小松 文成

Objective  This study compared intraoperative findings with preoperative computed tomography angiography (CTA) and computational fluid dynamics (CFD) analysis of perianeurysmal findings for the indication of possible vessel wall thinning. Materials and Methods  Participants comprised 38 patients with unruptured middle cerebral artery aneurysms treated by surgical clipping at our hospital between May 2020 and April 2021. We defined parent artery radiation sign (PARS) as the presence of each of the following three findings in CFD analysis based on preoperative CTA: (1) impingement of the stream line on the outer parent vessel wall of the aneurysm; (2) radiation of wall shear stress vectors outwards from the same site; and (3) increased wall pressure compared with the surrounding area. CFD analysis showing PARS was compared with intraoperative findings. Results  In all nine cases with PARS, no morphological abnormalities were found in the same area on CTA. However, intraoperative findings showed thinning of the parent artery wall in one of the nine cases and formation of a very small mass in three cases, differing from CTA findings. All nine patients underwent additional clipping and/or wrapping and coating at the site of PARS. Conclusion  Detecting thinning of the vessel wall or the presence of a microaneurysm may be difficult in endovascular therapy, which is based on the visualization of the vessel lumen. CFD analysis suggests the necessity of confirming findings for the vessel wall around an aneurysm by direct manipulation, as the presence of PARS may indicate partial thinning of the vessel wall or formation of a microaneurysm.

Objective  Trigeminal neuralgia (TN) is a neurological disorder that often presents as severe toothache. The majority of TN patients visit dental clinics first, so TN represents a potential pitfall for dental practitioners. This report describes the development of a trigeminal neuralgia questionnaire (TNQ), assessing 10 characteristics of TN, to assist dentists in screening for TN in dental clinics, and evaluates the effectiveness of TNQ. Materials and Methods  Fifty-three patients who visited the TN outpatient department in our institute and completed the TNQ were included in this study. All patients were examined by two neurosurgeons and neuroimaging was performed. Statistical Analysis  Patients were classified into a TN group and a non-TN group. TNQ score was retrospectively compared between groups. Furthermore, history and characteristics of TN were investigated in the TN group to clarify the status of the reference situation. Results  Thirty-seven cases were assigned to the TN group, and 16 cases to the non-TN group. Mean TNQ score was 8.3 in the TN group and 6.6 in the non-TN group. Setting a TNQ cutoff score of 7 offered 91% sensitivity and 56% specificity for TN. Investigation of the history of the present illness indicated that 39.2% of TN cases were improperly triaged and referred from initial dental clinics, and interdisciplinary practice was insufficient. Conclusion  TNQ offers a reliable, convenient method to triage TN patients, and may assist dentists in screening for TN. Multidisciplinary practice is necessary for total management of TN and the TNQ is expected to connect dentists and TN specialists.

OBJECTIVES: Superficial temporal artery (STA) to middle cerebral artery (MCA) bypass surgery is a common treatment for preventing cerebral ischemia in patients with intracranial artery stenosis. The aim of this study was to analyze the surgical outcomes of the STA-MCA bypass procedure, particularly with regard to the invasiveness of targeted bypass (TB) with preoperative planning using Amira® software. METHODS: Consecutive patients with single STA-MCA bypass performed by a single neurosurgeon from January 2019 to May 2022 were included. The clinical parameters of seven TB patients were compared with those of 11 patients treated with the conventional method (CM). RESULTS: Compared with CM patients, TB using Amira® software patients had a shorter scalp incision (median [interquartile range]=11.2 [9.7-12.7] cm vs. 16.9 [16.0-17.7] cm, respectively; p=0.004], smaller craniotomy size (11.8 [11.5-14.4] cm2 vs. 20.9 [17.1-22.2] cm2, respectively; p=0.01], shorter surgery duration (201 [195-218] min vs. 277 [229-310] min, respectively; p=0.003], and less intraoperative bleeding (10 [10-20] g vs. 23 [20-50] g, respectively; p=0.033]. However, there were no differences in surgical complications between the two groups. CONCLUSIONS: Detailed preoperative evaluation using Amira® software can reduce the invasiveness of the STA-MCA bypass procedure.

BACKGROUND: The trigeminocerebellar artery is a standard variant originating from the basilar artery and can be an infrequent cause of trigeminal neuralgia. METHOD: Total endoscopic microvascular decompression (eMVD) was performed using a 0-degree endoscope through a retro sigmoid keyhole. Multiple points of neurovascular conflict enhanced by indocyanine green angiography were identified, and the root entry zone was decompressed. The patient had an improvement in facial pain with no complications. CONCLUSION: Complete eMVD for a nerve-penetrating artery is a practical, minimally invasive, uncomplicated technique that improves visualization and patient comfort.

Unruptured cerebral aneurysms (UCAs) are usually asymptomatic and detected incidentally by intracranial examinations. The coexistence of neurovascular compression syndrome (NVCS) and UCAs has not been well described. The aim of this study was to clarify the characteristics of UCAs with the NVCS. A total of 103 cases that underwent microvascular decompression (MVD) for trigeminal neuralgia (TN) or hemifacial spasm (HFS) were assigned to the NVCS group. The prevalence of UCAs in the NVCS group was compared retrospectively to that in 110 control cases (a control group) by neuro-imaging. Overall treatment courses for NVCS and UCAs were investigated in the NVCS group. Sixteen (15.3%; TN 11 cases, HFS 5 cases) of 103 MVD cases had 19 UCAs, a significantly higher prevalence than the 3.6% in the control group. Binomial logistic regression showed that NVCS is a significant factor for predicting the presence of UCAs, with an odds ratio of 4.80. In the NVCS group, 18 UCAs (94.7%) were supratentorial aneurysms, and 17 UCAs (89.5%) were less than 5 mm in size. Nine UCAs were surgically treated with clipping or coiling. Of the surgical cases, 2 UCAs were treated before MVD for NVCS, whereas the other 7 UCAs were treated after MVD. No aneurysms ruptured during the treatment course. The NVCS occurred with UCAs at a significantly higher rate than in the control group. Most UCAs with the NVCS were supratentorial, small aneurysms that did not affect the surgical treatment of NVCS.

