Vascular malformations of the spinal cord. Diagnosis and therapy

A review is given on the current diagnosis and treatment of spinal vascular malformations with special emphasis on the use of selective spinal angiography. Spinal vascular malformations are mostly encountered within the thoraco-lumbar segment of the spinal cord. Typical clinical symptoms are due to either progressive myelopathy or hemorrhage (subarachnoidal hemorrhage). The prognosis of untreated spinal angiomas is poor. Therefore, therapeutic measures are aiming to prevent bleeding of angiomas resulting in subarachnoidal hemorrhages or hematomyelia or to prevent spinal cord ischemia due to compression. Using selective spinal angiography, spinal angiomas can now be treated successfully by complete surgical extirpation or embolization procedures. The evaluation of radicular myelopathies and subarachnoidal hemorrhages of unknown origin has also considerably benefited from this technique. It is concluded that early diagnosis of spinal vascular malformations is of crucial importance for good therapeutical results.     

Clinical features and MR imaging in children with repaired myelomeningocele

The aim of the study is to define the role of associated malformations in the clinical evolution of children affected by myelomeningocele. MRI investigation of the spinal cord was carried out on 25 patients between the age of 7.3 and 18.10 with MMC repaired and followed up for at least 7 years. The relation between associated malformations demonstrated by MRI and clinical trend was analysed. The results are the following: 1) presence of asymptomatic tethered cord in all cases; 2) high frequency (92%) of Chiari malformation; 3) presence of syringomyelia in 20% of patients, symptomatic in 1; 4) presence of ventricular enlargement in 72% of cases without increased intracranial pressure syndrome. For a better therapeutic approach prospective MRI studies are needed in order to follow up associated malformations.     

Unusual malformations in occult spinal dysraphism

Two cases of occult spinal dysraphism with different clinical symptoms, signs and congenital pathologies are presented. One had malformations including scoliosis, dermoid tumor, hydromyelia, diastematomyelia, dermal sinus, low conus, vertebrae anomalies and dextrocardia. The occurrence of dextrocardia in association with occult spinal dysraphism was found to be extremely unusual. The second case is presented in relation to the rarity of teratoma with dermal sinus and tethered cord in the lumbar area. Myelography, computed tomography, (CT), Myelo CT and magnetic resonance were used in making a diagnosis.     

Simplified hepatic resection utilizing absorbable polyglycolic acid-based tape and other ligature apparatus

Central nervous system malformations have been reported in a number of inherited enzyme defects. Ethylmalonic encephalopathy, an organic aciduria of unknown pathogenesis, has not been reported previously in association with brain or spinal cord malformations. We report on 2 sibs with confirmed ethylmalonic encephalopathy and malformations of the central nervous system; one with tethered cord, the other with cerebellar tonsillar ectopia (Chiari I malformation).     

A case of diastematomyelia (split cord malformation type I) with clinical manifestation in adulthood

We present the case of a female patient with split-cord malformation type I (diastematomyelia) who developed first symptoms as an adult and worsened markedly after intramedullary injection of local anaesthetics. Our own observations are compared with the small number of cases known from the literature. We are using the morphological and clinical classification of spinal malformations of Pang et al. (1992), which is based on a uniform disturbed embryonal development [18, 19]. The 52-year-old patient presented to her family physician in September 1991 with pain in the region of the vertebral column which had developed gradually over a period of two weeks. Following unsuccessful analgetic and muscle-relaxing therapy, the family physician had the patient transferred to the orthopedic department of a hospital. Lumbar peridural infiltrations were carried out there in February 1992 for a suspected disc prolapse. Since June 1992, she had no longer been able to walk. In addition, there had also been a progredient urinary incontinence since April 1992. The spinal CT scan reveals a duplication of spinal cord starting at the level of L4 as well as a bony spur dividing the spinal cord at levels L4 and L5. MRI of the vertebral column likewise reveals a duplication of the spinal cord starting at L4 as well as a low conus and a bone spur extending from L4 to L5 is also visualized here. Each primordial spinal cord is surrounded by its own dura mater. Altogether, this led to the diagnosis of split cord malformation type I according to Pang et al. [18].(ABSTRACT TRUNCATED AT 250 WORDS)     

