Intractable epilepsy impacts many children. Surgically resective and palliative treatments have developed to increase seizure freedom or palliate the seizure burden in those with medically refractory epilepsy. However, surgical epilepsy treatment can confer significant morbidity and death. Endoscope-assisted surgical approaches may be helpful in reducing the morbidity related to traditional open surgical approaches while allowing for good visualization of surgical targets. Here, the authors report a case utilizing an endoscope-assisted keyhole approach to perform a posterior quadrantectomy and corpus callosotomy, achieving the surgical goals of disconnection and reducing the need for large craniotomy exposure. They present the case of a 17-year-old male with medically refractory epilepsy treated with endoscope-assisted posterior quadrantectomy and corpus callosotomy through two mini-craniotomies to achieve a functional disconnection. To the authors’ knowledge, this is the first reported case of an endoscope-assisted approach for a posterior quadrantectomy for surgical epilepsy treatment in an adult or a pediatric patient. The case is reported to highlight the technical nuances and benefits of this approach in select patients as well as the expansion of applications of endoscope-assisted epilepsy surgery.
Melissa A. LoPresti, Kathryn Wagner, and Sandi Lam
Melissa A. LoPresti, Joshua Nguyen, and Sandi K. Lam
Head immobilization devices with skull pins are commonly used by neurosurgeons to stabilize the head for microsurgical techniques and to maintain accurate intraoperative neuronavigation. Pediatric patients, who may have open fontanelles, unfused sutures, and thin skulls, are vulnerable to complications during placement in pins. We review the various methods of pinning in pediatric neurosurgery and revisit the modified rubber stopper technique using a commonly available rubber stopper from a medication bottle over a standard adult pin of a Mayfield head clamp to prevent the pins from plunging through the thin pediatric skull.
Donald A. Ross
Nathan A. Shlobin, Melissa A. LoPresti, Rebecca Y. Du, and Sandi Lam
Neural tube defects (NTDs) are common congenital neurological defects, resulting in mortality, morbidity, and impaired quality of life for patients and caregivers. While public health interventions that increase folate consumption among women who are or plan to become pregnant are shown to reduce folate-sensitive NTDs, public health policy reflecting the scientific evidence lags behind. The authors aimed to identify the types of policies applied, associated outcomes, and impact of folate fortification and supplementation on NTDs worldwide. By identifying effective legislation, the authors aim to focus advocacy efforts to more broadly effect change, reducing the burden of NTDs in neurosurgery.
A systematic review was conducted exploring folate fortification and supplementation policies using the PubMed and Scopus databases. Titles and abstracts from articles identified were read and selected for full-text review. Studies meeting inclusion criteria were reviewed in full and analyzed for study design, aim, population, interventions, and outcomes.
Of 1637 resultant articles, 54 were included. Mandatory folate fortification was effective at reducing folate-sensitive NTDs. Mandatory fortification also decreased hospitalization rates and deaths after discharge and increased 1st-year survival for infants with NTDs. Recommended folate supplementation also resulted in decreased NTDs; however, issues with compliance and adherence were a concern and impacted effectiveness. Folate fortification and/or supplementation resulted in decreased NTD prevalence, although more change was attributed to fortification. Dual policies may hold the most promise. Furthermore, reductions in NTDs were associated with significant cost savings over time.
Both mandatory folate fortification and recommended supplementation policies were found to effectively decrease folate-sensitive NTD rates when applied. A comprehensive approach incorporating mandatory folate fortification, appropriate folate supplementation, and improved infrastructure and access to prenatal care may lead to decreased NTDs worldwide. This approach should be context-specific, emphasize education, and account for regional access to healthcare and social determinants of health. With wide implications for NTDs, associated health outcomes, quality of life of patients and caregivers, and economic impacts, policy changes can drastically improve global NTD outcomes. As caretakers of children with NTDs, the authors as neurosurgeons advocate for a comprehensive policy, the engagement of stakeholders, and a broader global impact.
Rebecca Y. Du, Melissa A. LoPresti, Roxanna M. García, and Sandi Lam
Road traffic accidents are the most frequent cause of severe traumatic brain injury (TBI), particularly among young populations worldwide. Helmets are proven to prevent injuries; however, estimates of helmet compliance are low globally. Surgical/critical care management of TBI is often used to treat these injuries, but primary prevention should be recommended. A key component in promoting TBI prevention among pediatric and young populations is through helmet legislation. The authors investigated helmet policies for motorcycles and bicycles globally to provide recommendations for how related legislation may impact TBI and guide advocacy in pediatric neurosurgery.
