The classical supraorbital minicraniotomy to approach the areas of origin of anterior skull base meningiomas: Anatomical nuances influencing accessibility, operability, and frontal lobe retraction.

Background: The classical supraorbital minicraniotomy (cSOM) constitutes a minimally invasive alternative for the resection of anterior skull base meningiomas (ASBM). Surgical success depends strongly on optimal patient selection and surgery planning, for which a careful assessment of tumor characteristics, approach trajectory, and bony anterior skull base anatomy is required. Still, morphometrical studies searching for relevant anatomical factors with surgical relevance when intending a cSOM for ASBM resection are lacking. Methods: Bilateral cSOM was done in five formaldehyde-fixed heads toward the areas of origin of ASBM. Morphometrical data with potential relevant surgical implications were analyzed. Results: The more tangential position of the cSOM with respect to the olfactory groove (OG) led to a reduction in surgical freedom (SF) in this area compared to others (P < 0.0001). Frontal lobe retraction (FLR) was also higher when approaching the OG (P < 0.05). Olfactory nerve mobilization was higher when accessing the planum sphenoidale (PS), tuberculum sellae (TS), and anterior clinoid process (ACP) (P < 0.0001). OG depth and the slope of the sphenoid bone between the PS and TS predicted lower SF and higher frontal retraction requirements along the OG and TS, respectively (P < 0.05). In contrast, longer distances to the ACP tip predicted lower SF over this structure (P < 0.01). Conclusion: Although clinical validation is still needed, the present anatomical data suggest that assessing minicraniotomy's position/extension, OG depth, the sphenoid's slope, and distance to ACP-tip might be of particular relevance to predict FLR, maneuverability, and accessibility when considering the cSOM for ASBM resection, thus helping surgeons optimize patient selection and surgical strategy.

Moyamoya syndrome in a patient with D-2-hydroxyglutaric aciduria type II: a rare association.

PURPOSE: Several underlying conditions of moyamoya syndrome (MMS) are well established, but so far, D-2-hydroxyglutaric aciduria (D-2-HGA) has not been mentioned. We are the first to describe a case of a patient suffering from D-2-HGA developing MMS. METHODS: The co-occurrence of D-2-HGA and MMS in a patient is reported. Furthermore, we describe the neurosurgical revascularization procedure performed and report on the follow-up. RESULTS: A 7-year-old girl suffering from D-2-HGA developed two transient ischemic attacks (TIAs). Using MRI/MRA and invasive angiography MMS was diagnosed. We performed an encephalo-duro-arterio-myo-synangiosis (EDAMS) as an indirect revascularization procedure first on the right and 2 months later on the left hemisphere. We have followed her up until the age of 10. Since the second surgery, she has not suffered further TIAs and is in a better general medical condition. CONCLUSION: Even though children with D-2-HGA often suffer epileptic attacks, every new (transient) neurological deficit should be followed up by an MRI/MRA so as not to oversee a possible underlying MMS. After diagnosis, EDAMS in combination with acetylsalicylic acid (ASA) is recommended to prevent further ischemic events.

Variants of the Anterior Subtemporal Approach to the Gasserian Ganglion and Related Structures: An Anatomical Study With Relevant Implications for Keyhole Surgery.

BACKGROUND: The advantages and limitations of different craniotomy positions and approach trajectories to the gasserian ganglion (GG) and related structures using an anterior subtemporal approach have not been studied systematically. Knowledge of these features is of importance when planning keyhole anterior subtemporal (kAST) approaches to the GG to optimize access and minimize risks. METHODS: Eight formalin-fixed heads were used bilaterally to assess temporal lobe retraction (TLR), trigeminal exposure, and relevant anatomical aspects of extra- and transdural classic anterior subtemporal (CLAST) approaches compared with slightly dorsally and ventrally allocated corridors. RESULTS: TLR to the GG and foramen ovale was found to be lower via the CLAST approach (P < 0.001). Using the ventral variant, TLR to access the foramen rotundum was minimized (P < 0.001). The overall TLR was maximal using the dorsal variant (P < 0.001) owing to interposition of the arcuate eminence. An extradural CLAST approach required wide exposure of the greater petrosal nerve (GPN) and middle meningeal artery (MMA) sacrifice. Both maneuvers were spared using a transdural approach. Using CLAST, medial dissection >39 mm can enter the Parkinson triangle, jeopardizing the intracavernous internal carotid artery. The ventral variant enabled access to the anterior portion of the GG and foramen ovale without the need for MMA sacrifice or GPN dissection. CONCLUSIONS: The CLAST approach provides high versatility to approach the trigeminal plexus, minimizing TLR. However, an extradural approach jeopardizes the GPN and requires MMA sacrifice. The risk of cavernous sinus violation exists when progressing medially beyond 4 cm. The ventral variant has some advantages to access the ventral structures and avoid MMA and GPN manipulation. In contrast, the usefulness of the dorsal variant is rather limited owing to the greater TLR required.