Vertebral body infarction as a confirmatory sign of spinal cord ischemic stroke: report of three cases and review of the literature

BACKGROUND: Acute spontaneous spinal cord syndromes often remain etiologically ambiguous despite extensive diagnostic efforts. In the previous literature five cases are described with acute spinal cord syndromes interpreted as spinal cord ischemic strokes because of association with vertebral body infarctions on MRI. CASE DESCRIPTIONS: Three cases are presented, and the literature is reviewed. In addition to an extensive diagnostic battery including an initial MRI without pathological signs, follow-up MRI at different time intervals from the onset of symptoms showed T2 hyperintense signals in vertebral bodies. Patient 1, who had plaques in the abdominal aorta, had suffered a thoracolumbar spinal infarction; this and a concomitant infarction of the left portion of T-12 could be demonstrated on follow-up MRI on day 12. Patient 2, who had incomplete transverse spinal artery syndrome below T-3, had an abnormal signal at the T-2 level of the spinal cord on follow-up MRI on day 5; this was one segment above infarction of the dorsal area of T-3, corresponding to the ascending course of the medullary artery. The spinal cord of patient 3, who had a posterior spinal artery syndrome below T-11, was unremarkable on follow-up MRI on day 14, but a T2 hyperintense signal was noted in the dorsal area of T-10. CONCLUSIONS: Vertebral body infarction represents the only confirmatory sign for the otherwise exclusionary diagnostic procedure for spinal cord ischemic stroke and must be searched for on follow-up MRI as a key to correct diagnosis.     

What''s new in pediatric neurosurgery

During the past year, there have been some significant papers dealing with pediatric neurosurgical problems. These papers deal with arteriovenous malformations, spinal cord injury, brain tumors and hydrocephalus. The papers are summarized in the following review.     

The biological resolution of malformations of the central nervous system

It has been known for years that the assembly of the nervous system is under genetic control. During the last 10 years, the genes that direct the formation of the brain and spinal cord have begun to be discovered at an amazing pace. Mutations in the fruit fly and advances in molecular genetics have led the way. Gene mutations that cause many of the malformations of the human brain and spinal cord are now known. This has many physician-scientists hoping that an understanding of cause might lead to cure.     

Cervical arachnoid cysts after craniocervical decompression for Chiari II malformations: report of three cases

OBJECTIVE AND IMPORTANCE: We describe three cases in which ventrally situated cervical arachnoid cysts led to spinal cord or cervicomedullary compression after repeat craniocervical decompression for Chiari II malformations. CLINICAL PRESENTATION: All three patients underwent craniocervical decompression when their Chiari malformations became symptomatic. The first patient developed chronic vertiginous spells and headache and was treated with repeated craniocervical decompression procedures during several years. Seven months after undergoing her third decompression procedure, she developed severe dizzy spells, which were determined to be of brain stem origin. The second patient had a small, asymptomatic arachnoid cyst anterior to the brain stem discovered at age 6 years. After undergoing repeat craniocervical decompression for headaches 8 years after undergoing his first procedure, the patient developed severe neck pain and acute quadraparesis. A third patient underwent repeat craniocervical decompression at age 14 years for cranial nerve dysfunction. Postoperatively, he acutely developed paresis of extraocular movements and incoordination of the upper extremities. All three patients were found to have anteriorly situated arachnoid cysts compressing the brain stem and/or cervical spinal cord. INTERVENTION AND TECHNIQUE: Fenestration of the arachnoid cyst or drainage with cystoperitoneal shunting adequately treated acute brain stem or cervical spinal cord compression. All three patients had achieved satisfactory relief from their acute symptoms of neural compression at their follow-up examinations. CONCLUSION: An association between spinal arachnoid cysts and neural tube defects has previously been reported. However, the development of previously undetected spinal arachnoid cysts after craniocervical decompression was unexpected. We hypothesize that extensive craniocervical decompression may alter the cerebrospinal fluid pressure dynamics in such a way that the anterior subarachnoid space, previously compressed, may dilate. Occasionally, because of perimedullary arachnoiditis, the cerebrospinal fluid may become loculated and act as a mass. Direct fenestration or shunting may successfully treat this problem, and less extensive craniocervical decompression may avoid it.     

