Bilateral Congenital Vocal Cord Paralysis: A 16-year Institutional Review

Otolaryngology–Head and Neck Surgery (2005) 133, 241-245

Bilateral Congenital Vocal Cord Paralysis: A 16-year Institutional Review R. Christopher Miyamoto, MD,* Sanjay R. Parikh, MD, Walid Gellad, MD,† and Greg R. Licameli, MD, Boston, Massachusetts and Bronx, New York OBJECTIVE: To review the management and outcome of bilateral congenital true vocal cord paralysis in 22 patients treated over a 16-year period and to review the role of tracheostomy in these patients. DESIGN: Retrospective chart review. SETTING: Pediatric tertiary hospital. PATIENTS: Twenty-two pediatric patients diagnosed with bilateral congenital true vocal cord paralysis. INTERVENTIONS: Flexible or rigid diagnostic evaluation, tracheostomy, and vocal cord lateralization procedures. MAIN OUTCOMES MEASURES: Vocal cord recovery and decannulation. RESULTS: With a mean follow up of 50 months, 15 of 22 patients (68%) with bilateral vocal cord paralysis required tracheostomy for airway securement. Of the 15 tracheotomized patients, 10 were successfully decannulated (8 had spontaneous recovery, whereas 2 required lateralization procedures). Eleven of these patients with tracheostomy had comorbid factors, including neurologic abnormalities (midbrain/brainstem dysgenesis, ArnoldChiari malformation, global hypotonia, and developmental delay). Of the 7 patients not requiring tracheostomy, 6 recovered vocal cord function (86%). CONCLUSION: In our series of 22 patients with bilateral vocal cord paralysis, 14 had spontaneous recovery of function. Patients managed with tracheostomy were noted to have a high incidence of comorbid factors. In this series, recovery rates were found to be higher in nontracheostomized patients than in tracheostomized patients. Patients can be carefully selected for observation versus From the Department of Otolaryngology and Communication Disorders, Children’s Hospital Boston and the Department of Otology and Laryngology, Harvard Medical School (Drs Miyamoto, Gellad, and Licameli); and the Department of Otolaryngology–Head and Neck Surgery, Children’s Hospital at Montefiore, Albert Einstein College of Medicine (Dr Parikh). *Dr Miyamoto is now affiliated with the Departments of Otolaryngology–Head and Neck Surgery and Department of Pediatrics, Indiana University School of Medicine.

tracheostomy at the time of diagnosis based on underlying medical conditions. © 2005 American Academy of Otolaryngology–Head and Neck Surgery Foundation, Inc. All rights reserved.

V

ocal cord paralysis in the neonate is a well-known cause of stridor.1 The majority of the reports in the literature address both unilateral and bilateral paralysis.2-7 Bilateral vocal cord paralysis is an uncommon condition. Murty et al8 estimated an incidence of 0.75 cases per million births per year. Traditional management has included tracheostomy to secure the airway before spontaneous recovery of the vocal cords. Tucker9 stated that “tracheotomy is a necessary measure in the management of virtually all children with bilaterally immobile vocal folds.” Gentile et al5 reviewed vocal cord paralyses at their institution from 1962-1985. They found 12 bilateral paralyses, 11 of which required tracheostomy. Seven of these patients recovered vocal cord function between 5 and 18 months. Over the past 20 years, management of this condition has evolved with the advent of flexible laryngoscopy. Given the ease of repeat evaluation of the larynx with flexible endoscopy, careful observation without tracheostomy is now possible in children with an adequate airway.8 Many different strategies regarding treatment options, timing of interventions, and prognosis for recovery are † Dr Gellad is now affiliated with the Department of Medicine, Brigham and Women’s Hospital, Harvard Medical School. Presented at the American Society of Pediatric Otolaryngology Annual Meeting, Boca Raton, FL, May 12-14, 2002. Reprint requests: R. Christopher Miyamoto, MD, Department of Otolaryngology–Head and Neck Surgery, Riley Hospital for Children, Suite 0860, Indiana University Medical Center, 702 Barnhill Dr, Indianapolis, IN 46202-5230. E-mail address: [email protected].

