Cervical cord tethering due to split cord malformation at the cervico-dorsal junction presenting with self-mutilation of the fingers

An unusual case of cervical spinal cord tethering with diplomyelia is described. A 12-month old female presented with self-mutilation of the fingers due to sensory loss in the hands, absent reflexes, poor muscle tone, and reduced distal upper-limb movements. There was a deep skin dimple overlying the T1 spinous process. Imaging showed angulation of the lower cervical cord and an operation revealed a low cervical meningocele and a split cord malformation with tethering of one half of the cord; the cord was untethered. In this report the literature is reviewed.     

Solitary Cervical Neurenteric Cyst in an Adolescent Patient

Spinal neurenteric cysts are uncommon congenital lesions, furthermore solitary neurenteric cysts of the upper cervical spine are very rare. A 15-year-old boy having an intraspinal neurenteric cyst located at cervical spine presented with symptoms of neck pain and both shoulders pain for 2 months. Cervical spine magnetic resonance (MR) imaging demonstrated an intradural extramedullary cystic mass at the C1-3 level without enhancement after gadolinium injection. There was no associated malformation on the MR imaging, computed tomography, and radiography. Hemilaminectomy at the C1-3 levels was performed and the lesion was completely removed through a posterior approach. Histological examination showed the cystic wall lined with ciliated pseudostratified columnar epithelium containing mucinous contents. Neurenteric cyst should be considered in the diagnosis of spinal solitary cystic mass.     

Cervical meningocele and associated spinal anomalies

Simple meningoceles are infrequent forms of dysraphism and are often benign. They have been associated with other spinal anomalies. The uncommon cervical meningocele may have a higher propensity to be associated with other spinal anomalies. Four patients with cervical meningocele are presented with radiographic evaluation and clinical course. Multiple abnormalities were documented radiographically and operatively, including hydrocephalus, Chiari malformation, hydromyelia, lipomeningomyelocele, tethered cord, thickened filum terminale, diastematomyelia, Klippel-Feil syndrome, and thoracic hemivertebrae. Prior to the development of any late neurological abnormality from associated spinal anomalies, magnetic resonance imaging is recommended early in a child born with a simple meningocele.     

Is meningocele really an isolated lesion?

OBJECT: We designed this study to elucidate the associated occult spinal lesions in patients with simple dorsal meningocele. METHODS: The study population was comprised of two groups. Group I comprised newly diagnosed patients with dorsal spinal meningocele, and group II comprised patients who had had surgery for meningocele and presented with progressive neurological deficits. Magnetic resonance imaging (MRI) scans of the whole spinal column were done. The associated spinal cord malformations were also treated at the same operation. There were 14 boys and 8 girls, with an age range from birth to 4 years (mean 3.9 months), in group I. Of 20 patients (90%) with associated spinal lesions, 6 had more than one lesion, excluding hydromyelia. Group II was made up of 6 patients who had been previously operated on for a meningocele and who presented with tethered cord syndrome. These were 4 boys and 2 girls, who ranged in age from 4 to 10 years (mean 6 years). RESULTS: The level of the conus terminalis was lower than L3 in all patients. The other findings on MRI, besides low conus, were as follows: tight filum, split cord malformation, epidermoid, dorsal lipoma and hydromyelia. CONCLUSIONS: Meningocele frequently camouflages a second, occult, spinal lesion. MRI of the whole spinal column should be performed. An intradural exploration performed with a microneurosurgical technique is needed to detect the fibrous bands that may lead to spinal cord tethering and to release the entrapped nerve roots. The other associated spinal anomalies should be operated on during the same operation.     

Neurenteric cyst of medulla oblongata--a curiosity

A case of a newborn infant with a complex cerebral malformation, including a neurenteric cyst of medulla oblongata is presented. This localization is extremely rare, only one such case has been reported in the world literature. Other cerebral malformations were spina bifida posterior with cervico-dorsal dysraphia, cerebellar hypoplasy, cervico-dorsal cystic meningocele and hydrocephalus. The etiology of this complex malformation remained unknown.     

A neurofibromatosis type 1 patient with severe kyphoscoliosis and intrathoracic meningocele.

