Intraspinal Space Research Articles

SPINAL INTRADURAL EXTRAMEDULLARY TUMORS AND SIMILAR PATHOLOGIES: A SINGLE CENTER STUDY

Background: We wanted to analyze the neurological symptoms and prognosis of five spinal IDEM (intradural extramedullary) tumors cases diagnosed, and to emphasize that a spinal cord tumor can be found incidentally in evoluation of the proximal regions of the vertebral column in patients who were mistakenly operated for lumbar disc herniation on lumbar MRI, but whosesymptoms did not resolve. Objective: To emphasize the need for examinations such as MRI along the vertebral columna in patients with different spinal pathologies and whose symptoms and clinical signs do not improve. Methods: Patients with spinal IDEM tumors treated with surgical excision and followed for more than 2 years were retrospectively analyzed. Issessed pain with the visual analo scale (VAS), and neurological function with the Nurrick grade.Preoperative complaint time,location of tumor in sagital and axial sections in MRI and pathological diagnosis were investigated. Results: The pathological diagnosis in our study was meningioma in two cases,dural arteriovenous fistula in one case,teratoma in one case ,and myxopapilary epandimoma in one case.İn one case,disc herniation surgery was performed in another hospital due to misdiagnosis, but because the patient’s complaints were resistant. In examination, an IDEM tumor at T11 level was detected, and he was operated. In all cases the VAS score (mean) was reduced from 7.8 to 0.4 as well as the Nurrick grade (mean) was from 2.0 to 0.6. In the dAVF case, spasticity in the lower extremities, bladder and bowel disfunction continued. Conclusion: In cases with severe neurological deficits, MRI scanning of the proximal parts of the vertebral column will help in the differential diagnosis of the lesion occupying the intraspinal space.

Hamman syndrome with a rare feature of pneumorrhachis – an unusual complication of polysubstance abuse and e-vaping

Hamman syndrome is defined as dissection of air in mediastinum and skin fascia usually due to increased intrathoracic pressure. The air leak tends to make its way into pleural and pericardial layers; however, in rare instances air can also dissect into epidural spaces, regarded as pneumorrhachis. We present a case of a young male with a history of polysubstance abuse and e-vaping, who presented with symptoms of altered mental status. Given the concerning physical examination, a computed tomography of the chest was undertaken, which showed pneumothorax, pneumomediastinum and pneumorrhachis. The patient was closely monitored in the intensive care unit and improved after symptomatic management. The symptoms of pneumorrhachis depend on the volume and location of air in intracranial and intraspinal space. Although asymptomatic in our case, it is crucial for clinicians to be aware that pneumorrhachis with Hamman syndrome can potentially cause neurological deficits and cardiopulmonary arrest in severe cases due to increased intraspinal and intracranial hypertension, emphasising the need for close monitoring.

High-viscosity versus low-viscosity cement for the treatment of vertebral compression fractures: A meta-analysis of randomized controlled trials.

High viscosity cement (HVC) and low viscosity cement (LVC) have been used to treat osteoporotic vertebral compression fractures (OVCFs). Our study was to assess the safety and efficacy of HVC and LVC in treating OVCFs. We searched the electronic database for randomized controlled trials of HVC and LVC to treat OVCFs. Random-effects model was performed to pool the outcomes about operation time, visual analogue scale (VAS), bone cement injection volume, oswestry disability index (ODI), bone cement leakage and adjacent vertebral fractures. Twelve randomized trials were included in the meta-analysis. The 2 groups had similar changes in terms of bone cement injection volume, ODI and adjacent vertebral fractures. The HVC group showed shorter operation time and better VAS score improvement. The bone cement leakage rate of the HVC group was significantly better than LVC group (P < .00001).According to the location of bone cement leakage, in the leakages of the veins (P < .00001), the intervertebral disc (P < .00001), the paravertebral area (P = .003) and the intraspinal space (P = .03), the HVC group were significantly better than the LVC group. In terms of bone cement injection volume, ODI and adjacent vertebral fractures, the 2 group are equivalent. HVC had a shorter operation time, lower bone cement leakage rate and better VAS score improvement, compared with LVC.

