Endoscopic treatment of a voluminous benign symptomatic cyst of the pineal region responsible for an obstructive hydrocephalus

Asymptomatic non neoplastic cysts of the pineal region are common incidental findings in adults. On the contrary, voluminous and symptomatic cysts of the pineal region are rare and their management are not well defined. We present the case of a 32-year-old woman suffering who suffered from mild intracranial hypertension, gait disturbance and vertigo for one year. The neuroradiological workup showed a voluminous cyst of the pineal region responsible for an obstructive hydrocephalus. An endoscopic etiological treatment was decided. The operation consisted in a marsupialization of the cyst in the third ventricle with a stereotactic guidance system. A frozen section of the cyst wall failed to show tumoral cells. Immediate postoperative course was uneventful. Intracranial hypertension symptoms resolved in 24 hours. Clinical examination and neuropsychological testing were normal at two years postoperatively. The two years follow-up cerebral MRI demonstrated a remnant cystic cavity without mass effect and the patency of the aqueduct of Sylvius. Endoscopic treatment of symptomatic pineal cysts constitutes an interesting therapeutic alternative in the management of this pathology.     

Symptomatic pineal cyst: case report and review of the literature

Asymptomatic cysts of the pineal region are common incidental findings in adults. In contrast, symptomatic pineal cysts are rare and their management is not well defined. We present the case of a 39-year-old woman suffering from intracranial hypertension, with visual disturbance and mild papilledema. The MR images showed a voluminous cyst of the pineal region responsible for an obstructive hydrocephalus. Endoscopic treatment with the aid of computerized neuronavigation consisting in third ventriculostomy and fenestration of the cyst was performed. Intracranial hypertension symptoms resolved in 24 hours. The one year follow-up cerebral MR images demonstrated the normalization of ventricular size with patency of the aqueduct of Sylvius. Reviewing the literature demonstrates that the endoscopic approach represents a minimally invasive and safe procedure in the treatment of symptomatic pineal cysts.     

Rare occurrence of intracerebellar colloid cyst. Case report.

Colloid cysts are rare intracerebral lesions that are preferentially encountered within the third ventricle. There are only a few reports in which colloid cysts are described in other locations such as the fourth ventricle. A symptomatic intracerebellar colloid cyst in a 45-year-old woman is described. The patient presented with headache, gait disturbance, and nausea. Neuroradiological imaging revealed compression of the fourth ventricle, hydrocephalus, and an intracerebellar cystic lesion measuring 4 x 5 cm that had a small peripheral solid portion. The cyst was successfully removed via a paramedian suboccipital approach. Postoperatively, the patient recovered quickly. The findings in the present report represent an additional example of the broad spectrum of cystic lesions encountered in the cerebellum.     

Benign symptomatic lesions of the pineal gland. Report of seven cases treated surgically

Summary Seven patients with non-neoplastic symptomatic lesions of the pineal gland have been operated on in our department since 1988. Clinical symptoms were slight and there were intermittent signs of a space occupying lesion in the quadrigeminal area, i.e. visual and gait disturbances. In three patients, obstructive hydrocephalus with related symptoms was found. Diagnosis was confirmed by use of MRI in sagittal planes, revealing a cystic lesion in five and a solid tumour in two patients. All patients were operated on without complications using an infratentorial supracerebellar approach. Histological examination showed glial cysts of the pineal gland in five patients. In the two solid specimens, normal pineal tissue was found. These lesions seem to be of special interest, as the only pathological property are their size: Both lesions — too large pineal glands — caused obstruction of the outlet of the third ventricle with subsequent hydrocephalus. Surgical treatment was curative in all cases with prompt relief of the symptoms. Clinical symptoms and signs, diagnostic and pathological findings, as well as the surgical results of these cases will be reported.     

The endoscopic intraventricular management of pineal cysts: a minimally invasive modus operandi

Background

The management of pineal cysts is still debatable, especially for asymptomatic incidental ones. For symptomatic cysts associated with hydrocephalus, the surgical management is mandatory and may include either classical microsurgical approaches to the pineal region or endoscopic trans-ventricular approaches in a minimally invasive philosophy.

