Cementifying Fibroma of the Sphenoid Wing in a Child: A Case Report.

"Cementifying fibroma" is a benign tumor of the fibroblastic tissue containing masses of cementum-like calcified tissue, usually occurring between the third and fourth decades. Cementifying-ossifying fibromas are rare non-odontogenic, fibro-osseous tumors of the periodontal ligament that arise from the mesodermal germ layer3. We report a 12-year-old male child, who presented with a swelling in the left temporal region near the angle of the left eye. These non-neoplastic, locally destructive tumors present as an osseous lesion involving the mandible, maxilla, zygoma, paranasal sinuses, orbit, and rarely the petromastoid regions. The preoperative diagnosis was not clear even with CT and MRI, and, hence, all fibro-osseous lesions were considered as differential diagnosis. The lesion was surgically resected completely, and the histology confirmed it as a cementifying fibroma. According to the WHO classification, this is a variant of cementifying fibromas, which represent a subgroup of cementomas, that is, fibro-osseous lesions containing cementum. Histologically, these are fibrous tissues with calcified structures resembling bone and cementum. Cranioplasty was done simultaneously, with successful clinical results.

Pediatric Skull Base Meningioma: Case Report and Review of Literature.

Meningiomas are frequent in adults but rare in children, though they are not uncommon. They are known to occur in the pediatric population in all age groups. In children, meningiomas are usually known to be large, cystic, and even aggressive. Among them, skull base meningiomas constitute a distinct entity. Meningiomas arising from the skull base in those younger than the age of two years are rarely reported in literature. We report one such skull base meningioma, involving the middle and posterior cranial fossa, in a child of one year and eight months. The challenges associated in its diagnosis and management are presented.

Multiloculated Hydrocephalus: Evolution of Treatments and Outcome.

Multiloculated hydrocephalus has an overall dismal functional outcome; imaging, early diagnosis, and technological advancements have made new roads in its management. Thirty infants with multiloculated hydrocephalus were studied. Progressive enlargement of the head was the most common presentation in 77%; of these, 47% were treated for neonatal meningitis and septicemia and 20% had suffered birth trauma. The majority required a single ventriculoperitoneal shunt; nine of them required multiple shunts; and six were managed with endoscopic fenestration and endoscopic third ventriculostomy. On follow-up, shunt malfunction was the most common complication. Only 26% of the survivors could achieve normal, neuropsychological developments. The mortality in this series is 6.7%.

Cavernous malformation of the optic chiasm: Neuro-endoscopic removal.

Cavernous malformations (CMs) arising from the optic nerve and chiasm are extremely rare. In large autopsy series, CMs were estimated to range from 0.02 to 0.13% in the general population. However, with introduction of MRI, these lesions were found more often than previously thought, ranging from 0.2% to 0.4%. Only 29 cases have been reported according to our knowledge. Most patients present with drop in visual acuity and visual field. Although MRI findings of cavernous malformations have been reported, they may not be diagnostic enough. Among the 29 reported, 16 underwent total resection with good results. In some, resection was complicated by damage to the surrounding neural tissue. Surgical removal is the recommended treatment to restore or preserve vision and to eliminate the risk of future hemorrhage. However, the anatomical location and eloquence of nearby neural structures can make these lesions difficult to access and remove. CMs appear to occur in every age group (range 4 months to 84 years mean-34.6 years) ith an approximately equal male to female ratio. They typically present with chiasmal apoplexy, characterized by sudden visual loss, acute headaches, retro orbital pain, and nausea.

Bilateral transplantation of allogenic adult human bone marrow-derived mesenchymal stem cells into the subventricular zone of Parkinson's disease: a pilot clinical study.

The progress of PD and its related disorders cannot be prevented with the medications available. In this study, we recruited 8 PD and 4 PD plus patients between 5 to 15 years after diagnosis. All patients received BM-MSCs bilaterally into the SVZ and were followed up for 12 months. PD patients after therapy reported a mean improvement of 17.92% during "on" and 31.21% during "off" period on the UPDRS scoring system. None of the patients increased their medication during the follow-up period. Subjectively, the patients reported clarity in speech, reduction in tremors, rigidity, and freezing attacks. The results correlated with the duration of the disease. Those patients transplanted in the early stages of the disease (less than 5 years) showed more improvement and no further disease progression than the later stages (11-15 years). However, the PD plus patients did not show any change in their clinical status after stem cell transplantation. This study demonstrates the safety of adult allogenic human BM-MSCs transplanted into the SVZ of the brain and its efficacy in early-stage PD patients.