0194-5998/$30.00 © 2005 American Academy of Otolaryngology–Head and Neck Surgery Foundation, Inc. All rights reserved. doi:10.1016/j.otohns.2005.02.019

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Table 1 Clinical characteristics of bilateral vocal cord paralysis Patient number

Age of symptom/ age at diagnosis

1 2 3

DOL 2/1 mo DOL 1/1 d DOL 1/1 d

4 5 6 7 8 9

DOL 1/2 DOL 1/2 DOL 1/2 DOL 1/1 1 mo DOL 1/1

d mo d d mo

10 11 12 13

DOL 1 DOL1/7 d DOL 1/13 d DOL 1/1 d

14 15 16

DOL 2/2 d DOL 1/1 d DOL 2

17 18

DOL 1/1 mo DOL 1/1 d

20

DOL1/1 d

21

DOL1/1 d

22

DOL 1/3 wk

Comorbidity Arthrogryposis Brainstem dysgenesis Small posterior fossa coloboma developmental delay Swallow dyscoordination/G-tube

Chiari II/oligohydramnios/IUGR Chiari II/hydrocephalus 25-wk preemie/BPD/intubated 32 d Subglottic stenosis 0 0 Forceps delivery

Mild laryngomalacia, GERD 35-wk preemie/BPD GERD s/p Nissen Hypotonia, developmental delay GERD/Nissen Goldenhar’s; severe Laryngomalacia Intraventicular bleed, 26-wk preemie, BPD polyhydramnios Intubated 6 wk, SGS Bilateral profound SNHL Intubated 3-wk postbirth Tetrology of Fallot

Tracheostomy Yes Yes No

No No No Yes Yes Yes Yes Yes

Age at tracheostomy 3 wk 1 wk

1 mo 2y 2 mo

Yes

2 mo DOL 11 No 24 mo

Yes Yes Yes

DOL 9 11 d 2 mo

No Yes

1 mo

Yes

DOL 20

Yes

3 mo

Yes

1 mo

VC, vocal cord; DOL, day of life; IUGR, intrauterine growth retardation; preemie, premature; BPD, bronchopulmonary dysplasia; GERD, gastroesophageal reflux disease; SGS, subglottic stenosis; LTR (ss ANT CCG), laryngotracheal reconstruction (single-staged anterior costal chondral graft).

found in the literature.2-7,10 In counseling families of children with bilateral vocal cord paralysis, the practitioner must consider the need for tracheostomy versus the morbidity of the procedure. We examined our institution’s experience with bilateral congenital vocal cord paralysis to provide prognostic information to the practitioner and parent.

MATERIAL AND METHODS A retrospective chart review was conducted of all patients treated at Children’s Hospital Boston from 1985 to 2001 who had the diagnosis of bilateral vocal cord paralysis. A search was completed of all patients treated during this period for the ICD-9 codes 478.33 and 478.34 (bilateral vocal cord paralysis, partial and complete, respectively). Forty-two patients were identified, 36 of whom had complete records available for

review at the time of this study. Fourteen patients were excluded from the study: 4 were acquired after cardiovascular surgery, 2 were acquired after tracheo-esophageal fistula repair at outside institutions, 2 were acquired after intubation, 3 were acquired from other conditions (encephalitis, mediastinal mass, hydrocephalus), and 3 were lost to follow-up. Twenty-two patients were identified with congenital bilateral vocal cord paralysis and included in this study. Initial diagnosis was made by either flexible fiberoptic laryngoscopy at the bedside or direct laryngoscopy in the operating room. All patients had a full airway evaluation in the operating room comprising direct laryngoscopy and rigid bronchoscopy. Arytenoid palpation was performed to differentiate arytenoid fixation from vocal cord paralysis. Etiologies of the paralyses were carefully reviewed and comorbidities noted. Initial interventions included tracheostomy or observation. Postoperative airway management and vocal cord recovery were studied.