Method

The authors expose a stepwise technique to treat a pineal cyst associated with an obstructive hydrocephalus in one procedure gathering a third ventriculostomy followed by an intraventricular marsupialisation of the pineal cyst.

Conclusion

This endoscopic approach allows the treatment of the hydrocephalus and the pineal cyst in one short minimally invasive procedure.     

Neuroendoscopy in pineal region tumor with obstructive hydrocephalus

Objective : Pineal region tumours are composed of a variety of tumour pathologies, for example germinoma, teratoma, pinealocytoma, pinealoblastoma, meningioma and benign pineal glial cyst. They commonly present with mass effect causing obstructive hydrocephalus or Parinaud’s Syndrome. This paper reviews seven patients with pineal region tumour who presented with obstructive hydrocephalus and who received neuroendoscopy as part of the management of their clinical condition. Method : Neuroendoscopic techniques include endoscopic third ventriculoscopy (ETV) and endoscopic third ventriculo‐cisternostomy (ETVC). Diagnostic ETV allows direct visualization, localization and biopsy of the lesion, as well as sampling of cerebrospinal fluid (CSF). Endoscopic third ventriculo‐cisternostomy allows CSF diversion for relief of obstructive hydrocephalus. Results and Conclusion : Four out of seven of the third ventriculoscopy cases had lesions successfully located and biopsy was performed in two cases. Endoscopic third ventriculo‐cisternostomy was performed in all 7 patients. Two patients with unknown pathology relied on ETVC as the only therapeutic procedure. Three patients were followed with subsequent definitive treatment (radiotherapy or surgery). All these patients showed clinical improvement or radiological relief of obstructive hydrocephalus. Minimal complications were recorded among these cases.      

Unusual intraventricular herniation of the suprasellar arachnoid cyst and its successful endoscopic management.

A child with a benign intrinsic tectal tumor and obstructive hydrocephalus developed a huge intraventricular cystic lesion following insertion of a shunt, which was complicated by a subdural-subarachnoid bleeding. The lesion was identified to be an entrapped chiasmatic cistern, which disrupted the septum pellucidum in front of the third ventricle and filled gradually the anterior horn of the lateral ventricle. The condition was successfully treated endoscopically by fenestration of the cyst and a third ventriculostomy. Probable mechanisms of such unusual cyst formation and general management strategies in patients with obstructive hydrocephalus are discussed.     

Frameless navigation and endoscopy

OBJECT: Frameless computerized neuronavigation has been increasingly used in intracranial endoscopic neurosurgery. However, clear indications for the application of neuronavigation in neuroendoscopy have not yet been defined. The purpose of this study was to determine in which intracranial neuroendoscopic procedures frameless neuronavigation is necessary and really beneficial compared with a free-hand endoscopic approach. METHODS: A frameless infrared-based computerized neuronavi- gation system was used in 44 patients who underwent intracranial endoscopic procedures, including 13 third ventriculostomies, nine aqueductoplasties. eight intraventricular tumor biopsy procedures or resections, six cystocisternostomies in arachnoid cysts, five colloid cyst removals, four septostomies in multiloculated hydrocephalus, four cystoventriculostomies in intraparenchymal cysts, two aqueductal stent placements, and fenestration of one pineal cyst and one cavum veli interpositi. All interventions were successfully accomplished. In all procedures, the navigational system guided the surgeons precisely to the target. Navigational tracking was helpful in entering small ventricles, in approaching the posterior third ventricle when the foramen of Monro was narrow, and in selecting the best approach to colloid cysts. Neuronavigation was essential in some cystic lesions lacking clear landmarks, such as intraparenchymal cysts or multiloculated hydrocephalus. Neuronavigation was not necessary in standard third ventriculostomies, tumor biopsy procedures, and large sylvian arachnoid cysts, or for approaching the posterior third ventricle when the foramen of Monro was enlarged. CONCLUSIONS: Frameless neuronavigation has proven to be accurate, reliable, and extremely useful in selected intracranial neuroendoscopic procedures. Image-guided neuroendoscopy improved the accuracy of the endoscopic approach and minimized brain trauma.     

