Spinal arachnoid cysts associated with syringomyelia: report of two cases and a review of the literature

We describe two cases of spinal arachnoid cyst associated with syringomyelia and report the clinical results after surgical treatment using excision of the cyst without a shunt operation for the syringomyelia. Case 1 is a 73-year-old woman who presented with a spastic gait and numbness of her bilateral lower extremities. Magnetic resonance imaging (MRI) showed the presence of a spinal arachnoid cyst extending from T3 to T8 and syringomyelia from T8 to T10. The cyst had compressed the spinal cord anteriorly. We excised the cyst without applying a shunt tube for the syringomyelia. Case 2 is a 68-year-old woman who presented with gait disturbance and numbness of her left lower extremity. MRI indicated that the spinal cord had been compressed anteriorly by a spinal arachnoid cyst extending from T10 to T11. Syringomyelia existed just caudal to the cyst at T11. In our surgical treatment, we excised only the cyst. In both cases, neurologic examination after the operation showed amelioration of the condition. Postoperative MRI indicated that the spinal cord had moved to the center, its original position, and the syringomyelia had decreased in size. Conclusively, spinal arachnoid cyst associated with syringomyelia can be treated by simple excision of the cyst without shunting the syrinx if the decompression effect resulting from removal of the cyst is sufficient.     

A case of intraventricular cyst associated with normal pressure hydrocephalic condition

A case of intraventricular cyst associated with normal pressure hydrocephalic condition (NPHC) is reported. A 72 year-old female, with 2-year-history of slowly progressing dementia and gait disturbance, was admitted to our hospital on September 19, 1989. On admission, she had mild dementia, unsteadiness of gait, and at times urinary incontinence. Cerebrospinal fluid (CSF) pressure was found to be 90mmH2O by lumbar tap. Plain computed tomographic (CT) scan and T1-weighted magnetic resonance image (MRI) showed asymmetrical enlargement of the trigon and posterior horn of the right lateral ventricle. CT cisternography showed a cyst in the trigon and in the posterior horn of the right lateral ventricle. T1-weighted MRI with Gd-DTPA demonstrated no enhancement of the cyst wall, and there was superior and posteromedial displacement of the choroid plexus at the trigon of the right lateral ventricle. The patient was diagnosed as having an intraventricular cyst in the right trigon with NPHC. Ventriculo-peritoneal shunt and partial removal of the cyst were performed. Histological examination of the cyst wall revealed collagenous strands and no epithelial cells. Developmental intracranial cysts, especially arachnoid or ependymal cysts, occasionally lack an epithelial layer, so their histological diagnosis is difficult. This case was considered to be an arachnoid cyst because there was adhesion between the cyst and the choroid plexus in the right trigon, and superior, posteromedial displacement of the choroid plexus, which indicated extension of the cyst from the extracerebral to the intracerebral region. NPHC was considered to be due to disturbance of CSF circulation caused by gradual expansion of the cyst.     

骶管内蛛网膜囊肿的MRI表现及诊断价值

目的 探讨骶管内蛛网膜囊肿的MRI特点及其诊断价值.方法 28例骶管内蛛网膜囊肿经手术病理证实.其中其中男19例,女9例,年龄16～70岁,平均41.6岁.所有病例均行MR检查.结果 囊肿位于骶管内,呈卵圆形、不规则形、串珠形.囊肿境界清楚,囊壁薄,囊液信号与脑脊液信号相似,T1WI囊液呈低信号,T2WI囊液呈高信号,其中4例囊肿内可见细条状神经根影,6例增强扫描囊液、囊壁无强化.结论 MRI是最好的影像学诊断方法;骶管内蛛网膜囊肿的发生,主要是先天的硬膜缺陷所致.     

A case of arachnoid cyst in the middle cranial fossa presenting with trigeminal neuralgia

A 50-year-old man had been suffering from left trigeminal neuralgia for 30 years. CT scan revealed an arachnoid cyst occupying the anterior two-thirds of the left middle cranial fossa. Cerebral angiography showed elevation of the left middle cerebral artery and medial shift of the left posterior cerebral artery. Metrizamide CT showed that the cyst was not communicating with the subarachnoid space. Air CT revealed the root of the left trigeminal nerve in contact with the arachnoid cyst. The patient became completely free from the trigeminal neuralgia 6 months after cystoperitoneal shunt. This case seems to be a very rare case of trigeminal neuralgia caused by an arachnoid cyst.     