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Bilateral Congenital Vocal Cord Paralysis . . .

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Table 1 (continued) Corrective procedures (age at procedure)

Decannulated (yes/no) No No

VC recovery No No Yes

Age at VC recovery

Follow-up (mo)

9 mo

21 7 14

Partial Yes Yes No Yes No

1y 8 mo 1 mo

Yes

Partial Yes Partial Yes

5.5 y 2 mo 6y 3y

65 5 82 99

Yes Yes Yes

Yes Yes Partial

2 mo 3 mo 6.5 y

2 70 76

No

Partial No

Epiglottoplasty (5 y)

No

No

180

LTR (ss ANT CCG) (88 mo); Partial arytenoidectomy (86 mo)

Yes

No

89

Right arytenoidectomy (5 y); Tucker proc (7 y)

No

No

141

No Yes No Yes Yes Right vestibulectomy, tracheostomy (32 mo)

RESULTS Twenty-two patients were identified with congenital bilateral vocal cord paralysis. Onset of symptoms (stridor, respiratory distress) was noted on day 1 in 18 patients, on day 2 of life in 3 patients, and at 2 months of age in 1 patient. Diagnosis was made between day 1 of life and 6 months (Table 1). Etiology of the paralysis was idiopathic in 19 patients, intraventricular hemorrhage in 1, and Arnold-Chiari II in 2. Arytenoid fixation was not found in any case. Fifteen patients were managed with tracheostomy (68%). Surgery was performed between day 9 of life and 3 months. Seven patients were managed without tracheostomy (32%). Of the tracheotomized patients, 8 were decannulated with return of vocal cord function (53%). Two of these patients required surgical correction before decannulation (13%). Surgical procedures to correct vocal cord paralysis were attempted in 3 patients (patients 13, 21, and 22; see Table

41 mo

12 8 1 15 112 84

12 36

1). These comprised arytenoidopexy, arytenoidectomy, cordectomy, laryngotracheal reconstruction, and laryngeal reinnervation (Tucker procedure). An additional procedure consisting of epiglottoplasty was done in another patient (patient 20) to correct severe laryngomalacia. The time to decannulation ranged 2 to 88 months, with an average of 31 months. Six of the 7 patients treated without tracheotomy recovered good vocal cord function, consisting of adequate abduction and adduction (80%) in a time frame of 1-82 months. One patient (patient 4) did not have true return of cord function with minimal motion and a 5-mm glottic airway yet had good respiratory, feeding, and voice function. Follow-up ranged from 1 to 180 months, with a mean of 50 months. As listed in Table 1, comorbidities of the 15 patients requiring tracheotomies were examined. Eleven patients were noted to have significant comorbidities, and 4 had none. Neurologic abnormalities were noted in 5 patients.

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These involved mild midbrain and brainstem dysgenesis in 1 patient, Arnold-Chiari II in 2, and global hypotonia and developmental delay in 1. Prematurity and bronchopulmonary dysplasia were seen in 3 patients. Significant laryngomalacia was noted in 2 patients. Gastroesophageal reflux disease requiring Nissen fundoplication occurred in 2 patients. Congenital heart disease (tetrology of Fallot) was seen in 1 patient. In this patient, the vocal cord paralysis was diagnosed before cardiovascular surgery. Subglottic stenosis was seen in 2 patients. Goldenhar’s syndrome occurred in 1 patient. One patient had arthrogryposis. Of the 7 patients who did not require tracheotomy, only 1 was noted to have any comorbidity. This patient had a small posterior fossa, developmental delay, and discoordinated swallow requiring a gastrostomy tube and colobomas. Surgical correction of vocal cord paralysis was undertaken in 3 patients. Options included arytenoidectomy, arytenoidopexy, cordectomy, cord lateralization, and reinnervation procedure (Tucker procedure). The age range at which procedures occurred was 22 months to 7 years. Patient 13 had a right arytenoidopexy at 22 months; right arytenoidectomy at 24 months; and tracheostomy, right vestibulectomy, and partial cordotomy at 32 months. She was successfully decannulated at 34 months. Patient 21 had partial arytenoidectomy performed at 86 months. Subglottic stenosis was then repaired at 88 months by using a singlestage laryngotracheal reconstruction with anterior costal chondral graft. Decannulation was performed 2 months postoperatively. Patient 22 underwent right arytenoidectomy at 5 years, which still required tracheostomy postoperatively. A bilateral reinnervation procedure (Tucker procedure) was done 2 years later at an outside institution. Decannulation was not successful.

