INTRODUCTION REVIEW ARTICLE. Key words: flexible bronchoscopy, children, stridor

Transcription

1 REVIEW ARTICLE Flexible Bronchoscopy as a Valuable Tool in the Evaluation of Children with Stridor Wei-Ju Lee 1, Pei-Jung Wu 2, Chin-Ching Ku 2, Hui-Lin Chiu 2, Wen-Cheu Lee 2, Chih-Min Tsai 1, Chen-Kuang Niu 1, Kai-Sheng Hsieh 1, Hung-Jen Yu 1 1 Department of Pediatrics, 2 Respiratory Therapy, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Taiwan Abstract The aim is to determine clinical characteristics, and flexible bronchoscopy (FB) findings including associated airway abnormalities and other conditions (ex. gastroesophageal reflux disease, GERD) in children with stridor. Medical records of children who underwent FB for the evaluation of stridor between November 2008 and December 2014 were retrospectively reviewed. Demographic characteristics, symptoms, FB finding and presence of associated conditions were assessed. Seventy-two children were enrolled in the study. Among the 72 children, 51 (70.8%) patients were less than one year old. Among all patients, laryngomalacia was the most common etiology of stridor (n=43, 59.7%),which was isolated in 12 patients, GERD in 10 patients, and 26 patients had secondary airway lesions (SALs). Diagnoses other than laryngomalacia such as subglottic hemangioma, adenoid hypertrophy, and laryngeal cyst were found in 29 patients. There was a high incidence of SALs in patients with laryngomalacia. The results of laryngomalacia were predominantly in the group with age of less than one year. There were seven patients more than 5 years of age but only one was diagnosed with laryngomalacia. Other diagnoses included pharyngomalacia, vocal cord paralysis, and airway inflammation. Noticeably, among the severe patients with stridor those more than 5 years of age, 6 patients had underlying neurological problems including cerebral palsy in 3, hypoxic ischemic encephalopathy in 1, and post encephalitis epilepsy in 2. In conclusion, FB is a helpful tool for identifying these airway anomalies among patients with stridor. Laryngomalacia with SALs accounted for a high fraction in our study. Stridor in elder children always has underlying diseases. (J Pediatr Resp Dis 2015;11:40-47) Key words: flexible bronchoscopy, children, stridor INTRODUCTION Stridor or noisy breathing usually reflects an obstruction of upper airway and is the most common Correspondence: Hong-Ren Yu, M.D., Ph.D. Department of Pediatrics, Kaohsiung Chang Gung Memorial Hospital, #123 Ta-Pei Road, Niao-Sung district, Kaohsiung 833 Taiwan Phone: ext. 8713, Fax: yuu2004taiwan@yahoo.com.tw Received: May 18, Accepted: Aug 12, Journal compilation 2015 Taiwan Society of Pediatric Pulmonology symptom of many congenital or acquired airway diseases. Although laryngomalacia is the most common etiology of stridor in infants, a number of acquired or congenital conditions such as tracheal stenosis, foreign body aspiration, gastroesophageal reflux disease, vocal cord paralysis, vascular rings, laryngeal web, hemangiomas, cysts, laryngocele and laryngeal papillomatosis may also cause stridor. 1,2 Flexible bronchoscopy (FB) is an important diagnostic tool to determine the etiology for stridor. Evaluation of lower airways is also important in patients with laryngomalacia because 68 % of these patients have secondary

