Video article: the subtemporal extradural approach to cavernous sinus tumour.

OBJECTIVE: Here we demonstrate via operative video the subtemporal extradural approach to a tumour in the cavernous sinus. METHODS: The extradural approach is performed here in a paediatric patient (a 15-year-old child) via a right extended pterional osteoplastic craniotomy with removal of the zygomatic arch. The operative microscope is introduced, and the dura is divided at the superior orbital fissure into endosteal and meningeal layers using a diamond knife. The middle cranial fossa floor is drilled flat to increase access, and the plane is further developed towards the cavernous sinus. The tumour is seen bulging from within the cavernous sinus, and the cavernous sinus is opened in the anteromedial triangle between cranial nerves Vi and Vii. After biopsy, the tumour is debulked with an ultrasonic aspirator. Doppler is used to identify the internal carotid artery and preserve it. The bone flap is replaced, and the wound is closed in layers in standard fashion. RESULTS: The patient recovered well and was discharged on post-operative day 3. Persistent sixth nerve palsy (present pre-operatively) was present; however, otherwise, there was good recovery from surgery. Good resection of tumour is demonstrated on post-operative MR imaging. CONCLUSIONS: This approach is uncommon but important as it enables extradural access to the cavernous sinus, minimising the complications associated with an intradural approach such as cortical injury. In this video, we also demonstrate the fundamental anatomy using annotation and cadaveric images to enhance understanding required for the neurosurgeon to successfully complete this approach. The patient consented to the procedure in the standard fashion.

Fronto-orbital advancement and reconstruction using reverse frontal bone graft without the use of orbital bar: a technical note.

INTRODUCTION: We describe our technique of using reverse frontal bone graft for FOAR for patients with metopic or coronal synostosis and present our complications using the Leeds classification system for complications in craniosynostosis surgery. METHODS: Since April 2015, seventeen patients have been operated using this technique. We perform a frontal bone graft that is then reversed, and supraorbital margins are drilled out. The orbital bar is then removed and drilled down to make bone dust and on-lay bone grafts which are then used to fill gaps on exposed dura and fill in around the temporal region. RESULTS: All 17 patients who underwent this technique have good cosmetic results. We report 5 (29%) complications and 8 (47%) blood transfusions (7 exposures, 1 cell salvage).

Chiari I malformation and altered cerebrospinal fluid dynamics-the highs and the lows.

PURPOSE: This paper reviews the altered cerebrospinal fluid dynamics that can be associated with paediatric Chiari I malformation and we present our own institutional experience. METHODS: We conducted a thorough review of the literature and retrospectively analyzed all cases of operatively managed paediatric Chiari 1 malformation at our institution between February 2006 and February 2019. RESULTS: Acquired Chiari malformation (ACM) can radiologically mimic Chiari I and has been associated with both intracranial hypotension (either secondary to lumboperitoneal shunting or spontaneous CSF hypotension) and idiopathic intracranial hypertension (IIH). At our institution, 61 paediatric cases (range, 2-15 years) underwent foramen magnum decompression (FMD) for Chiari I malformation. Whilst 80% (50/61) of cases underwent FMD with no preceding or post-operative problems of CSF dynamics, 8% (5/61) of cases had hydrocephalus at initial presentation requiring CSF diversion followed by FMD for persistent Chiari, and 10% (6/61) developed hydrocephalus following FMD and required long-term CSF diversion. CONCLUSIONS: In paediatric ACM, the management of intracranial hypotension involves thorough radiological assessment and inclusion/adjustment of a valve in the case of lumboperitoneal shunting or epidural blood patch or interventional techniques in the case of spontaneous CSF leak. Thereby, unwarranted posterior fossa decompression surgery is avoided. In the case of IIH and Chiari I malformation, children who have recurrent symptoms despite adequate posterior fossa decompression surgery (failed Chiari), there is a strong role for intracranial pressure monitoring as raised intracranial pressure may indicate long-term CSF diversion.

Outcome and prognostic features in paediatric pineoblastomas: analysis of cases from the Surveillance, Epidemiology, and End Results registry (1990-2007).

BACKGROUND: Paediatric pineoblastomas are rare central nervous system tumours. Patient and treatment factors associated with outcome are poorly defined and limited to small retrospective case series and single case reports. METHODS: Using the Surveillance, Epidemiology, and End Results (SEER) cancer registry, we investigated clinical and pathological factors associated with outcome in paediatric pineoblastomas. Paediatric patients (< 16 years old) with pineoblastomas diagnosed between 1990 and 2007 were identified from the SEER database. Kaplan-Meier survival analysis and Cox models were used to examine the effect of variables on overall survival. The variables analysed included patient's age at diagnosis, gender, race, tumour spread and size, surgical resection and the use of adjuvant radiotherapy. RESULTS: Seventy-eight patients were identified from the database. Twelve patients were excluded as 11 had no surgery and one patient was excluded as the surgical status was unknown. Analysis of the remaining 66 patients revealed a median age at diagnosis of 5.5 years. Three patients underwent biopsy. Seventeen patients underwent full and partial resection, respectively. A further 46 patients underwent surgery the nature of which was not recorded. Thirty-nine patients (59.1%) received adjuvant radiotherapy. Eight patients (12.1%) had both surgery (full or partial resection) and radiotherapy. The median overall survival was 40.5 months. Univariate analysis demonstrated that older age at diagnosis was the only positive predictor of overall survival. CONCLUSION: This study represents the largest analysis of paediatric pineoblastomas to date. The only clinically relevant prognostic factor was older age at diagnosis. The role of surgery and adjuvant radiotherapy on overall survival remains to be defined.

Neuronavigation as a diagnostic tool: An innovative application.

The role of neuronavigation in tumour surgery is well established. We present an innovative use for this software, as a tool to compare ventricular size between serial sets of cranial imaging. We conclude that neuronavigation software is potentially a powerful diagnostic tool that should not be overlooked when making comparative assessments between scans.

Case series of atypical high flow arterio-venous malformations in children.

Arteriovenous malformations (AVM) constitute a clinically significant form of vascular malformations in children. We present three cases of paediatric AVMs that demonstrated unusual features of high flow and significant shunting of blood without a clearly demonstrable nidus. Venous malformations were associated with the lesions. A discussion of the concepts underlying AVM physiology and how these case reports contradict these is included.

Surgical correction of midline nasal dermoid sinus cysts.

Nasal dermoid sinus cysts (NDSCs) are rare congenital anomalies affecting approximately 1 in 30,000 live births. Nasal dermoid sinus cysts are unsightly, prone to infection, and, importantly, may communicate with the central nervous system. Treatment is complete surgical excision. This study retrospectively evaluated management of a large single-center cohort of intracranial NDSCs.Nineteen patients with NDSC were identified from all patients presenting to the Leeds craniofacial service between June 2000 and August 2008. Patient demographics, clinical presentation, preoperative investigations, and surgical procedures undertaken were analyzed.Mean age at presentation and surgery were 6.3 and 7.6 years, respectively. Fifty-three percent were males. Computed tomography (CT) and magnetic resonance imaging (MRI) were performed in 15 and 17 patients, respectively. One patient (5.3%) required local excision only. Eighteen (96.7%) underwent a bicoronal approach, and 13 (68.4%) of these required a craniotomy. The dura was opened in 7 (36.8%) patients. Neither CT nor MRI predicted the presence or absence of intracranial extension in all patients. Positive and negative predictive values for intracranial extension were 85.7% and 50% for CT and were 100% and 50.0% for MRI. Mean follow-up of 4.1 years shows no deep recurrences and 5 (26.3%) were superficial nasal recurrences only.A multidisciplinary approach can achieve good results with infrequent intracranial recurrence. We used a bicoronal approach to facilitate craniotomy when required intraoperatively because imaging is unable to diagnose intracranial extension with sufficient accuracy.

Prognostic value of leukocytosis in pediatric traumatic brain injury.

