Cervical meningomyelocele—an institutional experience

Objective Cervical myelomeningoceles (CMMC) are a less common but distinct subgroup of myelomeningoceles. Their embryology and clinical characteristics vary from the more common thoracolumbar variant. Only a few small series have been published addressing this lesion in the literature with the largest one of them addressing nine patients. The authors present one of the largest series of cervical myelomeningoceles, review their embryology, clinical features, and their management strategies. Methods This study included all the children who were managed for cervical myelomeningocele between Jan 2001 to July 2006 at our center. Results There were a total of ten children (five boys and five girls) operated on for cervical myelomeningoceles. The ages ranged between 2 months to 14 months. Neurological examination was normal in majority of the children with absence of gross orthopedic deformity in all the children. Three patients had associated hydrocephalus, two had Chiari malformation, and four of them had a syrinx. Surgical excision of the sac was performed for all. Conclusion Cystic dysraphisms of the cervical differ embryologically, clinically, and structurally from thoracolumbar meningomyelocele and have a more favorable outcome. A good pre operative evaluation is recommended to assess any associated anomalies and identify the internal structures. Surgery excision of these lesions with intradural exploration of the sac to release any potential adhesion bands as well as other associated anomalies is recommended.     

Cutaneous silent periods in intramedullary spinal cord lesions

OBJECTIVE: The neurophysiological assessment of intramedullary spinal cord lesions has been unsatisfactory. Previous studies in patients with syringomyelia suggest that testing of cutaneous silent periods (CSPs) may be useful to assess centromedullary lesions. METHODS: The authors studied nine patients with intramedullary spinal cord lesions of different etiologies. Eight patients with cervical lesions presented with hypalgesia, hypothermesthesia, or pain in at least one upper extremity; five of them had also upper limb weakness or sensory impairment. One patient with a thoracic lesion had normal upper limb function. The authors recorded CSPs in abductor pollicis brevis muscle following digit II and digit V stimulation. Somatosensory evoked potentials (SEPs) were obtained following median and tibial nerve stimulation. Motor evoked potentials (MEPs) were obtained in biceps brachii, abductor digiti minimi and tibialis anterior muscles following transcranial magnetic or electrical stimulation. RESULTS: CSP abnormalities were found in all patients with cervical lesions, but not in the patient with a thoracic lesion. Cortical median nerve SEPs had normal latencies in all patients, while tibial nerve SEPs, upper limb MEPs, and lower limb MEPs were delayed in five patients each. In one patient, abnormal CSP were the only neurophysiological finding. CSP abnormalities were associated with hypalgesia and hypothermesthesia in 95% of the studies. CONCLUSION: Upper extremity CSP testing is a sensitive neurophysiological technique for the assessment of cervical intramedullary lesions. In particular, abnormal CSPs are highly associated with spinothalamic dysfunction.     

Limited dorsal myeloschisis: a not-so-rare form of primary neurulation defect

Purpose

Limited dorsal myeloschisis (LDM) is a distinctive form of spinal dysraphism characterized by two constant features: a focal “closed” midline skin defect and a fibroneural stalk that links the skin lesion to the underlying cord. The embryogenesis is hypothesized to be incomplete disjunction between cutaneous and neural ectoderms, thus preventing complete midline skin closure and allowing persistence of a physical link (fibroneural stalk) between the disjunction site and the dorsal neural tube.

Objective

We utilize the experience gained from the management of 63 patients with LDM to illustrate these features.

Methods

All patients were studied with MRI or CT myelogram, operated on, and followed for a mean of 9.4 years.

