Bhagvatula Indira Devi

Pediatric Intracranial Meningiomas – Do They Differ from Their Counterparts in Adults?

Aim: Meningiomas are very rare in children comprising only 0.44.1% of pediatric age tumors and only 1.5–1.8% of all intracranial neoplasms. We analyzed the clinical, pathological and management profile of these rare tumors and elucidated their differences from meningiomas in adults. Methods: From 1990 to 2005, 33 patients belonging to the pediatric age group with intracranial meningiomas were treated in NIMHANS. Results: There were 19 male and 14 female children. The duration of symptoms ranged from 1 to 60 months. The study had a mean follow-up of 23.4 months. The commonest presenting symptoms were headache (90.9%) and visual disturbances (51.5%). Three patients had associated neurofibromatosis. Calcification was noticed in 22% of the tumors. Intraventricular meningiomas were the commonest (24.2%). Gross total or near total resection was possible in 22 out of 33 patients. Nine patients had reversible postoperative motor deficits. Fibrous meningioma was the commonest histological subtype (24.2%). Five patients had atypical or anaplastic subtypes. Adjuvant radiotherapy was administered in 4 patients. Six patients had recurrences and underwent surgery. Ten patients required either staged procedures or multiple surgeries for the recurrence/residual tumors. Conclusion: Pediatric meningiomas are rare tumors and differ from those in adults by their male predominance, atypical locations, higher rates of malignant subtypes, recurrence and association with neurofibromatosis. Surgical management is challenging because of atypical location.

Anomaly of Arch of Atlas – A Rare Cause of Symptomatic Canal Stenosis in Children

Symptomatic canal stenosis at the level of atlas (C1) without atlantoaxial dislocation is thought to be very rare in children. Though common, anomalies of the arch of atlas are generally incidental findings in X-rays. High cord compression due to a narrow canal from a bifid posterior arch, or an absent posterior arch, is a very rare condition. We report 5 children with high cord compression from stenosis of C1 arch.

Subclinical autonomic nervous system dysfunction in compressive cervical myelopathy.

Study Design. Laboratory evaluation of autonomic nervous system (ANS) in patients with cervical compressive myelopathy (CCM). Objective. To study the autonomic functions and heart rate variability (HRV) in patients with CCM and compare the findings after surgery. Summary of Background Data. ANS dysfunction is well known after traumatic spinal cord injury. There are very few studies of ANS dysfunction in noncompressive myelopathy and there are no studies on compressive myelopathies. Methods. After excluding patients on cervical traction or with medical comorbidities, 29 adult patients with CCM were evaluated. Conventional autonomic function tests and HRV were studied in these patients. The same tests were done on 29 age- and sex-matched healthy controls. Student t test was used to find the significance of study parameters on continuous scale. Chi-square/Fisher exact test was used to find the significance of study parameters on categorical scale between two groups. Significance was assessed at 5% level. Results. Patients with CCM as compared with controls, showed significant difference in following parameters; deep breathing, Valsalva ratio, 30:15 (longest RR interval (duration between two consecutive R waves of ECG) around 30th second and the minimum RR interval around 15 seconds after standing up), and orthostatic fall of blood pressure. Except 30:15, there was no significant change of other autonomic function tests after surgery. Among the HRV parameters, there was a trend in increase in total power and decrease in root-mean-square differences of successive RR intervals; however, it did not reach statistical significance. Conclusion. Patients with CCM have definite ANS dysfunction as compared to healthy age- and sex-matched controls. There is significant improvement in 30:15 ratio after surgery. HRV indices are also impaired and there is a trend for change in total power and root-mean-square differences of successive RR intervals suggesting loss of HRV.

Large occipito-cervical encephalocele with Chiari III malformation.

Chiari type III is the rarest of the Chiari malformations and is usually associated with high morbidity and mortality. Treatment consists of primary closure of the encephalocele with or without cerebro-spinal fluid (CSF) shunting. In our case, the patient was treated with ventriculoperitoneal shunt followed by excision of the encephalocele. We propose that large encephaloceles should be treated with CSF shunting prior to repair of the sac so as to achieve optimal result.