OBJECTIVE: The purpose of this study was to assess leukocytosis and its prognostic value in pediatric isolated traumatic brain injury (TBI). METHODS: Two hundred one children with isolated TBI admitted to the authors' institution between June 2006 and June 2018 were prospectively followed and their data retrospectively analyzed. Initial blood leukocyte count (i.e., white cell count [WCC]), Glasgow Coma Scale (GCS) score, CT scans, duration of hospital stay, and Pediatric Cerebral Performance Category Scale (PCPCS) scores were analyzed. RESULTS: The mean age was 4.2 years (range 0.2-16 years). Seventy-four, 70, and 57 patients had severe (GCS score 3-8), moderate (GCS score 9-13), and mild (GCS score 14-15) TBI, respectively, with associated WCC of 20, 15.9, and 10.7 × 109/L and neutrophil counts of 15.6, 11.3, and 6.1 × 109/L, respectively (p < 0.01). Higher WCC and neutrophil counts were demonstrated in patients with increased intracranial mass effect on CT, longer hospital stay, and worse 6-month PCPCS score (p < 0.05). Multivariate regression revealed a cutoff leukocyte count of 16.1 × 109/L, neutrophil count of 11.9 × 109/L, and neutrophil-to-lymphocyte ratio (NLR) of 5.2, above which length of hospital stay and PCPCS scores were less favorable. Furthermore, NLR was the second most important independent risk factor for a poor outcome (after GCS score). The IMPACT (International Mission for Prognosis and Analysis of Clinical Trials in TBI) adult TBI prediction model applied to this pediatric cohort demonstrated increased accuracy when WCC was incorporated as a risk factor. CONCLUSIONS: In the largest and first prospective study of isolated pediatric head injury to date, the authors have demonstrated that WCC > 16.1 × 109/L, neutrophil count > 11.9 × 109/L and NLR > 5.2 each have predictive value for lengthy hospital stay and poor PCPCS scores, and NLR is an independent risk factor for poor outcome. Incorporating the initial leukocyte count into TBI prediction models may improve prognostication.

Impact of age at surgery on neurodevelopmental outcomes in sagittal synostosis.

OBJECTIVE: The aim of this study was to ascertain whether age at surgery has an impact on later neurodevelopmental outcomes for children with sagittal synostosis (SS). METHODS: The developmental outcome data from patients who had surgery for SS and who attended their routine preoperative, 6-7 months postoperative, and 5-year-old developmental assessments (yielding general quotients [GQs]) (n = 50), 10-year-old IQ assessment (n = 54), and 15-year-old IQ assessment (n = 23) were examined, comparing whether they had surgery at < 7 months, 7 to < 12 months, or ≥ 12 months). RESULTS: There was no significant effect for age at surgery for GQ at 5 years of age, but there was a significant effect (p = 0.0001) for those undergoing surgery at < 7 months in terms of preoperative gross locomotor deficit that resolved by 6-7 months postoperatively (increase of 22.1 points), and had further improved by 5 years of age (total increase of 29.4 points). This effect was lessened when surgery was performed later (total increase of 7.3 points when surgery was performed at ≥ 12 months). At 10 years of age, 1-way ANOVA showed a significant difference in Full Scale IQ (FSIQ) score (p = 0.013), with the highest mean FSIQ being obtained when surgery was performed at < 7 months of age (score 107.0), followed by surgery at 7 to < 12 months (score 94.4), and the lowest when surgery was performed at ≥ 12 months (score 93.6). One-way ANOVA for the Performance IQ (PIQ) was very similar (p = 0.012), with PIQ scores of 101.4, 91.4, and 87.3, respectively. One-way ANOVA for Verbal IQ (VIQ) was again significant (p = 0.05), with VIQ scores of 111.3, 98.9, and 100.4, respectively. At 15 years, 1-way ANOVA showed a significant difference in PIQ (p = 0.006), with the highest mean PIQ being obtained when surgery was performed at < 7 months (score 104.8), followed by surgery at 7 to < 12 months (score 90.0), and the lowest when surgery was at performed at ≥ 12 months of age (score 85.3). There were no significant results for FSIQ and VIQ, although there was a similar trend for better outcomes with early surgery. CONCLUSIONS: The findings of this study add to the literature that suggests that early surgery for SS may result in improved neurodevelopmental outcomes, with surgery optimally undertaken when patients are < 7 months of age, and with those undergoing surgery at ≥ 12 months performing the least well. These results also have potential implications for ensuring early diagnosis and referral and for the type of surgery offered. Further research is needed to control for confounding factors and to identify the mechanism by which late surgery may be associated with poorer neurodevelopmental outcomes.

Endoscopic biopsy during third ventriculostomy in paediatric pineal region tumours.

OBJECTS: The goal of the study was to establish if endoscopic biopsy during third ventriculostomy contributes to morbidity in the management of paediatric pineal region tumours presenting with hydrocephalus. MATERIALS AND METHODS: This study was a retrospective descriptive study in patients less than 18 years of age who have presented with a pineal region tumour between 2000 and 2006. Data were obtained from case notes. Twelve patients had presented with a pineal region mass with symptomatic hydrocephalus. Of these, eight had endoscopic biopsy during third ventriculostomy. CONCLUSIONS: No mortality or permanent morbidity was seen following endoscopic biopsy. Two cases of transient worsening of pre-operative diplopia were noted. Diagnostic sensitivity for endoscopic biopsy is 75%. Tumour markers were not significantly raised in any plasma and cerebrospinal fluid samples. Endoscopic biopsy during third ventriculostomy in paediatric pineal region tumours is safe and results in good diagnostic yields. It should play an integral role in the initial management of patients in this setting.

