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  • 1
    Article
    Article
    In: Neuroradiologie Scan, 2017, Vol.07(02)
    In: Neuroradiologie Scan, 2017, Vol.07(02), pp.117-128
    Keywords: Dandy-walker-syndrom ; Terminologie ; Diagnostik ; Therapie ; Prognose
    ISSN: 1616-9697
    E-ISSN: 1616-9700
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  • 2
    Article
    Article
    Language: German
    In: Neurochirurgie Scan, 2016, Vol.04(02), pp.129-139
    Description: Zystische Fehlbildungen der hinteren Schädelgrube (HSG) werden unter dem Begriff Dandy-Walker zusammengefasst. Synonyme hierfür sind Dandy-Walker-Syndrom (DWS), Dandy-Walker-Complex (DWC), Dandy-Walker-Spektrum, Dandy-Walker-Continuum. Hierzu zählen die klassische Dandy-Walker-Malformation (DWM), Dandy-Walker-Variante (DWV), persistierende Blakeʼs-Pouch-Zyste (BPC), Mega-Cisterna-Magna (MCM) und bei manchen Autoren die Arachnoidalzyste (AC) der HSG. Aufgrund der Variabilität der einzelnen Missbildungen sowie möglichen zusätzlichen weiteren Hirnfehlbildungen sind sowohl die Diagnose inkl. Differenzialdiagnosen oft schwierig und verwirrend als auch die Prognosen und therapeutischen Strategien unterschiedlich. Deshalb ist es wichtig, die anatomischen Unterschiede dieser Entitäten zu kennen, um die richtige Diagnose zu stellen und weitere Therapie zu planen. Mit verbesserter pränataler Diagnostik mittels Ultraschall und Amniozentese sowie fetaler MRT kann heutzutage die Diagnose früh gestellt und adäquate Therapie eingeleitet werden. Entscheidend für das weitere Outcome sind Kenntnisse über die Symptome und erfolgreiche Behandlung des Hydrozephalus. Der folgende Artikel soll helfen, Licht in die verwirrende Terminologie zu bringen, die Diagnosestellung zu erleichtern und entsprechend der zugrunde liegenden Malformation die adäquate Therapie zu wählen.
    Keywords: Pädiatrische Neurochirurgie und Hydrozephalus
    ISSN: 2195-9919
    E-ISSN: 2195-9927
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  • 3
    Language: English
    In: European Journal of Pharmacology, 15 May 2013, Vol.708(1-3), pp.95-104
    Description: We found that in mice the basal activity of monoamine oxidase B (MAO-B) in the medial prefrontal cortex (mPFC) is lower in BALB/C than in C57Bl/6J mice, whereas activity of MAO-A is similar between strains. BALB/C mice, in comparison to C57Bl/6N mice, have higher basal content of dopamine in the mPFC, in both microdialysates and tissue content. Novelty stress (open field test) elicits a further increase in the microdialysate levels of dopamine in BALB/C, but not in C57Bl/6N mice; a subsequent accumulation of extracellular 3,4-dioxyphenylacetic acid (DOPAC) reaffirms the difference in catabolic capacity of monoaminergic systems between the strains. We demonstrated that in stress-susceptible BALB/C mice the novel anxiolytic afobazole, 5 mg/kg, selectively mitigates trait anxiety; however it does not change the behavioral response in stress-resilient C57Bl/6N mice. Afobazole inhibits MAO-A in in vitro; it also lowers the microdialysate DOPAC levels in both strains (which testifies to its MAO-A inhibiting activity in vivo) and slightly suppresses dopamine release when elevated. Therefore, it is likely that the drug may mediate its anxiolytic activity via modulation of volume dopaminergic transmission at level of the mPFC.
