Abstract Infantile Spasms, first described by Dr. William West comprises of most severe, resistant, and disabling epileptic encephalopathy along with developmental delay, leaving the patient crippled. It often has a syndromic association warranting a thorough examination of the patient. Astute anesthetic care and vigilance for such a condition is a must for preventing collateral damage during their surgical management. A 3-year-old male, with developmental delay presents with 6–8 episodes of disabling myoclonic seizures with secondary generalization. The seizures are inadequately controlled despite six anti-epileptic medications in the maximum permissible dose and ACTH requiring surgical intervention. Several neck and peripheral joint contractures, associated cardiac and renal anomalies, multiple anti-epileptic medication-associated complications viz deranged liver functions, thrombocytopenia, drug metabolism, and ACTH-related complications impose a mammoth challenge in their peri-operative anesthesia management. It is meritorious to have a detailed understanding of this rare epileptic encephalopathy and an awareness of the bunch of other affiliated conditions to formulate a detailed peri-operative anesthesia management plan. This prevents any form of collateral peri-operative damage and can lead to better outcomes.

Abstract Diagnostic dilemmas are common in central nervous system (CNS) lesions. It is important to have a proper neuropathological diagnosis, as the treatment of benign conditions is different from malignant tumors. Our case report is an example of a diagnostic dilemma, which commonly accompanies rare malignancies lacking unique characteristics and literature support. An 11-year-old child was diagnosed with intracranial tuberculoma with meningoencephalitis and started on antituberculous drugs. After 6 months, the patient presented with a paroxysmal event and raised intracranial pressure. A gross total excision of the lesion was done, and the final histopathology report was CNS neuroblastoma FOXR2. Our case highlights the importance of tissue diagnosis in differentiating ring-enhancing lesions on imaging and contributes to the literature on rare CNS neuroblastoma FOXR2 tumors.

Abstract A 13-year-old boy affected by Seckel syndrome (SS) with multiple intracranial aneurysms is reported. SS is a rare, autosomal recessive syndrome characterized by severe intrauterine and postnatal growth retardation, microcephaly, and typical facial appearance with a beaklike protrusion of the midface (bird-headed). His diagnostic workup was done based on a significant family history and his phenotypic appearance of SS. The aneurysms were surgically treated, and the favorable outcomes were discussed.

Abstract Background: Spinal lipomas are congenital malformations often associated with spinal dysraphism and neurological deficits in pediatric patients. Surgical management aims to alleviate symptoms and prevent neurological deterioration. This retrospective analysis presents the clinical characteristics, surgical outcomes, and long-term follow-up of 55 pediatric patients with spinal lipomas managed at a single institution. Materials and Methods: The medical records of pediatric patients diagnosed with spinal lipomas between 2018 and 2023 were reviewed and followed up for 1.6 years (1–3 years). Demographic data, clinical presentation, radiological findings, surgical approaches, intraoperative details, postoperative complications, and long-term outcomes were collected and analyzed. Results: The study included 55 patients with a mean age of 10 years 9 months (13 days–17 years). Magnetic resonance imaging revealed filum terminale lipoma (43.6%, n = 24), caudal type (23.6%, n = 13), dorsal type (10.9%, n = 6), lipomyelomeningocele (12.7%, n = 7), and the mixed type (9%, n = 5). Surgical approaches varied based on lipoma location, with intraoperative neurophysiological monitoring utilized to minimize neurological complications. Postoperative complications occurred in 27.3% (n = 15) of patients, including cerebrospinal fluid leakage, urinary retention, and wound dehiscence. Long-term follow-up demonstrated a 21.8% rate of secondary tethering. Conclusion: This retrospective analysis provides valuable insights into the surgical management and outcomes of pediatric patients with spinal lipomas. Understanding clinical characteristics and surgical techniques can aid in optimizing patient care and improving long-term outcomes.

Abstract Background: Electroencephalography (EEG) is a valuable resource in pediatric epilepsy but tends to be over-used, a specific concern in resource-constrained settings. We aimed to study the utility of EEG in the diagnosis and treatment of epilepsy among children. Materials and Methods: A cross-sectional study was conducted among children in the age group of 1 month to 18 years who underwent EEG at our center. Clinical, treatment, and evaluation details including EEG findings were recorded. EEG was judged to be contributory to diagnosis and/or management on a follow-up visit at the epilepsy clinic. The contribution was allocated into one of the following four categories: supported in making the diagnosis, altered management, both supported diagnosis and altered management and did not contribute. Results: A total of 200 children were enrolled (59% male). The mean age of patients enrolled was 62.2 ± 51.9 months. The most common seizure type was generalized (n = 142, 71%) followed by focal (n = 54, 27%). The most common etiological category was structural (47.5%). Abnormal findings were noted in 52% of EEGs. EEG was determined to be contributory in 31.5% and noncontributory in 68.5% of the cases. EEG supported the diagnosis in 8.5%, altered management in 2%, and both supported diagnosis and altered management in 21% of cases. Conclusions: The present study demonstrates that EEG continues to be overused in our setting, with only one-third of EEG contributing to diagnosis and management among children. Strategies to enhance EEG yield and prevent over-utilization of EEG in resource-constrained settings are urgently needed.

