Endoscopic third ventriculostomy for treatment of noncommunicating syringomyelia associated with a Chiari I malformation and hydrocephalus: case report and pathophysiological considerations

OBJECTIVE AND IMPORTANCE: A Chiari I malformation associated with syringomyelia and hydrocephalus is a rare condition. We report the successful use of endoscopic third ventriculostomy for the treatment of this pathological entity. The successful use of this technique in such a case has not been previously described, and the results allow us to speculate on the pathophysiological mechanism involved. CLINICAL PRESENTATION: A 34-year-old woman presented with headaches, a motor deficit of the right upper limb, and gait dyspraxia. Magnetic resonance imaging scans demonstrated dilation of all ventricles, compression of the retrocerebellar cerebrospinal fluid space, downward displacement of the tonsils, and syringomyelia. Syringomyelia involved the cervicodorsal cord below C3, with a syrinx-free segment between C1 and C3 and no enlargement of the rostral part of the central canal. INTERVENTION: Endoscopic third ventriculostomy resulted in prompt improvement of the clinical symptoms. Postoperative magnetic resonance imaging scans demonstrated shrinkage of the syrinx and return of the cerebellar tonsils to their physiological positions. CONCLUSION: This experience demonstrates that endoscopic third ventriculostomy, which is a simple, safe technique, may be the treatment of choice for associated Chiari I malformations, hydrocephalus, and syringomyelia (even the noncommunicating type).     

Chiari I malformation: a rare cause of noncommunicating hydrocephalus treated by third ventriculostomy.

OBJECT: Hydrocephalus associated with Chiari I malformation is a rare entity related to an obstruction in the flow of cerebrospinal fluid (CSF) in the foramen of Magendie. Like all forms of noncommunicating hydrocephalus. it can be treated by endoscopic third ventriculostomy (ETV). The object of this study is to report a series of five cases of hydrocephalus associated with Chiari I malformation and to evaluate the use of ETV in the treatment of this anomaly. METHODS: Five patients (four women and one man with a mean age of 29.6 years) underwent ETV for hydrocephalus associated with Chiari I malformation between April 1991 and February 1997. All patients had presented with paroxysmal headaches, which in two cases were associated with visual disorders. All patients had also presented with hydrocephalus (mean transverse diameter of the third ventricle 12.79 mm; mean sagittal diameter of the fourth ventricle 18.27 mm) with a mean herniation of the cerebellar tonsils at 13.75 mm below the basion-opisthion line. Surgery was performed in all patients by using a rigid endoscope. No complications occurred either during or after the procedure, except in one patient who experienced a wound infection that was treated by antibiotic medications. The mean duration of follow up in this study was 50.39 months. Four patients became completely asymptomatic and remained stable throughout the follow-up period. One patient required an additional third ventriculostomy after I year, due to secondary closure, and has remained stable since that time. Postoperative magnetic resonance images demonstrated a significant reduction in the extent of hydrocephalus in all patients (mean transverse diameter of the third ventricle 6.9 mm [p = 0.0035]; mean sagittal diameter of the fourth ventricle 10.32 mm [p = 0.007]), with a mean ascent of the cerebellar tonsils from 13.75 mm below the basion-opisthion line to 7.76 mm below it (p = 0.01). In addition, CSF flow was identified on either side of the orifice of the third ventriculostomy in all patients postoperatively. CONCLUSIONS: Results in this series confirm the efficacy of ETV in the treatment of hydrocephalus associated with Chiari I malformation. It is a reliable, minimally invasive technique that also provides a better understanding of the pathophysiology of this malformation.     

Treatment of Chiari malformation, syringomyelia and hydrocephalus by neuroendoscopic third ventriculostomy.

This short paper illustrates a case with cervical myelomeningocoele, a Chiari malformation (CM), hydrocephalus (HC) and cervical syringomyelia treated by neuroendoscopic third ventriculostomy (NTV) with resolution of both the hydrocephalus and the syrinx. Two similar cases are discussed. The technique is advocated for the treatment of such complex dysraphic conditions.     

