Incidentally found brain tumors in the pediatric population: a case series and proposed treatment algorithm.

PURPOSE: With the increasing use of imaging, there has been an increase in the number of incidentally found brain lesions in pediatric patients resulting in a treatment dilemma for physicians and emotional strain for patients and families. Adult studies support initial surveillance of incidentally found low grade appearing lesions as the most appropriate approach. The aim of this study was to evaluate incidental lesions in the pediatric population and propose an initial treatment algorithm for such lesions. METHODS: Pediatric records were retrospectively reviewed at Johns Hopkins All Children's Hospital for incidentally found brain tumors between 2000 and 2017. Demographic data, presenting symptoms, treatment approach, and outcomes were reviewed for 55 patients, age 0-18. RESULTS: Of the 55 patients included in the study, 14 underwent surgical resection, 3 underwent biopsy, and 38 with benign imaging characteristics at presentation were monitored with radiology alone. Only one patient, out of the 17 that underwent resection or biopsy, had pathology consistent with a high grade glioma. Of the patients monitored radiographically 10 total patients showed an increase in the overall size of the lesion; however after a median follow up of 34.2 months only 2 increased to a degree that required surgical intervention. CONCLUSIONS: The majority of incidentally found brain lesions with benign imaging characteristics at presentation may be managed conservatively. Surveillance is an important part of the initial management of incidental lesions in the pediatric population, but careful scrutiny must be paid to the potential for higher grade lesions or malignant transformation.

Urological Outcome of the Xiao Procedure in Children with Myelomeningocele and Lipomyelomeningocele Undergoing Spinal Cord Detethering.

PURPOSE: Although previous studies have revealed high success rates (70% to 85%) after an intradural somatic-to-autonomic nerve transfer procedure in children with spinal dysraphism, no study has had a control group or blinded observers. We report a rigorously designed study to investigate the effectiveness of the Xiao procedure. MATERIALS AND METHODS: Children with neurogenic bladder dysfunction related to myelomeningocele or lipomyelomeningocele who required spinal cord detethering were randomized to 2 groups at surgery, with half undergoing only spinal cord detethering and half undergoing the Xiao procedure in addition to detethering. Double-blind evaluations were performed at regular intervals during the 3-year followup. RESULTS: A total of 10 patients underwent spinal cord detethering only and 10 underwent detethering plus the Xiao procedure. The Xiao procedure did not result in voluntary voiding or continence in any patient, but patients undergoing spinal cord detethering plus the Xiao procedure were more likely to have greater improvements in total bladder capacity, bladder overactivity and overall quality of life than those who underwent detethering only. By the end of the study no participant or evaluator was able to accurately predict to which group the patients had been assigned. CONCLUSIONS: The results of this randomized controlled trial are in agreement with recently published similarly poor results of the Xiao procedure in patients with spinal cord injury. Improvements in bladder parameters observed in this study may be related to sacral nerve root section, a necessary portion of the Xiao procedure, instead of reinnervation. Confirmatory animal studies are recommended before further clinical trials of the Xiao procedure are performed in humans.

Pediatric microcystic meningioma: a clinical, histological, and radiographic case-based review.

BACKGROUND: Microcystic meningioma (MM) is a World Health Organization grade I tumor that is rare in the pediatric population. Meningiomas account for approximately 2-4 % of all childhood central nervous system (CNS) tumors compared to approximately 20 % of all adult CNS tumors. The authors present one of the few confirmed cases of microcystic meningioma in a child and discuss the characteristic radiographic appearance and histological findings. HISTORY: We report the case of an 11-year-old boy who presented with first-time seizure and imaging consistent with brain tumor. There was significant vasogenic edema within the entire right hemisphere, disproportionate to the size of the falcine-based tumor. Histopathological analysis revealed the microcystic subtype of meningioma. DISCUSSION: We review the radiographic characteristics, histopathological findings, and reported pediatric cases of MM in conjunction with our case. CONCLUSION: MM has distinct radiographic characteristics (variable enhancement, lack of a dural tail, and disproportionate vasogenic edema) that can be misinterpreted in the pediatric population, suggesting a more aggressive tumor.

