Evaluation of surgically treated primary spinal cord tumors in a single Indian institution: A case series study of 178 patients.

Introduction: Primary spinal cord tumors are rare tumors that are usually heterogeneous having variable histopathological subtypes. Rapidly, growing space-occupying lesions in the spinal canal cause severe loss of function. This study aimed to analyze all adult patients diagnosed with primary spinal cord tumors and to describe their location, symptoms, histopathological types, surgical outcome, complications, recurrence in a single institution, and prognostic factors. Materials and Methods: The study was a retrospective study of 178 patients with spinal tumor who underwent surgical intervention at the department of neurosurgery at a tertiary care institute from 2014 to 2021. Patient demographic characteristics, symptoms, radiological features, spinal level of tumor, spinal compartment, preoperative and postoperative McCormick grade, and complications were recorded, and a comparison of outcomes after surgical intervention was done. Results: In our study, 97 patients were males and the rest of the 81 patients were females. The mean age at the time of surgery was 43.5 years. The thoracic region of the spinal canal was the most involved with 67 (37.64%) cases, whereas intradural extramedullary was the most common (65.73%). Schwannoma was the most common histopathological subtype (46.06%). Total excision was achieved in 154 cases. A total of 37 patients were in Grades 4 and 5 of modified McCormick's grade preoperatively. However, postoperatively, there were only 17 patients in this group. Two patients with cervical intramedullary tumor expired in the postoperative period. Conclusion: Early surgical intervention with sound microneurosurgical skills gives good outcomes independent of the type and location of tumor. Better outcomes are seen in extradural tumors, early surgical intervention, tumors better amenable to total resection, and in patients with better preoperative neurological status.

Clival Tuberculosis: A Case Report.

A 39-year-old female presented with complaints of occipital headaches, diplopia, numbness over left half of face and deviation of face to the right. On examination she had hypoesthesia over left half of face, associated with bilateral abductor and left facial palsy. Neuroradiology showed a well-defined lytic lesion involving the clivus and adjacent sphenoid sinus and sella. The patient underwent an endoscopic transnasal decompression of the clival lesion. Intraoperative squash preparation was reported to show tuberculous granulation, which was confirmed on postoperative histology. The patient was advised anti-tubercular therapy. At 12 months follow up neuroradiology showed a near total resolution of the clival lesion. The patient had completely recovered from her cranial nerve deficits. Tuberculous involvement of spheno-clival region is rare and the authors' literature search has yielded only three previous similar case reports. A surgical decompression followed by anti-tubercular therapy is the recommended approach for management of clival tuberculosis. The relevant literature on the subject is presented.

Transthoracic approach for ventrally situated paraspinal extradural hydatid cyst of the dorsal spine.

A 40-year-old female presented with back pain without any neurological deficits. Radiological investigations revealed a ventrally situated paraspinal lesion causing widening of the right D4 neural foramen. She underwent a right posterolateral thoracotomy and excision of the lesion in toto, which was reported as a hydatid cyst on histology. Ventrally situated paraspinal extradural hydatid cysts are rare. A Transthoracic surgical exposure offers a direct approach for complete excision of the lesion, minimizing the chances of rupture and spillage.

Congenital meningocoele presenting in an adult.

A 53-year-old male patient presented with low back pain radiating to both lower limbs for 2 years and urinary incontinence for 2 months. He had swelled over his lower back since birth. The neurological examination revealed a decreased perianal sensation. Local examination of the lumbar swelling showed a brilliantly transilluminant, cystic midline swelling in the lumbar area with underlying spina bifida. Magnetic resonance imaging revealed a low-lying conus at L3 level associated with spina bifida at L5 and a meningocoele sac. The patient underwent excision of the meningocoele and detethering of cord by extirpation of hypertrophied filum terminale. The patient had an immediate relief of his symptoms. At 3 month follow-up the lumbar wound had healed well, and there was a significant improvement in the perianal hypoesthesia. The adult presentation of a congenital meningocoele and spinal dysraphism is rare, especially in the fifth decade. The possible causes of this delayed presentation are analyzed, and the relevant literature on the subject is presented.

Hemangiopericytoma of the cervical spine.

A 28-year-old male presented with neck pain and dysesthesias in the right upper limb. On examination, he had a firm, well-defined midline posterior cervical mass discernible on palpation at the mid-cervical level. He had no neurological deficit. Neuroradiology revealed a variegated enhancing cervical mass is arising from C3 lamina. The mass extended into the right extradural space eroding the C3 lamina and posteriorly into the intermuscular plane. The tumor was excised totally. Histopathology of the tumor showed features of hemangiopericytoma (HPC). The patient underwent postoperative radiotherapy. Primary osseous spinal HPC are rare malignant extra-axial tumors that tend to recur and metastasize. Only two cases of primary osseous HPC have been reported earlier to involve the cervical spine. The clinical presentation and management of the present case with a review of the literature is presented.

