Management of cerebrospinal fluid pseudocysts in the laparoscopic age.

OBJECTIVE: Abdominal CSF pseudocysts are an uncommon but challenging complication of ventriculoperitoneal shunts. Pseudocysts consist of a loculated intraperitoneal compartment that inadequately absorbs CSF and may be infected or sterile at diagnosis. The treatment goal is to clear infection if present, reduce inflammation, and reestablish long-term function in an absorptive (intraperitoneal) space. This aim of this paper was to study the efficacy of primary laparoscopic repositioning of the distal shunt catheter for treatment of sterile abdominal CSF pseudocysts. METHODS: All patients treated for abdominal CSF pseudocysts at Dallas Children's Health from 1991 to 2021 were retrospectively reviewed. Patient history and pseudocyst characteristics were analyzed, with a primary outcome of pseudocyst recurrence at 1 year. RESULTS: Of 92 primary pseudocysts, 5 initial treatment strategies (groups) were used depending on culture status, clinical history, and surgeon preference: 1) shunt explant/external ventricular drain (EVD) placement (23/92), 2) distal tubing externalization (13/92), 3) laparoscopic repositioning (35/92), 4) open repositioning (4/92), and 5) other methods such as pseudocyst drainage or direct revision to another terminus (17/92). Seventy pseudocysts underwent shunt reimplantation in the peritoneal space. The 1-year peritoneal shunt survival for groups 1 and 2 combined was 90%, and 62% for group 3. In group 3, 1-year survival was better for those with normal systemic inflammatory markers (100%) than for those with high markers (47%) (p = 0.042). In a univariate Cox proportional hazards model, the risk of pseudocyst recurrence was increased if the most recent abdominal procedure was a nonshunt abdominal surgery (p = 0.012), and it approached statistical significance with male sex (p = 0.054) and elevated inflammatory markers (p = 0.056. Multivariate Cox analysis suggested increased recurrence risk with male sex (p = 0.05) and elevated inflammatory markers (p = 0.06), although the statistical significance threshold was not reached. The length of hospital stay was shorter for laparoscopic repositioning (6 days) than for explantation/EVD placement (21 days) (p < 0.0001). Ultimately, 62% of patients had a peritoneal terminus at the last follow-up, 33% (n = 30) had an extraperitoneal terminus (19 pleura, 8 right heart, and 3 gallbladder), and 5 patients were shunt free. CONCLUSIONS: Some sterile pseudocysts with normal systemic inflammatory markers can be effectively treated with laparoscopic repositioning, resulting in a significantly shorter hospitalization and modestly higher recurrence rate than shunt explantation.

Risk factors for clinical and radiological worsening following Chiari malformation type I surgery in the pediatric population.

