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.

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).

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Referred shoulder pain from ventriculoperitoneal shunts. Report of three cases.

The authors report on three pediatric patients with ventriculoperitoneal (VP) shunts who presented with chronic right shoulder pain. Imaging revealed that the distal peritoneal catheter was positioned between the right hemidiaphragm and liver. Following surgical repositioning of the distal tubing, all patients experienced resolution of their shoulder pain, which has not recurred. Although seemingly rare, referred shoulder pain from a VP shunt should be added to the list of complications seen with this method of cerebrospinal fluid diversion. The clinician who cares for patients with VP shunts may wish to evaluate cases of shoulder pain without obvious neural or musculoskeletal cause by performing imaging of the distal shunt tubing.

Supraplacode spinal cord transection in paraplegic patients with myelodysplasia and repetitive symptomatic tethered spinal cord.

OBJECT: The authors describe the technique of transecting the spinal cord in children born with myelomeningocele who have undergone multiple detherings and are functionally paraplegic. METHODS: The authors' technique involves identifying the neural placode and sectioning the normal spinal cord just superior to this site. No postoperative complications have been identified in 14 patients undergoing this procedure over an 11-year period. No patient at last follow up was found to have symptoms referable to a tethered spinal cord. The advantage of this procedure is to excise the normally pia-coated cord, which is unlikely to retether compared with the neural placode, which is often covered with scar tissue and does not have a well-formed pial surface--hence, predisposing it to frequent dorsal adhesions. CONCLUSIONS: The authors believe that this technique is of benefit in a small, carefully selected group of myelodysplastic patients with repetitive tethering of the spinal cord.

Refining the staging evaluation of pineal region germinoma using neuroendoscopy and the presence of preoperative diabetes insipidus.

Treatment strategies for CNS germinoma are currently evolving. Current approaches include reducing the volume and dose of radiation by adding pre-irradiation chemotherapy. Very accurate staging is necessary with such an approach to prevent failures. Eight consecutive patients with pineal germinoma at one institution underwent endoscopic surgery for tumor biopsy, direct visualization of the third ventricular region, and third ventriculostomy for those with hydrocephalus. All patients were treated with 4 cycles of chemotherapy. Conformal field radiation therapy followed, with the dose to the tumor bed dependent on the response to chemotherapy. Patients who had MRI, endoscopic, or cerebrospinal fluid evidence of multicentric or disseminated disease also received craniospinal radiation. Six patients had diabetes insipidus (DI) at presentation. All 6 had tumor studding the floor of the third ventricle on endoscopic visualization, while only 4 of those patients had MRI evidence of disease in that region. All patients have completed therapy and are alive, with no evidence of disease at median follow-up of 31.5 months from diagnosis. Direct endoscopic visualization of the third ventricular region may be more sensitive than MRI for evaluating the presence of suprasellar disease and appears to add important information. This parameter should be added to the staging evaluation when feasible. In this series, the presence of DI was 100% predictive of suprasellar disease, even when the MRI was negative for involvement of that region. Patients should be evaluated for DI as part of the initial staging, and if it is present, the patients should be treated for suprasellar disease regardless of MRI findings.

Sports and pediatric cerebrospinal fluid shunts: who can play?

OBJECTIVE: The purpose of this study was to collect and review available data on the incidence of sport-related complications in children with cerebrospinal fluid (CSF) shunts. METHODS: After review of medical and legal literature revealed no reports on sports and shunts, an Internet-based survey was conducted of pediatric neurosurgeons. Observed complications, recommendations for sport participation, and shunt volume data were collected. RESULTS: Ninety-two providers (55% sample) responded. Seventy-seven percent had never observed a sport-related shunt complication in their practice. The remaining 23% witnessed an estimated total of 25 to 30 complications. Broken shunt catheters and shunt dysfunction were the most common complications observed. Only one acute intracranial hematoma was reported in the entire series. The incidence of sport-related CSF complications in children seems to be significantly less than 1%. Ninety percent of pediatric neurosurgeons do not restrict their patients' participation in noncontact sports. For contact sports, approximately one-third of neurosurgeons preclude all participation, whereas another third restrict specific sports. The final third of neurosurgeons surveyed do not restrict participation in contact sports for children with CSF shunts. CONCLUSION: Sport-related complications in children with CSF shunts are very uncommon.

Long-term control of hydrocephalus via endoscopic third ventriculostomy in children with tectal plate gliomas.

OBJECTIVE: We report the control of hydrocephalus in children with presumed tectal plate gliomas by the use of endoscopic third ventriculostomy (ETV). METHODS: The hospital records, office charts, and imaging studies from children who underwent ETV at the Children's Hospital of Alabama were reviewed. Thirteen children with the diagnosis of tectal plate glioma and hydrocephalus were identified. ETV was the primary therapy instituted for all but one of these children. Successful treatment outcome was defined as shunt freedom, improvement in symptoms, and reduced ventricular size. RESULTS: Thirteen children underwent a total of 15 ETVs, and all children were shunt-free at their most recent follow-up examinations. One child underwent successful secondary ETV, one child underwent shunt removal concomitant with the initial ETV, and one child underwent shunt removal concomitant with secondary ETV. Symptoms and signs resolved in all patients. All postoperative cranial imaging studies revealed normal or reduced ventricular size as compared with preoperative cranial imaging scans. The median follow-up period was 31 months. CONCLUSION: In our surgical experience, ETV has been uniformly successful in the management of hydrocephalus caused by tectal plate gliomas in children. ETV should be considered the treatment of choice for hydrocephalus in pediatric patients with tectal plate gliomas.

Acquired Chiari I malformation following baclofen pump placement in a child. Case report.

The authors present a case of a child suffering from shunt-treated hydrocephalus and spastic quadriplegia who underwent surgery for placement of a baclofen pump. Magnetic resonance (MR) imaging performed prior to pump placement demonstrated no hindbrain herniation. Afterward, however, the patient exhibited symptoms of brainstem compression, and MR imaging revealed a significant Chiari I malformation along with a fully functioning ventriculoperitoneal shunt. Posterior fossa decompression was performed, and the patient's symptoms abated. The authors believe this to be the first report of an acquired Chiari I malformation in a patient with a baclofen pump. Clinicians should consider Chiari I malformation as a rare but severe complication of baclofen pump placement.