Tethered cord due to caudal lipomeningocele associated with a lumbar dural arteriovenous fistula.

A 29-year-old man presented with progressive paraparesis associated with sensory impairment in both lower limbs for the past 2 years. He was experiencing the sensation of incomplete urinary evacuation. The patient had undergone an earlier operation for a lumbar lipomeningocele at birth. Magnetic resonance images of the lumbosacral spine showed a low-lying conus medullaris adherent to a caudal lipoma. There was a leash of abnormal vascular channels in the adjacent subarachnoid space. The patient underwent spinal angiography that revealed a dural arteriovenous fistula (AVF) principally fed by the left fourth lumbar (L-4) radicular branch. At surgery the cord was detethered by disconnection of the sacral lipoma. The dural fistula was obliterated by occlusion of the L-4 radicular feeder close to the nidus of the fistula. Postoperatively, the patient experienced an immediate relief of sensation of tightness in both lower limbs. There was a gradual improvement of power and sensation at the 6-month follow-up examination. According to the authors' literature search, the present case is a unique report of a rare association of spinal cord tethering due to a caudal lipoma associated with a lumbar dural AVF. The present report discusses the etiopathology, presentation, and management of this case.

Traumatic cervical epidural hematoma in an infant.

An 8-month-old male infant had presented with a history of a fall from the crib a fortnight ago. He had developed progressive weakness of both lower limbs. On examination, the infant had spastic paraplegia. Magnetic resonance (MR) imaging of the cervical spine showed an epidural hematoma extending from the fourth cervical (C4) to the first dorsal (D1) vertebral level with cord compression. The patient had no bleeding disorder on investigation. He underwent cervical laminoplasty at C6 and C7 levels. The epidural hematoma was evacuated. The cervical cord started pulsating immediately. Postoperatively, the patient's paraplegia improved dramatically in 48 hours. According to the author's literature search, only seven cases of post-traumatic epidural hematoma have been reported in pediatric patients, and our patient is the youngest. The present case report discusses the etiopathology, presentation, and management of this rare case.

Intramedullary conus metastasis from carcinoma lung.

A 46-year-old male presented with progressive paraparesis and sensory impairment in both lower limbs since 2 months. He had urinary and bowel incontinence. On examination he had flaccid paraplegia with a sensory level at 11(th) dorsal vertebral level. Magnetic resonance imaging (MRI) scans of the lumbosacral spine showed an enhancing intramedullary lesion in the conus. The patient underwent excision of the conus mass. Histopathology confirmed the tumor to represent a poorly differentiated metastatic carcinoma from an unknown primary. A positron emission tomography-computed tomography (PET-CT) scan of the whole body revealed hypermetabolic activity in the hilum of the right lung confirmed to be a lung carcinoma on a CT-guided biopsy. The patient was undergoing chemoradiation at 1 month follow-up. The author's literature search has yielded only four other case reports of conus metastasis of which only one is in English literature. The present case report and review of literature are presented.

Multicompartmental cerebrospinal fluid dissemination of cerebellar pilocytic astrocytoma at presentation.

A 26-year-old male presented with a rare cerebellar pilocytic astrocytoma with multicompartmental subarachnoid metastases. Cerebrospinal fluid dissemination of low grade astrocytoma at presentation is rare in adults. In the present patient, clinical and neuroradiological follow-up at 4 years, without adjuvant treatment, revealed non progression of tumors. The occurrence of benign dormancy, low grade tumor histology and multifocal involvement of the neuraxis is rare. Close clinical observation is the best approach for management of such a patient. The relevant literature is discussed.

The importance of the cortical subarachnoid space in understanding hydrocephalus.

