Bingham, Catherine April, MD

Chennupati, Sri Kiran, MD

Greenawald, Lauren, DO

Kuntzman, Kaitlyn, DO

Tolaymat, Naser, MD

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Lehigh Valley Health Network LVHN Scholarly Works Department of Pediatrics Progressive Stridor: Extraintestinal Airway Manifestations in a Pediatric Patient With Inflammatory Bowel Disease Kaitlyn Kuntzman DO Lehigh Valley Health Network, kaitlyn.kuntzman@lvhn.org Sri Kiran Chennupati MD Lehigh Valley Health Network, sri_k.chennupati@lvhn.org Catherine April Bingham MD Lehigh Valley Health Network, c_april.bingham@lvhn.org Naser Tolaymat MD Lehigh Valley Health Network, Naser.Tolaymat@lvhn.org Lauren Greenawald DO Lehigh Valley Health Network, lauren.greenawald@lvhn.org Follow this and additional works at: https://scholarlyworks.lvhn.org/pediatrics  Part of the Pediatrics Commons Published In/Presented At Kuntzman, K., Chennupati, S., Bingham, C.A., Tolaymat, N., & Greenawald, L. (2020, June). Progressive Stridor: Extraintestinal Airway Manifestations in a Pediatric Patient With Inflammatory Bowel Disease. Poster Presented at: LVHN Pediatric Residency Research Day, Virtual. This Poster is brought to you for free and open access by LVHN Scholarly Works. It has been accepted for inclusion in LVHN Scholarly Works by an authorized administrator. For more information, please contact LibraryServices@lvhn.org. LVHN.org/children© 2020 Lehigh Valley Health NetworkProgressive Stridor: Extraintestinal Airway Manifestations in a  Pediatric Patient With Inflammatory Bowel DiseaseKaitlyn Kuntzman, DO,1 Sri Chennupati, MD,2 April Bingham, MD,3 Naser Tolaymat, MD,4 Lauren Greenawald, DO5 1Division of Pediatrics, 2Division of Pediatric Otolaryngology, 3Division of Pediatric Rheumatology, 4Division of Pediatric Gastroenterology, 5Division of Pulmonary Medicine, Lehigh Valley Reilly Children’s Hospital, Allentown, PAIntroductionAirway manifestations of Inflammatory Bowel Disease (IBD) are rare in pediatrics with few publications or case reports.Stridor in pediatric patients is typically caused by infection, vocal cord dysfunction, neoplasms,  or anatomical abnormalities. Tracheal abnormalities are commonly caused by airway trauma, infection, vasculitis, or malignancy in children. This case report presents a pediatric female with progressive stridor resultant from airway granulation tissue and ulceration formation in the setting of ulcerative colitis (UC).Clinical Presentation and Initial EvaluationA nine-year-old female of Indian descent with a past medical history of mild persistent asthma  and UC presented to the Lehigh Valley Reilly Children’s Hospital pediatric pulmonology office  with two months of worsening stridor and dyspnea. Spirometry revealed mild airway obstruction with forced expiratory volume in one second (FEV1) 61% with a diminished flow volume loop and flattened inspiratory limb. Her baseline inhaled steroids were subsequently increased and bronchodilator therapy initiated. Her symptoms  worsened over the subsequent 4 weeks and repeat spirometry showed an FEV1 of 41% with  flat inspiratory and expiratory limbs. Due to the restrictive appearance of the spirometry loop  and audible homophonous wheezing, there was concern for upper airway abnormalities, so  imaging was performed. Chest Xray was normal. Airway fluoroscopy noted fixed, diffuse tracheal narrowing measuring 4.7mm in diameter Radiology recommended CT of the neck/chest in follow-up which showed circumferential, heterogenous tracheal soft tissue thickening and diffuse nodular appearance  of the posterior trachea and mainstem bronchi with no parenchymal lung disease. Flexible and rigid bronchoscopy visualized white endophytic tracheal granulation tissue extending past the carina bilaterally with ulceration notable in the right mainstem at the division of the bronchus intermedius and right upper lobe. Granulation tissue was removed and sent to pathology. Bronchial lavage cultures were obtained.  Diagnostic Testing and Managementreferences1. Ji, Xiao-Qing. “Pulmonary Manifestations of Inflammatory Bowel Disease.” World Journal of Gastroenterology, vol. 20, no. 37, 2014, p. 13501., doi:10.3748/wjg.v20.i37.13501.2. Prince, Jeffrey S., et al. “Nonneoplastic Lesions of the Tracheobronchial Wall: Radiologic Findings with Bronchoscopic Correlation.” RadioGraphics, vol. 22, no. suppl_1, 2002, doi:10.1148/radiographics.22.suppl_1.g02oc02s215.Figure 2: CT Chest: Diffuse nodular thickening of the trachea and right and left mainstem bronchi, airway narrowing beginning at the level of the thoracic inlet and extending into the upper mediastinum.Figure 3: Visible friable tissue with white, cystic like tissue protruding from lateral walls. Branching of the bronchus intermedius shows ulceration with white circumferential inflammatory tissue extending past the carina with an ulcerative lesion in the right mainstem. Figure 1: Spirometry reveals worsening restrictive airway disease over time. Flow volume loops 1 through 3 represent pretreatment loops in chronological order from time of presentation to immediately prior to diagnosis and treatment. . Flow volume loop 4 represents lung function after treatment with systemic corticosteroids. Tracheal pathology noted tiny fragments of inflamed granulation tissue, large fragments of fibrin with leukocytes most likely representing the bottom of a chronic ulcer. Bronchoalveolar lavage cultures were negative for infectious etiology. Rheumatologic evaluation via laboratory analysis was negative for systemic inflammatory disorders (Relapsing Polychondritis and Granulomatosis with Polyangiitis). Antineutrophil cytoplasmic antibodies were positive with an atypical perinuclear pattern typical of UC.Treatment consisted of oral prednisone for 14 days and optimization of her asthma regimen. At present, she is doing well with occasional recurrence of stridor that responds well to inhaled steroid treatment. Surveillance plan includes frequent monitoring with both pulmonology and gastroenterology and serial lung function testing.DiscussionThis case presents a rare example of extraintestinal manifestations of IBD in a pediatric patient. Airway involvement occurs in 0.21 to 0.40% of all IBD patients. There is no  direct correlation between control of gastrointestinal IBD symptoms and development of extraintestinal manifestations. Additionally, there is no known rate of incidence reported  in children. This case shows stepwise progression to diagnosis using clinical history, spirometry, imaging, airway evaluation, laboratory analysis and multidisciplinary discussion. Clinical history and spirometry were paramount to diagnosis and will be used to track intrinsic changes and  follow progression over time.  1. 2. 3. 4.

Progressive Stridor: Extraintestinal Airway Manifestations in a Pediatric Patient With Inflammatory Bowel Disease

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