RT Journal Article
SR Electronic
T1 Severe haemophilia A in a neonate presenting as haemopneumothorax after tracheo-oesophageal fistula-oesophageal atresia repair
JF BMJ Case Reports
FD BMJ Publishing Group Ltd
SP bcr-2018-225526
DO 10.1136/bcr-2018-225526
VO 2018
A1 Zita Hung
A1 Mohammed Bahari
A1 Mark J Belletrutti
A1 Chloe Joynt
YR 2018
UL http://casereports.bmj.com/content/2018/bcr-2018-225526.abstract
AB 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.