Split cord malformation with diastematomyelia presenting as neurogenic claudication in an adult: a case report

STUDY DESIGN: This is a report of a rare presentation of a split cord malformation with diastometamyelia. OBJECTIVES: This report draws attention to the fact that the only manifestation of diastmetamyelia in the adult patient may be neurogenic claudication. SUMMARY OF BACKGROUND DATA: Patients with split cord malformations and diastometamyelia rarely have symptomatic onset in adulthood. When present, a traumatic event leading to an acute neurologic change is the usual presentation. METHODS: An adult patient presented with symptoms of neurogenic claudication in the left leg. Magnetic resonance imaging examination showed a split cord malformation and diastometamylia at L3-L4 with spinal stenosis of the left hemicord. Decompressive laminectomy and subtotal resection of the bony spur were performed. RESULTS: Two years after decompression, the patient has complete resolution of his leg symptoms and is back to work. CONCLUSIONS: Neurogenic claudication without any objective neurologic deficit or neurocutaneous stigmas of an underlying spinal cord abnormality may be the only presentation in the adult with diastometamyelia. Decompression to relieve both clinical and radiologic evidence of spinal stenosis obtained excellent outcome.     

Cervical diastematomyelia presenting in adulthood. Case report

A case of cervical diastematomyelia in an adult is reported. The patient first noted sensory and motor symptoms at 34 years of age after two episodes of cervical trauma. Metrizamide computerized tomography myelography of the cervical spine and cord showed the region of diastematomyelia and revealed a spur containing both bone and fat tissue projecting into the spinal canal and cord. The spur arose from the laminae and spinous processes of C-2 and C-3, and was successfully excised. Postoperatively, the patient's deficits gradually improved. The literature concerning adult cervical diastematomyelia is reviewed.     

Diastematomyelia presenting as progressive weakness in an adult after spinal fusion for adolescent idiopathic scoliosis.

BACKGROUND CONTEXT: Diastematomyelia is uncommon and rarely presents in adulthood. This report draws attention to the fact that patients who underwent spinal fusion for deformity before the widespread use of computed tomography (CT) and magnetic resonance imaging (MRI) may have unrecognized spinal cord abnormalities. This should be considered if revision surgery is contemplated. PURPOSE: This case report focuses on the late presentation of lower-extremity weakness in a 44-year-old woman with a split cord malformation (diplomyelia), diastematomyelia and tethered cord syndrome. STUDY DESIGN/SETTING: METHODS: The patient underwent instrumented posterior spinal fusion with a Harrington rod as a child for progressive thoracolumbar scoliosis. As an adult, she developed paraparesis after a traumatic event.The patient underwent decompressive laminectomy, subtotal resection of the old fusion mass and resection of the osseous septum. Postoperatively, an anterior spinal fluid leak in the lower thoracic region required repeated fascial grafting, resection of a pseudomeningocele and reverse left latissimus dorsi flap transfer. The leak was controlled, and the patient had near complete resolution of her paraparesis 1 year after her surgery. RESULTS: The case described herein is unusual in that patients with diplomyelia and diastematomyelia rarely are symptomatic in adulthood. However, trauma may precipitate the onset of neurologic symptoms. This patient underwent spinal surgeries to address deformity, pain and progressive lower-extremity weakness. Preoperative CT and MRI studies showed a split cord malformation and diastematomyelia at L1-L2 with spinal stenosis and tethering of both hemicords. CONCLUSIONS: Progressive weakness without any previous neurologic deficit or neurocutaneous stigmas of an underlying spinal cord abnormality may develop in the adult with unrecognized diastemotomyelia. This case demonstrates that a thorough preoperative workup of patients with complex spinal deformities is imperative.     

Adult onset of tethered spinal cord syndrome due to fibrous diastematomyelia: case report

Adult onset of the symptoms of tethered spinal cord is a rare entity that is occasionally associated with diastematomyelia. Only one case of fibrous diastematomyelia in an adult has been reported. The fibrous nature of this disease may present a diagnostic difficulty. A 32-year-old man with the adult onset of impairment of sacral functions with lumbar fibrous diastematomyelia is reported. Surgical release of the spinal cord was followed by improvement of the patient's function.     

