Yeh Yu-Chaing, Lin Ya-Jui, Yeh Chih-Hua, Hsieh Pao-Shiu, Wu Chieh-Tsai
Department of Neurosurgery, Chang-Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan City, Taiwan.
The Graduate Institute of Biomedical Sciences and.
J Neurosurg Case Lessons. 2021 May 17;1(20):CASE20159. doi: 10.3171/CASE20159.
Marfan syndrome is rarely accompanied by anterior sacral meningocele (ASM) resulting from erosion of the sacrum by dural ectasia. ASM may induce symptoms due to severe mass effects. ASM may also mimic ovarian cysts, and the risk of cerebrospinal fluid (CSF) leakage is high if spontaneous rupture of the cyst occurs. In this study, the authors presented a rare case of ASM with iatrogenic CSF leakage in a 34-year-old woman with suspected Marfan syndrome.
The patient initially presented with a giant ASM that was first misdiagnosed as an ovarian cyst. Previously, it had been partially resected, which was followed by iatrogenic CSF leakage. Symptoms of intracranial hypotension, including postural headache and dizziness, developed within 1 month. Brain magnetic resonance imaging (MRI) showed pituitary enlargement, bilateral subdural effusion, and tonsillar herniation. Preoperative computed tomography myelography provided three-dimensional (3D) examination of the deformed sacrum and CSF leakage site. Transabdominal approaches led to primary repair, and repair of the meningocele was achieved by intraoperative fluorescein fluorescence and 3D printed model-guided polymethyl methacrylate bone cement reconstruction. No CSF leakage or recurrent ASM was found at the 1.5-year follow-up visit.
Intraoperative intrathecal fluorescence and 3D-printed models are useful for ASM repair. Preoperative MRI is helpful for differentiating ASM from other causes of a huge pelvic mass, including ovarian cyst.
马方综合征很少伴有因硬脊膜扩张侵蚀骶骨导致的骶前脑脊膜膨出(ASM)。ASM可能因严重的占位效应而引发症状。ASM也可能类似卵巢囊肿,如果囊肿自发破裂,脑脊液(CSF)漏出的风险很高。在本研究中,作者报告了一例34岁疑似马方综合征的女性患者发生ASM并伴有医源性脑脊液漏的罕见病例。
患者最初表现为巨大的ASM,最初被误诊为卵巢囊肿。此前曾进行过部分切除,随后出现医源性脑脊液漏。在1个月内出现了颅内低压症状,包括体位性头痛和头晕。脑部磁共振成像(MRI)显示垂体增大、双侧硬膜下积液和扁桃体疝。术前计算机断层扫描脊髓造影对变形的骶骨和脑脊液漏出部位进行了三维(3D)检查。经腹入路进行了初次修复,通过术中荧光素荧光和3D打印模型引导的聚甲基丙烯酸甲酯骨水泥重建实现了脑脊膜膨出的修复。在1.5年的随访中未发现脑脊液漏或ASM复发。
术中鞘内荧光和3D打印模型对ASM修复有用。术前MRI有助于将ASM与其他导致巨大盆腔肿块的病因区分开来,包括卵巢囊肿。