Indications for cerebral revascularization for moyamoya syndrome in pediatric sickle cell disease determined by Delphi methodology.

OBJECTIVE: Cerebral revascularization surgery (CRS) has been used to prevent stroke in children with sickle cell disease (SCD) and cerebral vasculopathy (e.g., moyamoya syndrome). While results suggest that it may be an effective treatment, surgical indications have not been well defined. This study sought to determine indications for offering revascularization surgery in centers with established sickle cell programs in the US. METHODS: Three sequential surveys utilizing the Delphi methodology were administered to neurosurgeons participating in the Stroke in Sickle Cell Revascularization Surgery study. Respondents were presented with clinical scenarios of patients with SCD and varying degrees of ischemic presentation and vasculopathy, and the group's agreement to offer surgical revascularization was measured. Consensus was defined as ≥ 75% similar responses. RESULTS: The response rate to all 3 surveys was 100%. Seventeen neurosurgeons from 16 different centers participated. The presence of moyamoya collaterals (MMCs) and arterial stenosis matching an ischemic distribution yielded the strongest recommendations to offer surgery. There was consensus to offer revascularization in the presence of MMCs and at least 50% arterial stenosis matching an ischemic distribution. In contrast, there was no consensus to offer revascularization with 50%-70% stenosis not matching an ischemic presentation in the absence of MMCs. The presence of the ivy sign in the distribution of the stenotic artery also contributed to the consensus to offer surgery in certain scenarios. CONCLUSIONS: There were several clinical scenarios that attained consensus to offer surgery; the strongest was moderate to severe arterial stenosis that matched the distribution of ischemic presentation in the presence of MMCs. Radiological findings of decreased cerebral flow or perfusion also facilitated attaining consensus to offer surgery. The findings of this study reflect expert opinion about questions that deserve prospective clinical research. Determination of indications for CRS can guide clinical practice and aid the design of prospective studies.

Cerebral revascularization surgery reduces cerebrovascular events in children with sickle cell disease and moyamoya syndrome: Results of the stroke in sickle cell revascularization surgery retrospective study.

BACKGROUND: Recent studies suggest that cerebral revascularization surgery may be a safe and effective therapy to reduce stroke risk in patients with sickle cell disease and moyamoya syndrome (SCD-MMS). METHODS: We performed a multicenter, retrospective study of children with SCD-MMS treated with conservative management alone (conservative group)-chronic blood transfusion and/or hydroxyurea-versus conservative management plus surgical revascularization (surgery group). We monitored cerebrovascular event (CVE) rates-a composite of strokes and transient ischemic attacks. Multivariable logistic regression was used to compare CVE occurrence and multivariable Poisson regression was used to compare incidence rates between groups. Covariates in multivariable models included age at treatment start, age at moyamoya diagnosis, antiplatelet use, CVE history, and the risk period length. RESULTS: We identified 141 patients with SCD-MMS, 78 (55.3%) in the surgery group and 63 (44.7%) in the conservative group. Compared with the conservative group, preoperatively the surgery group had a younger age at moyamoya diagnosis, worse baseline modified Rankin scale scores, and increased prevalence of CVEs. Despite more severe pretreatment disease, the surgery group had reduced odds of new CVEs after surgery (odds ratio = 0.27, 95% confidence interval [CI] = 0.08-0.94, p = .040). Furthermore, comparing surgery group patients during presurgical versus postsurgical periods, CVEs odds were significantly reduced after surgery (odds ratio = 0.22, 95% CI = 0.08-0.58, p = .002). CONCLUSIONS: When added to conservative management, cerebral revascularization surgery appears to reduce the risk of CVEs in patients with SCD-MMS. A prospective study will be needed to validate these findings.

Significant brainstem dysfunction in neonates with myelomeningoceles: a comparison of prenatal versus postnatal closure.

