A systematic review of the power of standardization in pediatric neurosurgery.

In the current neurosurgical field, there is a constant emphasis on providing the best care with the most value. Such work requires the constant optimization of not only surgical but also perioperative services. Recent work has demonstrated the power of standardized techniques in limiting complication while promoting optimal outcomes. In this review article, protocols addressing operative and perioperative care for common pediatric neurosurgical procedures are discussed. These articles address how various institutions have optimized procedures through standardization. Our objective is to improve patient outcomes through the optimization of protocols.

Arteriovenous malformation with unique drainage through the emissary vein of the foramen ovale: illustrative case.

BACKGROUND: As part of the laterotrigeminal venous system (LTVS), the emissary vein of the foramen ovale (EVFO) is an underrecognized venous structure communicating between the cavernous sinus and pterygoid plexus. The sphenobasal sinus is an anatomical variation of the sphenoparietal sinus that drains directly into the EVFO. The authors present the case of a ruptured arteriovenous malformation (AVM) with a unique drainage pattern through the sphenobasal sinus and EVFO. OBSERVATIONS: A 9-year-old female initially presented with loss of consciousness and was subsequently found to have a ruptured AVM in the left basal frontal area. She underwent an immediate decompressive hemicraniectomy, with a computed tomography angiogram demonstrating a unique anatomical variation in which the sphenobasal sinus communicated with the EVFO and LTVS. The final venous drainage returned to the pterygoid plexus and external jugular vein. Postoperatively, the patient made a substantial recovery, with generalized right-sided weakness remaining as the sole deficit. LESSONS: The authors present the case of a ruptured AVM with unique venous drainage into the sphenobasal sinus and EVFO, for which the current literature remains limited. As exemplified by this illustrative case, technique modification may be warranted in the setting of this unique anatomical variation to avoid venous sinus injury.

Ventriculoperitoneal shunt placement following endoscopic third ventriculostomy failure in the treatment of pediatric hydrocephalus.

OBJECTIVE: The goal of this study was to evaluate the impact of endoscopic third ventriculostomy (ETV) failure on subsequent risk of ventriculoperitoneal shunt (VPS) placement. METHODS: A retrospective chart review was performed to identify pediatric patients receiving ETV followed by a VPS at Oklahoma Children's Hospital between January 1, 2016, and December 31, 2021. A control group of patients receiving a VPS alone was also gathered. Complication and shunt failure rates were compared between the 2 groups at 12 months postoperatively. RESULTS: A total of 222 patients were included in this study. The VPS placement after ETV failure (VPSEF) group included 21 patients; 53% were male and 47% were female, with a mean age of 2.2 years and standard deviation of 4.3 years. The etiology of hydrocephalus was chiefly intraventricular hemorrhage (43%) and neural tube defects (19%). At 12 months after VPS placement, the complication rate was 24%, predominantly including infection (19%) or CSF leakage (10%). The VPS-only (VPSO) group included 201 patients; 51% were male and 49% were female, with a mean age of 4.2 years and standard deviation of 6.5 years. The etiology of hydrocephalus was chiefly intraventricular hemorrhage (26%) and neural tube defects (30%). At 12 months postoperatively, the complication rate was 10%, predominantly including infection (6%) or catheter-associated hemorrhage (3%). The difference in complication rates between the VPSEF and VPSO groups was not significant at 12 months postoperatively (p = 0.07); however, on subgroup analysis there was a significantly higher rate of CSF leakage at 12 months in the VPSEF group compared to the VPSO group (p = 0.0371). CONCLUSIONS: There was no difference in overall complication rates for the treatment of pediatric hydrocephalus by VPS following failed ETV compared to VPS placement alone, yet prior ETV may predispose patients to a higher rate of CSF leaks within 12 months of VPS placement. Further study is indicated to determine whether a prior ETV procedure predisposes patients to a higher complication rate on VPS placement.

Evaluation of Osteopathic Principles in Cadaveric Specimens Using Radiological Assessment.