High- versus low-viscosity cement vertebroplasty and kyphoplasty for osteoporotic vertebral compression fracture: a meta-analysis.

To compare high- versus low-viscosity bone cement on the clinical outcomes and complications in patients with Osteoporotic vertebral compression fractures (OVCFs) who underwent percutaneous vertebroplasty (PVP) or percutaneous kyphoplasty (PKP). PubMed, Embase, and the Cochrane Library were searched for papers published from inception up to February 2021 for potentially eligible studies comparing high- versus low-viscosity cement for PVP/PKP. The outcomes were the leakage rate, visual analog scale (VAS), and Oswestry Disability Index (ODI). Eight studies (558 patients; 279 in each group) were included. The meta-analysis showed that the leakage rate was lower with high-viscosity cement than with low-viscosity cement (OR = 0.23, 95%CI 0.14-0.39, P < 0.001; I2 = 43.5%, Pheterogeneity = 0.088); similar results were observed specifically for the disk space, paravertebral space, and peripheral vein, but there were no differences regarding the epidural space and intraspinal space. The VAS was decreased more significantly with high-viscosity cement than with low-viscosity cement (WMD = - 0.21, 95%CI - 0.38, - 0.04, P = 0.015; I2 = 0.0%, Pheterogeneity = 0.565). Regarding the ODI, there was no difference between high- and low-viscosity cement (WMD = - 0.88, 95%CI - 3.06, 1.29, P = 0.426; I2 = 78.3%, Pheterogeneity < 0.001). There were lower cement leakage rates in PVP/PKP with high-viscosity bone cement than low-viscosity bone cement. The two groups have similar results in ODI, but the VAS scores favor high-viscosity bone cement. Therefore, the administration of high-viscosity bone cement in PVP/ PKP could be a potential option for improving the complications of leakage in OVCFs, while the clinical efficacy of relieving pain is not certain.

Neurenteric Cyst at Craniovertebral Junction: An Uncommon Presentation

Neurenteric cysts, also known as endodermal cysts and enterogenous cysts, are rare benign cysts. Neurenteric cysts may be associated with congenital malformations, such as spinal dysraphism, or may occur as solitary lesions. Most are located in the spine, in the intradural, extramedullary and intraspinal space. Rare examples may occur intracranially, and rarer examples may occur at the craniovertebral junction. Clinically, solitary neurenteric cysts present with mass effect symptoms such as compression of spinal cord and adjacent nerve roots. We present a case of a 28-year-old male patient with a histologically confirmed neurenteric cyst located at the cervicomedullary or craniovertebral junction with presenting symptoms of neck pain and severe debilitating weakness of all four limbs. The patient underwent successful surgical resection, with gradual improvement in his symptoms. We also present a review of literature, including the clinical features, histological characteristics, and management options for patients with neurenteric cyst in the craniovertebral junction.

Tumour collection chamber for space-occupying lesions in neurosurgery

Collection of tissue sample is one of the most important step in neurosurgery while removing intracranial or intraspinal space occupying lesions (SOL). It is required for tissue diagnosis, further treatment planning and prognostication. There is no mention of any tool except taking pieces using various biopsy/holding forceps or curettes. Author has designed a prototype of a tumour collection chamber (TCC), which is connected to the suction tubing for collection of solid tumours during surgical removal by suction by selectively allowing the suction flow through the TCC. It is easy to use as and when the tumour is being removed and suction flow can be bypassed by the surgeon or the assistant once tumour removal is done or otherwise.