Factors associated with 30-day ventriculoperitoneal shunt failure in pediatric and adult patients.

OBJECTIVE The aim of this study was to provide a comprehensive benchmark of 30-day ventriculoperitoneal (VP) shunt failure rates for a single institution over a 5-year study period for both adult and pediatric patients, to compare this with the results in previously published literature, and to establish factors associated with shunt failure. METHODS A retrospective database search was undertaken to identify all VP shunt operations performed in a single, regional neurosurgical unit during a 5-year period. Data were collected regarding patient age, sex, origin of hydrocephalus, and whether the shunt was a primary or secondary shunt. Operative notes were used to ascertain the type of valve inserted, which components of the shunt were adjusted/replaced (in revision cases), level of seniority of the most senior surgeon who participated in the operation, and number of surgeons involved in the operation. Where appropriate and where available, postoperative imaging was assessed for grade of shunt placement, using a recognized grading system. Univariate and multivariate models were used to establish factors associated with early (30-day) shunt failure. RESULTS Six hundred eighty-three VP shunt operations were performed, of which 321 were pediatric and 362 were adult. The median duration of postoperative follow-up for nonfailed shunts (excluding deaths) was 1263 days (range 525-2226 days). The pediatric 30-day shunt failure rates in the authors' institution were 8.8% for primary shunts and 23.4% for revisions. In adults, the 30-day shunt failure rates are 17.7% for primary shunts and 25.6% for revisions. In pediatric procedures, the number of surgeons involved in the operating theater was significantly associated with shunt failure rate. In adults, the origin of hydrocephalus was a statistically significant variable. Primary shunts lasted longer than revision shunts, irrespective of patient age. CONCLUSIONS A benchmark of 30-day failures is presented and is consistent with current national databases and previously published data by other groups. The number of surgeons involved in shunt operations and the origin of the patient's hydrocephalus should be described in future studies and should be controlled for in any prospective work. The choice of shunt valve was not a significant predictor of shunt failure. Most previous studies on shunts have concentrated on primary shunts, but the high rate of early shunt failure in revision cases (in both adults and children) is perhaps where future research efforts should be concentrated.

Low-Grade Glioma with Foci of Early Transformation Does Not Necessarily Require Adjuvant Therapy After Radical Surgical Resection.

BACKGROUND: Low-grade glioma (LGG) is a slow-growing tumor often found in young adults with minimal or no symptoms. As opposed to true low-grade lesions such as dysembryoplastic neuroepithelial tumors, they are associated with continuous growth and inevitable malignant transformation. METHODS: Case series of patients who have had en bloc resection of LGG with foci of anaplasia found embedded within the tumor specimen and not at margins. Patients were offered and agreed to a conservative approach avoiding adjuvant therapy. RESULTS: In the current case series, we describe a small subset of LGG that have shown foci of high-grade glioma but have shown behavior and growth tendencies similar to LGG after radical surgical resection. No patient to date has shown recurrent disease requiring adjuvant therapy. CONCLUSIONS: This case series supports the use of early aggressive surgical treatment of grade II gliomas that are premalignant. It acts as proof of concept that after radical resection, the presence of small foci of transformation embedded within grade II tumor may be treated with close radiologic surveillance rather than immediate adjuvant therapy.

Recurrent meningioma of the scalp after 13†years.

Meningiomas represent some of the most common types of tumour of the central nervous system. They are considered benign, with ∼1 in 1000 known to metastasise. We report a case of recurrent meningioma of the scalp 13 years following craniotomy to treat the primary. The recurrent tumour of the scalp was eventually excised 5 years later and the defect closed with an anterolateral thigh free flap in a joint procedure between the neurosurgeons and plastic surgeons. Histology of the scalp lesion confirmed a WHO grade I meningioma. Although a number of explanations for recurrent scalp meningiomas exist, the precise role of isolated genetic defects in scalp meningiomas is incomplete. The scalp meningioma in this case consisted of a complex karyotype suggestive of more aggressive disease. This case emphasises that cytogenetics may play a greater role in identifying more aggressive tumours than histology alone.

Intracranial hypertrophic calcification complicating neuroendoscopy. Report of three cases.

