Surgical management of syringomyelia associated with spinal adhesive arachnoiditis.

The authors describe a new surgical technique to minimise the postoperative recurrence of adhesion after microlysis of adhesion to treat syringomyelia associated with spinal adhesive arachnoiditis. A 47 year old male presented with numbness of the lower extremities and urinary disturbance and was demonstrated to have a case of syringomyelia from C1 to T2 which was thought to be secondary to adhesive spinal arachnoiditis related to a history of tuberculous meningitis. Following meticulous microlysis of the adhesions, maximal expansion of a blocked subarachnoid space was performed by expansive duraplasty with a Gore-Tex surgical membrane, expansive laminoplasty and multiple tenting sutures of the Gore-Tex graft. Postoperatively, the syringomyelia had be en completely obliterated and improvement of the symptoms had been also achieved. The technique described may contribute to improvement of the surgical outcome following arachnoid dissection by maintaining continuity of the reconstructed subarachnoid space.     

Spontaneous chronic spinal subdural hematoma associated with spinal arachnoiditis and syringomyelia

Spontaneous chronic spinal subdural hematoma is rare. We describe a case of spontaneous chronic spinal subdural hematoma associated with arachnoiditis and syringomyelia in a 76-year old woman who presented with a 14-year history of progressive myelopathy. MRI scan revealed a thoraco-lumbar subdural cystic lesion and a thoracic syrinx. The patient underwent thoracic laminectomy and decompression of the lesion, which was a subdural hematoma. A myelotomy was performed to drain the syrinx. Pathological examination revealed features consistent with chronic subdural membrane. This report attempts to elucidate the pathogenesis of chronic spinal subdural hematoma. We discuss possible etiological factors in light of the current literature and pathogenesis of both spinal subdural hematoma and syrinx formation.     

脊髓蛛网膜炎合并脊髓空洞的显微手术治疗

目的探讨脊髓蛛网膜炎合并脊髓空洞的显微手术治疗方法。方法回顾性分析经显微外科手术治疗的15例脊髓蛛网膜炎合并脊髓空洞病人的临床资料。根据脊髓蛛网膜炎症黏连的特点分为3型：I型（弥漫黏连型）10例,Ⅱ型（局限黏连型）3例,Ⅲ型（囊肿型）2例。I型行脊髓空洞一胸腔分流术,Ⅱ型行脊髓黏连松解术,Ⅲ型行囊肿探查切除术。结果术后症状明显改善13例,同术前2例。MRJ复查显示：脊髓空洞缩小12例,消失1例,同术前2例。13例术后随访8个月～3年,症状改善9例,同术前3例,加重1例。结论显微手术是治疗脊髓蛛网膜炎合并脊髓空洞的有效方法,根据分型选择合适的显微手术治疗方案,治疗效果较好。     

Familial spinal arachnoiditis with secondary syringomyelia: clinical studies and MRI findings

We report the clinical and MRI findings of two patients with familial spinal arachnoiditis. Although their initial symptoms were various, they both showed spastic paraparesis and sensory disturbance below the thoracic level. Cytokines and WBC in the CSF were studied, but they were not elevated at all. The spinal magnetic resonance images of each showed extensive arachnoiditis and a cystic structure. The other impressive features included: (i) an enhancement within the thickened arachnoid and an adhesion between the spinal cord and the dura mater, (ii) deformation of the thoracic cord where the arachnoid adhered, and (iii) secondary syrinx formation. Laminectomy may have an adverse outcome for such patients.     

Postoperative spinal adhesive arachnoiditis presenting with hydrocephalus and cauda equina syndrome

To our knowledge, the association between hydrocephalus and postoperative spinal adhesive arachnoiditis (SAA) has never been reported. Herein we describe an unusual case of a 45-year-old man with spinal adhesive arachnoiditis (SAA) who developed delayed-onset hypertensive hydrocephalus and cauda equina syndrome (CES) after multiple low-back surgeries. The patient's clinical presentation, imaging findings, surgical management, and the possible mechanisms are discussed in the light of the present literature.     

Cerebellar medulloblastoma associated with lumbosacral syringomyelia

Summary In a 9-year-old boy with cerebellar medulloblastoma, lumbosacral syringomyelia and a cystic change in the olfactory tract were found at autopsy. The cause of the syringomyelia and the olfactory tract cyst was considered to be secondary to dissemination of the tumor and its treatment.

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Zusammenfassung In einem Fall von Kleinhirnmedulloblastom bei einem 9jährigen Knaben zeigte die Autopsie eine Syringomyelie im Lumbosakralmark und eine zystische Höhle des Tractus olfactorius. Es wird angenommen, daß beide Höhlenbildungen durch die subarachnoidale Tumorausbreitung sowie durch die Bestrahlung und die Chemotherapie verursacht wurden.

     

Case of Foster-Kennedy syndrome in the course of limited adhesive arachnoiditis

A patient was observed with limited adhesive arachnitis of nontuberculous origin producing Foster-Kennedy syndrome. Ophthalmological, neurological and arteriographic investigations suggested unequivocally presence of a brain tumour. Limited non-tuberculous leptomeningitis is a very rare disease, difficult to diagnose and particularly difficult to differentiate against brain tumour. In the described case only intraoperative findings made possible the diagnosis.     

Spinal dural arteriovenous fistula associated with syringomyelia

The previously undescribed association of a spinal dural arteriovenous fistula with syringomyelia was found in a 60-year-old male, who developed increasing paresis, numbness of both lower extremities and sphincteric dysfunction. Symptoms and signs were attributed to a syringomyelia at T5-L1 and an arteriovenous spinal dural fistula at L1. The fistula was successfully immobilised with N-butyl-cyano-acrylate. Six months after the procedure, all abnormalities had nearly disappeared. Whether the relation between the fistula and the syringomyelia was coincidental or causative could not be determined.     