    Keywords: Stress ; Dopamine ; Mao ; Balb/C Mice ; C57bl/6 Mice ; Anxiety ; Afobazole ; Microdialysis ; Pharmacy, Therapeutics, & Pharmacology
    ISSN: 0014-2999
    E-ISSN: 1879-0712
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  • 4
    Language: English
    In: Journal of neurosurgery. Pediatrics, March 2016, Vol.17(3), pp.285-8
    Description: The authors present the unusual case of a 4-year-old boy who had a complex history of posthemorrhagic hydrocephalus and who underwent more than 40 surgeries related to this condition. In the course of trying to treat his condition, ventriculoperitoneal, ventriculoatrial, and ventriculopleural shunts were inserted and failed. The child presented with a dysfunction of his shunt system. A ventriculopleural shunt was inserted, but within days the patient developed dyspnea as a clinical symptom of pleural effusion that required repeated thoracentesis. A bipleural drainage system was inserted, and no relevant pleural effusions developed during the follow-up period. Although the authors' experience is based on a single case, they do suggest bipleural drainage in patients with clinically relevant pleural effusions when the more common alternatives are not a good choice. Bipleural drainage might particularly be an option in children, who are prone to pleural effusion because of the smaller absorbing pleural surface. The authors reviewed the English-language literature on PubMed dating back to 1952. To their knowledge, this is the only published case in which a patient was treated with a ventriculo-bipleural shunt.
    Keywords: IVH = Intraventricular Hemorrhage ; VA = Ventriculoatrial ; VP = Ventriculoperitoneal ; Vpl = Ventriculopleural ; Hydrocephalus ; Pleural Effusion ; Ventriculopleural Shunt ; Cerebrospinal Fluid Shunts -- Adverse Effects ; Drainage -- Methods ; Hydrocephalus -- Surgery
    ISSN: 19330707
    E-ISSN: 1933-0715
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  • 5
    In: Neurosurgery, 2018, Vol.65(CN_suppl_1 Suppl 1), pp.90-90
    Description: Abstract INTRODUCTION Diffuse intrinsic pontine glioma (DIPG) are highly aggressive brain tumors with limited treatment options and dismal outcome. Brainstem biopsy to enable molecular characterization of the tumor is currently controversial due to unclear benefits. The INFORM...
    ISSN: 0148-396X
    E-ISSN: 15244040
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  • 6
    In: Pediatric Blood & Cancer, March 2018, Vol.65(3), pp.n/a-n/a
    Description: Infants with low‐grade glioma (LGG) and diencephalic syndrome have a poor outcome. The patient described here had a desmoplastic infantile astrocytoma harboring a BRAF V600E mutation. After relapse following initial standard chemotherapy treatment, he was successfully treated with the BRAF V600E inhibitor vemurafenib at the age of 3 years 11 months and 5 years 0 months. A rapid response was observed on both occasions. This illustrates the possibility of continuous oncogenic addiction and the therapeutic potential of BRAF V600E inhibitor monotherapy in LGG, even in very young severely compromised children. BRAF V600E inhibition in LGG and possible (re‐)treatment regimens are briefly discussed.
    Keywords: Braf V600e Inhibitor ; Child ; Desmoplastic Infantile Astrocytoma ; Infant ; Low‐Grade Glioma ; Vemurafenib
    ISSN: 1545-5009
    E-ISSN: 1545-5017
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  • 7
    Language: English
    In: European Journal of Paediatric Neurology, July 2017, Vol.21(4), pp.678-681
    Description: Spontaneous intracranial hypotension is a rarely diagnosed cause of headache, especially in children and adolescents. It is due to cerebrospinal fluid (CSF) leakage via spinal fistulae occurring without major trauma. An adolescent patient presented with a 3-month history of strictly postural headache. Cranial magnetic resonance imaging (MRI) showed pronounced Chiari-like prolapse of the cerebellar tonsils, narrow ventricles and enlarged cerebral veins. On spinal MRI, myelographic sequences revealed a large collection of CSF around the first sacral roots. CT myelography proved extensive spinal CSF leakage. Hence, we applied epidural patches at multiple levels. Afterwards, symptoms and radiologic findings, including Chiari-like displacement, completely resolved. A Chiari-like descent of the cerebellar tonsils alone does not secure the diagnosis of a Chiari I malformation. Especially if other findings indicate spinal CSF leakage, a systematic work-up should be initiated. In most cases, interventional techniques seal the leak successfully, resulting in a favorable outcome.
