Congenital Airway Abnormalities in Patients Requiring Hospitalization

ORIGINAL ARTICLE Congenital Airway Abnormalities in Patients Requiring Hospitalization

Ken W. Altman, MD, PhD; Ralph F. Wetmore, MD; Roger R. Marsh, PhD

Objective: To determine the cause of congenital air- logical findings and/or clinical evaluation. Sixty-five pa- way abnormalities in pediatric patients requiring hospi- tients (37%) had multiple sites of airway abnormalities; talization for their respiratory status. laryngeal abnormalities were noted almost 3 times as often as tracheal abnormalities (161 vs 62, respectively). Design and Setting: Case series in a tertiary care Of the laryngeal abnormalities, laryngomalacia was the center. most common, followed by glottic web, subglottic stenosis, vocal-cord paralysis, and subglottic heman- Patients: A 5-year retrospective chart review was con- gioma. Tracheomalacia was the most common tracheal ducted at our institution. A total of 174 patients were iden- abnormality, followed by external compression and tra- tified who required hospitalization for their respiratory cheal stenosis. Thirty-three patients (19%) required tra- status as a result of a congenital airway abnormality. cheotomy for management of recurrent respiratory de- compensation. Results: Of the 174 patients, 114 (65.5%) were male and 60 (34.5%) were female. Eighty patients (47%) pre- Conclusions: While congenital airway abnormalities are sented within the first 3 months of life. Forty-six pa- usually self-limited, those patients requiring hospitaliza- tients (26%) were born prematurely, and 49 patients tion represent a group with a more severe respiratory sta- (28%) were diagnosed as having gastroesophageal re- tus who have a greater chance of requiring trache- flux. The majority of patients (139 [80%]) had multiple otomy. The recognizable percentage of patients with presenting symptoms or signs. Stridor was the most com- gastroesophageal reflux and prematurity accounts for co- mon (129 [74%]), followed by accessory respiratory ef- morbid factors in the need for hospitalization for respi- fort, cyanosis, apnea, and failure to thrive. Diagnosis was ratory issues related to congenital airway abnormalities. made at the time of surgical evaluation in 91% of the patients, with the remaining diagnoses made using radio- Arch Otolaryngol Head Neck Surg. 1999;125:525-528

ONGENITAL AIRWAY abnor- RESULTS malities in the pediatric population are usually A total of 174 patients were identified who mild in severity, often required hospitalization for their respira- presenting with stridor tory status as a result of a congenital air- (noisyC breathing from obstructed airway abnormality. One hundred fourteen flow) or stertor (snorting or gurgling patients (65.5%) were male, and 60 pa- from obstruction in the nose or phar- tients (34.5%), female. Seventy-three pa- ynx). However, exacerbation of symp- tients (42%) required endotracheal intu- toms with compromise of respiratory sta- bation prior to the time of diagnosis of a tus may become evident in patients with congenital airway abnormality, with a comorbid medical conditions, multiple mean duration of 22 days and a median sites of airway abnormalities (synchro- duration of 2 weeks. The age distribution nous lesions), and during upper respira- at the time of diagnosis is shown in tory tract infections. The purpose of this Figure 1, with a mean age of 7.6 months study is to determine the cause of con- and a median age of 3.2 months. Of the From the Department of Otolaryngology, Children’s genital airway abnormalities in pediatric 174 patients, 82 (47%) were diagnosed at Hospital of Philadelphia and patients requiring hospitalization for 3 months or younger. the University of Pennsylvania their respiratory status, with attention to Prematurity (Յ36 weeks’ estimated School of Medicine, prematurity, comorbid medical condi- gestational age at birth) was identified in Philadelphia. tions, and outcome. 46 patients (26%). Major cardiovascular,