OBJECTIVE: Foramen magnum (FM) decompression with or without duraplasty is considered a common treatment strategy for Chiari malformation type I (CM-I). The authors' objective was to determine a predictive model of risk factors for clinical and radiological worsening after CM-I surgery. METHODS: A retrospective review of electronic health records was conducted at an academic tertiary care hospital from 2001 to 2019. A multivariable Cox proportional hazards regression model was used to determine the risk factors. The Kaplan-Meier estimate was plotted to delineate outcomes based on FM size. FM was measured as the preoperative distance between the basion and opisthion and dichotomized into < 34 mm and ≥ 34 mm. Syrinx was measured preoperatively and postoperatively in the craniocaudal and anteroposterior directions using a T2-weighted MRI sequence. RESULTS: A total of 454 patients (231 females [50.9%]) with a median (range) age of 8.0 (0-18) years were included in the study. The median duration of follow-up was 21.0 months (range 3.0-144.0 years). The model suggested that patients with symptoms consisting of occipital/tussive headache (HR 4.05, 95% CI 1.34-12.17, p = 0.01), cranial nerve symptoms (HR 3.46, 95% CI 1.16-10.2, p = 0.02), and brainstem/spinal cord symptoms (HR 3.25, 95% CI 1.01-11.49, p = 0.05) had higher risk, whereas those who underwent arachnoid dissection/adhesion lysis had 75% lower likelihood (HR 0.25, 95% CI 0.10-0.64, p = 0.004) of clinical worsening postoperatively. Similarly, patients with evidence of brainstem/spinal cord symptoms (HR 7.9, 95% CI 2.84-9.50, p = 0.03), scoliosis (HR 1.04, 95% CI 1.01-2.80, p = 0.04), and preoperative syrinx (HR 16.1, 95% CI 1.95-132.7, p = 0.03) had significantly higher likelihood of postoperative worsening of syrinx. Patients with symptoms consisting of occipital/tussive headache (HR 5.44, 95% CI 1.86-15.9, p = 0.002), cranial nerve symptoms (HR 2.80, 95% CI 1.02-7.68, p = 0.04), and nonspecific symptoms (HR 6.70, 95% CI 1.99-22.6, p = 0.002) had significantly higher likelihood, whereas patients with FM ≥ 34 mm and those who underwent arachnoid dissection/adhesion lysis had 73% (HR 0.27, 95% CI 0.08-0.89, p = 0.03) and 70% (HR 0.30, 95% CI 0.12-0.73, p = 0.008) lower likelihood of reoperation, respectively. The Kaplan-Meier curve showed that patients with FM size ≥ 34 mm had significantly better clinical (p = 0.02) and syrinx (p = 0.03) improvement postoperatively when the tonsils were resected. CONCLUSIONS: These results showed that preoperative and intraoperative factors may help to provide better clinical decision-making for CM-I surgery. Patients with FM size ≥ 34 mm may have better outcomes when the tonsils are resected.

Cerebellar tonsil reduction for surgical treatment of Chiari malformation type I in children.

OBJECTIVE: The goal of this study was to review the efficacy and safety of different surgical techniques used for treatment of Chiari malformation type I (CM-I) in children. METHODS: The authors retrospectively reviewed 437 consecutive children surgically treated for CM-I. Procedures were classified into four groups: bone decompression (posterior fossa decompression [PFD]) and duraplasty (PFD with duraplasty [PFDD]), PFDD with arachnoid dissection (PFDD+AD), PFDD with tonsil coagulation of at least one cerebellar tonsil (PFDD+TC), and PFDD with subpial tonsil resection of at least one tonsil (PFDD+TR). Efficacy was measured as a greater than 50% reduction in the syrinx by length or anteroposterior width, patient-reported improvement in symptoms, and rate of reoperation. Safety was measured as the rate of postoperative complications. RESULTS: The mean patient age was 8.4 years (range 3 months to 18 years). In total, 221 (50.6%) patients had syringomyelia. The mean follow-up was 31.1 months (range 3-199 months), and there was no statistically significant difference between groups (p = 0.474). Preoperatively, univariate analysis showed that non-Chiari headache, hydrocephalus, tonsil length, and distance from the opisthion to brainstem were associated with the surgical technique used. Multivariate analysis demonstrated that hydrocephalus was independently associated with PFD+AD (p = 0.028), tonsil length was independently associated with PFD+TC (p = 0.001) and PFD+TR (p = 0.044), and non-Chiari headache was inversely associated with PFD+TR (p = 0.001). In the treatment groups postoperatively, symptoms improved in 57/69 (82.6%) PFDD patients, 20/21 (95.2%) PFDD+AD patients, 79/90 (87.8%) PFDD+TC patients, and 231/257 (89.9%) PFDD+TR patients, and differences between groups were not statistically significant. Similarly, there was no statistically significant difference in postoperative Chicago Chiari Outcome Scale scores between groups (p = 0.174). Syringomyelia improved in 79.8% of PFDD+TC/TR patients versus only 58.7% of PFDD+AD patients (p = 0.003). PFDD+TC/TR remained independently associated with improved syrinx outcomes (p = 0.005) after controlling for which surgeon performed the operation. For those patients whose syrinx did not resolve, no statistically significant differences between surgery groups were observed in the length of follow-up or time to reoperation. Overall, there was no statistically significant difference between groups in postoperative complication rates, including aseptic meningitis and CSF- and wound-related issues, or reoperation rates. CONCLUSIONS: In this single-center retrospective series, cerebellar tonsil reduction, by either coagulation or subpial resection, resulted in superior reduction of syringomyelia in pediatric CM-I patients, without increased complications.

