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.

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.

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.

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.

Cessation of chronic hypertension after posterior fossa decompression in a child with Chiari I malformation. Case report.

The authors report on the case of a patient evaluated for Valsalva maneuver-induced headache, dizziness, and ataxia. Neuroimaging revealed a Chiari I malformation without syringomyelia. A history of idiopathic hypertension was noted. After posterior fossa decompression, pathologically elevated blood pressure was absent, and at 24-month follow-up evaluation the patient remained normotensive. Although seemingly rare, this case illustrates that some patients with tonsillar ectopia may exhibit elevated blood pressure. Clinicians should consider hindbrain herniation a rare cause in idiopathic hypertension.

Inclination of the odontoid process in the pediatric Chiari I malformation.

OBJECT: The quantitative analysis of odontoid process angulation has had scant attention in the Chiari I malformation population. In this study the authors sought to elucidate the correlation between posterior angulation of the odontoid process and patients with Chiari I malformation. METHODS: Magnetic resonance images of the craniocervical junction obtained in 100 children with Chiari I malformation and in 50 children with normal intracranial anatomy (controls) were analyzed. Specific attention was focused on measuring the degree of angulation of the odontoid process and assigning a score to the various degrees. Postoperative outcome following posterior cranial fossa decompression was then correlated to grades of angulation. Other measurements included midsagittal lengths of the foramen magnum and basiocciput, the authors' institutions' previously documented pB-C2 line (a line drawn perpendicular to one drawn between the basion and the posterior aspect of the C-2 body), level of the obex from a midpoint of the McRae line, and the extent of tonsillar herniation. Higher grades of odontoid angulation (retroflexion) were found to be more frequently associated with syringomyelia and particularly holocord syringes. Higher grades of angulation were more common in female patients and were often found to have obices that were caudally displaced greater than three standard deviations below normal. CONCLUSIONS: These results not only confirm prior reports of an increased incidence of a retroflexed odontoid process in Chiari I malformation but quantitatively define grades of inclination. Grades of angulation were not found to correlate with postoperative outcome. It is the authors' hopes that these data add to our current limited understanding of the mechanisms involved in hindbrain herniation.

Complications of chronic vagus nerve stimulation for epilepsy in children.

OBJECT: The aim of this study was to define better the incidence of surgical complications and untoward side effects of chronic vagus nerve stimulation (VNS) in a population of children with medically refractory epilepsy. METHODS: The authors retrospectively reviewed the cases of 74 consecutive patients (41 male and 33 female) 18 years of age or younger (mean age 8.8 years, range 11 months-18 years) who had undergone implantation of a vagal stimulator between 1998 and 2001 with a minimum follow up of 1 year (mean 2.2 years). Of the 74 patients treated, seven (9.4%) had a complication ultimately resulting in removal of the stimulator. The rate of deep infections necessitating device removal was 3.5% (three of 74 patients who had undergone 85 implantation and/or revision procedures). An additional three superficial infections occurred in patients in whom the stimulators were not removed: one was treated with superficial operative debridement and antibiotic agents and the other two with oral antibiotics only. Another four stimulators (5.4%) were removed because of the absence of clinical benefit and device intolerance. Two devices were revised because of lead fracture (2.7%). Among the cohort, 11 battery changes have been performed thus far, although none less than 33 months after initial implantation. Several patients experienced stimulation-induced symptoms (hoarseness, cough, drooling, outbursts of laughter, shoulder abduction, dysphagia, or urinary retention) that did not require device removal. Ipsilateral vocal cord paralysis was identified in one patient. One patient died of aspiration pneumonia more than 30 days after device implantation. CONCLUSIONS: Vagus nerve stimulation remains a viable option for improving seizure control in difficult to treat pediatric patients with epilepsy. Surgical complications such as hardware failure (2.7%) or deep infection (3.5%) occurred, resulting in device removal or revision. Occasional stimulation-induced symptoms such as hoarseness, dysphagia, or torticollis may be expected (5.4%).

Superficial surgical landmarks for the frontal sinus.

