腰骶部脂肪瘤型脊髓栓系的手术治疗

目的 探讨脂肪瘤型脂肪脊髓脊膜膨出的病理改变以及如何手术去除病灶,彻底解除脊髓栓系,最大限度地恢复脊髓正常解剖结构.方法 手术治疗脂肪瘤型脂肪脊髓脊膜膨出73例,年龄1.5个月～18岁,临床症状有疼痛、大小便失禁及(或)双下肢功能障碍.手术切除皮下和椎管内脊髓外的脂肪瘤,脊髓内的脂肪瘤部分切除,将脊髓从两侧硬脊膜上剪开并分离,切断病变终丝.结果 随访67例,时间1-5年.术前有症状的患儿,术后多有不同程度改善,5例术后症状一过性加重;无症状者术后仍然无症状.结论 只有充分认识脂肪瘤型脂肪脊髓脊膜膨出的病理改变,才能最大限度地切除脂肪瘤,真正解除脊髓栓系,恢复神经功能.

Abstract：

Objective To study the pathological changes of lipomas of conus medullaris and the appropriate surgical treatment for removing such lipomas for optimal reconstruction of normal spinal cord anatomy.Methods The clinical data of 73 patients ( aged from 1.5 months to 18 years) who underwent surgical removal of lipoma of conus medullaris from January 2005 to July 2009 were collected.The neurological symptoms included pain, urine and stool incontinence, and bilateral lower limb dysfunction.The surgical procedures consisted of excision of subcutaneous and intradural extramedullary lipoma, partial excision of intramedullary lipoma, detachment of the spinal cord from the dural membrane, relief of tethered spinal cord, and excision of the affected filum terminale.Results The main pathological changes in patients with lipoma of the conus medullaris were ventral deviation of the spinal cord caused by compression from dorsal lipoma, traction on the spinal cord from attachment of intradural lipoma and subcutaneous lipoma, increased tight fit between the spinal cord and the dural membrane on both sides, and degeneration of the filum terminale.Sixty - seven patients were followed - up from 1 to 5 years.The improvement after surgery varied among the symptomatic patients.Five patients had transient deterioration of symptoms after surgery.All asymptomatic patients remained asymptomatic.Conclusion Only through thorough understanding of the pathology of lipoma of conus medullaris, we could optimally excise the lipoma, untether the spinal cord, reconstruct the normal anatomy of the spinal cord and rehabilitate neurological function.     

显微手术治疗脂肪瘤型脊髓栓系综合征的疗效分析(附611例报告)

目的 通过对611例脂肪瘤型脊髓栓系综合征临床数据分析评价其显微外科治疗的疗效.方法 患者均行神经系统和MRI检查,并经显微外科手术治疗.手术前后相应地应用尿动力学、神经电生理等检查手段.术后疗效根据Hoffman功能性分级、Kirollos术中松解分级、尿动力学和神经电生理等结果进行评定.结果 根据Hoffman功能性分级:术后无症状87例(14.2％),轻度289例(47.3％),中度214例(35.1％),重度21例(3.4％).按Kirollos术中松解分级评定方法:587例(96.0％)达到Ⅰ级,16例(2.6％)达到Ⅱ级,8例(1.3％)为Ⅲ级.尿动力学检查在术前和术后差异有统计学意义.神经电生理检查在术前和术后差异有统计学意义.结论 显微外科手术治疗脂肪瘤型脊髓栓系综合征可获得较好的疗效,尿动力学以及神经电生理的临床应用可以提高疗效.     

Pedicle subtraction osteotomy for adult tethered cord syndrome with lumbar canal stenosis: report of two cases

Tethered cord syndrome with spinal lipoma is the most common form of occult spinal dysraphism. For the symptomatic patients, surgical treatment is recommended; however, there are many patients who have not been encouraged to seek medical attention until adulthood, since their symptoms are not severe enough to interfere with their daily activities. We performed pedicle subtraction osteotomy (PSO) to achieve indirect untethering and neural decompression in two senior patients with tethered cord syndrome, who showed deteriorating neurological condition due to coexisting lumbar canal stenosis. Here we report two patients (aged 56 and 60 years) who underwent PSO of L3 or L4. The pain disappeared and the bladder dysfunction recovered significantly after surgery. Complete bone union and untethering were achieved in both patients. PSO is an alternative surgical technique for senior patients with tethered cord syndrome caused by lumbosacral spinal lipoma, when the syndrome occurs along with lumbar canal stenosis.     

