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[Duraplasty with Neuropatch versus autologous fascia lata for Chiari I malformation with syringomyelia: a comparative study].

OBJECTIVE: To evaluate the outcome and postoperative reaction of dural substitute (Neuropatch) applying in the treatment of Chiari I malformation(CMI) associated with syringomyelia(SM). METHODS: Forty patients of CMI associated with SM were operated in our department from Jul. 2002 to Jul. 2004. All patients underwent posterior cranial fossa decompression and duraplasty. They were divided into two groups, 20 patients being repaired with Neuropatch (Neuropatch group), and the others with autologous fascia lata (fascia group). There were 6 males and 14 females in Neuropatch group and 10 males and 10 females in fascia group. The operations were performed under general anesthesia via suboccipital approach and the extent of posterior cranial fossa decompression ranged from 20 cm(2) (5 cmx4 cm) to 35 cm(2) (5 cmx7 cm). The removal of posterior arch of atlas depended on the extent of tonsillar herniation, and the dura was opened in Y shape. The Neuropatch was cut into triangular shape, and the same sized autologous fascia lata was used in fascia group. The patches were sutured tightly to the dura matter in each group. The incision was closed layer by layer and drainage was used, if necessary. Antibiotics and hormone were routinely used. The duration of operation, postoperative fever were evaluated, the outcome of the operation was evaluated by Tator scale, and the data were analyzed with statistic software SPSS 10.0. RESULTS: There were12 patients (60%) who suffered from postoperative fever in the Neuropatch group, and 9 patients (45%) in the fascia group(chi(2)=0.902,P=0.342). Seventeen patients in each group were improved postoperatively. The duration of operation, postoperative fever and antibiotics used were compared between the two groups. No significant difference was found, but the duration of postoperative fever and the time of hormone used were different. There were no postoperative infections that occurred after the follow up for 1 to 2 years, except for one patient in fascia group who developed infective granuloma and recovered later by treatment. CONCLUSION: Neuropatch is a useful dural substitute for the repair of dural defects in the treatment of CMI associated with syringomyelia.

Arnold-Chiari Malformation↗

[Clinico-immunological and pathomorphological correlations in syringomyelia].

The authors provide the results of studying humoral immunity of the blood serum, CSF and cystic fluid and pathomorphological alterations in the spinal membranes of 16 operated patients with communicating syringomyelia, which form the basis for viewing the immune mechanism as an important component of the pathogenesis of cyst formation. The indicators of humoral immunity mirror the activity of syringomyelia pathomorphosis and can be used for estimation of the time course of changes in the process and of the efficacy of the treatment given to the patients.

Adolescent↗

[The magnetic resonance tomographic method in the diagnosis of syringomyelia].

Eighteen patients with a typical clinical picture of syringomyelia were examined by a complex of methods including magnetic resonance tomography (MRT) (T1-suspended tomograms of sagittal and transverse section). MRT made it possible to recognize in 12 patients an occlusive type of the disease with a distinctly visualized expanded cyst against the background of hernia of the cerebellar tonsils and hydrocephalus. A gliomatous variant (with multiple small cysts or without such, without the Arnold-Chiari malformation) was found in 6 patients. The MRT method allows simultaneous determination of the localization and size of the cyst, ectopia of the cerebellar tonsils, and hydrocephalus, which makes it very valuable in considering the indications for and tactics of operative treatment. MRT brings the diagnosis closer to the pathologicoanatomical one and may be used successfully in studying the clinical picture and pathogenesis of various types of syringomyelia.

Adult↗

[Syringomyelia: diagnostic value of magnetic resonance and computed tomography in 5 cases].

We present five cases of Syringomyelia, three of which are associated with Arnold-Chiari malformation, establishing a comparison between the diagnostic effectivity of CT scan and Nuclear Magnetic Resonance (NMR). In all five cases the lesion was detected by NMR, but not by CT scan. The advantages of NMR over CT scan were evaluated concluding that NMR is more sensible and should be of first choice in the diagnosis of Syringomyelia, permitting also, a better study of the extension of the cavities as well as its control after surgical treatment.

Adult↗

[MRT in syringomyelia--follow-up control after syringo-arachnoid shunt surgery with clinical correlation].