Frameless neuronavigation in intracranial endoscopic neurosurgery

OBJECT: Frameless computerized neuronavigation has been increasingly used in intracranial endoscopic neurosurgery. However, clear indications for the application of neuronavigation in neuroendoscopy have not yet been defined. The purpose of this study was to determine in which intracranial neuroendoscopic procedures frameless neuronavigation is necessary and really beneficial compared with a free-hand endoscopic approach. METHODS: A frameless infrared-based computerized neuronavigation system was used in 44 patients who underwent intracranial endoscopic procedures, including 13 third ventriculostomies, nine aqueductoplasties, eight intraventricular tumor biopsy procedures or resections, six cystocistemostomies in arachnoid cysts, five colloid cyst removals, four septostomies in multiloculated hydrocephalus, four cystoventriculostomies in intraparenchymal cysts, two aqueductal stent placements, and fenestration of one pineal cyst and one cavum veli interpositi. All interventions were successfully accomplished. In all procedures, the navigational system guided the surgeons precisely to the target. Navigational tracking was helpful in entering small ventricles, in approaching the posterior third ventricle when the foramen of Monro was narrow, and in selecting the best approach to colloid cysts. Neuronavigation was essential in some cystic lesions lacking clear landmarks, such as intraparenchymal cysts or multiloculated hydrocephalus. Neuronavigation was not necessary in standard third ventriculostomies, tumor biopsy procedures, and large sylvian arachnoid cysts, or for approaching the posterior third ventricle when the foramen of Monro was enlarged. CONCLUSIONS: Frameless neuronavigation has proven to be accurate, reliable, and extremely useful in selected intracranial neuroendoscopic procedures. Image-guided neuroendoscopy improved the accuracy of the endoscopic approach and minimized brain trauma.     

Hydrocephalus and racemose cysticercosis: surgical alternative by endoscopic third ventriculostomy

BACKGROUND AND PURPOSE: Hydrocephalus is a frequent and potentially serious complication of neurocysticercosis. Its treatment often requires ventricular shunting. The complication rate is high due to obstruction or material infection, which may justify endoscopic third ventriculostomy (ETV). OBSERVATION: We report a case of obstructive hydrocephalus in a 46-year-old man in the context of racemose cysticercosis, presenting with headaches and transient disorders of consciousness. Imaging showed cystic lesions of the cisterna magna, responsible for hydrocephalus which was treated effectively by ETV. Treatment with albendazole decreased the volume of the cisterna magna cysts. RESULTS: The patient was followed for 6 years after ETV with no recurrence of hydrocephalus despite two more symptomatic episodes of the disease with extension of the cysts into the lumen of the fourth ventricle and into the perispinal subarachnoid spaces, effectively treated by albendazole each time. CONCLUSIONS: Treatment of obstructive hydrocephalus secondary to cerebral racemose cysticercosis by ETV seems to be an effective and safety technique. The role of ETV should be evaluated in this indication.     

Multiple epidermoid cysts located in the pineal and extracranial regions treated by neuroendoscopy

A 22-year-old woman presented with a rare case of multiple epidermoid cysts located in the pineal and extracranial regions. Magnetic resonance (MR) imaging showed a lesion in the pineal region as hypointense on the T(1)-weighted image and hyperintense on the T(2)-weighted image, without enhancement. Neuroendoscopic treatment was performed under a diagnosis of pineal cyst. However, the cyst wall was too thick to perforate, although third ventriculostomy was performed. Diffusion-weighted MR imaging demonstrated the lesions in the pineal and extracranial regions as marked hyperintensity. The diagnosis was epidermoid cyst. Subsequently, neuroendoscopic treatment of the pineal epidermoid cyst was performed. Careful preoperative diagnosis of epidermoid cysts based on diffusion-weighted MR imaging is required.     

Arachnoid cyst of the craniocervical junction: case report.