The patient presented with neurofibromatosis and a dystrophic kyphoscoliosis around the cervico-thoracic junction. When the patient was 59 years old, he started to suffer from dyspnea caused by an intrathoracic meningocele in the upper left thoracic cavity. A wide laminectomy from T2 to T5 was performed and the meningocele was resected. Although the dyspnoea disappeared postoperatively, the patient started to neurologically deteriorate. Laminectomy alone caused instability around the apex of the kyphosoliosis and spinal cord compression. Halo cast was applied and brought remarkable recovery of neurologic deficits. This result encouraged us to perform posterior fusion in situ from C3 to L2 with bone graft from the iliac crests and the Luque technique in conjunction with the Isola system. This resulted in the patient being able to walk again. The removal of the posterior element predisposes the patient to unstable postlaminectomy kyphosis and removes valuable bone stock required for posterior spinal fusion. For this reason, spinal fusion should have been conducted during surgery for the patient's meningocele.     

Spinal dysraphism: MR imaging rationale

Spinal cord development occurs through the three consecutive periods of gastrulation (weeks 2-3), primary neurulation (weeks 3-4), and secondary neurulation (weeks 5-6). Spinal cord malformations derive from defects in these early embryonic stages, and are collectively called spinal dysraphisms. Spinal dysraphisms may be categorized clinically into open and closed, based on whether the abnormal nervous tissue is exposed to the environment or covered by skin. Open spinal dysraphisms include myelomeningocele and other rare abnormalities such as myelocele, hemimyelomeningocele, and hemimyelocele, and are always associated with a Chiari II malformation. Closed spinal dysraphisms are further divided into two subsets based on whether a subcutaneous mass is present in the low back. Closed spinal dysraphisms with mass comprise lipomyelocele, lipomyelomeningocele, meningocele, and myelocystocele. Closed spinal dysraphisms without mass comprise simple dysraphic states (tight filum terminale, filar and intradural lipomas, persistent terminal ventricle, and dermal sinuses) and complex dysraphic states. The latter category involves abnormal notochordal development, either in the form of failed midline integration (ranging from complete dorsal enteric fistula to neurenteric cysts and diastematomyelia) or of segmental agenesis (caudal agenesis and spinal segmental dysgenesis). Magnetic resonance imaging is the imaging modality of choice for evaluation of this complex group of disorders.     

Spontaneous hemorrhage into spinal neurenteric cyst

Case report A case of spontaneous hemorrhage into an intradural extramedullary neurenteric cyst at the C4–C6 level in a 6-year-old boy is reported. The child presented with sudden neck pain, torticollis, and myelopathy. The cyst was of high signal intensity on both T1- and T2-weighted magnetic resonance images, suggesting hemorrhage into the cyst. The combination of CT and MR provided useful preoperative information regarding the nature and the location of the cyst. A posterior approach via osteoplastic laminotomy was used for this anteriorly localized cyst. The use of intraoperative ultrasound was helpful in identifying and puncturing the cyst to minimize the spinal cord manipulation during the excision of the cyst wall.Discussion We report the first documented case of hemorrhage into a spinal neurenteric cyst that has been confirmed surgically and histopathologically.     

Supratentorial neurenteric cysts—A fascinating entity of uncertain embryopathogenesis

The histopathological, immunologic, and ultrastructural findings of neurenteric cysts support an endodermal derivation. These developmental cystic lesions are generally located in the posterior mediastinum, abdomen, and pelvis and may also contain some mesodermal and neuroectodermal elements. In contrast, neurenteric cysts of the central nervous system are very infrequent and occur most commonly in the spinal canal. Intraspinal neurenteric cysts are usually encountered in the cervicothoracic region with an intradural, extramedullary location and are commonly associated with congenital defects of the overlying skin and/or vertebral bodies.     