Magnetic resonance imaging-guided stereotactic laser ablation therapy for the treatment of pediatric epilepsy: a retrospective multiinstitutional study.

OBJECTIVE: The authors of this study evaluated the safety and efficacy of stereotactic laser ablation (SLA) for the treatment of drug-resistant epilepsy (DRE) in children. METHODS: Seventeen North American centers were enrolled in the study. Data for pediatric patients with DRE who had been treated with SLA between 2008 and 2018 were retrospectively reviewed. RESULTS: A total of 225 patients, mean age 12.8 ± 5.8 years, were identified. Target-of-interest (TOI) locations included extratemporal (44.4%), temporal neocortical (8.4%), mesiotemporal (23.1%), hypothalamic (14.2%), and callosal (9.8%). Visualase and NeuroBlate SLA systems were used in 199 and 26 cases, respectively. Procedure goals included ablation (149 cases), disconnection (63), or both (13). The mean follow-up was 27 ± 20.4 months. Improvement in targeted seizure type (TST) was seen in 179 (84.0%) patients. Engel classification was reported for 167 (74.2%) patients; excluding the palliative cases, 74 (49.7%), 35 (23.5%), 10 (6.7%), and 30 (20.1%) patients had Engel class I, II, III, and IV outcomes, respectively. For patients with a follow-up ≥ 12 months, 25 (51.0%), 18 (36.7%), 3 (6.1%), and 3 (6.1%) had Engel class I, II, III, and IV outcomes, respectively. Patients with a history of pre-SLA surgery related to the TOI, a pathology of malformation of cortical development, and 2+ trajectories per TOI were more likely to experience no improvement in seizure frequency and/or to have an unfavorable outcome. A greater number of smaller thermal lesions was associated with greater improvement in TST. Thirty (13.3%) patients experienced 51 short-term complications including malpositioned catheter (3 cases), intracranial hemorrhage (2), transient neurological deficit (19), permanent neurological deficit (3), symptomatic perilesional edema (6), hydrocephalus (1), CSF leakage (1), wound infection (2), unplanned ICU stay (5), and unplanned 30-day readmission (9). The relative incidence of complications was higher in the hypothalamic target location. Target volume, number of laser trajectories, number or size of thermal lesions, or use of perioperative steroids did not have a significant effect on short-term complications. CONCLUSIONS: SLA appears to be an effective and well-tolerated treatment option for children with DRE. Large-volume prospective studies are needed to better understand the indications for treatment and demonstrate the long-term efficacy of SLA in this population.

Retrospective analysis of open surgical versus laser interstitial thermal therapy callosotomy in pediatric patients with refractory epilepsy.

OBJECTIVE: Corpus callosotomy remains an established surgical treatment for certain types of medically refractory epilepsy in pediatric patients. While the traditional surgical approach is often well tolerated, the advent of MR-guided laser interstitial thermal therapy (LITT) provides a new opportunity to ablate the callosal body in a minimally invasive fashion and minimize the risks associated with an open interhemispheric approach. However, the literature is sparse regarding the comparative efficacy and safety profiles of open corpus callosotomy (OCC) and LITT callosotomy. To this end, the authors present a novel retrospective analysis comparing the efficacy and safety of these methods. METHODS: Patients who underwent OCC and LITT callosotomy during the period from 2005 to 2018 were included in a single-center retrospective analysis. Patient demographic and procedural variables were collected, including length of stay, procedural blood loss, corticosteroid requirements, postsurgical complications, and postoperative disposition. Pre- and postoperative seizure frequency (according to seizure type) were recorded. RESULTS: In total, 19 patients, who underwent 24 interventions (16 OCC and 8 LITT), were included in the analysis. The mean follow-up durations for the OCC and LITT cohorts were 83.5 months and 12.3 months, respectively. Both groups experienced reduced frequencies of seizure and drop attack frequency postoperatively. Additionally, LITT callosotomy was associated with a significant decrease in estimated blood loss and decreased length of pediatric ICU stay, with a trend of shorter length of hospitalization. CONCLUSIONS: Longer-term follow-up and a larger population are required to further delineate the comparative efficacies of LITT callosotomy and OCC for the treatment of pediatric medically refractory epilepsy. However, the authors' data demonstrate that LITT shows promise as a safe and effective alternative to OCC.

