Persistent Left Mainstem Bronchial Stenosis After Surgical Treatment for Neuromuscular Kyphoscoliosis

To the Editor, A 14-year-old adolescent with complex medical background was admitted for respiratory failure, polyserositis and left lung collapse (Figure 1). He has a history of de novo variant in RHEB (Ras Homolog Enriched in Brain) protein, macrocephaly, truncal hypotonia, global developmental delay, GMFCS 4 cerebral palsy, seizure disorder and kyphoscoliosis, status post posterior spinal fusion in 2022. He is gastrostomy fed and does not need oxygen nor ventilatory support at baseline. On admission in May 2024, he required High Flow Nasal Cannula support, bilateral thoracocentesis and pericardial drainage at the PICU. Left lung collapse persisted despite successful drainage of the pericardial and pleural effusions. Nasopharyngeal swab showed positive human rhinovirus/enterovirus. Computed tomography of the chest (CT Chest) showed complete left lung collapse and obscured left mainstem bronchus. There was no adjacent mass, lymphadenopathy, or obvious reason for lung collapse (Figure 2). Bronchoscopy showed a short segment bronchial stenosis at the junction of proximal and middle third of the left mainstem bronchus (Figure 3). The distal left mainstem bronchus, left segmental bronchi and bronchial tree on the right side were unremarkable. Copious secretions were seen at the left bronchial tree throughout. Secretions were suctioned clearly. Bronchoalveolar lavage was performed, which was paucicellular on cytology and negative for microbiological tests. Vigorous airway clearance therapy was provided. His left lung collapse resolved and he returned to his normal baseline upon discharge (Figure 4). He was not known to have any tracheobronchial or pulmonary abnormalities before this admission. His past chest x-ray's were reviewed. Retrospectively, we were able to identify a short and narrowed segment of the left main stem bronchus on chest x-ray's taken in 2022 which likely represents a prior distortion of the bronchus as a result of his significant kyphoscoliosis (Figure 5). The natural history of neuromuscular kyphoscoliosis would usually progress as the child grows [1], timely treatment is important to prevent long term complications. While standard lung function test may not be achievable for every patient, other forms of airway or respiratory assessment would be necessary. Three-dimensional models of spine and airway lumen could be reconstructed for preoperative assessment [2] which may provide some guidance. Complications of cardiorespiratory failure with untreated kyphoscoliosis was well described [3]. However, the clinical course in children with neuromuscular kyphoscoliosis after corrective surgery is less clear. Relief of central airway obstruction following spinal release in patient with idiopathic scoliosis was reported before [4], however, our report has suggested that the airway complication may persist, even at 2 years after corrective spinal surgery in a developing adolescent. Caution and individualized plan would be warranted for the management of children with neuromuscular kyphoscoliosis. Jeff Wong: writing–original draft, writing–review and editing, conceptualization, investigation. The author deeply appreciated the patient's parents for permission to publish this case. The author declares no conflicts of interest. The author has obtained approval from the IWK Research Ethics Board, Project#: 1030922, for the use of deidentified information collected from the patient. Written informed consent was also obtained from patient's mother for the publication of this case report. Data sharing not applicable to this article as no datasets were generated or analyzed during the current study.

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