Prenatal diagnosis of diastematomyelia

Introduction Diastematomyelia, also termed split cord malformation, is a form of occult spinal dysraphism characterized by a cleft in the spinal cord. Prenatal diagnosis of this anomaly is possible by ultrasonography (US), and fetal MRI can be used to diagnose the type of diastematomyelia precisely. Diastematomyelia can be isolated or associated with other dysraphisms, segmental anomalies of the vertebral bodies, or visceral malformations (horseshoe or ectopic kidney, utero-ovarian malformation, and anorectal malformation). We present three cases of fetal diastematomyelia investigated using a multimodal prenatal work-up (US, MRI, 3D-CT).Cases The first case, detected at 20 weeks' gestation, had a lumbar meningocele. At 30 weeks' gestation, direct US visualization revealed the division of the spinal cord into two hemicords. This patient illustrates an isolated type II diastematomyelia with a favorable prognosis. The second case, detected at 22 weeks' gestation, presented with disorganization of bony process of the vertebral column with a midline echogenic bony spur, asymmetrical hemicords, and a foot malposition. Fetal MRI at 26 weeks' gestation and CT/3D reconstructed at 32 weeks' gestation confirmed a type I diastematomyelia with orthopedic malposition. The third case, detected at 22 weeks' gestation, presented with widening of the lumbar canal and scoliosis. Prenatal work-up (US, MRI) disclosed other visceral malformations (pelvic kidney), which led to the assumption of a complex polymalformative syndrome. The pregnancy was terminated. Fetopathologic examination disclosed even more visceral malformations (anal atresia and unicorn uterus).     

Unusual split cord with neurenteric cyst and cerebellar heterotopia over spinal cord

OBJECTIVE: To report a case of splitting of a cord, which could neither be classified as type I nor type II according to Pang's classification. The child had associated neurenteric cyst and cerebellar tissue heterotopia. CASE REPORT: A 3-year-old girl presented with a tuft of hair and dermal sinus at her upper dorsal spine. She had splitting of cord into two halves by two intradural horny osseocartilaginous spurs leaving a gap of 3 mm in between (two spurs and two halves of cords). A neurenteric cyst was seen passing from the anterior to posterior aspect of the cords through this gap. An associated heterotopic cerebellar tissue mass was encountered over the dorsum of cord proximal to splitting. The dermal sinus, neurenteric cyst, and spurs were excised to detether the cord. The heterotopic tissue was biopsied. The child had no neurological deficit at 2.5 years follow-up. CONCLUSION: An unusual splitting of cord by unusual spurs may leave a gap between two spurs and two halves of cords to pass a neurenteric cyst. Rarely, heterotopic tissue may be associated with split cord syndrome. A dermal sinus may lead into underlying neurenteric cyst (rather than dermoid).     

Giant intrasacral meningocele: a case report

A peculiar case of intrasacral meningocele and spinal cord tethering is reported. Contents of the intrasacral meningocele and importance of CSF flow analyses with MRI are discussed. Demonstration of CSF flow from the thecal sac to meningocele in the CSF flow MR imaging may be helpful for determining the possibility of meningocele growth. In this report, we have presented the determination of CSF flow as a new surgical indication in this type of cases.     

脊髓中央管隔膜形成与Chiari畸形并脊髓空洞

目的探讨显微外科治疗Chiari畸形并脊髓空洞的手术方法.方法 127例Chiari畸形并脊髓空洞经MRI确诊,采用小脑扁桃体下疝切除,并脊髓中央管开口隔膜切开术. 结果术中发现脊髓中央管开口隔膜形成;术后随访89例,MRI检查示,所有患者下疝的小脑扁桃体均消失,并存脊髓空洞明显缩小和消失.结论小脑扁桃体下疝,脊髓中央管开口隔膜形成是造成Chiari畸形并脊髓空洞的原因之一;小脑扁桃体下疝切除,脊髓中央管隔膜切开术是手术治疗Chiari畸形并脊髓空洞症的有效方法.     

Intramedullary neurenteric cyst of the conus medullaris without associated spinal malformation: a case report and review of the literature

Spinal neurenteric (NE) cysts are rare congenital anomalies that may occur either alone or in the context of a complex malformative disorder. They are usually intradural-extramedullary lesions. Intramedullary NE cysts not associated with other congenital anomalies are very rare and only a few cases have been reported in the conus medullaris region. Intramedullary neurenteric cysts not associated with other spinal anomalies are very rare especially in the conus medullaris region. MRI is useful to define the cyst and the osseous anomalies associated with this lesion. The goal of treatment of an intramedullary neurenteric cyst is total excision at the first operation, if possible. Life-long follow-up with annual MRI is recommended due to the risk of cyst recurrence. We report an intramedullary NE cyst of the conus medullaris without associated malformation and the relevant literature is briefly reviewed.     