Results

There were 11 cervical, 16 thoracic, 8 thoracolumbar, and 28 lumbar lesions. Two main types of skin lesion were: saccular (26 patients; consisting of a skin base cerebrospinal fluid sac topped with squamous epithelial dome or a thin membranous sac) and nonsaccular (37 patients; with a flat or sunken squamous epithelial crater or pit). The internal structure of a saccular LDM could be a basal neural nodule, a stalk that inserts on the dome, or a segmental myelocystocoele. In nonsaccular LDMs, the fibroneural stalk has variable thickness and complexity. All fibroneural stalks contain glioneuronal tissues accompanied by variable quantities of nerves and mesodermal derivatives. In all LDMs, the fibroneural stalk was tethering the cord. Twenty-nine patients had neurological deficits. There was a positive correlation between neurological grade and age suggesting progression with chronicity. Treatment consisted of detaching the stalk from the cord. Most patients improved or remained stable. LDMs were associated with three other dysraphic malformations in more than coincidental frequencies: six LDMs were contiguous with dorsal lipomas, four LDMs shared the same tract or traveled in parallel with a dermal sinus tract, and seven LDMs were related to a split cord malformation. The embryogenetic implications of these associations are discussed.

Conclusion

LDM is a distinctive clinicopathological entity and a tethering lesion with characteristic external and internal features. We propose a new classification incorporating both saccular and flat lesions.     

Spinal cord MRI in multiple sclerosis with multicoil arrays: a comparison between fast spin echo and fast FLAIR.

OBJECTIVES: To compare the sensitivity of fast spin echo (FSE) and of fast fluid attenuated inversion recovery (fast FLAIR) in detecting spinal cord lesions in multiple sclerosis. METHODS: With a 1.5 Tesla machine and a multicoil receiver array, FSE images (with two different pixel sizes) and fast FLAIR images of the spinal cord were obtained from 13 patients with multiple sclerosis. RESULTS: Twenty three lesions (10 cervical, 12 thoracic, and one lumbar) were found in seven patients (54%) using FSE with the larger pixel size. Seventeen lesions (seven cervical and 10 thoracic) were detected in the same seven patients using FSE with smaller pixel size. Nine lesions (five cervical and four thoracic) were found using fast FLAIR in six patients (46%). All the lesions found using fast FLAIR were detected using the other two techniques and all the lesions detected by FSE with smaller pixel size were detected using FSE and greater pixel size. CONCLUSION: Fast FLAIR sequences detect substantially fewer cord lesions in patients with multiple sclerosis.     

颈髓髓内血管母细胞瘤的诊断与治疗

目的 探讨颈髓髓内血管母细胞瘤的诊断与治疗。方法 分析19例患者的MRI表现，及其手术治疗的效果。结果 这类肿瘤在MRI上可分为三型：A.空洞型，肿瘤大小不等，且继发延、脊髓空洞；B.囊肿型，为囊内小的附壁结节；C.实体型，为巨大的实体性肿瘤。所有患者均经手术全切除肿瘤，并经病理证实为血管母细胞瘤。术后患者神经系统状态好转者18例，加重1例。结论 颈髓MRI能对颈髓髓内血管母细胞瘤作出定位、定性诊断，并可将其分型，以利选择不同的手术方法；诊断时，还需与胸廓出口综合征和颈椎病相鉴别。认为颈髓髓内、即使累及延髓的血管母细胞瘤宜行积极手术治疗；手术方法随肿瘤类型不同而各异，最为重要是：需沿正确的界面分离，并应在离断供血动脉后切除肿瘤。     

Posterior fossa cystic lesions — magnetic resonance imaging manifestations

Cystic lesions of the posterior fossa remain a controversial subject as to clinical classification and diagnosis, especially for those in combination with other intracranial abnormalities. During the period of November 1985 to June 1991, 16 patients with cystic lesions of the posterior fossa were retrospectively reviewed on neuroradiological evaluation with MR images. The patients were 9 males and 7 females aged from 5 days to 15 years old (medium 1 year old). They were classified into three groups as Dandy-Walker cyst (group A, 5 patients), mega cisterna magna (group B, 4) and retrocerebellar pouch or cyst (group C, 7). Nine patients had associated intracranial anomalies, holoprosencephaly in 3 (group A, 3), dysgenesis of the corpus callosum in 4 (group A, 2; group C, 2), and occipital meningoceles in 2 (group A, 2). Hydrocephalus or ventricular enlargement was found in 10 patients (group A, 5; group B, 2; group C, 3). Surgical treatment (cyst-peritoneal shunt or cystectomy) was performed for 10 patients with clinical symptoms due to cyst. Postoperatively, clinical symptoms due to increased intracranial pressure or hydrocephalus improved in 7 patients. For long-term results, the cyst was reduced in 1 of 5 patients of group A and 2 of 5 group C. Most of our patients have a poor prognosis because of the associated intracranial anomalies or atrophy of the cerebellum.