Magnetic resonance-guided stereotactic laser ablation therapy for the treatment of pediatric brain tumors: a multiinstitutional retrospective study.

OBJECTIVE: This study aimed to assess the safety and efficacy of MR-guided stereotactic laser ablation (SLA) therapy in the treatment of pediatric brain tumors. METHODS: Data from 17 North American centers were retrospectively reviewed. Clinical, technical, and radiographic data for pediatric patients treated with SLA for a diagnosis of brain tumor from 2008 to 2016 were collected and analyzed. RESULTS: A total of 86 patients (mean age 12.2 ± 4.5 years) with 76 low-grade (I or II) and 10 high-grade (III or IV) tumors were included. Tumor location included lobar (38.4%), deep (45.3%), and cerebellar (16.3%) compartments. The mean follow-up time was 24 months (median 18 months, range 3-72 months). At the last follow-up, the volume of SLA-treated tumors had decreased in 80.6% of patients with follow-up data. Patients with high-grade tumors were more likely to have an unchanged or larger tumor size after SLA treatment than those with low-grade tumors (OR 7.49, p = 0.0364). Subsequent surgery and adjuvant treatment were not required after SLA treatment in 90.4% and 86.7% of patients, respectively. Patients with high-grade tumors were more likely to receive subsequent surgery (OR 2.25, p = 0.4957) and adjuvant treatment (OR 3.77, p = 0.1711) after SLA therapy, without reaching significance. A total of 29 acute complications in 23 patients were reported and included malpositioned catheters (n = 3), intracranial hemorrhages (n = 2), transient neurological deficits (n = 11), permanent neurological deficits (n = 5), symptomatic perilesional edema (n = 2), hydrocephalus (n = 4), and death (n = 2). On long-term follow-up, 3 patients were reported to have worsened neuropsychological test results. Pre-SLA tumor volume, tumor location, number of laser trajectories, and number of lesions created did not result in a significantly increased risk of complications; however, the odds of complications increased by 14% (OR 1.14, p = 0.0159) with every 1-cm3 increase in the volume of the lesion created. CONCLUSIONS: SLA is an effective, minimally invasive treatment option for pediatric brain tumors, although it is not without risks. Limiting the volume of the generated thermal lesion may help decrease the incidence of complications.

Thirty-day readmission risk after intracranial tumor resection surgeries in children.

OBJECTIVE: The risk of readmission after brain tumor resection among pediatric patients has not been defined. The authors' objective was to evaluate the readmission rates and predictors of readmission after pediatric brain tumor resection. METHODS: Nationwide Readmissions Database (NRD) data sets from 2010 to 2014 were searched for unplanned readmissions within 30 days of the discharge date after pediatric brain tumor resection. Patient demographic variables included sex, age, expected payment source (Medicaid or private insurance), and median annual household income. Readmission events for chemotherapy, radiation therapy, or further tumor resection were not included. RESULTS: Of 282 patients (12.7%) readmitted within 30 days of the index event, the median time to readmission was 10 days (IQR 5-19 days). The most common reason for readmission was hydrocephalus, which accounted for 19% of readmission events. Other CNS-related complications (24%), surgical site infections or septicemia (14%), seizures (7%), and hematological disorders (7%) accounted for other major readmission events. The median charge for readmission events was $35,431, and the median length of readmission stay was 4 days. In multivariate regression, factors associated with a significant increase in readmission risk included Medicaid as the primary payor, discharge from the index event with home health services, and fluid and electrolyte disorders during the index event. CONCLUSIONS: More than 10% of pediatric brain tumor patients have unplanned readmission events within 30 days of discharge after tumor resection. Medicaid patients and those with preoperative or early postoperative fluid and electrolyte disturbances may benefit from early or frequent outpatient visits after tumor resection.

Inchoate guidelines of endoscopic resection of colloid cysts.

