Neurological Disorders DOI: 10.4172/2329-6895.1000204 ISSN: 2329-6895

Neurological Disorders DOI: 10.4172/2329-6895.1000204 ISSN: 2329-6895

olog eur ica N l D f i o s l o a r n d r e u r s o J Li-Hua, et al., J Neurol Disord 2015, 3:1 Neurological Disorders DOI: 10.4172/2329-6895.1000204 ISSN: 2329-6895 Research Article Open Access The Primary Large Fourth Ventricular Schwannoma Removed by the Cerebellomedullary Fissure Approach. Case Report and Review of the Literature Chen Li-Hua*, Ru-Xiang Xu, Wei Qun, Li Yun-Jun, Li Wen-De, Zhao Hao, Gao Jin-Bao and Yu Bin The affiliated Bayi Brain Hospital, the Military General Hospital of Beijing PLA, Beijing, 100700, PR China *Corresponding author: Chen Li-Hua, The affiliated Bayi Brain Hospital, the Military General Hospital of Beijing PLA, Beijing, 100700, PR China, Tel: 86-10-13801187508; E-mail: 13801187508@sinasn Rec date: Dec 24, 2014, Acc date: Jan 07, 2015, Pub date: Jan 09, 2015 Copyright: © 2014 Chen Li-Hua, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Abstract The objective of this study was to present a case of a solitary fourth intraventricular schwannoma with a review of the literature, and to analyzing the current theories of its origin and application of trancerebellomedullary fissure approach. The authors presented a primary intraventricle schwannoma occurring in the lateral recess of the fourth ventricle in a 53-year-old female. She presented with severe headache, dysphagia, horizontal nystagmus, ataxia and quadriparesis were detected on initial examination. Imaging studies showed a large mass in the fourth ventricle and a marked hydrocephalus. The tumor was removed by microsurgical dissection using the cerebellomedullary fissure approach at our Institution. Complete removal was achieved and no reccurrence was noted follow-up period of 8 months after surgery. To our knowledge, this is the ninth reported case schwannoma of fourth intraventricule in the literature, which is unrelated to the cranial nerves and cerebellar hemisphere parenchyma, blood vessel or to the dura, is extremely rare. The etiology and natural history of intraventricular schwannomas is not well understood. The presenting clinical, radiological and pathological features of this tumor are summarized. We have discussed the effectiveness of this approach for removal of bulky tumors of the fourth ventricle, the other cases reported in the literature are reviewed and the benefits and potential hazards of trancerebellomedullary fissure (T-CMF) approach. Intraventricular schwannomas are benign tumours that are amenable to complete surgical removal, having a good prognosis without the need of adjuvant therapy. Keywords: Intraventricular schwannoma; Fourth ventricle; Case Report Cerebellomedullary fissure; Microsurgical technique Signs and symptoms Introduction A previously healthy 53-year-old woman who was admitted to our Schwannomas are generally benign tumors of the peripheral nerve neurosurgery department of Bayi Brain Hospital in Sept, 2012. She sheath and account for 8% of all primary intracranial tumors [1,2]. presented with clinical signs of intracranial hypertension, severe Most intracranial schwannomas arise from the vestibular portion of tussive headache that was associated to vomiting episodes during one the eighth cranial nerve and are more frequently found in middle-aged month before he presented, intermittent episodic vomiting and visual females, preferentially involving the superior vestibular branch of the obscuration, progressive hoarseness and diplopia, dysphagia and did acoustic nerve. Schwannomas have been reported from various not swallowing that insertion gastric canal is mandatory, progressively unusual intracranial locations like cerebellum, ventricle, sella, brain worsening generalized weakness result in lying the bed, memory stem, etc. [3-6]. Intraventricular schwannomas are an exceedingly rare disturbances and gait instability, moderately facial paresis tumors [5,7-21], with only eight fourth intraventricular schwannomas accompanying with low nasolabial groove in the left-side, developed in being described in the literature [5,12,16,18,21-24]. That, however, the last five months before presentation. She was performed remains a challenge even for the most skillful surgeons because of the ventriculoperitoneal shunt in other hospital because of severe vicinity of important brainstem and cranial nerve structures involved hydrocephalus, began to experience progressive slightly drowsy and is particularly difficult for large intraventricular schwannomain of mentality and worsening vomiting 45 days prior to admission. She was the fourth ventricule. Here, the authors report an extremely rare also performed incision of tracheal due to pulmonary infection, primary intraventricular