OBJECTIVE: The purpose of this study was to compare the incidence of significant brainstem dysfunction (SBD) in neonates with myelomeningocele who have been treated with prenatal versus postnatal closure at a single institution. METHODS: The records and imaging of all children undergoing either prenatal (n = 27) or postnatal (n = 60) closure of myelomeningocele at the authors' institution from December 2014 through May 2021 were reviewed. SBD, fetal ventricular size, gestational age at fetal imaging and delivery, postnatal ventricular size, need for and type of hydrocephalus treatment, spinal neurological level at birth, anatomical Chiari severity, death, and prenatal or postnatal repair were factors recorded. SBD was defined by need for airway surgery or gastrostomy tube, or endotracheal intubation because of apnea, aspiration, or airway control problems. Comparisons between prenatal and postnatal cohorts and between the cohorts with and without SBD were performed. RESULTS: SBD occurred in 25% and 0% of neonates who underwent postnatal and prenatal closure, respectively. There were no differences in fetal ventricular size or spinal neurological level between the prenatal and postnatal cohorts or between those with or without SBD. Anatomical severity of the Chiari malformation after birth was worse in the postnatal cohort. Hydrocephalus treatment was required in 70% and 33% of infants who underwent postnatal and prenatal closure, respectively. All three deaths were in the postnatal group from SBD. CONCLUSIONS: Prenatal closure of myelomeningocele is associated with a significant reduction in SBD.

Ventricular size measurement methods in fetuses considered for prenatal closure of myelomeningocele.

OBJECTIVE: Prenatal closure of myelomeningocele is associated with a reduced rate of hydrocephalus treatment. This need for hydrocephalus treatment is positively correlated with fetal ventricular width. When ventricular width is 15 mm or greater, the benefits of prenatal closure, as a method to decrease hydrocephalus treatment, are reduced. Thus, fetal ventricular size is an important factor when counseling families who are considering intrauterine surgery with mitigation of hydrocephalus as the primary goal. This study sought to determine whether imaging modality (ultrasound [US] vs MRI) and interobserver variability were factors in any ventricular size disparity seen on imaging studies. METHODS: The imaging studies of 15 consecutive fetuses who underwent prenatal myelomeningocele repair at Children's Mercy Fetal Health Center, Kansas City, Missouri, were reviewed. All fetuses were imaged with US and fetal MRI; on average (range), procedures were performed 3.8 (0-20) days apart. Three comparisons were performed to analyze interobserver and intermodality variability in ventricular width measurements: 1) retrospective comparison of dictated ventricular widths measured with MRI and US by pediatric radiologists (PRs) and maternal-fetal medicine specialists (MFMs), respectively; 2) blinded measurements obtained with US by PRs versus initial US-based measurements by MFMs, and blinded measurements obtained with MRI by PRs versus initial MRI-based measurements by PRs; and 3) blinded measurements obtained with MRI by PRs versus those obtained with US. RESULTS: Retrospective comparison showed that measurements with MRI by PRs were on average 2.06 mm (95% CI 1.43-2.69, p < 0.001) larger than measurements with US by MFMs. Blinded measurements with US by PRs were on average larger than dictated measurements obtained with US by MFMs, but by only 0.6 mm (95% CI 0.31-0.84, p < 0.001). When PRs measured ventricular size in a blinded fashion with both US and MRI, the mean width determined with MRI was significantly larger by 2.0 mm (95% CI 1.26-2.67, p < 0.0001). CONCLUSIONS: The ventricular width of these fetuses was larger when measured with MRI than US by an amount that could impact recommendations for fetal surgery. Every center involved in counseling families about the risks and benefits of fetal intervention for spina bifida needs to be aware of these possible imaging-based disparities.

Treatment of pediatric unstable os odontoideum with adjacent degenerative cyst: case presentation and literature review.

Degenerative cysts associated with an unstable os odontoideum in pediatric patients are uncommon lesions. Reported treatments of such lesions have varied and yielded mixed results with the optimal surgical strategy remaining unclear. The authors report the clinical and surgical outcome of a 13-year-old patient presenting with degenerative cyst adjacent to an abnormal os odontoideum motion segment. The patient was asymptomatic from this lesion which was an incidental finding while undergoing workup for atypical headaches. Clinical and radiologic findings, operative details, and postoperative outcome are described. The patient was successfully treated with posterior cervical fusion without direct cyst decompression. Complete resolution of the cyst was demonstrated on magnetic resonance imaging at 6 months. Computed tomography 8 months postoperatively showed solid bony fusion and normal alignment. Regarding treatment goals in pediatric patients with os odontoideum degenerative cysts, the current case and literature review supports posterior instrumented fusion without direct surgical cyst resection.

A rare case of a pediatric white epidermoid cyst.

White epidermoid cysts are a rare entity that present with atypical signal characteristics on MRI in comparison to typical epidermoid cysts. This can mislead a practitioner when trying to formulate a differential diagnosis based on imaging. We present the case of 15-year-old girl who presented with a pontine-medullar junction mass. This mass did not follow typical signal characteristics for known lesions. The mass was ultimately found to be an epidermoid cyst based on pathology findings. This lesion has rarely been reported in pediatric patients (J Pediatr Neurosci 9(1):52-54, 2014; Singap Med J 53(8):179-181, 2012). Here we present a rare case of an intracranial white epidermoid in a pediatric patient (Neurol India 62(5):577-579, 2014; Singap Med J 53(8):179-181, 2012; Am J Neuroradiol 19:1111-1112, 1998).