Colloid cyst are cystic lesions in the third ventricle and could render patients asymptomatic. However, there is an inherent risk of symptomatic progression, acute decompensation, and sudden death. Therefore, there is no clear consensus as how to observe or when to treat a newly diagnosed patient with a colloid cyst. The authors' objective is to identify the risk factors and then develop a risk stratification score to guide neurosurgeons during acute or chronic presentation. Radiological imaging characteristics have been outlined for the risk stratification as well preoperative evaluation. A baseline neuropsychological evaluation is helpful to obtain during an incidental presentation because history and neurological examination could be inconclusive in these cases. Radiological imaging with an MRI brain scan plays a vital role for the initial screening (determination of the cyst size, exact location, and the imaging characteristics) as well as for the preoperative planning. Stereotactic guidance is a high yield, followed by neuroendoscopic resection of the colloid cyst has been an established approach to resect these lesions. Modified colloid cyst risk scoring (mCCRS) system is robust and detailed for the optimal risk stratification of colloid cyst presentation. Stereotactic guided neuroendoscopic resection of the colloid cyst is a safe and efficacious approach to manage these lesions. The intended use, crucial steps involved, and the limitations of the technique have been discussed especially with a focus on the recurrence. Moreover, a comprehensive treatment algorithm has been presented.

Diffuse intrinsic pontine gliomas: Diagnostic approach and treatment strategies.

Diffuse intrinsic pontine gliomas (DIPG) are high grade gliomas of the brainstem with fatal outcomes. Radiation is known to be partially effective to control the immediate flare but relapse is frequent. There has been ongoing research to study the role of molecular subgroups and identification of specific targets but this is not possible with histopathological diagnosis alone. The authors' objective is to highlight the need for and discuss ongoing molecular research. There is an inherent need for the availability of tumor tissue to be able to conduct research studies. The authors advocate the use of neuronavigation assisted stereotactic technique for tumor biopsy. The technique is feasible with a predefined surgical trajectory. After obtaining tissue diagnosis further work can be performed to isolate and identify histone protein genetic mutations and methylation changes responsible for DIPG molecular subgrouping. Moreover, convection enhanced delivery of therapeutic agents is being developed for better instillation of future drug agents. Despite identification of genetic/epigenetic mutations, growth factors, receptors, and tissue biomarkers, the oncogenesis of DIPG remains elusive. The authors' effort to provide a comprehensive review on DIPG to better understand the disease, need for tissue diagnosis, described surgical technique, and need for pre-clinical and clinical future research is novel.

Concomitant ruptured anterior circulation and unruptured posterior circulation aneurysms: Treatment strategy and review of literature.

Basilar artery apex or bifurcation is the most common location for aneurysms arising from posterior cerebral circulation. Reports of unruptured aneurysms of the basilar bifurcation associated with ruptured anterior circulation aneurysms are rare. The presence of multiple intracranial aneurysms poses a significantly high risk to management than a single aneurysm due several factors involved. Surgical management is considered the best treatment modality for most aneurysmal types and location with quite a few limitations when applicable. Authors have conducted a literature review of anterior and posterior circulation concomitant aneurysms and report their own experience with a case of anterior communicating artery blister type aneurysmal rupture presented with the symptoms and signs of subarachnoid hemorrhage concomitant with an unruptured basilar artery bifurcation aneurysm. Moreover, the anomalous origin of thalamoperforators at the basilar apex instead of the posterior cerebral artery makes it reasonably challenging for the microsurgical clipping. Discussed is the clinical presentation, radiological studies obtained, surgical approach utilized with an adequate exposure of the entire circle of Willis as well as the critical decision making when managing these challenging cases.

Predictors of 90-Day Readmission in Children Undergoing Spinal Cord Tumor Surgery: A Nationwide Readmissions Database Analysis.