In 8 patients with syringomyelia, MR long-term follow-ups (observation period 25-46 months) were done after syringosubarachnoidal shunt operations. MR showed directly after surgery in 6 cases an extensive collapse of the syringomyelias and in 2 cases a lesser reduction of the diameter of the syrinx. During the subsequent course the size of the cavities increased again in 3 cases. The size of the syrinx visualised by MR did not correlate with the clinical status during the follow-up studies. This seems to point to an insufficient representation of the disease process by the morphological visualisation of the size of the syrinx.

Adult↗

[Significance of delayed myelography and computed tomography in the diagnosis of syringomyelia. An analysis of 19 cases].

We compared and analysed results of three diagnostic methods including Physical Examination Myelography and DMCT from 19 cases who were considered to have Syringomyelia at first visit. It was shown that DMCT is a reliable radiodiagnostic method in diagnosis of syringomyelia. The trustworthy basis can be given by DMCT to surgical treatment and diagnosis of atypical cases. Misdiagnosis may be made by DMCT for cases accompanied with intraspinal hemorrhage or instances of unsuitable scanning time. We, therefore, think that the history and the physical examination are still the main diagnostic method for this disease.

Adult↗

[A case of disproportionately large communicating fourth ventricle (DLCFV) combined with syringomyelia and Chiari malformation].

We report a rare case of disproportionately large communicating fourth ventricle (DLCFV) combined with syringomyelia and Chiari malformation. The case was a 27-year-old male who underwent ventriculoperitoneal (V-P) shunt on the right side for hydrocephalus caused by traumatic intracerebral and intraventricular hemorrhage. One month later, he became somnolent with posterior fossa symptoms (nausea, vomiting and nystagmus). CT scan demonstrated enlarged fourth ventricle, which was diagnosed as DLCFV because the ventriculogram revealed patency of the aqueduct. One and half month later a second V-P shunt was made on the left side to increase the shunt flow. He became ambulatory with a cane, although the fourth ventricle remained moderately dilated on CT scan. Two months after the additional V-P shunt, he slipped and hit the occiput and immediately became tetraparetic. The patient was treated conservatively under the diagnosis of central spinal cord injury. The MRI taken 2 months after the accident revealed Chiari malformation (type 1), syringomyelia and a dilated fourth ventricle which was compressing the brainstem. After the fourth ventriculoperitoneal (FV-P) shunt, the tetraparesis transiently improved but then again worsened. On the CT scan the syrinx did non change in size, while the size of the fourth ventricle became normal. After syringoperitoneal (S-P) shunt the patient showed a moderate improvement of tetraparesis. Unfortunately he suffered appendicitis complicated with peritonitis and all the shunts were immediately changed to external drainage. However, the patient developed meningitis and became paraplegic. The motor function of the upper extremities slightly improved by aspiration of fluid via the external drainage system from the syrinx.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Syringomyelia: a neurological and surgical spectrum.

Because of the variation in the natural history, anatomy, surgical treatment, and follow-up periods reported to date, very few firm conclusions can be drawn regarding syringomyelia. With the advent of magnetic resonance scanning, cases of syringomyelia are being detected earlier and a better understanding of the disease has been facilitated. The authors report their personal experiences with 21 patients followed for 5-20 years. No single surgical procedure appears to predictably remedy any syrinx for more than a brief period of several years. Complete collapse of the syrinx does not eradicate all symptoms. Direct treatment of the syrinx makes intuitive sense for those syrinxes that do not communicate with the fourth ventricle. On the other hand, for syrinxes that are likely to communicate with the fourth ventricle, shunting of the ventricular system, particularly where ventriculomegaly exists (i.e., any degree of radiographic evidence of ventricular enlargement), should be considered initially. Procedures such as posterior fossa decompression may be necessary as dictated by the neurological condition of the patient. In the authors' experience, shunting procedures provided better improvement, with longer duration of improvement than with posterior fossa decompression. The timing and surgical details of posterior fossa decompression remain to be elucidated.

Adolescent↗

[An autopsy case of amyotrophic lateral sclerosis associated with cervical syringomyelia].