Hemangioblastoma of the conus medullaris associated with cutaneous hemangioma

A 6-month-old infant is reported with a spinal cord hemangioblastoma located in the conus medullaris associated with an overlying congenital dermal sinus and cutaneous capillary hemangioma. There were no neurologic deficits either preoperatively or following removal of the tumor. The skin and spinal cord lesions were believed to represent an isolated vascular malformation. This spinal cord hemangioblastoma is unusual because of the age of the patient, manner of clinical presentation, location in the caudal spinal cord, and pathologic characteristics. We review the literature and discuss the associations of spinal cord hemangioblastomas with cutaneous and other lesions.     

脊髓血管畸形合并胸腔血管畸形

脊髓血管畸形的临床发病率较低,仅为颅内血管畸形或脊髓新生物的1/10左右.本病可能与其它组织的血管异常同时存在,如皮肤血管痣(Cobb综合征),皮肤、粘膜病变,和Klipple-Trenaunay-Weber综合征等.本文报告的与胸腔血管畸形同时存在的脊髓血管畸形,我们尚未见诸报道.本文还结合文献就脊髓血管畸形导致脊髓功能损害的病理生理作了阐述.     

Cervical neurenteric cyst associated with Klippel-Feil syndrome: a case report and review of the literature.

A neurenteric cyst of the spine is a rare congenital disorder secondary to alimentary duplication and vertebral malformation. It should, however, be included in the differential diagnosis of an intradural, extramedullary spinal lesion. We present a case of a cervical neurenteric cyst associated with Klippel-Feil syndrome and discuss the clinical, radiologic, histopathologic, immunohistochemical and embryologic characteristics of this disorder.     

Anterior cervical arachnoid cyst presenting with traumatic quadriplegia

Introduction Intradural spinal arachnoid cysts are rare. Rarer still are cysts located anterior to the cervical spinal cord. To date, only 10 such cases have been reported in the English-language literature.Case report Two cases of anterior cervical arachnoid cysts that presented as traumatic quadriplegia are reported.     

Holocord syringomyelia secondary to tethered spinal cord associated with anterior sacral meningocele and tailgut cyst: case report and review of literature

Background and importance

Anterior sacral meningoceles are lesions that are uncommonly reported and can be associated with other pathology including presacral masses, tethered spinal cord, and syringomyelia. Tethered spinal cord and syringomyelia can result in neurologic deficits, while large meningoceles and presacral masses can have gastroenterologic, urologic, reproductive, and oncologic consequences.

Clinical presentation

The authors report a case of a 14-year-old girl with an anterior sacral meningocele, tailgut cyst, and tethered cord with holocord syringomyelia who presented with a tethered cord syndrome, manifested by constipation, urinary retention, bilateral lower extremity weakness, and sensory deficits. After extensive radiographic and urodynamic workups were performed, the patient was treated by the neurosurgery and pediatric surgery teams with a posterior sagittal approach for cord detethering, resection of an intradural cystic mass, resection of the anterior sacral meningocele, and resection of the adjacent presacral mass. After surgical treatment, motor weakness and sensory deficits were resolved, though urinary symptoms persisted. The syrinx resolved after detethering alone. Pathology of the intradural cystic mass and the presacral mass inferior to the anterior sacral meningocele were consistent with tailgut cyst.

Conclusion

The patient’s clinical and surgical management are discussed, and a literature review related to anterior sacral meningoceles and their related pathologies is presented. An interdisciplinary approach is required for the best treatment of this constellation of findings.     

脑干脊髓腹侧肠源性囊肿的显微手术治疗

目的 探讨位于脑干及脊髓腹侧肠源性囊肿的诊断及显微手术方法.方法 回顾位于脑干脊髓腹侧肠源性囊肿8例,其中4例颅颈交界部囊肿采用远外侧手术入路,3例颈段采用后正中入路,1例胸段采用胸椎侧前方入路进行手术切除.结果 MRI是早期诊断肠源性囊肿的有效手段.7例囊肿获得全切除,1例大部切除;所有患者术后神经功能恢复良好.结论 肠源性囊肿虽然是先天性良性肿瘤,但多位于脑干、脊髓腹侧,手术易造成脑干、脊髓损伤,全切除难度大;采用枕下远外侧经髁入路或胸椎侧前方入路,通过显微外科操作可在对脑干、脊髓最小干扰下获得全切除.