Journal of Otolaryngology and Ophthalmology of Shandong University ›› 2025, Vol. 39 ›› Issue (3): 77-80.doi: 10.6040/j.issn.1673-3770.0.2024.514

• Shanghai Sixth Peoples Hospital Otorhinolaryngology & Head and Neck Surgery Department dedicated to “120th Anniversary of the Sixth Hospital” Commemorative Theme • Previous Articles    

Adenotonsillectomy in a child with congenital fibrinogen deficiency and literature review

WANG Yao, CHEN Jingyi, YANG Yingchao, SU Kaiming   

  1. Department of Otorhinolaryngology & Head and Neck Surgery, Shanghai Sixth People's Hospital Affiliated to Shanghai Jiao Tong University School of Medicine / Shanghai Key Laboratory of Sleep Disordered Breathing / Otolaryngology Institute of Shanghai Jiao Tong University, Shanghai 200233, China
  • Published:2025-06-04

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Abstract: Objective To investigate the perioperative management strategies for adenotonsillectomy in pediatric patients with Congenital Hypofibrinogenemia(CFD). Methods We reviewed and analyzed the perioperative management of a pediatric patient with CFD who underwent adenotonsillectomy using plasma ablation at our hospital, in conjunction with a review of the relevant literature. Results With perioperative fibrinogen replacement therapy, the patient's fibrinogen level was successfully raised from 0.23 g/L to 0.92 g/L, allowing for the safe performance of adenotonsillectomy. The patient recovered well postoperatively without any bleeding complications. A literature search revealed no previous reports of adenotonsillectomy in patients with CFD. However, the target fibrinogen level for supplementation in CFD patients undergoing other surgical procedures is typically 0.5-1.0 g/L or appropriately higher. Conclusion Adenotonsillectomy can be safely performed in pediatric patients with CFD through effective perioperative fibrinogen replacement therapy.

Key words: Congenital hypofibrinogenemia, Tonsil hypertrophy, Adenoidal hypertrophy, Tonsillectomy &, adenoidectomy

CLC Number: 

  • R762.3
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