The role of flexible hollow core carbon dioxide lasers in resection of lumbar intraspinal lipomas

Purpose

The authors assess the role of flexible hollow core CO₂ laser system (Omniguide system) in resection of intraspinal lipomas.

Methods

Eight patients with intraspinal lipomas were operated using an Omniguide laser system over a 22-month period. The age range varied between 6?months and 16?years. All of them had lipoma of the conus medullaris associated with lumbar subcutaneous lipoma. Two of these had previous surgery with resection of the subcutaneous part of the lipoma; in one of them, a partial resection of the conus medullaris lipoma had also been performed. In six, there were no obvious motor or sensory deficits whereas two had gross neurologic deficits, with the deficits occurring in one after the previous decompression. Resection of the conus lipoma, untethering of the spinal cord, and reconstruction of the decompressed conus was performed in all aided with Omniguide laser system. The power settings of the laser system ranged from 4 to 8?W.

Results

Subtotal to near-total resection was achieved in all. None of the patients developed any new motor or sensory deficits. Three had postoperative CSF leaks which initially required reoperation with graft resuturing and subsequently placement of lumboperitoneal shunt. One child with preexisting neurologic deficits improved in power over the next 12?months.

Conclusion

Flexible CO₂ laser system was precise and convenient in decompressing the conus lipomas and untethering of the cord in lumbar spinal lipomas.     

脂肪瘤型脊髓栓系综合征的手术技巧

目的 探讨脂肪瘤型脊髓栓系综合征的手术治疗技巧. 方法 自2003年至2006年,我科共收治33例脂肪瘤型脊髓栓系患者,均在电生理监测下切除脂肪瘤,显微手术分离粘连、松解脊髓,以无损伤不可吸收缝线(Prolene线,5-0)连续缝合硬脊膜,一期行硬脊膜重建;翻转腰背部筋膜行先天性缺损修补与加固. 结果 29例患者切口一期愈合:4例患者出现脂肪坏死导致皮下积液,其中2例患者出现脑脊液漏,经缝合及引流后治愈.随访1～3年,28例患者临床症状有显著改善,5例患者虽无改善亦无神经系统症状加重;复查均无再栓系形成. 结论 脂肪瘤型脊髓栓系综合征早期电生理监测下行栓系松解术,同时行硬脊膜的修补与重建,对于预防术后再栓系至关重要.     

Spinal cord abnormalities in caudal regression syndrome

Summary Caudal regression syndrome includes malformations ranging from mild forms of sacral agenesis to severe limb anomalies referred to as sirenomelia. The latter, in addition to sacral anomalies, shows malformed single lower limb and agenesis of rectum and genitourinary tracts. We report the neuropathologic examination in four infants, three with sirenomelia and one with lumbosacral agenesis. Brain and spinal cord were normal except for the structures in the caudal region of the spinal cord that were abnormal in all four cases. The first sirenomelic case with a mild sacral hypoplasia had only minor fusion of a few sacral roots and a slightly low-positioned conus medullaris. The second sirenomelic case with an intermediate degree of sacral hypoplasia had fusion of some of the sacral roots and ganglia, spinal ganglion cell heterotopias, filar lipoma and absence of the last sacral roots and ganglia. The third sirenomelic case with a severe degree of sacral hypoplasia showed additional tethering of the spinal cord. Case four with the agenesis of the lumbosacral spine had a total lack of the lumbosacral spinal cord, and dysplasia of the T11 and T12 cord segments. These findings suggest that the extent of anomalies of the caudal spinal cord structures in the caudal regression syndrome are roughly proportional to the anomalies of the vertebral column, and that they may constitute some of the main components of this syndrome.     