A case of diastematomyelia with meningomyelocele]

A case of diastematomyelia with meningomyelocele is presented here. The patient was a newly born infant with a soft tumor in the lumbo-sacral region. The lower extremities were not paretic, but anal reflex was negative. A plain X-ray and a CT scan of the spine showed a bone spur at L3, 4. An MRI showed a longitudinal slit in the spinal conus. The removal of the septum and repair of the cele was carried out. The operative view showed a bony septum which divided the spinal conus into two parts and nerves had spread out on the cele wall. After removal of the septum, the tethered cord was released. The postoperative condition remained stable. The reason for the occurrence of a combined case of diastematomyelia with meningomyelocele is not clear. We presume that abnormality of the mesoderm at the embryonic stage may have an effect on this sort of disorder.     

脊髓纵裂分型及临床意义

目的　研究脊髓纵裂临床病理分型及临床意义。方法　通过对 65例脊髓纵裂临床资料的回顾 ,根据影像学、病理解剖和临床表现 ,结合手术方法和效果 ,进行脊髓纵裂分型。结果　按硬脊膜形态及纵裂与脊髓关系分为双管型和单管型。双管型特征 :硬脊膜为两独立的次管 ,其内有各自分裂的半脊髓 ,两硬膜管间隙有骨性、软骨性或纤维性间隔。单管型硬膜管维持单管状 ,管腔扩大 ,其内有两个呈“镜影状”分裂的脊髓 ,少有间隔。结论　脊髓纵裂应按分型确定治疗方法。双管型应尽早手术治疗 ,如切除骨嵴 ,切断纤维性带状终丝 ,脊髓水囊肿内引流等。然后 ,进行先天性脊柱畸形矫正。单管型一般不主张脊髓探查 ,可考虑早期进行畸形脊柱的矫正治疗。     

Occult intraspinal anomalies in congenital scoliosis

Thirty consecutive patients with congenital spinal deformity underwent magnetic resonance imaging (MRI) to determine the incidence of occult intraspinal anomaly. These congenital spinal deformities included 29 cases of congenital scoliosis and one case of congenital kyphosis. Physical examination findings and plain radiographs were reviewed in an attempt to correlate these findings with subsequent intraspinal pathology. Nine patients had intraspinal anomalies identified on MRI consisting of five with tethered cord, four with syringomyelia, three with lipoma, and one with diastematomyelia. One patient required surgery for diastematomyelia; another underwent release of his tethered cord. Only one patient, with diastematomyelia associated with a syrinx and bifocal tethering, had his anomaly suggested by physical examination and plain radiographs. Two other patients had findings on plain radiographs previously associated with high prevalence of occult intraspinal anomalies; one patient with congenital kyphosis had a tethered cord, and one patient with a unilateral hemivertebrae associated with a contralateral bar had a tethered cord. Two of nine patients with occult intraspinal anomalies required surgery for their anomaly. In patients with a congenital spinal deformity, we found nine (30%) of 30 to have an associated anomaly within the spinal canal. Only three of these nine had plain radiographs and physical examination findings suggestive of their subsequent MRI findings. Given the poor correlation between findings on physical examination, plain radiographs, and subsequent occult intraspinal anomalies on MRI, we believe that MRI is helpful in evaluating patients with congenital spinal anomalies.     

Diastematomyelia--a harmless finding or cause of late neurological disturbance? (author's transl)

Diastematomyelia is said to produce neurological lesions. Several new cases of diastematomyelia are reported without any neurological defect. They are discussed and compared with similar cases from the literature. Apparently the fixation of the spinal cord by diastematomyelia seems not to be the primary cause of the "tethering cord syndrome". Other conditions which are sometimes associated with diastematomyelia (myelodysplasia, dysraphia of the spinal cord etc.) may be the primary cause of progressive neurological lesions.     

Coexistence of spinal intramedullary teratoma and diastematomyelia in an adult

STUDY DESIGN: A case report of intramedullary spinal cord teratoma with remote diastematomyelia in a female adult. OBJECTIVE: To present a rare case of a dysembryogenic spinal tumor with concurrent split cord malformation and to define the importance of early surgical removal of the tumor. SETTING: A department of neurosurgery in Greece. METHODS: A 44-year old woman, presented with progressive lower limb muscle weakness, gait disturbances and dysesthesias in the trunk and lower extremity. She underwent plain radiographs, CT and MRI scan, which revealed an exophytic intramedullary spinal cord tumor at the level of T8-T10 and distant diastematomyelia in the upper lumbar spine (L2-L3). She underwent surgical intervention. The tumor was subtotally removed. No attempt was made to treat diastematomyelia. RESULTS: Postoperatively, the patient's neurological status started to improve gradually. After 1 year she exhibited better strength in the lower limb muscles and improved sensation. The histological examination demonstrated mature spinal teratoma consisting of ectodermal, mesodermal and endodermal elements. CONCLUSIONS: In adult patients with intramedullary masses of possible dysembryogenic origin, the whole spine must be examined for additional dysraphic lesions. The choice and the timing of a surgical intervention are strongly dependent on the clinical picture.     