OBJECT: In this paper the authors define the role of the cortical subarachnoid space (CSAS) in poorly understood forms of hydrocephalus to cerebrospinal fluid (CSF) dynamics to improve understanding of the importance of the CSAS and its role in selecting patients for endoscopic third ventriculostomy (ETV). The secondary purpose of this work was to define testable hypotheses to explain enigmatic disorders of CSF dynamics and to suggest how these concepts could be tested. METHODS: The magnitude of the contribution of the CSAS is explored using the solid geometry of concentric spheres. With this starting point, clinical conditions in which CSF dynamics are not easily understood are explored regarding the potential role of the CSAS. Overall, problems of CSF dynamics are easily understood. Insights may be gained when the results of a pathological process or its treatment vary from what has been expected. RESULTS: Acute changes in ventricular volume at the time that hydrocephalus develops, the failure of shunts, and the changes in ventricular volume with shunt repair may occur very rapidly. Changes in the volume of water in the brain, especially in the brain substance itself, are unlikely to occur at this rapid rate and may be interpreted as a simple redistribution of the CSF between the ventricle and CSAS with no initial change in the actual volume of brain parenchyma. Problems such as pseudotumor cerebri, shunt failure with nonresponsive ventricles, and negative-pressure hydrocephalus can be explained by assessing the ability of ventricular CSF to flow to the CSAS and the ability of this fluid to exit this compartment. Ventricular enlargement at the time of shunt failure implies a failure of flow between the ventricles and CSAS, implying that all patients who show this phenomenon are potential candidates for ETV. CONCLUSIONS: The important role of the CSAS in the pathophysiology of various forms of hydrocephalus has been largely ignored. Attention to the dynamics of the CSF in this compartment will improve understanding of enigmatic conditions of hydrocephalus and improve selection criteria for treatment paradigms such as ETV. These concepts lead to clearly defined problems that may be solved by the creation of a central database to address these issues.

Concurrent use of a lumboperitoneal shunt with programmable valve and ventricular access device in the treatment of pseudotumor cerebri: review of 40 cases.

OBJECT: The authors evaluated the efficacy of treating patients with pseudotumor cerebri (PTC) and headaches due to increased intracranial pressure (ICP) by using a lumboperitoneal (LP) shunt with a programmable valve and ventricular access device (VAD). METHODS: Forty patients in whom PTC was diagnosed were treated using LP shunts with programmable valves and wand-guided placement of a VAD. All patients had papilledema and high opening pressure during spinal tap. The mean follow-up was 18 months (range 3-72 months). When patients complained of headaches that suggested shunt malfunction, the ventricular reservoir was tapped at bedside to assess ICP. The programmable valve was adjusted based on the patient's headache and ICP. RESULTS: The VAD was tapped in 21 patients, and the LP valve was redialed in 14. Shunt malfunction was diagnosed accurately. The 10 patients undergoing revision were all found to have shunt obstruction except 1 whose valve was replaced because it could not be reprogrammed. No patient treated with a shunt developed a Chiari malformation. The VAD was exposed in 4 patients with infection or wound breakdown. The LP shunt was revised in 2 patients who developed a pseudomeningocele. In 1 patient, a small bowel obstruction responded to conservative management. Seven patients had headaches despite documented normal ICP. That is, the headaches were unrelated to shunt function, and these patients were referred to a pain management clinic. CONCLUSIONS: Lumboperitoneal shunts with programmable valves effectively controlled the outflow of lumbar cerebrospinal fluid to ameliorate the symptoms of PTC. The VAD permitted assessment of ICP and thus, indirectly, LP shunt function, and benefits outweighed risks. The programmable valve permitted cerebrospinal fluid flow to be adjusted based on patients' clinical status and ICP to be measured by the VAD.

Chudley McCullough syndrome.

BACKGROUND: Chudley McCullough syndrome is characterized by partial agenesis of the corpus callosum, interhemispheric cyst, cerebral and cerebellar cortical dysplasias, and hydrocephalus. This syndromic form of sensorineural hearing loss is rare. Our literature search has located 13 siblings in 6 families with this syndrome. We report a case of Chudley McCullough syndrome and discuss the relevant literature. It is amply clear from the review of literature that treatment of ventricular dilatation or drainage of arachnoid cysts in these cases will not improve the sensorineural hearing loss. CASE ILLUSTRATION: A 14-month-old female child presented with bilateral profound sensorineural hearing loss. Neuroimaging revealed partial agenesis of the corpus callosum, colpocephaly, and an interhemispheric arachnoid cyst. These associations suggested a diagnosis of Chudley McCullough syndrome.

Cranial migration of complete ventriculo-peritoneal shunt assembly.

A 10-month-old male child with severe congenital hydrocephalus due to aqueduct stenosis presented with cranial migration of the entire ventriculo-peritoneal (VP) shunt. The complete shunt assembly, including the shunt chamber, was noted in the dilated ventricles. The migrated shunt was left in situ. A VP shunt was performed on the opposite side. The complete intraventricular migration of a VP shunt is a rare complication. This complication may be avoided by firm anchoring of the connector sites to the periosteum, and avoidance of large burr holes and dural openings. The possible mechanisms of such an event and the relevant literature are discussed.

Dandy-Walker malformation associated with polycystic kidneys: Goldston syndrome revisited.