BACKGROUND: Endoscopic microvascular decompression (eMVD) provides excellent visualization and minimally invasive surgical treatment of trigeminal neuralgia (TN). The transposition technique is desirable for long-term outcomes of TN. METHOD: A two-step transposition technique is performed during eMVD for TN due to the arch-shaped superior cerebellar artery (SCA). First, the cerebellomesencephalic segment of the SCA is pulled out using oxycellulose balls. Second, the retracted lateral pontomesencephalic segment of the SCA is detached from the trigeminal nerve and is transposed for fixation at the tentorium cerebelli. CONCLUSION: The two-step transposition technique offers simple, reliable decompression for TN due to the arch-shaped SCA.

Introduction  Preoperative illustration is a part of an important exercise to study the configuration, direction, and presence of any perforations, and is the weakest point in the wall of the cerebral aneurysm. The same illustration is used to study the surrounding brain structures to decide the best and safe surgical approach prior to any surgical procedure. With the evolution of the aneurysm wall study and study of flow dynamic within the involved artery and its aneurysm wall using computational fluid dynamics (CFD), a better surgical plan can be formulated to improve the flow dynamics. As one of the clinical applications of CFD, we propose a study using a composite image that combines preoperative illustration and CFD, which is traditionally widely used in neurosurgery. Methods and Materials  We study the use of illustrations of the unruptured cerebral aneurysm of internal carotid-posterior communicating (ICPC) artery and anterior communicating artery (AcomA) treated at our hospital. The combinations of both preoperative illustrations and CFD images by using "ipad Pro" were used. Result and Conclusion  Medical illustration in the preoperative study of unruptured cerebral aneurysm with combinations of CFD and surrounding brain structures is helpful to decide the surgical approaches and successful surgical treatments.

BACKGROUND: Mechanical stimulation may lead to internal carotid artery (ICA) dissection and aneurysm. CASE DESCRIPTION: We encountered a rare case of ICA dissection and aneurysm with prolonged styloid process (SP) fracture. A 37-year-old sales worker presented with right-sided amaurosis fugax. After admission to a nearby optical clinic, he was admitted to our hospital. Computed tomography angiography (CTA) and digital subtraction angiography showed dissection and apparent aneurysmal change in the right cervical portion of the ICA. CTA also showed elongated SPs, so we diagnosed Eagle's syndrome, and fracture of the right-side process. After 2 weeks of antiplatelet therapy, the aneurysm enlarged and dissection remained, so we treated the patient with coil embolization and stenting. CONCLUSION: We encountered a rare case of ICA dissection and aneurysm with Eagle's syndrome. Endovascular treatment was performed because the SP was fractured.

BACKGROUND: Careful evaluation of the preoperative imaging for extracranial-intracranial bypass performed for conditions like intracranial stenosis and Moya disease is important. The traditional use of 2D imaging has a significant limitation for neurosurgeons, primarily to determine the optimal location of the recipient artery for performing the surgical bypass. Therefore, many neurosurgeons use 3D angiograms more frequently to overcome these shortcomings. MATERIALS AND METHODS: We performed the preoperative evaluation of the possibility of performing an anastomosis between the superficial temporal artery and the middle cerebral artery (STA-MCA) bypass by synthesizing images of computerized tomography (CT), magnetic resonance imaging (MRI), and digital subtraction angiography (DSA) using Amira® of 3D visualization analysis software (Thermo Scientific Co.). RESULTS: The 3D images prepared before surgery using this software showed good agreement with the intraoperative findings. CONCLUSION: Preoperative image processing using tools like Amira provide optimal information for good planning and communication for performing STA-MCA bypass and may become a helpful tool.

BACKGROUND: A repair strategy for venous bleeding from the superior petrosal vein (SPV) is essential during endoscopic microvascular decompression. METHOD: Sliced oxycellulose seats are rounded off, making balls around 10 mm in diameter. When venous bleeding arises from the SPV, the first oxycellulose ball is placed just behind the SPV in the surgical view. A second ball is then applied in front of the SPV. The SPV is thus immediately and entirely covered by oxycellulose, and hemostasis is safely achieved with the preservation of the SPV. CONCLUSION: This oxycellulose ball technique offers simple, reliable control of venous bleeding from the SPV.

BACKGROUND: Microvascular decompression (MVD) for glossopharyngeal neuralgia (GPN) is associated with high complication and incomplete cure rates because of its poor ability to visualize neurovascular conflicts. METHOD: Fully endoscopic MVD for GPN was carried out through a retrosigmoid keyhole approach. Neurovascular conflicts were clearly demonstrated with a loop of the posterior inferior cerebellar artery (PICA) under a 30° endoscopic view, and no significant cerebellar retraction was observed. The loop of the PICA was safely decompressed and the perforators were preserved while offering an excellent operative view. CONCLUSION: Endoscopic MVD is a reliable and minimally invasive method for GPN.

Introduction We previously reported centripetal propagation of vasoconstriction at the time of thunderclap headache remission in patients with reversible cerebral vasoconstriction syndrome. Here we examine the clinical significance of centripetal propagation of vasoconstriction. Methods Participants comprised 48 patients who underwent magnetic resonance angiography within 72 h of reversible cerebral vasoconstriction syndrome onset and within 48 h of thunderclap headache remission. Results In 24 of the 48 patients (50%), centripetal propagation of vasoconstriction occurred on magnetic resonance angiography at the time of thunderclap headache remission. The interval from first to last thunderclap headache in patients with centripetal propagation of vasoconstriction (14 ± 10 days) was significantly longer than that of patients without centripetal propagation of vasoconstriction (4 ± 2 days). In the patients with centripetal propagation of vasoconstriction at the time of thunderclap headache remission, the incidence of another cerebral lesion (38%, 9 of 24 cases) was significantly higher than in patients without centripetal propagation of vasoconstriction (0%). From findings of sequential magnetic resonance angiography before and after thunderclap headache remission, we observed tendencies in which centripetal propagation of vasoconstriction gradually progressed after the onset of reversible cerebral vasoconstriction syndrome and peaked at the time of thunderclap headache remission. The progress of centripetal propagation of vasoconstriction concluded with thunderclap headache remission. Conclusions Centripetal propagation of vasoconstriction has clinical significance as an indicator of the severity of reversible cerebral vasoconstriction syndrome. The presence of centripetal propagation of vasoconstriction is associated with an increased risk of brain lesions and a longer interval from first to last thunderclap headache. Moreover, repeat magnetic resonance angiography to assess centripetal propagation of vasoconstriction during the time from onset to thunderclap headache remission can help diagnose reversible cerebral vasoconstriction syndrome.

