Aneurysmal bone cyst of the lumbar spine.

An aneurysmal bone cyst (ABC) is a benign, locally proliferative vascular disorder of non-neoplastic osseous lesions in children and young adults. Seventy-five percent of ABCs occur before the age of 20 years. They comprise 1.4% of all primary bone tumors, and commonly occur in the long bones. Spinal ABCs are much rarer. We present to you one such rare case of ABC involving the lumbar spine which was successfully treated with surgery. The clinical pathological and radiological features are described. The treatment options available are discussed.

Is there an ideal shunt? A panoramic view of 110 years in CSF diversions and shunt systems used for the treatment of hydrocephalus: from historical events to current trends.

OBJECT: The goal of this study is to evaluate whether an "ideal shunt" exists. METHODS: This is a retrospective analysis based on original papers in the field of Hydrocephalus and Shunts. Patients of all age groups, who had hydrocephalus, and underwent some form of CSF diversion were included. The study has been divided into four stages: from 1900 to 1949, 1950 to 1974, 1975 to 1999, and from 2000 to 2010. RESULTS: In stage 1 (historical era): Saphenous vein grafts, rubber conduits, and other materials were used in CSF diversions. In 1949, the first implantable shunt tube was developed by Nulsen. In stage 2 (experimental stage): the Holter valve was developed. Newer innovations were developed in relation to the ventriculo-atrial shunt, which was the preferred CSF diversion. In stage 3 (developmental stage), a large number of different design shunt systems were developed, with the aim of reducing complications. The ventriculo-peritoneal shunt had become the preferred CSF diversion. Also, the programmable valve was born. In stage 4 (era of programmable valve, there is a preference for the use of programmable shunt systems. However, shunt failure rate at 1 year being around 25 to 40%, and shunt survival at 1 and 2 years are 50-70 and 47-53% in most series. CONCLUSION: Every shunt is an ideal shunt provided the choice of the shunt used should be made by the matching performance of the shunt system to the altered profile of CSF dynamics of a given patient. The most important factor being the opening pressure.

Complication avoidance in transcallosal transforaminal approach to colloid cysts of the anterior third ventriclen: An analysis of 80 cases.

OBJECT: The objective of the present study is to analyze the complications and their avoidance in a series of 80 patients operated by transcallosal transforaminal approach to colloid cysts of the anterior third ventricle. MATERIALS AND METHODS: The surgical outcome and complications of 80 patients operated by transcallosal transforaminal approach for colloid cysts in the anterior third ventricle was analyzed. A detailed pre- and post-operative neurological assessment was done in all patients. Neurocognitive assessment of corpus callosal function was done in the last 22 cases. CT scan of the brain was done in all patients, before and after surgery. RESULTS: All patients underwent transcallosal transforaminal approach. Total excision of the lesion was achieved in 79 patients and subtotal in one. The complications encountered were postoperative seizures in six, acute hydrocephalus in four, venous cortical infarct in four, transient hemiparesis in four, transient memory impairment, especially for immediate recall in nine, mutism in one, subdural hematoma in one, meningitis in three, and tension pneumocephalus in one patient. There were two mortalities. There was no incidence of postoperative disconnection syndrome. CONCLUSION: Colloid cyst is surgically curable. With good knowledge of the regional anatomy and meticulous microsurgical techniques, there is a low mortality and minimum morbidity, when compared to the natural history of the disease. With increasing experience, most of the complications are avoidable. The limited anterior callosotomy does not result in disconnection syndromes.

Management of pediatric intracranial meningiomas: an analysis of 31 cases and review of literature.

