Faculty Bibliography

Brain tumors collectively represent the most common solid tumors in childhood and account for significant morbidity and mortality. Until recently, pediatric brain tumors were diagnosed and classified solely based on histologic criteria, and treatments were chosen empirically. Recent research has greatly enhanced our understanding of the diverse biology of pediatric brain tumors, their molecular and genetic underpinnings, leading to improved diagnostic accuracy and risk stratification, as well as the development of novel biomarkers and molecular targeted therapies. For subsets of patients, these new treatment options have already resulted in improved survival and decreased treatment toxicity. In this article, we provide an overview of the most common childhood brain tumors, describe recent key advances in the field, and discuss the therapeutic challenges that remain.

BACKGROUND: Tenecteplase, a modified plasminogen activator with higher fibrin specificity and longer half-life, may have advantages over alteplase in acute ischemic stroke thrombolysis. AIMS: We undertook an individual patient data meta-analysis of randomized controlled trials that compared alteplase with tenecteplase in acute stroke. METHODS: Eligible studies were identified by a MEDLINE search, and individual patient data were acquired. We compared clinical outcomes including modified Rankin Scale at three months, early neurological improvement at 24 h, intracerebral hemorrhage, symptomatic intracerebral hemorrhage, and mortality at three months between all dose tiers of tenecteplase and alteplase. RESULTS: Three relevant studies (Haley et al., Parsons et al., and ATTEST) included 291 patients and investigated three doses of tenecteplase (0.1, 0.25, 0.4 mg/kg). There were no differences between any dose of tenecteplase and alteplase for either efficacy or safety end points. Tenecteplase 0.25 mg/kg had the greatest odds to achieve early neurological improvement (OR [95%CI] 3.3 [1.5, 7.2], p = 0.093), excellent functional outcome (modified Rankin Scale 0-1) at three months (OR [95%CI] 1.9 [0.8, 4.4], p = 0.28), with reduced odds of intracerebral hemorrhage (OR [95%CI] 0.6 [0.2, 1.8], P = 0.43) compared with alteplase. Only 19 patients were treated with tenecteplase 0.4 mg/kg, which showed increased odds of symptomatic intracerebral hemorrhage compared with alteplase (OR [95% CI] 6.2 [0.7, 56.3]). CONCLUSIONS: While no significant differences between tenecteplase and alteplase were found, point estimates suggest potentially greater efficacy of 0.25 and 0.1 mg/kg doses with no difference in symptomatic intracerebral hemorrhage, and potentially higher symptomatic intracerebral hemorrhage risk with the 0.4 mg/kg dose. Further investigation of 0.25 mg/kg tenecteplase is warranted.

OBJECTIVE: To provide recommendations for the comprehensive management of young infants who present with signs or symptoms concerning for laryngomalacia. METHODS: Expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG). RESULTS: Consensus recommendations include initial care and triage recommendations for health care providers who commonly evaluate young infants with noisy breathing. The consensus statement also provides comprehensive care recommendations for otolaryngologists who manage young infants with laryngomalacia including: evaluation and treatment considerations for commonly debated issues in laryngomalacia, initial work-up of infants presenting with inspiratory stridor, treatment recommendations based on disease severity, management of the infant with feeding difficulties, post-surgical treatment management recommendations, and suggestions for acid suppression therapy. CONCLUSION: Laryngomalacia care consensus recommendations are aimed at improving patient-centered care in infants with laryngomalacia.

INTRODUCTION: Although rare, complications in cochlear implantation may result from surgical or technical mishaps, reaction to the foreign body, infection, or mechanical device failure. Delayed cerebrospinal fluid (CSF) leak is a rarely reported condition that may present with asymptomatic swelling over the receiver-stimulator (RS). In our practice, meticulous drilling of a bony well is important in preventing device migration and maintaining a low device profile but there is the potential for immediate or delayed complication from this technique. OBJECTIVE: We report two cases of the diagnosis and management of delayed extradural CSF collection of the RS bony well and describe its successful management. PATIENTS: Two pediatric cochlear implant patients, 10 and 17 months of age with devices from different manufacturers. INTERVENTION(S): Operative exploration and repair without device removal. MAIN OUTCOME AND RESULTS: Although the initial postoperative course was uncomplicated with both patients receiving benefit from their device, both presented at varying intervals month(s) later with swelling over the RS. There were no signs of infection but the swelling prevented use of the device. Extradural CSF collection was suspected, confirmed operatively, and repaired with complete resolution without the need for reimplantation. CONCLUSION: Delayed CSF leak may present as an asymptomatic swelling over the RS after cochlear implantation. Sterile fluid aspiration may confirm the diagnosis and management can proceed conservatively or with operative exploration and repair. Future device designs with lower profiles may facilitate device fixation while allowing for a more shallow well, further reducing the risk of this rare complication.

A craniotomy over the superior cerebellar convexity for approaches to this region typically involves a small infratentorial craniotomy and then drilling down of the bone to expose some portion of the transverse/sigmoid sinuses. The authors describe the anatomy of the region and the method for a two-part paramedian occipital and suboccipital craniotomy (supra and infratentorial) that may have time-saving, safety, and cosmetic advantages. For this technique, a supratentorial craniotomy is used to expose the transverse sinus from above, and subsequently, dissection across the sinus over the cerebellar convexity can be done under direct vision. The two bone pieces are joined on the inner table side while plates for fixation above the superior nuchal line can be counter-sunk to avoid post-operative pain from the prominence of screws. There is no need for cranioplasty materials since there is no burring down of bone for adequate exposure of the transverse sinus. The technique has been used by two senior surgeons over the years convincing them of the speed, safety, and utility of the technique. Here, the authors present a single example of the technique.