Multiplanar MR images may provide sufficient evidence for the diagnosis of posterior fossa cysts, especially in the case of rotation or upward displacement of the cerebellar vermis. However, the cyst membrane, and the communication of fluid between the cyst and the cistern, cannot be demonstrated on MR images. Further investigation on these subjects and indications for surgical intervention is highly necessary.     

Spinal mediation of thermally induced sweating.

The sweat responses of nine patients with physiologically complete lesions of the spinal cord (six cervical and three thoracic) were recorded by two different techniques while the patients were exposed to elevated environmental temperatures. Oral temperatures, heart rate and respiration were monitored throughout the observational periods. Oral temperature invariably rose during exposure to heat and both heart rate and respiration tended to increase. Sweating was detected on all of the test areas by both the iodine-starch-paper technique and the quinizarin technique, but it was of widely varying intensity in different portions of the body. In the patients with cervical lesions sweating was generally profuse on the head and neck and occurred in progressively decreasing intensity down to the level of the umbilicus. It was invariably present, but only in very low intensity, on the lower extremity. Sweating was frequently present as a result of manipulation of the patient during the initial preparations, but this generally declined or stopped before the heat was turned on. With application of heat, sweating was recruited on previously dry areas or increased in intensity on those areas in which it was previously present. After oral temperature had increased moderately, the heat was turned off and the doors of the chamber opened widely so that the heat stimulus was suddenly removed. Despite a continued rise in oral temperature, sweating stopped or decreased dramatically. These results are interpreted to indicate the direct mediation by the isolated spinal cord of reflex sweating responses to a heat stimulus applied to the skin. The general distribution of sweating was similar to that associated with distension of the urinary bladder, and careful attention was taken to avoid this complication. The distribution of sweating on the patients with lesions in the thoracic cord was quite different, being most obvious and profuse on the lower extremities and lower trunk and completely absent from the upper trunk, head and upper extremities.     

Syringomyelia Associated with a Spinal Arachnoid Cyst

While syringomyelia is not a rare spinal disorder, syringomyelia associated with a spinal arachnoid cyst is very unusual. Here, we report a 62-year-old man who suffered from gait disturbance and numbness of bilateral lower extremities. Spinal magnetic resonance imaging (MRI) showed the presence of a spinal arachnoid cyst between the 7th cervical and 3rd thoracic vertebral segment and syringomyelia extending between the 6th cervical and 1st thoracic vertebral segment. The cyst had compressed the spinal cord anteriorly. Syringomyelia usually results from lesions that partially obstruct cerebrospinal fluid flow. Therefore, we concluded that the spinal arachnoid cyst was causing the syringomyelia. After simple excision of the arachnoid cyst, the symptoms were relieved. A follow-up MRI demonstrated that the syringomyelia had significantly decreased in size after removal of the arachnoid cyst. This report presents an unusual case of gait disturbance caused by syringomyelia associated with a spinal arachnoid cyst.     

Cystic degeneration of the telencephalic subependymal germinal layer in newborn infants.

Cystic lesions were found in the telencephalic germinal layer of 12 newborn babies. According to their location, the cysts could be divided into three groups: anterior, middle or thalamostriate, and posterior. The histological appearance of all cysts was essentially the same, but in three cases the germinal layer had a peculiar alveolar type of microcystic degeneration. A constant feature was the presence in the cyst wall of small white granulations composed of germinal cells and/or glial tissue. Cystic degeneration of the germinal layer was usually bilateral and sometimes quite extensive. After the involution of the germinal layer, these lesions are likely to persist as subependymal cysts, characterized by their specific location and the presence of glial granulations.     