The authors' initial experience with the endoscopic extradural supraorbital approach to the temporal pole and adjacent area is reported. Fully endoscopic surgery using the extradural space via a supraorbital keyhole was performed for tumors in or around the temporal pole, including temporal pole cavernous angioma, sphenoid ridge meningioma, and cavernous sinus pituitary adenoma, mainly using 4-mm, 0° and 30° endoscopes and single-shaft instruments. After making a supraorbital keyhole, a 4-mm, 30° endoscope was advanced into the extradural space of the anterior cranial fossa during lifting of the dura mater. Following identification of the sphenoid ridge, orbital roof, and anterior clinoid process, the bone lateral to the orbital roof was drilled off until the dura mater of the anterior aspect of the temporal lobe was exposed. The dura mater of the temporal lobe was incised and opened, exposing the temporal pole under a 4-mm, 0° endoscope. Tumors in or around the temporal pole were safely removed under a superb view through the extradural corridor. The endoscopic extradural supraorbital approach was technically feasible and safe. The anterior trajectory to the temporal pole using the extradural space under endoscopy provided excellent visibility, allowing minimally invasive surgery. Further surgical experience and development of specialized instruments would promote this approach as an alternative surgical option.

OBJECTIVE This study attempted to determine whether a previous minor leak correlated with the occurrence of symptomatic delayed cerebral ischemia (sDCI). METHODS The authors retrospectively evaluated sDCI-related clinical features and findings from MRI, including T1-weighted imaging (T1WI)-FLAIR mismatch at the time of admission, in 151 patients admitted with subarachnoid hemorrhage (SAH) within 48 hours of ictus. RESULTS The overall incidence of sDCI was 23% (35 of 151 patients). In all subjects, multivariate analysis revealed that World Federation of Neurosurgical Societies Grades II-V, age 70 years or older, presence of rebleeding after admission, a previous minor leak before the major SAH attack as diagnosed by T1WI-FLAIR mismatch, acute infarction on diffusion-weighted imaging, and CT SAH score were significantly associated with occurrence of sDCI. In patients with no previous minor leak before major SAH as diagnosed by T1WI-FLAIR mismatch, the incidence of sDCI was only 7% (7 of 97 patients). CONCLUSIONS A previous minor leak before major SAH as diagnosed by T1WI-FLAIR mismatch represents an important sDCI-related factor. When the analysis was restricted to patients with true acute SAH without a previous minor leak diagnosed by T1WI-FLAIR mismatch, the incidence of sDCI was extremely low.

BACKGROUND: Microvascular decompression (MVD) of hemifacial spasm (HFS) associated with the vertebral artery (VA) shows higher rates of incomplete cure and complications compared to non-VA-related HFS. METHOD: Purely endoscopic MVD for VA-associated HFS via a retrosigmoid keyhole was performed. Neurovascular conflicts by a directly offending artery and VA around the root exit zone of the facial nerve were clearly demonstrated under 30° endoscopic view without significant cerebellar retraction. The VA and directly offending artery were safely transposed with preservation of perforators under excellent view. CONCLUSION: Endoscopic MVD offers reliable decompression for VA-associated HFS with minimal invasiveness.

BACKGROUND: Skull base reconstruction is an essential technique for repairing cerebrospinal fluid (CSF) leakage. A reliable method for middle cranial fossa (MCF) reconstruction with minimal invasiveness has not been reported. An initial case of endoscopic MCF reconstruction with a subtemporal keyhole is described. CASE DESCRIPTION: A 57-year-old man developed severe meningitis and was diagnosed with spontaneous CSF leakage from bone defects on the tegmen tympani. Endoscopic MCF reconstruction with a subtemporal keyhole was carried out. Three skin incisions, including 1 subtemporal incision for a subtemporal keyhole and 2 temporal line incisions on the superior temporal line, were made, and a 0-degree endoscope was introduced into the subcutaneous space. The deep temporal fascia (DTF) was bluntly dissected and separated from the superficial temporal fascia and the temporal muscle, and the DTF was incised to shape a pedicled flap under endoscopic view. Blood supply to the pedicled DTF flap was confirmed with indocyanine green angiography. A subtemporal keyhole was then made, and a 30-degree endoscope was used to explore the extradural space of the MCF floor, visualizing the bone defects on the tegmen tympani. The vascularized DTF flap passed easily through the subtemporal keyhole and adequately overlaid the bone defects. The patient's postoperative course was uneventful, and the CSF leakage disappeared without mastication problems. CONCLUSIONS: This purely endoscopic technique using a vascularized DTF flap provided reliable MCF reconstruction through a subtemporal keyhole. This technique is also expected to be applicable for MCF reconstruction after subtemporal keyhole surgery for skull base tumors.

Background Endoscopic microvascular decompression (MVD) offers reliable identification of neurovascular conflicts under superb illumination, and it provides minimally invasive surgery for trigeminal neuralgia and hemifacial spasm. Transposition techniques have been reported as a decompression method to prevent adhesion and granuloma formation around decompression sites, providing better surgical outcomes. The feasibility and effects of transposition under endoscopic MVD were evaluated. Material and Methods Fully endoscopic MVD was performed using 4-mm 0- and 30-degree endoscopes. The endoscope was fixed with a pneumatic holding system, and a bimanual technique using single-shaft instruments was performed. Transposition was performed with Teflon felt string and fibrin glue. Surgical results were evaluated using the scoring system proposed by Kondo et al. Results The endoscope was introduced via a retrosigmoid keyhole. The 0-degree endoscope was advanced through the lateral aspect of the cerebellar tentorial surface to the trigeminal nerve in cases of trigeminal neuralgia and through the petrosal surface of the cerebellum to the facial nerve in cases of hemifacial spasm. Neurovascular conflicts and perforators from the offending artery were clearly demonstrated under the 30-degree endoscopic view, and transposition of the offending artery was safely performed with preservation of perforators. Clinical symptoms improved without permanent complications. Conclusion Endoscopic MVD with the transposition technique is feasible. Superb endoscopic views demonstrate perforators arising from the offending artery behind the corner, allowing damage to perforators to be avoided during the transposition technique. Endoscopic MVD using the transposition technique is expected to offer excellent surgical results.