BACKGROUND: Osteopathic manipulative treatment (OMT) of the spine requires the physician to first be able to diagnose with palpation of the spinous processes, transverse processes, and facets, test for movement of the anatomy, and evaluate changes in tissue texture at each level. Physicians should then apply these changes to Fryette's Principles to effectively understand the corresponding somatic dysfunction and provide treatment. Continuing education in osteopathic principles and practices is important throughout an osteopathic physician's training. AIM: Diagnosis and treatment require an understanding of the complex neuroanatomy and physiology of patients. We sought to evaluate the diagnostic capabilities of osteopathic physicians. This was done by evaluating the accuracy of diagnosis of somatic dysfunction on a cadaver specimen and verifying via fluoroscopy and blunt dissection. MATERIALS & METHODS: Fresh refrigerated cadavers were palpated for lesions in the thoracic spine by residents and attendings, and diagnoses of somatic dysfunction were made. Anterior-posterior X-rays were taken with a C-arm. These levels were then exposed by blunt dissection, and somatic dysfunctions were visualized and recorded. Comparative analyses were conducted to evaluate the accuracy of diagnosis.  Results: The accuracy of diagnoses was correct in those who had OMT skills reassessed throughout training and continuing medical education. Osteopathic physicians who routinely kept up with their training were better able to make diagnoses of somatic dysfunction. CONCLUSION: Continuing osteopathic medical education with an emphasis on the maintenance of palpatory skills is important. Those physicians with the greatest accuracy of somatic dysfunction diagnosis were physicians who routinely underwent reassessment and continuing medical education of osteopathic skills.

Infrastructural Limitations in Establishing Neurosurgical Specialty Services in Liberia.

Introduction Liberia recently employed the first neurosurgeon in the country's history. In a country with a population of 4.7 million people and staggering rates of cranial and spine trauma, as well as hydrocephalus and neural tube defects, neurosurgery is considered a luxury. Our study documents the experience of a team of neurosurgeons, critical care nurses, scrub technicians, nurses, and biomedical engineers who carried out a series of neurosurgical clinics and complex brain and spine surgeries in Liberia. Specifically, we aim to highlight some of the larger obstacles, beyond staff and equipment, facing the development of a neurosurgical or any other specialty practice in Liberia.  Methods Our institutions, in collaboration with the Korle-Bu Neuroscience Foundation, spent 10 days in Liberia, based in Tappita, and performed 18 surgeries in addition to seeing several hundred clinic patients. This is a retrospective review of the cases performed along with outcomes to investigate obstacles in providing neurosurgical services in the country. Results Before arriving in Liberia, we evaluated, planned, and supplied staff and materials for treating complex neurosurgical patients. Sixteen patients underwent 18 surgeries at a hospital in Tappita, Liberia, in November 2018. Their ages ranged from 1 month to 72 years (average 20 years). Five patients (28%) were female. Ten patients (56%) were under the age of 18. Surgeries included ventriculoperitoneal shunting (VP-shunt), lumbar myelomeningocele repair, encephalocele repair, laminectomy, and a craniotomy for tumor resection. Ten patients (55%) underwent VP-shunting. Two patients (11%) had a craniotomy for tumor resection. Three patients (17%) had laminectomy for lumbar stenosis. Two patients (11%) had repair of lumbar myelomeningocele. Conclusion After an aggressive and in-depth approach to planning, conducting, and supplying complex neurosurgical procedures in Liberia, the greatest limiting factor to successful outcomes lie in real-time is access to health care, which is largely limited by overall infrastructure. Our study documents the experience of a team of neurosurgeons, critical care nurses, scrub technicians, nurses, and biomedical engineers who carried out a series of neurosurgical clinics and complex brain and spine surgeries in Liberia. Specifically, we aim to highlight some of the larger obstacles, beyond staff and equipment, facing the development of a neurosurgical or any other specialty procedural practice in the country of Liberia. Most notably, we focus on infrastructure factors, including power, roads, water, education, and overall health care.

Radiographic Predictors of Shunt Dependency in Intracranial Hemorrhage With Intraventricular Extension.