OBJECTIVE AND IMPORTANCE: An arachnoid cyst at the craniocervical junction presenting with obstructive hydrocephalus as a result of blockage of the outflow of the fourth ventricle is described. This is a very rare anatomic site, with only five other cases described in the literature. CLINICAL PRESENTATION: A 37-year-old woman presented with a 9-month history of severe neck pain, persistent vomiting, visual disturbances, and numbness of the nose, cheek, and lips. She had severe bilateral papilledema on ophthalmoscopy. Magnetic resonance imaging revealed a midline cystic lesion extending down to C2. INTERVENTION: The patient underwent posterior fossa craniectomy and excision of the arachnoid cyst. She made a full recovery and was asymptomatic at follow-up examination. CONCLUSION: The symptomatology of these rare craniocervical arachnoid cysts and their development are discussed.     

Evaluation of complicated renal cyst: a comparison of CT and MR imaging]

We report 12 cases of renal cystic lesions that could not be diagnosed preoperatively. Sonography, computed tomography (CT), magnetic resonance (MR) imaging, and angiography were performed in all cases. Angiography was not helpful in evaluating cystic lesions, because all cystic lesions were hypovascular or avascular within the cystic mass. CT was useful in depicting the fine structural abnormalities, and especially bolus-contrast CT study was useful in the differentiation of cystic renal cell carcinoma from simple renal cyst. However, there were 5 false positive cases. MR imaging is superior to CT in distinguishing a hemorrhagic cyst or multiloculated cystic mass. In conclusion, CT is currently the primary imaging modality for evaluating complicated renal cysts, and surgical exploration is warranted for the undiagnosed cystic lesion by CT. However, MR imaging also has an important role in detecting the benign complicated cyst. Therefore if complicated cyst is thought to be benign by MR imaging, parenchyma-sparing surgery should be considered preoperatively.     

Resolution of a fourth ventricle epithelial cyst after ventriculoperitoneal shunting: case report

BACKGROUND: Symptomatic cysts of epithelial origin occurring in the fourth ventricle are very rare. When such a cyst is encountered, the treatment strategy includes surgical removal or fenestration of the cyst into subarachnoid space. CASE 1: A 23-year-old male was diagnosed as having a cyst located in the fourth ventricle causing hydrocephalus; the patient underwent cyst removal via craniotomy. The histopathologic diagnosis was neuroepithelial cyst. Because clinical and neuroradiological findings persisted, he underwent VP shunting. The cyst disappeared and did not recur. CASE 2: A 54-year-old woman was diagnosed as having a cystic mass in the fourth ventricle and dilatation of the ventricles. Magnetic resonance imaging showed the same findings as those of the first case. The patient refused craniotomy for total mass excision. Therefore, a VP shunt was applied. Postoperatively, the clinical findings and hydrocephalus improved, and complete disappearance of the cystic mass was observed unexpectedly. Both cases had 2 years of follow-up. CONCLUSION: There is no proven mechanism to explain resolution of fourth ventricle cysts after a supratentorial VP shunting. We hypothesize that disappearance of the cyst could result from rupture of its wall because of pressure gradient, which might be facilitated by a VP shunt. The current report should not be taken as an argument against cyst removal, which is the established way of treatment. However, considering that the pathogenesis and pathophysiology of these cysts are unclear, VP shunting should be considered especially for recurrent cases accompanied by hydrocephalus.     

Endoscopic surgery of third ventricle lesions

The endoscopic approach of the tumors of the third ventricle interests mainly the colloid cysts but also offers the possibilities of biopsies. Twenty two patients (16 men and 6 women, average age 41 years) presenting with hydrocephalus related to a tumor of the pineal area were treated by a ventriculostomy with attempt at biopsy : they are outside of the limits of this report. Twenty two other patients (15 men, 7 women, average age 39 years) were operated on from 1994 to 1999 for a colloid cyst, and 2 of them were admitted in emergency in sudden coma. The CT scan showed a colloid cyst (hyperdense in 16 patients) associated with an hydrocephalus, except for a patient previously shunted. The diameter of the cyst varied from 4 to 50 mm (average of 20 mm). All the patients were operated on using a rigid endoscope. Among the 20 patients presenting a tumor of the pineal area, a biopsy was possible only in 4 cases (20%). There were no hemorrhage nor neurological disorders. In all the cases, the size and the number of the specimens were sufficient to allow the histological diagnosis. For the patients presenting with colloid cyst, the average follow-up is 2 years. All the preoperative symptoms disappeared except for the memory disorders which were improved. The post-operative Evans index decreased significantly. No residual cyst was observed on the post-operative MRI in 14 patients (63%). Among these patients, an asymptomatic recurrence was observed and remained stable after 44 months of follow-up. A residual cyst was observed in 8 patients (36%), with a diameter from 5 to 25 mm (average 9 mm). No patient required a shunt procedure, and no patient presented hemorrhagic complication. Endoscopy is especially useful in the first line treatment of the colloid cysts of the third ventricle.     