Recurrent spinal cord tethering by sacral nerve root following lipomyelomeningocele surgery. Case report

A 21-year-old woman had recurrent progressive weakness/hypesthesia and pain in both lower extremities. At the age of 5 and 19 years, she had undergone surgical resection of a lipomyelomeningocele at L5-S1. Surgical exploration revealed that the cord was tethered and pulled over to the side by an excessively short right S-1 nerve root. The contralateral L-5 and S-1 nerve roots were markedly stretched. Division of the right S-1 nerve root resulted in prompt disappearance of pain in the lower extremities and improvement in neurological function.     

Interhemispheric arachnoid cyst; report of three cases]

Three operated cases of the interhemispheric arachnoid cyst were reported. Case 1: a 58-year-old female suffering from progressive right hemi-rigidity and gait disturbance for the past two years. Case 2: a 66-year-old female was admitted with chronic headache. MRI demonstrated a large interhemispheric cyst in these two patients. Case 3: a 6-month-old male had frequent episodes of tonic seizure. MRI demonstrated interhemispheric cyst and agenesis of the anterior part of corpus callosum. The resection of the cystic wall via the interhemispheric approach was performed for all cases. General convulsive seizure developed in the early stage following operation in case 1 and case 2, in spite of routine administration of prophylactic anticonvulsant. The seizures were well controlled thereafter. In all cases, the cyst disappeared on the follow up CT. Simple resection of cyst wall is effective in the surgical treatment of interhemispheric arachnoid cysts.     

症状性骶管内囊肿的诊断与治疗

目的：探讨症状性骶管内蛛网膜囊肿的诊断与治疗方法.方法：对12例以骶管内神经受压表现为主,如腰骶部疼痛、下肢痛、会阴部疼痛,鞍区麻木不适,大小便障碍,下肢无力患者,行MRI检查.结合临床症状、体征及MRI表现明确诊断为症状性骶管内囊肿后行手术治疗.手术均在显微镜下操作.对未见明显交通孔的7例中5例行囊壁大部切除后残存囊壁修补缝扎,2例因硬脊膜缺如,无法修补而行囊肿部分切除旷置;5例有交通孔的患者中,2例囊肿大部切除后用肌肉填塞交通孔,2例因交通孔处理困难未作特殊处理,1例囊肿大部切除后,切开交通孔处硬脊膜以扩大交通孔至脑脊液通畅流出,消除交通孔的单向阀门作用.严密缝合切口,术后采取头低臀高俯卧位.结果：骶管内囊肿在MRI上表现为骶管内单发或多发类圆形或椭圆形的软组织影,呈长T1、长T2表现,信号与脑脊液相同.术后2例出现少量皮下积液,加压包扎2～3个月后自行吸收,无1例脑脊液漏.随访3个月～4年6个月,平均18.3个月,症状完全缓解8例,部分缓解3例,1例3个月后症状复发,MRI检查示囊肿较术前轻微扩大,未再次手术.结论：MRI检查是正确诊断骶管内蛛网膜囊肿的有力手段,伴有临床症状、体征者应考虑手术治疗.对囊肿的处理以囊肿大部切除为主,严密缝合切口各层及术后合理体位可以有效防止并发症的发生.     

Anterior sacral meningocele associated with tethered cord syndrome]

A case of anterior sacral meningocele associated with tethered cord syndrome is reported. A 5-year-old boy was admitted for urinary and fecal incontinence which had persisted since his birth. Abdominal MRI and sacral CT showed a presacral cystic mass communicating with the spinal dural sac through a neck traversing a sacral bony defect and tethered spinal conus. Neurological examination showed that the patient had a neurogenic bladder, atrophy of the legs and anesthesia below the S3 level. Operative finding revealed a thickened filum terminale attached to the wall of the presacral meningocele. Agenesis of the nerve root below the S2 level was identified, and no neural elements entered into the sac. Untethering was performed. Postoperative course was uneventful and the patient is doing well with his neurogenic bladder gradually improving.     