An 89-year-old male presented with cerebrospinal fluid (CSF) rhinorrhea associated with head trauma sustained as a pedestrian in a traffic accident. Computed tomography (CT) showed pneumocephalus and multiple cranial bone fractures, including the clivus. Although the CSF rhinorrhea was treated conservatively for a week, clinical symptoms did not improve and surgical repair was performed. Preoperative thin-sliced bone CT and steady-state magnetic resonance images revealed a bone defect at the middle clivus and a collection of CSF fluid from the clival fistula in the sphenoid sinus. Endoscopic endonasal reconstruction was performed, and the 3-mm diameter dural tear and bone defect at the middle clivus were well visualized. The fistula was repaired using a pedicled nasoseptal mucosal flap. The CSF rhinorrhea completely disappeared as a result of the endoscopic endonasal surgery. The present report describes a rare case of CSF rhinorrhea caused by a traumatic clival fracture and surgical management by endoscopic endonasal surgery.

We describe a supra-diaphragmatic ectopic pituitary adenoma that was safely removed using the extended endoscopic endonasal approach, and discuss the value of three-dimensional (3D) endoscopy and intra-operative magnetic resonance imaging (MRI) to this type of procedure. A 61-year-old-man with bitemporal hemianopsia was referred to our hospital, where MRI revealed an enhanced suprasellar tumor compressing the optic chiasma. The tumor extended on the planum sphenoidale and partially encased the right internal carotid artery. An endocrinological assessment indicated normal pituitary function. The extended endoscopic endonasal approach was taken using a 3D endoscope in the intraoperative MRI suite. The tumor was located above the diaphragma sellae and separated from the normal pituitary gland. The pathological findings indicated non-functioning pituitary adenoma and thus the tumor was diagnosed as a supra-diaphragmatic ectopic pituitary adenoma. Intra-operative MRI provided useful information to minimize dural opening and the supra-diaphragmatic ectopic pituitary adenoma was removed from the complex neurovascular structure via the extended endoscopic endonasal approach under 3D endoscopic guidance in the intra-operative suite. Safe and effective removal of a supra-diaphragmatic ectopic pituitary adenoma was accomplished via the extended endoscopic endonasal approach with visual information provided by 3D endoscopy and intra-operative MRI.

BACKGROUND: Identification of the internal carotid artery (ICA) is essential for successful endoscopic endonasal cavernous sinus tumor surgery. This study aimed to develop a method for identifying the ICA in cavernous sinus tumors at the superior part of the cavernous sinus. METHODS: Ten fresh cadavers were studied with a 4-mm 0° and 30° endoscope to identify surgical landmarks of the ICA in the cavernous sinus. Clinical cases of cavernous sinus tumors were surgically treated using an endoscopic transpterygoid approach. RESULTS: Anatomical study indicated the ICA at the superior part of the cavernous sinus can be identified using three steps: 1) exposure of the optic nerve sheath by drilling the optic canal; 2) identification of the proximal orifice of the optic nerve sheath at the transition of the optic nerve sheath and dura mater of the tuberculum sellae; and 3) identification of the clinoid segment of the ICA at the distal dural ring just below the proximal orifice of the optic nerve sheath. Although the ICA was encased and transposed by tumors in preliminary surgical cases, the clinoid segment of the ICA was safely exposed at the superior part of the cavernous sinus using this method. CONCLUSIONS: Dural structures around the cavernous sinus are key to identifying the ICA at the superior part of the cavernous sinus. This method is expected to reduce the risk of ICA injury during endoscopic endonasal surgery for cavernous sinus tumors.

The lateral limit of endoscopic endonasal surgery has yet to be defined. The aim of this study was to investigate the lateral limit of endoscopic endonasal surgery at the level of the sphenoid sinus. Access from the sphenoid sinus to the middle cranial fossa through the cavernous sinus triangles was evaluated by cadaver dissection. Anatomical analysis demonstrated that the medial temporal dura mater was exposed through the anterior area of the clinoidal triangle, anteromedial triangle, and superior area of the anterolateral triangle, indicating potential corridors to the middle cranial fossa. This study suggests that the cavernous sinus triangles are applicable in selected cases to manage middle cranial fossa lesions by endoscopic endonasal surgery.

The lateral limit of endoscopic endonasal surgery has yet to be defined. The aim of this study was to investigate the lateral limit of endoscopic endonasal surgery at the level of the sphenoid sinus. Access from the sphenoid sinus to the middle cranial fossa through the cavernous sinus triangles was evaluated by cadaver dissection. Anatomical analysis demonstrated that the medial temporal dura mater was exposed through the anterior area of the clinoidal triangle, anteromedial triangle, and superior area of the anterolateral triangle, indicating potential corridors to the middle cranial fossa. This study suggests that the cavernous sinus triangles are applicable in selected cases to manage middle cranial fossa lesions by endoscopic endonasal surgery.

OBJECTIVE: Endoscopy has provided a less invasive approach to skull base surgery, mainly through endonasal routes, but has been limited in its applications due to potential complications. The aims of this study were to evaluate the feasibility of the purely endoscopic extradural transcranial approach to lateral and central skull base through a subtemporal keyhole and to better understand potential distortions of the related anatomy via endoscopy. METHODS: Ten fresh cadaver heads were studied with 4-mm 0° and 30° endoscopes to develop the surgical approach and to identify surgical landmarks. RESULTS: The endoscopic extradural subtemporal approach was divided into 3 sections after exposure of the extradural space in the middle cranial fossa: 1) exposure of the lateral wall of the cavernous sinus and the preauricular infratemporal fossa; 2) anterior petrosectomy and posterior cranial fossa exploration; and 3) unroofing of the tympanic cavity and exposure of the facial nerve. This keyhole endoscopic technique clearly visualized anatomical landmarks of the lateral and central skull base via an extradural subtemporal route. CONCLUSIONS: The endoscopic extradural subtemporal approach was feasible. This approach could display a wide range of lateral and central skull base structures with minimal invasiveness. The use of extradural space would be key to performing safe and effective endoscopic skull base surgery.

