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Severe haemophilia A in a neonate presenting as haemopneumothorax after tracheo-oesophageal fistula-oesophageal atresia repair
  1. Zita Hung1,
  2. Mohammed Bahari1,
  3. Mark J Belletrutti2,
  4. Chloe Joynt1
  1. 1Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada
  2. 2Department of Pediatrics, Division of Hematology Oncology and Palliative Care, University of Alberta, Edmonton, Alberta, Canada
  1. Correspondence to Dr Zita Hung, zhung{at}


A male infant with oesophageal atresia and distal tracheo-oesophageal fistula (TEF type C) underwent right thoracotomy and transpleural repair of TEF on day 4 of life. He did not have a family history of coagulation disorders. A preoperative finding of prolonged partial thromboplastin time (PTT)>200 s was overlooked, and he went to surgery. There were no concerns with haemostasis prior to and even during the operation. The prolonged PTT was treated with one 10 mL/kg dose of fresh frozen plasma in the immediate postoperative period. On the fourth postoperative day, the infant developed a right haemopneumothorax, requiring fresh frozen plasma and packed cell transfusions. He was subsequently diagnosed with severe haemophilia A due to intron 22 inversion in the factor VIII gene, with factor VIII level <0.01 IU/mL.

  • neonatal intensive care
  • haematology (incl blood transfusion)
  • paediatric surgery
  • otolaryngology / ENT
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  • Contributors ZH and MB conceptualised this report, gathered the data, drafted the manuscript and approved the case report as submitted. CJ critically reviewed, revised and approved the case report as submitted. MJB provided additional data, critically reviewed and approved the case report as submitted.

  • 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 Parental/guardian consent obtained.

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

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