OBJECTIVE: A fair number of hospital admissions occur after 30 days; thus, the true readmission rate could have been underestimated. Therefore, we hypothesized that the 90-day readmission rate might better characterize the factors contributing to readmission for pediatric patients undergoing spinal tumor resection. METHODS: The Nationwide Readmissions Database was used to study the patient demographic data, comorbidities, admissions, hospital course, spinal tumor behavior (malignant vs. benign), complications, revisions, and 30- and 90-day readmissions. RESULTS: Of the 397 patients included in the 30-day cohort, 43 (10.8%) had been readmitted. In comparison, the 90-day readmission rate was significantly greater; 52 of 325 patients were readmitted (16.0%; P < 0.04). Patients aged 16-20 constituted the largest subgroup. However, the highest readmission rate was observed for patients aged <5 years (30-day, 21.7%; 90-day, 26.4%). Medicaid patients were more likely to be readmitted than were private insurance patients (30-day odds ratio [OR], 3.3 [P < 0.001]; 90-day OR, 2.29 [P < 0.02]). In both cohorts, patients with malignant tumors required readmission more often than did those with benign tumors (30-day OR, 2.78 [P < 0.02]; 90-day OR, 1.92 [P = 0.08]). In the 90-day cohort, the patients had been readmitted 26.4 days after discharge versus 10.6 days in the 30-day cohort. Within the 90-day cohort, 18.6% of the readmissions were for spinal reoperation, 28.3% for chemotherapy or hematologic complications, and 25.6% for other central nervous system disorders. The median charges for each readmission were ∼$50,000 and ∼$40,000 for the 30- and 90-day cohorts, respectively. Medicaid insurance, malignant tumors, and younger age were significant predictors of readmission in the 90-day cohort. CONCLUSIONS: The prevalence and charges associated with unplanned hospital readmissions after spinal tumor resection were remarkably high. Younger age, Medicaid insurance, malignant tumors, and complications during the initial admission were significant predictors of 90-day readmission.

A history of the Jannetta procedure.

Peter Jannetta was a neurosurgery resident when he proposed the neurovascular compression theory. He built upon the astute observations of Dandy, Gardner, and others who, in the era before the operating microscope, had successfully ventured into the posterior fossa. In 1965, Jannetta performed cranial nerve microdissections for dental students and identified the trigeminal portio intermedia. He proposed that preservation of these sensory fibers may avoid complete facial numbness, and together with Robert Rand developed a subtemporal transtentorial approach for selective rhizotomy for trigeminal neuralgia (TN). Such rash surgery, using an operating microscope, was then forbidden at their University of California, Los Angeles center, so they collaborated with John Alksne to perform the first surgery at Harbor General Hospital. Upon visualizing the trigeminal nerve root, Jannetta was surprised to see a pulsating superior cerebellar artery compressing the nerve and said "That's the cause of the tic." He also hypothesized that alleviating the observed vascular cross-compression may be curative.A few months later, while assessing a patient with hemifacial spasm, Jannetta had the epiphany that this was the same disease process as TN, but instead affecting the facial nerve. The patient consented to what would become Jannetta's first microvascular decompression procedure. The senior faculty members who had forbidden such surgery were away, so the supervising neurosurgeon, Paul Crandall, granted the approval to perform the surgery and assisted. Via a retromastoid approach with the patient in the sitting position and using the operating microscope, Jannetta identified and alleviated the culprit neurovascular compression, with a cure resulting.Jannetta presented his neurovascular compression theory and operative findings to the neurosurgical patriarchy of the time. Elders of the field were generally not inclined to accept the bold speculations of an untested neurosurgeon, and were often determined to discredit the new "cure" of the old diseases. Over decades of refining his surgical technique, documenting the outcomes, and enduring the skepticism he often faced, Jannetta's theory and his microvascular decompression procedure withstood critical analysis and have become recognized as one the great discoveries and advances in neurosurgery and medicine.

Intraventricular bone dust migration after neuroendoscopy: report of 2 cases.