2 Flexible bronchoscopy for children with stridor associated lower respiratory tract abnormalities. 3 FB is not routinely indicated in every infant with stridor; however, performing the procedure in children with severe/persistent symptoms or in the presence of associated symptoms such as hoarseness or apnea is a widely accepted approach. Presence of an underlying condition other than laryngomalacia or associated abnormalities called as secondary airway lesions (SALs) may alter the management of these patients and prevent unnecessary treatments. 4,5 There are a number of studies evaluating the etiology of stridor and presence of SALs other than laryngomalacia. While some studies suggest that the lower airways should be visualized in all children with stridor due to the possibility of finding a concomitant airway lesion, others suggest that it is an unnecessary and invasive procedure because the observed lesions are rarely clinically significant The aim of this study was to describe the clinical characteristics, FB findings including associated airway abnormalities, and other conditions (i.e. GERD, neurological abnormalities) of children with congenital stridor. Patients and Methods We retrospectively reviewed the medical records of children who underwent FB for the evaluation of stridor between November 2008 and December 2014 in Kaohsiung Chang Gung Memorial Hospital, Division of Pediatric Pulmonology. Indications for FB were as follows: stridor with or without associated findings such as hoarseness, respiratory distress, and radiologic abnormalities. Demographic characteristics, symptoms at presentation, FB findings, presence of associated conditions such as GERD, neurological abnormalities were obtained from the patients medical records. Patients who had symptoms and signs suggestive of swallowing problems and GERD were also investigated by bronchoscopy for esophago-gastric junction and gastric mucosa. Structural or functional airway lesions other than laryngomalacia associated with the etiology of stridor were defined as SALs. 16 FB was performed by the same pediatric pulmonologist with a flexible bronchoscope (Olympus) 2.5 mm, 3.2 mm or 5.0 mm in diameter. Informed consent was obtained from the parents before the procedure. FB was performed transnasally while the patient was breathing spontaneously and under conscious sedation with intravenous midazolam or Ketamine. Lidocaine 2 % and 1 % were used for topical anesthesia of nasal cavities, vocal cords and carina respectively. However, supraglottic structures were examined from above before applying topical anesthesia, because topical anesthesia during FB may exaggerate the findings in children with laryngomalacia. 17 Continuous oxygen was delivered via nasal cannula in order to keep SpO2 above 90 % and patients were monitored for heart rate and arterial oxygen saturation with a pulse oximeter during the entire procedure. Following the procedure, flumazenil was sometimes used in order to reverse the extreme sedative effect and patients were observed until they were fully awake. Results Seventy-two children who underwent FB for the evaluation of stridor were evaluated. FB was performed at ages ranging from 26 days to 16 years. Age distribution is illustrated in Figure 1. Among the 72 children, 51 (70.8%) patients were less than one year old. FB findings of the patients are presented in Table 1. Laryngomalacia was the most common etiology (n=43, 59.7%) and pharyngomalacia followed this (n=13, 18.0%). Nine patients concomitantly presented these two problems as pharyngo-laryngomalacia. Excluding pharyngeal or laryngomalacia, 25 patients (34.7%) still had stridor, which had been induced by other etiologies such as airway inflammation, tracheal malacia, and vocal cord paralysis. Other less common but significant findings such as vallecula cyst (Figures 2a, 2b), hemangioma (Figures 3a, 3b), adenoid hypertrophy (Figures 4a, 4b), tracheitis (Figures 5a, 5b), and granulation (Figures 6a, 6b) usually needed surgical intervention. Among patients with laryngomalacia, isolated laryngomalacia accounted for 12 patients (28%); GERD in 10 patients (23.3%); and secondary associated airway abnormalities were found in 26 patients (60.5%) as Table 2 shows. Although stridor was the main presenting symptom for all patients, the following symptoms were respiratory distress (n=8), and hoarseness (n=5) in addition to stridor. 41

3 Lee WJ, et al. 42 Noticeably, among the seven patients with stridor those more than 5 years old, there were six patients with underlying neurological problems, including cerebral palsy in three, hypoxic ischemic encephalopathy in one, and post encephalitis epilepsy in two. Gastro esophageal reflux disease (GERD): Oesophago-gastrography was also performed in these patients and the results of esophagus-gastric junction erythema or injected were consistent with GERD in 10 (13.8%) patients, which were all concomitant with laryngomalacia in our study. Antireflux treatment was started in these patients. DISCUSSION Laryngomalacia is the most common etiology for stridor in children. Most infants with laryngomalacia do not have severe respiratory compromise or require surgery. 16 Surgery for isolated laryngomalacia is reserved for very severe cases, such as patients with a history of failure to thrive, severe obstructive apnea, or cor pulmonale. However, the presence of SALs and a number of other conditions may also cause or increase stridor and delayed diagnosis and treatment may increase morbidity and mortality in these patients. FB is an important diagnostic tool for definitive diagnosis and may prevent unnecessary treatments. SALs were reported in % of the patients with laryngomalacia. 8, 9, 13, 14, 16, In our study, laryngomalacia was the most common finding and 60.5% of the patients had associated SALs. Several studies suggest that patients with SALs may have more 8, 15, 19, 20 severe symptoms. Importance of diagnosing SALs in these children is controversial. Some studies suggest that only a few children with SALs require surgical intervention. 13,14 The presence of SALs has been reported as a potentially life-threatening condition possibly requiring immediate intervention in other studies. 16,21 Dickson et al. reported that patients with SALs were more likely to require surgical intervention than those without SALs (27 vs. 5.6 %, p = ). 19 Conditions other than laryngomalacia may be the underlying etiology of stridor. Martins reported that infraglottic stenosis, laryngeal and/or tracheal inflammation and bilateral vocal cord paralysis were the most common endoscopic findings in the evaluation of stridor. 21 In our study, 56 children (77.8%) had stridor, which was not only pharyngeal or laryngeal malacia-einduced. In these patients, the etiology of stridor was airway inflammation, tracheomalacia, vocal cord paralysis, laryngeal cyst, and adenoid hypertrophy according to priority. Diagnosis of these conditions would have been missed without FB. There is a high rate of GERD in children with laryngomalacia and GERD is considered as a predisposing factor for the development of laryngomalacia with a rate ranging from 23 to 100 %. 19,25-27 GERD has been shown to stimulate inflammation and edema of posterior glottic and supra-arytenoid tissue that may further compromise airway patency. 27 Upper airflow obstruction may result in negative intrapleural pressure, which induces malfunction of the lower gastroesophageal sphincter and causes GERD. 26 In the current study, 49.5 % of the patients had persistent GERD symptoms and the duration of stridor was significantly longer in these patients compared to those without reflux symptoms. This association of GERD with laryngomalacia is well documented and may have important implications for the evaluation and management of patients with laryngomalacia. 26,28 Figure 1. Seventy-two children were enrolled in our study. Among the 72 children, 51 (70.8%) patients were less than one year old person <1 1~5 5~10 10~15 15~20 Age (year)