The authors conducted a systematic review of helmet laws and/or policies by using the National Library of Medicine PubMed and SCOPUS databases. Additional articles were identified using citation searches of key publications. Abstracts from articles of all sources were read and selected for full-text review. Details of relevant full articles were extracted and analyzed for the following: bibliographic data, study aim, design and duration, study participants, intervention characteristics, and intervention effect data.
Of 618 search results, 53 full-text articles were analyzed for recommendations. Helmet legislation is associated with increased helmet use among bicyclists and decreased road traffic accident–related head injuries and fatalities among motorcyclists and bicyclists. Laws are more effective if comprehensive and inclusive of the following: both primary riders and passengers, all age groups, all modes of transportation made safer by helmets, a proper use clause, and standardized helmet quality measures. Cultural, socioeconomic, and infrastructural circumstances are important as well, and legislation must consider enforcement mechanisms with penalties significant enough to incentivize behavioral changes, but proportional to community socioeconomic status.
Compulsory use laws are the optimal primary intervention; however, concurrent programs to support financial access to helmets, change cultural attitudes, increase health literacy, and improve road infrastructure will augment legislative benefits. Pediatric neurosurgeons are caretakers of children suffering from TBI. Although extensive study has explored the surgical management of TBI, the authors believe that primary prevention is instrumental to improving outcomes and reducing injury. All helmet laws are not equal; based on these findings, a comprehensive, context-specific approach is the key to success, especially in resource-limited countries.
Melissa A. LoPresti, Eric A. Goethe, James C. Bayley, Brandon Bertot, Peter T. Kan, and Sandi Lam
Neonatal intracerebral hemorrhage is associated with substantial morbidity and mortality. Treatment is largely conservative, though interventions to evacuate intraventricular and intraparenchymal hematomas (IPHs) have been applied. Endoscopic ultrasonic aspiration for the treatment of IPH has increasingly been shown to be a useful strategy in adults; however, it has not been studied in children, and the technology has been more commonly applied to intraventricular hemorrhage (IVH). Here, the authors describe, to the best of their knowledge, the first use of endoscopic ultrasonic aspiration for IPH in a newborn.
An 8-week-old female presented with IPH secondary to left M3 aneurysm rupture, which was treated with coil embolization for aneurysm securement and vessel sacrifice, followed by IPH evacuation using endoscopic ultrasonic aspiration. Through applying this approach in a newborn, the authors gained technical insight not previously reported in the application of this technique in similar cases in adults or in cases of IVH. They highlight this case to share learning points and technical challenges regarding the application of endoscopic ultrasonic aspiration in a newborn along with learning points for imaging and visualization. Endoscopic ultrasonic aspiration can be used to treat IPH in select newborns. Further study is needed to improve efficacy and ease when applying this approach in very young patients.
Jacob Cherian, Kristen A. Staggers, I-Wen Pan, Melissa Lopresti, Andrew Jea, and Sandi Lam
Due to improved nutrition and early detection, myelomeningocele repair is a relatively uncommon procedure. Although previous studies have reviewed surgical trends and predictors of outcomes, they have relied largely on single-hospital experiences or on databases centered on hospital admission data. Here, the authors report 30-day outcomes of pediatric patients undergoing postnatal myelomeningocele repair from a national prospective surgical outcomes database. They sought to investigate the association between preoperative and intraoperative factors on the occurrence of 30-day complications, readmissions, and unplanned return to operating room events.
The 2013 American College of Surgeons National Surgical Quality Improvement Program Pediatric database (NSQIP-P) was queried for all patients undergoing postnatal myelomeningocele repair. Patients were subdivided on the basis of the size of the repair (< 5 cm vs > 5 cm). Preoperative variables, intraoperative characteristics, and postoperative 30-day events were tabulated from prospectively collected data. Three separate outcomes for complication, unplanned readmission, and return to the operating room were analyzed using univariate and multivariate logistic regression. Rates of associated CSF diversion operations and their timing were also analyzed.
A total of 114 patients were included; 54 had myelomeningocele repair for a defect size smaller than 5 cm, and 60 had repair for a defect size larger than 5 cm. CSF shunts were placed concurrently in 8% of the cases. There were 42 NSQIP-defined complications in 31 patients (27%); these included wound complications and infections, in addition to others. Postoperative wound complications were the most common and occurred in 27 patients (24%). Forty patients (35%) had at least one subsequent surgery within 30 days. Twenty-four patients (21%) returned to the operating room for initial shunt placement. Unplanned readmission occurred in 11% of cases. Both complication and return to operating room outcomes were statistically associated with age at repair.
The NSQIP-P allows examination of 30-day perioperative outcomes from a national prospectively collected database. In this cohort, over one-quarter of patients undergoing postnatal myelomeningocele repair experienced a complication within 30 days. The complication rate was significantly higher in patients who had surgical repair within the first 24 hours of birth than in patients who had surgery after the 1st day of life. The authors also highlight limitations of investigating myelomeningocele repair using NSQIP-P and advocate the importance of disease-specific data collection.