Myxomas of the mandible and maxilla

Two cases of congenital absence of a cervical vertebral pedicle are reported. This condition includes hypoplasia of the ipsilateral posterior arch and may predispose to spinal cord injury. The radiographic and computed tomography (CT) findings are reviewed, and the importance of the ipsilateral oblique radiographic view and of CT in diagnosis is stressed.     

Intraoperative monitoring for spinal cord tumor by spinal cord evoked potential following unilateral spinal cord stimulation

We described our experiences with intraoperative spinal cord monitoring in 6 cases of spinal cord tumor. During the operation, spinal cord evoked potential following unilateral spinal cord stimulation was recorded from subdural monitoring electrodes. This series included two cases of intradural extramedullary tumor (one case each of neurinoma and of meningioma) and four cases of intramedullary tumor (2 cases of cavernous angioma, one case each of ependymoma, and of glioblastoma multiforme). Before the removal of the tumor, the spinal cord evoked potential showed lower amplitude or no response on the more affected side in all 6 cases. During the operation, the different intraoperative changes were shown on each side. The authors think that the detection of unilateral damage to the spinal cord is possible in spinal cord evoked potential using unilateral spinal cord stimulation.     

Intradural parenchymal involvement in the spinal subarachnoid space associated with primary lung cancer

BACKGROUND: Intradural parenchymal involvement (IPI) in the spinal subarachnoid space associated with primary lung cancer is rare. A retrospective study was undertaken to investigate the clinical and pathologic features of IPI. METHOD: A total of 1215 cases of primary lung cancer were studied at autopsy; the results were reviewed retrospectively. RESULTS: Twenty (1.65%) of the cases revealed IPI in the spinal subarachnoid space. The histologic diagnoses were small cell carcinoma in ten cases, adenocarcinoma in eight cases, and squamous cell carcinoma in two cases. In 14 (70%) cases, the IPI was located between the lumbar and cauda equina of the spinal cord. However, no metastases were observed in the cervical spinal cord. Brain metastasis, vertebral metastasis, and meningeal carcinomatosis were seen in 70%, 60%, and 40% of the 20 cases, respectively, suggesting that these metastases may be related to the metastatic pathway to the spinal cord. Most patients had neurologic symptoms or signs referable to IPI; IPI could be diagnosed before death in only one patient by magnetic resonance imaging. The median interval between diagnosis of lung cancer and development of IPI and median survival after the onset of neurologic symptoms referable to IPI were 415 days and 110 days, respectively. CONCLUSION: The authors retrospectively received 1215 autopsies of patients with primary lung cancer and found 20 (1.65%) with IPI.     

The hazard of persistent cigarette smoking in later life

Two cases of post-traumatic syringomyelia studied macro- and microscopically are reported and compared with three previous autopsy cases described in the literature reviewed. The histological findings suggest that the pathogenesis may be explained by a pressure gradient in a small primary cavity. Although syringomyelia is a rare complication of traumatic spinal cord lesions, it is important to draw attention to the condition because it may be improved by surgical intervention.     

Mucosal immunity in the human female reproductive tract: cytotoxic T lymphocyte function in the cervix and vagina of premenopausal and postmenopausal women

Hydromyelia is a dilation of the spinal cord central canal. In man this may be due to congenital malformations such as Dandy-Walker syndrome and Chiari malformations or may be acquired as result of infection, trauma or neoplasia. In dogs hydromyelia may be accidentally diagnosed during routine cisterna magna myelography. Hydromyelia, and its possible etiology, may be confirmed by means of computed tomography or magnetic resonance imaging. Three dogs with hydromyelia due to differing etiologies are described.     

Fibroblast growth factor-2 stimulates cell proliferation and decreases sexually dimorphic cell death in an avian song control nucleus

Congenital anorectal malformations are found in many forms, and are frequently associated with other anomalies, especially of the spinal cord, spine, and urogenital system. Decisions concerning initial management of children with anorectal malformations can be made only after accurate determination of (a) the level and type of malformation, (b) the type of fistula, (c) the developmental state of the sphincter muscle complex, and (d) the presence of associated anomalies. Magnetic resonance imaging has proven to be the only modality to answer all these crucial questions, and has contributed to a better insight in the morphology and pathogenesis of such complex congenital malformations.     