Intracranial subdural empyemas and epidural abscesses in children

OBJECTIVE: The authors conducted a retrospective analysis of a consecutive series of children with intracranial subdural empyemas (SEs) and epidural abscesses (EAs) to highlight the important clinical difference between these two entities. They describe the delays and pitfalls in achieving accurate diagnoses and make treatment recommendations based on clinical and imaging findings. METHODS: They reviewed their experience with children who had presented with intracranial SE and/or EA in the period from January 2013 to May 2018. They recorded presenting complaint, date of presentation, age, neurological examination findings, time from presentation to diagnosis, any errors in initial image interpretation, timing from diagnosis to surgical intervention, type of surgical intervention, neurological outcome, and microbiology data. They aimed to assess possible causes of any delay in diagnosis or surgical intervention. RESULTS: Sixteen children with SE and/or EA had undergone evaluation by the authors' neurosurgical service since 2013. Children with SE (n = 14) presented with unmistakable evidence of CNS involvement with only one exception. Children with EA alone (n = 2) had no evidence of CNS dysfunction. All children older than 1 year of age had sinusitis. The time from initial presentation to a physician to diagnosis ranged from 0 to 21 days with a mean and median of 4.5 and 6 days, respectively. The time from diagnosis to neurosurgical intervention ranged from 0 to 14 days with a mean and median of 3 and 1 day, respectively. Delay in treatment was due to misinterpretation of images, a failure to perform timely imaging, progression on imaging as an indication for surgical intervention, or the managing clinician's preference. Among the 14 cases with SE, initial imaging studies in 6 were not interpreted as showing SE. Four SE collections were dictated as epidural even on MRI. The only fatality was associated with no surgical intervention. Endoscopic sinus surgery was not associated with reducing the need for repeat craniotomy. CONCLUSIONS: Regardless of the initial imaging interpretation, any child presenting with focal neurological deficit or seizures and sinusitis should be assumed to have an SE or meningitis, and a careful review of high-resolution imaging, ideally MRI with contrast, should be performed. If an extraaxial collection is identified, surgical drainage should be performed expeditiously. Neurosurgical involvement and evaluation are imperative to achieve timely diagnoses and to guide management in these critically ill children. ABBREVIATIONS: EA = epidural abscess; SE = subdural empyema.

Reverse Distraction for Treatment of Hydrocephalic Macrocephaly in Late Childhood.

Macrocephaly diminishes quality of life for children whose head size inhibits independent mobility and appropriate interaction with caregivers. Cranial reduction is a method of addressing these issues, historically with a high morbidity due most commonly to bleeding and shunt complications. The authors present a 9-year-old girl with holoprosencephaly and severe macrocephaly from progressive hydrocephalus who underwent cranial reduction via reverse distraction osteogenesis, a method to slowly reduce the skull volume. The patient underwent circumferential occipital temporoparietal frontal craniotomy with placement of 4 cranial distractors, followed approximately 1 month later by removal of the distractors and cranioplasty with resorbable fixation devices. The patient demonstrated significant postoperative improvement in head control and interaction in school activities. This is the oldest patient with macrocephaly treated with reverse distraction in the literature to date. The slow contraction of the cranial vault with limited bony surgery at the time of initial reduction provides an additional safety margin, and should be considered in older children presenting with profound macrocephaly.

Hemiballismus as a complication of an intratumoral chemotherapy catheter.

We report an unusual case of delayed bilateral, right greater than left hemiballismus in a 15-year-old female patient with a history of a craniopharyngioma 2years following the insertion of a right intratumoral chemotherapy catheter. Following cyst decompression, the catheter was found to have changed position, traversing the basal ganglia structures, namely the right subthalamic nucleus. Her movement disorder near-completely resolved immediately following removal of the catheter. A review of the current literature and proposed pathophysiological mechanisms are discussed.

Surgical management of intracranial capillary hemangiomas in children: report of 2 cases.