RETROSPECTIVE ANALYSIS OF INTRACRANIAL AND INTRASPINAL SPACE OCCUPYING LESIONS AT A TERTIARY CARE CENTER- A FIVE YEAR STUDY

Background: Tumors of Central Nervous System constitute approximately 2% of all malignancies. Cancers of the central nervous system (CNS) are considered to be among the most notorious of all cancers. Low-grade cns tumors have been found over time to progress to high grade tumors. CNS is a common site for metastasis from other organs. Due to newer techniques used in radio diagnosis and stereotactic biopsies, more &amp; more CNS tumors are being diagnosed &amp; are being operated upon.&#x0D; Aims and objectives: The objective of this article is to provide an overview of intracranial and intraspinal space occupying lesions at a single tertiary care referral center. The aim was to study incidence of various lesions in light of the WHO classification of tumours of the central nervous system, 2016 and to study relevant statistics.&#x0D; Material and methods: A total of 124 cases were received between January,2007 &amp; December, 2011 at Bharati Vidyapeeth Deemed University Medical College and Hospital, Pune. Intraoperative diagnosis was desired in 70 cases. The final diagnoses in all the cases were made on Hematoxylin and Eosin stained slides of routinely processed tissue.&#x0D; Results: Neoplastic lesions comprised 83.88 %, including metastatic tumors while 16.12 % were nonneoplastic. A wide range of histopathological spectrum of CNS tumors was observed and was classified according to WHO classification of CNS tumors, 2016. The primary CNS tumors were graded from Grade I to IV according to WHO grading system, 2016. Astrocytic tumors constituted the largest category with 33 cases and most of the astrocytomas were grade II at the time of diagnosis. Incidence was more in females than males and maximum number of lesions were seen in fifth and sixth decades of life.&#x0D; Conclusion: This study highlights the histological diversity of CNS tumors in both adults and children. The most common destructive but nonneoplastic lesions like, infections/abscesses, infarct and cysts can be diagnosed, by crush cytology and frozen section for the definitive management.

Cervical laminoplastic in patients with cerebro-spinal cord injury

Cervical laminoplasty is a surgical method of treating the stenosis of the spinal canal in the cervical region, which increases the intraspinal space and allows preserving the integrity of the posterior elements of the vertebrae.Purpose: The aim of the study was to investigate the efficacy and develop the treatment tactics for patients with posttraumatic cervical spondylotic myelopathy due to stenosis of the spinal canal.Materials and methods. On treatment in Dnepropetrovsk Regional Clinical Hospital after named I.I. Mechnikov , from April 2014 to February 2018 there were 7 patients with posttraumatic cervical spondylotic myelopathy due to stenosis of the spinal canal. The age of the patients was 54-68 years. To assess the effectiveness of the treatment, the ASIA neurological disorders scale was used. The degree of severity of neurological disorders of patients corresponded to C-D. All patients underwent standard SCT and MRI study.Results of the study. In the early postoperative period, 6 patients showed improvement in their neurological status. Patients of group C - 2 observations (before surgery, muscle strength in the limbs was below 3 points), recovered to category D (muscular strength was more than 3 points). In patients included in the category D - 4 examinations (before surgery, muscle strength was more than 3 points, but did not reach 5 points), there was a complete restoration of muscular strength in the limbs - 5 points. One patient in Group D did not have a neurological recovery.Conclusions:1. The operation is organ-preserving and after the operation the spinal cord remains covered with a bone tissue behind it, which prevents the formation of scar tissue directly on the dura mater.2. Decompression of the spinal cord is achieved without significant disruption of stability, thus reducing the likelihood of postoperative kyphotic deformity and spondylolisthesis.3. Laminoplasty does not require subsequent spondylodesis, movement remains in the segment, in contrast to the methods that include the installation of the fixation structure, in this connection, the severity of degeneration at adjacent levels may decrease.4. Carrying out decompressive laminoplasty in patients with traumatic myelopathy, most effective in the early days after trauma, the first 7 days

Comparison of high- and low-viscosity cement in the treatment of vertebral compression fractures