ARCH OTOLARYNGOL HEAD NECK SURG/ VOL 125, MAY 1999 525

15 A retrospective chart review was conducted in all patients treated at the Children’s Hospital of Philadel- phia, Philadelphia, Pa, with congenital airway abnor- Patients, % 10 malities requiring hospitalization between July 1, 1991, and June 30, 1996. Patients were selected if their di- 5 agnoses were categorized from the following Interna- tional Classification of Diseases, Ninth Revision1 diagnostic codes: 748.2 (laryngeal web), 748.3 (congenital 0 1 2 3 4 5 6 7 8 9 10 11 12 >12 anomalies of the larynx, trachea, and bronchus), and Age, mo 478.30 to 478.34 (vocal-cord or vocal-fold paralysis). Patients with the following anomalies that were Figure 1. Age distribution of patients at the time of diagnosis. Note that 47% presented at 3 months or younger, and 67% presented at 6 months or presumed to be acquired were excluded: subglottic ste- younger. nosis, tracheomalacia or bronchomalacia as a sole finding in patients who had received ventilation through an endotracheal tube for longer than 6 days, and vocal-fold paralysis in newborn patients without Cardiovascular central nervous system findings. Medical records were reviewed for patient demographics, clinical Chromosomal presentation, comorbid medical conditions, diagnostic evaluation, treatment, and outcome. Patients whose medical records had incomplete documenta- Neurologic tion in these areas were also excluded.

Congenital

neurologic, chromosomal abnormalities, and/or congeni- None

tal syndromes were present in 34 patients (19.5%; 0 20 40 60 80 Figure 2). Five patients had multiple major abnormali- Patients, % ties. Cardiovascular abnormalities were present in 25 patients and included tetralogy of Fallot, abnormalities of Figure 2. Major comorbid medical abnormalities. the aortic arch, transposition of the great vessels, severe atrial or ventricular septal defect, and pulmonary artery and valvular deformity. Three patients had congenital Stridor

syndromes, and 7 had chromosomal abnormalities in- Accessory cluding Down syndrome. Neurologic abnormalities Respiration were present in 4 patients and included spina bifida, mi- crocephalus, communicating hydrocephalus, and cere- Cyanosis bral palsy. Seventy-two patients (41%) had 1 or more of Apnea the above malformations and/or were born prematurely. Gastroesophageal reflux was identified in 49 pa- Failure to tients (28%). Thrive

Stridor was the most common presenting symp- Other tom or sign, found in 128 patients (74%) as shown in Figure 3. This was followed by accessory respiratory 0 20 40 60 80 100 effort in 71 patients (41%), cyanosis in 45 (26%), apnea Patients, % in 43 (25%), failure to thrive in 39 (22%), and cough in Figure 3. Symptoms and diagnoses of patients at presentation. “Other” 30 (17%). One hundred forty patients (80%) had mul- includes cough, oxygen desaturation and/or bradycardia while being tiple presenting symptoms or signs. Other presenting com- monitored, tachypnea, poor pulmonary toilet, weak cry, upper respiratory tract infection, asthma or wheezing, and restless sleep. plaints included oxygen desaturation and/or bradycardia while being monitored, tachypnea, poor pulmonary toilet, weak cry, upper respiratory tract infection, asthma Sixty-five patients (37%) were found to have mul- or wheezing, and restless sleep. tiple sites of airway abnormalities. There were a total of A diagnosis was made during surgery in 158 pa- 257 diagnostic findings, with laryngeal abnormalities tients (91%). Direct laryngoscopy was performed in 111 noted almost 3 times as often as tracheal abnormalities (64%), flexible laryngobronchoscopy in 117 (67%), and (161 vs 62). Overall, 154 patients (86%) had laryngeal rigid bronchoscopy in 54 (31%). The remaining pa- abnormalities, 60 patients (34%) had tracheal abnor- tients were diagnosed clinically or via radiographic evalu- malities, 18 patients (10%) had bronchial abnormalities ation with airway fluoroscopy and/or barium swallow. (all bronchomalacia except 1 with bronchial stenosis),

