Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature. Bilateral Vocal Cord Paralysis in Children Bilateral abductor vocal cord paralysis is a distressing condition, often life-threatening, and not infrequently a direct cause of asphyxial death. Early detection of pediatric laryngeal paralysis requires a high index of suspicion and is important to prevent catastrophes during periods of acute respiratory embarrassment. The etiology of vocal cord paralysis may be divided into two broad categories: congenital and acquired. Vocal cord paralysis accounts for 10% of all congenital laryngeal lesions and ranks second only to laryngomalacia in occurrence. Unfortunately, the majority of these infants have multiple congenital defects. Hydrocephalus, meningomyelocele, and meningocele have been associated with the Arnold-Chiari malformation in this illness; the medulla and cerebellum protrude through the foramen magnum into the spinal canal. The subsequent caudal displacement of the brain stem by increasing intracranial pressure stretches the vagi and causes recurrent nerve and vocal cord paralysis. Other developmental anomalies of the central nervous system such as encephalocele, cerebral agenesis, and nucleus ambiguus dysgenesis have been complicated by vocal cord paralysis. Congenital anomalies of the cardiovascular system are frequently associated with vocal cord paralysis. Ventricular and atrial septal defects have been described in these patients. Abnormalities of the major vessels have also been found. Laryngeal paralysis has been reported with subglottic stenosis, laryngomalacia, laryngeal clefts, and laryngoceles. The occurrence of congenital bilateral vocal cord paralysis in families has been documented. Acquired bilateral vocal cord paralysis in the pediatric age group may be divided into three broad categories: traumatic, infectious, and miscellaneous. Newborns with vocal cord paralysis and no apparent cause are thought to have had unavoidable stretching of the laryngeal nerves during delivery. The anatomy of the recurrent laryngeal nerves makes them vulnerable to injury as they course around the subclavian artery on the right and aorta on the left. During delivery the vessels provide counter traction against the nerves, thus stretching them, with subsequent paralysis which may be temporary or permanent. Vocal cord paralysis secondary to endotracheal intubation is a controversial subject. It has been shown that the recurrent laryngeal nerve can be susceptible to pressure injury between the endotracheal tube and the superior portion of the thyroid cartilage. Surgical correction of many of the thoracic and cardiovascular anomalies may be complicated by injury to the recurrent laryngeal nerves. It may occur following repair of tracheoesophageal fistulas, Arnold-Chiari malformations, or myelomeningoceles. Antibiotics and vaccinations have greatly reduced the infectious types of vocal cord paralysis. Whooping cough encephalitis, polio, diphtheria, rabies, tetanus, syphilis and botulism have been sources of laryngeal paralysis that are fortunately rarely seen in present day pediatrics. Tumors of the CNS may present with bilateral vocal cord paralysis. Chondroma and spongioblastoma multiforme have been reported. Vocal cord paralysis may affect any of the normal laryngeal functions of voice production, respiration, or deglutition. The cry of the patient with bilateral vocal cord paralysis is often quite normal. This is because in bilateral vocal cord paralysis both cords may remain weakly adducted, in which position they vibrate during expiration and phonation, passively but equally. Patients with this problem have a limited airway and persistent, soft, inspiratory stridor which may be barely audible or may become quite loud. Any additional edema of the cords results in extreme dyspnea, for which control of the airway may become necessary. Infants and children may remain stridorous but undiagnosed until an upper respiratory infection leads to obstruction. Patients with bilateral vocal cord paralysis may have cyanosis, apnea, or intercostal retractions. Drooling, recurrent choking and aspiration suggest either a developmental abnormality or a neuromuscular disorder of the