Three cases are presented in which progressive hypertrophic calcification formed following ventricular endoscopy. After a ventricular endoscopy has been performed, it has been the authors' practice to seal the burr hole with bone dust. They believe that the calcifications formed from bone dust that fell into the track left by the endoscope. This is the first time this complication has been described.

Paediatric ventriculoperitoneal shunt infection caused by Actinomyces neuii.

We present the first reported case of ventriculoperitoneal shunt infection secondary to Actinomyces neuii in a paediatric patient. Our patient was managed with temporary shunt removal, intrathecal antibiotics and a prolonged course of intravenous and then oral antibiotics. She went on to make a complete recovery. Subsequent cerebrospinal fluid analysis at 5 months post-treatment demonstrated no evidence of residual infection.

Ventriculoperitoneal shunt 30-day failure rate: a retrospective international cohort study.

BACKGROUND: With the need for transparency of surgical results, 30-day outcome measures have become increasingly important. Ventriculoperitoneal (VP) shunt failure is a substantial burden to patients and health care systems. OBJECTIVE: This study introduces the 30-day VP shunt failure rate as a possible barometer of surgical outcome and demonstrates its use in a national (United Kingdom [UK]) study and makes comparison with 2 published randomized, controlled trials (RCT). METHODS: A cohort study of all (except 1) pediatric neurosurgical centers in the UK and Ireland. All new and revision VP shunt operations were recorded for 2008 and 2009. Both newly placed and revised VP shunts were subject to Kaplan-Meier analysis, and 30-day failure rate was obtained. Data from 2 RCTs investigating new VP shunt technology were analyzed, and the 30-day failure rate was extracted for comparative purposes. RESULTS: The overall 30-day and 1-year failure rates for new shunts were 12.9% and 28.8%, respectively. The 30-day failure rate from 2 RCTs was comparable (14% and 16%, respectively). The failure rate of the subsequent revision of those new shunts was 20.7% at 30 days and 40.4% at 1 year. According to these data, shunt survival appears to be better if performed by a consultant pediatric neurosurgeon for revision surgery only. CONCLUSION: VP shunt survival in the UK is comparable to the published multicenter data investigating shunt survival. The 30-day failure rate may represent a better barometer of surgical outcome and should be used as a separate outcome measure in the design of future trials.

Does patient ethnicity affect site of craniosynostosis?

OBJECT: There are no published papers examining the role of ethnicity on suture involvement in nonsyndromic craniosynostosis. The authors sought to examine whether there is a significant difference in the epidemiological pattern of suture(s) affected between different ethnic groups attending a regional craniofacial clinic with a diagnosis of nonsyndromic craniosynostosis. METHODS: A 5-year retrospective case-notes analysis of all cases involving patients attending a regional craniofacial clinic was undertaken. Cases were coded for the patients' declared ethnicity, suture(s) affected by synostosis, and the decision whether to have surgical correction of synostosis. The chi-square test was used to determine whether there were any differences in site of suture affected between ethnic groups. RESULTS: A total of 312 cases were identified. Of these 312 cases, ethnicity data were available for 296 cases (95%). The patient population was dominated by 2 ethnic groups: white patients (222 cases) and Asian patients (56 cases). There were both more cases of complex synostosis and fewer cases of sagittal synostosis than expected in the Asian patient cohort (χ(2) = 9.217, p = 0.027). CONCLUSIONS: There is a statistically significant difference in the prevalence of the various sutures affected within the nonsyndromic craniosynostosis patient cohort when Asian patients are compared with white patients. The data from this study also suggest that nonsyndromic craniosynostosis is more prevalent in the Asian community than in the white community, although there may be inaccuracies in the estimates of the background population data. A larger-scale, multinational analysis is needed to further evaluate the relationship between ethnicity and nonsyndromic craniosynostosis.

Patients with autosomal-recessive osteopetrosis presenting with hydrocephalus and hindbrain posterior fossa crowding.

Osteopetrosis is a heterogeneous group of disorders characterized by abnormal bone sclerosis. As a result, patients often require input regarding various neurological complications. Although autosomal-recessive osteopetrosis has been associated with hydrocephalus, it has not been linked to hindbrain abnormalities. The authors present 3 cases of auto-somal-recessive osteopetrosis in patients who presented with hydrocephalus. In each of these patients, cerebrospinal fluid diversion procedures were required and hindbrain compression developed. To date, only 1 patient has needed craniocervical decompression due to symptomatic brainstem compression.