Samir Kumar Kalra

Pediatric Intracranial Aneurysms: An Institutional Experience

Introduction: Intracranial aneurysms are extremely uncommon in the pediatric population. Their epidemiology is poorly understood, and certain features make them unique. In our study we analyzed pediatric intracranial aneurysm patients to gain an insight into the epidemiology, clinicoradiological profile and outcome. Material and Methods: Out of 36 children (≤18 years of age; male:female ratio = 1.076:1; mean age 13.19 ± 3.72 years, age range 5–18 years) presenting with subarachnoid hemorrhage (SAH; n = 33; 91.67%) and mass effect (n = 3; 8.33%), 27 who were positive on digital subtraction angiography and treated for intracranial aneurysms between January 1991 and February 2007 were included in this study. Results: At presentation, the majority (n = 21) of the pediatric intracranial patients showed a good grade, and 23 (85.18%) presented with SAH. Sudden severe headache (n = 19; 70.37%) and loss of consciousness (n = 14; 51.85%) were the most common symptoms, and meningeal signs (n = 18; 66.66%) most commonly elicitable. There were 7 patients with giant aneurysms and 8 patients with posterior circulation aneurysms. Internal carotid artery (ICA) bifurcations (n = 6; 18.18%) followed by middle cerebral artery (MCA) bifurcations (n = 4; 12.12%) were the most common sites. At a mean follow-up of 18.67 ± 10.85 months (range 1–42 months), there were 21 (77.77%) patients with favorable outcome and 3 patients died. Conclusions: Intracranial aneurysms in children commonly present with SAH; there is a male predominance, and ICA bifurcations followed by MCA bifurcations are the most common sites. The incidence of posterior circulation aneurysms and giant aneurysms is higher as compared to adults. The pediatric patients present with better grades and have better overall surgical results.

Chiari I malformation: surgical experience over a decade of management

Chiari malformations are a pathological continuum of hindbrain maldevelopment and Chiari I malformation (CMI) represents caudal decent of cerebellar tonsils. The surgery aims to relieve tonsillar compression with restoration of the normal cerebrospinal (CSF) flow dynamics. We report the salient features of our surgical experience with CMI; over a decade of management of 87 patients. Most (57) presented with pain and weakness was predominant motor complaint (n = 34). The associated bony anomalies were seen in 25 patients. For clinical grading and outcome analysis, modified Klekamp and Samii scoring system was used. The majority of patients were in poor grades (n = 43); 79 patients improved following surgical intervention at a mean follow-up of 34.21 + 21.53 months. The bony decompression and establishment of CSF flow dynamics are needed, and posterior fossa decompression in symptomatic patients offers success in the majority of patients with minimal complications.

A clinical scoring system for neurological assessment of high cervical myelopathy: measurements in pediatric patients with congenital atlantoaxial dislocations.

OBJECTIVEThe assessment of response to treatment in pediatric patients with congenital atlantoaxial dislocation (AAD) is performed using a disability grading system but may be better determined by a score based on clinical parameters. This study proposes a scoring system based on a comprehensive neurological examination to assess surgical outcome in these patients. METHODSSixty-seven patients with congenital AAD aged 14 years or younger were included and analyzed prospectively. A scoring system based on six factors (motor power, gait, sensory involvement, sphincteric involvement, spasticity, and respiratory difficulty) was designed at the beginning of the study and all patients were assessed using this score as well as the Di Lorenzos grade preoperatively, postoperatively, and at the time of each follow-up visit. RESULTSThere was a very high incidence of occipitalized arch of atlas and fusion of the second and third cervical vertebrae in the irreducible variety. Most patients were classified in poor grades preoperatively; however, the changes in score were seen more often when using the scoring system we developed compared with the Di Lorenzos grade. Our score also corroborated better with the clinical improvement. CONCLUSIONThe clinical profiles of pediatric patients with AAD are similar with a higher incidence of atlas arch anomalies in patients with irreducible AAD. A scoring system based on clinical parameters is proposed for clinical evaluation of such patients. This system is easy to use and interpret and is more sensitive to the changes in the neurological status of patients.