OBJECT: There is a lack of reports in the literature that contain descriptions of superficial anatomical landmarks for the identification of the internally located frontal sinus. Neurosurgeons must often enter the cranium through the frontal bone and knowledge of the frontal sinus is essential to minimize complications. METHODS: Seventy adult cadaveric frontal sinuses were evaluated. Measurements included both the lateral and superior extent of the frontal sinus in reference to a midpupillary line, and the superior extent of the frontal sinus from the nasion. Frontal sinuses were found bilaterally in all specimens. The mean height of the frontal sinus superior to the nasion was 2.8 cm. In 71.4% and 74.3% of specimens the lateral extent of the frontal sinus was found to be medial to the left and right midpupillary line, respectively. Distances superior to a plane drawn through the supraorbital ridges at a midpupillary line included a mean of 2.5 mm for the left side and 1.8 mm for the right side. CONCLUSIONS: Of 70 sinuses, none extended more than 5 mm lateral to a midpupillary line. At this same midpupillary line and at a plane drawn through the supraorbital ridges, the frontal sinus was never higher than 12 mm. Finally, in the midline the frontal sinus never reached more than 4 cm above the nasion. These measurements will assist surgeons who must manipulate the frontal bone.

Atlantoaxial interlaminar distances in cervical flexion in children.

OBJECT: To the best of the authors' knowledge, no quantitative analysis of the atlantoaxial interlaminar distance in flexion (ILD) in children exists in the medical literature. In this study they sought to determine the age-matched relationship between the posterior elements of the atlas and axis in children in cervical spine flexion, to be used as an adjunct to the atlantodental interval in common clinical use. METHODS: Lateral radiographs of the cervical spine in full flexion were analyzed in 74 children. The atlantoaxial ILD was defined as the distance between a midpoint of the anterior cortices of the atlantal and axial posterior arches. The mean ILD for the entire group was 19 mm (range 8-30 mm). No significant difference was seen between male and female patients (p = 0.084). When stratified by age, the mean ILD was 12.3 +/- 3 mm (15 cases) in children age 3 years or younger and 20.5 +/- 4.7 mm (59 cases) in children age older than 3 years. Further stratification of the groups yielded a mean ILD of 10.4 +/- 1.4 [eight cases]) in children age 1 to 2 years, and 14.4 +/- 4.7 mm (seven cases) in children age 3 years. In children older than 3 years of age the mean ILD was consistently approximately 20 +/- 5 mm regardless of age. CONCLUSIONS: Rapid, safe, and accurate diagnosis of the cervical spine is essential in critical care. Knowledge of the distance between the posterior elements of the atlas and axis in flexion should enhance the clinicians' (those who clear cervical spines) ability to diagnose accurately atlantoaxial instability on lateral radiographs obtained in flexion.

Neuroendoscopic findings in patients with intracranial germinomas correlating with diabetes insipidus.

OBJECT: Intracranial germinomas commonly occur in the pineal region, the floor of the third ventricle (hypothalamus), or both, and they are often associated with diabetes insipidus (DI). The authors conducted a study to correlate preoperative DI with the endoscopic and magnetic resonance (MR) imaging evidence of tumor on the third ventricle floor. METHODS: The authors reviewed hospital records, office charts, and MR imaging studies obtained in patients in whom a biopsy sampling procedure was performed with or without endoscopic third ventriculostomy (ETV) at Children's Hospital, Birmingham, Alabama between May 1998 and July 2002. Ten patients with the pathological diagnosis of pure germinoma were identified. Preoperative MR imaging findings and presenting symptoms were correlated with intraoperative neuroendoscopic findings. Seven patients presented with symptomatic hydrocephalus and underwent concomitant ETV. Six patients presented with DI and MR imaging evidence of involvement of the third ventricle floor. Two patients presented with DI and no initial MR imaging evidence of neoplastic involvement of the third ventricle floor; in both there was endoscopic evidence of neoplastic involvement of the floor of the third ventricle. In two children without DI, neither MR imaging nor endoscopic evidence of involvement of the third ventricle floor was observed. CONCLUSIONS: In the authors' experience with intracranial germinoma, endoscopic tumor biopsy sampling, and ETV provide an effective, safe, and minimally invasive means of obtaining diagnostic tissue and treating any concomitant hydrocephalus. The authors found that preoperative DI is an absolute predictor of neoplastic involvement of the hypothalamus regardless of MR imaging findings. Therefore, in the setting of DI and intracranial germinoma without neuroimaging evidence of hypothalamic involvement, germinomatous involvement of the hypothalamus should be assumed present, if not confirmed endoscopically at the time of biopsy sampling or third ventriculostomy, when devising adjuvant treatment plans for such patients.

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.