Background:High-viscosity cement (HVC) has been gradually applied in percutaneous vertebroplasty (PVP) and percutaneous kyphoplasty (PKP). Although HVC has been reported to reduce cement leakage, different opinions exist. To assess the complications of HVC in cement leakage in the treatment of vertebral compression fractures and to evaluate the clinical effect of HVC compared with low-viscosity cement (LVC).Methods:EMBASE, PubMed, Science Direct, Google Scholar and Cochrane Library databases were comprehensively searched from their inception to August 2017. Two researchers independently searched for articles and reviewed all retrieved studies. Forest plots were used to illustrate the results. The Q-test and I2 statistic were employed to evaluate between-study heterogeneity. Potential publication bias was assessed by funnel plot.Results:HVC reduced the occurrence of cement leakage (risk ratio (RR) = 0.38, 95% confidence interval (CI) = 0.29 to 0.51, P < 0.00001), especially in the disc space (RR = 0.45, 95% CI = 0.45 to 0.80, P = 0.007) and the vein (RR = 0.54, 95% CI = 0.35 to 0.85, P = 0.008) but not in the intraspinal space (RR = 0.48, 95% CI = 0.19 to 1.23, P = 0.13) or the paravertebral area (RR = 0.63, 95% CI = 0.32 to 1.22, P = 0.17). No significant differences in the visual analogue scale (VAS), Oswestry Disability Index (ODI), injected cement volume or adjacent vertebral fracture were noted between HVC and LVC (P > 0.05).Conclusion:Compared with LVC, HVC results in a reduced incidence of cement leakage for the treatment of vertebral compression fractures, especially in the disc space and vein but not in the intraspinal space or the paravertebral area. In addition, HVC yields the same satisfactory clinical effect as LVC.

Spinal Epidural Cavernous Hemangioma: A Clinical Series of 7 Patients.

To investigate imaging characteristics of spinal epidural cavernous hemangiomas (ECHs) and improve understanding of this rare disease. This retrospective study included 7 cases of ECH, confirmed with surgical pathology and imaging. All patients underwent computed tomography and conventional magnetic resonance imaging. One patient underwent diffusion-weighted imaging. ECH originated from vertebrae with coarsened trabeculae in 5 patients. Among these, 1 tumor was mainly in the intervertebral foramen and paravertebral space, 2 extended to the intraspinal and paravertebral spaces and appeared dumbbell-shaped, and 2 were located in the intraspinal space. Purely epidural hemangiomas were present in 2 patients; 1 of these patients had adjacent bony erosion. Epidural hemangiomas were isointense in 6 patients and hyperintense in 1 patient on T1-weighted images; lesions were hyperintense on T2-weighted images in all 7 patients. Peripheral fat was present in 4 ECHs; 2 were of vertebral origin, and 2 were of epidural space origin. In the patient who underwent diffusion-weighted imaging, the lesion showed restricted diffusion. The affected vertebra had coarsened trabeculae, which may be a critical finding for differentiating ECHs of vertebral origin from foraminal nerve sheath tumors. Peripheral fat around hemangiomas of vertebral origin and primary epidural space hemangiomas indicates chronicity. ECHs are classified either as epidural hemangiomas of vertebral origin or as primary epidural hemangiomas. Awareness of imaging characteristics of ECHs may facilitate diagnosis and treatment of these lesions.