OBJECTIVE: The purpose of this study is to understand the epidemiology, clinical features, behavior, and the prognostic factors that influence the outcome of intracranial meningiomas in children. METHODS: Thirty-one patients with age less than 18 years who underwent surgery for intracranial meningiomas during the period of at 1988-2012 at Voluntary Health Services Hospital, Chennai, India were studied. These formed 3.7 % of the total 831 cases of intracranial meningiomas operated at the institute. RESULTS: The study group included 22 (71 %) males and 9 (29 %) females. The mean age of presentation was 15 years, with the youngest being 9 months old. Two (6 %) had evidence of neurofibromatosis type 2 (NF2). The most common symptoms at presentation were seizures in 11 (35.5 %), weakness of limbs in 11 (35.5 %), and raised intracranial pressure in 10 (32 %). Convexity meningiomas were seen in 16 (51 %) and skull base in 15(49 %). Multiple meningiomas was seen in two (6 %) of the patients and intraventricular location was found in one patient (3 %). Two (6 %) had evidence of NF2. Gross total excision was achieved in 26 (83 %) and subtotal excision in 5 (17 %). WHO grade I was found in 20 (64 %) and higher grade was seen in 11 (36 %). The mean follow-up was 46.2 months. Recurrence was seen in 20 patients (64 %). In patients with higher grade or with recurrence resurgery and radiotherapy was given. Three (9 %) had multiple recurrences. On a mean follow-up of 46.2 months, 25 patients (81 %) were neurologically intact, 5 (16 %) were having moderated disability, and 1 (3 %) patient died. CONCLUSIONS: Childhood meningiomas are uncommon lesions with a slight male predominance. They can have a varied clinical presentation. Higher grade is found more frequently compared with adults. Gross total resection is the goal and in higher grade meningiomas radiotherapy helps as a good adjuvant. Though the rate of recurrence is high, resurgery and radiotherapy gives a good outcome.

Theories of cerebrospinal fluid dynamics and hydrocephalus: historical trend.

According to the CSF bulk flow theory, hydrocephalus is caused by an imbalance between CSF formation and absorption, or a block at various locations in the major CSF pathway. New theories, however, have been proposed in which minor CSF pathways may play a significant role in the development of congenital hydrocephalus. The authors review major contributions to the literature and analyze the evolution of theories of CSF dynamics in relation to hydrocephalus, dividing their development into 4 stages on the basis of historical trends. In Stage I (prior to 1950), 2 systems of classifying hydrocephalus were proposed, namely Dandy's classifications of communicating and noncommunicating hydrocephalus and Russell's nonobstructive and obstructive hydrocephalus. In Stage II (1950-1974), based on these theories of major CSF pathway dynamics, treatment focused on ventriculostomy as an alternative to reduction of CSF production by choroid plexus coagulation. In Stage III (1975-1999), some of the specific forms of hydrocephalus, especially in premature infants, were found to be unsuitable for ventriculostomy. In Stage IV (2000-2008), selection of treatment modalities evolved further, with a focus on analysis of the chronological changes in CSF dynamics and the differences in absorption pathways in the developing and mature brains. The authors focus on "minor pathway hydrocephalus" in the immature brain, differentiating it from the conventional classification of obstructive and nonobstructive "major pathway hydrocephalus."

Isolated skull base primary Ewing's sarcoma: an extremely rare location.

A 16-year-old boy presented with history of headache and blurring of distant vision. He had UMN facial paresis and papilledema. Imaging revealed a heterogenous space occupying lesion in the middle cranial fossa base with mass effect and midline shift and non homogenous contrast enhancement with erosion of the petrous bone. He underwent gross total excision of the lesion. Histopathology and immunohistochemistry were suggestive of skull base Ewing's sarcoma. Bone scan was done to rule out primary involvement of other extracranial location. We have discussed the epidemiology, presentation, management and prognosis of such cases.

Management of pediatric colloid cysts of anterior third ventricle: A review of five cases.

OBJECT: Colloid cysts are congenital benign lesions, associated with more aggressive clinical and radiological picture in children than in adults. We present our experience in management of five pediatric patients with colloid cyst of the anterior third ventricle. They have an excellent chance of surgical cure, or they can be devastating and even fatal, if not recognized on time and treated. MATERIALS AND METHODS: Five pediatric patients (aged 16 years or less) who were surgically treated for a colloid cyst, between 1987 and 2011 were analyzed. The clinical features included raised intracranial pressure in all five cases, of which one patient was brought unconscious with decerebrate posturing. Computed Tomography (CT) scan of the brain was done in all patients. The density of the lesion, enhancement with contrast and the presence of hydrocephalus were analyzed. Four patients underwent a detailed postoperative neurological assessment. RESULTS: Three patients underwent the transcallosal-transforaminal approach and total excision of the lesion. One patient underwent revision of the pre-existing ventriculoperitoneal shunt. One patient who was brought in an unconscious state, an external ventricular drain was inserted and she was ventilated. She died 4 hours after the admission. On follow-up, none of the three patients who underwent the transcallosal-transforaminal approach had disconnection syndromes or behavioral disturbances. CONCLUSION: Colloid cysts in children are rarer and more aggressive than their adult counterparts. It is surgically curable. Early detection and total excision of the lesion is a permanent cure with minimum morbidity, when compared with the natural history of the disease. The limited anterior callosotomy does not result in disconnection syndromes or behavioral disturbance.