Background Intracranial hemorrhage (ICH) may be complicated by intraventricular hemorrhage (IVH) and hydrocephalus, which can require the placement of a ventriculoperitoneal shunt (VPS). ICH and IVH risk scores using radiographic and clinical characteristics have been developed but utilization for assessment of future need for VPS placement is limited. Methods This is a single-institution retrospective review for patients with primary ICH with IVH from 2018-2020. Initial CTs and charts were analyzed to determine ICH, IVH, LeRoux and Graeb scores, Evans' index, ICH and IVH volumes, and comorbidities. Outcomes including Glasgow coma scale (GCS), National Institute of Health Stroke Scale (NIHSS), length of stay, and shunt placement were evaluated with bivariate correlations, t-tests, chi-squared tests, and receiver operating characteristic (ROC) curves (p=0.05). Results A total of 130 patients were included of which 102 underwent full treatment beyond hospital day one. VPS placement was significantly associated with longer length of stay (p<0.001), discharge NIHSS (p=0.001), arrival Evans' index (p<0.001), IVH (p=0.033), LeRoux (p=0.049), but not comorbidities, ICH score, or admission GCS. When treated beyond hospital day one, Evans' index (p<0.001), IVH volume (p=0.029), Graeb (p=0.0029), IVH (p=0.004), Slice (p=0.015), and Leroux scores (p=0.006) were associated with shunt placement of which an Evans' index of 0.31 or greater had highest sensitivity and specificity (area under the ROC curve (AUC) 0.81, sensitivity 81%, specificity 0.76). Conclusions The higher the Evans' index, Graeb, IVH, Slice, and LeRoux scores on admission, the higher the risk of shunt dependency in patients undergoing full treatment beyond hospital day one. Admission imaging scores significantly predict the development of shunt dependence and may be considered in treatment.

Osteopathic Manipulative Treatment to Optimize the Glymphatic Environment in Severe Traumatic Brain Injury Measured With Optic Nerve Sheath Diameter, Intracranial Pressure Monitoring, and Neurological Pupil Index.

Background Traumatic brain injury (TBI) has a complex pathophysiology that has historically been poorly understood. New evidence on the pathophysiology, molecular biology, and diagnostic studies involved in TBI have shed new light on optimizing rehabilitation and recovery. The goal of this study was to assess the effect of osteopathic manipulative treatment (OMT) on peripheral and central glial lymphatics in patients with severe TBI, brain edema, and elevated intracranial pressure (ICP) by measuring changes in several parameters regularly used in management. Methodology This was a retrospective study at a level II trauma center that occurred in 2018. The study enrolled patients with TBI, increased ICP, or brain edema who had an external ventricular drain placed. Patients previously underwent a 51-minute treatment with OMT with an established protocol. Patients received 51 minutes of OMT to the head, neck, and peripheral lymphatics. The ICP, cerebrospinal fluid (CSF) drainage, optic nerve sheath diameter (ONSD) measured by ultrasonography, and Neurological Pupil Index (NPi) measured by pupillometer were recorded before, during, and after receiving OMT. Results A total of 11 patients were included in the study, and 21 points of data were collected from the patients meeting inclusion criteria who received OMT. There was a mean decrease in the ONSD of 0.62 mm from 6.24 mm to 5.62 mm (P = 0.0001). The mean increase in NPi was 0.18 (P = 0.01). The mean decrease in ICP was 3.33 mmHg (P= 0.0001). There was a significant decrease in CSF output after treatment (P = 0.0001). Each measurement of ICP, ONSD, and NPi demonstrated a decrease in overall CSF volume and pressure after OMT compared to CSF output and ICP prior to OMT. Conclusions This study demonstrates that OMT may help optimize glial lymphatic clearance of CSF and improve brain edema, interstitial waste product removal, NPi, ICP, CSF volume, and ONSD. A holistic approach including OMT may be considered to enhance management in TBI patients. As TBI is a spectrum of disease, utilizing similar techniques may be considered for all forms of TBI including concussions and other diseases with brain edema. The results of this study can better inform future trials to specifically study the effectiveness of OMT in post-concussive treatment and in those with mild-to-moderate TBI.

Toxic Leukoencephalopathy due to Inhalational Heroin Abuse.

