'What's in a name', a systematic review of the pterional craniotomy for aneurysm surgery and its many modifications with a proposal for simplified nomenclature.

BACKGROUND: The pterional or frontosphenotemporal craniotomy has stood the test of time and continues to be a commonly used method of managing a variety of neurosurgical pathology. Already described in the beginning of the twentieth century and perfected by Yasargil in the 1970s, it has seen many modifications. These modifications have been a normal evolution for most neurosurgeons, tailoring the craniotomy to the patients' specific anatomy and pathology. Nonetheless, an abundance of variations have appeared in the literature. METHODS: A search strategy was devised according to the 2020 Preferred Reporting Items of Systematic Reviews and Meta-Analyses (PRISMA) statement. To identify articles investigating the variations in the pterional approach, the following search terms were applied: (pterional OR minipterional OR supraorbital) AND (approach OR craniotomy OR technique). RESULTS: In total, 3552 articles were screened with 74 articles being read in full with 47 articles being included for review. Each article was examined according the name of the technique, temporalis dissection technique, craniotomy technique and approach. CONCLUSION: This systematic review gives an overview of the different techniques and modifications to the pterional craniotomy since it was initially described. We advocate for the use of a more standardised nomenclature that focuses on the target zone to simplify the management approach to supratentorial aneurysms.

Nonprogrammable Shunts for Communicating Hydrocephalus and Three-Dimensional Volumetry: A Retrospective Analysis.

OBJECTIVE: Although the use of different types of valves has been extensively studied in shunt surgery for communicating hydrocephalus (cHC), a consensus about the valve type remains absent. The objective of this study is to evaluate our results with the primary placement of nonprogrammable valves (NPVs) for this indication. METHODS: We retrospectively analyzed all first NPVs implanted between 2014 and 2020 for cHC. We studied the revision rate, clinical outcome described by modified Rankin Scale (mRS), and radiologic evolution using Evans Index (EI) and ventricular volumes three-dimensional semi-automatic segmentation (vv-3DSAS). RESULTS: Forty-one patients were shunted for posthemorrhagic (61%), posttraumatic (24.4%), and tumoral (14.6%) hydrocephalus. Mean age was 65 years (range, 25-89 years). Overall, 59 procedures were performed including 18 revision surgeries in 12 patients (29.3%). The underlying reasons for first shunt revision were valve type related (valve dysfunction, overdrainage, and underdrainage) and nonvalve type related (malpositioning, infection, and shunt migration). The shunt-related revision rate was 17.1%. Twenty-eight patients (68.3%) had an mRS score improvement of 1 or more points. We found a good correlation between ventricle volumes (VV) and EI and a significant reduction in VV measured by EI and vv-3DSAS was observed. However, the mRS improvement was not correlated with a reduction in ventricle volumes. CONCLUSIONS: Overall, our results in terms of shunt revisions as well as clinical and radiologic evolution are comparable to the literature for NPV. vv-3DSAS can be used and could be useful to detect small changes in VV in patients with cHC.

Randomized Trial Comparing Burr Hole Craniostomy, Minicraniotomy, and Twist Drill Craniostomy for Treatment of Chronic Subdural Hematoma.

BACKGROUND: The mainstay of treatment for symptomatic or large chronic subdural hematoma (CSDH) is surgery, but controversy still exists regarding the best surgical technique. Three different techniques are commonly used: burr hole craniostomy (BHC), minicraniotomy (MC), and twist drill craniostomy (TDC). OBJECTIVE: To determine which surgical technique for drainage of CSDH offers best results. METHODS: We set up a multicenter prospective randomized trial (Comparison of Chronic Subdural Hematoma Treatment [COMPACT] trial) comparing BHC, MC, and TDC for the surgical treatment of CSDH. The primary end point was reoperation rate, and secondary end points included complication rates and clinical outcome. Patients were considered to have good outcome when they did not undergo reoperation, suffered no surgical or medical complication, and had no related mortality. Clinical outcome was also evaluated by evolution of the Markwalder score and the modified Rankin score. RESULTS: Two-hundred forty-five patients were included in the final analysis: 79 BHC, 84 MC, and 82 TDC. Mean duration of surgery was shorter for TDC than for BHC and MC ( P < .001). Reoperation rate was 7.6% for BHC, 13.1% for MC, and 19.5% for TDC ( P = .07). This trend toward better results for BHC was not statistically significant in logistic regression analysis. The proportion of patients with good outcome was 78.5% for BHC group, 76.2% for MC, and 69.5% for TDC ( P = .4). Evolution of the Markwalder score and modified Rankin score were not significantly different between treatment groups. CONCLUSION: All 3 techniques are effective at treating patients with CSDH with eventual 6-month outcome being similar. Although not reaching statistical significance in our study, BHC offers the lowest recurrence rate combined with manageable complication rate.