Primary intrasellar schwannoma.

Intracranial schwannomas commonly arise from the eighth cranial nerve in the cerebellopontine angle. Schwannoma arising in the sella and extending into the suprasellar region is very rare and is easily mistaken for pituitary adenoma. To our knowledge, there have been only 12 previous reports. We present a patient with primary intrasellar schwannoma that clinically and radiologically resembled a pituitary adenoma (PA). Intra-operative findings differed from a PA, as the tumour had a firmer consistency. Gross total excision of the lesion was done via a transethmosphenoidal approach. Post-operatively the patient improved in visual acuity and visual fields. We have reviewed the literature and described the characteristics of such lesions.

Pediatric multifocal glioblastoma multiforme with fulminant course.

Glioblastoma multiforme (GBM) is the most common primary malignant brain tumor. GBM in children is less common than in adults and has a better prognosis. Pediatric GBM is a rare entity, and a multifocal development in a pediatric GBM is much rarer. We report to you one such rare case of pediatric multifocal GBM in a 5-year-old child who developed rapidly increasing multiple lesions after radiotherapy. More studies are required to study the genetic analysis, tumor behavior, management and outcome of these rare tumors.

Primary spinal intradural extramedullary lymphoma causing cauda equina syndrome.

We report a case of lumbar intradural extramedullary lesion in an 11-year-old boy who presented with cauda equina syndrome and acute bladder disturbance. He underwent emergency surgical resection of the lesion, which was proved to be a lymphoma by histopathology and immunohistochemistry. He has improved neurologically and after 1 year, he is leading a normal life with near normal neurological functions. This is the second case of primary spinal intradural extramedullary lymphoma. This is the first such case in the pediatric age group and causing cauda equina syndrome. We describe the characteristics of such tumors along with pathogenesis and management.

Management outcome of the transcallosal, transforaminal approach to colloid cysts of the anterior third ventricle: an analysis of 78 cases.

BACKGROUND: Colloid cysts are not common brain lesions and account for 0.2-2.0% of all brain lesions. Transcallosal, transforaminal approach is a safe route and the most direct path to excise third ventricular colloid cyst, without dependence on hydrocephalus. AIM: To assess the surgical outcome of patients with colloid cysts of the anterior third ventricle treated by the transcallosal, transforaminal approach. PATIENTS AND METHODS: Seventy-eight patients operated by the above approach over a period of 20 years were analyzed. A pre- and postoperative neurological assessment was done in all the patients. Neuro-cognitive evaluation of corpus callosum function was done in the last 20 patients. Computer tomography scan of the brain was done in all patients pre- and postoperatively. RESULTS: Clinical features of raised intracranial pressure without localizing signs were the commonest presenting feature in 52 (66.7%) patients. Hydrocephalus was present in 65 (83.3%) patients. All patients underwent the transcallosal, transforaminal approach, and total excision of the lesion was achieved in 77 patients and subtotal in 1. Four patients required a postoperative shunt for acute hydrocephalus. There was no incidence of postoperative disconnection syndrome. In two patients, there was recurrence of the lesion after 2 and 6 years, respectively. Two patients died in the postoperative period. CONCLUSION: Colloid cyst is surgically curable. Early detection and total excision of the lesion can be a permanent cure with low mortality and minimum morbidity, when compared to the natural history of the disease. The limited anterior callosotomy does not result in disconnection syndromes or behavioral disturbance.

Malignant Course of a Metastatic Melanoma During Pregnancy: A Case Report.

Cutaneous melanoma can metastasize to any organ, including brain and spinal cord. A 27-year-old lady, four months after conception presented with generalized seizures and was diagnosed to have subarachnoid hemorrhage. Further investigation did not reveal aneurysm. She underwent right ventriculo-peritoneal shunt for hydrocephalus and MTP for unprotected radiation from CT scan. Six weeks later she came to our institution with symptoms of dorsal compression. Imaging showed multiple intradural extramedullary spinal lesions at D3-D4, D8 and D10-D11. Surgical excision of the lesions was done and histopathology was consistent with metastatic deposits from malignant melanoma which was confirmed by immunohistochemistry studies also. Her conscious level deteriorated on the second postoperative day and CT scan showed multiple small tumor emboli with evidence of right temporal bleed and diffuse cerebral edema. In spite of aggressive treatment she could not be saved. This reported case concludes that pregnancy aggravates the clinical course of metastatic melanoma.