Heroin-induced spongiform leukoencephalopathy (HSLE) is a rare condition that is strongly associated with heroin vapor inhalation which has become a popular method among heroin addicts because it poses a less immediate danger to the user and makes the drug much easier to use. We present a case of a 22-year-old male who presented with dysarthria and cerebellar symptoms starting, after 3 months of heroin inhalation. Diagnosis was confirmed to be HSLE after extensive diagnostic testing. HSLE is a rare complication of which the pathogenesis is poorly understood. Clinical history and characteristic findings on magnetic resonance imaging (diffuse, symmetric T2-hyperintensity, and diffusion restriction in frontal, parietal, occipital lobs, basal ganglia, and superior cerebellum) are diagnostic; however, care should be taken to exclude other etiologies. Treatment is primarily supportive; however, there is anecdotal evidence that coenzyme Q10 may be of benefit. The growing number of victims of the opioid crisis requires that physicians be aware of and counsel patients on the devastating neurological complications that can occur with abuse of these drugs.

Spontaneous Intracranial Hypotension: Case Study and Review of the Literature.

Spontaneous intracranial hypotension (SIH) is a pathology characterized by orthostatic headaches, diffuse pachymeningeal enhancement on magnetic resonance imaging (MRI), and low to normal cerebrospinal fluid (CSF) pressures. We present the case of a 46-year-old male with refractory postural headaches, found to have a diffuse CSF leak throughout the cervicothoracic (C1-T12) spine. His neurological status declined rapidly to a Glasgow Coma Scale (GCS) of eight, necessitating bilateral subdural drain placement. Despite an overall brisk neurologic recovery, the patient remained unable to speak for nearly a week after the return of the remainder of his function. This raised the concern for possible cerebellar mutism. We review the multiple modalities used in this patient's treatment and explore possible explanations for the failure of initial therapy. The placement of bilateral subdural drains was a temporizing measure to treat the patient's neurologic decline, but it was likely the epidural blood patch with prolonged bedrest that hastened the patient's recovery. His speech function also returned with time and repeated therapy.

Pathophysiologic Mechanisms of Concussion, Development of Chronic Traumatic Encephalopathy, and Emerging Diagnostics: A Narrative Review.

Pathophysiological mechanisms and cascades take place after a mild traumatic brain injury (mTBI) that can cause long-term sequelae, including chronic traumatic encephalopathy in patients with multiple concurrent TBIs. As diagnostic imaging has become more advanced, microanatomical changes present after mTBI may now be more readily visible. In this narrative review, the authors discuss emerging diagnostics and findings in mTBI through advanced imaging, electroencephalograms, neurophysiologic processes, Q2 biochemical markers, and clinical tissue tests in an effort to help osteopathic physicians to understand, diagnose, and manage the pathophysiology behind mTBI, which is increasingly prevalent in the United States.

Diagnosis of Rhinocerebral Mucormycosis by Treatment of Cavernous Right Internal Carotid Artery Occlusion With Mechanical Thrombectomy: Special Case Presentation and Literature Review.

Background: Mucormycosis is a rapidly progressive, angioinvasive fungal infection that has a predilection for the paranasal sinuses and adjacent mucosa. Rhinocerebral mucormycosis (RCM) is the most common form and is known to invade the skull base and its associated blood vessels-leading to mycotic aneurysms, ischemic infarcts, and intracerebral hemorrhage. There are documented cases of mechanical thrombectomy in ischemic stroke due to RCM, however, there are no known cases that were diagnosed primarily by histological and pathological analysis of the embolus. We present a case of treatment of large vessel occlusion that led to the diagnosis and treatment of RCM. Case Presentation: A 21 year-old male inmate with history of type 1 diabetes presented with generalized weakness, abdominal pain, right eye blindness, and ophthalmoplegia after an assault in prison. He underwent treatment for diabetic ketoacidosis, but subsequently developed left hemiplegia and was found to have complete occlusion of his right internal carotid artery. He underwent successful mechanical thrombectomy and pathological analysis of the embolus revealed a diagnosis of mucormycosis. He completed a course of amphotericin B, micafungin, and posaconazole. With the aid of acute rehabilitation he achieved a modified Rankin score of 2. Discussion: We review the pathogenesis, diagnosis, and treatment of RCM. A comprehensive multidisciplinary approach is critical in the management of this often-fatal disease. Early diagnosis and treatment are essential in RCM as delaying treatment by more than 6 days significantly increases mortality. Treatment includes surgical debridement and intravenous antifungal therapy (amphotericin B + micafungin or caspofungin) for a minimum of 6-8 weeks.