Adolescent Disc Disease: Risk Factors and Treatment Success-Related Factors.

OBJECTIVE: A paucity of literature is available discussing the associated risk factors, treatment options (including the use of minimally invasive surgery), and outcomes related to lumbar disc herniation (LDH) in children. We have discussed the risk factors for disc disease among pediatric patients and evaluated the efficacy of the minimally invasive approach. METHODS: A retrospective review of pediatric patients with lumbar disc disease who had undergone microdiscectomy at our institution from 2005 to 2016 was conducted. The preoperative presentation, hospital course, postoperative course, and follow-up data (≥3 years) were reviewed. We evaluated the risk factors for LDH and the surgical outcomes for both groups. RESULTS: A total of 52 pediatric patients had undergone 61 lumbar disc surgeries for LDH in our department from 2005 to 2016. Their average age at surgery was 16.65 years. Of the 61 procedures, 48 (78.7%) had been performed via the minimally invasive spine microdiscectomy approach and 13 (21.3%) via the open microdiscectomy approach. The average body mass index for all cases was 29.3 kg/m2. The average interval to diagnosis was 7.9 months. Of the 61 cases, 21 (34.4%) had been required for patients who were competitive athletes. In addition, 15 had been for LDH related to trauma (24.6%). In 46 of the 61 cases, complete resolution of the symptoms had occurred at the 1-year follow-up visit (79.2% of minimally invasive spine microdiscectomy vs 61.5% of open microdiscectomy). CONCLUSION: Risk factors similar to those for adult LDH, such as an elevated body mass index, can be seen in the pediatric population. However, some unique risk factors such as post-traumatic LDH were found in the pediatric age group. Minimally invasive techniques are demonstrably safe and useful in this patient population.

Pediatric mild head trauma: is outpatient follow-up imaging necessary or beneficial?

OBJECTIVE: Pediatric traumatic brain injury (TBI) is the leading cause of death among children and is a significant cause of morbidity. However, the majority of injuries are mild (Glasgow Coma Scale score 13-15) without any need for neurosurgical intervention, and clinically significant neurological decline rarely occurs. Although the question of repeat imaging within the first 24 hours has been discussed in the past, the yield of short-term follow-up imaging has never been thoroughly described. In this paper, the authors focus on the yield of routine repeat imaging for pediatric mild TBI (mTBI) at the first clinic visit following hospital discharge. METHODS: The authors conducted a retrospective review of patients with pediatric brain trauma who had been admitted to Johns Hopkins All Children's Hospital (JHACH). Patients with mTBI were identified, and their presentation, hospital course, and imaging results were reviewed. Those pediatric patients with mTBI who had undergone no procedure during their initial admission (only conservative treatment) were eligible for inclusion in the study. Two distinct groups were identified: patients who underwent repeated imaging at their follow-up clinic visit and those who underwent only clinical evaluation. Each case was assessed on whether the follow-up imaging had changed the follow-up course. RESULTS: Between 2010 and 2015, 725 patients with TBI were admitted to JHACH. Of those, 548 patients qualified for analysis (i.e., those with mTBI who received conservative treatment without any procedure and were seen in the clinic for follow-up evaluation within 8 weeks after the trauma). A total of 392 patients had only clinic follow-up, without any diagnostic imaging study conducted as part of their clinic visit, whereas the other 156 patients underwent repeat MRI. Only 1 patient had a symptomatic change and was admitted after undergoing imaging. For 30 patients (19.2%), it was decided after imaging to continue the neurosurgical follow-up, which is a change from the institutional paradigm after mTBI. None of these patients had a change in neurological status, and all had a good functional status. All of these patients had one more follow-up in the clinic with new MRI, and none of them required further follow-up. CONCLUSIONS: Children with mTBI are commonly followed up in the ambulatory clinic setting. The authors believe that for children with mTBI, normal clinical examination, and no new symptoms, there is no need for routine ambulatory imaging since the clinical yield of such is relatively low.