An autopsy case of pericallosal lipoma with mental retardation]

The patient was a 69-year-old male who had visited our psychological department due to mental retardation. Pericallosal lipoma was indicated by MRI taken during treatment. On December 30, 1998, the patient fell from steps and struck his forehead hard, and then limb palsy occurred. He was emergently brought to our hospital. Under the suspected diagnosis of traumatic cervical spinal cord injury, preventive therapy was performed to observe the clinical course in which paralysis gradually improved. On January 22, 1999, however, he died of cardiopulmonary arrest due to sudden suffocation. In general, the connection of choroid plexus lipoma and pericallosal lipoma is bilateral in most cases. In our patients, MRI suggested that a connection with cerebral ventricles through choroidal fissure existed with no right and left difference, however, autopsy findings revealed that the lipoma existed along the left cerebral arch with obvious connection only with the left choroid plexus. The present case seemed to be a very rare case.     

Bilateral abductor vocal cord paralysis (Gerhardt syndrome) in the Shy-Drager syndrome

Ten consecutive patients with a progressive pan-autonomic failure of the Shy-Drager syndrome were investigated. Movement disorders of the vocal cords were examined with a fiber-optic laryngoscope as well as a video-recorder. Moderate to severe vocal cord paralysis was present in five of ten patients. The vocal cords were almost immobile during inspiration, while there was no limitation of the adduction during phonation. In two cases, grade of vocal cord paralysis was asymmetric. One patient developed peculiar twisting-like dystonic movements of the vocal cord. Polygraphic studies revealed that SaO2 was lowered in spite of tachypnea during sleep. In two cases, the expiratory flow volume curve in effort-dependent portion near TLC showed a plateau and the inspiratory part of the curve also showed a plateau indicating constant flow. These functional disorders suggest an upper airway obstruction probably due to the vocal cord dysfunction. There was no vocal cord paralysis in two patients who had neither snore nor stridor. Development of a severe vocal cord dysfunction usually manifested itself clinically as stridor, snore or respiratory failure requiring tracheostomy. There was little information on the pathology of the vagal nerves and nuclei supplying motor control to the laryngeal muscles. The mechanism of the selective involvement of abductor muscle (posterior muscle) of the vocal cord (Gerhardt syndrome) remains unsolved. Vocal cord paralysis should be looked for since it can result in respiratory failure leading to death.     

Congenital lumbosacral lesions with late onset in adult life

Seven adult patients with congenital lumbosacral lesions and clinical onset in adult life are reported. All also had an abnormally low and tethered spinal cord. A thickened filum terminale and a spinal lipoma are the most common lesions in adults, whereas others, including diastematomyelia, fibrous adhesions, previous meningocele repair and dermal sinus are more rare. Although water-soluble myelography visualizes the abnormally low conus well and may diagnose a diastematomyelia and a thickened filum, computerized tomography, CT myelography and magnetic resonance may provide a better diagnostic definition of the other abnormalities. Surgical treatment should be performed in all adult patients with progressive symptoms. Transection of the thickened filum or of fibrous adhesions and the removal of a spinal lipoma, diastematomyelia septum or dermal sinus tract, result in remission of the pain and improvement of the sensorimotor deficits, or at least prevent the progression of the neurological troubles.     

软膜下脂肪瘤的外科治疗与分型：附5例报告

目的 对不合并脊柱裂的软膜下脂肪瘤进行临床回顾,根据临床特点提出一种基于影像学的新的亚组分类,并对其手术策略进行探讨.方法 回顾分析了2013年1月~2014年12月在北京积水潭医院神经外科接受手术治疗的5例软膜下脂肪瘤患者.所有病例均有完整的临床资料,常规进行术前的评估,定期门诊随访进行神经功能评估和影像学评估.基于磁共振影像特点将本组5例病例分为两组:单纯型组和侵袭型组,比较两种类型的术式及临床情况.结果 所有5例患者在术前的神经功能均有轻中度的损害,均行手术治疗,手术过程均较顺利,单纯型中1例达到了完全切除,1例大部分切除;侵袭型中3例均为部分切除;肿瘤切除情况在术后的MRI检查中得到证实,所有病例最终均经病理学证实为脂肪瘤.两者表现为不同的影像特点和不同的神经功能恢复过程.单纯型并发脊髓空洞,术中肿瘤边界较易探查;侵袭型肿瘤中有神经组织穿行,切除过程中即产生损伤,在术后病理中可得到证实.两种类型在术后均有短暂的神经功能下降,但单纯型在术后两年基本恢复到术前情况,预后较后者好.结论 单纯型和侵袭型的分类方法对认识不伴脊柱裂的脊髓软膜下脂肪瘤可能有一定帮助,单纯型一般手术中可发现肿瘤与脊髓的边界,可能完全切除;而侵袭型肿瘤呈浸润性生长,任何试图切除肿瘤的操作可能导致脊髓功能的进一步损伤,减压性手术可能是最佳的选择.     