The surgical management of intraoperative intracranial internal carotid artery injury in open skull base surgery-a systematic review.

Intraoperative internal carotid artery (ICA) injury during open skull base surgery is a catastrophic complication. Multiple techniques and management strategies have been reported for endoscopic skull base surgery; however, the literature on managing this complication in open skull base surgery is limited. To perform a systematic review and give an overview of the different techniques described to manage this complication intraoperatively, a systematic review was conducted in PubMed, Ovid Medline, Ovid Embase and Scopus for literature published until July 2021. Titles and abstracts were screened. Studies meeting prespecified inclusion criteria were reviewed in full. PRISMA guidelines were strictly adhered to. Out of 4492 articles, only 12 articles could be included, reflecting an underreporting of open skull base ICA injuries. Multiple techniques can be used depending on the location and size of the injury as well as the surgeon's experience. Described techniques include the following: a primary repair via a micro-suture or nonpenetrating clips; wrapping or plugging; coating; occlusion of the parent artery with or without a bypass; packing with further endovascular management. A treatment algorithm is proposed.

Cumulative surgical morbidity in patients with multiple cerebellar and medullary hemangioblastomas.

OBJECTIVE: Patients affected with von Hippel-Lindau disease often develop multiple hemangioblastomas in the cerebellum and spinal cord. Timing of surgical intervention is difficult and depends largely on the anticipated surgical morbidity. However, data regarding surgical outcome after multiple cerebellar and medullary surgeries are scarce. Our objective was to evaluate cumulative surgical morbidity in patients operated on multiple cerebellar and medullary hemangioblastomas and to deduce recommendations for treatment. METHODS: We performed a retrospective analysis for a consecutive cohort of von Hippel-Lindau patients with surgical treatment of at least two cerebellar and/or medullary hemangioblastomas. Pre- and postoperative functional grades were reviewed in patients' files and compared by Modified Ranking Scale (cerebellar surgeries) or by Modified McCormick Score (medullary surgeries). RESULTS: Thirty-six patients were surgically treated for at least two cerebellar hemangioblastomas (12 patients), at least two medullary hemangioblastomas (19 patients) or at least two hemangioblastomas in both locations (5 patients). Fourthy-eight cerebellar and 80 medullary procedures were performed in total. On average, multiple cerebellar surgeries caused no clinical deterioration, whereas multiple medullary surgeries led to a slight cumulative deterioration of postoperative functional grades. The severity of this deterioration did not correlate to the number of performed medullary surgeries. CONCLUSION: Resection of multiple cerebellar hemangioblastomas is not associated with cumulative morbidity. Although there is a certain cumulative surgical morbidity caused by medullary surgeries, its extent does not increase with the number of performed surgeries. Microsurgical removal of asymptomatic tumors with radiographic progression can also be considered for patients with multiple tumors and previous surgeries.

Predictors of dopamine agonist resistance in prolactinoma patients.