Helmetlike skull deformity with a large arachnoid cyst

BACKGROUND: It is not difficult to find localized skull ballooning or macrocrania in patients with intracranial arachnoid cysts. However, there have been no previous reports regarding large localized skull protuberant deformities resembling a war helmet. The authors report with a review of literature a case of an adult with helmetlike skull deformity resulting from a large supratentorial arachnoid cyst. CASE DESCRIPTION: A 35-year-old man presented with a large head deformity since his early childhood that had been the result of gradual progression from infantile macrocrania. He also had mental retardation, sixth cranial nerve palsy with recent aggravation of headache, reduced activity, poor voiding control, and walking disturbance. Magnetic resonance imaging of the head showed hydrocephalus with a large supratentorial arachnoid cyst located in the bilateral parietooccipital area compressing the hemisphere anteriorly, and the tentorium and cerebellum inferiorly. Magnetic resonance venogram demonstrated low-lying short transverse and lateral sinuses, and the superior sagittal sinus and falx were displaced to the right side. Radioisotopic cisternogram showed nonfilling of the isotope in the bilateral parietooccipital area. Cerebrospinal fluid pressure measured by lumbar puncture was 17 cm H(2)O. We tentatively diagnosed the condition as normopressure hydrocephalus with a large supratentorial arachnoid cyst. His headache, reduced activity, poor voiding control, and walking disturbance improved after a cystoperitoneal shunt. CONCLUSIONS: This might suggest that large arachnoid cysts found in childhood should be treated for prevention of skull deformity and late aggravation of increased intracranial pressure.     

骶神经根周围囊肿的诊断及外科治疗

目的 探讨骶神经根周围囊肿的临床表现、影像学特点及外科治疗效果。方法 诊断22例骶管内神经根周围囊肿患者。对有明显症状的16例进行手术治疗。行病变部位后路椎板减压，13例囊肿切除，3例囊肿大部分切除，9例硬膜破裂神经根裸露采用胶原蛋白海绵覆盖。结果 MRI检查显示出椎管内神经根囊肿的形态，CT显示骶骨压迫性改变。16例切口均一期愈合，术后随访6个月～7年，平均2年9个月，患者术后腰骶疼痛、间歇跛行、骶神经症状均得到明显缓解，无感染病例，2例脑脊液漏1周内愈合。结论 骶管内神经周围囊肿临床上少见，容易误诊。MRI是最好的诊断方法，采用手术摘除囊肿和胶原蛋白海绵覆盖治疗，其治疗效果满意。     

An intradural arachnoid cyst of the craniovertebral junction; a case report]

An intradural arachnoid cyst of the craniovertebral junction possibly of traumatic origin is reported. A 59-year-old man was admitted to our hospital with a 10-month history of progressive gait disturbance. He had a history of head injury with a fracture of the occipital bone. Myelography revealed pooling of the contrast medium in the posterior fossa and on the dorsal sides of C1 and C2. Metrizamide-enhanced computed tomography also showed pooling at the same level. Magnetic resonance imaging indicated a large cystic lesion at the craniovertebral junction. Craniectomy of the posterior fossa and laminectomy of C1, C2 and C3 were performed, and an intradural cyst with thickened dura and arachnoid was found. The cyst wall was opened to communicate with the subarachnoid space. Histological findings of the specimen showed that the arachnoid was thickened. There are over 130 reports of intradural arachnoid cyst of the spine, but those of traumatic origin are rare, and cysts located in the intracranial to spinal region are extremely rare.     

Surgical treatment of progressive visual loss in traumatic optic neuropathy. Report of two cases

In two patients with traumatic optic neuropathy progressive visual loss was reversed by surgical decompression of the optic nerve sheath. The first patient with hemorrhage beneath the optic nerve sheath had progressive loss of vision from counting fingers to no light perception within 24 hours after the injury. Surgical evacuation of the hematoma improved visual acuity to 8/30. The second patient had progressive visual loss from 20/20 to 20/400 within the 1st week after injury. Drainage of an arachnoid cyst of the optic nerve sheath improved visual acuity to 20/25. Computerized axial tomography disclosed the hemorrhage in the first case and enlargement of the optic nerve sheath in the second. While the management of traumatic optic neuropathy is controversial, surgical intervention for an arachnoid cyst and hematoma involving the optic nerve is clearly beneficial.     

