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Paediatrics
Alveolar capillary dysplasia complicated by subglottic stenosis
  1. Kotaro Nagamoto1,
  2. Hidehiko Maruyama2,
  3. Akira Ishuguro1 and
  4. Yushi Ito2
  1. 1 Center for Postgraduate, Education and Training, National Center for Child Health and Development, Tokyo, Japan
  2. 2 Neonatology, National Center for Child Health and Development, Setagaya-ku, Japan
  1. Correspondence to Dr Hidehiko Maruyama; maruyamahidehiko{at}gmail.com

Abstract

Alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV) is an interstitial lung disease. In ACDMPV, respiratory impairment with severe pulmonary hypertension occurs from the early hours of life. Anomalies in the cardiovascular, gastrointestinal and genitourinary systems have been reported. However, little is known about upper airway abnormalities. We encountered a genetically diagnosed ACDMPV infant who presented with subglottic and bronchial stenosis. The prenatal diagnosis was hypoplastic left heart syndrome. Her respiratory condition worsened at 16 hours of life. We found subglottic stenosis when intubating. She died on day 7. Autopsy imaging with CT scan showed bilateral main bronchial stenosis. Chromosomal microarray revealed a 531 kb deletion in chromosome 16q24.1, including FOXF1.

  • Pulmonary hypertension
  • Neonatal and paediatric intensive care

https://doi.org/10.1136/bcr-2024-261004

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    Footnotes

    • Contributors The following authors were responsible for drafting of the text, sourcing and editing of clinical images, investigation results, drawing original diagrams and algorithms, and critical revision for important intellectual content: KN, HM, AI. The following authors gave final approval of the manuscript: KN, HM, AI, YI.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.