BACKGROUND: Surgical resection of prolactinomas resistant to dopamine agonists is frequently incomplete due to fibrotic changes of the tumour under pharmacological therapy. In order to identify a subgroup of patients who may benefit from early surgery, we thought to investigate possible predictive factors of pharmacological resistance of prolactinomas to dopamine agonists. METHODS: We retrospectively analyzed a database of a Belgian tertiary reference center for patients with pituitary tumours from 2014 to 2016. The groups of interest were patients with dopamine agonist responsive and resistant prolactinomas. The possible predictive factors, including MRI findings, endocrinological parameters, response of tumour and patient factors for dopamine agonist resistance were investigated. RESULTS: We included 69 patients of whom 52 were women (75,4%) and 17 were men (24,6%). Rate of dopamine agonist resistance was 15.9%. We identified four significant predictors of dopamine agonist resistance: male gender, a large tumour volume, prolonged time to prolactin normalization and presence of a cystic, hemorrhagic and/or necrotic component. In addition, symptoms due to mass effect, high baseline prolactin level and a high contrast capture on MRI are factors that can be taken into consideration. CONCLUSION: We identified predictive factors for pharmacological resistance and developed a scoring system for patient specific prediction of resistance to dopamine agonists. This scoring system may have impact on the timing and decision of surgery in prolactinoma patients after further prospective evaluation.

A focused 35-minute whole body MRI screening protocol for patients with von Hippel-Lindau disease.

BACKGROUND: Von Hippel-Lindau (VHL) disease is an autosomal dominantly inherited tumor syndrome. Affected patients develop central nervous system hemangioblastomas and abdominal tumors, among other lesions. Patients undergo an annual clinical screening program including separate magnetic resonance imaging (MRI) of the brain, whole spine and abdomen. Consequently, patients are repeatedly subjected to time-consuming and expensive MRI scans, performed with cumulative Gadolinium injections. We report our experience with a 35-min whole body MRI screening protocol, specifically designed for detection of VHL-associated lesions. METHODS: We designed an MRI protocol dedicated to the typical characteristics of VHL-associated lesions in different imaging sequences, within the time frame of 35 min. Blank imaging of the abdomen is carried out first, followed by abdominal sequences with Gadolinium contrast. Next, the full spine is examined, followed by imaging of the brain. A single dose of contrast used for abdominal imaging is sufficient for further highlighting of spine- and brain lesions, thus limiting the Gadolinium dosage. We used 1.5 Tesla equipment, dealing with fewer artifacts compared to a 3 Tesla system for spine- and abdominal imaging, while preserving acceptable quality for central nervous system images. In addition, imaging on a 1.5 Tesla scanner is slightly faster. RESULTS: From January 2016 to November 2018, we performed 38 whole body screening MRIs in 18 VHL patients; looking for the most common types of VHL lesions in the abdomen, spine, and brain, both for new lesions and follow-up. The one-step approach MRI examinations lead to 6 surgical interventions for clinically significant or symptomatic hemangioblastomas in the brain and spine. One renal cell carcinoma was treated with radiofrequency ablation. In comparison with previous conventional MRI scans of the same patients, all lesions were visible with the focused protocol. CONCLUSIONS: Annual screening in VHL disease can be done in a rapid, safe and sensitive way by using a dedicated whole body MRI protocol; saving MRI examination time and limiting Gadolinium dose.

Epilepsy Surgery in the First 3 Years of Life: Predictors of Seizure Freedom and Cognitive Development.

BACKGROUND: Although the majority of children undergoing epilepsy surgery are younger than 3 yr at epilepsy manifestation, only few actually receive surgical treatment in early childhood. Past studies have, however, suggested that earlier intervention may correlate with superior developmental outcomes. OBJECTIVE: To identify predictors for long-term seizure freedom and cognitive development following epilepsy surgery in the first 3 yr of life and determine the appropriate timing for surgical treatment in this age group. METHODS: We retrospectively analyzed the data of 48 consecutive children aged 1.1 ± 0.7 yr at surgery. RESULTS: Final surgeries comprised 52% hemispherotomies, 13% multilobar, and 35% intralobar resections. Etiology included cortical malformations in 71%, peri- or postnatal ischemic lesions in 13%, and benign tumor or tuberous sclerosis in 8% each. At last follow-up (median 4.3, range 1-14.3 yr), 60% of children remained seizure-free: 38% had discontinued antiepileptic drugs. Intralobar lesionectomy resulted more often in seizure control than multilobar or hemispheric surgery. Postsurgical seizure freedom was determined by the completeness of resection. Early postsurgical seizures were key markers of seizure recurrence. Presurgical adaptive and cognitive developmental status was impaired in 89% children. Longer epilepsy duration and larger lesion extent were detrimental to presurgical development, which, in turn, determined the postsurgical developmental outcome. CONCLUSION: Our study demonstrates that epilepsy surgery in very young children is safe as well as efficient regarding long-term seizure freedom and antiepileptic drug cessation in selected candidates. Longer epilepsy duration is the only modifiable predictor of impaired adaptive and cognitive development, thus supporting early surgical intervention.