Subarachnoid hemorrhage secondary to spinal arteriovenous malformation and aneurysm. Report of a case and review of the literature

A patient with recurrent subarachnoid hemorrhage was seen initially with intermittent signs and symptoms of intracranial and spinal cord dysfunction. Myelography and spinal angiography revealed an arteriovenous malformation (AVM) and aneurysm of the spinal cord. Extensive investigation failed to reveal any intracranial lesion. The relationship of subarachnoid hemorrhage at a spinal level to the development of remote neurological abnormalities is discussed, and previous reports of aneurysms associated with spinal AVM are reviewed.     

Pharmacology of descending noradrenergic systems in relation to motor function

The function of descending noradrenergic systems in the spinal ventral horn has not been fully elucidated. We have reviewed our own findings and those of others relating to motor function of these noradrenergic systems. We studied the effects of adrenergic drugs on spinal reflexes, decerebrate rigidity, and noradrenaline release from the spinal cord in rats, and motoneuron activity in spinal cord slices isolated from adult rats. It was shown that the descending noradrenergic systems were facilitatory to the motor system, and that alpha 1-antagonistic action at the spinal cord and alpha 2-agonistic action at the brainstem inhibited spinal motor activity by blocking spinal alpha 1-receptors and by reducing the release of noradrenaline in the spinal cord, respectively.     

Ischemic spinal cord disorders

Infarcts in the territory of the anterior spinal artery usually are located in the Adamkiewicz artery. Onset is either sudden or progressive with clinical signs of deficit and spinal pain of short duration. The extent and distribution of the deficit depends on the extension and the level of the spinal lesions. A perfect knowledge of the spinal cord vascularization is required to discern the complex and variable clinical expression. Unlike cerebral vascular events, emboli or cardiac origin are rarely involved in spinal cord events. Aortic pathology, including surgery of the aorta, is however of major importance in ischaemic spinal cord syndromes. Several reports have shown that atheroma causing spinal cord ischaemia are not located in the perispinal arterial network but lie on the aorta and its spinal branches. Dissection of the aorta is found responsible in 2 to 8% of the cases. Isthma stenosis can also give spinal cord signs and syphylitic arteritis is exceptionally encountered. Other rare causes are gas emboli and fibrocalcium emboli.     

The subthalamic nucleus: a possible target for stereotaxic surgery in Parkinson''s disease

Agenesis of the sacrum is a rare anomaly that is associated with numerous visceral abnormalities, spinal cord malformation, and lower limb defects. A fatal case of sacral and lower lumbar agenesis in a 3-day-old female infant born at 38 weeks of gestation is reported. The extraneural malformations comprised an imperforate anus, a rectovaginal fistula, and musculoskeletal abnormalities, including several thoracocervical hemivertebrae and aplasia of the sacrum and the fourth and fifth lumbar vertebrae. The cervical and high thoracic spinal cord segments were normal. Disruption of secondary neurulation, possibly due to notochord dysfunction, was suggested by malformation of the ventral half of the lower thoracic spinal cord with relative preservation of the dorsal horns and, more caudally, by loss of all normal histological landmarks, including the central canal. Neither skeletal muscle nor myoblasts were found in muscle compartments that would normally have received motor innervation from the levels of the spinal cord from which anterior horn cells were absent, indicating parallel, segmental failure of myotomal differentiation in the caudal eminence.     

Extensive epidural hemangioma with skin and bone involvement. A case report

STUDY DESIGN: This is a case report. OBJECTIVE: To report and to examine the clinical findings and management of epidural cavernous hemangioma. SUMMARY OF BACKGROUND DATA: Spinal epidural cavernous hemangiomatous malformation is a very rare entity. Only 20 cases of this disease have been reported in the literature. METHODS: The case of a 20-year-old woman with spinal epidural cavernous hemangioma was reviewed. RESULTS: The clinical presentation, diagnosis, and therapy of this disease are discussed. CONCLUSION: The treatment of epidural hemangioma consists of total or subtotal excision of the lesion, depending on the relation of the lesion to the spinal cord.