Case Report
Idiopathic cervical tracheal stenosis in an 11-year-old male

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Abstract

Idiopathic laryngotracheal stenosis is a fibroinflammatory stenosis in persons without alternative explanation such as trauma, intubation, or autoimmune disease. Patients are usually females of child-bearing age. We report on an 11-year-old male who developed progressive dyspnea and stridor. Bronchoscopy revealed 90% stenosis of the cervical trachea. He underwent serial balloon dilation with steroid injection; stenosis decreased to 20%. He had no history of intubation or trauma. Histologic and laboratory workup for autoimmune disease was negative. This report highlights occurrence of a well-known disease in an uncommon population, and alerts providers to consider this when seeing new pediatric patients with symptoms of progressive airway restriction.

Introduction

Idiopathic laryngotracheal stenosis (ITS) is a fibroinflammatory disease resulting in narrowing of the airway at the cricoid and/or proximal trachea [1,2]. It was initially described by Brandenburg in 1972 [3] and represents a diagnosis of exclusion when common etiologies such as intubation, granulomatosis with polyangiitis (formerly Wegener's disease), trauma, or prior surgery have been excluded [4]. Etiology is not known, but may potentially be related to estrogen [5], gastroesophageal reflux disease [6], or disruption of local blood supply secondary to telescoping of the first tracheal ring within the cricoid cartilage [7].

The vast majority of cases occur in females of childbearing age [8]. In reviewing multiple prior patient series, 181 of 184 patients were female with typical mean age of 40–50 years [4,7,[9], [10], [11], [12], [13]]. Notably, there were two outlying female patients aged 13 and 15 years [4,9]. These patients were not separately discussed.

Laryngotracheal stenosis occurs in children, but is typically congenital or related to prior intubation or trauma. Idiopathic laryngotracheal stenosis in this population is extremely rare. In addition to the two aforementioned patients in the larger case series, three single patient case reports are described of idiopathic subglottic stenosis occurring in children. Jazbi et al. reported on a 2.5-year-old male in 1977 who presented with one year of progressive cough, stridor, and mild respiratory distress [14]. Bronchoscopy showed an isolated subglottic stenosis. Rigid dilation was performed. The patient developed recurrent symptoms requiring tracheotomy, but was able to be decannulated five months later with no further sequelae. There is some suspicion, given the young age at presentation, the stenosis may have been secondary to a developmental defect of the cricoid cartilage or conus elasticus [13]. Bodart et al. reported on a 9-year-old male who presented with progressive dyspnea with exertion [15]. Bronchoscopy showed false membranes with inflammatory granulomas at the upper trachea. The granulomas were removed and systemic steroids were administered. Histology demonstrated non-specific inflammatory granulation tissue with epidermoid metaplasia, in contrast to the non-inflammatory pearl-like appearance seen in congenital subglottic stenosis [15]. He underwent carbon dioxide laser incision of the stenosis two months later for progressive symptoms followed by budesonide nebulizers for recrudescent symptoms with good effect. Modgil et al. reported on a 12-year-old female who presented with six months of progressive inspiratory stridor and dyspnea with exertion not relieved by bronchodilators and inhaled steroids [16]. Interestingly, symptoms began with menarche and worsened with menstruation. Bronchoscopy showed a 75% concentric subglottic stenosis. Omeprazole was given with no improvement in symptoms. Tracheotomy was then performed, followed by carbon dioxide laser incision and mitomycin-C injections. The patient was decannulated six weeks later. There were no further reported complications.

In addition to idiopathic subglottic stenosis, idiopathic tracheal stenosis can also occur. This is a rare condition in pediatric patients with few prior published reports. Beddow et al. reported on two 11-year-old patients with symptoms of progressive airway restriction who were found to have tracheal stenosis without identifiable cause and were managed with open surgery [17]. Watson et al. described the case of an 8-year-old with progressive cough, dyspnea, and stridor managed with endoscopic dilations and high-dose steroids [18].

We report on an 11-year-old male who presented with progressive dyspnea and stridor and was found on bronchoscopy to have 90% concentric stenosis of the cervical trachea on bronchoscopy with negative workup for alternative etiologies for subglottic stenosis. This report adds to the small body of literature on pediatric idiopathic laryngotracheal stenosis and raises awareness of this disease entity.

Section snippets

Case report

An 11-year-old male presented to his family practitioner with one month of progressive dyspnea and two weeks of noisy breathing. He had no history of prior intubation, neck trauma, recent infectious disease, or autoimmune disease. Expiratory peak flow was 100 L/min (normal value for height: 370 L/min) that did not change after an albuterol nebulizer. He was prescribed albuterol, prednisone, and fluticasone nasal sprays. Symptoms did not improve over the next three weeks. A chest x-ray was

Discussion

Laryngotracheal stenosis represents a diagnostic challenge for both the primary provider and otolaryngologist; accordingly, delays in diagnosis are common [19]. Noise with respiration can lead to a presumptive diagnosis of asthma and treatment with bronchodilators. It is important to note when the noise occurs in the respiratory cycle, as noise with inspiration suggests an extrathoracic rather than intrathoracic obstruction. In our patient, the presence of biphasic stridor and respiratory

Conflicts of interest

None.

References (21)

  • E.J. Damrose

    On the development of idiopathic subglottic stenosis

    Med. Hypotheses

    (2008)
  • E. Beddow et al.

    Idiopathic tracheal and bronchial stenosis in two pediatric patients

    Ann. Thorac. Surg.

    (2004)
  • G. Watson et al.

    Relapsing diffuse idiopathic tracheal stenosis in a child: dilemmas in management

    Int. J. Pediatr. Otorhinolaryngol. Extra

    (2006)
  • A. Gelbard et al.

    Causes and consequences of adult laryngotracheal stenosis

    Laryngoscope

    (2014)
  • S.M. Kraft et al.

    Using pulmonary function data to assess outcomes in the endoscopic management of subglottic stenosis

    Ann. Otol. Rhinol. Laryngol.

    (2015)
  • J.H. Brandenburg

    Idiopathic subglottic stenosis

    Trans. Am. Acad. Ophthalmol. Otolaryngol.

    (1972 Sep-Oct)
  • T.A. Valdez et al.

    Idiopathic subglottic stenosis revisited

    Ann. Otol. Rhinol. Laryngol.

    (2002)
  • B. Benjamin et al.

    Idiopathic subglottic stenosis: diagnosis and endoscopic laser treatment

    Ann. Otol. Rhinol. Laryngol.

    (1997)
  • J.R. Jindal et al.

    Gastroesophageal reflux disease as a likely cause of “idiopathic” subglottic stenosis

    Ann. Otol. Rhinol. Laryngol.

    (1994)
  • F. Moldanado et al.

    Idiopathic subglottic stenosis: an evolving therapeutic algorithm

    Laryngoscope

    (2014)
There are more references available in the full text version of this article.

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