Early postnatal cranial vault reduction and fixation surgery for severe hydrocephalic macrocephaly.

OBJECTIVE Infants with severe hydrocephalus and extreme macrocephaly typically undergo CSF diversion early in life, which can result in significant cranial deformity due to CSF overdrainage. In this scenario, overlap of the cranial plates can precede the development of secondary synostosis and/or severe, permanent cranial deformity. As a result, extensive cranial vault remodeling is sometimes undertaken later in life, which is often challenging and has been associated with mortality and a high morbidity rate. The authors have previously described a technique for early postnatal cranial vault reduction and fixation (CVRF), in which the calvarial bones are stabilized using absorbable fixation plates in the neonatal period, in an attempt to facilitate patient positioning, simplify hydrocephalus management, and improve cosmesis. Here, the authors describe their institutional experience managing patients with extreme neonatal hydrocephalus with CSF diversion, with and without CVRF, over the past 12 years. METHODS The authors retrospectively reviewed the charts of infants with extreme hydrocephalus (head circumference > 49 cm) treated at their children's hospital with ventriculoperitoneal shunting, with or without CVRF, between 2005 and 2017. Data collected included age, sex, etiology of hydrocephalus, type of CVRF performed (anterior, posterior, or combined), follow-up duration, orbitofrontal circumference, craniometric measurements, intraoperative blood loss, operative duration, and postoperative complications. Developmental data were collected using the third edition of the Ages and Stages Questionnaire. Photographic imaging was used to demonstrate esthetic outcomes, and family questionnaires were used to evaluate satisfaction with the esthetic outcome. RESULTS Eleven patients with extreme neonatal hydrocephalus underwent CSF shunting; 5 underwent shunting alone and 6 patients underwent shunting and CVRF. For patients who underwent shunting and CVRF, the median age at CVRF was 6 days and the median interval between shunt placement and CVRF was 2.5 days. The mean extent of calvarial vault volume reduction was 44.5% (± 3.9%). The mean duration of the CVRF procedure was 108 minutes, and 5 of 6 patients required intraoperative transfusion. Of the 5 patients who underwent shunting alone, 3 developed severe cranial deformities. Of 6 patients who underwent shunting and CVRF, 1 had a poor cosmetic outcome. In the shunting-alone group, 2 patients died and 1 required extensive cranial vault correction at 10 years of age. One patient in the shunting and CVRF group also died. CONCLUSIONS CVRF in combination with CSF shunting in the neonatal period can simplify the treatment of the rare case of severe hydrocephalic macrocephaly and leads to cosmetic outcomes that are considered good by their families.

Management of hydrocephalus and subdural hygromas in pediatric patients after decompression of Chiari malformation type I: case series and review of the literature.

OBJECTIVE: Hydrocephalus associated with subdural hygromas is a rare complication after decompression of Chiari malformation type I (CM-I). There is no consensus for management of this complication. The authors present a series of 5 pediatric patients who underwent CM-I decompression with placement of a dural graft complicated by posterior fossa hygromas and hydrocephalus that were successfully managed nonoperatively. METHODS: A retrospective review over the last 5 years of patients who presented with hydrocephalus and subdural hygromas following foramen magnum decompression with placement of a dural graft for CM-I was conducted at 2 pediatric institutions. Their preoperative presentation, perioperative hospital course, and postoperative re-presentation are discussed with attention to their treatment regimen and ultimate outcome. In addition to reporting these cases, the authors discuss all similar cases found in their literature review. RESULTS: Over the last 5 years, the authors have encountered 194 pediatric cases of CM-I decompression with duraplasty equally distributed at the 2 institutions. Of those cases, 5 pediatric patients with a delayed postoperative complication involving hydrocephalus and subdural hygromas were identified. The 5 patients were managed nonoperatively with acetazolamide and high-dose dexamethasone; dosages of both drugs were adjusted to the age and weight of each patient. All patients were symptom free at follow-up and exhibited resolution of their pathology on imaging. Thirteen similar pediatric cases and 17 adult cases were identified in the literature review. Most reported cases were treated with CSF diversion or reoperation. There were a total of 4 cases previously reported with successful nonoperative management. Of these cases, only 1 case was reported in the pediatric population. CONCLUSIONS: De novo hydrocephalus, in association with subdural hygromas following CM-I decompression, is rare. This presentation suggests that these complications after posterior fossa decompression with duraplasty can be treated with nonoperative medical management, therefore obviating the need for CSF diversion or reoperation.