An autopsied case of amyotrophic lateral sclerosis complicated by cervical syringomyelia was reported. The case was a 59-year-old man, who first noticed weakness of both lower extremities at 54-year-old. The weakness spread to both upper extremities within 2 years. Cervical myelography revealed multi-level cervical spondylosis and anterior fusion of C5-C7 was done. But the weakness and atrophy of proximal muscle, diminished deep tendon reflex on upper extremities, hyperreflexia and pathological reflexes on both legs, tongue fasciculation and respiratory muscle weakness developed successively, and the patient died of respiratory distress at 59-year-old. Autopsy revealed multiple independent four syrinxes located at the level between C2-C7. One of these syrinxes had ependymal cell lining and thought to be idiopathic syringomyelia. The other three syrinxes were considered to be the cavitation in association with cervical spondylotic myelopathy. Degeneration and decreasing of spinal anterior horn cells, atrophy of medullary pyramis and Bunina bodies were observed as features of typical amyotrophic lateral sclerosis. Cervical spondylosis as causative lesion of multiple syrinxes was discussed, and relationship between ALS and the syrinxes was not indicated clearly.

Amyotrophic Lateral Sclerosis↗

[Clinical and neuroradiological features of syringomyelia associated with Chiari malformation].

The clinical presentation and radiological features were analyzed in 30 cases of syringomyelia associated with Chiari malformation. None of the patients had spinal dysraphism. The age on admission ranged from 6 to 59 years with a mean of 27 years. Syringomyelia was diagnosed by CT myelography and or MRI from 1982 to 1988. The initial symptoms were skeletal abnormality (43%) such as scoliosis (12 cases) or pes cavus (one case), unilateral pain or numbness (40%) and unilateral motor weakness (17%). Frequently seen signs on admission were sensory deficit (100%), scoliosis (57%), muscle weakness (57%), muscle atrophy (37%) and lower cranial nerve palsy (40%). The neurological findings were asymmetrical in all patients. The characteristic neurological findings in the cases presenting under 20 years of age were unilateral sensory and motor deficits (61%) with decreased or absent deep tendon reflex on the same side. The localization of the syrinx in axial section varied according to the level even in the same case. In 15 cases with unilateral sensory disturbance or unilateral sensory and motor deficit, the syrinx was located in the region corresponding to the posterolateral portion on the same side as that of sensory disturbance in the cervical or thoracic level. On the other hand, in 15 cases with bilateral sensory and motor deficit, the syrinx was located in the central portion and extended into the posterolateral portion of the more affected side.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Flexed neck posture due to cervical posttraumatic syringomyelia.

A flexed neck posture, especially with prolonged sitting, developed in three quadriplegic patients as a manifestation of posttraumatic syringomyelia (PTS). This posture developed at 1, 6, and 20 years after spinal cord injury. All patients complained of increasing weakness of the neck with several hours of sitting, and all required analgesics for aching neck pain. Neck weakness preceded an ascent of the level of sensory or motor deficit in two patients, and followed it in one. Current muscle testing failed to demonstrate weakness of neck extension in any patients, but electromography revealed chronic denervation and reinervation of cervical paraspinal muscles in each patient. Magnetic resonance imaging revealed a syrinx extending from the site of injury to the medulla oblongata in each case. We conclude that a flexed neck posture can represent: (1) a loss of stamina in denervated head support musculature, (2) either a precursor or successor to the conventional signs of PTS, and (3) high cervical syringomyelia.

Adolescent↗

[Atlanto-occipital luxation and syringomyelia: 2 rare complications of cervical injury. Diagnostic and therapeutic effects. Apropos of a case].

The authors report the case of a 19 years old patient with an undiagnosed traumatic atlanto-occipital dislocation complicated after two years by a rapidly evolutive syringomyelia. This clinical case which associates two rare complications of cervical traumatology allows to discuss the problems of both pathologies especially on the following points: --The early diagnosis of the dislocation by tomography and CT scan is the only mean to treat totally these lesions and perhaps to avoid the complications like syringomyelia. --In case of delayed diagnosis, when the dislocation is "fixed" the complete decompressive surgery both by a posterior and a trans-oral way is for the authors the treatment of choice.

Adult↗

Asymmetrical growth of scalp hair in syringomyelia.

Trophic alterations in the skin take place consequent to interruption of its motor, sensory, and autonomic innervation. The hair in denervated areas is often less abundant and grows more slowly. Syringomyelia is a common cause of cutaneous trophic alterations that are often unilateral. They occur predominantly in the upper extremities and seldom involve the skin over the face and scalp. A patient with syringomyelia exhibited a unilateral impairment of pain and temperature sensation in the entire right side of the scalp. A dissimilarity of hair length between the right and left side of the scalp was noticed, with a sharp demarcation along the midline. This peculiar trophic change has not been previously described in the medical literature.