Minimally invasive surgery to the posterolateral craniovertebral junction (CVJ) has not been sufficiently described. The aims of this study were to evaluate the feasibility of an endoscopic far-lateral approach to the posterolateral craniocervical junction and to better understand the related anatomy under distorted endoscopic view. Ten fresh cadavers were studied with 4-mm 0° and 30° endoscopes to develop the surgical approach and to identify surgical landmarks. After making a 3-cm straight incision behind the mastoid process, the superior oblique and rectus capitis posterior major muscles were partially exposed. An endoscope was then introduced and the two muscles were followed inferiorly until the posterior arch of the atlas appeared. The two muscles were removed to create ample working space without violating the posterior atlanto-occipital membrane. The vertebral artery was identified by the landmark of the posterior arch of the atlas, and the atlanto-occipital joint and foramen magnum were exposed. In addition to suboccipital craniectomy, transcondylar, supracondylar, and paracondylar extension by drilling were applicable through the narrow corridor under superb visualization. The intradural neurovascular structures from the acousticofacial bundle to the dorsal root of C2, anterolateral space of the foramen magnum, cerebellomedullary fissure, and fourth ventricle were clearly demonstrated. This endoscopic far-lateral approach offers excellent exposure of surgical landmarks around the posterolateral CVJ with minimal invasiveness. Endoscopic soft tissue dissection is key to creating the surgical corridor. This approach could offer an alternative to the conventional far-lateral approach in selected cases.

OBJECT: The course of the trigeminal nerve straddles multiple fossae and is known to be very complex. Comprehensive anatomical knowledge and skull base techniques are required for surgical management of trigeminal schwannomas. The aims of this study were to become familiar with the endoscopic anatomy of the trigeminal nerve and to develop a minimally invasive surgical strategy for the treatment of trigeminal schwannomas. METHODS: Ten fresh cadavers were studied using 5 endoscopic approaches with the aid of 4-mm 0° and 30° endoscopes to identify surgical landmarks associated with the trigeminal nerve. The endoscopic approaches included 3 transcranial keyhole approaches (the extradural supraorbital, extradural subtemporal, and retrosigmoid approaches), and 2 endonasal approaches (the transpterygoid and the transmaxillary transpterygoid approaches). RESULTS: The trajectories of the extradural supraorbital, transpterygoid, and extradural subtemporal approaches corresponded with the course of the first, second, and third divisions of the trigeminal nerve, respectively. The 3 approaches demonstrated each division in intra- and extracranial spaces, as well as the Meckel cave in the middle cranial fossa. The interdural space at the lateral wall of the cavernous sinus was exposed by the extradural supraorbital and subtemporal approaches. The extradural subtemporal approach with anterior petrosectomy and the retrosigmoid approach visualized the trigeminal sensory root and its neighboring neurovascular structures in the posterior cranial fossa. The transmaxillary transpterygoid approach revealed the course of the third division in the infratemporal fossa. CONCLUSIONS: The 5 endoscopic approaches effectively followed the course of the trigeminal nerve with minimal invasiveness. These approaches could provide alternative options for the management of trigeminal schwannoma.

The telovelar approach allows reliable access to the fourth ventricle and avoids the splitting of the vermis and its associated "posterior vermal split syndrome." Our objective was to describe the endoscopic topographical anatomy of the telovelum approach to the fourth ventricle as accessed by the cerebellomedullary corridor. A series of 20 fresh and fixed injected anatomical specimens were used. The endoscopic equipment consisted of rigid endoscopes with different lens angles, while the extradural step required the use of the microscope and/or the exoscope. All the anatomical landmarks and relationships within the fourth ventricle and the cerebellomedullary fissure were identified by means of the endoscopic microscope/exoscope-assisted telovelar approach. In conclusion, we showed that the endoscope is a valid tool to gain an anatomic understanding of the fourth ventricle reached by means of the telovelar approach.

BACKGROUND: In the past, sporadic demonstrations of the existence of a subarachnoid subdiaphragmatic cistern have been published. The aim of this study was to evaluate the anatomical characteristics of the subdiaphragmatic cistern of the pituitary gland. METHODS: After a complete review of the literature published on the topic, we report anatomical observations of the subdiaphragmatic cistern and its relationship to the pituitary gland and to the chiasmatic cistern. Ten cadaveric heads were studied using different techniques and surgical methods (plastination, plastic casts of the subarachnoid spaces, microscopic and transsphenoidal endoscopic approaches). Moreover, 3-T magnetic resonance images of ten healthy volunteers were analyzed to investigate the presence and anatomical variability of the subdiaphragmatic cistern. RESULTS: By means of our qualitative radioanatomic study, we found that the roof of the subdiaphragmatic cistern is formed by the diaphragma sellae, the floor by the superior face of the pituitary gland, the lateral walls by the arachnoidea extending laterally through the medial walls of the cavernous sinus, and the medial walls by the infundibular stem. The subdiaphragmatic cistern communicates by means of the ostium of the diaphragm with the chiasmatic cistern. CONCLUSION: We confirmed the existence of the subdiaphragmatic cistern. The overused term "suprasellar cistern" refers more to a complex of cisterns, formed by the subdiaphragmatic cistern, below the diaphragma sella, and by the chiasmatic cistern, above it, in direct communication with the lamina terminalis and carotid cisterns.

BACKGROUND: Reconstruction of the skull base is essential to prevent postoperative leakage of cerebrospinal fluid (CSF). However, a reliable method of reconstructing the middle cranial fossa via a subtemporal keyhole is not available. OBJECTIVE: To determine whether less invasive reconstruction of the middle cranial fossa under endoscopic guidance with a pedicled deep temporal fascia approach via a subtemporal keyhole is feasible and useful. METHODS: The middle cranial fossa in 4 fresh cadaver heads was reconstructed with a 4-mm 0° rigid endoscope. RESULTS: A subtemporal skin incision (subtemporal incision) was followed by 2 small skin incisions (temporal line incisions) made on the superior temporal line. The endoscope was inserted through the temporal line incisions, and then the deep temporal fascia was separated from the superficial temporal fascia and temporal muscle under endoscopic view. A pedicled flap was harvested from the subtemporal incision and applied to the middle cranial fossa after subtemporal keyhole craniotomy. The pedicled deep temporal fascial flap was flexible, long, and large enough to overlay skull base defects. CONCLUSION: This purely endoscopic technique using a pedicled deep temporal fascial flap provided reliable reconstruction of the middle cranial fossa through a subtemporal keyhole. This technique would also be applicable in preventing CSF leakage or treating traumatic, acquired nontraumatic, or congenital encephalocele in the middle cranial fossa.

We report herein a case of lower clival chondroid chordoma, focusing on the surgical procedure of endoscopic endonasal surgery. A 36-year-old woman presented with progressive headache, right shoulder pain, and right hypoglossal nerve palsy. Computed tomography (CT) and magnetic resonance (MR) imaging revealed an extradural tumor located in the lower clivus, including the anterior aspect of the foramen magnum, deeply compressing the medulla and upper cervical spinal cord. Endoscopic endonasal surgery was performed via two nostrils. Since the basiocciput was destroyed by the tumor, removal of the tumor allowed identification of the middle clivus superiorly, the anterior arch of the atlas inferiorly, and anteromedial parts of occipital condyles bilaterally without drilling the basiocciput. The tumor was removed except for laterally and inferiorly extended lesions. Pathological diagnosis was chondroid chordoma. Postoperative course was uneventful, and the patient was discharged without further neurological deterioration. Endonasal endoscopic surgery provided safe and reliable tumor resection for a lower clival lesion. We believe that this minimally invasive procedure should be considered as an alternative to traditional surgical treatment.

BACKGROUND: Anterior clinoidectomy is an essential preliminary step for parasellar and pericavernous sinus surgery. Endoscopy is a widely accepted modality for neurosurgical strategies and is becoming more important in treating conditions involving the cranial base. OBJECTIVE: To determine the feasibility of endoscopic extradural anterior clinoidectomy via the supraorbital keyhole. METHODS: Eight fresh cadaver heads were studied using 4-mm, 0- and 30-degree rigid endoscopes to perform endoscopic extradural anterior clinoidectomy. We also evaluated a bony landmark for this technique in 36 dry craniums. RESULTS: An endoscope was introduced into the extradural space created via a supraorbital keyhole craniotomy. The periorbita and the duplication of the dura extending to the temporal lobe dura and periorbita were exposed by drilling. Anterior clinoidectomy proceeded using a diamond drill under endoscopic visualization without a dural incision. A submerged view with continuous irrigation through an endoscopic sheath maintained clear visibility while drilling. A small bony eminence at the transition between the sphenoid ridge and the anterior clinoid process, which is an anatomic landmark for endoscopic extradural anterior clinoidectomy, was identified in 57.4% of 36 adult dry craniums. CONCLUSION: The endoscopic extradural procedure can accomplish reliable anterior clinoidectomy under superb endoscopic visualization. This method would be applicable to parasellar and cavernous sinus surgery combined with keyhole or conventional craniotomy.

OBJECT: The cavernous sinus is a small complex structure located at the central base of the skull. Recent extensive use of endoscopy has provided less invasive approaches to the cavernous sinus via endonasal routes, although transcranial routes play an important role in the approach to the cavernous sinus. The aims of this study were to evaluate the feasibility of the purely endoscopic transcranial approach to the cavernous sinus through the supraorbital keyhole and to better understand the distorted anatomy of the cavernous sinus via endoscopy. METHODS: Eight fresh cadavers were studied using 4-mm 0° and 30° endoscopes to develop a surgical approach and to identify surgical landmarks. RESULTS: The endoscopic supraorbital extradural approach was divided into 4 stages: entry into the extradural anterior cranial fossa, exposure of the middle cranial fossa and the periorbita, exposure of the superior cavernous sinus, and exposure of the lateral cavernous sinus. This approach provided superb views of the cavernous sinus structures, especially through the clinoidal (Dolenc) triangle. The lateral wall of the cavernous sinus, including the infratrochlear (Parkinson) triangle and anteromedial (Mullan) triangle, was also clearly demonstrated. CONCLUSIONS: An endoscopic supraorbital extradural approach offers excellent exposure of the superior and lateral walls of the cavernous sinus with minimal invasiveness via the transcranial route. This approach could be an alternative to the conventional transcranial approach.

The incidence of vitamin K deficiency in infancy has decreased markedly, due to prophylactic administration of vitamin K during the neonatal period. However, vitamin K deficiency bleeding may occur during or after the neonatal period despite prophylactic administration in Japan. Two cases are reported of intracranial hemorrhage associated with coagulopathy in full-term infants who had received prophylactic administration of vitamin K. More reliable methods for prophylactic administration should be established.

A 38-year-old woman presented with a case of post-traumatic ethmoidal cerebrospinal fluid (CSF) leak that was repaired using a purely endoscopic endonasal approach. Six weeks after a mild domestic maxillofacial trauma, she started complaining of clear, watery discharge from the left nostril and headache. Neuroimaging investigations disclosed a linear fracture of the left anterior ethmoidal roof without evidence of large bony breaches or any brain tissue damage. After conservative medical treatment with carbonic anhydrase inhibitors failed, she was referred to our hospital for surgical repair of the osteodural defect. An endoscopic endonasal transethmoidal approach was successfully performed, and an overlay technique was used to reconstruct the defect. The patient was discharged without clinical evidence of CSF rhinorrhea and no leaks were apparent at the 3-month follow-up clinical and radiological examinations. Post-traumatic CSF rhinorrhea occurs in cases of dural tears associated with small bone breaks, most frequently involving the anterior skull base. Recovery is often spontaneous following only bed rest, or with medical treatments such as inhibitors of carbonic anhydrase diuretics, steroids, or eventually stool softeners to help reduce CSF pressure. Surgical repair is required when first-line conservative treatment proves ineffective. The present case shows that the endoscopic endonasal technique for the management of CSF leaks provides a less invasive surgical route to achieve valid dural repair.