Abstract Cranial migration of the shunt assembly is a rare complication following ventricular-peritoneal (VP) shunting, a common treatment for hydrocephalus. Despite its prevalence, VP shunting is associated with numerous complications, such as shunt failure, infection, and various types of shunt migration. This reports three cases of complete intracranial migration of the Chabra VP shunt system, including a cylindrical reservoir and a single spring-loaded valve. The potential causes and mechanisms of this rare complication are discussed, including technical errors during surgery, pressure gradients, excessive neck movements in neonates, and the physical characteristics of the ventricles and shunt path. This paper also suggests mitigation strategies such as using larger spherical chambers or flat round-chambered shunts instead of cylindrical Chabra chambers and providing adequate care to avoid excessive neck movements in children.

Abstract Congenital hypopituitarism is a rare disorder, ranging from isolated growth hormone deficiency (GHD) to more complex disorders. We present a case series of seven cases, including septo-optic dysplasia, pituitary stalk interruption syndrome, persistent craniopharyngeal canal, and Rathke cleft cysts, illustrating the spectrum of magnetic resonance imaging (MRI) abnormalities in congenital pituitary anomalies. Structural pituitary abnormalities were seen to be associated with central nervous system anomalies, and the presence of MRI abnormalities corresponded to severe GHD. Thus, by recognizing conditions beyond pituitary adenomas, MRI can play a vital role in identifying, managing, and prognosticating the myriad nontumoral causes of childhood pituitary dysfunction.

Abstract Introduction: Sacrococcygeal teratoma (SCT) is one of the most common neonatal tumors. While most SCTs are benign and can be removed without complications, intraspinal extension is rare but presents challenges, potentially necessitating laminectomy for complete excision. Our review aims to elucidate the difficulties in evaluating patients with intraspinal extension and their subsequent management. Objective: We aimed to explore the clinical features and complexities of management of SCT cases with spinal extension in children. Materials and Methods: We utilized the preferred reporting items for systematic reviews and meta-analyses extension for scoping reviews (PRISMA-ScR) guidelines to conduct the present review. Our inclusion criteria encompassed studies involving pediatric cases of SCTs with intraspinal extension, irrespective of the study type. Exclusion criteria involved studies that mentioned SCTs with intraspinal extension, but did not describe case characteristics. Additionally, we excluded adult literature, letters, editorials, comments, animal studies, and non-English literature studies. Results: Among the 11 included studies, comprising three case series and eight case reports, we described 14 cases, predominantly operated on within the first month of life, with a majority of female patients. Antenatal detection of intraspinal extension was possible in 21.43% of cases. Associated anomalies were present in 14.29% of cases, with one patient exhibiting preoperative neurological deficits. Imaging modalities primarily included magnetic resonance imaging (MRI), with the extension observed ranging from sacrum to thoracic levels. Lesion sizes varied greatly, and the Altman classification indicated predominance of type III tumors. Laminectomy was common for tumor removal, with a subset mentioning sacral nerve root sacrifice. Histopathological findings revealed predominantly mature teratomas, with few cases of immature teratomas. Postoperative neurological deficits were present in 28.57% of cases, with one patient experiencing recurrence and another mortality. Conclusion: A high index of suspicion is crucial in cases of SCT. Antenatal and postnatal ultrasonography should thoroughly evaluate for possible intraspinal extension and associated neural tube defects. Any suspicion warrants further assessment with MRI for detailed examination and determination of the extent of spinal extension. A collaborative team approach involving pediatric surgeons and pediatric neurosurgeons is essential for complete tumor removal, aiming to reduce the risk of recurrence and minimize neurological deficits.

Abstract Anti-N-methyl-d-aspartate receptor encephalitis (NMDARE) is an important and most common cause of pediatric autoimmune encephalitis. Neuroimaging in NMDARE is usually normal, but there can be involvement of the hippocampus or temporal cortex in some cases. However, imaging findings involving the thalamus, basal ganglia, and parietal cortex have rarely been reported. Awareness about the varied imaging patterns of this treatable condition is important as diagnostic delay can affect management and prognosis. We must also be aware of the overlap syndromes and rule out co-existing etiologies before attributing the extensive changes to one condition alone.