PDF(Pubmed)
Abstract：
UNASSIGNED: Vigilant monitoring for postoperative complications, including bleeding and dysrhythmia, is crucial in patients with craniosynostosis syndromes like Crouzon syndrome undergoing craniofacial surgery, with a thorough evaluation, including coagulation tests, assisting in diagnosing underlying conditions such as von Willebrand disease subtype 1 to inform appropriate management strategies.
UNASSIGNED: Crouzon syndrome is a rare genetic disorder affecting craniofacial structures. Its etiology is the premature fusion of cranial sutures. The LeFort III advancement surgery is a commonly used approach to correct malformations related to midface hypoplasia. Complications following surgical treatment of craniosynostosis and craniofacial syndromes can include both intracranial and extracranial problems. Reporting of this syndrome and the surgery complications, in addition to consideration of other differential diagnoses, can help improve the treatment plan and surgery outcomes. The aim of the article is to report a 14-year-old female with Crouzon syndrome who underwent the modified LeFort III osteotomy and developed unexpected massive bleeding during the surgery. Post-surgery, she experienced complications including dysrhythmia, hypothermia, and cyanosis. Treatment included fluid therapy, blood transfusions, and antibiotic therapy for suspected septic shock. Differential diagnosis was disseminated intravascular coagulation but was ruled out. Post-discharge, coagulation tests suggested von Willebrand disease subtype 1 as the diagnosis. Excessive bleeding during surgery for craniosynostosis syndromes is a significant and concerning issue in the surgical management of Crouzon syndrome. For patients with von Willebrand disease who are candidates for elective surgeries, von Willebrand factor concentrates or recombinant von Willebrand factor can be used.
UNASSIGNED: Crouzon syndrome is a rare genetic disorder affecting craniofacial structures. Its etiology is the premature fusion of cranial sutures. The LeFort III advancement surgery is a commonly used approach to correct malformations related to midface hypoplasia. Complications following surgical treatment of craniosynostosis and craniofacial syndromes can include both intracranial and extracranial problems. Reporting of this syndrome and the surgery complications, in addition to consideration of other differential diagnoses, can help improve the treatment plan and surgery outcomes. The aim of the article is to report a 14-year-old female with Crouzon syndrome who underwent the modified LeFort III osteotomy and developed unexpected massive bleeding during the surgery. Post-surgery, she experienced complications including dysrhythmia, hypothermia, and cyanosis. Treatment included fluid therapy, blood transfusions, and antibiotic therapy for suspected septic shock. Differential diagnosis was disseminated intravascular coagulation but was ruled out. Post-discharge, coagulation tests suggested von Willebrand disease subtype 1 as the diagnosis. Excessive bleeding during surgery for craniosynostosis syndromes is a significant and concerning issue in the surgical management of Crouzon syndrome. For patients with von Willebrand disease who are candidates for elective surgeries, von Willebrand factor concentrates or recombinant von Willebrand factor can be used.
摘要：
■术后并发症的警惕监测，包括出血和心律失常,对于接受颅面手术的颅骨融合综合征如克鲁松综合征患者至关重要，经过全面评估，包括凝血测试，协助诊断基础疾病，如vonWillebrand病亚型1，以告知适当的管理策略。
■克鲁松综合征是一种罕见的影响颅面结构的遗传性疾病。其病因是颅骨缝线过早融合。LeFortIII前移手术是一种常用的方法，用于纠正与中面发育不全有关的畸形。手术治疗颅骨融合和颅面综合征后的并发症可能包括颅内和颅外问题。报告这种综合征和手术并发症，除了考虑其他鉴别诊断，可以帮助改善治疗计划和手术效果。本文的目的是报告一名14岁的Crouzon综合征女性，她接受了改良的LeFortIII截骨术，并在手术过程中出现了意外的大出血。手术后,她经历了包括心律失常在内的并发症,体温过低,和紫癜。治疗包括液体治疗，输血,以及疑似感染性休克的抗生素治疗。鉴别诊断为弥散性血管内凝血，但被排除。放电后,凝血测试表明vonWillebrand病亚型1作为诊断。颅骨融合综合征手术期间的过度出血是克鲁松综合征手术治疗中的一个重要且令人担忧的问题。对于接受择期手术的vonWillebrand病患者，可以使用血管性血友病因子浓缩物或重组血管性血友病因子。
■克鲁松综合征是一种罕见的影响颅面结构的遗传性疾病。其病因是颅骨缝线过早融合。LeFortIII前移手术是一种常用的方法，用于纠正与中面发育不全有关的畸形。手术治疗颅骨融合和颅面综合征后的并发症可能包括颅内和颅外问题。报告这种综合征和手术并发症，除了考虑其他鉴别诊断，可以帮助改善治疗计划和手术效果。本文的目的是报告一名14岁的Crouzon综合征女性，她接受了改良的LeFortIII截骨术，并在手术过程中出现了意外的大出血。手术后,她经历了包括心律失常在内的并发症,体温过低,和紫癜。治疗包括液体治疗，输血,以及疑似感染性休克的抗生素治疗。鉴别诊断为弥散性血管内凝血，但被排除。放电后,凝血测试表明vonWillebrand病亚型1作为诊断。颅骨融合综合征手术期间的过度出血是克鲁松综合征手术治疗中的一个重要且令人担忧的问题。对于接受择期手术的vonWillebrand病患者，可以使用血管性血友病因子浓缩物或重组血管性血友病因子。