Multiple burr hole surgery as a treatment modality for pediatric moyamoya disease.

OBJECTIVE: To re-emphasize that indirect revascularization surgery alone, where multiple burr holes and arachnoid openings are made over both cerebral hemispheres, is beneficial in the treatment of moyamoya disease (MMD) in children. CLINICAL PRESENTATION: We report a 10-year-old boy who presented with complaints of episodic headache for the last 5 years. At the peak of his headache he had visual disturbances and acute onset weakness of left-sided limbs, recovering within a few minutes. He had no focal neurological deficits. Radiological investigations revealed abnormal findings, demonstrating the features of MMD. SURGICAL MANAGEMENT: He underwent bilateral multiple burr holes, dural and arachnoid opening over the frontal, parietal and temporal regions of each hemisphere. The elevated periosteal flap was placed in contact with the exposed brain through each burr hole. RESULTS: On 6-months follow-up he had only one episode of transient ischemic attack. Postoperative four vessel angiogram demonstrated excellent cerebral revascularization around the burr hole sites, and single photon emission computerized tomography imaging showed hypoperfusion in the right temporo-occipital area suggestive of an old infarct with no other perfusion defect in the rest of the brain parenchyma. CONCLUSION: In children with MMD this relatively simple surgical technique is effective and safe, and can be used as the only treatment without supplementary revascularization procedures. This procedure can be done in a single stage on both sides and the number of burr holes made over each hemisphere depends on the extent of the disease.

Intracranial Rosai Dorfman Disease: report of three cases and literature review.

BACKGROUND: Rosai-Dorfman Disease (RDD) is a rare idiopathic non-neoplastic histioproliferative disease characterized clinically by massive painless cervical lymphadenopathy, fever and weight loss. Extranodal involvement has also been recognized. Central nervous system (CNS) manifestations are extremely rare and patients with intracranial involvement usually present with clinical and radiological findings suggestive of a meningioma. CASE DESCRIPTION: We report our experience in the management of three patients with RDD. Two patients had dural based lesions, radiologically in favour of a meningioma, and one patient had a parenchymal lesion suggestive of a tuberculous granuloma. Treatment consisted of total excision in one case, and subtotal excision followed by conventional radiotherapy in two cases. The diagnosis was confirmed by histopathology and immunochemistry which is essential for a definite diagnosis of RDD.

Exophytic choroid plexus papilloma of the fourth ventricle presenting with cerebrospinal fluid rhinorrhea: a case report.

BACKGROUND: Choroid plexus papillomas are rare benign neoplasms that arise from the ventricular choroid plexus and are considered to be of ependymal origin. They are slow-growing lesions, are commonly associated with hydrocephalus, and are prone to hemorrhage spontaneously. Most of them are located in the posterior fossa in adults, the fourth ventricle being the most common location. CASE DESCRIPTION: We report a case of a 61-year-old male patient with a fourth ventricular exophytic choroid plexus papilloma extending caudally into the foramen magnum causing obstructive hydrocephalus. In February 2005, he presented with spontaneous CSF rhinorrhea to an ENT surgeon and underwent an endoscopic transnasal repair. Six months later, he came to us with progressive loss of vision due to raised intracranial pressure. CONCLUSION: The lesion may not be detected on CT scans and MRI scan is the imaging modality of choice. Excision of the tumor takes precedence over any attempt to repair the fistula, as many a time, the CSF leak may stop.

Intracranial epithelioid hemangioendothelioma.

INTRODUCTION: Epithelioid hemangioendothelioma (EHE) is an uncommon soft-tissue vascular neoplasm. Although a well-defined entity outside the neuraxis, its intracranial occurrence is rare. Literature review revealed 35 reported cases till date. CASE REPORT: The authors report a case of intracranial EHE in a 15-year-old girl, who presented with a short history and unusual radiology. Following radical removal, the lesion recurred within 1 month and progressed to terminal stages. This tumor is thought to have an indolent intermediate malignancy potential and such rapid progression has not been reported so far.