CONCLUSIONS Several series over the past 20 years have reviewed pediatric vocal cord paralysis. Most reports include both unilateral and bilateral paralysis with multiple etiologies. Investigators list a variety of management options for bilateral paralysis and give differing ranges for recovery. Cohen et al2 had 100 cases; 62 were bilateral and 50 were congenital. De Gaudemar et al3 reviewed 113 cases; 52 were bilateral congenital paralysis. Daya et al4 reviewed 102 patients with 44 bilateral congenital cases. Gentile et al,5 Murty et al,8 Emery and Fearon,6 and Zbar and Smith,7 respectively, reviewed 12, 11, 9, and 4 cases of bilateral congenital paralyses. In those series, the rate of tracheostomy for bilateral vocal cord paralysis as initial management was 9%80%, and recovery of vocal cord function occurred in 29%71% over a period of 6 months to 11 years. Fifteen of our 22 patients (68%) required tracheotomy for initial management. Ten of these patients were decannulated (8 had spontaneous recovery [53%], and 2 required surgical lateraliza-

tion). Among the 7 patients not requiring tracheostomy, 6 (86%) recovered vocal cord function, whereas 1 patient had no functional recovery but maintained a patent airway with little vocal cord motion. Our experience with tracheostomy placement, decannulation rates, and recovery periods fits within these ranges. We chose to review comorbidities (Table 1) because bilateral vocal cord paralysis occurs rarely in isolation,1 and coexisting conditions affect outcomes. Our patients with prematurity and bronchopulmonary dysplasia (BPD) fared the worse among those with comorbidities. Patient 9 was not decannulated, patient 16 required 74 months for decannulation, and patient 21 required an arytenoidectomy and laryngotracheal reconstruction before decannulation at 88 months. Of the 7 patients not requiring tracheostomy, only 1 patient (patient 3) had any comorbidity. Traditionally, the management of congenital bilateral vocal cord paralysis dictated tracheostomy in most cases. The advent of flexible endoscopy has allowed conservative management using close medical follow-up without tracheostomy in selected cases. In general, our patients requiring tracheostomy had other significant medical problems such as pulmonary illness, whereas those observed were less medically complicated. The decision for observation was made based on repeated flexible endoscopic exam and consideration of the overall medical condition. This includes respiratory status, swallowing function, growth rate, and social issues such as compliance and proximity of the home to the hospital. Surgical correction for vocal cord paralysis is a viable and attractive treatment. The dilemma lies in deciding when to undertake surgery. Triglia et al10 performed external arytenoidopexy as early as 1 month with a mean of 20 months of age. De Gaudemar et al3 recommend waiting until 1 year of age before performing arytenoid surgery because many of their patients recovered by 6 months. Emery and Fearon6 found some patients recovered up to 9 years after tracheostomy. Daya et al4 had patients with recoveries as long as 11 years; thus, they advise waiting until adolescence so that the patient may decide between better voice quality with tracheostomy versus weaker voice with surgery. The task of the clinician lies in interpreting the literature and choosing which therapy is best for their patient. Our series shows that conservative management utilizing tracheostomy while awaiting return of vocal cord function was a reasonable option in 8 of the 15 patients. No significant morbidity or mortality was incurred with the tracheostomy in place. Of the 7 patients still with a tracheostomy in place, 4 of them are still very young (patients 1, 2, 7, and 19; follow-up, 7-36 months after tracheostomy), and we are allowing time for spontaneous recovery. The other patients not decannulated (patients 9, 20, and 22; 84-143 months after tracheostomy) have other clinical reasons for leaving the tracheostomy in place. Patient 20 has Goldenhar’s syndrome with upper airway obstruction precluding decannulation. Patient 22 has a history of congenital heart disease and did not regain vocal cord function or a