ARCH OTOLARYNGOL HEAD NECK SURG/ VOL 125, MAY 1999 526

©1999 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/29/2021 and 17 patients (10%) had upper airway obstruction (glos- soptosis, pharyngeal collapse, micrognathia, and choa- Prevalence of Tracheotomy and Endotracheal nal atresia). Intubation Prior to Diagnosis* Laryngeal anomalies are listed below. No. (%) of Patients Abnormality No. (%) of Patients Laryngomalacia 119 (68.4) Abnormality Tracheotomy Endotracheal Intubation Glottic web 15 (8.6) Subglottic hemangioma 2 (100) 0 (0) Subglottic stenosis 12 (6.9) External compression 6 (60)† 4 (40) Vocal-fold paralysis 8 (4.6) Subglottic stenosis 7 (58)† 9 (75)‡ Glottic or subglottic cyst 3 (1.7) Glottic stenosis 1 (50) 0 (0) Subglottic hemangioma 2 (1.1) Glottic web 6 (40) 10 (67)‡ Laryngeal stenosis or atresia 2 (1.1) Vocal-fold paralysis 2 (25) 3 (38) Tracheal anomalies are listed below. Tracheomalacia 14 (33)† 21 (50) Glottic or subglottic cyst 1 (33) 1 (33) Abnormality No. (%) of Patients Bronchomalacia or stenosis 5 (28) 12 (67)‡ Tracheomalacia 42 (24.1) Upper airway obstruction 3 (18) 4 (24) External compression 10 (5.7) Laryngomalacia 15 (13) 47 (39) Tracheal stenosis 5 (2.9) Transesophageal fistula 0 (0) 3 (100) Tracheoesophageal fistula 3 (1.7) Tracheal web 0 (0) 1 (100) Tracheal web 1 (0.6) Tracheal stenosis 0 (0) 2 (40) Tracheal agenesis 1 (0.6) All 33 (19) 73 (42)

Of the laryngeal abnormalities, laryngomalacia (congeni- *Multiple airway abnormalities (synchronous lesions) were present in 19 tal laryngeal stridor) was the most common, followed by (58%) of the 33 patients requiring tracheotomy and 31 (42%) of the 73 glottic web, subglottic stenosis, and vocal-fold paraly- patients requiring endotracheal intubation. †Higher than sample mean ( PϽ.05). sis. Tracheomalacia was the most common tracheal ab- ‡Higher than sample mean ( PϽ.01). normality, while other anomalies included tracheal compression, tracheal stenosis, tracheoesophageal fistula, and tracheal agenesis. Tracheal compression was due to vas- known comorbidity; they underwent carbon dioxide la- cular ring (2 patients), anomalous innominate artery (2), ser aryepiglottoplasty (along with 1 other patient with and tetralogy of Fallot (1), cardiomegaly secondary to pul- laryngomalacia) at or after the time of tracheotomy. All monic vein stenosis (1), severe atrial septal defect (1), 3 patients who required laser aryepiglottoplasty under- cystic teratoma (1), supraglottic cystic hygroma (1), and went successful decannulation after tracheotomy. soft tissue hemangioma of the neck (1). However, 18 pa- There were 6 deaths (3.4%): cardiovascular col- tients with major cardiovascular abnormalities were not lapse in 3 patients (2 after cardiac surgery or tracheo- diagnosed as having tracheal compression as a surgical plasty and 1 while receiving extracorporal membrane oxy- finding. genation), and 1 each due to gastrointestinal hemorrhage, Eighty-one patients (47%) were followed up by the lymphoproliferative disease, and tracheal agenesis (the otolaryngology service or required additional hospital- patient was unable to maintain an open airway at birth). ization after the initial diagnosis, with a mean follow-up of 1.5 years and a median follow-up of 1 year. Thirty- COMMENT three patients (19%) required tracheotomy for severe, un- relenting respiratory symptoms and/or prolonged ven- Airway abnormalities of congenital origin represent more tilatory support following diagnosis. The prevalence of than 85% of those infants and children referred to the tracheotomy and endotracheal intubation prior to sur- otolaryngologist for evaluation of chronic stridor and res- gical diagnosis is listed in the Table. Patients with di- piratory symptoms.2,3 The male preponderance of al- agnoses of external compression, subglottic stenosis, or most 2:1, of which there is no obvious explanation, is tracheomalacia were significantly more likely to require not unique to our study.2,4,5 Stridor is the most common tracheotomy than the sample as a whole (PϽ.01: exact presenting sign of congenital airway abnormalities, with binomial probability for nϽ20; z scores for others). There exacerbations or severity producing accessory respira- was a higher-than-average likelihood of intubation for tory effort, cyanosis, and apnea.6 subglottic stenosis, glottic web, and bronchomalacia or While a diagnosis based on the clinical history is stenosis (PϽ.05). Thirteen patients underwent trache- sometimes sufficient for an isolated lesion, surgical di- otomy at the time of laryngobronchoscopy without prior agnosis with flexible or rigid laryngobronchoscopy is of- intubation. ten necessary for definitive evaluation. Synchronous le- Of the 15 patients with laryngomalacia requiring tra- sions (multiple sites of airway abnormalities) were noted cheotomy, 12 patients (80%) had major medical comor- in 64 patients (37% compared with 12% in another study3 bidity (including cardiovascular, congenital, neuro- of patients with stridor), underscoring the need for di- logic, chromosomal, or syndromal abnormalities, or rect visualization. Radiological evaluation of films of the bronchopulmonary dysplasia requiring prolonged intu- lateral side of the neck, airway fluoroscopy, and barium bation prior to tracheotomy). Six patients (40%) had mul- esophagography are useful, but the results are not as de- tiple airway abnormalities, 3 patients with 3 abnormali- finitive as those of laryngobronchoscopy.7 ties each. Six patients (40%) were premature at birth (Յ36 The prevalence of laryngomalacia in our study is con- weeks estimated gestational age). Only 2 patients had no sistent with other series of pediatric patients with chronic