Two cases of intracranial capillary hemangiomas (ICHs) occurring in children are presented to highlight the surgical challenges encountered with these extremely rare lesions. The author describes their clinical presentation, preoperative imaging features, intraoperative findings, and operative management. The pertinent literature is reviewed. Recommendations for preoperative planning and intraoperative management are made based on the author's experience and the literature for when ICH is considered in the differential diagnosis and encountered intraoperatively.

Long-term change in ventricular size following endoscopic third ventriculostomy for hydrocephalus due to tectal plate gliomas.

OBJECT: Endoscopic third ventriculostomy (ETV) is an alternative to shunt placement in children with hydrocephalus due to tectal plate gliomas (TPGs). However, controversy remains regarding the amount of ventricular size reduction that should be expected after ETV. This study investigates ventricular size change after ETV for TPGs. METHODS: Twenty-two children were identified from a 15-year retrospective database of neuroendoscopic procedures performed at the authors' institution, Children's Hospital of Alabama, in patients with a minimum of 1 year of follow-up. Clinical outcomes, including the need for further CSF diversion and symptom resolution, were recorded. The frontal and occipital horn ratio (FOR) was measured on pre- and postoperative, 1-year, and last follow-up imaging studies. RESULTS: In 17 (77%) of 22 children no additional procedure for CSF diversion was required. Of those in whom CSF diversion failed, 4 underwent successful repeat ETV and 1 required shunt replacement. Therefore, in 21 (96%) of 22 patients, CSF diversion was accomplished with ETV. Preoperative and postoperative imaging was available for 18 (82%) of 22 patients. The FOR decreased in 89% of children who underwent ETV. The FOR progressively decreased 1.7%, 11.2%, and 12.7% on the initial postoperative, 1-year, and last follow-up images, respectively. The mean radiological follow-up duration for 18 patients was 5.4 years. When ETV failed, the FOR increased at the time of failure in all patients. Failure occurred 1.6 years after initial ETV on average. The mean clinical follow-up period for all 22 patients was 5.3 years. In all cases clinical improvement was demonstrated at the last follow-up. CONCLUSIONS: Endoscopic third ventriculostomy successfully treated hydrocephalus in the extended follow-up period of patients with TPGs. The most significant reduction in ventricular size was observed at the the 1-year followup, with only modest reduction thereafter.

Cervical osteochondroma with postoperative recurrence: case report and review of the literature.

INTRODUCTION: Osteochondromas of the cervical spine are rare. We report an 8-year-old girl presenting with neck pain and a known familial predisposition for osteochondromas. CASE REPORT: Imaging revealed a presumed osteochondroma of the cervical spine located at C3. A cervical hemilaminectomy of the lesion was performed. Histopathology confirmed the lesion as an osteochondroma. Six months later, the child was found to have a recurrence of the previously resected lesion. CONCLUSION: The child was reoperated and the lesion removed along with the entire remaining lamina of C3. At 6-year follow-up, there has been no recurrence of the child's cervical lesion. We believe this to be the third reported case of recurrence of a cervical osteochondroma. Surgeons dealing with such lesions should be mindful of this complication.

Medial pectoral nerve to musculocutaneous nerve neurotization for the treatment of persistent birth-related brachial plexus palsy: an 11-year institutional experience.

OBJECT: Medial pectoral nerve (MPN) to musculocutaneous nerve (MCN) neurotization for recovery of elbow flexion by biceps reinnervation is a valid option following traumatic injury to the upper brachial plexus. A major criticism of the application of this technique in infants is the smaller size of the MPN and mismatch of viable axons. We describe our institutional experience utilizing this procedure and critically examine functional outcomes. METHODS: Office charts and hospital records of children from over an 11-year period beginning January 1997 were reviewed. Of the 53 children of various ages undergoing brachial plexus exploration for traumatic injury of any nature, 20 underwent MPN to MCN neurotization as a part of an overall procedure in the first year of life to treat birth-related brachial plexus palsy and had at least 9 months' follow-up. Medial pectoral nerve to MCN neurotization was chosen if the results of clinical examination and intraoperative electrophysiological evidence were consistent with medial cord function. Functional recovery was defined as the ability of the child to bring their hand to their mouth. RESULTS: Sixteen patients (80%) gained functional recovery. The median age at surgery was 7 months. Median time to first clinic visit documenting recovery was 11.5 months and median overall follow up was 21.5 months. Preoperative hand function was a useful predictor of recovery of elbow flexion. CONCLUSIONS: Medial pectoral nerve to MCN neurotization is a valid surgical option for the reinnervation of the biceps muscle for birth-related brachial plexus palsy when the hand is functional preoperatively. Useful elbow flexion can be expected in the majority of these children.