A Modified Technique for Occipitocervical Fusion Using Compressed Iliac Crest Allograft Results in a High Rate of Fusion in the Pediatric Population.

BACKGROUND: In children, high rates of occipitocervical (OC) fusion have been demonstrated with the use of rigid instrumentation in combination with harvested autograft, with or without bone morphogenetic protein (BMP). Historically, the use of allograft materials demonstrated inferior OC fusion outcomes compared with autograft. However, autograft harvest harbors an increased risk of patient morbidity, and the use of BMP is costly and controversial in children. Thus, there remains a need for safer, less costly, yet efficacious techniques for OC fusion in the pediatric population. METHODS: We retrospectively reviewed the charts of patients younger than 21 years of age who underwent OC fusion with structural allograft placement at our institution from 2010 to 2015. Data collected included age, sex, follow-up duration, fusion outcomes, and postoperative complications. RESULTS: A total of 19 patients (8 female and 11 male) underwent OC fusion with our surgical technique. Mean age was 8.5 ± 4.3 years. Radiographic follow up data were available for 18 of 19 patients. One patient was lost to clinical follow up but had radiographic confirmation of fusion. Thus, 18 of 18 (100%) of patients with radiographic follow-up achieved successful arthrodesis as determined by computed tomography. Median duration to documented fusion was 4.5 months. Clinical follow-up was available for 17 of 19 patients and was on average 18.8 ± 13.5 months. One patient required reoperation for graft fracture 8 months after radiographic confirmation of successful fusion. There were no vertebral artery injuries or other postoperative complications. CONCLUSIONS: We demonstrate a modified technique for OC fusion in children with unique structural allograft shaping and affixation, leading to excellent fusion outcomes at follow up. This technique obviates the need for autograft harvest or BMP, and may decrease postoperative morbidity.

Allograft versus autograft for pediatric posterior cervical and occipito-cervical fusion: a systematic review of factors affecting fusion rates.

OBJECT Fusion rates are high for children undergoing posterior cervical fusion (PCF) and occipito-cervical fusion (OCF). Autologous bone has been widely used as the graft material of choice, despite the risk of donor-site morbidity associated with harvesting the bone, possibly because very low fusion rates were reported with posterior allograft cervical fusions in children several decades ago. Higher overall fusion rates using allograft in adults, associated with improvements in internal fixation techniques and the availability of osteoinductive substances such as bone morphogenetic protein (BMP), have led to heightened enthusiasm for the use of bank bone during pediatric PCF. A systematic review was performed to study factors associated with successful bone fusion, including the type of bone graft used. METHODS The authors performed a comprehensive PubMed search of English-language articles pertaining to PCF and OCF in patients less than 18 years old. Of the 561 abstracts selected, 148 articles were reviewed, resulting in 60 articles that had sufficient detail to be included in the analysis. A meta-regression analysis was performed to determine if and how age, fusion technique, levels fused, fusion substrate, BMP use, postoperative bracing, and radiographic fusion criteria were related to the pooled prevalence estimates. A systematic review of the literature was performed according to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) statement. RESULTS A total of 604 patients met the specific inclusion and exclusion criteria. The overall fusion rate was 93%, with a mean age of 9.3 years and mean follow-up of 38.7 months. A total of 539 patients had fusion with autograft (94% fusion rate) and 65 patients with allograft (80% fusion rate). Multivariate meta-regression analysis showed that higher fusion rates were associated with OCF compared with fusions that excluded the occiput (p < 0.001), with the use of autograft instead of allograft (p < 0.001), and with the use of CT to define fusion instead of plain radiography alone. The type of internal fixation, the use of BMP, patient age, and the duration of follow-up were not found to be associated with fusion rates in the multivariate analysis. CONCLUSIONS Fusion rates for PCF are high, with higher rates of fusion seen when autograft is used as the bone substrate and when the occiput is included in the fusion construct. Further study of the use of allograft as a viable alternative to autograft bone fusion is warranted because limited data are available regarding the use of allograft in combination with more rigid internal fixation techniques and osteoinductive substances, both of which may enhance fusion rates with allograft.