Efficacy of Trauma Catheter and Mushroom Tip Catheter in Evacuation of Chronic Subdural Hematoma and Complications of Drain Placement.

Objective The aim of this study was to assess the efficacy and complications of trauma catheter versus mushroom tip catheter placement in the evacuation of chronic subdural hematoma via twist drill craniostomy with closed system drainage. Background Chronic subdural hematoma (cSDH) is one of the most frequent neurosurgical pathologies in patients >70 years of age with an estimated incidence of 8.2 per 100,000 people per year. The most common risk factors for cSDH are advanced age, alcohol abuse, seizures, cerebrospinal fluid (CSF) shunts, coagulopathies, blood thinners, and patients at risk for falling. Twist drill craniostomy can be performed at the bedside under local anesthesia, making it an attractive treatment option, especially in poly-morbid patients who are poor surgical candidates. A closed drainage system is placed at the time of surgery to allow continuous drainage and promote postoperative brain expansion. Despite the increasing prevalence, limited literature exists to guide surgical management, particularly in terms of drain management and selection of catheter.  Methods This is a retrospective review of 205 patients from January 2007 to May 2017 at two-level high volume centers for the evaluation and treatment of cSDH. Inclusion criteria include patients >18 years of age with the radiographic presence of a subdural hematoma for greater than three weeks. All patients were managed with either a trauma catheter or mushroom tip catheter. All patients received computed tomography (CT) of the head without contrast prior to subdural drain placement and within 24 hours after subdural drain removal. Exclusion criteria include patients <18 years of age and patients with depressed skull fractures, vascular malformations, subdural empyema, subdural hygroma, or who initially underwent open craniotomy or burr-hole craniotomy. Results Drain efficiency in evacuating the cSDH was assessed using both radiographic and clinical markers. Analysis of 205 patients treated by twist drill craniostomy and the subsequent closed system drainage utilizing either the mushroom tip catheter or trauma catheter revealed that neither catheter was superior in producing a statistically significant change in the maximum thickness of the cSDH (p = 0.35) and midline shift (p = 0.45). Furthermore, when assessing patients clinically via utilization of the Glasgow Coma Scale (GCS), both the trauma catheter and the mushroom catheter did not show a statistically significant difference in improving GCS after the evacuation of the cSDH (p = 0.35). Neither catheter was associated with an increased incidence of hemorrhage with drain placement requiring open surgery (p = 0.12), need for additional drain placement (p = 0.13) or decline in GCS with intervention (p = 0.065). Conclusion Analysis of the 205 patients treated by twist drill craniostomy with closed system drainage for the evacuation of chronic subdural hematoma utilizing either the mushroom tip or trauma catheters revealed that neither catheter was statistically significant in radiographic or clinical improvement in evacuating cSDH. Furthermore, neither catheter was found to be associated with an increased complication risk.

Pathogenesis and treatment of intracranial arachnoid cysts in pediatric patients younger than 2 years of age.

OBJECT: Arachnoid cysts can cause a variety of clinical signs and symptoms in infants. The authors sought to determine whether the clinical presentation of pediatric patients younger than 2 years old and harboring arachnoid cysts influenced the type of intervention that would be required. METHODS: A retrospective chart review was conducted for all patients younger than 2 years of age who had undergone craniotomy for fenestration of an arachnoid cyst at the Children's Hospital Los Angeles between 1995 and 2006. Forty-two patients were included in the study. The mean age was 10.4 months. The median follow-up time was 33 months. Clinical presentations were as follows: macrocephaly without ventriculomegaly (21 patients, 50%), hydrocephalus (six patients, 14%), and other symptoms (15 patients, 36%). After fenestration of the arachnoid cyst, 12 of 21 patients (57%) presenting with nonspecific macrocephaly required placement of a cystoperitoneal or ventriculoperitoneal shunt, compared with 1 of 15 patients (7%) presenting with other symptoms (p value = 0.0039). Five of six patients with hydrocephalus (83%) were shunt dependent following fenestration. Overall, 18 of 42 patients (43%) were shunt dependent after fenestration. Ten of these patients (55%) required revisions during the follow-up period. CONCLUSIONS: Patients younger than 2 years of age and harboring an arachnoid cyst commonly present with macrocephaly. These patients are more likely to require shunts than are those presenting with other findings, such as seizures or incidental lesions. The development and expansion of arachnoid cysts may be related to aberrant cerebrospinalfluid dynamics, and these lesions may be a form fruste of hydrocephalus. Arachnoid cysts should be treatedwith craniotomy and cyst fenestration, taking into account the likelihood of perioperative shunt dependency.