Neuroendoscopy has demonstrated safety and efficacy in the treatment of a host of pediatric neurosurgical pathologies. With the increase in its applicability, several associated complications have been described in the literature. A common practice in pediatric neurosurgery is the use of Gelfoam sponge pledget in the burr hole, followed by bone fragments and dust (obtained from the created burr hole), to cover the dural defect. This technique is used to enhance burr hole sealing and potentially prevent CSF leakage from the surgical site. Reports on intracranial bone dust migration associated with this technique are scarce. The authors report 2 cases of intracranial migration of bone fragments after an endoscopic third ventriculostomy and an endoscopic colloid cyst resection. The bone fragment migration was thought to be caused by negative pressure from a lumbar puncture in one case and external trauma to the head in the other. As endoscopy becomes more widely used, it is important to be aware of this potential complication that may in some cases require an intervention. A review of the cases reported in the literature is provided and a technique is suggested to help prevent this complication.

Isolated Dorsal Thoracic Neuroenteric Cyst with Spinal Cord Compression: Case Reports in Pediatrics.

BACKGROUND: Neuroenteric cysts are rare developmental anomalies of the central nervous system that account for approximately 0.7%-1.3% of all spinal cord lesions. The majority of spinal neuroenteric cysts are located ventral to the spinal cord. Dorsally located neuroenteric cysts are rare and are almost always associated with other spinal anomalies. Isolated dorsal spinal neuroenteric cysts, without other associated spinal anomalies, are extremely rare. These lesions can be mistaken for the more commonly encountered spinal cysts (e.g., arachnoid cysts, ependymal cysts, cystic teratomas). CASE DESCRIPTION: We present the first pediatric case of a 9-month-old boy with an isolated dorsal thoracic neuroenteric cyst and cord compression that underwent surgical resection. The patient tolerated the procedure well and remained neurologically stable after surgery. Pathology was consistent with a neuroenteric cyst. Complete resection was not achieved at the time of surgery. In retrospect, knowing the lesion is a neuroenteric cyst, a more aggressive approach aiming for a complete surgical resection should have been undertaken. To our knowledge, only 2 adult cases of isolated dorsal spinal neuroenteric cysts have been reported in the literature. CONCLUSIONS: Neuroenteric cysts can occur dorsal to the spinal cord without any associated spinal anomalies. The gold standard treatment for neuroenteric cysts is surgical resection, and the goal of surgery is complete resection.

Limited reduction cranioplasty for the treatment of hydrocephalic macrocephaly.

BACKGROUND: Hydrocephalic macrocephaly, occurring despite adequate cerebrospinal fluid shunting, is rare, and most publications advocate near-total cranial vault reduction procedures. The authors reviewed our series of limited reductions (designed to minimize complications while still providing functional benefits) to evaluate outcomes. METHODS: All patients undergoing posterior reduction cranioplasties were retrospectively reviewed for outcomes, including operative data, length of stay, preoperative and postoperative anthropometrics, and complications. In addition, preoperative and postoperative motor function was assessed using a novel scale. These data were then compared with published series. RESULTS: Ten patients (five male, five female) underwent reduction cranioplasties for macrocephaly at an average age of 17.9 months (range, 6 to 53 months) and were followed for an average of 41.5 months. The mean operative time was 4.9 hours (range, 4.3 to 6.5 hours), estimated blood loss was 530 ml (range, 200 to 1500 ml), and 78 percent received blood transfusions. The average length of hospitalization was 2.6 days. Three patients experienced complications, including one shunt revision. The mean functional assessment scores increased from 2.3 to 3.9 (p = 0.022), with all patients able to support their heads postoperatively. CONCLUSIONS: Use of a limited cranioplasty technique was associated with a hospitalization that was over 7 days shorter than has been reported in the literature for total cranial vault reductions and with a much lower shunt revision rate. Measurable improvements in motor function and subjective benefits in appearance were noted, despite a more limited reduction. Surgeons faced with this unusual condition may wish to consider performing this smaller procedure.

Screw fixation of the upper cervical spine in the pediatric population. Clinical article.