ARCH OTOLARYNGOL HEAD NECK SURG/ VOL 125, MAY 1999 527

©1999 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/29/2021 stridor in the United States2,3 and internationally.4,5 In a centage of patients with cardiovascular or other major series of 191 patients with congenital stridor, Holinger2 malformations and prematurity accounts for comorbid found laryngeal abnormalities in 69%, tracheal abnor- factors in the need for intervention (hospitalization, in- malities in 18%, and bronchial abnormalities in 6%. La- tubation, and tracheotomy). The presence of synchro- ryngomalacia was the most common abnormality (41% nous lesions also portends a need for intervention. of all congenital lesions), followed by congenital subglottic stenosis in 14% and vocal-fold paralysis in 9%. This CONCLUSIONS distribution is consistent with the results of this study. The origin of laryngomalacia is thought to include This demographic study of patients with congenital air- anatomical, histological, and neurologic features, al- way abnormalities requiring hospitalization showed a 2:1 though the current theory recognizes mild localized hy- male preponderance of these lesions. Comorbid factors potonia of the musculature supporting supraglottic struc- were present in 41% of the patients and included pre- tures.8 Laryngomalacia tends to be self-limited with a maturity, cardiovascular malformations, and neuro- natural history toward resolution by 18 months as the logic and congenital or chromosomal abnormalities. Syn- child grows and develops neuromuscular maturity.3 In chronous lesions were found in 37% of the patients, a series9 of 985 patients with laryngomalacia, 12% re- contributing to the need for tracheotomy in 19% of the quired operative intervention (such as aryepiglotto- patients and endotracheal intubation in 42% prior to di- plasty) and only 2 patients required tracheotomy. agnosis. While laryngomalacia is the most common con- Tracheomalacia was the second most common congenital airway abnormality, it is an uncommon reason genital airway abnormality in the present study. The for tracheotomy in the absence of comorbid factors. pathophysiologic characteristic of congenital tracheo- bronchomalacia is an abnormal shape or consistency of Accepted for publication December 7, 1998. the tracheobronchial rings leading to an inherent weak- Presented as a poster at the Annual Meeting of the ness.6,10 Both tracheomalacia and external compression American Society of Pediatric Otolaryngology, Scottsdale, of the trachea are sometimes associated with anomalous Ariz, May 14-16, 1997. vascular structures compressing the trachea, resulting in Corresponding author: Ralph F. Wetmore, MD, respiratory symptoms in the majority of patients.11 In a Department of Otolaryngology, Children’s Hospital of study10 of 50 infants and children with tracheobroncho- Philadelphia, 34th Street and Civic Center Boulevard, malacia, 48% were primary (congenital) and 52% were Philadelphia, PA 19104. secondary (acquired). Overall, 62% required tracheotomy, but the rate was almost twice as high for pa- REFERENCES tients who were born prematurely. Laryngeal webs, atresia, and stenosis result from fail- 1. World Health Organization. International Classification of Diseases, Ninth Revi- ure of the epithelial tissue over the laryngotracheal groove sion (ICD-9). Geneva, Switzerland: World Health Organization; 1977. to resorb by 10 weeks of gestation.12 While patients with 2. Holinger LD. Etiology of stridor in the neonate, infant and child. Ann Otol Rhinol these abnormalities are relatively uncommon in our study, Laryngol. 