Delayed repair of large myelomeningoceles

Introduction Repair of myelomeningoceles larger than 5 cm in diameter is not easy. We describe a simple technique.Patients and methods The patients with a large myelomeningocele sac were included in this study. Ten patients with a large myelomeningocele underwent surgical repair using this technique. The skin was incised in the midline proximal to the myelomeningocele sac. The incision was carried caudally and away from the midline to open the sac after identifying the fascia. The incision is carried circumferentially around the neural placode and the skin is saved as much as possible. About a 1-cm-width of the dura mater beneath the skin is left to ease the subcutaneous suturing. The dura is sutured in the midline. The skin is undermined bilaterally in the plane between the muscle and subcutaneous fat and closed with interrupted sutures in the midline.Conclusion This simple technique is appropriate for the closure of large myelomeningoceles and does not need more extensive and complex procedures.Presented at the 31st Annual Meeting of the International Society for Pediatric Neurosurgery, 14–18 September 2003, Monaco     

Post-surgical thoracic pseudomeningocele causing spinal cord compression

Pseudomeningoceles are extradural cerebrospinal fluid collections categorized into three groups: traumatic, congenital, and iatrogenic. Iatrogenic pseudomeningoceles occur after durotomy, usually after cervical or lumbar spine surgery. Although many remain asymptomatic, pseudomeningoceles can compress or herniate the spinal cord and nerve roots. We present a 57-year-old woman who had a thoracic laminectomy and discectomy. Two weeks after surgery, she presented with lower extremity weakness and gait difficulty. Physical examination revealed hyperreflexia and a T11 sensory level. MRI revealed a pseudomeningocele compressing the thoracic spinal cord. The patient underwent surgical drainage of the cyst. On follow-up, she had complete resolution of her symptoms, and MRI did not show a residual lesion. To our knowledge, this is the second documented post-operative pseudomeningocele causing symptomatic spinal cord compression of the thoracic spine. In this article, a review of the literature is presented, including four reported patients with post-traumatic pseudomeningocele causing myelopathic symptoms and 20 patients with iatrogenic pseudomeningocele that resulted in neurological decline due to herniation or compression of neural tissue. Treatment options for these lesions include conservative management, epidural blood patch, lumbar subarachnoid drainage, and lumbo-peritoneal shunt placement. Surgical repair, usually by primary dural closure, remains the definitive treatment modality for iatrogenic symptomatic pseudomeningoceles.     

Diplomyelia and diastematomyelia. Case reports]

Diplomyelia and diastematomyelia. Case report. Three cases of diplomyelia [1] and diastematomyelia [2] were studied and treated between 1980 and 1990 years. All patients had symptomatic onset in adulthood. Pathological features were caused by localization on the lesion in cervical, thoracic and lumbar segment of the spinal cord. Plain X-rays showed a variety of osseous malformations associated with spinal dysraphism. The most usefull radiographic examination was computerized tomography (CT). Indications to surgical treatment are discussed.     

Primary solitary hydatid cyst in paraspinal cervical muscles: a case report and review of the literature

Hydatid disease caused by Echinococcus granulosus and Echinococcus multilocularis commonly presents with pulmonary and hepatic cysts. Primary paraspinal muscle cysts are a rare presentation. We report a case of hydatid cyst within paraspinal muscles presenting with cervical mass and associated pain. The hydatid disease serological test was negative. Neither hepatic nor pulmonary cystic lesions were found. Radiographic findings were unspecific for hydatid cysts. Surgical resection was planned due to the provisional diagnosis of muscular cystic neoplasm. During surgery, a cyst containing a clear liquid was found. The cyst wall was excised and the surgical field was irrigated with hypertonic saline. The patient's symptoms resolved by discharge day. Postoperative pathological examinations revealed a muscular hydatid cyst.     