CNS lipoma in patients with epidermal nevus syndrome

Epidermal nevus syndrome (ENS) is a congenital neurocutaneous disorder characterized by linear nevus with a significant involvement of the nervous, ophthalmological and skeletal systems. Clinical manifestations of ENS include neurological features such as mental retardation, seizures, and movement disorders which are caused by a wide range of neuropathological lesions. We describe three patients with ENS, all of whom had in addition to the characteristic features of ENS intracranial and/or intraspinal lipomas. In one patient the lipoma extended from the thoracal vertebra 8 to the 4th ventricle; in the second patient it was localized on T9, and in the third patient an intracranial lipoma was located at the right cerebellopontine angle. The intraspinal lipomas caused a significant spastic movement disorder. So far, CNS lipomas have not been described as typical neuropathological findings in ENS. The differential diagnosis to encephalocraniocutaneous lipomatosis with the typical finding of CNS lipoma is discussed.     

儿童脊髓拴系综合征的显微外科治疗

目的 研究显微手术治疗儿童脊髓拴系综合征的疗效。方法 回顾性分析35例儿童脊髓拴系综合征，平均年龄2.2岁。均伴有脊柱裂，合并单纯脊髓脊膜膨出20例，脊髓脊膜膨出合并椎管内脂肪瘤13例，畸胎瘤1例，皮样囊肿1例。行显微手术松解黏连的脊髓和神经，切断牵拉的终丝。结果 会阴部感觉异常总有效率为80．0％，排尿功能障碍总有效率为77．1％．结合矫形手术可明显改善运动障碍。结论 显微外科手术松解脊髓圆锥和马尾神经及切断牵张的终丝．是治疗儿童脊髓拴系综合征的关键．结合矫形手术可提高病儿的生存质量。     

Congenital lumbosacral lesions with late onset in adult life

Abstract

Seven adult patients with congenital lumbosacral lesions and clinical onset in adult life are reported. All also had an abnormally low and tethered spinal cord. A thickened filum terminate and a spinal lipoma are the most common lesions in adults, whereas others, including diastematomyelia, fibrous adhesions, previous meningocele repair and dermal sinus are more rare. Although water-soluble myelography visualizes the abnormally low conus well and may diagnose a diastematomyelia and a thickened filum, computerized tomography, CT myelography and magnetic resonance may provide a better diagnostic definition of the other abnormalities. Surgical treatment should be performed in all adult patients with progressive symptoms. Transection of the thickened filum or of fibrous adhesions and the removal of a spinal lipoma, diastematomyelia septum or dermal sinus tract, result in remission of the pain and improvement of the sensorimotor deficits, or at least prevent the progression of the neurological troubles.     

Lumbosacral dysraphia and "long spinal cord" of delayed appearance (9 cases)

Urinary disorders, deformities of the feet, and sensory of trophic disturbances appearing during, or increasing in severity at adolescence, were observed in 9 patients. In seven cases there appeared to be an abnormality of the teguments of the lumbosacral region, and in all cases, as shown by straight radiological examination of a spina bifida of L5 or S1, a sacral dehiscence and wide sacral canal, and a subjacent nervous tissue abnormality was suspected. Radiculography showed the presence of a "long spinal cord" terminating in the sacrum by a tumor mass. Examination of this mass in the seven operated cases demonstrated that the tumor was an intradural lipoma with a wedge-shaped penetration into the end of the medulla, fixing the cord in the sacrococcygial region, in the absence of the filum terminale and cauda equina. The etiology of this "long spinal cord" is discussed, and it appears to be the consequence of a dysraphia, which is also the cause of the lipoma. Mechanical factors appear later and produce neurological signs.     

