Nixon R G, Brindley G W
Department of Orthopedic Surgery, Scott and White Memorial Hospital, Temple, TX 76508.
Clin Orthop Relat Res. 1989 Jul(244):176-81.
A three-month-old male infant with undiagnosed hemophilia presented with fever and irritability. During the diagnostic evaluation a venipuncture in the right antecubital fossa led to hemorrhage and a compartment syndrome in the brachium. Differential diagnosis included osteomyelitis and septic arthritis but a 99technetium bone scan revealed only delayed washout of radioactive material from the right brachium without increased uptake. Despite elevated pressures in the muscle compartments of the right arm, surgical decompression was delayed until a complete blood coagulation profile was obtained. After replacement of deficient blood products, the compartment syndrome resolved without surgical decompression. Defective coagulation leading to hemorrhage into the muscle compartments of the arm resulted in a significant elevation of compartmental pressure. The unusual clinical presentation of the patient, as well as a lack of a positive family history, delayed the diagnosis of hemophilia and the subsequent replacement therapy.
一名三个月大的男婴，患有未确诊的血友病，出现发热和易怒症状。在诊断评估期间，右肘前窝静脉穿刺导致出血，并引发了臂部骨筋膜室综合征。鉴别诊断包括骨髓炎和化脓性关节炎，但锝骨扫描显示仅右上肢放射性物质清除延迟，摄取未增加。尽管右臂肌肉骨筋膜室内压力升高，但手术减压推迟至获得完整的血液凝固指标后进行。在补充缺乏的血液制品后，骨筋膜室综合征未经手术减压即得到缓解。凝血功能缺陷导致血液渗入臂部肌肉骨筋膜室，致使骨筋膜室内压力显著升高。患者不寻常的临床表现以及缺乏阳性家族史，延误了血友病的诊断及后续替代治疗。