Pigmented villonodular synovitis of the thoracic spine

Pigmented villonodular synovitis (PVNS) is a proliferative lesion of the synovial membranes. Knees, hips, and other large weight-bearing joints are most commonly affected. PVNS rarely presents in the spine, in particular the thoracic segments. We present a patient with PVNS in the thoracic spine and describe its clinical presentation, radiographic findings, pathologic features, and treatment as well as providing the first comprehensive meta-analysis and review of the literature on this topic, to our knowledge. A total of 28 publications reporting 56 patients were found. The lumbar and cervical spine were most frequently involved (40% and 36% of patients, respectively) with infrequent involvement of the thoracic spine (24% of patients). PVNS affects a wide range of ages, but has a particular predilection for the thoracic spine in younger patients. The mean age in the thoracic group was 22.8 years and was significantly lower than the cervical and lumbar groups (42.4 and 48.6 years, respectively; p = 0.0001). PVNS should be included in the differential diagnosis of osteodestructive lesions of the spine, especially because of its potential for local recurrence. The goal of treatment should be complete surgical excision. Although the pathogenesis is not clear, mechanical strain may play an important role, especially in cervical and lumbar PVNS. The association of thoracic lesions and younger age suggests that other factors, such as neoplasia, derangement of lipid metabolism, perturbations of humoral and cellular immunity, and other undefined patient factors, play a role in the development of thoracic PVNS.     

Intradural extramedullary arachnoid cyst of the thoracic spine associated with cord compression.

In this report, a 55-year-old Caucasian women with an arachnoid cyst of the thoracic spine is presented. This cyst remained undiagnosed because of the nonspecific nature of her symptoms over approximately three months. Only when she started to complain of ataxia, a posterior fluid collection compressing the spinal cord was found in MRI. Even though preoperative diagnosis remained uncertain, this additional neurological dysfunction warranted surgical treatment. Surgery was successful with respect to in-toto removal of the intradural, extramedullary cyst, reversal of cord compression and symptoms. Histological diagnosis was of an arachnoid cyst.     

Enterogene Cyste des Halsmarkes mit Mucomyelie

Summary A woman of 22 years developed heavy pain in the neck and arms with weakness and paresthesia. She died two days after a myelography which was normal. At autopsy an enterogenous extra- and intra-medullary cyst of the cervical cord (C 3, C 4) was found. In addition the cervical cord apart from the cyst and the uppermost thoracic cord presented central clefts which were filled with mucilage. These findings are named mucomyelia and have not been reported previously.

     

Experience with endoscopic interventions in diseases of the vertebral column

The paper analyzes the results of treatment for various diseases of the vertebral column, by applying endoscopic technologies. Group 1 included 5 patients with cervical hernias appeared as cervical radiculopathy. Group 2 comprised patients with diseases of the thoracic vertebral column, including 8 patients with discal hernias, 6 with primary or metastatic lesions of the thoracic vertebrae, 2 with sympathalgia, and 5 with uncomplicated thoracic vertebral body fractures. Group 3 consisted of 86 patients with lumbosacral hernias, of whom 33 patients were endoscopically operated on and the remaining 53 patients made up a control group and were microsurgically operated on. The clinical findings were analyzed by using the current scales and questionnaires to make an objective assessment of subjective index, such as pain syndrome. A preliminary comparison of endoscopic and microscopic surgical treatments for diseases and lesions of the vertebral column demonstrate the noticeable advantages of endoscopic technology. Further development of neuroendoscopy in spinal neurosurgical care is expected to substantially enhance the efficiency of treatment due to the lower traumaticity rates of surgical interventions. Furthermore, mastering endoscopictechniques in surgery of bone tumors, but also spinal cord extra- and intramedullary neoplasms is promising, as considered by the authors.     

Clinical Experiences and Usefulness of Cervical Posterior Stabilization with Polyaxial Screw-Rod System

Objective

The objective of this study is to investigate the safety, surgical efficacy, and advantages of a polyaxial screw-rod system for posterior occipitocervicothoracic arthrodesis.

