Department of Surgery, University of Hawaii, John A. Burns School of Medicine; and.
Department of Surgery, Kaiser Permanente Moanalua Medical Center, Honolulu, Hawaii.
J Neurosurg Spine. 2015 Jul;23(1):77-80. doi: 10.3171/2014.11.SPINE131086. Epub 2015 Apr 24.
Defective collagen biosynthesis in Marfan syndrome predisposes to dural defects such as dural ectasia, meningocele, and pseudomeningocele; thus, an increased index of suspicion for these conditions should be present in the clinical setting of Marfan syndrome. The authors describe a young woman with Marfan syndrome who was being treated with anticoagulants for a prosthetic heart valve and who presented with a spontaneous retroperitoneal hemorrhage requiring surgical evacuation. No CSF leak was encountered at surgery, but she developed progressively more severe positional headaches over the following year. She then experienced the sudden onset of acute urinary obstruction, at which time CT revealed a 17 × 15 × 13-cm presacral pseudomeningocele communicating with the thecal sac through a sacral bone defect. An anterior surgical approach was used for drainage of the pseudomeningocele as well as for primary closure of the dural defect with a bovine pericardial patch and autologous subcutaneous fat graft. After a short period of lumbar subarachnoid drainage of the CSF, the patient was able to resume normal activity without recurrent symptoms. To the authors' knowledge, such a pseudomeningocele in a patient with Marfan syndrome has been reported only twice, and this case features the largest pseudomeningocele to date. They also review the pertinent literature regarding presentation, diagnosis, and management of these lesions.
马凡综合征患者胶原生物合成缺陷易导致硬脑膜缺陷，如硬脑膜扩张、脑脊膜膨出和假性脑脊膜膨出；因此，在马凡综合征的临床环境中，应该对这些情况有更高的怀疑指数。作者描述了一位患有马凡综合征的年轻女性，她因人工心脏瓣膜正在接受抗凝治疗，因自发性腹膜后出血需要手术清除。手术时未发现脑脊液漏，但在接下来的一年中，她逐渐出现更严重的体位性头痛。然后，她突然出现急性尿梗阻，此时 CT 显示 17×15×13 厘米的骶前假性脑脊膜膨出通过骶骨缺损与脊膜囊相通。采用前路手术引流假性脑脊膜膨出，并使用牛心包补片和自体皮下脂肪移植物对硬脑膜缺损进行一期修补。在进行短暂的腰蛛网膜下腔脑脊液引流后，患者能够恢复正常活动，无复发症状。据作者所知，马凡综合征患者的这种假性脑脊膜膨出仅报告过两次，而本例是迄今为止最大的假性脑脊膜膨出。他们还回顾了关于这些病变的表现、诊断和治疗的相关文献。
