for at least 1.5 years. Bilateral selective laryngeal reinnervation surgery. Flexible laryngoscopy findings, subjective and objective measures of voice quality, subjective swallowing function, and decannulation in patients who were previously dependent on a tracheostomy tube. Participants included 6 boys and 2 girls with a median age of 9.3 (range, 2.2 to 18.0) years at the time of surgery. All 8 patients were decannulated; 6 patients had preoperative tracheostomies and 2 had perioperative tracheostomies. Voice quality, as measured using the GRBAS (grade, roughness, breathiness, asthenia, strain) scale, improved in 6 of 8 patients after reinnervation, and swallowing was not impaired in any patients. In 2 patients, GRBAS scale scores remained the same before and after surgery. Inspiratory vocal fold abduction was observed on both sides in 5 patients and on 1 side in 2 patients, with no active abduction observed in 1 patient. The follow-up period was more than 5 years in 7 of 8 patients and at least 1.5 years in all patients. Bilateral SLR appears to be a promising treatment option for children with BVFP; it is currently the only option, to our knowledge, with the potential to restore abductor and adductor vocal fold movement. In patients with complete paralysis, this procedure may provide a strategy for airway management and restoration of the dynamic function of the larynx. It could be considered as a first-line technique before endolaryngeal or airway framework procedures, which carry a risk of compromising voice quality.

CNS Imaging is Essential in Acquired Bilateral Vocal Fold Dysfunction in Children

Dear Sir,
 
 I greatly enjoyed reading the paper by David and Lim on “Congenital Bilateral Vocal Fold Paralysis”1 and complement the authors on their excellent surgical outcome. They also very appropriately underscored the primacy of careful clinical assessment over costly investigations, particularly in an environment of scarce resources.
 
 We have previously reported on the MRI findings in 23 children with bilateral vocal fold dysfunction (BVFD) as an isolated abnormality that was present at birth. We found MRI abnormalities in 35%, but these were all non-specific.2 This study indicated that MRI was of research and clinical value, but did not identify any major CNS structural abnormalities in this patient group.
 
 However, the case reported by David and Lim involved the delayed onset of symptoms at 2 years of age that may have increased over the next 3 years. This acquired and possibly progressive disease process differs from that of congenital BVFD. The likelihood of Arnold-Chiari malformation or another significant structural CNS abnormality being present would appear to be higher when symptoms of BVFD are acquired later in life compared to when they are present at birth.
 
 Anecdotally, I have been involved with a case of acquired BVFD presenting at a similar age where the underlying cause was found to be a posterior fossa tumour. The 3 year history of symptoms in the child in the case report would very likely preclude this diagnosis, but certainly another CNS cause needs to be considered to explain the development of delayed-onset brainstem dysfunction, and this would require MRI.
 
 
 Robert Berkowitz
 Department of Otolaryngology
 Royal Children’s Hospital
 Melbourne, Australia

Congenital Bilateral Vocal Fold Paralysis in a Two-Year-Old Girl

Congenital Bilateral Vocal Fold Paralysis in a Two-Year-Old Girl

A novel endoscopically placed stent to relieve glottic obstruction from bilateral vocal fold paralysis

Objective Congenital bilateral vocal fold paralysis (BVP) is a rare but serious condition often requiring a tracheostomy to temporize the airway. In cases of idiopathic BVP, studies suggest waiting twelve months prior to laryngeal surgery because of a high rate of spontaneous recovery. Therefore a less invasive and reversible intervention would be optimal. A prospective study in a piglet model was undertaken to evaluate the efficacy of a novel spring-loaded stenting device designed to maintain laryngeal patency in an in vivo animal model of BVP. Methods Eight Yorkshire piglets had BVP induced by surgical division of the recurrent laryngeal nerves. Stents were endoscopically deployed between the arytenoid vocal processes. Animals were recovered and monitored for stridor, dietary intake, and weight gain. Animals were sacrificed after five days. Airway resistance using a calibrated manometer was measured at four time-points: baseline, BVP induction, stent insertion, and pre-sacrifice. Results Six of eight animals survived greater than five days with an average weight gain of 1.9 kg ( p = 0.003). Relative inspiratory resistance increased from baseline after inducing BVP (1.00 vs. 1.468, p = 0.0315) and decreased to baseline levels with stent insertion (1.468 vs. 1.092, p = 0.0238). Expiratory resistance was not significantly influenced by stage of measurement ( p = 0.236). Of the two animals not surviving the protocol, one had an unrelated anesthesia complication and the other a malpositioned stent. Conclusion The novel stent was successful in relieving the inspiratory resistance associated with BVP, without compromising swallowing and daily function. This may hold promise in temporarily securing the pediatric airway in the setting of BVP.

Congenital Bilateral Vocal Fold Paralysis and Charcot-Marie-Tooth Disease

We present the case of a patient with Charcot-Marie-Tooth disease (CMT) type 1 with congenital bilateral vocal fold paralysis in order to emphasize the treatment options and long-term outcome. The case is reviewed with regard to presentation, differential diagnosis, and treatment. We also reviewed the literature to determine the frequency of congenital and childhood presentations of bilateral vocal fold paralysis associated with CMT, most specifically CMT type 1. We found only 14 children reported to have bilateral vocal fold paralysis associated with CMT, and only 1 of these cases was associated with CMT type 1. None of these patients had congenital vocal fold paralysis. Because of the degenerative nature of the disease, our patient underwent endoscopic cordotomy to avoid tracheotomy. We conclude that CMT should be included in the differential diagnosis in evaluating neonates with bilateral vocal fold paralysis. If CMT is definitively diagnosed, it could alter the course of treatment.

Natural history of tracheostomy-dependent idiopathic congenital bilateral vocal fold paralysis

To identify the age at which spontaneous improvement in vocal fold function occurs that will allow decannulation to be performed in tracheostomy-dependent children with isolated idiopathic congenital bilateral vocal fold paralysis (BVFP). Retrospective chart review in tertiary pediatric center. Three children were identified who underwent tracheostomy between 13 and 45 days old. Two patients have been decannulated at age 5 years 11 months and 7 years 1 month, but both have persistent symptoms of upper airway obstruction. One patient remains tracheostomy-dependent at 4 years of age with only minimal vocal cord abduction. Spontaneous improvement in vocal fold function sufficient to allow decannulation appears to occur during the second quinquennium of life. Conservative treatment could be considered as an alternative to surgery in severe idiopathic congenital BVFP.

A Unique Treatment Option for a Case of Congenital Bilateral Vocal Fold Paralysis

A Unique Treatment Option for a Case of Congenital Bilateral Vocal Fold Paralysis