Scoliosis associated with syringomyelia presenting in children

The clinical presentations and radiological features of scoliosis accompanying syringomyelia were analyzed in 14 cases of syringomyelia associated with a Chiari malformation in children. Scoliosis was the initial symptom in 11 out of 16 patients (64%) with syringomyelia and present in 14 (88%) at the initial examination. The scoliosis associated with syringomyelia was characterized by a higher incidence of a single curve (6 cases, 43%) and convexity to the left (7 cases, 50%) than seen in idiopathic scoliosis. The syrinx was shifted to the convex side of scoliosis on the axial section at the middle or lower thoracie level in patients with a single curve, and at the cervical or upper thoracic level in patients with a double curve. The authors think that the scoliosis develops in children as a result of damage done to the anterior horn, which innervates the muscles of the trunk, by an asymmetrically expanded syrinx.     

Acute brainstem symptoms associated with cervical syringomyelia

Syringomyelia classically presents with slowly progressing dissociated sensory and upper and lower motor deficits. Atypical and acute manifestations have rarely been described. We report here on 3 patients with syringomyelia, who had acute and atypical brainstem symptoms with regard to the underlying disease. These symptoms occurred after acute elevation of the intrathoracic and intra-abdominal pressure, respectively, and remitted subsequently. Vertebrobasilar ischemia was initially suspected.     

Surgical treatment of syringomyelia associated with spinal dysraphism

Clinical and radiological features of syringomyelia in 15 patients with spinal dysraphism are reported. There were 8 patients with occult spinal dysraphism (lumbosacral lipoma) and 7 with spina bifida aperta (meningomyelocele). Syringomyelia with spinal dysraphism can be radiologically divided into two types according to the dysraphic state. The syrinx in the patients with occult spinal dysraphism occurred immediately rostral to the lipoma and was localized to the lower thoracic to lumbar levels, while in the meningomyelocele patients the syrinx extended from the cervical to the thoracic level. Large syrinx formation was recognized in 1 of the 7 occult spinal dysraphism cases and 3 of the 8 meningomyelocele cases. For syringomyelia with occult spinal dysraphism, 4 patients underwent syringo-subarachnoid shunting (S-S shunt, 2 cases) or syringostomy (2 cases) during an untethering operation. In the case of meningomyelocele, S-S shunts were placed in 2 patients. Collapse of the syrinx was achieved in all 6 patients who underwent S-S shunting or syringostomy. Decreased size of the syrinx was also noted in 3 occult spinal dysraphism patients who underwent untethering operations alone. In conclusion, a large syrinx in the case of spinal dysraphism should be surgically treated. S-S shunting is effective in both types of syringomyelia. Foramen magnum decompression may be an alternative method of surgical treatment for syringomyelia in patients with meningomyelocele.     

Craniofacial dysostosis with syringomyelia and associated anomalies.

A 16-year old boy had craniofacial dysostosis, hydrocephalus, and syringomyelia. Other anomalies included platybasia, a Klippel-Feil anomaly, webbed toes, and a cutaneous hemangioma. Evaluation included cerebral angiography, ventriculography, and myelography.     

Hypothesis on the pathophysiology of syringomyelia based on simulation of cerebrospinal fluid dynamics

OBJECTIVES: Despite many hypotheses, the pathophysiology of syringomyelia is still not well understood. In this report, the authors propose a hypothesis based on analysis of cerebrospinal fluid dynamics in the spine. METHODS: An electric circuit model of the CSF dynamics of the spine was constructed based on a technique of computational fluid mechanics. With this model, the authors calculated how a pulsatile CSF wave coming from the cranial side is propagated along the spinal cord. RESULTS: Reducing the temporary fluid storage capacity of the cisterna magna dramatically increased the pressure wave propagated along the central canal. The peak of this pressure wave resided in the mid-portion of the spinal cord. CONCLUSIONS: The following hypotheses are proposed. The cisterna magna functions as a shock absorber against the pulsatile CSF waves coming from the cranial side. The loss of shock absorbing capacity of the cisterna magna and subsequent increase of central canal wall pressure leads to syrinx formation in patients with Chiari I malformation.     

An efficacy analysis of posterior fossa decompression techniques in the treatment of Chiari malformation with associated syringomyelia

A retrospective study of the efficacy of posterior fossa decompression (PFD) was carried out in 132 patients with Chiari malformation (CM) with associated syringomyelia (SM). Of these 132 patients, 69 received extended PFD (large craniotomy group), and the other 63 patients received only local PFD (small craniotomy group). At the short-term postoperative evaluation (1–4 weeks) the extended PFD appeared to be more effective than the local PFD (p < 0.05). However, there was no significant difference in long-term analysis (6 months–11 years) (p > 0.05). In the large craniotomy group, there was no difference between the short-term and long-term efficacy (p > 0.05). However, in the small craniotomy group, long-term efficacy clearly improved (p < 0.05). Furthermore, patients who had undergone local PFD exhibited more obvious radiological improvement of SM (p < 0.05) and fewer postoperative complications compared to patients undergoing extended PFD (p < 0.05). Therefore, local PFD is preferable for the surgical treatment of CM with associated SM.     

Trigeminal neurinoma associated with Chiari malformation and syringomyelia.

We report a 36 year-old woman who presented with headaches and hypoesthesia of the face. MRI revealed a large dumbbell shaped trigeminal neurinoma extending into both the middle and the posterior cranial fossae. In addition, there was a Chiari I malformation and syringomyelia. Within two months of tumor resection, both the Chiari malformation and the syringomyelia resolved. The association of an intracranial space-occupying lesion with Chiari malformation and syringomyelia is reviewed.