A novel technique for balloon dilation of multifocal bronchial stenosis in granulomatosis with polyangiitis

Bronchial stenosis is a rare and potentially life-threatening condition in children that can be challenging to address.1 Bronchoscopic treatment of bronchial stenosis in adults is more well-established, however adapting endoscopic approaches to children can be limited by the smaller diameter of their airways, with limited reports on the use of fiberoptic bronchoscopic balloon dilation in children. In this report, we describe the case of a child with multifocal bronchial stenosis in the context of granulomatosis with polyangitis (GPA), who successfully underwent bronchial recanalization and balloon dilation using a novel fluoroscopic and bronchoscopic approach. A 12-year-old male with a diagnosis of GPA developed debilitating dyspnea and worsening obstruction on spirometry (FEV1 = 37% predicted) despite treatment with systemic steroids, rituximab, and cyclophosphamide. Bronchoscopy was performed, revealing membranous obliteration of the segmental bronchi of the right upper lobe (RUL) and superior segment of the left lower lobe (LLL) and diffuse bronchial stenosis. computed tomography (CT) chest showed RUL atelectasis and right lower lobe (RLL) bronchiectasis. He was referred for management of bronchial stenosis. Flexible bronchoscopy was performed in our hybrid operating room in collaboration with our colleagues from interventional radiology, where we had access to an Intraoperative C-arm cone beam CT and fluoroscopy. Bronchoscopy performed using an Olympus 4.2 mm video bronchoscope (BF-P190) with a 2.0 mm working channel identified membranous occlusion of the three RUL segments and severe stenosis of the bronchus intermedius, right middle lobe (RML), left mainstem bronchus (LMSB), left upper lobe (LUL), and lingula, and occlusion of the LLL superior segment (Figure 1A–D). A 21-gauge PeriView Flex Olympus needle was advanced via the working channel under fluoroscopic guidance to traverse the membrane of the obliterated bronchi in the RUL (Figure 2A,D). Once an opening was created in the membrane, the Olympus 1.9 mm EndoJaw biopsy forceps were traversed in a closed position through the small hole created by the needle, and then opened and pulled back to enlarge the opening. Then, a 0.018 Boston Scientific Thruway guidewire was introduced through a five French Cobra catheter to help direct the guidewire, and was advanced in tandem via the endotracheal tube alongside the bronchoscope through the opening (Figure 2B,E). Balloon dilation was performed over this guidewire, with a 4 mm and 6 mm × 2 cm monorail balloon catheter, with the balloon inflated up to 10–12 atmospheres until the waist in the balloon was eliminated for 2 min (Figure 2C,F). The LLL superior segment was similarly recannulated (Figure 2G–L), however, the guidewire was passed through the PeriView needle while inserted through the working channel. The needle was removed over the guidewire, and a 6 mm × 2 cm balloon was passed over the guidewire, though the working channel of the bronchoscope, while leaving the bronchoscope in place for visualization. Following re-expansion of the RUL, which was found to be severely bronchiectatic, the patient developed a pneumothorax that was evacuated with a pigtail and resolved within 24 h. Unfortunately, on follow-up evaluations, the openings to the RUL were completely obliterated (Figure 1E). Over the following weeks, he underwent dilations of the bronchus intermedius, RML, LMSB, LUL, and lingula (Figure 1E–H), which were similarly dilated over multiple procedures with balloons up to 12 mm in diameter through the working channel of the 4.2 mm bronchoscope. Before balloon dilations, kenalog (40 mg in 1 mL solution) was injected via the PeriView needle into the stenotic segments. Following balloon dilations, a 1.1 or 1.7 mm flexible cryoprobe (Erbe) was used to perform tissue devitalization using 60 s freeze cycles. There were no other occurrences of air leak, no significant bleeding, mucosal lacerations or perforations, and no episodes of hypoxemia. Following serial dilations, his FEV1 improved to 73% predicted, and he's had near complete resolution of his exertional dyspnea. He is currently undergoing monthly surveillance bronchoscopy. Multilevel bronchial stenosis is a rare and potentially life-threatening manifestation of GPA in children, and its management remains challenging.2 While there is growing interest in pediatric interventional bronchoscopy, with increasing applications being recognized,3 the use of interventional bronchoscopic tools in children can be limited by their smaller airway size and smaller working channel of pediatric bronchoscopes.4 The optimal approach to managing bronchial stenosis in children remains unknown, often requiring repeated bronchoscopic interventions with the primary objective to maintain intraluminal patency.5 Balloon dilation for stenotic airway diseases in children has been previously described mainly for subglottic and tracheal stenosis,6 however, data on the treatment of bronchial obstruction in children is limited.1 Angioplasty balloon catheters are the most widely used for bronchial balloon dilation in children, and have been described in conjunction with intralesional steroids, application of topical mitomycin C, laser treatment, cryotherapy, and stent placement.1, 2, 7 Given the challenges of treating progressive multilevel bronchial stenosis in GPA, it has been previously suggested that the use of cryotherapy via flexible bronchoscopy for cryodevitalization may be of particular benefit in patients with GPA to prevent scarring and decrease the risk of recurrence.7, 8 Reports have established the safety of balloon dilation in pediatric age patients for treating tracheobronchial stenosis.1 To our knowledge, this is the first reported case in a child of using a flexible bronchoscope needle to create an opening in an occlusive bronchial membrane to permit passage of a guidewire for balloon dilation. With this approach, we successfully advanced the angioplasty balloon catheters both in parallel and through the working channel of the 4.2 mm bronchoscope to perform balloon dilation of several bronchial segments without complication. This technique for the management of bronchial stenosis with balloon dilation via the working channel of the bronchoscope to the level of the segmental bronchi can be adopted to any young child who can accommodate a 4.2 mm bronchoscope, and when balloon dilation is performed in parallel with the bronchoscope, it is even feasible in any pediatric patient that can accommodate an even smaller bronchoscope of any size. Patient-specific considerations on selecting equipment size, including the choice of angioplasty balloon size poses additional challenges in children. Additional risks include maintaining adequate oxygenation and ventilation, prolonged procedure time, and airway trauma including laceration, perforation, and bleeding.1 In our patient, the lack of alternative treatment options coupled with debilitating dyspnea and obstruction prompted the necessity of this intervention. This undertaking should only be considered in specialized centers with appropriate bronchoscopy experience and can successfully result in increased airway diameter thereby immediately improving symptoms. Our case also highlights the importance of interdisciplinary and multicenter collaborations in the advancement of pediatric interventional bronchoscopy. Carolyn Wallace: Writing—review and editing; resources. Nicole Hilvert: Writing—review and editing; Resources. Evans M. Machogu: Writing—review and editing. Pi Chun Cheng: Writing—review and editing. Olivia A. Kwan: Writing—review and editing. Douglas C. von Allmen: Writing—review and editing; methodology; conceptualization. John M. Racadio: Conceptualization; writing—review and editing; supervision; methodology; visualization. Erik Hysinger: Conceptualization; writing—review and editing; supervision; methodology; visualization. The authors declare no conflict of interest.

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