Hair↗

[Neuroradiological studies in syringomyelia].

In the years 1973--1975 20 patients with syringomyelia were treated surgically. The surgical interventions were preceded by careful radiological examinations which was often indispensable for confirmation of diagnosis, establishing of indications to operation and choice of surgical method. On plain radiograms in 16 cases cervicothoracic scoliosis was found, in 11 cases the vertebral canal was dilated in its cervical part, in 6 cases atlanto-occipital malformations were disclosed. The basic diagnostic examination was ascending myelography which was performed in 17 cases. In 3 cases the contrast medium failed to pass to the atlanto-occipital junction because of marked dilatation of the spinal cord in the cervical part. In 9 out of the remaining 14 cases radiological findings were compatible with the diagnosis of Arnold-Chiari syndrome which was confirmed during the operation. In 1 case positive contrast central pneumoencephalography was performed demonstrating a communication between the 4 th ventricle and the cavity in the spinal cord. Early detailed neuroradiological diagnosis is indispensable in syringomyelia for early surgical treatment.

Adolescent↗

Posttraumatic syringomyelia.

Posttraumatic syringomyelia is becoming increasingly recognized as a sequel to major and minor spinal cord injury, paralleling the development and widespread availability of magnetic resonance imaging as a diagnostic modality for evaluating possible spinal pathologic lesions. Delayed, subacute, or progressive neurologic deterioration in victims of traumatic spinal injury with "fixed deficits" should raise the suspicion of posttraumatic syringomyelia. Alternatively, it may present as sensory or motor complaints occurring on a delayed basis after minor spinal trauma causing no initial neurologic impairment. At our institution, we have treated six of eight patients with this condition by shunting fluid from the intramedullary cyst to the peritoneal cavity by means of a simple valveless shunt, resulting in sustained neurologic improvement in five patients.

Adolescent↗

Posttraumatic syringomyelia associated with heavy weightlifting exercises: case report.

Posttraumatic syringomyelia is a well-recognized late sequel to spinal trauma occurring in 1% to 3.2% of spinal cord injured patients. Its clinical presentation is usually marked by pain, ascending sensory loss, increased muscle weakness, and depressed deep tendon reflexes. The case of a 25-year-old man with C8 complete quadriplegia, who developed a syrinx five years after his initial injury, is presented. This patient kept a log of his daily physical workout which consisted of lifting weights of 50 to 60 pounds with his neck extensors and biceps. The diagnosis was made clinically and confirmed by magnetic resonance imaging. Repeated valsalva maneuvers from daily heavy weightlifting exercises most likely predisposed this patient to the development and extension of his syringomyelia. Dramatic improvement followed surgical placement of a subarachnoid shunt.

Adult↗

The natural history of syringomyelia.

A retrospective analysis was made of the clinical course in 24 patients with syringomyelia who had not been treated surgically. 5 patients died during follow-up, and the survivors were followed for 1 to 37 (median 9) years after diagnosis. 8 of the 19 survivors followed a slowly progressive course over 4 to 46 (median 27) years but only 3 were severely disabled. 3 patients had an intermittent progression of symptoms, often with long intervening periods of stability. 8 patients had no progression in symptoms after presentation; none of these patients was severely disabled at last follow-up. It is concluded that the natural history of syringomyelia is unpredictable. Some patients have no further progression in symptoms for many years after onset. Prolonged survival is usual even among those patients with a slowly progressive deficit. The uncertain progression of the clinical course presents considerable difficulties in the timing of surgical intervention and in interpretation of the results.

Adolescent↗

[Syringomyelia and scoliosis in children and adolescents. Apropos of 14 cases].

The authors report their experience of 14 cases of scoliosis in children or young adults in association with syringomyelia. The neurological lesion was discovered at a variable time during the development of the scoliosis which may appear to be idiopathic for several years. There were 11 cases of syringomyelia or hydromyelia and three intramedullary tumours with cyst formation. The neurological lesion must be treated first and sufficiently early to avoid the development of muscle weakness and scoliosis. The scoliosis can be treated by normal without risk of additional complications.

Adolescent↗