OBJECT: Rigid fixation of the upper cervical spine has become an established method of durable stabilization for a variety of craniocervical pathological entities in children. In children, specifically, the use of C1-2 transarticular screws has been proposed in recent literature to be the gold standard configuration for pathology involving these levels. The authors reviewed the use of rigid fixation techniques alternative to C1-2 transarticular screws in children. Factors evaluated included ease of placement, complications, and postoperative stability. METHODS: Seventeen patients, ranging in age from 3 to 17 years (mean 9.6 years), underwent screw fixation involving the atlas or axis for a multitude of pathologies, including os odontoideum, Down syndrome, congenital instability, iatrogenic instability, or posttraumatic instability. All patients had preoperative instability of the occipitocervical or atlantoaxial spine demonstrated on dynamic lateral cervical spine radiographs. All patients also underwent preoperative CT scanning and MR imaging to evaluate the anatomical feasibility of the selected hardware placement. Thirteen patients underwent C1-2 fusion, and 4 underwent occipitocervical fusion, all incorporating C-1 lateral mass screws, C-2 pars screws, and/or C-2 laminar screws within their constructs. Patients who underwent occipitocervical fusion had no instrumentation placed at C-1. One patient's construct included sublaminar wiring at C-2. All patients received autograft onlay either from from rib (in 15 patients), split-thickness skull (1 patient), or local bone harvested within the operative field (1 patient). Nine patients' constructs were supplemented with recombinant human bone morphogenetic protein at the discretion of the attending physician. Eight patients had surgical sacrifice of 1 or both C-2 nerve roots to better facilitate visualization of the C-1 lateral mass. One patient was placed in halo-vest orthosis postoperatively, while the rest were maintained in rigid collars. RESULTS: All 17 patients underwent immediate postoperative CT scanning to evaluate hardware placement. Follow-up was achieved in 16 cases, ranging from 2 to 39 months (mean 14 months), and repeated dynamic lateral cervical spine radiography was performed in these patients at the end of their follow-up period. Some, but not all patients, also underwent delayed postoperative CT scans, which were done at the discretion of the treating attending physician. No neurovascular injuries were encountered, no hardware revisions were required, and no infections were seen. No postoperative pain was seen in patients who underwent C-2 nerve root sacrifice. Stability was achieved in all patients postoperatively. In all patients who underwent delayed postoperative CT scanning, the presence of bridging bone was shown spanning the fused levels. CONCLUSIONS: Screw fixation of the atlas using lateral mass screws, in conjunction with C-2 root sacrifice in selected cases, and of the axis using pars or laminar screws is a safe method for achieving rigid fixation of the upper cervical spine in the pediatric population.

Failure to treat obstructive hydrocephalus with endoscopic third ventriculostomy in a patient with neurodegenerative Langerhans cell histiocytosis.

The second most frequent central nervous system involvement pattern in Langerhans cell histiocytosis (LCH) is a rare condition documented in a number of reports called "neurodegenerative LCH" (ND-LCH). Magnetic resonance images confirming the presence of the disease usually demonstrate striking symmetric bilateral hyperintensities predominantly in the cerebellum, basal ganglia, pons, and/or cerebral white matter. The authors here describe for the first time in the literature a patient with ND-LCH and concomitant hydrocephalus initially treated using endoscopic third ventriculostomy (ETV). This 9-year-old boy, who had undergone chemotherapy for skin and lung LCH without central nervous system involvement at the age of 10 months, presented with acute ataxia, headaches, and paraparesis and a 1-year history of gradually increasing clumsiness. Magnetic resonance images showed obstructive hydrocephalus at the level of the aqueduct of Sylvius and signs of ND-LCH. After registering high intracranial pressure (ICP) spikes with an intraparenchymal pressure monitor, an ETV was performed. A second ETV was required months later because of ostomy occlusion, and finally a ventriculoperitoneal shunt was placed because of ostomy reocclusion. Endoscopic third ventriculostomy was initially considered the treatment of choice to divert cerebrospinal fluid without leaving a ventriculoperitoneal shunt and to obtain biopsy specimens from the periinfundibular recess area. The third ventriculostomy occluded twice, and an endoscopic aqueduct fenestration was unsuccessful. The authors hypothesized that an inflammatory process related to late ND disease was responsible for the occlusions. Biopsy specimens from the infundibular recess and fornix column did not show histopathogical abnormalities. Increased ICP symptoms resolved with cerebrospinal fluid diversion. This case is the first instance of ND-LCH with hydrocephalus reported in the literature to date. Shunt placement rather than ETV seems to be the favorable choice in relieving elevated ICP.