An updated comparison of high- and low-viscosity cement vertebroplasty in the treatment of osteoporotic thoracolumbar vertebral compression fractures: A retrospective cohort study

ObjectiveThis study mainly aimed to evaluate complications of cement leakage for osteoporotic thoracolumbar vertebral compression fractures by PVP using HVC, and access the clinical efficacy. MethodsBetween May 2013 and June 2015, 66 patients with osteoporotic thoracolumbar vertebral compression fractures, who underwent PVP (36 HVC and 30 LVC) in our hospital, were enrolled. Cement leakage, Visual Analog Scale (VAS), Oswestry Disability Index (ODI), refracture of the cemented vertebrae, and adjacent vertebral fractures were evaluated. The follow-up time was 1 year. ResultsThe overall cement leakage rate was 30.55% in the HVC group, lower than 77.77% obtained in the LVC group (P = 0.00). The incidence rates of cement leakage into paravertebral area (P = 0.02) and vein (P = 0.04) in the HVC group were significantly lower than those of the LVC group; however, no differences were found for disc space (P = 0.72) and intraspinal space (P = 0.58). There were no differences in VAS, ODI, refracture of cemented vertebrae, and adjacent vertebral fracture between the two groups (P > 0.05). ConclusionsPVP using HVC not only can reduce cement leakage, especially in the paravertebral area and peripheral vein, but also has satisfactory clinical effect.

The Role of Minimal Invasive Split Laminotomy, the Alternative Parasplit Approach and the “Arch bone” Technique in Surgical Removal of Axial Longitudinal Pathologies in the Spinal Canal

Introduction Conventional approaches to open the spinal canal carries the risk of spinal deformities, instability, subluxation, and invariably separate the muscle attachments from the spinous processes and laminae. Damage to these muscles and bony connections can lead to persistent axial pain and cervical malalignment. With the aim of preserving and reconstructing the posterior structures, the authors developed a novel minimal invasive multilevel spinous process splitting and distraction laminotomy approach, the “arch bone” and the additional rescue parasplit technique. Patients and Methods These novel techniques were used in 70 adult patients with mainly midline located intramedullary pathologies of the cervical, thoracic, and thoracolumbar spine. All muscle attachments on the spinous processes and laminas, as well as the laminas themselves, were completely preserved. With the aim of moderate enlargement of the spinal canal, a spacer was placed between the bony parts facing each other in case of total resection of an intramedullary tumor was not possible. When midline splitting is not feasible or convenient because of anatomical differences of the spinous processes and laminas, we used the parasplit approach. The patients were followed with regular MRI, CT scans, fluoroscopy, and neurological examinations. Results The midline opening of the spinal canal gave a key-hole approach, achieving an adequate view of the intraspinal space for surgery in its entire longitudinal extension. The approach used did not affect the extent of resection proved by MRI evaluation or neurological outcome. The numbers of splitted laminae were 3 to 10. The incidence of postoperative local pain was lower, within acceptable limits (VAS: 2–4). The average length of hospital stay was 6.7 days. Average follow-up was 58 months. Instability and deformity was detected in none of the patients on the flexion–extension lateral radiographs during the follow-up period. Conclusion These surgical approaches suitable for exploring and removing different intramedullary pathologies, help in preventing damage to crucial posterior stabilizers of the spine. Its major advantage is that unnecessary exposure and tissue trauma is reduced, and structures not directly involved in the pathologic process are preserved. In contrast to conventional spinal canal approaches, leaves the muscle attachments intact, disintegration of vertebral arches and facet joints is reduced. Furthermore, these techniques are suitable methods for all spinal segments in all age group. Although the clinical and radiological results are very promising, the limited follow-up period precludes conclusions regarding the long-term results of the procedure, especially with respect to kyphotic deformity.

Chronic dura erosion and intradural lumbar disc herniation: CT and MR imaging and intraoperative photographs of a transdural sequestrectomy

A 47-year-old male with a history of recurrent low-back pain presented with acute left radiculopathy. The CT and MR scans showed a severe osteochondrosis of the L4/5 segment, a broad protrusion of the disc annulus and extrusion of nucleus material into the spinal canal on the left side. The caudally dislocated sequester pieces were visualised intradurally and the intraoperative finding confirmed this rare pathology. After dorsal durotomy-free sequester material was found between the nerve rootlets within the subarachnoid space and altogether ten fragments were removed. Further transdural exploration visualised ventrally a round dura defect surrounded by a thickened arachnoid layer with enlarged veins as a sign of a chronic erosive process. Patients history, imaging and the intraoperative findings support the thesis, that chronic degenerative disc disease and adhesions between the posterior longitudinal ligament and the dura are the predisposing pathogenetic factors for an intradural disc herniation. Intradural disc herniation is a rare condition and requires durotomy to remove the pathology. Therefore an actual high resolution MRI is mandatory in all cases of intraspinal space occupying lesions.