Chiari Type I malformation presenting as glossopharyngeal neuralgia: case report

OBJECTIVE AND IMPORTANCE: Chiari Type I malformation is an important pathological state in which the brainstem is compressed by the cerebellar tonsil. We present a case of glossopharyngeal neuralgia caused by Chiari Type I malformation. CLINICAL PRESENTATION: A 50-year-old male patient was admitted with glossopharyngeal neuralgia. Magnetic resonance imaging studies revealed caudal displacement of the left cerebellar tonsil. INTERVENTION: Small occipital craniectomy and C1 laminectomy were performed. The left cerebellar tonsil was resected. CONCLUSION: This glossopharyngeal neuralgia was caused by compression of the lower cranial nerves and brainstem by the displaced left cerebellar tonsil. Decompression and pain relief were obtained with resection of the cerebellar tonsil. The patient was pain-free 30 weeks after the operation.     

Initial management of hydrocephalus associated with Chiari malformation Type I-syringomyelia complex via endoscopic third ventriculostomy: an outcome analysis

OBJECT: The aim of this study was to evaluate the efficacy of endoscopic third ventriculostomy (ETV) in patients with Chiari malformation Type I (CM-I) and hydrocephalus with or without syringomyelia. METHODS: The authors identified, in a prospective endoscopy database, 16 adults and children (age range 2-68 years) with CM-I and hydrocephalus that had been managed with ETV. They reviewed the clinical features and radiographic findings for all patients. Fifteen patients underwent ETV as a primary treatment, whereas 1 patient underwent the procedure at the time of shunt failure. All patients had symptomatic hydrocephalus with either aqueductal or fourth ventricular outflow obstruction. The mean duration of follow-up was 42 months. RESULTS: Fifteen patients (94%) remain shunt free following ETV for CM-I. Five (83%) of the 6 patients with a syrinx had improvement or resolution of the syrinx following ETV. Six patients (37.5%) underwent foramen magnum decompression for persistent CM-I -- or syrinx-related symptoms. There was no cerebrospinal fluid leakage or intracranial pressure-related problem following foramen magnum decompression. CONCLUSIONS: Endoscopic third ventriculostomy provides a durable method of treatment for hydrocephalus associated with CM-I. It is effective as a primary treatment, and the authors advocate its use as a replacement for routine ventriculoperitoneal shunt insertion in these patients. Management of the hydrocephalus alone is often sufficient and may obviate decompression, although a significant proportion of patients will still need both procedures.     

Familial osteodysplasia associated with trigeminal neuralgia: case report

A 24-year-old woman suffering from left trigeminal neuralgia presented with the familial occurrence of skeletal anomalies simulating Hajdu-Cheney syndrome. She was of a short stature and had low-set ears, protrusion of the bilateral temporal and occipital bones, platybasia, basilar impression, and dental anomaly. Vertebral angiography disclosed marked displacement to the left and dorsally of the basilar artery, which was confirmed during the operation to have compressed the left trigeminal nerve in the cerebellopontine angle narrowed by the unusually protruded bony structure. Cases of Hajdu-Cheney syndrome have occasionally been reported, a few combined with facial spasm, but Hajdu-Cheney syndrome with trigeminal neuralgia is very rare. The patient's family members were surveyed. The possible cause of the neuralgia with its relation to the osteodysplasia is briefly discussed.     

Endoscopic third ventriculostomy for hydrocephalus.

The authors report on 125 patients who underwent endoscopic third ventriculostomy for obstructive hydrocephalus in three Italian Neurosurgical Centers. The series includes 77 cases of primary aqueductal stenosis, 33 with triventricular hydrocephalus due to external tumor compression, and 15 with tetraventricular hydrocephalus. The operations were carried out mainly under general anesthesia, using a flexible endoscope. Decrease of size of the third ventricle and the presence of a signal void at the level of the fenestration are the main postoperative MRI findings. Signs of intracranial hypertension, increased head circumference and Parinaud syndrome respond more frequently to the endoscopic treatment. The overall rate of good results (shunt-independent patients) in this series is 86.4%; primary aqueductal stenosis (93.5%) and triventricular hydrocephalus due to external compression (84.8%) are associated to the higher rate of good postoperative results than tetraventricular hydrocephalus (53.3%). Because of the very low invasivity of this technique, the absence of postoperative mortality and the scarce and usually transient postoperative complications, the authors advise to enlarge the indications for endoscopic third ventriculostomy to all patients with obstructive hydrocephalus when the third ventricle is large enough and there are no alterations of the CSF resorption.     

Trigeminal neuralgia presenting as Chiari I malformation.