Cerebral Aneurysmal Childhood Arteriopathy

Cerebrovascular complications are a rare occurrence in children with AIDS [1] . Patsalides et al. [2] found the incidence of vascular abnormalities in pediatric AIDS to be 2.6% in their cohort of 426 patients. Involvement of the small and medium arteries is a more common manifesta-tion of HIV-associated cerebral vasculitis [3]mor . f Fi usdilatation of cerebral vessels termed CACA is a rare com-plication of HIV infection in children [4] . Only 35 cases C erebral aneurysmal childhood arteriopathy (CACA) is a fusiform dilatation of vessels of the circle of Willis. It is a unique manifestation of cerebrovascular complica-tion due to HIV infection in children. We describe a case of CACA who did not have other clinical manifestation of AIDS. A 13-year-old boy presented with irritability and re-duced speech output for 1 week, and acute-onset right hemiplegia with aphasia of 1 day duration. He had had an episode of left lower limb weakness 2 years back which resolved spontaneously over 3 h. He was not investigated at that time. Birth, developmental, personal and family history were unremarkable. On examination, he was con-scious, aphasic and had right hemiplegia. A cranial CT scan revealed multiple ovoid enhancing lesions in the suprasellar and medial sylvian cisterns with hydrocephalus (f ig. 1) . The radiological diagnosis was gi-ant internal carotid artery (ICA) aneurysm or an arterio-venous malformation. A cerebral angiogram revealed dilatation of the bilateral supraclinoid ICAs extending beyond the bifurcation suggestive of fusiform aneurysm or ectasia ( fig. 2 ). Blood investigations were normal ex-cept for positive serology for HIV-1 and -2. He deterio-rated following the angiogram and developed extensor posturing. An external ventricular drainage was institut-ed immediately but the patient did not show any improve-ment and expired within few hours.

Peculiarities and Patterns of Cervical Spine Injuries in Children and Adolescents: A Retrospective Series of 84 Patients from a Single Institute.

Cervical spine injuries occur infrequently in children but are associated with significant disability and mortality. A retrospective analysis was performed of 84 consecutive pediatric spine injuries treated at our institute from January 2002 to December 2011. The mean age was 14.7 years. There were 18 patients (21%) in group A (0-12 years) and 66 patients (79%) in group B (13-18 years). Overall, injury was more common in boys (ratio of 6:1). Trivial fall was the predominant cause in group A and fall from height in group B. There were 30 children (36%) with injuries of the upper cervical spine, 53 (63%) with injuries of the lower cervical spine and 1 patient (1%) with a combined injury of upper cervical spine and thoracic spine. Overall, 22% of the group A children and 67% of the group B patients had more severe injuries (Frankel grades A, B and C); 21% (18/84) were treated by surgical fusion. Where follow-up was available, 17 out of 22 children (77%) had good outcome (Frankel grade >C). In conclusion, mechanisms and patterns of injury in children are age related and the majority of the children had good outcome.

Post-traumatic cervical spondyloptosis: A rare entity with multiple management options

Post-traumatic cervical spondyloptosis is a rare condition associated with high energy injuries, and to our knowledge only case reports are available. There are no universally accepted treatment paradigms for these cases and management is individualised according to the case and surgeon preference. We retrospectively analysed our management and clinical outcomes of this condition. From January 2007 to August 2014 we treated eight patients with cervical spondyloptosis at our institute. Only two patients had no neurological deficits; all the remaining patients had partial cord injury. Seven were treated surgically with preoperative traction followed by anterior cervical discectomy and fusion with fixation in three patients, and combined anterior and posterior fusion and fixation in four. Depending on the presence of anterior compression by a disc an anterior first or posterior first approach was advocated. All four combined anterior and posterior fusion and fixation patients needed to be turned more than once (540°). There was no neurological deterioration in any of the patients, as they either improved or remained stable neurologically. Post-traumatic cervical spondyloptosis is a challenging entity to manage. Surgery can be done safely with good clinical and radiological outcome and needs to be tailored to the individual patient.