Hydrocephalus Indian scenario - A review.

Hydrocephalus is a common clinical problem seen in pediatric neurosurgical practice. Hydrocephalus involves dilatation of the cerebral ventricular system with corresponding, compressive effects on the parenchyma. It can be communicative or obstructive types. Congenital, acquired, infective, and secondary hydrocephalus have different clinical features with different modality of treatments. Ventriculoperitoneal shunt is the gold standard of treatment. Endoscopic 3(rd) ventriculostomy is rapidly gaining prominence as an alternative. Various kinds of hydrocephalus, their pathophysiology, treatment and complications are reviewed.

Spinal dysraphism.

To review the clinical features and current understanding of spina bifida with an emphasis on the Indian Scenario. Selected articles and current English language texts were reviewed. The authors experience was also reviewed and analysed. Spina bifida is a common congenital anomaly encompassing a wide spectrum of neural tube defects.It is broadly classified as spina bifida aperta and occulta. With the prenatal screening, the incidence of aperta is gradually declining, whereas the detection of occulta has increased with the advent of magnetic resonance imaging. Over the years, the understanding of pathophysiology has made a significant changein the management of these anomalies. Early detection and complete correction can significantly reduce the neurological disability. This article is an overview of spina bifida with a special emphasis on Indian scenario.

Evaluation of functional outcomes in congenital hydrocephalus.

AIM: The long term outcomes of congenital hydrocephalus are still not clearly known despite it being a common clinical condition. Several clinical, radiological factors were correlated to predict the functional outcomes. This study aimed to correlate the clinical, radiological parameters with the regional functional outcomes of the brain. MATERIALS AND METHODS: Children with congenital hydrocephalus were divided into Group A with hydrocephalus alone and Group B hydrocephalus with spina bifida. Ventriculoperitoneal shunt surgery was performed by the same surgeon. CT scans and neuropsychological assessments were performed before and serially after the shunt. The clinical and the radiological findings were correlated with the developmental levels during the follow-up. RESULTS: There were 25 children in Group A and 15 children in Group B; 72% in Group A and 93% in Group B were less than 6 months of age at the time of treatment. Forty percent in Group A and 92% in Group B had the signs of hydrocephalus at admission. Cerebrospinal fluid (CSF) diversion results in the reduction in ventricular dilatation and corresponding increase in the cortical mantle thickness. The ventricular size and the cortical mantle thickness were measured serially and correlated with the development in the neuropsychological function. In this study, 80% in Group B reached near normal development in comparison to 33% in Group A. We have noticed a significant correlation in the increase in the regional cortical mantle thickness with corresponding improvement in the functional development. This clearly ratifies the improvement in the frontal and parietal areas having their distinctive effect on the functional development of the child. CONCLUSION: Early CSF diversion and timely intervention seems to benefit functional recovery. It is interesting to note that reconstitution of cortical mantle in different areas of the brain showing corresponding improvement in their respective areas. Large ventricles (head circumference more than 50 cm) recurrent subdural collections and repeated shunt obstructions have a bad influence on the long-term outcome. Unlike the previous belief the children with myelomeningocele can have equal benefit in terms of neuropsychological development after the shunt surgery.

Targeted therapy for high-grade glioma with the TGF-ß2 inhibitor trabedersen: results of a randomized and controlled phase IIb study.

This randomized, open-label, active-controlled, dose-finding phase IIb study evaluated the efficacy and safety of trabedersen (AP 12009) administered intratumorally by convection-enhanced delivery compared with standard chemotherapy in patients with recurrent/refractory high-grade glioma. One hundred and forty-five patients with central reference histopathology of recurrent/refractory glioblastoma multiforme (GBM) or anaplastic astrocytoma (AA) were randomly assigned to receive trabedersen at doses of 10 or 80 µM or standard chemotherapy (temozolomide or procarbazine/lomustine/vincristine). Primary endpoint was 6-month tumor control rate, and secondary endpoints included response at further timepoints, survival, and safety. Six-month tumor control rates were not significantly different in the entire study population (AA and GBM). Prespecified AA subgroup analysis showed a significant benefit regarding the 14-month tumor control rate for 10 µM trabedersen vs chemotherapy (p= .0032). The 2-year survival rate had a trend for superiority for 10 µM trabedersen vs chemotherapy (p = .10). Median survival for 10 µM trabedersen was 39.1 months compared with 35.2 months for 80 µM trabedersen and 21.7 months for chemotherapy (not significant). In GBM patients, response and survival results were comparable among the 3 arms. Exploratory analysis on GBM patients aged ≤55 years with Karnofsky performance status >80% at baseline indicated a 3-fold survival at 2 and 3 years for 10 µM trabedersen vs chemotherapy. The frequency of patients with related or possibly drug-related adverse events was higher with standard chemotherapy (64%) than with 80 µM trabedersen (43%) and 10 µM trabedersen (27%). Superior efficacy and safety for 10 µM trabedersen over 80 µM trabedersen and chemotherapy and positive risk-benefit assessment suggest it as the optimal dose for further clinical development in high-grade glioma.

