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Pediatric Cystic Deep Neck Mass Presenting With Stridor and Dysphagia

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 6-month-old girl presented to the emergency department for evaluation of fever and was noted to have mild inspiratory stridor. Her parents stated that her stridor began acutely at 4 months of age without any inciting illness or event. The stridor was worse with agitation but was without positional exacerbation. The family reported that the stridor had remained stable since onset. Her parents noted mild dysphagia but denied shortness of breath, cyanosis, or apnea. Laboratory test results revealed acute COVID-19 infection. Physical examination was unremarkable other than mild inspiratory stridor. Findings from bedside flexible laryngoscopy revealed intact vocal fold motion bilaterally with glottic competence. No supraglottic or glottic masses were identified. Anterior-posterior neck and chest radiography demonstrated narrowing and tortuosity of the trachea with displacement to the right proximal to the thoracic inlet and diffuse patchy heterogenous airspace opacities (Figure, A). Subsequent neck ultrasonography and computed tomography scan demonstrated a 3.2 × 2.0-cm low-density lesion abutting the trachea and hypopharynx at the level of thoracic inlet, with rightward displacement of the trachea and larynx (Figure, B-D). Operative laryngoscopy, bronchoscopy, and esophagoscopy revealed extrinsic compression of the trachea, right mainstem bronchus, and esophagus with rightward deviation of the trachea and esophagus. No mucosal tract was identified.

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B. Foregut duplication cyst

Foregut duplication cysts originate from persistent heterotopic rests of foregut-derived epithelium. Traditional diagnostic criteria include (1) attachment to some portion of the foregut, (2) the presence of an outer smooth muscle layer, and (3) an epithelial lining consistent with some portion of the foregut.1 These cysts contain epithelial and mesenchymal elements related to the organ of origin. Accordingly, foregut duplication cysts are subclassified as bronchogenic cysts, esophageal duplication cysts, or enteric duplication cysts, depending on the epithelium.2,3 Bronchogenic cysts are lined with ciliated respiratory epithelium, which may demonstrate squamous metaplasia and seromucinous glands, with underlying smooth muscle and hyaline cartilage resembling a bronchus. These are the most common, comprising more than 50% of mediastinal cysts.2 Esophageal duplication cysts arise within the esophageal wall and are lined with squamous or respiratory-type epithelium and contain smooth or striated muscle reminiscent of the esophagus. Enteric duplication cysts are less commonly connected to the esophagus, but may be closely associated with vertebrae, and are lined with gastric or intestinal mucosa with an overlying muscle layer.3 Of note, the cysts often demonstrate mixed mucosal types and may not represent the typical mucosa found at the anatomic level of the cyst.3 In the described case, pathologic findings demonstrated mixed squamous and ciliated respiratory epithelium with stroma containing both smooth and skeletal muscle and benign mucosal glands, most consistent with bronchogenic duplication cyst.

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Article Information

Corresponding Author: Teresa G. Vos, MD, Department of Otolaryngology–Head & Neck Surgery, School of Medicine, University of North Carolina at Chapel Hill, 170 Manning Dr, Houpt Building, CB 7070, Chapel Hill, NC 27599-7070 (teresa.vos@unchealth.unc.edu).

Published Online: September 22, 2022. doi:10.1001/jamaoto.2022.2788

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient’s parents for granting permission to publish this information.

References
1.
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2.
Kieran  SM , Robson  CD , Nosé  V , Rahbar  R .  Foregut duplication cysts in the head and neck: presentation, diagnosis, and management.   Arch Otolaryngol Head Neck Surg. 2010;136(8):778-782.PubMedGoogle ScholarCrossref
3.
Bush  A , Chitty  L , Harcourt  J , Hewitt  RJ , Nicholson  AG . Congenital lung disease. In:  Kendig’s Disorders of the Respiratory Tract in Children. Elsevier; 2019:289-337.
4.
Veeneklaas  GMH .  Pathogenesis of intrathoracic gastrogenic cysts.   AMA AJDC. 1952;83(4):500-507.PubMedGoogle Scholar
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Azzie  G , Beasley  S .  Diagnosis and treatment of foregut duplications.   Semin Pediatr Surg. 2003;12(1):46-54.PubMedGoogle ScholarCrossref
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Fraser  L , Howatson  AG , MacGregor  FB .  Foregut duplication cyst of the pharynx.   J Laryngol Otol. 2008;122(7):754-756.PubMedGoogle ScholarCrossref
7.
Kim  JK , Park  K-K .  Foregut duplication cyst of the hypopharynx: a rare cause of upper airway obstruction.   J Pediatr Surg. 2007;42(6):E5-E7.PubMedGoogle ScholarCrossref
8.
Edwards  J , Pearson  S , Zalzal  G .  Foregut duplication cyst of the hypopharynx.   Arch Otolaryngol Head Neck Surg. 2005;131(12):1112-1115.PubMedGoogle ScholarCrossref
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Canty  TG , Hendren  WH .  Upper airway obstruction from foregut cysts of the hypopharynx.   J Pediatr Surg. 1975;10(5):807-812.PubMedGoogle ScholarCrossref
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Fitch  SJ , Tonkin  IL , Tonkin  AK .  Imaging of foregut duplication cysts.   Radiographics. 1986;6(2):189-201.PubMedGoogle ScholarCrossref
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