Craniopharyngioma in childhood.

In summary, much progress has been made in our understanding of childhood craniopharyngiomas. These histologically benign but "geographically malignant" tumors are challenging to treat and require experienced clinicians from multiple disciplines including neurosurgery, radiology, hematology/oncology, ophthalmology, endocrinology, and general pediatrics to address the multiple issues that arise with diagnosis, treatment, and long-term follow-up of affected children. The study and close observation of patients who have craniopharyngiomas may also be beneficial for our general understanding of pathophysiologic processes such as the observed "growth without growth hormone" phenomenon or the well-described and studied hypothalamic obesity phenotypes.

Spinal arachnoid cysts in the pediatric population: report of 31 cases and a review of the literature.

OBJECT: The goal of this study was to review all cases of pediatric spinal arachnoid cysts (SACs) surgically treated at the authors' institution between 1992 and 2008 and to compare these cases to the published literature for the general population. METHODS: The charts of all pediatric patients with SACs were reviewed for demographics, medical history, presenting symptoms, imaging findings, operative procedure(s), complications, and outcomes. Following a complete literature review, the pediatric data were compared with data from the general population and unique findings associated with pediatric patients were identified. RESULTS: Thirty-one pediatric patients (median age 6.9 years) underwent operative intervention for SACs between 1992 and 2008 (median duration of follow-up 4.2 years). There were 17 female patients (55%) and 14 male patients (45%). Twenty-one patients (68%) presented with symptoms of radiculopathy or myelopathy. The most common presenting symptoms were pain (42%), lower-extremity weakness (39%), gait instability (32%), spasticity (19%), sensory loss (10%), and bladder dysfunction (7%). In 3 patients (10%) SACs were incidental findings. Intradural SACs were more common (18 patients, 58%) than extradural SACs (11 patients, 36%). One patient (3%) had extradural and intradural components. One patient (3%) had a purely intramedullary cyst, and 1 patient (3%) had both an intradural and intramedullary component. Of the 18 intradural SACs, 9 (50%) were located ventral to the spinal cord and 9 (50%) were dorsally situated. One dorsal intradural SAC had an intramedullary component. All extradural SACs were located dorsal to the spinal cord. Intradural SACs were primarily concentrated in the cervical and thoracic regions (67%), whereas extradural cysts were more evenly distributed between the thoracic, lumbar, and sacral regions. Of the 18 patients with intradural SACs, 13 (72%) had significant previous CNS abnormalities, compared with 3 (27%) of 11 patients with extradural SACs. There were 2 operative complications. One patient had a CSF leak treated with a lumbar drain, and the second patient had a pseudomeningocele. No patients had neurological deterioration as a result of surgical intervention. Twenty-one patients (68%) had complete remission of symptoms, 6 (19%) had improvement, 3 (10%) were stable, and 1 (3%) has worsening of symptoms with recurrence that ultimately required cystoperitoneal shunting, despite multiple failed attempts at fenestration. CONCLUSIONS: Spinal arachnoid cysts are rare lesions in the pediatric population. Affected patients present with back pain, weakness, and/or gait instability. In children, SACs predominantly develop in the thoracic region and are more likely to occur intradurally, compared with SACs in the general population. Overall outcomes following surgical fenestration or excision of SACs are excellent, with complete remission or improvement of symptoms achieved in 87% of cases.