Neurogenic disorders of bladder emptying in closed spinal dysraphism]

Closed (occult) spinal dysraphism, e.g. lipomyelomeningocele, intraspinal lipoma, diastematomyelia, the tethered spinal cord in its various forms and dysgenesis of the sacrum, is often diagnosed late and only symptoms of neurogenic bladder dysfunction are present. A lipomyelomeningocele mostly causes detrusor and sphincter dysfunction, as was the case in five of six children among our patients. However, improvement of neurological and urological symptoms after the operation can only be achieved in about 40%. Four of eight children with diastematomyelia suffered from neurogenic bladder dysfunction; three have meanwhile undergone surgery with complete recovery in one, no relevant change in the second, and worsening in the third. Originally a specific term, the "tethered spinal cord" when associated with spinal dysraphism has taken on a more general meaning. Nowadays this term is not only used for a short, thickened and tight filum terminale, but comprises any pathology, which prevents the spinal cord from ascending. MRI examination of the craniovertebral junction and spinal cord of patients with treated myelomeningocele often reveals secondary pathologic changes: these may be areas of cord atrophy, hydromyelic cavitation or ventral compression from arachnoid cysts with clinical symptoms mostly after the age of 5 years. In these children a changing urodynamic pattern may therefore be caused by such a pathology and is an indication for a thorough neurological examination including MRI. Of all the dysrhaphic states mentioned above, sacral dysgenesis is the most frequent. The sacral osteological anomaly, as a numerical and as a structural anomaly, also determines the neuro-urological deficit.(ABSTRACT TRUNCATED AT 250 WORDS)     

Adult diastematomyelia: A complex dysrhaphic state

It is rare for patients with diastematomyelia to manifest initial symptoms in adult life. Clinical, radiological, and surgical features of a patient with symptomatic adult diastematomyelia are presented. Pathogenetic mechanisms are considered to include a variety of congenital spinal anomalies, distinct from the spur, as well as mechanical factors.     

Diastematomyelia combined with disc herniation at T 6/7 in an adult. Case report

A case of thoracic diastematomyelia associated with acute disc herniation is reported. The female patient noted sensory and motor symptoms at 49 years of age, four months before hospitalization. Myelography, myelo-computerized tomography and nuclear resonance tomography of the thoracic spine and cord showed the region of diastematomyelia with an additional disc herniation at T 6/7. The bone spur and the disc was successfully excised. Post-operatively, the patient's deficits improved.     

脊髓纵裂分型治疗的疗效分析

目的探讨脊髓纵裂分型治疗的疗效。方法对1978年5月至2006年11月收洽的有完整资料的121例单管型及双管型脊髓纵裂患者的病历资料进行回顾性分析，并通过临床神经功能评分及胫后神经皮层体感诱发电位（FTNCSEP）P40波峰监测，评价其手术及非手术治疗的疗效。结果所有患者均得到随访，随访时间6个月～15年，平均2年6个月。96例双管型患者中手术治疗86例，术后观察和随访发现24例痊愈、22例有明显改善、28例有一定改善、12例无明确效果，临床神经功能评分及PTNCSEP术后有明显改变，尤其是以疼痛为主诉的患者恢复最为明显，手术总有效率为86％；10例双管型患者未行手术治疗，随访发现临床症状无明显改善且有加重趋势。单管型患者25例，手术治疗16例，术前、术后临床神经功能评分及胛NCSEP无明显变化；未手术治疗的9例，随访时上述指标亦无明显变化。结论双管型脊髓纵裂患者脊髓损害重且呈进行性加重，精细的手术治疗可以使大多数患者症状得到缓解，终止神经进行性损伤，确诊后应尽早手术。单管型脊髓纵裂患者脊髓损害轻，无须手术探查。     