Use of a contoured bioresorbable plate with a hemostatic plug to control life-threatening bleeding from the superior sagittal sinus during hemispherotomy: technical note.

Profuse bleeding originating from an injured cerebral sinus can be a harrowing experience for any surgeon, particularly during an operation on a young child. Common surgical remedies include sinus ligation, primary repair, placement of a hemostatic plug, and patch or venous grafting that may require temporary stenting. In this paper the authors describe the use of a contoured bioresorbable plate to hold a hemostatic plug in place along a tear in the inferomedial portion of a relatively inaccessible part of the posterior segment of the superior sagittal sinus in an 11-kg infant undergoing hemispherotomy for epilepsy. This variation on previously described hemostatic techniques proved to be easy, effective, and ultimately lifesaving. Surgeons may find this technique useful in similar dire circumstances when previously described techniques are ineffective or impractical.

Lack of efficacy of an intradural somatic-to-autonomic nerve anastomosis (Xiao procedure) for bladder control in children with myelomeningocele and lipomyelomeningocele: results of a prospective, randomized, double-blind study.

OBJECTIVE Xiao et al. and other investigators have studied an intradural somatic-to-autonomic (e.g., L-5 to S3-4) nerve transfer as a method to create a reflex arc to allow bladder emptying in response to cutaneous stimulation (the Xiao procedure). In previous clinical studies of patients with spinal dysraphism who underwent the Xiao procedure, high success rates (70%-85%) were reported for the establishment of a "skin-CNS-bladder" reflex arc that allows spontaneous, controlled voiding in children with neurogenic bladder dysfunction. However, many of these studies did not use blinded observers, did not have control groups, and/or featured only limited follow-up durations. METHODS A randomized, prospective, double-blind trial was initiated in March 2009, enrolling children with myelomeningocele (MM), lipomyelomeningocele (LMM), and neurogenic bladder dysfunction who were scheduled for spinal cord detethering (DT) for the usual indications. At the time of DT, patients were randomized between 2 arms of the study: half of the patients underwent a standard spinal cord DT procedure alone (DT group) and half underwent DT as well as the Xiao procedure (DT+X group). Patients, families, and study investigators, all of whom were blinded to the surgical details, analyzed the patients' strength, sensory function, mobility, voiding, and urodynamic bladder function before surgery and at regular intervals during the 3-year follow-up. RESULTS Twenty patients were enrolled in the study: 10 underwent only DT and the other 10 underwent DT+X. The addition of the Xiao procedure to spinal cord DT resulted in longer operative times (p = 0.024) and a greater chance of wound infection (p = 0.03). Patients in both treatment arms could intermittently void or dribble small amounts of urine (< 20% total bladder capacity) in response to scratching in dermatomes T-9 through S-2 using a standardized protocol, but the voiding was not reproducible and the volume voided was not clinically useful in any patient. Voiding in response to scratching was not more frequent in patients who underwent DT+X compared with those who underwent only DT. Bladder contractions in response to scratching occurred in both treatment arms at various intervals after surgery, but they were not more reproducible or more frequent in the patients who underwent the Xiao procedure than in the patients who did not. No patient in either treatment arm was continent of urine before, during, or after the study. CONCLUSIONS Patients with MM and LMM who underwent the Xiao procedure during spinal cord DT were no more likely to be able to void, to control their urination, to achieve continence, or to have a demonstrable urodynamic bladder contraction in response to cutaneous stimulation than patients who underwent only spinal cord DT. This study, in the context of disappointing results reported in other recent studies of the Xiao procedure, raises doubts about the clinical applicability of this procedure in humans until further basic science research is performed.