1980;89(5, pt 1):397-400. 3. Friedman EM, Vastola AP, McGill TJI, Healy GB. Chronic pediatric stridor: etiol- patients with glottic webs and congenital subglottic ste- ogy and outcome. Laryngoscope. 1990;100:277-280. nosis have about a 43% likelihood of requiring trache- 4. Elango S, Htun YN. Aetiology of stridor in Malaysian children. Ann Trop Paedi- otomy.13 Similarly, as many as 73% of patients with bi- atr. 1994;14:329-332. lateral vocal-fold paralysis are also likely to require 5. Rupa V, Raman R. Aetiological profile of paediatric laryngeal stridor in an Indian tracheotomy.14 hospital. Ann Trop Paediatr. 1991;11:137-141. 6. Richardson MA, Cotton RT. Anatomic abnormalities of the pediatric airway. We report a 19% tracheotomy rate in our series, com- Pediatr Clin North Am. 1984;31:821-834. 2,3 parable to the 10% to 14% rate previously reported. Of 7. Tunkel DE, Zalzal GH. Stridor in infants and children: ambulatory evaluation and the patients requiring tracheotomy in our series, 58% had operative diagnosis. Clin Pediatr (Phila). 1992;31:38-55. synchronous lesions. Based on the discussion herein, it 8. Belmont JR, Grundfast K. Congenital laryngeal stridor (laryngomalacia): etio- logic factors and associated disorders. Ann Otol Rhinol Laryngol. 1984;93: is not surprising to find that 40% to 58% of our patients 430-437. with congenital subglottic stenosis and glottic web re- 9. Roger G, Denoyelle F, Triglia JM, Garabedian EN. Severe laryngomalacia: surgi- quired tracheotomy. Also, the diagnosis of tracheoma- cal indications and results in 115 patients. Laryngoscope. 1995;105:1111- lacia carried a 33% prevalence of tracheotomy in our 1117. series, consistent with previously reported findings.10,15 10. Jacobs IN, Wetmore RF, Tom LWC, Handler SD, Potsic WP. Tracheobroncho- malacia in children. Arch Otolaryngol Head Neck Surg. 1994;120:154-158. In our study, the risk of requiring tracheotomy or intu- 11. Chun K, Colombani PM, Dudgeon DL, Haller JA Jr. Diagnosis and management bation (Table) is only statistically significant for those of congenital vascular rings: a 22-year experience. Ann Thorac Surg. 1992;53: abnormalities with explanatory footnotes. This risk re- 597-603. flects our population of hospitalized patients with stated 12. Carpenter BLM, Merten DF. Radiographic manifestations of congenital anomalies affecting the airway. Radiol Clin North Am. 1991;29:219-240. comorbidity and synchronous lesions and may not ap- 13. Cohen SR. Congenital glottic webs in children: a retrospective review of 51 ply to strictly outpatient populations. patients. Ann Otol Rhinol Laryngol. 1985;121(suppl):2-16. Gastroesophageal reflux was noted in 28% of our 14. Bower CM, Choi SS, Cotton RT. Arytenoidectomy in children. Ann Otol Rhinol patients, similar to 23% to 53% found in studies of pa- Laryngol. 1994;103:271-278. tients with laryngomalacia.8,16 It is possible that gastro- 15. Altman KW, Wetmore RF, Marsh RR. Congenital airway abnormalities requiring tracheotomy: a profile of 56 patients and their diagnoses over a 9 year period. esophageal reflux is a factor that aggravates respiratory Int J Pediatr Otorhinolaryngol. 1997:41:199-206. symptoms; however, it also may represent a concomi- 16. Holinger LD, Konior RJ. Surgical management of severe laryngomalacia. Laryn- tant, yet unrelated finding. The relatively large per- goscope. 1989;99:136-142.

ARCH OTOLARYNGOL HEAD NECK SURG/ VOL 125, MAY 1999 528