Hemangiopericytoma of thoracic spine: a rare bony tumor

Case reportWe report the case of a 16-year-old girl who developed hemangiopericytoma of the thoracic spine; the main clinical symptoms were of spastic paraparesis with sensory involvement and uro-fecal incontinence. She was initially put on antitubercular treatment keeping in mind the endemicity of tuberculosis in the region. When she deteriorated on conservative management, she was operated upon, and the histopathological report was suggestive of hemangiopericytoma.Materials and methodsAdditional immunocytochemistry was performed in the paraffin-embedded tumor sections.ConclusionsAn extremely rare case of primary epidural malignant hemangiopericytoma of the thoracic spinal column is described. It is a rare tumor, which is locally aggressive, and a potentially malignant tumor. The tumor is more commonly found in the cranium, and spinal involvement is rare, and only few case reports could be retrieved from the literature. We discuss the clinical profile, management, and outcome of spinal hemangiopericytomas along with pertinent review of the literature.

Lhermitte–Duclos disease

BackgroundLhermitte–Duclos disease (LDD; dysplastic gangliocytoma of the cerebellum) is a rare hamartoma of the cerebellum. It was first described by Lhermitte and Duclos in 1920 as “Sur un ganglioneurome diffus du cortex du cervelet” (Lhermitte and Duclos [Bull Assoc Fr Etude Cancer 9:99–107, 1920]). Since then, due to its rarity and the variability of its presentation, the diagnosis is often missed. It is not only important for the neurosurgeon and the pathologist to be aware of this entity so as to differentiate from malignant lesions but also to distinguish it from other cerebellar malformations, which may be hypoplastic or dysplastic, focal or diffuse in nature, with brain stem and cerebral involvement, and varied natural course.Illustrative caseWe report a case of LDD in a 16-year-old boy. This case exemplifies the rarity of the disease leading to lack of awareness with which the diagnosis was easily missed. We also take this opportunity to discuss the clinical presentation, neuroradiological appearance, and a brief summary of histopathological findings, pathogenetic considerations, and neurogenetical aspects.

Tubercular atlantoaxial dislocation in children: an institutional experience

OBJECT In this paper the authors analyzed the clinical and imaging-documented profile of pediatric patients with tubercular atlantoaxial dislocation (AAD). METHODS Seventeen children 16 years of age or younger with tubercular AAD were included in the study. Patients with reducible AAD underwent direct posterior stabilization. All patients were treated with a four-drug antituberculosis therapy (ATT) regimen (10-20 mg/kg/day rifampicin, 10-20 mg/kg/day isoniazid, and 15 mg/kg/day ethambutol in a single daily dose; and pyrazinamide 20-35 mg/kg/day in two divided doses) for 3 months. The pyrazinamide was then discontinued after 3 months and the ethambutol after 1 year. The rifampicin and isoniazid were continued for 18 months. RESULTS Most of the patients had irreducible AAD. There was a high incidence of long tract signs, and the restriction of neck movements, as well as neck pain, was also very common. There was a significant delay in seeking neurosurgical consultation. Most patients were assigned poor preoperative grades, but they experienced excellent improvement postoperatively. CONCLUSIONS The presence of tubercular AAD in children can have subtle manifestations leading to delayed diagnosis. The successful management of tubercular AAD can be achieved after determining the extent of the disease process and the underlying instability. The goal of surgery is tissue diagnosis and relief of neural compression and stabilization. Medical treatment with ATT is an integral part of the treatment protocol.

Craniovertebral Junction Anomaly with Atlas Assimilation and Reducible Atlantoaxial Dislocation: A Rare Constellation of Bony Abnormalities

A rare case of craniovertebral junction anomaly with associated reducible C₁-C₂ dislocation and assimilation of the atlas is reported. The patient presented with neck pain with spastic quadriparesis. A posterior stabilization utilizing a contour rod, sublaminar wire fixation and onlay bone grafts between the occiput, and C₃ and C₄ vertebrae was performed followed by symptomatic improvement.

Intraventricular Neurocysticercus Cyst(s) in Indian Children

Eleven cases of intraventricular neurocysticercosis were managed over a 10-year period. All patients presented with features of hydrocephalus. In 9 cases the cysts were blocking the cerebrospinal fluid (CSF) pathway resulting in hydrocephalus. The 4 cases of lateral ventricular cyst had communicating hydrocephalus, while 2 of these had asymmetrical ventriculomegaly. Two of 4 cases had more than one cyst. One of 4 children with lateral ventricular cyst had narrowing of the foramen of Monro causing asymmetrical hydrocephalus. Following cyst removal, CSF diversion was not required in 6 cases, while 5 required CSF diversion despite removal of their cysts in 4 of 5. One patient had a cyst at the aqueductal inlet and he was managed by shunt and albendazole therapy. Thus, we managed 6 of our cases with excision of the cysts obstructing the CSF pathway, and in 4 cases excision of cysts with ventriculoperitoneal shunt was adequate.

