Kazuaki Shimoji

Surgical considerations in fourth ventricular ependymoma with the transcerebellomedullary fissure approach in focus.

IntroductionWithin the existing consensus for the best management of pediatric infratentorial ependymomas (PIE), surgery is the most important stage, where complete removal should be the perfect aim, before complementing it with chemo- or radiotherapy. That, however, remains a challenge even for the most skillful surgeons because of the vicinity of important brainstem and cranial nerve structures involved and is particularly difficult in lateral extensions.Materials and methodsThe paper analyzes the current trends of PIE treatment with emphasis on resection difficulties created by lateral extensions. Anatomical analysis and clinical application of the cerebellomedullary fissure dissection has created specific approaches, providing safe route to the lateral recess and cerebellopontine area by dividing safely tenia and tonsils and biventer lobes retraction.Discussion and conclusionBilateral and unilateral approaches have been developed. This approach prevents the damage of transvermian access and the resulting cerebellar mutism in some cases. Indications, technique and benefits of transcerebellomedullary fissure types of approaches are discussed.

Unique radiological appearance of a microcystic meningioma.

Summary A 35-year-old female presented with partial complex seizures. Computed tomography (CT) showed a slightly high density mass over the right frontal convexity, with heterogeneous contrast enhancement. T1-weighted magnetic resonance (MR) imaging showed the tumour as a hypo-intense lesion, with faint reticular enhancement after intravenous injection of gadolinium-diethylenetriaminepenta-acetic acid. The tumour was totally removed. The specimen was extremely soft and moist. The histological diagnosis was microcystic meningioma. The tumour cells were composed of typical meningothelial cells and stellate cells. The degenerative character of the tumour may be reflected in the poor enhancement on CT and MR imaging. This faint enhancement effect may be a neuro-imaging characteristic indication of this rare microcystic variant of meningioma.

Endoscopic and Microsurgical Treatment of Sylvian Fissure Arachnoid Cysts—Clinical and Radiological Outcome

OBJECTIVE A Sylvian fissure arachnoid cyst (SAC) is a well-recognized location for an intracranial arachnoid cyst in the pediatric population. For those cysts, which can rupture and be accompanied by a subdural hygroma or hematoma, several treatment modalities have been reported. We report clinical and radiological outcome of fenestration of these cysts by either endoscopy or microsurgery. METHODS A retrospective review of the database of operative procedures revealed 24 procedures (20 endoscopic and 4 microsurgical procedures) to fenestrate a SAC at university hospitals in Berlin, Germany and Tokyo, Japan. RESULTS With the applied technique, a reduction of SAC volume of more than 10% was achieved in 83.3% of all patients. The median volume of SACs (n = 24) was significantly reduced from 83.5 mL (range 21-509 mL) preoperatively to 45.5 mL (range 8.4-261 mL; P < 0.01) after 3.5 months and to 29.0 mL (range 0-266 mL; P < 0.01) after 15 months. In children (n = 8) with a ruptured SAC the combined extraaxial volume of a SAC and accompanying hygroma/hematoma was reduced from 166 mL (range 111-291 mL) before surgery to 127 mL (range 87-329 mL) after 2 months and to 77 mL (range 25-140 mL; P < 0.05) after 11 months. Acute clinical symptoms were generally resolved postoperatively; headaches were resolved or improved in 75%. A significant association of resolution or improvement of headaches and volume reduction was demonstrated. CONCLUSIONS The study demonstrated efficacy in a predominantly endoscopically treated patient cohort with Sylvian fissure arachnoid cysts, as indicated by improvement of clinical symptoms and diminished radiological SAC volume after treatment.

Posterior Cranial Vault Distraction Osteogenesis with Barrel Stave Osteotomy in theTreatment of Craniosynostosis

Twenty years have passed since distraction osteogenesis was introduced into the field of craniomaxillofacial surgery, with distraction osteogenesis gradually consolidating its position for midface advancement in syndromic craniosynostosis. On the other hand, no consensus has been reached regarding its adaptation to calvarial bone. We reported that distraction osteogenesis was useful in posterior cranial vault expansion, and subsequently, similar reports have been successively observed worldwide. In posterior cranial vault distraction, intracranial capacity could be greatly expanded due to its simultaneous expansion with the scalp, with little risk of relapse because new bone is regenerated in the distraction gap. The possibility was suggested that the standard of first carrying out fronto-orbital advancement (FOA) for brachycephaly observed in syndromic craniosynostosis will greatly change posterior cranial vault distraction.

Sturge-Weber syndrome with spontaneous intracerebral hemorrhage in childhood

A girl aged 2 years 10 months suddenly went into a deep coma and demonstrated left hemiplegia. At birth, she had exhibited a left-sided facial port-wine stain typical of Sturge-Weber syndrome (SWS) and involving the V1 and V2 distributions of the trigeminal nerve. Computed tomography showed a right thalamic hemorrhage with acute hydrocephalus. Magnetic resonance imaging with Gd enhancement 8 months before the hemorrhage had shown a patent superior sagittal sinus (SSS) and deep venous system. Magnetic resonance imaging and MR angiography studies 2 months before the hemorrhage had revealed obstruction of the SSS and right internal cerebral vein (ICV). Given that a digital subtraction angiography study obtained after the hemorrhage did not show the SSS or right ICV, the authors assumed that impaired drainage was present in the deep venous system at that stage. The authors speculated that the patients venous drainage pattern underwent compensatory changes because of the occluded SSS and deep venous collectors, shifting outflow through other cortical venous channels to nonoccluded dural sinuses. Sudden congestion (nearly total to total obstruction) of the ICV may have caused the thalamic hemorrhage in this case, which is the first reported instance of pediatric SWS with intracerebral hemorrhage and no other vascular lesion. Findings suggested that the appearance of major venous sinus occlusion in a child with SWS could be a warning sign of hemorrhage.

Use of targeted next-generation sequencing for molecular diagnosis of craniosynostosis: Identification of a novel de novo mutation of EFNB1.

Craniofrontonasal syndrome (CFNS; MIM#304110) is characterized by asymmetric facial features with hypertelorism and a broad bifid nose due to synostosis of the coronal suture. CFNS shows a unique X‐linked inheritance pattern (most affected patients are female and obligate male carriers exhibit a mild manifestation or no typical features at all) associated with the ephrin‐B1 gene (EFNB1) located in the Xq13.1 region. In this study, we performed targeted, massively parallel sequencing using a next‐generation sequencer, and identified a novel EFNB1 mutation, c.270_271delCA, in a Japanese female patient with craniosynostosis. Because subsequent Sanger sequencing identified no mutation in either parent, this mutation was determined to be de novo in origin. After obtaining molecular diagnosis, a retrospective clinical evaluation confirmed the clinical diagnosis of CFNS in this patient. Comprehensive molecular diagnosis using a next‐generation sequencer would be beneficial for early diagnosis of the patients with undiagnosed craniosynostosis.

Conservative Treatment of Large Aplasia Cutis Congenita of the Scalp With Bone Defect With Basic Fibroblast Growth Factor Application

The object of this report is to share our experience of conservative management of giant aplasia cutis congenita (ACC) of the scalp with the topical application of basic fibroblast growth factor (bFGF). Complete epithelialization of the 9 × 8 cm sized defect was achieved in 33 weeks. Careful conservative management could eliminate the requirement of surgery for giant ACC defects of the scalp with bone defects and should be tried if surgery is thought to be risky or has consecutive morbidity. Topical bFGF application seems to accelerate healing, also providing a better epithelium for later reconstructive treatments and its usage could be standardized in the future.

Panventriculomegaly with a wide foramen of Magendie and large cisterna magna: clinical, radiological, and genetic analysis.

The purpose of this study is to provide the first clinical, radiological, and genetic analysis of panventriculomegaly (PaVM) defined by a wide foramen of Magendie and large cisterna magna.

Role of Artificial Cerebrospinal Fluid as Perfusate in Neuroendoscopic Surgery: A Basic Investigation

Neuroendoscopic surgery is distinct from usual craniotomy as it is performed in water. We have previously reported that the use of artificial cerebrospinal fluid (CSF) as perfusate in third ventriculostomy is more efficacious in minimizing severe host reaction than normal saline or lactated Ringers solution. In this study, we investigated the effects of different perfusion solutions in human cultured astrocytes. We cultured human astrocytes in growth medium. Then each of them was further cultured for 6 h in artificial CSF, lactated Ringers solution, or normal saline. Using DNA microarray, RNAs were extracted from each of the cells and were comprehensively analyzed to identify differences in patterns of gene manifestation. Compared to the use of artificial CSF, in cases where lactated Ringers solution or normal saline was used, there was little difference in the pattern of gene manifestation, but there was an increase in gene manifestation related to apoptosis and inflammatory reaction. For neuroendoscopic surgery, the use of artificial CSF as a perfusate is considered effective in maintaining brain homeostasis compared to the use of normal saline or lactated Ringers solution.

Shunt Techniques—Cystoperitoneal Shunt

Abstract In treating arachnoid cysts (AC), cystoperitoneal (CP) shunting is reported to produce good outcomes in terms of symptom improvement and cyst size reduction. However, CP shunting has several drawbacks, including shunt dependency, in addition to the complications associated with all shunts, such as shunt infections and malfunctions. In a survey on facilities’ preferred method for a middle fossa AC, microscopic fenestration was chosen by 66.6%, endoscopic fenestration by 28.8%, and CP shunting just by 6.6%. However, no consensus has been reached regarding the relative merits and demerits of fenestration and cystoperitoneal shunting, as the treatment outcome varies according to each study. Here we discuss the advantages and complications of CP shunting, and especially the complication unique to shunts, the shunt dependency syndrome .