32例颈髓肿瘤显微外科治疗研究

目的 探讨颈髓肿瘤的诊断、手术入路选择及手术操作技巧.方法 回顾性分析湘雅二院神经外科自2003年6月至2005年6月经手术治疗的32例颈髓肿瘤患者的临床资料.所有患者均经术前MRI确诊,经显微手术切除,术后均行MRI复查.结果 手术显微镜下全切肿瘤29例,大部分切除3例,其中2例为星形细胞瘤,1例为脂肪瘤.术后无神经功能障碍加重及死亡者.结论 MRI对颈髓肿瘤的诊断具有最重要的价值.据此可以明确肿瘤的大致类型、位置及毗邻关系.选择合适的手术入路.显微外科手术是治疗颈髓肿瘤的有效方法,熟练应用显微外科技术和手术操作技巧是治疗成功的关键因素.     

A bony human tail causing tethered cord syndrome: case report

Introduction

Dorsal cutaneous appendages, sometimes referred to as “human tails,” are considered to be markers of underlying occult spinal dysraphism. Rarely, these tail-like structures can themselves be the cause of tethered cord syndrome. However, to date, a “bony human tail” causing tethered cord has not been reported in the literature. One such rare lesion is being reported.

Discussion

A 2-days-old female child was brought for neurosurgical consultation with a skin-covered bony protuberance in the lower back. Examination of the child did not reveal any neurological deficits. Plain radiographic and CT evaluation showed a dorsal bony protuberance arising from the posterior elements of L1 vertebra. MRI showed the cord to be displaced posteriorly and adherent to the undersurface of the bony tail through a lipoma. During surgery, the bony “tail” was excised, and the cord was untethered with excision of the lipoma, which was tethering the cord to the bony “tail.” When examined 1 year later, the child was developing normally without any focal neurological deficits.

Conclusions

This case is being reported for its rarity and to highlight the hitherto unreported occurrence of “bony human tail” causing tethered cord syndrome.     

Magnetic resonance imaging of the spine in children

We reviewed the magnetic resonance imaging (MRI) scans of the spine of 42 children who had neurologic signs compatible with lesions of the spinal cord. Twenty-three of the children had abnormalities identified by MRI. The spectrum of abnormalities included posttraumatic lesions, tumor, tethered cord, and syringohydromyelia. Tethered cord with or without lipoma and syringohydromyelia were the most common findings, affecting six and four children, respectively. Thirteen children studied by MRI also underwent conventional metrizamide myelography and/or computed tomography. In 12 cases, the findings of MRI were comparable to those of myelography and computed tomography. These observations indicate that MRI effectively detects lesions of the spinal cord in children. Because MRI can be performed on an outpatient basis and avoids the risks of metrizamide myelography, we conclude that MRI should be considered to be the preferred screening technique for children with suspected spinal cord disorders.     