Primary midbrain germinoma.

An 11-year-old boy presented with a 4-month history of progressive holocranial headache, intermittent vomiting and visual blurring. Later, he began walking unsteadily, with progressive bilateral visual and hearing loss. He had had two episodes of abnormal tonic posturing the day prior to admission. On examination, the patient was drowsy and did not respond to commands. Imaging showed an enhancing midbrain mass with an exophytic component with hydrocephalus. Emergency ventriculoperitoneal shunt was performed, following which there was neurological improvement. Later he underwent a midline suboccipital craniectomy with vermian splitting and decompression of the exophytic component of the tumor from the midbrain. Biopsy of the tumor revealed a germinoma. Post-operatively, the patient received chemotherapy followed by radiotherapy. Primary midbrain germinoma is an extremely rare entity and to the best of our knowledge only two cases to date have been reported in the literature.

Lord Ganesha: the idol neurosurgeon

Ganesha (also spelled Ganesa or Ganesh) is one of the bestknown and most-worshipped divinities in Hinduism. Although there are many descriptions, Ganesha’s elephant head makes him easy to identify (see cover illustration). There are many mythological anecdotes associated with his birth and life history. Ganesha is worshipped as the lord of beginnings, of obstacles, patron of arts and sciences, and of intellect and wisdom. Ganesha appears as a distinct deity in clearly recognizable form beginning in the fourth to fifth centuries, during the Gupta Period. The name Ganesha is a Sanskrit compound, joining the words gana meaning a group, multitude, or categorical system and isha, meaning lord or master. The translation “Lord of Hosts” may convey a familiar sense to Western readers. Unlike some deities, representations of Ganesha show wide variation with distinct patterns changing over time. Ganesha images were prevalent in many parts of India by the sixth century. A statue of Lord Ganesha has been dated between 973 and 1200 by Martin-Dubost [2]. He has the head of an elephant and a big belly. While some texts say that Ganesha was born with an elephant head, in most stories he acquires the head later, with several accounts given. The most common motif in these stories is that Ganesha was born with a human head and body and that Shiva beheaded him when Ganesha came between Shiva and Parvati. Shiva then replaced Ganesha’s original head with that of an elephant [2]. Details of the battle and where the replacement head came from vary according to different sources. It is widely believed that Lord Shiva wanted his son to have divine attributes and hence he performed the first documented and successful transplant surgery of the brain, which the neurosurgical fraternity still strives to achieve [1]. Lord Ganesha hence became the recipient of the first brain transplant. The Lord had many attributes, which could be a neurosurgeons’ delight. Ganesha is considered to be the Lord of Intelligence. In Sanskrit, the word buddhi is a feminine noun that is variously translated as intelligence, wisdom, or intellect. The concept of buddhi is closely associated with the personality of Ganesha, especially in the Puranic period, where many stories showcase his cleverness and love of intelligence. On a symbolic level, Ganesha was blessed with wide ears and a small mouth so as to listen more clearly and at all decibels and speak less, with a very active eight-nerve function system that every neurosurgeon must desire for all his patients, irrespective of his caste, creed and socioeconomic status. He possessed a large head, meant to think big and have large memory, which easily are the qualities every neurosurgeon aspires for. The small eyes gave him power to concentrate and have focus, which in a microscopic era would be desired by all neurosurgeons. This may be likened to the neuronavigational systems, which we want to emulate. The number of Ganesha’s arms varies; his bestknown forms have between two and 16 arms. The Robotic arms, which we utilize and wish to have, were possessed by the Lord Ganesha. One tusk, which he had, represented the ability to retain the good and discard the poor. Is it not the essence of what neurosurgery is all about? The trunk represented high efficiency and adaptability, which we feel is the bottomline of neurosurgical practice, taking instant and stable decisions. Ganesha’s protruding belly appears as Childs Nerv Syst (2008) 24:287–288 DOI 10.1007/s00381-007-0507-2