Methods

Charts and radiographs of 32 patients who underwent posterior cervical fixation between October 2004 and February 2006 were retrospectively reviewed. Posterior cervical polyaxial screw-rod fixation was applied on the cervical spine and/or upper thoracic spine. The surgical indication was fracture or dislocation in 18, C1-2 ligamentous injury with trauma in 5, atlantoaxial instability by rheumatoid arthritis (RA) or diffuse idiopathic skeletal hyperostosis (DISH) in 4, cervical spondylosis with myelopathy in 4, and spinal metastatic tumor in 1. The patients were followed up and evaluated based on their clinical status and radiographs at 1, 3, 6 months and 1 year after surgery.

Results

A total of 189 screws were implanted in 32 patients. Fixation was carried out over an average of 3.3 spinal segment (range, 2 to 7). The mean follow-up interval was 20.2 months. This system allowed for screw placement in the occiput, C1 lateral mass, C2 pars, C3-7 lateral masses, as well as the lower cervical and upper thoracic pedicles. Satisfactory bony fusion and reduction were achieved and confirmed in postoperative flexion-extension lateral radiographs and computed tomography (CT) scans in all cases. Revision surgery was required in two cases due to deep wound infection. One case needed a skin graft due to necrotic change. There was one case of kyphotic change due to adjacent segmental degeneration. There were no other complications, such as cord or vertebral artery injury, cerebrospinal fluid leak, screw malposition or back-out, or implant failure, and there were no cases of postoperative radiculopathy due to foraminal stenosis.

Conclusion

Posterior cervical stabilization with a polyaxial screw-rod system is a safe and reliable technique that appears to offer several advantages over existing methods. Further biomechanical testings and clinical experiences are needed in order to determine the true benefits of this procedure.     

Neonatal anterior cervical arachnoid cyst: case report and review of the literature

OBJECTIVE: Anterior cervical arachnoid cysts are rare in the pediatric population, with only 16 cases reported. We present the first case of an anterior cervical arachnoid cyst in a neonate and review the literature on pediatric cervical arachnoid cysts. CLINICAL PRESENTATION: A 16-day-old baby girl with a history of myelomeningocele repair progressively developed symptoms of upper extremity weakness over the course of 2 weeks. Magnetic resonance imaging (MRI) demonstrated a compressive arachnoid cyst extending from C2 to C7. INTERVENTION: The child was taken for posterior cervical laminoplasty and cyst fenestration. Intraoperatively, diffuse cervical arachnoiditis was noted. Rapid improvement in upper extremity paresis was noted within 24 h of surgery, and MRI confirmed decompression of the cyst. However, flaccid upper extremity paresis recurred within 2 weeks. MRI confirmed recurrence of the anterior cervical arachnoid cyst. The child was taken for a secondary fenestration and stenting of the cyst. Only partial improvement in arm function was noted by 1 month following reoperation. CONCLUSION: Arachnoid cysts can be effectively treated with surgical fenestration, shunting, and complete or partial excision. Rapid identification and treatment results in improvement in myelopathic symptoms; however, the most efficacious treatment modality remains unknown. Of the 17 cases of anterior cervical arachnoid cysts reported in the literature, 11 (65%) have had either prior myelomeningocele repair or a history of spinal trauma. Anterior cervical arachnoid cysts should be considered in the differential diagnosis of acute onset myelopathy in the pediatric population especially in cases with a history of spinal trauma or myelomeningocele repair.     

Giant intramedullary epidermoid extending from the brain stem to the upper thoracic spinal cord

Epidermoid cysts are benign tumors originating from ectoderm remnants. Most epidermoid cyst cases are intracranial. Spinal epidermoid cysts are uncommon and most of the reported cases are in the thoracic and lumbar spine. Occurrence of intramedullary epidermoid cysts in the cervical spine is extremely rare. An 18-year-old male with a giant intramedullary tumor extending from the brain stem to the upper thoracic spinal cord presented at our outpatient department. The patient underwent total excision of a silvery white lesion through a midline myelotomy. Histopathological examination was suggestive of an epidermoid cyst. We present a brief report of the case and discuss the relevant literature.