Dengue encephalitis associated with hydrocephalus. A case report.

We describe a case of dengue encephalitis with bilateral thalamus and cerebellum involvement. The cerebellar mass effect led to hydrocephalus requiring drainage. The patient recovered completely on follow-up.

Pediatric anterior skull base tumors: Our experience and review of literature.

Surgery for skull base lesions has advanced considerably in the past few years. The improvement in surgical results could be attributed to the availability of refined imaging modalities, modern technological advances and multidisciplinary team approach. In this report, we present our personal experience in the surgical management of 45 children with a variety of skull base lesions treated over 10 years. This article includes a retrospective analysis of the surgical approaches used and their results with a review of the literature.

Sinus pericranii presenting with macrocephaly and mental retardation.

We present a rare case of right parietal sinus pericranii in a 2-year-old female child who presented with a compressible swelling on the right side of the scalp since 3 months of age, with a large head. Magnetic resonance imaging along with venography and conventional angiogram was performed, which confirmed the diagnosis.

Cerebral high grade glioma with crossed cerebellar metastases. A report of three cases.

We describe three patients with high grade supratentorial gliomas who were operated and later presented with cerebellar mass lesions on the opposite side. Supratentorial infarct with crossed cerebellar atrophy is a well-known entity called crossed cerebellar diaschisis. We hypothesize that supratentorial high grade tumours might also spread to the opposite cerebellum through crossed cerebrocerebellar fiber tracts similar to crossed cerebellar diaschisis.

Primary hypothyroidism presenting as a sellar mass which regressed with thyroid hormone therapy. A case report.

We describe a15-year-old girl presenting with compensatory pituitary enlargement due to primary hypothyroidism. The hyperplasia regressed following thyroxine administration. The clinical, laboratory and MRI features of the patients are described and the mechanisms of pituitary gland enlargement are discussed.

Clinical studies for improving radiotherapy with 2-deoxy-D-glucose: present status and future prospects.

Higher rates of glucose usage generally correlate with poor prognosis in several types of malignant tumours. Experimental studies (both in vitro and in vivo) have shown that 2-deoxy-D-glucose (2-DG), a glucose analog and glycolytic inhibitor, enhances radiation-induced damage selectively in tumor cells while protecting normal cells, thereby suggesting that 2-DG can be used as a differential radiomodifier to improve the efficacy of radiotherapy. Clinical trials undertaken to study the feasibility, safety, and validity of this suggested approach will be described. Based on 2-DG-induced radiosensitization observed in primary organ cultures of cerebral glioma tissues, clinical trials were designed taking into consideration the radiobiology of gliomas and pharmacokinetics of 2-DG. Phase I/II clinical trials have unequivocally demonstrated that a combination of 2-DG (200-300 mg 2-DG per kg body weight orally administered after overnight fasting, 20 min before irradiation) with large weekly fractions (5 Gy/fraction) of low-LET radiotherapy is well tolerated without any acute toxicity or late radiation damage to the normal brain tissue. Nonserious transient side effects similar to hypoglycemia induced disturbances like restlessness, nausea, and vomiting were observed at the 2-DG doses used. Data from these trials involving more than 100 patients have clearly indicated a moderate increase in the survival, with a significant improvement in the quality of life with clinicopathological evidence of protection of normal brain tissue. A phase III multicentric trial to evaluate the efficacy of the combined treatment is in progress. Directions for future studies are discussed.

Recurrent cerebral larva migrans: A case report and review of literature.

Cerebral larva migrans (CLM) is an uncommon and rarely diagnosed entity. We report here a histopathologically proven case of CLM in a 13 year-old girl, who presented with bilateral focal seizures, right hemiparesis, and lapsed into altered sensorium prior to admission. Her CT scan was suggestive of a granulomatous lesion which eventually turned out to be CLM (toxocariasis). Interestingly, the lesion recurred after excision, with a confusing clinical picture. Following the second surgery, the pathology was reconfirmed and cure was provided for the disease. There has been no further intracranial recurrence during the past ten years although she developed cutaneous lesions.