Cumulative diagnostic radiation exposure in children with ventriculoperitoneal shunts: a review.

INTRODUCTION: Children may be more vulnerable to diagnostic radiation exposure because of the increased dose-volume ratio and the increased lifetime risk per unit dose of radiation from early exposure. Moreover, recent radiological literature suggests that exposure to ionizing radiation from imaging studies may play a role in the later development of malignancies. MATERIALS AND METHODS: We review the literature and present two illustrative clinical examples of children (each child developed head and neck malignancies during their late teen years) with hydrocephalus requiring multiple cerebrospinal fluid (CSF) shunt revisions and diagnostic computerized tomography (CT) scans throughout their life. DISCUSSION: The literature reviewed suggests that children are more prone to diagnostic radiation exposure. Although it is not possible to prove that the multiple diagnostic studies result in malignancies, our review of the literature and illustrative cases describing malignancy risk and radiation exposure should give clinicians pause when considering requesting multiple diagnostic CT studies in children during the evaluation of possible CSF shunt dysfunction. Alternative tests such as "shunt MRI" protocols should be considered for patients and used whenever possible to minimize exposure to ionizing radiation.

Urgent surgical intervention in pediatric patients with Chiari malformation type I. Report of two cases.

Patients with Chiari malformation Type I (CM-I) most commonly present with chronic symptoms. A search of the current medical literature revealed scant information regarding acute presentations of CM-I in either pediatric or adult patients. The authors report on two children who presented with rapidly worsening neurological symptoms attributable to a previously undiagnosed CM-I. One patient became profoundly hypopneic with dysphagia and right hemiparesis over a less than 48-hour period. The second patient presented with a few days of worsening right hemiparesis, gait disturbance, and anisocoria. In addition to a CM-I, magnetic resonance imaging in the second patient revealed a holocord syrinx. Following urgent posterior fossa decompression, both patients rapidly improved in the 24 hours immediately following surgery and continued to improve in the subsequent weeks. Few reports detail acute symptoms due to CM-I and those that do exist almost exclusively involve adult patients. Although seemingly rare, the clinician should consider CM-I in the differential diagnosis in pediatric patients presenting with acute brainstem or long tract signs.

Chiari I malformation and idiopathic growth hormone deficiency in siblings: report of three cases.

INTRODUCTION: The authors report a case of three brothers. CASE DESCRIPTIONS: Two of these siblings presented with congenital growth hormone deficiency (GHD) and Chiari I malformation (CIM). The third younger brother has been found not to have GHD or the CIM. DISCUSSION: Sparse cases of these two clinical occurrences have been reported. Further, the posterior cranial fossa (PF) has been determined to be altered in patients with CIM and GHD. CONCLUSION: Our current case reports strengthen the association between these two pathological entities and, to our knowledge, is the first description of both defects in siblings.

Spinal accessory nerve meningioma in a paediatric patient: case report.

Accessory nerve meningiomas are exceedingly rare. We present a case of a nine-year-old patient with neurofibromatosis type 2 who had radiologic evidence of spinal cord compression from an upper cervical/foramen magnum lesion. He was asymptomatic from this lesion, but it progressed in size. The tumor was resected and histologic investigation revealed frequent tight whorls and psammoma bodies consistent with meningioma. To the authors' knowledge, this is the first reported spinal accessory nerve meningioma in a pediatric patient.

Vagus nerve stimulation in children less than 5 years old.

INTRODUCTION: Vagus nerve stimulation (VNS) has been used in both adults and older children with varying success. MATERIALS AND METHODS: We retrospectively reviewed our experience with VNS in very young children (below 5 years old). The mean age at stimulator implantation was 20.5 months. Two patients were below 2 years old at implantation and two patients were below 1 year old at their initial surgery. The average follow up time for this group was 22 months. RESULTS: Of the six patients (three males and three females) with long-term follow up, 83% had a significant decrease in the frequency of their seizure. Of these, two are seizure-free (33%), three are improved (50%), and one (17%) has had no change in seizure status at their most recent clinical examination. Age at implantation of the vagus nerve stimulator did not seem to correlate with patient success. In this group, atonic seizures were found to best respond to VNS with cessation of this type of seizure in two patients. No patients were made worse by the procedure and no morbidity was observed related to VNS. CONCLUSIONS: Based on our small patient cohort, it appears that VNS in very young children with life-threatening epilepsy can be efficacious. Larger groups and other institutional experiences are now needed to verify our findings.