4 Flexible bronchoscopy for children with stridor The main limitation of this study is the absence of follow-up and surgery record. Our study is a retrospective study and the surgical interventions were not available when patients visited for a second opinion for surgery. The possibility of SALs should be considered in children with persistent symptoms or in patients with hoarseness, respiratory distress, and Figure 2A. One 1-month old girl, who presented with decreased appetite and progressive stridor noted for 1 day. One huge white-gray vallecula cyst over tongue bass was found, which compressed the epiglottis and obstructed the airway under FB finding. history of long-term intubation during neonatal period. Although the role of FB in evaluating the cause of stridor is still controversial, we suggest that FB be performed in infants with stridor because underlying or associated abnormalities requiring surgical treatment is not rare. Early treatment in these patients may decrease morbidity, mortality and treatment cost. Figure 2B. Head and neck CT showed homogeneous low-density mass over tongue bass (arrow) Figure 3A. One 26-day-old girl, who presented with progressive respiratory distress with stridor noted for 4 days. Vessel-rich lesion (arrow) over upper segment tracheal mucosa r/o hemangioma invasion under FB finding. Figure 3B. Head and neck CT with contrast showed enhanced hemangioma (arrow) over para-tracheal area compressed to trachea and tracheal deviated to right side. 43

5 Lee WJ, et al. Figure 4A. One 5-month-old girl presenting with severe stridor and respiratory distress for nearly one month. One soft tissue mass of adenoid hypertrophy protruding from dome of pharynx area. (Arrow) Figure 4B. Head and neck CT showed one soft tissue density mass over pharynx area, which just obstructed the post-nasal choana. Figure 5A. One 9-month-old girl, presenting with fever and stridor for 4 days. Severe erythematous change of mucosa with pus-like discharge coating was noted by FB.(Arrow) Figure 5B. Head and neck CT lung window showed sludgy shadow in tracheal lumen.(arrow) 44

6 Flexible bronchoscopy for children with stridor Figure 6A. One 7-month-old boy presenting with stridor for 2 months after operation of herniorrhaphy. One granulation-like lesion over subglottic area was noted under FB.(Arrow) Figure 6B. Head and neck CT showed one soft tissue density mass over subglottis area.(arrow) Table 1. Flexible bronchoscopy findings of the patients with stridor. Bronchoscopy findings n(%) Laryngomalacia 43 (59.7%) Pharyngomalacia 13 (18.0%) Airway inflammation 13 (18.0%) Tracheomalacia 11 (15.2%) GERD 10 (13.8%) Vocal cord paralysis 7 (9.7%) Bronchostenosis 4 (5.5%) Macroglossia 3 (4.1%) Laryngeal cysts (Vallecular cyst) 3 (4.1%) Adenoid hypertrophy 3 (4.1%) Macroglossia 3 (4.1%) Vocal cord swelling 3 (4.1%) Subglottic hemangioma 2 (2.7%) Subglottic stenosis 2 (2.7%) Granulation 2 (2.7%) Tracheal stenosis 1 (1.3%) Vascular ring 1 (1.3%) Trachitis 1 (1.3%) Post nasal choanal stenosis 1 (1.3%) Table 2. Among patient with laryngomalacia, 60.5% patients also had secondary airway lesions Laryngomalacia 43(100%) Isolated laryngomalacia 12 (28%) GERD 10 (23.3%) SALs 26 (60.5%) (100%) Pharyngomalacia 9 (34.6%) Tracheomalacia 7 (26.9%) Adenoid hypertrophy 6 (23.1%) Vocal cord paralysis 3 (11.5%) Airway inflammation 3 (11.5%) Laryngeal cysts 2 (7.7%) Bronchus stenosis 2 (7.7%) Macroglossia 2 (7.7%) Granulation 1 (3.8%) Vascular rings 1 (3.8%) Tracheal stenosis 1 (3.8%) 45

