Endovascular management of pediatric neurovascular malformations – A single‑center experience from South India

Background: Neurovascular malformations (NVMs) in pediatric population are highly challenging to manage and treatment options include open surgery, endovascular therapy, and radiosurgery or combined. Recently, there has been a gradual shift from conventional surgical approach toward endovascular therapies with increasing availability of technical expertise and gadgetry. Objective: We aimed to study the clinical profile and immediate outcome of children with NVMs, who underwent endovascular therapy. Materials and Methods: This retrospective observational study was conducted in a tertiary care center in South India between February 2017 and August 2018. We included children admitted in pediatric intensive care unit (PICU) with NVM and needed neuroradiological intervention. Children with thromboembolism or other NVMs who did not require intervention were excluded from the study. Data on clinical profile, endovascular procedure done, supportive therapy given, and immediate outcome were collected and analyzed. Results: Of 1615 children admitted in PICU, 13 had NVM (0.8%), of which five had arteriovenous malformation (AVM), three had vein of Galen arteriovenous malformation (VGAM), one had VGAM with dural AVM, one had acquired carotid-cavernous fistula, two had berry aneurysm, and one had mycotic aneurysm. VGAM presented as hydrocephalus, whereas AVM and aneurysm as intracranial hemorrhage. Endovascular embolization was done using platinum detachable coils, onyx, N-butyl cyanoacrylate glue, and coil assist stents. One child needed decompressive craniectomy and another child needed extraventricular drainage. Four children needed pre-procedure ventilation and seven children needed prolonged post-procedure ventilation. Mortality was 15%; and among the survivors, 72% had an uneventful recovery. One child had seizures and two had hemiparesis at discharge. Conclusion: Endovascular management is an effective intervention for pediatric NVM. Multidisciplinary team approach and good pediatric intensive care are important for successful outcome. Further studies with long-term follow-up are needed to assess the durability of endovascular therapy

Profile and outcome of children with inborn errors of metabolism in a tertiary pediatric intensive care unit in South India

Introduction: Inborn errors of metabolism (IEM) can present as acute metabolic emergencies resulting in significant morbidity or death. Good intensive care supportive management and specific metabolic crisis treatment by tertiary pediatric intensive care unit (PICU) play a crucial role in optimizing the outcomes. Objectives: We aimed to study the clinical profile and outcome of children with IEM presenting as an acute metabolic crisis in a tertiary PICU. Methods: This retrospective descriptive study was conducted in a tertiary care center in south India between June 2016 and December 2018. We included children admitted in PICU as medical emergency and diagnosed to have IEM either earlier or at the time of PICU presentation by biochemical testing (basic testing and tandem mass spectrometry [TMS], gas chromatography-mass spectrometry [GCMS] and high-performance liquid chromatography [HPLC], and/or specialized testing) and/or molecular analysis. Clinical profile, details of diagnostic workup, and outcome were collected and analyzed. Results: Out of 2815 children admitted in PICU, 15 had IEM (0.9%). Median (interquartile range) age of presentation was 15 months (10–30 months). Consanguinity was found in 80%. The most common disorder was protein metabolic disorder. Seven patients were diagnosed in the newborn period, five during evaluation for developmental delay before PICU admission, and three were newly diagnosed during PICU admission. Supportive therapy of invasive/non-invasive ventilation and peritoneal dialysis was initiated in 10 and 4, respectively. Special formula was started in nine patients. Five (33%) died in spite of intensive care management. TMS, GCMS, and HPLC yielded definitive diagnosis in 12 (80%) patients. Molecular analysis was done in 12 patients. Conclusion: The most common cause for metabolic crisis in PICU is due to protein metabolic disorder. Aggressive intensive care and IEM directed therapy can be lifesaving, but still, the mortality is high