脊髓纵裂30例诊治分析

目的探讨脊髓纵裂的临床诊治。方法回顾性研究1995—2002年间我院收治的脊髓纵裂病例，分析其临床诊治过程。结果脊髓纵裂30例，女性22例(73．3％)，男性8例(26．7％)，临床表现主要有：脊柱畸形30例(100%)，背部皮肤病变13例(43．3％)，下肢神经缺陷12例(40．0％)。X线片发现椎体异常27例(90%)，脊髓造影、CT、CTM、MRI检查异常表现的比例分别为77．8％(14／18)、88．9％(8／9)、100％(16／16)、91．7％(11／12)。纵裂位于胸段15例，腰段4例，颈段及胸段l例，胸段及腰段9例，颈段、胸段及腰段1例；骨性纵裂6例。纤维性纵裂21例，混合性纵裂3例。治疗方法包括单纯骨嵴切除椎管减压1例(3．3％)。脊柱畸形矫形手术同时切除骨嵴4例(13．3％)，单纯行脊柱畸形矫形手术而未处理脊髓纵裂23例(76．7％)，未行任何手术2例。有神经缺陷者中有4例在术后获得改善。结论脊髓纵裂临床少见，以女性患者较多，往往以脊侧凸为首发表现，患者多合并有背部皮肤异常及下肢神经缺陷，CTM扫描常能明确诊断。纵裂多位于胸段及腰段，在脊柱畸形矫形手术时要注意对脊髓纵裂的影响。     

Magnetic resonance imaging of the spinal cord in spinal dysraphisms

Magnetic resonance imaging (MRI) was performed 49 times in 42 patients with spinal dysraphism. Scoliosis and a changing neurological picture were the primary indications. Spinal cord anomalies included hydromyelia, diastematomyelia, lipoma, thickened filum terminali, and spinal cord atrophy. All but one patient exhibited Arnold-Chiari malformation. Twenty-two of the 42 patients had computed tomography (CT) scans, myelograms, or operations that corroborated the 41 MRI findings. Three false-positive MRI findings of hydromyelia and no false-negative studies were observed. MRI is a noninvasive investigative technique that provides more information than myelography or CT in defining spinal cord anatomy in spinal dysraphism.     

Diastematomyelia of the cervical spinal cord with tethering in an adult

A case of cervical diastematomyelia associated with spinal cord tethering in an adult is presented. The differences between cervicodorsal and lumbar region spinal dysraphic states are emphasized with a review of the embryological factors involved. Possible causes and subsequent treatment are outlined.     

Split cord malformation: Part I: A unified theory of embryogenesis for double spinal cord malformations.

Much confusion still exists concerning the pathological definitions and clinical significance of double spinal cord malformations. Traditional terms used to describe the two main forms of these rare malformations, diastematomyelia and diplomyelia, add to the confusion by their inconsistent usage, ambiguities, and implications of their dissimilar embryogenesis. Based on the detailed radiographic and surgical findings of 39 cases of double cord malformations and the autopsy data on two other cases, this study endorses a new classification for double cord malformations and proposes a unified theory of embryogenesis for all their variant forms and features. The new classification recommends the term split cord malformation (SCM) for all double spinal cords. A Type I SCM consists of two hemicords, each contained within its own dural tube and separated by a dura-sheathed rigid osseocartilaginous median septum. A Type II SCM consists of two hemicords housed in a single dural tube separated by a nonrigid, fibrous median septum. These two essential features necessary for typing, the state of the dural tube and the nature of the median septum, do not ever overlap between the two main forms and can always be demonstrated by imaging studies so that accurate preoperative typing is always possible. All other associated structures in SCM such as paramedian nerve roots, myelomeningoceles manqué, and centromedian vascular structures frequently do overlap between types and are not reliable typing criteria. The unified theory of embryogenesis proposes that all variant types of SCMs have a common embryogenetic mechanism. Basic to this mechanism is the formation of adhesions between ecto- and endoderm, leading to an accessory neurenteric canal around which condenses an endomesenchymal tract that bisects the developing notochord and causes formation of two hemineural plates. The altered state of the emerging split neural tube and the subsequent ontogenetic fates of the constituent components of the endomesenchymal tract ultimately determine the configuration and orientation of the hemicords, the nature of the median septum, the coexistence of various vascular, lipomatous, neural, and fibrous oddities within the median cleft, the high association with open myelodysplastic and cutaneous lesions, and the seemingly unlikely relationship with fore and midgut anomalies. The multiple facets of this theory are presented in increasing complexity against the background of known embryological facts and theories; the validity of each facet is tested by comparing structures and phenomena predicted by the facet with actual radiographic, surgical, and histopathological findings of these 41 cases of SCM.     