7 Lee WJ, et al. REFERENCES 1. Holinger LD, Congenital laryngeal anomalies, In: Holinger LD, Lusk RP, Gren CG (eds) Pediatric Laryngology & Bronchoesophagology. Lippincott- Raven, Philadelphia, pp 1997; Holzki J, Laschat M, Stratmann C, Stridor in the neonate and infant, Implications for the paediatric anaesthetist, Prospective description of 155 patients with congenital and acquired stridor in early infancy. Paediatr Anaesth 1998;8: Nussbaum E, Maggi JC Laryngomalacia in children. Chest 1990;98: Balfour Lynn IM, Spencer H Bronchoscopy-how and when? Paediatr Respir Rev 2002;3: Midulla F, de Blic J, Barbato A, Bush A, et al. Flexible endoscopy of paediatric airways. Eur Respir J 2003;22: Kuo CH, Niu CK, Yu HR, et al. Applications for flexible bronchoscopy in infants with congenital vocal cord paralysis: a 12 year experience. Pediatr Neonatol 2008;49: O Sullivan BP, Finger L, Zwerdling RG Use of nasopharyngoscopy in the evaluation of children with noisy breathing. Chest 2004;125: Vijayasekaran D, Gowrishankar NC, Kaplana S, et al. Lower airway anomalies in infants with laryngomalacia. Indian J Pediatr 77: Masters IB, Chang AB, Patterson L, et al. Series of laryngomalacia, tracheomalacia, and bronchomalacia disorders and their associations with other conditions in children. Pediatr Pulmonol 2002;34: Sa nchez I, Pesce C, Navarro H, et al. Clinical characteristics of children with tracheobronchial anomalies. Pediatr Pulmonol 2003;35: Boogaard R, Huijsmans SH, Pijnenburg MWH, et al. Tracheomalacia and bronchomalacia in children: incidence and patient characteristics. Chest 2005;128: Parente Herna ndez A, Garcı a-casillas MA, Matute JA, et al. Is stridor a banal symptom in infants? An Pediatr (Barc) 2007;66: Krashin E, Ben-Ari J, Springer C, et al. Synchronous airway lesions in laryngomalacia. Int J Pediatr Otorhinolaryngol 2008;72: Yuen HW, Tan HK, Balakrishnan A Synchronous airwaylesions and associated anomalies in children with laryngomalacia evaluated with rigid endoscopy. Int J Pediatr Otorhinolaryngol 70: Bluestone CD, Healy GB, Cotton RT Diagnosis of laryngomalacia is not enough. Arch Otolaryngol Head Neck Surg 1996;122: Mancuso RF Laryngomalacia: the search for the second lesion. Arch Otolaryngol Head Neck Surg 1996;122: Nielson DW, Ku PL, Egger M Topical lidocaine exaggerates laryngomalacia during flexible bronchoscopy. Am J Respir Crit Care Med 2000;161: Hartzell LD, Richter GT, Glade RS et al. Accuracy and safety of tracheoscopy for infants in a tertiary care clinic. Arch Otolaryngol Head Neck Surg 2010;136: Dickson JM, Richter GT, Meinzen-Derr J, et al. Secondary airway lesions in infants with laryngomalacia. Ann Otol Rhinol Laryngol 2009;11: Schroeder JW Jr, Bhandarkar ND, Holinger LD Synchronous airway lesions and outcomes in infants with severe laryngomalacia requiring supraglottoplasty. Arch Otoloaryngol Head Neck Surg 2009;135: Martins RHG, Dias NH, Castilho EC, et al. Endoscopic findings in children with stridor. Rev Bras Otorrinolaringol 2006;72: Gandhi S, Oswal V, Thekedar P, et al. Role of transoral CO2 laser surgery for severe pediatric laryngomalacia. Eur Arch Otorhinolaryngol 2011;268: Belmont JR, Grundfast K Congenital laryngeal stridor (laryngomalacia): etiological factors and associated disorder. Ann Otol Rhinol Laryngol 1984;93: Wiggs JRWJ, DiNardo LJ Acquired laryngomalacia: resolution after neurologic recovery. Otolaryngol Head Neck Surg 1995;112: Thompson DM Abnormal sensorimotor integrative function of the larynx in congenital laryngomalacia: a new theory of etiology. Larngoscope 2007;117: Bibi H, Khvolis E, Sheseyov D, et al. The 46

8 Flexible bronchoscopy for children with stridor prevalence of gastroesophageal reflux in children with tracheomalacia and laryngomalacia. Chest 119: Matthews BL, Little JP, Mcquirt WF Jr et al. Reflux in infants with laryngomalacia: results of 24 hour double-probe ph monitoring. Otoloaryngol Head Neck Surg 1999;120: Giannoni C, Sulek M, Friedman EM, et al. Gastroesophageal reflux association with laryngomalacia: a prospective study. Int J Pediatr Otorhinolaryngol 1998;43: