Association of meningitis and clival canal defect: case illustration, management, and systematic review of the literature

OBJECTIVE Improper embryological development of the clivus, a bony structure that comprises part of the skull base, can lead to a clival canal defect. Previously thought to be a benign condition, clival canals have been reported to be associated with meningitis and meningoceles. In this review, the authors sought to present an unpublished case of a patient with a clival canal defect associated with meningitis and to evaluate all other reported cases. METHODS In October 2020, a search of PubMed, Web of Science, and Scopus was conducted to identify all cases of clival canals reported from January 1, 1980, through October 31, 2020. RESULTS Including the case presented herein, 13 cases of clival canals, 11 in children (84.6%) and 2 in adults (15.4%), have been identified. Of the pediatric patients, 5 (45.5%) had an associated meningocele, and 8 (72.7%) had meningitis. Nine of the 13 patients (69.2%) had defects that were treated surgically, 5 (38.5%) by a transnasal approach and 4 (30.8%) by a transoral approach. Two patients (15.4%) were treated with drainage and antibiotics, 1 patient (7.7%) was treated solely with antibiotics, and 1 patient (7.7%) was not treated. In the literature review, 8 reports of clival canals were found to be associated with meningitis, further contributing to the notion that the clival canal may be an overlooked source of recurrent infection. In several of these cases, surgical repair of the lesion was curative, thus preventing continued episodes of meningitis. CONCLUSIONS When a patient has recurrent meningitis with no clear cause, taking a closer look at clival anatomy is recommended. In addition, if a clival canal defect has been identified, surgical repair should be considered a safe and effective primary treatment option.

Surgical management of quadrigeminal cistern arachnoid cysts: case series and literature review

OBJECTIVE Quadrigeminal cistern arachnoid cysts (QACs) are congenital lesions that can cause pineal region compression and obstructive hydrocephalus when sufficiently large. Management of these cysts is controversial and rates of reintervention are high. Given the limited data on the management of QACs, the authors retrospectively reviewed 20 years of cases managed at their institution and performed a literature review on this topic. METHODS The authors performed a retrospective analysis of patients treated for QAC at their institution between 2001 and 2021. They also performed a literature review of studies published between 1980 and 2021 that reported at least 5 patients treated for QACs. Patient characteristics, radiographic findings, management course, and postoperative follow-up data were collected and analyzed. RESULTS A total of 12 patients treated for a QAC at the authors’ institution met the inclusion criteria for analysis. Median age was 9 months, mean cyst size was 5.1 cm, and 83% of patients had hydrocephalus. Initial treatment was endoscopic fenestration in 92% of these patients, 27% of whom had an endoscopic third ventriculostomy (ETV) performed concurrently. Reintervention was required in 42% of patients. Cases that required reintervention had a statistically significant lower median age at the initial intervention (5 months) than the cases that did not require reintervention (24.33 months; p = 0.018). There were no major complications. At a mean follow-up of 5.42 years, 83% of patients had improvement or resolution of their symptoms. A literature review revealed 7 studies that met the inclusion criteria, totaling 108 patients with a mean age of 8.8 years. Eighty-seven percent of patients had hydrocephalus at presentation. Ninety-two percent of patients were initially treated with endoscopic fenestration, 44% of whom underwent concurrent ETV. Complications occurred in 17.6% of cases, and reintervention was required in 30.6% of cases. The most frequent reason for reintervention was untreated or unresolved hydrocephalus after the initial procedure. CONCLUSIONS Endoscopic fenestration is the most common treatment for QACs. While generally safe and effective, there is a high rate of reintervention after initial treatment of QACs, which may be associated with a younger age at the first intervention. Additionally, identifying patients who require initial treatment of hydrocephalus is critically important, as the literature suggests that untreated hydrocephalus is a common cause of reintervention.

Complete posterior cranial vault distraction osteogenesis to correct Chiari malformation type I associated with craniosynostosis

OBJECTIVE Posterior vault distraction osteogenesis (PVDO) is an effective tool to increase intracranial volume and expand the posterior cranial fossa. During PVDO, the authors extended osteotomy posterior to the foramen magnum to fully expand the posterior cranial fossa. The aim of this study was to investigate the efficacy of complete PVDO in posterior fossa expansion and treatment of Chiari malformation type I (CM-I) in patients with craniosynostosis. METHODS Patients with craniosynostosis who had undergone complete PVDO between January 2012 and May 2020 were reviewed retrospectively. A coronal osteotomy extending to the foramen magnum was performed and the foramen magnum was decompressed by removing its posterior rim with a 1-mm Kerrison rongeur. Four distractor devices were placed and the vector of distraction was controlled from the posterior to the inferior-posterior direction, depending on the deformity. Changes in the intracranial volume, posterior cranial fossa area, and cerebellar tonsillar descent were measured after complete PVDO by using CT and MRI. RESULTS A total of 11 patients with craniosynostosis and concurrent CM-I were included in the study. The mean age was 34.6 ± 24.0 months (continuous variables are expressed as the mean ± SD throughout). One patient had sleep apnea, which was consistent with CM-I, and another patient had a headache, which was nonspecific. The intracranial volume increased from 1179.6 ± 180.2 cm3 to 1440.6 ± 251.5 cm3 (p = 0.003; 24.5% increase compared to the preoperative volume). The posterior skull base area increased from 44.9 ± 19.3 cm2 to 72.7 ± 18.1 cm2 (p = 0.004). Cerebellar tonsillar descent decreased in all 11 patients after complete PVDO (preoperative: 10.8 ± 3.7 mm, postoperative: 2.7 ± 3.0 mm; p = 0.003). Among the 11 patients, 5 showed complete resolution of cerebellar tonsillar herniation. CONCLUSIONS Complete PVDO can more efficiently expand the posterior cranial fossa, unlike conventional methods. Moreover, it helps to relieve cerebellar tonsillar herniation. Complete PVDO is a powerful tool to increase the intracranial and posterior fossa volumes in patients with craniosynostosis and concurrent CM-I.

A propensity-adjusted comparison of endoscopic endonasal surgery versus transcranial microsurgery for pediatric craniopharyngioma: a single-center study

OBJECTIVE When comparing endoscopic endonasal surgery (EES) and transcranial microsurgery (TCM) for adult and mixed-age population craniopharyngiomas, EES has become an alternative to TCM. To date, studies comparing EES and TCM for pediatric craniopharyngiomas are sparse. In this study, the authors aimed to compare postoperative complications and surgical outcomes between EES and TCM for pediatric craniopharyngiomas. METHODS The data of pediatric patients with craniopharyngiomas who underwent surgery between February 2009 and June 2021 at a single center were retrospectively reviewed. All included cases were divided into EES and TCM groups according to the treatment modality received. The baseline characteristics of patients were compared between the groups, as well as surgical results, perioperative complications, and long-term outcomes. To control for confounding factors, propensity-adjusted analysis was performed. RESULTS Overall, 51 pediatric craniopharyngioma surgeries were identified in 49 patients, among which 35 were treated with EES and 16 were treated with TCM. The proportion of gross-total resection (GTR) was similar between the groups (94.3% for EES vs 75% for TCM, p = 0.130). TCM was associated with a lower rate of hypogonadism (33.3% vs 64.7%, p = 0.042) and a higher rate of growth hormone deficiency (73.3% vs 26.5%, p = 0.002), permanent diabetes insipidus (DI) (60.0% vs 29.4%, p = 0.043), and panhypopituitarism (80.0% vs 47.1%, p = 0.032) at the last follow-up. CSF leakage only occurred in the EES group, with no significant difference observed between the groups (p > 0.99). TCM significantly increased the risk of worsened visual outcomes (25.0% vs 0.0%, p = 0.012). However, TCM was associated with a significantly longer median duration of follow-up (66.0 vs 40.5 months, p = 0.007) and a significantly lower rate of preoperative hypogonadism (18.8% vs 60.0%, p = 0.006). The propensity-adjusted analysis revealed no difference in the rate of recurrence, hypogonadism, or permanent DI. Additionally, EES was associated with a lower median gain in BMI (1.5 kg/m2 vs 7.5 kg/m2, p = 0.046) and better hypothalamic function (58.3% vs 8.3%, p = 0.027) at the last follow-up. CONCLUSIONS Compared with TCM, EES was associated with a superior visual outcome, better endocrinological and hypothalamic function, and less BMI gain, but comparable rates of GTR, recurrence, and perioperative complications. These findings have indicated that EES is a safe and effective surgical modality and can be a viable alternative to TCM for pediatric midline craniopharyngiomas.

Single-dose antibiotic prophylaxis compared with multiple-dose protocol in clean pediatric neurosurgical interventions: a nonrandomized, historically controlled equivalence trial

OBJECTIVE Guidelines recommend antimicrobial prophylaxis (AMP) preoperatively for "clean" spinal and cranial surgeries, while dose and timing remain controversial. The use of multiple-dose AMP for such surgeries is under debate in the pediatric context. In this clinical study, the authors aimed to compare single-dose with multiple-dose prophylactic antibiotic usage in cranial and spinal neurosurgical interventions of pediatric patients. METHODS All neurosurgical patients aged 28 days to 18 years who underwent surgery at a single tertiary center were assessed. Three cohorts (noninstrumented clean spinal, noninstrumented cranial, and instrumented cranial interventions), each of which comprised two 50-patient arms (i.e., single-dose AMP and multiple-dose AMP), were included after propensity score–matched retrospective sampling and power analysis. Records were examined for surgical site infections. Using a previously published meta-analysis as the prior and 80% acceptance of equivalence (margin of OR 0.88–1.13), logistic regression was carried out for the total cohort and each subcohort and adjusted for etiology by consideration of multiple-dose AMP as reference. RESULTS The overall sample included 300 age- and sex-matched patients who were evenly distributed in 3 bi-arm cohorts. There was no statistical intercohort difference based on etiology or type of operation (p < 0.05). Equivalence analysis revealed nondiscriminating results for the total cohort (adjusted OR 0.65, 95% CI 0.27–1.57) and each of the subcohorts (noninstrumented clean spinal, adjusted OR 0.65, 95% CI 0.12–3.44; noninstrumented cranial, adjusted OR 0.52, 95% CI 0.14–2.73; and instrumented cranial, adjusted OR 0.68, 95% CI 0.13–3.31). CONCLUSIONS No significant benefit for multiple-dose compared with single-dose AMPs in any of the pediatric neurosurgery settings could be detected. Since unnecessary antibiotic use should be avoided as much as possible, it seems that usage of single-dose AMP is indicated.

Ivy sign: a diagnostic and prognostic biomarker for pediatric moyamoya

OBJECTIVE Ivy sign is a radiographic finding on FLAIR MRI sequences and is associated with slow cortical blood flow in moyamoya. Limited data exist on the utility of the ivy sign as a diagnostic and prognostic tool in pediatric patients, particularly outside of Asian populations. The authors aimed to investigate a modified grading scale with which to characterize the prevalence and extent of the ivy sign in children with moyamoya and evaluate its efficacy as a biomarker in predicting postoperative outcomes, including stroke risk. METHODS Pre- and postoperative clinical and radiographic data of all pediatric patients (21 years of age or younger) who underwent surgery for moyamoya disease or moyamoya syndrome at two major tertiary referral centers in the US and Israel, between July 2009 and August 2019, were retrospectively reviewed. Ivy sign scores were correlated to Suzuki stage, Matsushima grade, and postoperative stroke rate to quantify the diagnostic and prognostic utility of ivy sign. RESULTS A total of 171 hemispheres in 107 patients were included. The median age at the time of surgery was 9 years (range 3 months–21 years). The ivy sign was most frequently encountered in association with Suzuki stage III or IV disease in all vascular territories, including the anterior cerebral artery (53.7%), middle cerebral artery (56.3%), and posterior cerebral artery (47.5%) territories. Following surgical revascularization, 85% of hemispheres with Matsushima grade A demonstrated a concomitant, statistically significant reduction in ivy sign scores (OR 5.3, 95% CI 1.4–20.0; p = 0.013). Postoperatively, revascularized hemispheres that exhibited ivy sign score decreases had significantly lower rates of postoperative stroke (3.4%) compared with hemispheres that demonstrated no reversal of the ivy sign (16.1%) (OR 5.5, 95% CI 1.5–21.0; p = 0.008). CONCLUSIONS This is the largest study to date that focuses on the role of the ivy sign in pediatric moyamoya. These data demonstrate that the ivy sign was present in approximately half the pediatric patients with moyamoya with Suzuki stage III or IV disease, when blood flow was most unstable. The authors found that reversal of the ivy sign provided both radiographic and clinical utility as a prognostic biomarker postoperatively, given the statistically significant association with both better Matsushima grades and a fivefold reduction in postoperative stroke rates. These findings can help inform clinical decision-making, and they have particular value in the pediatric population, as the ability to minimize additional radiographic evaluations and tailor radiographic surveillance is requisite.

Outcome of endoscopic transcortical intraventricular biopsy of isolated thickened pituitary stalk lesions in children

OBJECTIVE The purpose of this study was to investigate the efficacy and safety of endoscopic transcortical intraventricular biopsy in pediatric patients with isolated thickened pituitary stalk lesions. METHODS From 1994 to 2018, 16 pediatric patients (8 males and 8 females) underwent endoscopic transcortical intraventricular biopsy at a single institution to establish an accurate pathological diagnosis for appropriate treatment strategies. RESULTS Preoperatively, the median diameter of the pituitary stalks was 5.31 ± 1.67 mm (range 3.86–9.17 mm). Overall, the diagnostic yield at endoscopic intraventricular biopsy was 93.8%. Histopathological diagnosis included germinoma (n = 10, 62.5%), Langerhans cell histiocytosis (n = 4, 25%), pilocytic astrocytoma (n = 1, 6.3%), and pituicytoma (n = 1, 6.3%). Two patients developed new diabetes insipidus after the procedure, and 3 patients had new postoperative hypothyroidism. There were no postoperative neurological deficits in this series. CONCLUSIONS Neuroendoscopic biopsy via the transcortical intraventricular corridor was shown to be safe and to aid in a reliable histopathological diagnosis in the management of isolated pituitary stalk lesions in pediatric patients. It can be considered a minimally invasive alternative to open biopsy via transcranial or endonasal corridors.

Hydrocephalus in achondroplasia: efficacy of endoscopic third ventriculostomy

OBJECTIVE Ventriculoperitoneal shunts (VPSs) for hydrocephalus in patients with achondroplasia are known to have a higher failure rate than in other hydrocephalus populations. However, the etiology of hydrocephalus in this group is considered “communicating,” and, therefore, potentially not amenable to endoscopic third ventriculostomy (ETV). ETV has, nonetheless, been reported to be successful in a small number of patients with achondroplasia. The authors aimed to investigate the long-term results of ETV in this population. METHODS Patients with achondroplasia who had undergone surgical treatment for hydrocephalus (ETV or VPS placement) were identified. In patients who had undergone ETV, medical records and neuroimages were reviewed to determine ventricular volumes and frontal and occipital horn ratios (FOHRs) pre- and postoperatively, as well as the incidence of surgical complications and reoperation. Patients who underwent VPS placement were included for historical comparison, and their medical records were reviewed for basic demographic information as well as the incidence of surgical complications and reoperation. RESULTS Of 114 pediatric patients with achondroplasia referred for neurosurgical consultation, 19 (17%) were treated for hydrocephalus; 10 patients underwent ETV only, 7 patients underwent VPS placement only, and 2 patients had a VPS placed followed by ETV. In patients treated with ETV, ventricular volume and FOHRs were normal, if measured at birth, and increased significantly until the time of the ETV. After ETV, all patients demonstrated significant and sustained decreases in ventricular measurements with surveillance up to 15 years. There was a statistically significant difference in rates of repeat CSF surgery between the ETV and VPS cohorts (0/12 vs 7/9, p < 0.001). CONCLUSIONS ETV was efficacious, safe, and durable in the treatment of hydrocephalus in patients with achondroplasia. Although many studies have indicated that hydrocephalus in these patients is “communicating,” a subset may develop an “obstructive” component that is progressive and responsive to ETV.

Estimating the prevalence of neurosurgical interventions in adults with spina bifida using the Health Facts data set: implications for transition planning and the development of adult clinics

OBJECTIVE As the care of patients with spina bifida continues to evolve, life expectancy is increasing, leading to a critical need for transition planning from pediatric-based to adult-based care. The burden of neurosurgical care for adults with spina bifida remains unknown. In this study, the authors sought to use a large national data set to estimate the prevalence of neurosurgical interventions in adults with spina bifida. METHODS This study utilized Health Facts, which is a de-identified proprietary data set abstracted from all Cerner electronic health records. It includes 69 million unique patients with > 500 million encounters in 580 centers. Validation, technical exclusions, and data filters were applied to obtain an appropriate cohort of patients. The ICD-9 and ICD-10 codes for all types of spinal dysraphism, as well as the Current Procedural Terminology (CPT) codes for hydrocephalus procedures, spinal cord untethering, and Chiari decompression, were queried and records were retrieved. Demographic variables along with differences in age groups and temporal trends were analyzed. RESULTS Overall, 24,764 unique patients with ≥ 1 encounter with a spinal dysraphism diagnosis between 2000 and 2017 were identified. The pediatric cohort included 11,123 patients with 60,027 separate encounters, and the adult cohort included 13,641 patients with 41,618 separate encounters. The proportion of females was higher in the adult (62.9%) than in the pediatric (51.4%) cohort. Annual encounters were stable from 2 to 18 years of age, but then decreased by approximately half with a precipitous drop after age 21 years. The sex distribution of adults and children who underwent procedures was similar (54.6% female adults vs 52.4% female children). Surgical interventions in adults were common. Between 2013 and 2017, there were 4913 procedures for hydrocephalus, with 2435 (49.6%) adult patients. Similarly, 273 (33.3%) of the 819 tethered cord procedures were performed in adults, as were 307 (32.9%) of 933 Chiari decompressions. CONCLUSIONS The Health Facts database offered another option for studying care delivery and utilization in patients aging with spina bifida. The median age of this population has now reached early adulthood, and a significant number of neurosurgical procedures were performed in adults. An abrupt drop in the rate of encounters occurred at 21 years of age, possibly reflecting transition issues such as access-to-care problems and lack of coordinated care.