schwannoma originating in the fourth excessive phlegm on account of severe dysphagia at 16 days before ventricle where it did not have any attachment to the surrounding admission. There was no stigmata of a neurocutaneous syndrome and structures (cranianl nerve, blood vessel, cerebellar hemisphere there was no history in her family of inheritable neurological disorder, parenchyma), this solitary schwannoma was removed using the such as neurofibromatosis. cerebellomedullary fissure approach, and the pertinent literature is reviewed. The technique and benefits of transcerebellomedullary fissure approaches and the possible origins of this tumor in this unique Examination location are also discussed. On admission, the patient was slightly drowsy, bedridden and uncooperative neurological examination. The general physical J Neurol Disord Volume 3 • Issue 1 • 1000204 ISSN:2329-6895 JND, an open access journal Citation: Li-Hua C, Xu R-C, Qun W, Yun-Jun L, Wen-De L, et al. (2015) The Primary Large Fourth Ventricular Schwannoma Removed by the Cerebellomedullary Fissure Approach. Case Report and Review of the Literature. J Neurol Disord 3: 204. doi: 10.4172/2329-6895.1000204 Page 2 of 9 examination was normal, heart, kidney and liver function was normal. brainstem, cerebellum, and fourth ventricle was filled with the mass. No cutaneous pigmented lesions were identified. His routine The sagittal T1-weighted MRI confirmed its location in the fourth biochemical and hematological parameters were also normal. The ventricle, and demonstrated a heterogeneously enhancing lesion with a acuity and visual fields could not be performed becuase of un- cystic component, displacing the brian stem. The tumor is an cooperation. Shunt latch was founded in the right forehead. There was asymmetrical, solitary spherical tumor. The lesion had regular border features of increased intracranial pressure, funduscopy revealed with two components, the larger, periphery portion was nearly bilateral papilledema. Detailed cranial nerve examination revealed isointense to the cerebellum and the smaller, and center segment was slight bilateral horizontalis nystagmus and diplopia gazing either side, slightly hypointense relative to CSF. The lesion included particularly with gaze to the left side, moderately left facial paresis with multiloculated cystic component and had a nodular portion that an asymmetrical smile. The dysarthric speech, a weak gag reflex, and seemed to extend into cisterna magna, with perilesional oedema. The apparent quadriparesis were detected on examination. Inspection of lesion is also found to be extending through the foramen magnum up the left eye revealed a marked esotropia with an inability to abduct the to C1. The brainstem was displaced anteriorly and right side, the eye beyond the midline and right side gaze. He had bilateral upper cerebellum was displaced posteriorly. This tumor was hypo- to extremity dysmetria on finger-nose testing as well as isointense on axial T1-weighted image (T1-WI) (Figure 2A), mixed dysdiadochokinesia, and ataxia of the lower extremities on heel-shin hyperintense lesion on T2-weighted image (T2-WI). It was mixed testing, respectively. Her deep tendon reflexes were accentuation in the hyperintense on the T2-fluid attenuated inversion recovery (T2- bilateral lower extremities. FLAIR) and occupying the all part of the 4th ventricle (Figure 2B), mixed hyperintense on the diffusion-weighted image (DWI) sequences Radiological examination (Figure 2C). It was iso- to hypointense on the T1- FLAIR (Figure 2D) and iso- to hyperintense on the T2-FLAIR (Figure 2E). The tumor was Pre-operative computerized tomography (CT) scan of the brain as asymmetrical and confounding peripheral enhancement on T1-WI identified an regular isointensity to hyperdense dorsal pontomedullary (Figure 2F-H). These radiographic features and location of the tumor, tumor, projecting into the fourth ventricle, third and lateral ventricles in conjunction with the clinical findings, suggested a tentative were moderately dilated. This tumor is a well-defined, solid-cytic diagnosis of fourth ventricular ependymoma. Other diagnostic lesion, the solid component of the lesion showed a plain tumor considerations included exophytic brainstem astrocytoma, and boundary (Figure 1), within the fourth ventricle, with associated choroid plexus papilloma or epidermoid. hydrocephalus and had been performed ventriculoperitoneal shunt. Surgical technique The patient was positioned between lateral decubitus and prone position, while the head was flexed and stretched backward. A posterior midline incision was made from inion to the spinous process of C3. The patient underwent a standard midline suboccipital

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