A Rare Case of Docetaxel-Induced Hydrocephalus Presenting with Gait Disturbances Mimicking and Coexisting with Taxane-Associated Polyneuropathy: The Relevance of Differential Diagnosis, Clinical Assessment, and Response to Ventriculoperitoneal Shunt.

Docetaxel constitutes a widely used chemotherapeutic agent as a first-line treatment for several neoplastic diseases. One of the most common side effects induced by this drug is polyneuropathy, which among other symptoms can cause gait disbalance. However, in exceptional cases gait disturbances could be related to docetaxel-induced hydrocephalus, a rare event that up to the present has been overseen throughout the medical literature and should be meticulously differentiated from polyneuropathy, since its clinical features, treatment, and prognosis differ drastically. We present the case of a woman with a progressive gait disturbance that started immediately after having been treated with docetaxel for breast cancer resembling the same clinical characteristics as seen in patients affected by normal pressure hydrocephalus. The symptoms had been observed for about 2 years as having been caused only by polyneuropathy, given the high incidence of this side effect and the accompanying numbness of distal extremities. However, brain MRI evidenced a marked enlargement of the ventricular system. Brain metastases as well as carcinomatous meningitis were ruled out. After having placed a ventriculoperitoneal shunt, the patient showed a rapid, long-lasting and outstanding improvement of gait performance. We discuss the coexistence, in this case, of taxane-associated hydrocephalus and polyneuropathy and describe the clinical features, assessment and surgical outcome of docetaxel-induced hydrocephalus, since its early recognition and differentiation from the highly frequent taxane-associated polyneuropathy has relevant consequences for the management and treatment of these patients.

Spinal Intradural Intramedullary Dissemination in the Absence of Intracranial Relapse of a Previously Radically Treated Temporal Lobe Glioblastoma Multiforme.

Intracranial glioblastoma multiforme (GBM) constitutes the most frequent and unfortunately aggressive primary central nervous system malignancy. Despite the high tendency of these tumors to show local relapse within the brain after primary therapy, dissemination into the spinal axis is an infrequent event. If spinal metastases occur they are leptomeningeal in the vast majority of cases and always in the context of intracranial progressive disease. Spinal intramedullary metastases of intracranial GBM have rarely been described to date. We report the unique case of a young woman with subacute progressive paraparesis due to spinal intramedullary metastases of a temporal lobe GBM despite the remarkable absence of intracranial tumor relapse. The patient had undergone gross total resection of a left temporal GBM in contact with the ventricles and cisternal space followed by radio- and chemotherapy 13 months before. At the moment of diagnosis of spinal intramedullary metastases, there were no signs of intracranial tumor recurrence as revealed by MRI scans. Since a high level of suspicion may be needed to detect this rare evolution of intracranial GBM and other differential diagnoses must be ruled out at presentation, we discuss the important features of this case regarding clinical manifestation, diagnosis, surgery, and management. Furthermore, we mention possible factors that may have contributed to the development of these metastases in the context of intracranial remission.

Myelomeningocele - a single institute analysis of the years 2007 to 2015.

BACKGROUND: After the introduction of folate supplementation, the number of open spinal dysraphism was successfully reduced over time. In 2007, the department for pediatric neurosurgery was established in the children's hospital. Since then, newborns with myelomeningocele (MMC), the most common form of open neural tube defects (NTD), are treated here. The aim of this study is to present the concepts applied to and experiences resulting from treatment of MMC. METHODS: Records of all newborns with MMC treated surgically during the period January 2007 to August 2015 in our institution were analyzed. Children, who were previously operated in utero were excluded. The type of neural tube defect, its location, associated comorbidities, and ambulation were recorded. RESULTS: Forty-eight children (25 males, 23 females) with spinal dysraphism were included in the analysis. In nearly 90 % of the cases, the repair of the MMC was done on the day of delivery. The follow-up period ranges from 9 weeks to 8 9/12 years (loss of follow-up in 2 cases). In 19 %, the defect remained undetected during gestation and in one case, carbamazepine was taken despite pregnancy. In 36 children (75 %), we found a Chiari malformation type II (CMII) associated with myelomeningocele. 85.4 % suffered from hydrocephalus and implantation of a shunt was necessary. In cases of bladder impairment, an intermittent catheterization was the most common management (83.3 %); no bladder augmentation was required. Twelve children required orthopedic surgery. Twenty-three of 33 patients (70 %) are ambulatory w/wo orthoses and devices. The 13 children who are younger than 2 years were considered separately to assess the motor activity safely. CONCLUSIONS: Our data show that neural tube defects to this day can remain undetected despite medical care during pregnancy. The most common associated diseases with MMC are Chiari II malformations and hydrocephalus. In the seven cases of simultaneous repair of MMC with shunt implantation, no additional complications were encountered. An interdisciplinary approach was allowed in a high percentage independence and social continence.

High risk of cerebrospinal fluid leakage in surgery of a rare primary intraosseous cavernous hemangioma of the clivus showing meningeal infiltration: A case report and review of the literature.

BACKGROUND: Primary intraosseous cavernous hemangiomas (PICH) of the skull represent an infrequent bone tumor. Although some rare cases of PICHs located in the skull base have been published, to our concern only three cases have been reported in the English literature of PICHs arising within the clivus. CASE DESCRIPTION: We present the case of a patient presenting an isolated abducens paresis due to a rare PICH of the clivus showing also an unusual destruction of the inner table as well as infiltration of the dura mater. Due to this uncommon infiltrative pattern of an otherwise expected intraosseous tumor, a cerebrospinal fluid (CSF)-fistula occurred while performing a transnasal biopsy. The patient recovered successfully without need of lumbar drainage or re-surgery. Additionally, intratumoral decompression was sufficient to relief the abducens paresis. CONCLUSIONS: Our case provides new and meaningful information about clinical features as well as growth pattern of these rare clival tumors. We also discuss the importance of knowing these peculiarities before surgery in order to plan the optimal operative management as well as to avoid complications while approaching PICHs localized in such a delicate cranial region.

Revascularisation surgery and long-term follow-up in juvenile Moyamoya syndrome: a retrospective analysis.

Due to its low incidence in Western countries, physician awareness of juvenile Moyamoya disease should be improved. The benefits of revascularisation surgery have only been proven in the juvenile version of the disease. Therefore, early revascularisation may prevent irreversible ischaemic deficits and rapidly progressive mental retardation in young patients.From 1984 to 2009, a total of 19 children (mean age 8 years, range 1-18 years, female predominance 2:1, 17/19 European white patients, 2/19 Asian origin of at least one parent) were treated for juvenile Moyamoya disease by surgical revascularisation. The leading symptoms were epilepsy (17/19), followed by transient ischaemic attacks (TIA) or prolonged reversible ischaemic neurologic deficits (PRIND) (15/19) and mental retardation (11/19). Angiography showed a clear neovascularisation in the majority of patients after indirect bypasses after 6 months. The mean follow-up was 17 years and 3 months (maximum 25 years, minimum 2 years). Two patients were lost to follow-up. In accordance with the literature, ischaemic symptoms were eliminated by the revascularisation operation in 94% of our patients with a very low rate of complications, and no lasting morbidity and mortality in any of the patients.Early diagnosis and surgical treatment seem to potentiate the benefits independently of the type of revascularisation procedure.

Posthemorrhagic hydrocephalus in very low birth weight infants--a new gentle surgical technique for external ventricular drainage.

OBJECTIVE: The germinal matrix hemorrhage in premature infants is related to the immaturity of the subependymal vascular bed. Posthemorrhagic ventricular dilation (PVD) in extremely low birth weight infants (ELBI) is a medical challenge and is associated with a high risk of long-term disability. Our aim was to find a safe and gentle surgical technique for external ventricular drainage implantation for this patient population. PATIENTS AND METHODS: We implanted eight external ventricular drainages in ELBI with birth weights ranging from 479 to 884 g (24 to 27 weeks gestational age) and a grade III to IV hemorrhage, who developed a PVD soon after birth. A replacement of the drainage was required three times in one infant with a birth weight of 479 g. Due to the subcutaneous tunneling and drawing of the catheter through the sleeve, the skin contact remains slight, and thus, infections are avoided. RESULTS: There were no complications during the procedures which can also be carried out in the neonatal intensive care unit. Only in one case (479 g birth weight and severe concomitant diseases) was an infection observed, though the origin was most likely abdominal. In the same case, a temporary leakage was treated by suture. This patient died of non-neurological complications related to the patient's prematurity. Three of the five preterms were shunted over time. CONCLUSIONS: Although there is only a small number of extremely low birth weight infants with posthemorrhagic hydrocephalus who were treated with this technique, our findings suggest that this method is very safe and useful. It reduces the risk of infection and cerebrospinal fluid leaks and might be a better alternative to serial lumbar or transfontanel punctures in extremely low birth weight infants for bridging the first weeks of life.

Acute epidural hematoma with infarction of the right hemisphere in a 5-month-old child: case report with a long-term follow-up and a review of the literature.

Two hours after a fall, a 5-month-old girl was admitted to our hospital because of an extended galea hematoma and restlessness. Five hours after the trauma, a left hemiparesis developed. The child became drowsy. The hematocrit had fallen to 7.1 g/dL. Cranial computerized tomography disclosed a huge frontoparietal epidural hematoma on the right side and a hypodensity in the territories of the middle and posterior cerebral arteries. Immediately before surgery, the right pupil dilated. After evacuation of the epidural hematoma and ligation of the middle meningeal artery, the girl recovered satisfactorily. Nevertheless, magnetic resonance imaging showed a vast defect zone in the territories of the right middle and posterior cerebral arteries. Six weeks after the injury, the visual-evoked potentials were unavailable on both sides. Four years after the accident, visual assessment revealed normal acuity and stereopsis. Cognitive and neuromotor development were undisturbed and appropriate to the age.

Progressive moyamoya syndrome associated with de novo formation of the ipsilateral venous and contralateral cavernous malformations: case report.

BACKGROUND: Association of moyamoya disease with multiple vascular malformations is extremely rare. CASE PRESENTATION: A 33-year-old man, who had been affected with moyamoya syndrome with TIA during the past 3 years, was admitted to our hospital because of crescendo TIAs with numbness in his right hand and dysarthria. Magnetic resonance angiography showed apparent progression of the steno-occlusive changes bilaterally. We then planned surgical revascularization on the symptomatic side. At the time of readmission for surgery, an asymptomatic small intracerebral hemorrhage in the right frontal cortex was found, which presented on MRI as a CCM. Cerebral angiogram showed avascular lesion corresponding to the location of CCM as well as a newly formed venous malformation on the left side. One month later, STA-MCA anastomosis with pial synangiosis was performed without complications. Significant improvement in CBF on the left hemisphere was confirmed by postoperative single-photon emission CT. There were no ischemic changes on a postoperative MRI, and STA-MCA bypass was apparently patent by MRA. The TIA completely disappeared after surgery, and the patient was discharged without neurologic deficit. Persistence of the CCM and venous malformation were confirmed by T2*-weighted image and 3-dimensional contrast-enhanced gradient echo/SWI 4 months after surgery, respectively. CONCLUSION: The association of moyamoya syndrome with CCM and venous malformation is extremely rare. The coincidence of the progressive moyamoya syndrome with these newly formed vascular malformations may give a clue to the underlying mechanism of the progression of this rare entity.