laryngopharynx. A neonate, infant, or child with an airway problem, feeding difficulty, weak or absent cry, deglutition abnormality or known anomaly of the esophagus, heart or central nervous system must be suspected of having a vocal cord problem. The method of choice to evaluate cord mobility is laryngoscopy. A significant advancement in the diagnosis of vocal cord paralysis in children has been the advent of the flexible fiberoptic nasopharyngolaryngoscope. The infant can be examined at the bedside without requiring transport to the operating room or general anaesthesia. The advantage of direct rigid laryngoscopy is that it provides the opportunity to palpate the vocal cords and thus to differentiate between vocal cord paralysis and conditions that mimic the problem, including interarytenoid web, posterior glottic stenosis, or cricoarytenoid fixation. Direct rigid laryngoscopy is best performed without anesthesia. If there is no apparent pathology to explain the stridor or partial upper airway obstruction, direct laryngoscopy under general anesthesia should be done to rule out the presence of a subglottic lesion that is only visible through the cords when they are relaxed. When other congenital anomalies of the respiratory or digestive systems are suspected, bronchoscopy and esophagoscopy should be part of the management protocol. Workup of the child with a bilateral vocal cord paralysis should be systematic. Particular attention should be paid to the birth history with complications of labor and delivery noted. Plain chest x-rays can help rule out intrathoracic abnormalities and barium swallow can be diagnostic in congenital vascular disorders and pharyngoesophageal dysfunction. Head CT or MRI is recommended because of the high percentage of neurological abnormalities in these patients. The goal of management of bilateral vocal cord paralysis is to obtain an adequate airway. If airway compromise is severe, endotracheal intubation or tracheotomy may be necessary. However, if a neurosurgical procedure is planned to relieve hydrocephalus and secondarily improve the function of the vagus nerve, intubation is indicated with a waiting period prior to tracheotomy to see if function will return. If tracheotomy is performed because of inability to extubate, it may be desirable to wait six months to one year before planning more definitive therapy because of this possibility. For the child that does not experience a return of function, tracheal decannulation may be a major treatment problem. Endoscopic surgery with posterior cordectomy or cord lateralization with sutures may be performed. Cordectomy and arytenoidectomy with the CO2 laser have been used, but disadvantages include postoperative scarring and inability to correctly quantify the amount of tissue removed. Arytenoidectomy has a failure rate of 20% to 40% The nerve-muscle pedicle technique described by Dr. Harvey Tucker involves dissecting the ansa hypoglossi and retrieving the small branch that innervates the omohyoid muscle along with a portion of the muscle itself. This is sutured to the posterior cricoarytenoid muscle. Although Tucker's success rates approach 90% for decannulation, other authors have had failures approaching 50% with this method. Unfortunately the ideal corrective treatment for the child with bilateral vocal cord paralysis is not well established. Case Presentation A term infant was born to a multigravida black female by cesarean delivery. He had a lumbar myelomeningocele that was closed on day one of life. Intubation was required for that procedure, but the patient was extubated immediately afterward without respiratory distress. He had a progressively increasing head fronto-occipital circumference, and thus a head CT scan was ordered. He was noted to have hydrocephalus and an Arnold-Chiari malformation. Because of lumbosacral wound breakdown, ventriculoperitoneal shunt was deferred until flap closure of the wound was performed. He underwent ventricular tap to relieve hydrocephalus on one occasion. On day 44 of life, the patient developed inspiratory stridor when agitated. It had been over 48 hours since he had been extubated following the lumbosacral procedure. His oxygen saturation remained high, he