Congenital Airway Abnormalities in Patients Requiring Hospitalization

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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 of- ten 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 ste- nosis, 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 pa- tients, 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- C(noisy breathing from obstructed air- way 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 (#36 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 ©1999 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/29/2021 25 PATIENTS, MATERIALS, AND METHODS 20 15 A retrospective chart review was conducted in all pa- tients 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 diag- nostic 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 paraly- sis). 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 find- ing 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, diagnos- tic 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 pa- tients 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 prema- turely. 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 bradycar- dia 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
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