骶管内囊肿的临床研究

目的探讨骶管内囊肿的发病机理、临床特点及治疗方法。方法回顾性分析35例手术治疗的骶管内囊肿的临床资料，根据术前的影像学检查、术中所见、术后病理和手术后疗效，分析其特点。结果28例随访结果发现，囊壁部分切除治疗骶管内囊肿的疗效满意，随访中未发现囊肿复发和神经损伤再加重。结论骶管内囊肿是相对较常见的疾病，该病尤其是在40岁以上人群中发病较多，与退变性腰椎疾病的表现有很大的相似处。本病最好的诊断方法是行腰骶椎MRI检查。治疗上采用手术简单的囊壁部分切除具有较好的疗效。     

Spinal arachnoid cysts in the pediatric age group: an association with neural tube defects.

Between 1979 and 1991, spinal arachnoid cysts were found in 11 patients aged 19 months to 18 years (mean age 5 1/2 years). Of the 11 patients, six had a myelomeningocele and one diastematomyelia. The presenting symptoms included radicular pain (one patient), progressive weakness (three), increasing scoliosis (one), worsening spasticity (three), and recurrent urinary tract infections and progressive constipation (one). Two patients showed no symptoms from the spinal arachnoid cyst. The distribution of lesions was as follows: cervicomedullary (one patient), cervical (one), cervicothoracic (two), thoracic (four), lumbar (two), and sacral (one). Four of the 11 arachnoid cysts (all intradural) were located anterior to the spinal cord, three of which were in children with a myelomeningocele. Only two of the cysts were extradural; both were found in the lumbosacral region, and one was associated with diastematomyelia. Eight patients were treated with fenestration and/or resection of the cyst wall. Three patients with anterior cysts were treated with shunts, a cyst-to-pleural space shunt in two and a cyst-to-subarachnoid space shunt in one. All of the patients either improved or exhibited an arrest in the progression of their symptoms. Spinal arachnoid cysts are a treatable cause of progressive neurological deficits and, in this series, were frequently found in patients with neural tube defects.     

Extradural spinal arachnoid cysts associated with spina bifida occulta

Summary Introduction. Spinal extradural arachnoid cysts are an uncommon cause of neural compression in children. Even more uncommon is the association of such cysts with spina bifida occulta. Material. Two girls, 12 and 8-years-old, presented with left leg pain, deteriorating gait, clinical signs of left L5 and S1 root compression, without bladder or bowel symptoms. The first patient had left foot drop. The second patient had muscle wasting and smaller left foot with pes cavus. Radiographs showed spina bifida occulta of S1 in both. MRI revealed an extradural cyst at the S1 level, indenting the thecal sac and the L5 and S1 roots. At operation in both patients a large arachnoid cyst arising from a small dural defect in the axilla of the left S1 root was compressing and displacing it and the dural sac. It was removed and the defect was repaired. The first patient improved with complete recovery of the foot drop. An MRI at 12 months showed no cyst recurrence. The second patient made good recovery initially, but at 10 months developed recurrent symptoms. An MRI scan showed recurrence of the cyst with root compression. On repeat exploration a different dural defect was identified in a more anterior position and was repaired. Discussion. The coexistence of extradural arachnoid cyst and corresponding bifid spinal segment has not been described previously. It raises the suspicion that the dural defect giving rise to the arachnoid cyst may be due to segmental dural dysgenesis in the context of the dysrhaphic neuroectodermal malformation.     

A case of sacral meningeal cyst with Marfan syndrome]

A rare case of sacral meningeal cyst Marfan syndrome is reported. A 40-year-old male who had a past history of Marfan syndrome was admitted to Tokyo Metropolitan Ebara Hospital due to sensory disturbance in the right S1-2 nerve root area on October 1998. On admission, neurological deficits were sensory disturbance and decrease right Achilles reflex. Plain sacral X-ray was normal. Magnetic resonance imaging revealed a cystic mass in the intrasacral space. MR myelography showed the cyst followed the thecal sac. Signal intensity of the cystic mass was the same as the cerebrospinal fluid. In the prone position, laminectomy of the sacrum was carried out. The neck of the meningeal cyst was ligated with the help of an aneurysmal needle. The patient's sensory disturbance disappeared postoperatively. As MRI becomes more frequently used in Marfan syndrome, it is important to keep the presence of such an entity in mind. MR myelography made it easy to diagnose the meningeal cyst.     