Surgical outcome of video-assisted thoracic surgery for acute thoracic empyema using pulsed lavage irrigation

The essential points of video-assisted thoracic surgery (VATS) for acute thoracic empyema are the decortication of thickened pleura, resection of necrotic tissues and fibrin blocks, and drainage. Pulsed lavage irrigation, which is commonly used in orthopedic surgery as a method of sufficiently performing the technique, was used under a thoracoscope to study the efficacy of the treatment for acute thoracic empyema. The subjects comprised 31 patients who had undergone VATS for acute thoracic empyema. There were 26 men and 5 women with an average age of 60.5 years. For the surgical technique, the thickened pus-producing pleura were decorticated under a thoracoscope. The pulsed lavage irrigation system was used after the intrathoracic space had become a single cavity. Using the tip for an intraspinal space, lavage and suctioning were repeated with 5-10 l of a pressurized warm saline solution. Fibrin blocks and necrotic tissues were easily removed by spray washing with pressurized fluid. The operating time was 150.8 min; the amount of bleeding, including suctioned pleural effusion, was 478.5 g; and the postoperative duration of drainage was 10.7 days. During the postoperative course, the addition of open window thoracotomy due to the relapse of empyema due to methicillin-resistant Staphylococcus aureus was observed in only one patient (3.2%). All of the other patients improved despite their concomitant diseases. The use of pulsed lavage irrigation under a thoracoscope for acute thoracic empyema provides simple, efficient débridement or drainage.

Management of intramedullary spinal cord abscess: experience with four cases, pathophysiology and outcomes

Intramedullary spinal cord abscess (ISCA) in children is extremely a rare infection of the central nervous system (CNS); and probably a devastating neurological condition. Clinical awareness of patients at risk is crucial for early diagnosis and intervention; as this entity is one of the treatable conditions of paraparesis. Association with congenital neuro-ectodermal abnormality in children is frequent. This pathology highlights the importance of complete neurological checks of infants as a part of their routine physical examination and early management of patients with congenital dermal sinus, prophylactic surgical resection of such a congenital anomaly is recommended by most authors to prevent serious infections of the CNS. However, once the abscess is established; immediate surgical drainage along side adequate antibiotics should be instituted. This may guarantee improving neurological outcome. In this communication, the authors present their experience with four cases of ISCA in children treated successfully with surgical drainage, intravenous antibiotics and neuro rehabilitation between 2001 and 2006 and discuss their results. We concluded that early diagnosis and treatment is essential; before a devastating mechanico-vascular insult of the spinal cord is established from rapid formation of the abscess and a swift expansion of the spinal cord within the limited intraspinal space.

Degenerative Lumbar Spinal Stenosis – Current Strategies in Diagnosis: Effective Measure

The article ends where the excitement begins for patients with inoperable lumbar spinal stenosis and for pain therapists. Analgesics as needed are not an option in this scenario. In my opinion, epidural therapy using water soluble corticoids and low dose local anesthetics (sequentially injected in a sterile manner at the level of the sacral cornua, provided that the patient’s clotting mechanisms are intact). The postulated effective mechanism entails reduction of edema and creation of intraspinal space. This is a painless effective measure that I learnt from other anesthesiological pain therapists. I recommend evaluating this treatment in patients with lumbar spinal stenosis. We may need it ourselves one day!