Scoliosis and Chiari malformation Type I in children.

OBJECT: The identification of Chiari malformations Type I (CM-Is) has increased in recent years, commonly during MR imaging for evaluation of a possible cause of scoliosis. The treatment of this abnormality remains controversial, and the expected success of treatment is unclear. The goal of the present study is to evaluate the effects of a craniotomy for CM-I decompression on scoliosis in children and adolescents. METHODS: The authors conducted a 10-year retrospective review of pediatric patients who were found to have a CM-I during evaluation for scoliosis. Seventy-nine patients were identified, ranging in age from 6 months to 18 years (median 12 years). There were 42 girls (54%) and 37 boys (46%). All were noted on MR imaging to have hydrosyringomyelia of the spinal cord. Forty-nine patients had curvatures less than 20° prior to treatment. The other 30 patients had curves ranging from 25° to 80° and underwent orthopedic follow-up and treatment. None of these patients were referred for specific neurological complaints, but 12 (16%) had neurological signs on physical examination. All were treated with a craniocervical decompression in a standard fashion. Follow-up ranged from 6 to 93 months with a median of 35 months. Magnetic resonance images obtained at 6 months postoperatively and serial standing anteroposterior spine radiographs were used to evaluate outcomes. RESULTS: On the MR images obtained 6 months postoperatively, 70 patients (89%) had a significant reduction in the syrinx with an associated ascent of the cerebellar tonsils. Persistent large syringes were treated with reoperation in 6 patients, and shunts were inserted for hydrocephalus in 2 patients. None of the 49 patients with curves less than 20° had progression of their curvature postoperatively. Of the 30 patients with curves greater than 25°, 9 had no change in the scoliosis or had a reduction in curve magnitude after Chiari decompression. This group required no further therapy and was effectively treated by Chiari decompression alone. Twenty-one patients required further scoliosis treatment after Chiari decompression; 12 required orthotic treatment, 11 received spinal instrumentation and fusion surgery, and 2 received orthoses followed by fusion and instrumentation. The severity of the curvature beyond 20° did not predict the need for spinal surgery. CONCLUSIONS: This large series reports on the efficacy of treatment for scoliosis associated with a CM-I and syrinx in children. A CM-I decompression alone was adequate treatment for mild scoliosis of less than 20°. Patients with scoliosis greater than 20° required bracing and/or spinal fusion surgery 70% of the time in addition to the CM-I decompression.

Evaluation of intracranial cerebrospinal fluid cytology in staging pediatric medulloblastomas, supratentorial primitive neuroectodermal tumors, and ependymomas.

OBJECT: The objective of this study was to determine the role of intracranial CSF examination in detecting true cases of early tumor dissemination. Cerebrospinal fluid dissemination is an ominous feature of pediatric brain tumors, occurring in as many as 30% of medulloblastomas, 25% of supratentorial primitive neuroectodermal tumors (PNETs), and 5% of ependymomas at diagnosis. Detecting early dissemination is important for determining both treatment and prognosis. Dissemination can be detected by evaluating imaging of the full neuraxis and by examining CSF cytology. Neuraxis MR imaging and lumbar CSF cytology evaluation are widely accepted methods for determining dissemination. However, the value of examining intracranial CSF cytology in detecting early dissemination is uncertain. METHODS: Under an institutional review board-approved protocol, medical records, pathology reports, and radiology reports for 150 patients who had undergone resection of brain tumors (88 with medulloblastomas, 21 with supratentorial PNETs, and 41 with ependymomas) and who had been evaluated using neuraxis MR imaging studies in the last 15 years were retrospectively reviewed. Radiology results were compared with the CSF cytology results and long-term disease outcomes. RESULTS: Between lumbar and intracranial CSF cytology results, 7 of 40 were discordant: in 2 intracranial CSF was negative and lumbar CSF was positive, and in 5 the reverse was true. The discordance percentage was 18%, with a kappa statistic of 0.36. Between MR imaging and lumbar CSF cytology results, 11 of 65 were discordant: in 9 the lumbar CSF was negative and MR imaging was positive, and in 2 the reverse was true. The discordance percentage is 17%, with a kappa statistic of 0.27. Between MR imaging and intracranial CSF cytology results, 8 of 52 were discordant: in 3 intracranial CSF was negative and MR imaging was positive, and in 5 the reverse was true. The discordance rate was 15%, with a kappa statistic of 0.41. Patients with positive and negative results on perioperative neuraxis MR imaging studies had a median survival of 26.8 and 33.1 months, respectively (p = 0.02). Patients with positive and negative results on perioperative lumbar CSF cytology had a median survival of 20.1 and 31.4 months, respectively (p = 0.11). Patients with positive and negative results on intracranial CSF cytology had a median survival of 31 and 31.4 months, respectively (p = 0.84). CONCLUSIONS: Discordance exists between the results of neuraxis MR imaging and lumbar and intracranial CSF cytology in perioperative detection of tumor dissemination for pediatric medulloblastoma, supratentorial PNETs, and ependymoma. In 1 case in this series, perioperative dissemination was detected by intracranial CSF cytology, but not by lumbar CSF cytology or neuraxis MR imaging. Isolated intracranial CSF cytology positivity may represent an earlier stage of disseminated disease. Complementary use of perioperative neuraxis MR imaging and lumbar and intracranial CSF cytology can reduce the incidence of missed diagnoses of dissemination. Survival analysis revealed that perioperative neuraxis MR imaging findings are correlated with survival, whereas perioperative lumbar and intracranial CSF cytology findings are not.