A 23-year-old man was admitted with a rare case of radiation-induced astrocytoma manifesting as 3-month history of unstable gait. He had received 50 Gy of irradiation therapy for a germ cell tumor in the right basal ganglia 13 years earlier. Magnetic resonance (MR) imaging on admission showed a non-enhanced mass lesion in the right cerebellar hemisphere with expansion to the vermis. The histological diagnosis of the stereotaxic biopsy specimen was grade II astrocytoma. Two months later, he developed drowsiness, and MR imaging demonstrated that the tumor had enlarged and was enhanced after gadolinium injection. The clinical diagnosis was high-grade glioma resulting from malignant transformation. The tumor had compressed the mesencephalic aqueduct, leading to obstructive hydrocephalus. Endoscopic third ventriculostomy was performed to improve the cerebrospinal fluid circulation. He underwent chemotherapy with temozolomide postoperatively, but died 8 months after the initial diagnosis of astrocytoma. The clinical course of radiation-induced astrocytoma is not benign. The potential for malignant transformation necessitates careful postoperative follow up for patients with this tumor.

A 57-year-old female presented with a left putaminal hemorrhage, intraventricular hematoma (IVH), and acute obstructive hydrocephalus. Neuroendoscopic surgery was performed for the IVH. Three days after successful IVH removal and improvement of her consciousness, the patient lapsed into a comatose state due to hydrocephalus caused by obstruction of the mesencephalic aqueduct by a small remnant clot. The small clot was identified by constructive interference with steady state magnetic resonance imaging and was removed during a second-look operation. Even after the cerebrospinal fluid circulation improves following neuroendoscopic surgery for IVH, the patient may nevertheless develop a life-threatening condition without warning signs in the subacute phase. Awareness of this complication will lead to early diagnosis and correct management.

BACKGROUND: Symptomatic Rathke's cleft cyst is usually accompanied by a long history of headache, visual disturbance, and hypopituitarism; however, rare cases present with acute onset and the clinical features in such cases remain uncertain. We report herein the clinical features of Rathke's cleft cyst with acute onset and discuss the clinical significance. METHOD: In this study, we defined acute onset as the clinical course with clinical symptoms within a 7-day history. From among 35 cases of symptomatic Rathke's cleft cyst that were pathologically diagnosed at Fukuoka University Hospital between 1990 and 2009, five cases presented with acute onset. The symptoms, endocrinological findings, MR image findings, and pathological findings of these cases were analyzed retrospectively. FINDINGS: Mean age was 56.8 years. Initial symptoms included headache (n = 3), general malaise (n = 2), polyuria (n = 2), and fever (n = 1). MR imaging revealed an intrasellar cystic lesion with suprasellar extension in all cases and showed rim enhancement in three cases. All cases were treated by transsphenoidal surgery. Pathological findings included hemorrhage (n = 2), hypophysitis (n = 2), and abscess formation in the cyst (n = 1). Postoperatively, all symptoms, except for hypopituitarism, improved in all cases. CONCLUSIONS: Rathke's cleft cysts sometimes present with acute onset, and the presentation is consistent with the features of pituitary apoplexy caused by pituitary adenoma. Although pituitary apoplexy due to hemorrhage, inflammation, or infection due to an underlying Rathke's cleft cyst is difficult to diagnose pre-operatively, Rathke's cleft cyst should be included in the differential diagnosis, and early surgical treatment is needed, as for pituitary apoplexy caused by pituitary adenoma.

We report a case of intramedullary cervical metastasis from cerebellar anaplastic oligodendroglioma without any MR appearance for CSF dissemination or recurrence in the primary tumor site. The case was a 73-year-old female who underwent tumor resection for cerebellar anaplastic oligodendroglioma following irradiation and TMZ chemotherapy. One year and a half later, a small nodular lesion developed at the temporal lobe. While treatment with TMZ was restarted during the course, another intramedullary cervical lesion produced additionally without any CSF dissemination or recurrence at the primary site. The histological examination obtained in spinal surgery revealed evidence of similar features consistent with a previous cerebellar tumor with anaplastic oligodendroglioma. Although CSF dissemination by malignant glioma with leptomeningeal enhancement is relatively well recognized at their terminal stage, cases with intramedullary cervical metastasis without any leptomeningeal enhancement have only been rarely described. We discussed the mechanisms of intramedullary cervical metastasis from intracranial malignant glioma.

A 37-year-old man involved in a motor vehicle accident was admitted to our hospital with disturbed consciousness. Computed tomography (CT) showed an acute, right-sided epidural hematoma and pneumocephalus. Emergency evacuation of the acute epidural hematoma was carried out, and his condition gradually recovered. However, cerebrospinal fluid (CSF) rhinorrhea continued despite conservative treatment. Three-dimensional reconstructed CT revealed a bone defect, which was approximately 20 mm in size, in the planum sphenoidale and tuberculum sellae, and magnetic resonance imaging demonstrated a traumatic meningo-encephalocele through the defect, with CSF collection in the sphenoid sinus. Endoscopic endonasal transsphenoidal surgery was performed. The 9-mm diameter dural defect was clearly visualized in the sphenoid sinus and was reconstructed with a combination of suturing and overlay with abdominal fascia, and absorbable polyglycolic acid felt. The CSF leakage from a traumatic meningo-encephalocele through the planum sphenoidale was successfully repaired by endoscopic endonasal surgery. Thorough preoperative evaluation of the feasibilities of the endoscopic and transcranial approaches should be based on the preoperative identification of the fistula, the bone defect, and vital structures.

The efficacy of treatment for intraventricular hematoma by neuroendoscopic surgery and extraventricular drainage was compared in 10 patients with intraventricular hematoma and hydrocephalus who underwent neuroendoscopic surgery (endoscopic group), and eight patients with intraventricular hematoma and hydrocephalus treated with extraventricular drainage (EVD group). The outcomes in each group were assessed retrospectively using the Graeb scores on the pre- and postoperative computed tomography (CT), duration of extraventricular drainage, requirement for a shunt operation, and modified Rankin scale score at 12 months. The Graeb scores on the preoperative CT were not significantly different between the two groups, but the duration of catheter placement was significantly shorter (69.3%) in the endoscopic group (2.7 days) than in the EVD group (8.8 days). None of the patients in either group required a shunt procedure for communicating hydrocephalus 12 months after surgery. Neuroendoscopic removal is a safe and effective procedure for intraventricular hematoma. Advantages include rapid removal of hematoma in the ventricular systems and reliable improvement of non-communicating hydrocephalus in the acute phase. The procedure resulted in faster removal of the catheter in the postoperative period and earlier patient ambulation.