Accidental intracranial infusion of parenteral nutrition in a preterm neonate.

Vascular access in the neonate can be challenging, especially in preterm infants. When other access is not available, superficial scalp veins can be safely used for vascular access. However, rare and potentially catastrophic complications can occur due to unique features of the neonatal skull and soft-tissue anatomy. The authors report a rare complication of vascular access in a preterm infant, which led to the direct infusion of parenteral nutrition into the intracranial space. The child had an excellent outcome after open drainage and irrigation of bilateral intracranial spaces and the spinal thecal sac. Relevant anatomy is illustrated, and an outcome-based literature review is presented on this rarely reported condition. Surgical and conservative management strategies are discussed, along with clinical and radiographic follow-up. Drainage and irrigation is advocated in patients with mass effect, viscous effusions, or declining neurological examination findings.

Cranial reduction and fixation with a resorbable plate combined with cerebrospinal fluid shunting for difficult-to-manage macrocephaly related to hydrocephalus.

The management of newborns with extreme macrocephaly related to hydrocephalus can be difficult; balancing the treatment of severe cranial deformity with optimal hydrocephalus management can be complicated. Excessive CSF drainage can result in significant suture overlap that leads to difficulties in patient positioning, secondary synostosis, and long-term aesthetic complications. Delayed cranial reduction and remodeling procedures carry significant risk, and the aesthetic outcomes have sometimes been poor. The authors describe a newborn with severe macrocephaly who underwent shunt placement followed by a limited cranial reduction and fixation procedure using an absorbable plate within the 1st week of life. The procedure produced an immediate intracranial volume reduction of 49%. This novel management strategy facilitated patient positioning, simplified hydrocephalus management, and provided an excellent aesthetic outcome.

Attempted bladder reinnervation and creation of a scratch reflex for bladder emptying through a somatic-to-autonomic intradural anastomosis.

An intradural somatic-to-autonomic anastomosis, or Xiao procedure, has been described to create a "skin-CNS-bladder" reflex that improves bladder and bowel function in patients with neurogenic bladder and bowel dysfunction. The authors present their experience with a 10-year-old boy with chronic neurogenic bladder and bowel dysfunction related to spinal cord injury who underwent the Xiao procedure. After undergoing a left L-5 ventral root to left S2-3 intradural anastomosis, the patient reported that his bladder and bowel dysfunction improved between 6 and 12 months. Two years after the procedure, however, he reported that there was no change in his bladder or bowel dysfunction as compared with his condition prior to the procedure. Frequent, systematic multidisciplinary evaluations produced conflicting data. Electrophysiological and histological evaluation of the previously performed anastomosis during surgical reexploration 3 years after the Xiao procedure revealed that the anastomosis was in anatomical continuity but neuroma formation had prevented reinnervation. Nerve action potentials were not demonstrable across the anastomosis, and stimulation of the nerve above and below the anastomosis created no bladder or perineal contractions. This is the first clinical report on the outcome of the Xiao procedure in a child with spinal cord injury outside of China. It is impossible to draw broad conclusions about the efficacy of the procedure based on a single patient with no demonstrable benefit. However, future studies should carefully interpret transient improvements in bladder function, urodynamic findings, and the patient's ability to void in response to scratching after the Xiao procedure. The authors' experience with the featured patient, in whom reinnervation could not be demonstrated, suggests that such changes could be related to factors other than the establishment of a skin-CNS-bladder reflex as a result of a somatic-to-autonomic anastomosis.

Decompressive craniectomy with massive intractable intraoperative cerebral edema: utilization of silicone sheet for temporary scalp closure.

The authors present a case of extreme brain herniation encountered during decompressive craniectomy in a 21-month-old boy who suffered a trauma event that necessitated temporary scalp closure in which a sterile silicone sheet was placed. Although the clinical situation is usually expected to lead to brain death or severe disability, the patient's 3-year follow-up examination revealed a highly functional child with a good quality of life. The authors discuss the feasibility and advantages of temporary scalp expansion as a treatment option when extreme brain herniation is encountered during craniotomy.