had no cardiac arrhythmias, and had no cyanotic episodes. The Otolaryngology Head and Neck Service was consulted and on flexible fiberoptic laryngoscopy he was found to have a bilateral abductor true vocal cord paralysis with a small glottic chink. Ventriculoperitoneal shunt was recommended to relieve the hydrocephalus and presumed vagus nerve traction. This was performed with no significant improvement in the patient's symptoms. Tracheotomy was then recommended. This was deferred by the pediatric and pulmonary services because the patient never displayed any signs of hypoxia or central apnea. At three months of age, he was discharged home without a tracheotomy. The inspiratory stridor he experienced upon agitation spontaneously resolved over the following few months. He is now one year old and without respiratory symptoms. Bibliography Charney EB, Rorke LB. Management of Chiari II complications in infants with myelomeningocele. J Pediatr 1987;111:364371. Cohen SR. Pseudolaryngeal paralysis: a postintubation complications. Ann Otol Rhinol Laryngol 1981;90:483488. Cummings CW. Bilateral vocal cord paralysis/ankylosis. In: Cummings CW, Fredrickson JW, editors. Otolaryngology Head and Neck Surgery. St. Louis: Mosby, 1986:21812189. Dedo DD. Pediatric vocal cord paralysis. Laryngoscope 1979;89:13781384. Dedo HH, Sooy CD. Endoscopic laser repair of posterior glottic, subglottic and tracheal stenosis by division or microtrapdoor flap. Laryngoscope 1984;94:445450. Gentile RD, Miller RH, Woodson GE. Vocal cord paralysis in children 1 year of age and younger. Ann Otol Rhinol Laryngol 1986;95:622625. Grundfast KM, Harley E. Vocal cord paralysis. Pediatr Otolaryngol 1989;22:569. Grundfast KM, Milmoe G. Congenital hereditary bilateral abductor vocal cord paralysis. Ann Otol Rhinol Laryngol 1982;91:564566. Holinger LD, Holinger PC, Holinger PH. Etiology of bilateral abductor vocal cord paralysis: a review of 389 cases. Ann Otol Rhinol Laryngol 1976;85:428436. Lim RY. Laser arytenoidectomy. Arch Otolaryngol Head Neck Surg 1985;111:262. Magnussen CR, Patanella HP. Herpes simplex virus and recurrent laryngeal nerve paralysis. Report of a case and review of the literature. Arch Intern Med 1979;139:14231424. Oren J, Helly DH, Todres D, Shannon DC. Respiratory complications in patients with myelodysplasia and ArnoldChiari malformation. AJDC 1986;140:221224. Pfenninger J. Bilateral vocal cord paralysis after severe blunt head injury a cause of failed extubation. Crit Care Med 1987;15:701702. Robertson JR, Birck HG. Laryngeal problems following infant esophageal surgery. Laryngoscope 1976;86:965970. Rosin DF, Handler SD, Potsic WP, Wetmore RF, Tom LW. Vocal cord paralysis in children. Laryngoscope 1990;100:11741179. Ruff ME, Oakes WJ, Fisher SR, Spock A. Sleep apnea and vocal cord paralysis secondary to type I Chiari malformation. Pediatrics 1987;80:231234. Sessions DG, Ogura JH, Heeneman H. Surgical management of bilateral vocal cord paralysis. Laryngoscope 1976;86:559566. Sivula A, Ronni Sivula H. Observations on 334 patients operated on for primary hyperparathyroidism. Ann Chir Gynaecol 1985;74:6673. Thompson JW, Rosenthal P, Camilon FS, Jr. Vocal cord paralysis and superior laryngeal nerve dysfunction in Reye's syndrome. Arch Otolaryngol Head Neck Surg 1990;116:4648. Tucker HM. Congenital bilateral recurrent nerve paralysis and ptosis: a new syndrome? Laryngoscope 1983;93:14051407. Tucker HM. Human laryngeal reinnervation: longterm experience with the nervemuscle pedicle technique. Laryngoscope 1978;88:598604. Tucker HM. Longterm results of nervemuscle pedicle reinnervation for laryngeal paralysis. Ann Otol Rhinol Laryngol 1989;98:674676. Woodman D, Pennington CL. Bilateral abductor paralysis: 30 years experience with arytenoidectomy. Ann Otol Rhinol Laryngol 1976;85:437439. Woodson GE. The timing of surgical intervention in vocal cord paralysis. Otolaryngol Head Neck Surg 1981;89:264267. Yamada H, Tanaka Y, Nakamura S. Laryngeal stridor associated with the Chiari II malformation. Childs Nerv Syst 1985;1:312318. Zager EL, Ojemann RG, Poletti CE. Acute presentations of syringomyelia. Report of three cases. J Neurosurg 1990;72:133138. 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