Craniospinal Langerhans cell histiocytosis in children: 30 years' experience at a single institution.

OBJECTIVES: The goal of this study was to review a large series of patients with Langerhans cell histiocytosis (LCH) who had craniospinal lesions to assess the long-term course, outcome, and efficacy of treatment of the disease. METHODS: Forty-four patients with LCH who presented to a single pediatric neurosurgical department between 1976 and 2006 were retrospectively reviewed. RESULTS: This series included 29 boys and 15 girls, ranging in age from 2 months to 13 years, with a mean follow-up duration of 4.5 years. Twenty-seven patients (61%) had unifocal bone lesions, 12 (27%) had multifocal bone disease, 2 (5%) had solitary hypothalamic-pituitary axis lesions, and 3 (7%) had multiple organ involvement at presentation. Five (19%) of the 27 patients with unifocal bone disease and 4 (33%) of the 12 patients with multifocal bone disease had delayed development of new bone lesions during the follow-up period. The time to development of new bone lesions ranged from 1 month to 1 year. Two of the 3 patients with multiple-organ LCH died. Patient age < or = 2 years at the time of initial presentation was a risk factor for both initial multifocality and eventual dissemination. In all patients with initial multifocal bone involvement or later dissemination of unifocal bone disease, LCH was controlled by chemotherapy, except for 2 who were treated by surgery alone. Three patients had histological evidence of spontaneous resolution of their lesions. CONCLUSIONS: Patients with unifocal LCH can be effectively treated with surgery alone. Very young patients are more likely to have multifocal disease and disseminations, and will usually require chemotherapy to control their disease. Spontaneously regressing lesions need not be resected; however, a biopsy procedure can be performed for diagnostic purposes.

Effects of surgical resection and adjuvant therapy on pediatric intracranial ependymomas.

The optimal therapy of pediatric ependymomas is controversial. The benefit of surgical resection is widely accepted, but the role of adjuvant therapy is subject to debate. Due to the relatively low survival rates of ependymoma patients, as well as the tumor's high recurrence rates, further research into the efficacy of treatment strategies and adjuvant therapy is necessary. Extent of resection remains the most important determinant of survival in patients with ependymomas. Expectantly, gross total resection yields the best outcome for patients. The optimal roles of chemotherapy and radiation therapy are poorly understood. A closer look at the efficacy of tailored radiation therapy and the possible use of chemotherapy to delay radiation therapy sheds light on potential treatment modalities for ependymomas. The greatest increase in survival on the ependymoma population will likely come from an increase in the rate of complete resections. An improvement in the efficacy of radiation therapy in addition to an understanding of chemotherapy protocols and treatment durations will hopefully provide further means for successfully treating ependymomas.