Neuroepithelial (colloid) cyst of the third ventricle in identical twins. Case report

Colloid cysts of the third ventricle are described in middle-aged twin brothers. One of them presented with recurrent attacks of headache. In this patient the cyst had reached a size large enough to obstruct the cerebrospinal fluid pathway, resulting in hydrocephalus. The twin brother, although asymptomatic, was suspected of the anomaly and investigated because of the similarity of his ocular signs. The diagnosis was confirmed by computerized tomography in both the patient and his brother. The latter proved to have a smaller colloid cyst situated anteriorly in the third ventricle with no obstructive hydrocephalus. The patient was successfully operated on, while the brother is still under observation. Both brothers have had bilateral cataracts, retinal detachments, and left lateral rectus palsies. The familial occurrence of colloid cysts and their association with these ocular findings have apparently not been described before.     

Paired colloid cysts of the third and lateral ventricles

We report the case of a man of aged 27 years who presented with obstructive hydrocephalus caused by a colloid cyst of the third ventricle. He was found to have an additional and larger colloid cyst lying adjacent to it, but within the lateral ventricle. The contents of the two cysts were of different consistency. We have been able to find only one previous report of more than one colloid cyst occurring in the same patient and none where one of the cysts lay within the lateral ventricle.     

Spontaneous rupture of a presumed colloid cyst of the third ventricle--case report

An 83-year-old man presented with gait disturbance, dementia, and urinary incontinence that had progressed over 2 months. Computed tomography (CT) of the brain revealed hydrocephalus due to a well-demarcated, round hyperdense mass in the third ventricle, which was not enhanced by contrast agent. Ten days after the initial evaluation, CT revealed that the cyst in the third ventricle had disappeared. Magnetic resonance imaging revealed spontaneous rupture of the lesion and remnants of cyst wall anchored to the anterior roof of the third ventricle. Thereafter, the symptoms of hydrocephalus subsided. However, 6 months later the patient's condition gradually deteriorated and the ventricles dilated without any evidence of tumor regrowth. Surgical intervention was not performed as the family of the patient withheld consent. The natural history of colloid cysts of the third ventricle remains unclear. Spontaneous rupture of a presumed colloid cyst of the third ventricle should be considered when planning treatment.     

Endoscopic third ventriculostomy is the treatment of choice for obstructive hydrocephalus due to pediatric pineal tumors.

Pineal lesions in the pediatric patient are often complicated by the development of hydrocephalus due to obstruction of the aqueduct or the third ventricle by tumor masses. In such cases, hydrocephalus treatment has the highest priority and should be performed prior to any surgical treatment of the pineal tumor itself. The golden standard in obstructive hydrocephalus treatment remains placement of a temporary or permanent cerebrospinal fluid shunt, although there are many long-term complications associated with a shunt system. To avoid these and to render the patients independent from a failure-prone shunt system, we employed endoscopic third ventriculostomy for permanent relief of elevated intracranial pressure prior to surgical removal of the pineal lesions. The present study summarizes the results of this approach in 7 pediatric patients with obstructive hydrocephalus. No complications of the endoscopic procedure were encountered, and the ventriculostomy remained patent in all cases, as confirmed by motion sensitive MRI. The advantages of endoscopic third ventriculostomy as compared with other techniques are discussed, and its increasing role in the management of children with space occupying lesions of the pineal region is defined.