Silastic duraplasty in pediatric patients

The role of adhesions in the retethering of the neural elements after surgical treatment of spinal dysraphism has produced refinements in the technique of the closure of the neural tube. Silastic, because of its relative inert property, has been used for duraplasty, but a few reports of late complications of hemorrhage or fibrotic capsule formation have caused concern over its use. We report 33 patients with the following diagnoses--eight spinal lipomyelomeningoceles, three myelomeningoceles, six symptomatic Chiari II malformations, seven tethered cords, six spinal cord tumors, two diastematomyelia, and one cerebellar astrocytoma--in whom Silastic dural grafts were used to prevent the adherence of neural structures to the overlying tissues. Our surgical technique is presented here. The patients have been observed for up to 6 years. Only one became infected, was treated with antibiotics without graft removal, and has remained without sequelae for over 3 years. One had an incidental pseudomeningocele that was noted on follow-up magnetic resonance imaging scan that was not clinically apparent. There have been no hemorrhages, leakage of cerebrospinal fluid, nor other complications from using nonreinforced Silastic sheeting. In one patient, Dacron-reinforced Silastic was used and, upon reexploration, a marked fibroblastic response was noted. A review of the literature suggests that fibrotic complications are related to this Dacron-reinforced Silastic. The hemorrhagic complications that were reported occurred in instances where Silastic grafts were large and no technical modifications were made preventively. We conclude that the use of nonreinforced Silastic dural grafts, with appropriate technical modifications in surgical technique, is safe and may prevent retethering of neural tissues in a variety of neurosurgical lesions.     

先天性脊柱侧凸合并脊髓分裂症的诊治探讨

目的探讨先天性脊柱侧凸合并脊髓分裂症的诊断和手术治疗方法及效果。方法1999年5月至2004年6月共收治先天性脊柱侧凸患者353例,术前经脊髓造影和(或)CTM检查发现58例合并脊髓分裂症。按照脊髓分裂畸形(SCM)的分类方法分为双管型(SCMⅠ型)和单管型(SCMⅡ型)。对其中的56例患者进行了手术治疗,除1例合并蛛网膜囊肿的患者行椎管内探查手术外,其他患者在脊柱矫形前未切除分裂脊髓间的间隔(骨性、软骨性或纤维性)。分别对SCMⅠ型和SCMⅡ型患者的临床表现、影像学改变、手术治疗的方法和效果进行回顾性分析。结果SCMⅠ型11例,占19%,SCMⅡ型47例,占81%。17例术前合并神经系统的异常表现,其中SCMⅠ型2例、SCMⅡ型15例。接受手术的56例患者中,SCMⅠ型11例,术前Cobb角(60±25)°;SCMⅡ型45例,术前Cobb角(67±21)°。术后SCMI型Cobb角(29±13)°,矫形率(51±17)%;SCMⅡ型Cobb角(39±19)°,矫形率(41±24)%。以上两组之间的差异无统计学意义。术后所有患者未出现新的神经损伤症状或原有神经症状加重。26例(45%)患者获得了平均17.3个月(4～59个月)的随访,矫形丢失(6±10)°,SCMⅠ型的7例患者丢失(5±8)°,SCMⅡ型的19例患者丢失(6±11)°,两组之间的差异无统计学意义。术后随访时患者神经症状稳定。结论先天性脊柱侧凸常合并椎管内异常病变,手术治疗前应常规行椎管内的影像学检查(脊髓造影、CTM或MRI检查)。并不是所有合并SCM的先天性脊柱侧凸患者在脊柱矫形前都需要处理椎管内的病变。对于SCMⅠ型的先天性脊柱侧凸患者,如果术前无脊髓栓系的临床表现并且影像学显示骨性间隔的上下方存在分裂的脊髓,侧凸矫形前可不必处理椎管内的骨嵴。对于合并SCMⅡ型的先天性脊柱侧凸患者,如果术前患者无进行性加重的神经系统表现,位于单管内分裂的脊髓可以像正常脊髓一样耐受脊柱矫形。     

Importance of early radiologic diagnosis of congenital anomalies of the spine

Manifest or occult spinal dysraphism in the absence of neurological symptoms is likely to remain undetected. Therefore, accurate and early diagnosis of such underlying anomalies is of prime importance for early surgical intervention in order to avoid late irreparable damage. During a period of just over 1 year, 17 such cases of spinal congenital anomalies were diagnosed by myelography with metrizamide and computed tomography-myelography. There were 16 cases of tethered cord, six with myelomeningocele, two with diastematomyelia, two with a split spinal cord, three with a lipoma, and the remaining three with vertebral anomalies. Scoliosis was present in 60%, weakness of both legs in 45%, and asymmetry of the feet in 25%. Some of these patients were initially brought to the orthopedic department for corrective surgery before undergoing a complete neurological evaluation; thus the radiologist was acting as the link between patient and neurosurgeon. Clinical experience has shown that surgical treatment can be successful and can thereby obviate further progression of neurological symptoms.