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Bilateral Congenital Vocal Cord Paralysis . . .

patent airway despite arytenoidectomy and bilateral Tucker procedures. Her course has been complicated by obstruction at the oropharyngeal level by redundant tissue. This allows her to plug the tracheostomy during the day, but the oropharyngeal collapse prevents decannulation. Patient 9 has a minimal glottic airway. Because of her good voice, the family prefers observation rather than surgical intervention. Corrective surgical procedures were done on 3 of our patients between 22 and 86 months after tracheostomy. Two of these patients were decannulated. As mentioned above, patient 9 was not decannulated. Historically, tracheostomy has been advocated for cases of bilateral true vocal cord paralysis until function recovered or surgery was performed to improve the glottic airway. The approach at our institution incorporates assessment of the overall medical status of the patient when deciding to pursue tracheostomy or careful observation. Thirty-two percent of our patients were managed without tracheostomy. This reflects individual treatment preferences by the attending surgeon as well as careful consideration of the child’s medical and social condition. Close monitoring with serial evaluation of respiratory pattern, swallowing function, and growth is required. Other concurrent illnesses may preclude this approach. The family must be reliable for follow-up and must live close to a medical facility should respiratory distress occur. Discussion with the family regarding the nature of spontaneous recovery with the time

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course of several months to years is undertaken when presenting options for surgical correction of vocal cord paralysis.

REFERENCES 1. de Jong AL, Kuppersmith RB, Sulek M, et al. Vocal cord paralysis in infants and children. Otolaryngol Clin North Am 2000;33:131– 49. 2. Cohen SR, Geller KA, Birns JW, et al. Laryngeal paralysis in children: a long-term retrospective study. Ann Otol Rhinol Laryngol 1982;91: 417–24. 3. de Gaudemar I, Roudaire M, Francois M, et al. Outcome of laryngeal paralysis in neonates: a long term retrospective study of 113 cases. Int J Pediatr Otorhinolaryngol 1996;34:101–10. 4. Daya H, Hosni A, Bejar-Solar I, et al. Pediatric vocal fold paralysis: a long-term retrospective study. Arch Otolaryngol Head Neck Surg 2000;126:21–5. 5. Gentile RD, Miller RH, Woodson GE. Vocal cord paralysis in children 1 year of age and younger. Ann Otol Rhinol Laryngol 1986;95:622–5. 6. Emery PJ, Fearon B. Vocal cord palsy in pediatric practice: a review of 71 cases. Int J Pediatr Otorhinolaryngol 1984;8:147–54. 7. Zbar RI, Smith RJ. Vocal fold paralysis in infants twelve months of age and younger. Otolaryngol Head Neck Surg 1996;114:18 –21. 8. Murty GE, Shinkwin C, Gibbin KP. Bilateral vocal fold paralysis in infants: tracheostomy or not? J Laryngol Otol 1994;108:329 –31. 9. Tucker HM. Vocal cord paralysis in small children: principles in management. Ann Otol Rhinol Laryngol 1986;95:618 –21. 10. Triglia JM, Belus JF, Nicollas R. Arytenoidopexy for bilateral vocal fold paralysis in young children. J Laryngol Otol 1996;110:1027–30.