Chiari I malformation usually presents with headache, numbness, weakness and gait abnormalities. We present a 38-year-old female with trigeminal neuralgia who was found to have a Chiari I malformation. She had a foramen magnum decompression with complete resolution of her symptoms.     

Ectopic choroid plexus associated with trigeminal neuralgia: case report

Introduction

A 55-year-old man underwent a microvascular decompression procedure for a pharmacoresistant trigeminal neuralgia. Preoperative MRI showed a neurovascular conflict between the Vth nerve root and the superior cerebellar artery.

Methods

Dissection of the intracisternal trigeminal root was undertaken, and a piece of Teflon was positioned between the artery and the nerve.

Results

Choroid plexus was found squeezing the root entry zone of the Vth nerve and partially removed. The patient did not improve after the vascular decompression procedure. Trigeminal neuralgia could be due to a mechanical irritation of the intracisternal nerve root.

Conclusion

Since vascular decompression of the trigeminal root did not relieve the pain, we suggest that the presence of choroid plexus at the root entry zone of the nerve may have induced trigeminal neuralgia in this patient.     

Chiari malformation (type I) associated with bilateral abducens nerve palsy: case report

There are still many questions concerning the pathology and therapeutic strategy for Chiari malformation. We recently encountered a case of Chiari malformation associated with only bilateral abducens nerve palsy which was successfully treated by foramen magnum decompression. The pathology and mechanisms of Chiari malformation (type I) were evaluated in this case. The patient was a 12-year-old girl who had visited our institute 1 month previously complaining of diplopia. She had no particular past or family history and her general appearance was also normal. Bilateral abducens nerve palsy was the only neurological deficit. Her cerebrospinal pressure was 9 cm H2O. Neuroradiological findings revealed basilar impression, C4-6 syringomyelia and Chiari malformation (type I), but no hydrocephalus or meningocele. Foramen magnum decompression with lateral extension, upper cervical laminectomy and open duraplasty were performed. The position of both eyes recovered fully shortly after surgery, and diplopia occurred only when the patient looked sideways. Eye movements also returned completely to normal 3 months after surgery. MRI showed no syringomyelia 4 months after surgery, the upward shift of the pendulous portion of the cerebellar tonsil, and the expansion of the subarachnoidal space of the pons-oblongata junction. In conclusion, it was thought that the mechanism of bilateral abducens nerve palsy in this case was due to the strangulation and the downward traction of the pons-oblongata junction. The nerve was relieved from this traction by foramen magnum decompression.     

Unsuccessful third ventriculostomy for occlusive hydrocephalus.

For non-communicating hydrocephalus, neuroendoscopic third ventriculostomy has become a major choice. But sometimes, the procedure results in failure. Typically, impairment of a distal CSF absorption, a preexisting arachnoid membrane just below the fenestrated site and a glial scarring of fenestrated site were pointed out as a factors of failure. On the other side, the intraventricular pressure dynamics of a functioning third ventriculostomy is in the process of study. Recently some reports have noticed the importance of the flow of CSF into the prepontine cistern, mimicking the flow through the aqueduct of Sylvius. We report an unsuccessful trial of third ventriculostomy in a case with huge posterior fossa tumor.     

Successful neuroendoscopic third ventriculostomy for hydrocephalus and syringomyelia associated with fourth ventricle outlet obstruction. Case report

The authors report the use of neuroendoscopic third ventriculostomy to treat successfully both hydrocephalus and syringomyelia associated with fourth ventricle outlet obstruction. A 27-year-old woman presented with dizziness, headache, and nausea. Magnetic resonance (MR) imaging demonstrated dilation of all ventricles, downward displacement of the third ventricular floor, obliteration of the retrocerebellar cerebrospinal fluid (CSF) space, funnellike enlargement of the entrance of the central canal in the fourth ventricle, and syringomyelia involving mainly the cervical spinal cord. Cine-MR imaging indicated patency of the aqueduct and an absent CSF flow signal in the area of the cistema magna, which indicated obstruction of the outlets of the fourth ventricle. Although results of radioisotope cisternography indicated failure of CSF absorption, neuroendoscopic third ventriculostomy completely resolved all symptoms as well as the ventricular and spinal cord abnormalities evident on MR images. Neuroendoscopic third ventriculostomy is an important option for treating hydrocephalus in patients with fourth ventricle outlet obstruction.