Polyglobulia in patients with hemangioblastomas is related to tumor size but not to serum erythropoietin.

BACKGROUND: Hemangioblastomas are associated with elevated hemoglobin (Hb) levels (polyglobulia), which is associated with a higher risk for cerebral stroke, cardiac infarction and pulmonary embolism. The pathomechanism of polyglobulia remains unclear and different theories have been postulated. Among those are elevated serum erythropoietin (EPO) levels caused by secretion of the tumor or associated tumor cyst. METHODS: To elucidate the pathomechanism, we systematically investigated the relation between polyglobulia, serum EPO level, size of the solid tumor and associated cyst in hemangioblastomas. We prospectively evaluated hemoglobin and EPO levels in a series of 33 consecutive patients operated on hemangioblastomas in our center. We measured the size of the solid tumor and associated cyst in magnetic resonance imaging. Statistical evaluations were performed using the Fisher's exact test and student's t-test. RESULTS: As a result five patients had elevated hemoglobin levels. Only one of these had an elevated serum EPO level. Of 26 patients with normal hemoglobin levels, 4 patients had elevated EPO levels.Patients with low or normal hemoglobin levels (84%) had an average tumor size of 0.8 cm3, which differed significantly from patients with elevated hemoglobin levels (16%), who had an average solid tumor size of 8.0 cm3 (p < 0.05). We did not observe a significant correlation between EPO levels or polyglobulia and associated cysts. CONCLUSIONS: We therefore conclude that in contrast to previous case reports and interpretations, our data show no correlation between polyglobulia and EPO levels or associated cysts in patients with hemangioblastomas. In fact, it is the size of the solid tumor that correlates with polyglobulia.The study was retrospectively registered in the German Clinical Trial Registry on 10 July 2014; Trial registration: DRKS00006310.

Posterior approach for anteriorly located cervical spinal cord hemangioblastomas: technical note.

The surgical management of anteriorly located spinal cord hemangioblastomas remains a challenge. Different approaches have been published, of which the anterior approach seems to be the most obvious and commonly used. A posterior approach might be more suitable in certain patients, especially in cases of cystic hemangioblastomas. The authors present 3 cases of anterior spinal hemangioblastomas, which were all resected via a posterior approach. The authors discuss the rationale for choosing this approach and explain the technique in detail.

Central nervous system gadolinium accumulation in patients undergoing periodical contrast MRI screening for hereditary tumor syndromes.