Congenital lumbosacral lipomas

Congenital lumbosacral lipomas can be responsible for progressive defects. The general feeling is that tethering of roots, filum, or cord probably explains this evolution, and that untethering of these structures could prevent late deterioration. Like the vast majority of neurosurgeons, we too have routinely and systematically operated on lumbosacral lipomas, even in the absence of neurological deficits. This policy stemmed from our belief that spontaneous neurological deterioration was frequent, recovery from preoperative deficits rare, and surgery both efficient and benign in nature. After 22 years of experience, we felt that it was necessary to review our series of 291 lipomas (38 lipomas of the filum and 253 of the conus) operated on from 1972 to 1994. To reassess the value of pro-phylactic surgery, we attempted an accurate evaluation of (1) the risk of pathology, (2) the risks involved in surgery, (3) the postoperative outcome with respect to preoperative deficits, and (4) the postoperative outcome in asymptomatic patients at 1 year and at maximum follow-up. Special attention was paid to 93 patients whose postoperative follow-up was more than 5 years (average 8.7, median 8, range 5–23 years). Of these 93 patients, 39 were asymptomatic preoperatively (7 with lipoma of the filum and 32 with lipoma of the conus). Lipomas of the filum and of the conus are entirely different lesions and were studied separately. In 6 cases prenatal diagnosis had been possible. The mean age at surgery was 6.4 years. Low back skin stigmata were present in 89.4% of cases. Preoperative neurological deficits existed in 57% of the patients and were congenital in 22%. Clinical signs and symptoms recorded were pain in 13.3% of the patients and/or neurological deficits affecting sphincter (52%), motor (27.6%) and sensory (22.4%) functions. Deficits were progressive in 22.4% of cases, slowly progressive in 58.8% of these and rapidly progressive in the remaining 41.2%. In 36 patients (13.2%) the lipomas were seen to grow either subcutaneously or intraspinally. Among these patients, 21 were infants, 2 were obese adolescents, and 10 were pregnant women. The metabolism of the fat within the lipomas was studied in 11 patients and found to be similar to that at other sites. Lipomas were associated with various other malformations, either intra- or extraspinal. These associated anomalies were rare in the case of lipomatous filum (5.2%) but frequent with lipomas of the conus, except for intracranial malformations (3.6%). Therapeutic objectives were spinal cord untethering and decompression, sparing of functional neural tissue and prevention of retethering. Procedures used to achieve these goals were subtotal removal of the lipoma, intraoperative monitoring, duroplasty, and sometimes closure of the placode. Histologically, lipomas consisted of normal mature fat. However, 77% of them also included a wide variety of other tissues, originating from ectoderm, mesoderm, or entoderm. This indicates that lipomas are either simple or complex teratomas. The results of the study are as follows. (1) Surgery was easy and safe when performed for treatment of lipomas of the filum (no complications), but difficult and hazardous in the case of lipomas of the conus (20% local, 3.9% neurological complications). (2) All types of deficit could be improved by surgery, which was beneficial in all cases of lipoma of the filum and 50% of cases of lipoma of the conus. (3) In asymptomatic patients long-term surgical results depended on the anatomical type of the lipoma. They were excellent in lipomas of the filum. In lipomas of the conus they were good in the short term but eroded with time. At more than 5 years of follow-up only 53.1% of the patients were still free of symptoms. (4) Reoperations were performed in 16 patients (5.5%), 5 (31.2%) of whom improved postoperatively, while in 7 (43.7%) progression stopped, in 3 (18.7%) deterioration continued and in 1 (6.2%) the condition was worse after surgery. (5) The natural history of the malformation, that is to say the risk of spontaneous aggravation, has only been evaluated in hospital in-patients, so that the true level of risk remains unknown. This means we cannot interpret the actuarial curve following surgery for asymptomatic lipoma of the conus. In conclusion, there are two different types of lipoma: lipoma of the filum, for which surgery is harmless and beneficial in both the short and the long term, and lipoma of the conus, for which surgery involves considerable risks and is of questionable benefit in the long term. This raises the question as to whether prophylactic surgery is indicated for patients with asymptomatic lipomas of the conus, and whether the outcome is any better than it would be if the lipoma were left to take its natural course. The lack of basic information remains a stumbling block to management of these patients. Until this is remedied, we are unable to recommend prophylactic surgery in patients with asymptomatic lipomas of the conus. Received: 25 November 1996     

Encephalocraniocutaneous lipomatosis associated with iris coloboma, chorioretinitis and spinal cord involvement: a case report

A 9-year-old boy with a history of lipoma excision and laminectomy at the Th10-11 levels, resulting in incomplete paraparesis and neurogenic bladder, was admitted for a comprehensive rehabilitation programme. Physical examination revealed an ipsilateral focal dermal hypoplastic defect within an area of alopecia and a subcutaneous lipomatous tissue on the left temporo-parietal region of the scalp. Iris coloboma and chorioretinitis were diagnosed on the left eye. He also had mild mental retardation and triparesis. Magnetic resonance imaging of the brain and the spine demonstrated hyperintense masses which were consistent with lipoma. Although in the literature three cases of encephalocraniocutaneous lipomatosis (ECCL) concomitant with spinal cord involvement have been reported, to our knowledge iris coloboma and chorioretinitis in ECCL have not been reported previously. In conclusion, we would like to stress that aside from known ophthalmological malformations, iris coloboma and chorioretinitis may also be observed in ECCL and that all patients who have been diagnosed as having ECCL should be examined for spinal cord involvement.