Vijay M. Ravindra, Stephen F. Kralik, Julius Griauzde, Nisha Gadgil, Melissa A. LoPresti, and Sandi Lam
Moyamoya disease is a progressive occlusive arteriopathy for which surgical revascularization is indicated. In this retrospective study, the authors investigated the use of preoperative CT perfusion with the aim of establishing pathological data references.
The authors reviewed the medical records of children with moyamoya disease treated surgically at one institution between 2016 and 2019. Preoperative CT perfusion studies were used to quantify mean transit time (MTT), cerebral blood volume (CBV), cerebral blood flow (CBF), and time to peak (TTP) for the anterior, middle, and posterior cerebral artery vascular territories for each patient. CT perfusion parameter ratios (diseased/healthy hemispheres) and absolute differences were compared between diseased and normal vascular territories (defined by catheter angiography studies). Sensitivity, specificity, and positive (PPV) and negative (NPV) predictive values for CT perfusion parameters for severe angiographic moyamoya were calculated.
Nine children (89% female) had preoperative CT perfusion data; 5 of them had evidence of unilateral hemispheric disease and 4 had bilateral disease. The mean age at revascularization was 77 months (range 40–144 months). The etiology of disease was neurofibromatosis type 1 (3 patients), Down syndrome (2), primary moyamoya disease (2), cerebral proliferative angiopathy (1), and sickle cell disease (1). Five patients had undergone unilateral revascularization. Among these patients, pathological vascular territories demonstrated increased MTT in 66% of samples, increased TTP in 66%, decreased CBF in 47%, and increased CBV in 87%. Severe moyamoya (Suzuki stage ≥ 4) had diseased/healthy ratios ≥ 1 for MTT in 78% of cases, for TTP in 89%, for CBF in 67%, and for CBV in 89%. The MTT and TTP region of interest ratio ≥ 1 demonstrated 89% sensitivity, 67% specificity, 80% PPV, and 80% NPV for the prediction of severe angiographic moyamoya disease.
Pathological hemispheres in these children with moyamoya disease demonstrated increased MTT, TTP, and CBV and decreased CBF. The authors’ results suggest that preoperative CT perfusion may, with high sensitivity, be useful in deciphering perfusion mismatch in brain tissue in children with moyamoya disease. More severe angiographic disease displays a more distinct correlation, allowing surgeons to recognize when to intervene in these patients.
Melissa A. LoPresti, Vijay M. Ravindra, Monika Pyarali, Eric Goethe, Nisha Gadgil, Kathyrn Wagner, Peter Kan, and Sandi Lam
Intracranial arteriovenous malformations (AVMs) are a common cause of intracranial hemorrhage in children. Exploring outcomes of treatment and factors that predict recurrence may help guide the management of pediatric AVMs. In this study, the authors review a single-center experience with pediatric AVMs, aiming to identify factors contributing to recurrence and areas for improvement in treatment.
A single-center retrospective review of patients ages 0–18 years with AVMs in the period from 2005 to 2018 was conducted. Demographic, clinical, and radiographic data were collected and examined to compare the clinical and functional outcomes of patients managed with and those managed without open microsurgical resection.
One hundred five patients were diagnosed and treated during the study period. Fifty (47.6%) had been treated surgically and 35 (33.3%) nonsurgically, and 20 (19%) had been treated primarily at an outside institution and thus were excluded from further analysis. Of the 50 treated surgically, 92% had complete obliteration on postoperative imaging, with the remaining patients having residual AVM and undergoing re-resection or SRS, ultimately resulting in a 96% radiographic cure rate. The surgically treated patients were followed up for an average of 3.34 years. Eighty-two percent had a modified Rankin Scale (mRS) score of 0–2. There was a recurrence rate of 12%, with the time to recurrence averaging 327.86 days (range 85–596 days). Of the 35 patients managed without open surgery, 77% underwent serial imaging surveillance, 20% underwent endovascular embolization, and 3% underwent stereotactic radiosurgery. Radiographic cure was seen in 6% of those treated nonsurgically, and all had undergone endovascular treatment alone. The nonsurgically treated patients were followed up for an average of 2.14 years. Eighty percent had an mRS score of 0–2.
The data revealed that patients with lower-grade AVMs treated surgically and those with higher-grade AVMs managed without surgery have good functional and clinical outcomes. A high rate of recurrence was noted, and the possible contributing factors were explored. The authors encourage further study into mechanisms to reduce AVM recurrence in pediatric patients and look forward to advances in the medical management of lesions that are considered unresectable.