Multiple short-segment laminoplasties in children: a novel technique to avoid postoperative spinal deformity

Spinal deformity is a recognized important complication after laminectomies, especially in children. Even with laminoplasties, spinal deformity is a concern, especially when laminoplasties involve multiple levels. The author reports a novel technique to avoid postoperative spinal deformity using multiple short-segment replacement laminoplasties to access pathology over a long segment of the intraspinal space. The technique was used to evacuate an organized extradural hematoma from C4 to T9 in a 9-month-old infant with incomplete quadriplegia. Three separate, short-segment, and superiorly reflected laminoplasties (C5-C7; T2-T4; T6-T8) were performed, leaving intact the laminae (T1; T5) between each laminoplasty flap. At 1 year postoperatively, there was complete neurological recovery and no spinal deformity. The laminae re-fused anatomically. Multiple short-segment laminoplasties allow good access to a long intraspinal segment, may preserve the stability of the spine, prevent spinal deformity, allow good reconstitution of the laminar anatomy, and facilitate surgical stabilization should that be required in the future. This technique may be applicable to other intraspinal lesions, such as tumors.

Klippel-Feil Syndrome and Sprengel Deformity Combined with an Intraspinal Course of the Left Subclavian Artery and a Bovine Aortic Arch Variant

We present a case of Klippel-Feil syndrome and Sprengel deformity with a bovine aortic arch and an aberrant course of the left subclavian artery in a 14-year-old boy. CT and MR imaging of the neck and upper thorax demonstrated a cervical osseous segmentation anomaly, a left common carotid artery originating from the innominate artery, and a left subclavian artery coursing through the intraspinal space at the C6 through T1 level. Possible embryonic mechanisms and clinical significance of this variant are reviewed.

Multiple Extradural Arachnoid Cysts

Report of 2 operated cousin cases of the multiple spinal extradural arachnoid cysts. To report the operative findings of cousin cases of the multiple spinal extradural arachnoid cysts and analyze a genetic etiology with the pedigree of all family members. Spinal extradural arachnoid cyst is a relatively rare condition that occupies the intraspinal space and sometimes causes neurologic disturbances. The exact etiology is unknown since most reported cases are sporadic. Minimally invasive treatments have been recently applied to a single extradural arachnoid cyst. However, it is still unknown which minimally invasive treatments can be applied to the multiple extradural arachnoid cysts. We experienced 2 cousin cases of multiple spinal extradural arachnoid cysts. Operative findings were described. The pedigree of all family members was made, and a genetic etiology was discussed. Minimally invasive treatment was tried in Case 1. However, it failed to remove all cysts completely. Each cyst had each stalk communicating with subarachnoid space in both cases. Pedigree showed that the mode of inheritance was likely autosomal dominant. When the cysts are multiple, it is necessary to identify a dural defect in each cyst. The etiology of the multiple extradural arachnoid cysts may be hereditary.

Spinal extradural arachnoid cysts associated with distichiasis and lymphedema

Spinal extradural arachnoid cysts (SEDAC) are lesions communicating to the subarachnoid space of the spinal canal via a dural defect. SEDAC occupies intraspinal space and sometimes causes neurological disturbances. Although most reported cases are sporadic, several familial cases have been described, suggesting a genetic etiology. Here we report on a family with SEDAC inherited in an autosomal dominant mode. Detailed study showed that the family has the lymphedema-distichiasis syndrome. Among family members examined, a total of ten in two generations manifested all or some of the following features: SEDAC, distichiasis and lymphedema. Seven had spinal cysts, four had both SEDAC and distichiasis, and one had SEDAC distichiasis and lymphedema; three did not have SEDAC. These findings, together with rarity of both distichiasis and lymphedema in the general population, support that all of the ten members were affected with one clinical entity, the lymphedema-distichiasis syndrome. The distribution of features illustrates the variable expressivity of clinical manifestations. Although FOXC2 mutation analysis was not performed in our family, it is likely that SEDAC is a component manifestation of lymphedema-distichiasis syndrome and more consistent in our family than those reported.