BACKGROUND: Patients with hereditary tumor syndromes undergo periodical magnetic resonance imaging (MRI) screening with Gadolinium contrast. Gadolinium accumulation has recently been described in the central nervous system after repeated administrations. The prevalence and rate of accumulation in different subgroups of patients are unknown. Neither are the mechanism nor clinical impact. This may cause uncertainty about the screening. To explore the prevalence and rate of Gadolinium accumulation in different subgroups, we retrospectively analyzed MRIs of patients with von Hippel-Lindau disease (VHL) and Tuberous Sclerosis Complex (TSC). METHODS: We determined the prevalence and rate of accumulation in the dentate nucleus and globus pallidus on unenhanced T1-weighted MRI from VHL and TSC patients. We compared the signal intensities of these regions to the signal intensity of the pons. We evaluated the impact of number of MRIs, kidney function and liver function on Gadolinium accumulation. RESULTS: Twenty eight VHL patients and 24 TSC patients were included. The prevalence of accumulation in the dentate nucleus and globus pallidus increased linearly according to number of Gadolinium enhanced MRIs and was higher in the VHL group (100%). A significant linear correlation between number of MRIs and increased signal intensity was observed in the VHL group. CONCLUSIONS: Gadolinium accumulation occurs in almost all patients undergoing contrast MRI screening after >5 MRIs. We advocate a screening protocol for patients with hereditary tumor syndromes that minimizes the Gadolinium dose. This can be accomplished by using a single administration to simultaneously screen for brain, spine and/or abdominal lesions, using an MRI protocol focused on either VHL- or TSC-specific lesions. Higher prevalence and rate of accumulation in VHL patients may be explained by the typical vascular leakage accompanying central nervous system hemangioblastomas.

Frontal Lobe Epilepsy Surgery in Childhood and Adolescence: Predictors of Long-Term Seizure Freedom, Overall Cognitive and Adaptive Functioning.

BACKGROUND: Although frontal lobe resections account for one-third of intralobar resections in pediatric epilepsy surgery, there is a dearth of information regarding long-term seizure freedom, overall cognitive and adaptive functioning. OBJECTIVE: To identify outcome predictors and define the appropriate timing for surgery. METHODS: We retrospectively analyzed the data of 75 consecutive patients aged 10.0 ± 4.9 yr at surgery that had an 8.1 yr mean follow-up. RESULTS: Etiology comprised focal cortical dysplasia (FCD) in 71% and benign tumors in 16% cases. All patients but one had a magnetic resonance imaging-visible lesion. At last follow-up, 63% patients remained seizure-free and 37% had discontinued antiepileptic drugs. Presurgical predictors of seizure freedom were a shorter epilepsy duration, strictly regional epileptic discharges in electroencephalography (EEG), and an epileptogenic zone and/or lesion distant from eloquent cortex. Postsurgical predictors were the completeness of resection and the lack of early postoperative seizures or epileptic discharges in EEG. Higher presurgical overall cognitive and adaptive functioning was related to later epilepsy onset and to a sublobar epileptogenic zone and/or lesion. Following surgery, scores remained stable in the majority of patients. Postsurgical gains were determined by higher presurgical performance and tumors vs FCD. CONCLUSION: Our findings highlight the favorable long-term outcomes following frontal lobe epilepsy surgery in childhood and adolescence and underline the importance of early surgical intervention in selected candidates. Early postsurgical relapses and epileptic discharges in EEG constitute key markers of treatment failure and should prompt timely reevaluation. Postsurgical overall cognitive and adaptive functioning is stable in most patients, whereas those with benign tumors have higher chances of improvement.

Posterior cortex epilepsy surgery in childhood and adolescence: Predictors of long-term seizure outcome.

OBJECTIVE: We aimed to investigate the long-term seizure outcome of children and adolescents who were undergoing epilepsy surgery in the parietooccipital cortex and determine their predictive factors. METHODS: We retrospectively analyzed the data of 50 consecutive patients aged 11.1 (mean) ± 5.1 (standard deviation) years at surgery. All patients but one had a magnetic resonance imaging (MRI)-visible lesion. Resections were parietal in 40%, occipital in 32%, and parietooccipital in 28% cases; 24% patients additionally underwent a resection of the posterior border of the temporal lobe. Etiology included focal cortical dysplasia in 44%, benign tumors (dysembryoplastic neuroepithelial tumor, ganglioglioma, angiocentric glioma, and pilocystic astrocytoma) in 32%, peri- or postnatal ischemic lesions in 16%, and tuberous sclerosis in 8% cases. RESULTS: At last follow-up (mean 8 years, range 1.5-18 years), 60% patients remained seizure-free (Engel class I): 30% had discontinued and 20% had reduced antiepileptic drugs. Most seizure recurrences (71%) occurred within the first 6 months, and only three patients presented with seizures ≥2 years after surgery. Independent predictors of seizure recurrence included left-sided as well as parietal epileptogenic zones and resections. Longer epilepsy duration to surgery was identified as the only modifiable independent predictor of seizure recurrence. SIGNIFICANCE: Our study demonstrates that posterior cortex epilepsy surgery is highly effective in terms of lasting seizure control and antiepileptic drug cessation in selected pediatric candidates. Most importantly, our data supports the early consideration of surgical intervention in children and adolescents with refractory posterior cortex epilepsy.

