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Reinforcing the vascular disruption theory of the genesis of Poland’s syndrome: a rare association of diaphragmatic eventration in a preterm infant with severe musculoskeletal defects
  1. Nadira Shahrul Baharin1,
  2. Eman Awadh Hashim1,2,
  3. Quek Bin Huey1,3,4 and
  4. Suresh Chandran1,3,4,5
  1. 1Neonatology, KK Women's and Children's Hospital, Singapore
  2. 2Neonatology, Salmanya Medical Complex, Kingdom of Bahrain, Bahrain
  3. 3Neonatology, NUS Yong Loo Lin School of Medicine, Singapore
  4. 4Neonatology, Duke NUS Medical School, Singapore
  5. 5Neonatology, Lee Kong Chian School of Medicine, Singapore
  1. Correspondence to Professor Suresh Chandran; profschandran2019{at}


A preterm female infant was admitted at birth with respiratory distress. On examination, she had an asymmetric right chest wall and ipsilateral small hand. Air entry was reduced over the right chest. A clinical diagnosis of Poland’s syndrome was made based on the hypoplasia of the right pectoral muscles, absent nipple, deformed ribs and symbrachydactyly of the ipsilateral hand. Chest X-ray suggested and ultrasound confirmed eventration of the right hemidiaphragm. ‘Subclavian artery supply disruption sequence’ (SASDS) theory by Bavnick and Weaver remains the most accepted pathogenic mechanism in Poland’s syndrome. This case reinforces SASDS theory associated with the genesis of Poland’s syndrome that relates to the pathogenicity of vascular disruption of subclavian artery, characteristics of which are unilateral pectoral defects, symbrachydactyly and eventration of the diaphragm. At 2 months, she underwent diaphragm plication. She is under review by our multidisciplinary surgical team for reconstruction of the chest deformity.

  • radiology (diagnostics)
  • musculoskeletal and joint disorders
  • respiratory system
  • genetics
  • neonatal intensive care

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  • Contributors NSB and EAH: manuscript preparation and review of literature. SC and QBH: review and editing of the final manuscript.

  • 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.

  • Competing interests None declared.

  • Patient consent for publication Parental/guardian consent obtained.

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