A 32-year-old woman was diagnosed with chronic bilateral frontoparietal and interhemispheric subdural haematomas (SDH). Abdominal ultrasonography revealed chronic renal failure due to bilateral large cystic kidneys. Brain MRI demonstrated Dandy-Walker malformation associated with hypoplastic vermis, in addition to the chronic subdural haematomas. This association of polycystic kidneys with Dandy-Walker malformation has been referred to as Goldston or cerebrorenal syndrome. The case is rare and is the first to be reported in an adult patient. The relevant literature is reviewed.

Primary intramedullary primitive neuroectodermal tumor of the cervical spinal cord. Case report.

Primary intramedullary primitive neuroectodermal tumors (PNETs) of the spinal cord are rare. Only six cases have previously been reported, all involving tumors in the thoracic or lumbar spine. The authors report the case of a 54-year-old woman who presented with quadriplegia and bladder and bowel dysfunction. The patient had suffered symptoms of neck pain for 1 month and left shoulder weakness for 10 days. Magnetic resonance imaging of the cervical spine revealed an intramedullary mass extending from C-2 to C-5 with an exophytic component in the adjacent left subarachnoid space. Multiple biopsy specimens were obtained, and a partial excision was performed. Histological examination revealed nodular growth and neuronal differentiation, with a striking resemblance to desmoplastic medulloblastoma. A positron emission tomography scan did not reveal uptake at any site. These findings confirmed the diagnosis of a primary intramedullary PNET. Postoperatively, the patient was given craniospinal radiotherapy with a radiation boost to the tumor bed.

Hemangioblastoma of the filum terminale.

A 52-year-old man presented with low backache, paraesthesiae and spasticity of both lower limbs. He had urinary retention and constipation. Investigations revealed a vascular intradural cauda equina-conus tumor. MRI scan demonstrated an enhancing mass at the second and third lumbar vertebral levels. There were multiple dilated and tortuous veins draining from both poles of the tumor. Digital subtraction spinal angiogram showed the tumor to be supplied mainly by the radiculo-medullary artery from first lumbar artery and dural branches of the second and third lumbar arteries. At surgery, after pre-operative embolization, a well-defined tumor with an orange hue and fleshy consistency was encountered arising from the filum terminale. The tumor was excised en bloc. A sporadic hemangioblastoma arising from the filum terminale should be considered as a rare cause of back pain and sciatica. Total excision of the tumor offers cure.

Spontaneous cerebrospinal fluid rhinorrhea following excision of a massive torcular meningioma.

A massive supratentorial torcular meningioma was completely resected in a 50-year-old woman. On the tenth postoperative day she presented with spontaneous cerebrospinal fluid (CSF) rhinorrhea. The site of the CSF leak was through the region of the cribriform plate, remote from the site of surgery. The possible cause of the leak and the relationship with the massive meningioma are analyzed.

Pineal epidermoid cysts: a study of 24 cases.

BACKGROUND: A retrospective analysis of clinical features and management strategy in 24 cases of epidermoids in the pineal region is done. Long-term outcome of radical surgical resection is analyzed. METHODS: Twenty-four patients having epidermoids in the pineal region were surgically treated in our department during an 11-year period (1992 up to 2003). These cases were retrospectively analyzed. The average duration of follow-up was 5.2 years. RESULTS: The age at the time of presentation ranged from 13 to 51 years (mean, 29.2 years). There were 15 male and 9 female patients. The average duration of complaints was 7 months. Headache and ataxia were the commonest symptoms. The infratentorial-supracerebellar approach and the interhemispheric posterior parietooccipital route were used to excise the epidermoids in 20 and 3 patients, respectively. In one patient, the tumor was removed in 2 stages, first by the infratentorial-supracerebellar approach and subsequently by the interhemispheric posterior parietooccipital surgical approach. Total excision of the solid part of the tumor and the capsule was achieved in 6 patients. In 13 cases, the solid part of the tumor was completely resected, but at least, a part of the tumor capsule was left behind. In 5 cases, a part of the solid portion of the tumor was either deliberately or inadvertently left behind. Incomplete tumor resection was primarily due to adhesions of the capsule to large veins in the region, dissection around which was considered hazardous, and extensive and invasive nature of extension of the tumor. During the period of follow-up, one patient had asymptomatic growth of the residual tumor after 6 years of surgery. One patient with residual tumor continued to get generalized seizures. All patients are leading active lives. CONCLUSION: Radical surgery for pineal region epidermoid tumors is associated with an excellent immediate postoperative and long-term outcome.

Cerebellar oligodendroglioma in a young adult.

A rare case of an oligodendroglioma of the cerebellum in an 18-year-old male is presented. Radical surgical excision and craniospinal radiotherapy appear to be the treatment options for these potentially malignant lesions.