Search

Purpose: To assess the outcome of a pediatric population operated for drug-resistant epilepsy from a large tertiary care center in India. Methods: Retrospectively: quality of life (QOL); prospectively: preoperative assessment included interictal EEG, MRI (as per epilepsy protocol), video-EEG. Ictal SPECT (with subtraction) and PET were performed when required. QOL scores were assessed using the HASS or SSQ for seizure severity, Quality of Life in Childhood Epilepsy (QOLCE) for QOL, and Child Behavior Check List (CBCL) for behavior. Results: 142 were operated from January 2000 to June 2011 by the senior author. 118 patients with at least 1 year of follow-up were included in the study. Mean age at surgery was 9.8 ± 4.3 years. In addition, 40 patients underwent QOL assessment prospectively both before and after surgery. Mean duration of epilepsy was 5.3 ± 3.3 years. A class I outcome (Engel’s) was seen in 79.5% patients, class II in 8.6%, class III in 10.7%, and class IV in 1 patient. As per surgical procedures, class I outcome in patients who underwent temporal resection, hemispherotomy and extratemporal resection was 76, 87 and 72%, respectively. QOL scores correlated with duration of seizures, epileptic encephalopathy and outcome of surgery, but not with side of surgery, age and sex. Conclusions: This study, the largest reported from India, has demonstrated satisfactory results for epilepsy surgery in children.

Endoscopic third ventriculostomy (ETV) is one of the efficacious surgical options available for obstructive hydrocephalus, and is preferable to a ventriculoperitoneal shunt in those eligible. We retrospectively studied 115 cases, who underwent ETV at our institute over the last 5 years. Thirty-eight patients were infants. Major indications for ETV were aqueductal stenosis (n = 60/115, 52.2%), Dandy-Walker malformation (15/115, 13%), associated arachnoid cyst (n = 13/115, 11.3%) and post-meningitic hydrocephalus (n = 12/115, 10.4%). Average duration of the follow-up was 10.6 months. Ninety-nine patients (86.1%) showed clinical improvement after surgery. Clinical improvement was seen in 27 out of 38 infants (71%) followed up. ETV is a highly effective tool in all age groups of patients for the management of hydrocephalus. Clinical features should be considered as indications of the success or failure of ventriculostomy in infants who have low-pressure hydrocephalus.

Introduction: Nondysraphic intramedullary lipomas of the spinal cord are rare lesions. They are most commonly seen in the second or third decade of life. Their occurrence in the pediatric age group is even rarer. Case Report: The authors describe a 14-month-old child who presented with delayed motor milestones involving the bilateral lower limbs. The child was unable to sit or stand. MRI revealed a dorsally located intramedullary lipoma extending from C7 to D12. The child underwent C7–L1 laminotomy and gross total excision of the lipoma. Postoperatively, the child improved neurologically and attained normal power in the bilateral lower limbs. Discussion: Very few cases of extensive intramedullary lipomas involving the dorsal cord have previously been reported. Only two cases of pediatric extensive dorsal lipomas have been mentioned to date. The authors describe successful surgical excision of a holodorsal intramedullary lipoma in a 14-month-old child. To the best of the authors’ knowledge, this is the first case being reported of a holodorsal intramedullary lipoma in the youngest patient.

Uterus-like mass is an extremely rare choristoma of müllerian origin arising in association with neural tube defects. In this article, we describe the case of a 9-year-old girl with spina bifida, a mass lesion within the conus, and a subcutaneous lipoma in the lumbosacral region. Histopathological examination of the conus lesion revealed a uterus-like structure comprising of endometrial glands and stroma surrounded by fascicles of smooth muscle. This case differs from the few previously described cases in absence of neurological symptoms and early age at diagnosis. Thorough histopathological examination of resected tissue is therefore recommended for the diagnosis of this rare entity, as it may not have a typical presentation in all instances.

A 4-week-old infant presented with hemifacial spasms noticed from the 4th day after delivery. These progressed in severity, with generalization every 3–4 h. On admission, the infant went into refractory status epilepticus and had to be electively ventilated and taken for surgery on a semi-emergency basis. MRI showed a fourth ventricular hamartoma and video EEG showed spikes that were synchronous with the facial ‘twitches’ with generalization. Following the first surgery, the infant had an initial complete recovery, but developed recurrence of facial twitches after 2 weeks. Repeat MRI showed a small residual tumor which was re-operated and completely excised (at 8 weeks). Following this, the patient had complete recovery from seizures (5-year follow-up). This is the youngest patient reported presenting with status epilepticus with a fourth ventricular hamartoma operated successfully.

Ewing’s sarcoma was first described by James Ewing in 1921. It is most often seen in the long bones of the lower limbs and pelvis, and typically affects children and young adults [1, 2] . Metastatic Ewing’s sarcoma to skull bones is seen frequently, but primary Ewing’s sarcoma of the skull bone is rarely encountered in neurosurgical practice [1, 2] . These patients usually present with headache due to a local mass effect, but presentation with obstructive hydrocephalus is distinctly rare [3] . The present case is the first in the existing medical literature of Ewing’s sarcoma of skull bone presenting as obstructive hydrocephalus and blindness.