    Keywords: Intracranial Hypotension ; Csf Leak ; Headache ; Chiari Malformation ; Pediatric ; Epidural Blood Patch ; Medicine
    ISSN: 1090-3798
    E-ISSN: 1532-2130
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  • 8
    Language: English
    In: Journal of Cranio-Maxillo-Facial Surgery, July 2014, Vol.42(5), pp.503-507
    Description: Resorbable osteosynthesis is a widespread tool in craniofacial surgery, however only a limited number of studies have focused on ultrasound-assisted pinned resorbable systems in the treatment of craniosynostosis. Thirty-eight children with various types of craniosynostosis including scaphocephaly, trigonocephaly, anterior and posterior plagiocephaly were treated using the Sonic Welding resorbable osteosynthesis system. All patients were evaluated for operation time, stability of the surgical results, rate of local infections and visibility or palpability of the osteosynthesis material in the follow-up ranging from 15 to 21 month. Mean operation time was not significantly higher compared to conventional osteosynthesis material and all remodelled cranial vaults showed immediate stability. Only one patient showed signs of an inflammatory skin reaction, which recovered spontaneously. The number of palpable or visible plates, respectively, increased during the first months with a maximum at 12 months (34 (89%) plates palpable, 26 (68%) plates visible). After this time point, the number decreased continuously until the end of the follow-up period at 21 months when 3 (20%) plates were palpable, 0 (0%) plates were visible). Ultrasound-assisted pinned resorbable systems seem to be a promising tool in craniofacial surgery providing a timesaving and stable osteosynthesis. An initial swelling of the plates during the first 12 months before the complete degradation might result in a palpable and visible bulge.
    Keywords: Craniofacial Surgery ; Craniosynostosis ; Resorbable Material ; Ultrasound-Assisted Pinned Resorbable Osteosynthesis ; Dentistry
    ISSN: 1010-5182
    E-ISSN: 1878-4119
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  • 9
    Language: English
    In: Journal of Cranio-Maxillo-Facial Surgery, June 2015, Vol.43(5), pp.593-598
    Description: There is still disagreement regarding the intracranial volumes of patients with metopic synostosis compared with healthy patients. This study aimed to compare the intracranial volume of children with metopic synostosis before and after surgery to an age- and sex-matched control cohort using three-dimensional (3D) photogrammetry. Eighteen boys with metopic synostosis were operated on using standardized fronto-orbital advancement. Frontal, posterior and total intracranial volumes were measured exactly 1 day pre-operatively and 10 days post-operatively, using 3D photogrammetry. To establish an age- and sex-matched control group, the 3D photogrammetric data of 634 healthy boys between the ages of 3 and 13 months were analyzed. Mean age at surgery was 9 months (SD 1.7). Prior to surgery, boys with metopic synostosis showed significantly reduced frontal and total intracranial volumes compared with the reference group, but similar posterior volumes. After surgery, frontal and total intracranial volumes did not differ statistically from the control group. As children with metopic synostosis showed significantly smaller frontal and total intracranial volumes compared with an age- and sex-matched control group, corrective surgery should aim to achieve volume expansion. Furthermore, 3D photogrammetry provides a valuable alternative to CT scans in the measurement of intracranial volume in children with metopic synostosis, which significantly reduces the amount of radiation exposure to the growing brain.
    Keywords: 3d Photogrammetry ; Fronto-Orbital Advancement ; Intracranial Volume ; Metopic Synostosis ; Dentistry
    ISSN: 1010-5182
    E-ISSN: 1878-4119
    Source: ScienceDirect Journals (Elsevier)
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  • 10
    In: Journal of Craniofacial Surgery, 2016, Vol.27(7), pp.e671-e673
    Description: ABSTRACT: Regarded singly, both Sturge-Weber syndrome and trigonocephaly are rare congenital disorders. The cardinal features of Sturge-Weber syndrome are facial cutaneous capillary malformation (port-wine stain), leptomeningeal angiomatosis, and glaucoma. Premature closure of the metopic suture results in trigonocephaly. However, to the best of our knowledge, the diagnosis of a combination of both Sturge-Weber syndrome and trigonocephaly has not as yet been reported. This brief clinical study thus presents a patient with the unusual findings of a Sturge-Weber syndrome and simultaneous trigonocephaly induced by premature metopic synostosis. Thus, the rare combination of a port-wine stain involving the first division of the trigeminal nerve with the diagnosis of a craniosynostosis justifies the indication of a prophylactic magnetic resonance imaging acquisition before craniofacial surgeries, in order to prevent seizures and stroke-like episodes triggered by the surgical intervention.
    Keywords: Abnormalities, Multiple ; Craniosynostoses -- Diagnosis ; Sturge-Weber Syndrome -- Diagnosis;
    ISSN: 1049-2275
    ISSN: 15363732
    E-ISSN: 15363732
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