Lesional cerebellar epilepsy: a review of the evidence.

Classical teaching in epileptology localizes the origins of focal seizures solely in the cerebral cortex, with only inhibitory effects attributed to subcortical structures. However, electrophysiological and neuroimaging studies over the last decades now provide evidence for an initiation of epileptic seizures within subcortical structures. Intrinsic epileptogenicity of hypothalamic hamartoma has already been established in recognition of subcortical epilepsy, whereas a seizure-generating impact of dysplastic cerebellar lesions remains to be clarified. Herein, we examine the supportive evidence and clinical presentation of cerebellar seizures and review therapy options.

Early Vasospasm after Aneurysmal Subarachnoid Hemorrhage Predicts the Occurrence and Severity of Symptomatic Vasospasm and Delayed Cerebral Ischemia.

BACKGROUND: Cerebral vasospasm usually develops several days after subarachnoid hemorrhage (SAH) and is generally acknowledged as a strong outcome predictor. In contrast, much less is known about the nature and eventual consequences of early angiographic vasospasm (EAVS) seen on admission digital subtraction angiography (DSA). Therefore, we aimed at identifying the risk factors and clinical impact of EAVS after SAH. METHODS: Five hundred and thirty-one SAH patients with admission DSA performed within 72 h after the bleeding event were selected from a comprehensive database containing all consecutive SAH patients treated at our institution between January 2005 and December 2012. Predictors of EAVS, as well as associations between EAVS and delayed vasospasm-related complications, and unfavorable outcome (defined as modified Rankin scale >3) were evaluated in univariate and multivariate analyses. RESULTS: EAVS was seen on 60 DSAs (11.3%) and was independently correlated with delayed symptomatic vasospasm requiring intra-arterial spasmolysis (OR 5.24, p < 0.0001), angioplasty (OR 2.56, p = 0.015) and repetitive endovascular treatment (OR 4.71, p < 0.0001). EAVS also increased the risk for multiple versus single territorial infarction on the follow-up CT scan(s) (OR 2.04, p = 0.047) and independently predicted unfavorable outcome (OR 2.93, p = 0.008). The presence of radiographic signs suspicious for fibromuscular dysplasia were independently associated with the occurrence of EAVS (OR 2.98, p = 0.026) and the need for repetitive endovascular vasospasm treatment (OR 3.95, p = 0.019). CONCLUSIONS: In view of the strong correlation with delayed symptomatic vasospasm and its ischemic complications, EAVS can be considered an alerting signal for severe symptomatic vasospasm. Therefore, more attention should be paid to the presence of EAVS on admission DSA.

Intracerebral Hematoma Due to Aneurysm Rupture: Are There Risk Factors Beyond Aneurysm Location?