骶管内蛛网膜囊肿的外科治疗

目的 :探讨骶管内蛛网膜囊肿的诊断及治疗方法。方法 :2 3例骶管内蛛网膜囊肿病人行椎板减压后 ,对囊肿的处理有 3种方法 :13例行囊肿大部分切除后 ,重新缝合残余的囊肿以包绕囊内神经根 ;8例囊肿大部分切除后用肌肉填塞交通孔 ;2例囊肿切开旷置。平均随访 3 0 2个月 ,观察疗效。并对其临床表现 ,手术中的病理特点 ,手术后的并发症 ,以及X线片 ,CT ,CTM ,MRI等影像学资料进行分析。结果 :MRI可清楚显示囊肿呈长T1及T2信号 ,信号强度与脑脊液一致。临床症状以骶管内神经受压表现为主 ,囊肿与硬膜囊一般有交通孔。囊肿切除后重新缝合包绕神经组与囊肿切除肌肉填塞组优良率并无统计学差异。术后并发症有皮肤糜烂和颅内感染。结论 :MRI是最好的影像学诊断方法 ;骶管内蛛网膜囊肿的发生是由于先天的硬膜缺隐所致。对囊肿的处理以囊肿切除 ,肌肉填塞封堵交通孔最为合理。术后不宜放引流及平卧。     

Sacral meningeal cyst associated with valve-like mechanism--case report

A 58-year-old woman presented with low back pain radiating to the lower extremities. Magnetic resonance imaging revealed a cystic lesion in the sacrum compressing the nerve roots. At operation, a valve-like communication was found between the subarachnoid space and the cyst cavity in the vicinity of the sacral nerve root. The communication was obliterated with a purse-string suture and reinforced with a free muscle graft. Postoperatively, she reported improvement of the pain. Valve-like communication between the cyst cavity and subarachnoid space can cause enlargement of spinal meningeal cyst, and could also explain enlargement of sacral meningeal cyst. Surgical obliteration of the communication rather than the cyst resection is more important for sacral meningeal cyst.     

Sacral cyst managed with cyst-subarachnoid shunt: a technical case report

STUDY DESIGN: This report describes the cyst-subarachnoid shunt, a novel surgical treatment, for sacral cysts. OBJECTIVE: To introduce a new surgical technique for sacral cysts. SUMMARY OF BACKGROUND DATA: There is no consensus on the appropriate treatment for symptomatic sacral cysts. The hydrostatic and pulsatile forces of cerebrospinal fluid are attributed to the growth of the cyst and their becoming symptomatic. METHODS: The clinical and radiologic features of a 41-year-old man with a symptomatic sacral cyst are detailed. A cyst-subarachnoid shunt was set to equalize the cerebrospinal fluid pressure between the cephalad thecal sac and the cyst. RESULTS: Immediately after surgery, the patient had no pain in his left leg and was free of pain at 2 years. Magnetic resonance imaging 1 year after surgery showed a decrease in the size of the cyst. CONCLUSION: Although this is a preliminary study, a cyst-subarachnoid shunt can be a useful alternative for symptomatic sacral cysts.     

骶管内囊肿的诊断及其发生机制的探讨

目的：探讨骶管内蛛网膜囊肿的影像学特点发生机制。方法：观察24例手术证实的骶管内蛛网膜囊肿病人的X线片、CT、造影后CT、MRI等影像资料，分析其临床特点及术中的病理特点。结果：5例X线片显示骶骨侵蚀性改变，5例CT显示骶骨侵蚀性改变及骶管内囊肿，1例造影后CT囊肿内造影剂显影，22例MRI显示囊肿呈长T1及T2信号，信号强度与脑脊液一致。临床症状以骶管内神经受压表现为主，囊肿与硬膜囊一般有交通孔。交通孔为瓣膜样。结论：MRI是最好的影像学诊断方法；骶管内蛛网膜囊肿的发生是由于先天的硬膜缺陷所致；瓣膜样交通孔是病程进展的结果。