BACKGROUND: Along with subarachnoid hemorrhage (SAH), a ruptured aneurysm may also cause an intracerebral hematoma (ICH), which negatively impacts the functional outcome of SAH. OBJECTIVE: To identify independent risk factors of aneurysmal ICH. METHODS: Six hundred thirty-two consecutive patients with aneurysmal SAH treated at our institution from January 2005 to December 2012 were eligible for this study. Demographic parameters and preexisting comorbidities of patients, as well as various clinical and radiographic characteristics of SAH were correlated with the incidence and volume of aneurysmal ICH. RESULTS: One hundred fifty-five patients (25%) had ICH on initial computed tomography with a mean volume of 26.7 mL (±26.8 mL). Occurrence and volume of ICH were associated with the location (distal anterior or middle cerebral artery >proximal anterior cerebral or internal carotid artery >posterior circulation, P < .001/P < .001) and size (>12 mm, P = .026/P < .001) of the ruptured aneurysm. Vascular risk factors independently increased the risk of ICH as well (arterial hypertension: odds ratio [OR] = 1.62, P = .032; diabetes mellitus: OR = 3.06, P = .009), while the use of aspirin (P = .037) correlated with the volume of ICH. The predictors of ICH were included into a risk score (0-9 points) that strongly predicted the occurrence of ICH (P = .01). Poor functional outcome after SAH was independently associated with the occurrence of ICH (P = .003, OR = 2.77) and its volume (P = .001, OR = 1.07 per-mL-increase). CONCLUSION: Aneurysmal ICH is strongly associated with poorer functional outcome and seems to be predictable even before the bleeding event. The proposed risk factors for aneurysmal ICH require further validation and may be considered for treatment decisions regarding unruptured intracranial aneurysms. ABBREVIATIONS: ACA, anterior cerebral arteryDHC, decompressive hemicraniectomyEVD, external ventricular drainageICA, internal carotid arteryICH, intracerebral hematomaMCA, middle cerebral arterymRS, modified Rankin scalePC, posterior circulationSAH, subarachnoid hemorrhageSIRS, systemic inflammatory response syndromeTCD, transcranial Doppler sonography.

Photophobia in a blind eye after removal of a progressive orbital optic glioma with denervation.

UNLABELLED: Optic glioma (OPG) accounts for 4-8% of all brain tumors in children. En-block removal of intraorbital tumor is recommended in cases with disfiguring exophthalmos and impaired vision. Surgical resection of intraorbital optic nerve (ON) poses the risks of permanent ptosis and globe atrophy. We present here the case of a 4-year-old boy with exophthalmos and near blindness due to an intraorbital OPG. Despite chemotherapy he showed progressive exophthalmos and vision loss. Bony orbital decompression with ON transection temporally reduced his exophthalmos. OPG resection was required later for recurrence of his exophthalmos secondary to tumor progression. Post operatively, he had preserved oculomotor nerve functions but developed globe ischemia. Unusually, his ischemic globe caused him to have pain and severe photophobia, which later lead to enucleation. Photophobia has been reported in blind patients. Animal models and MRI functional imaging showed activation of trigeminal pathway during photophobia in completely transected ON. However, the exact neuro-ophthalmology pathway requires further study. CONCLUSION: This is the first described case of photophobia after excision of OPG with ON denervation. Photophobia can be a serious side effect that significantly lowers the patient's quality of life.

Decompressive hemicraniectomy in the management of acute stroke in children with Moyamoya disease: the difficult balance between the spontaneous postoperative revascularization process and the need for cranial reconstruction.

BACKGROUND AND IMPORTANCE: The decompressive hemicraniectomy (DCH) after malignant middle cerebral artery infarction in children is a rare procedure, and the indication is discussed as being controversial. Etiological Moyamoya disease has some additional challenges concerning the therapeutic strategy that have not mentioned in the recent literature, as it is dependent on special pathophysiology. CLINICAL PRESENTATION: We report a case of a four-year-old patient with a decompressive hemicraniectomy after malignant middle cerebral artery infarction on the right hemisphere based on a Moyamoya syndrome with proximal MCA occlusions on both sides. After the decompression there was a good restitution of the hemiparesis, aphasia, and consciousness loss on admission. The bone flap replacement is usually done after three month in our department to ensure brain swelling has subsided. In this patient the cranioplasty was not arranged because of the development of collateral vessels to the right motor region through the craniotomy defect, to protect the supply of the eloquent cortex. CONCLUSION: We conclude that the indication of DCH and postoperative treatment should be discussed individually, especially when neovascularisation developments can occur like in Moyamoya disease. An important point is the right timing for bone flap replacement, which should be directly after cerebral edema has subsided prior to the evolution of collaterals through the craniotomy defect. Additionally, leaving the opportunity for neovascularization through smaller defects has to be taken into account.