A 13-year-old girl presented with an exceedingly rare case of primary yolk sac tumor located within the lateral ventricle, manifesting as headache, nausea, and diplopia. Magnetic resonance imaging revealed a 4-cm-diameter solid enhanced mass within the left inferior horn of the lateral ventricle. The tumor was removed subtotally via left middle temporal corticotomy. The histological and immunohistochemical diagnosis was pure yolk sac tumor. The serum alpha-fetoprotein (AFP) level was elevated at 1957.2 ng/ml and the serum beta-human chorionic gonadotropin level was 4 mIU/ml after surgery. The patient underwent radiotherapy (whole brain, 30 Gy; tumor bed, 21 Gy; whole spinal axis, 30 Gy) and chemotherapy (ifosfamide, cisplatin, etoposide). After three treatment cycles, the serum AFP level had decreased to 4.5 ng/ml. However, the tumor recurred with cerebrospinal fluid dissemination after nine cycles of chemotherapy. She died 18 months after surgery. The possibility of germ cell tumor should be considered in pediatric patients with brain tumors occurring outside the pineal or suprasellar region.

BACKGROUND: Numerous studies have reported the clinical efficacy of temozolomide (TMZ) treatment for high-grade glioma, but information on Japanese populations has been limited. This study assessed the safety and early outcomes of TMZ treatment, with or without combination therapy. PATIENTS AND METHODS: The subjects comprised ten patients with high-grade glioma [glioblastoma multiforme (GBM), n=3, gliosarcoma (GS), n=1, anaplastic oligodendroglioma (AO), n=3, anaplastic mixed oligoastrocytoma (AOA), n=1, and anaplastic ependymoma (AE), n=2]. All the patients were initially treated with conventional radiotherapy following surgical resection with or without adjuvant chemotherapy. As second- or third-line chemotherapy, patients received TMZ for recurrence or tumor progression. As combination therapy, the local administration of tumor necrosis factor-alpha and the addition of carboplatin and etoposide were included for three patients during the course of oral TMZ treatment. RESULTS: Partial response (PR) to TMZ therapy was achieved by four out of the ten patients (objective response rate, 40%), while three patients displayed stable disease (SD) and three showed disease progression (PD). One of the patients receiving combination therapy has continued to show shrinkage of the relapsed tumor. Despite prior radio- and chemotherapy, most patients experienced only grade 1-2 hematotoxicity that was well-controlled by conservative therapy. CONCLUSION: TMZ chemotherapy is effective for the treatment of high-grade glioma in some patients without serious toxicity. Assessing the true efficacy of TMZ will require a larger study with comparison of long-term outcomes between other agents or combined therapeutic modalities.

BACKGROUND: Pilomyxoid astrocytoma (PMA) shows a higher rate of recurrence and cerebrospinal fluid (CSF) dissemination than does pilocytic astrocytoma (PA). In this article, we discuss the treatment of PMA. MATERIALS AND METHODS: Between 1992 and 2007, the authors treated 5 patients. Two of these were male, three female. Their ages ranged from 3 months to 11 years. RESULTS: Three patients showed CSF dissemination on the initial radiographic examination. All patients received chemotherapy; the most commonly used combination drugs were cisplatin (CDDP)/carboplatin (CBDCA) and etoposide. When these drugs were unsuccessful, they were changed or other drugs added to the combination. After chemotherapy, four patients showed remarkable tumor regression. Nevertheless, one patient died 22 months after initial diagnosis, due to tumor progression. CONCLUSION: While our series was limited to a small number of patients, we have a positive impression of the value of chemotherapy. Even if initial chemotherapy is ineffective, we recommend continued CDDP/CBDCA-based chemotherapy with new drug combinations.

We report a case of ruptured tectal arteriovenous malformation (AVM) that was demonstrated angiographically only after removal of an unruptured occipital AVM. A 57-year-old man presented with sudden onset of diplopia and tinnitus. Computed tomography revealed a small hemorrhage in the right tectum mesencephali with intraventricular hemorrhage. Magnetic resonance imaging and angiography disclosed AVM in the right occipital lobe which was separate from the hemorrhagic lesion. Angiography demonstrated that the right occipital AVM was fed by the parieto-occipital artery and drained into the superior sagittal sinus and vein of Galen. However, no abnormal vascular lesion was detected near the tectum mesencephali. As venous hypertension was considered the reason for hemorrhage, the occipital AVM was completely resected. Postoperative angiography demonstrated disappearance of the occipital AVM, but it also disclosed a small tectal AVM fed by branches from the superior cerebellar artery, which had not been detected on preoperative angiography. This was considered the true cause of hemorrhage, and gamma knife surgery was accordingly performed. Even if an AVM is demonstrated, if the lesion does not correspond to the hemorrhage we recommend serial angiographical evaluation so that a small AVM is not missed.

We present an extremely rare case of an orbital melanocytoma that occurred in a 51-year-old man. The patient suffered from diplopia and mild exophthalmos of the right eye for 2 months. Brain magnetic resonance imaging showed a well-demarcated round mass 3.5 cm in diameter in the right orbit. We performed total resection of this tumor. Histological findings revealed a proliferation of large polygonal cells with fine pigment granules in the cytoplasm and prominent nucleoli. Although these tumor cells revealed immunohistochemical reactivity in HMB-1, there was no S-100 or Melan A antibody reactivity. Also, there were no malignant findings of nuclear polymorphism, mitoses, or necrosis. The brown pigments were confirmed to be melanin by bleaching and the Fontana-Masson silver stain method. The MIB-1 labeling index was less than 1%. This tumor also consisted of 50% melanophages, which revealed immunohistochemical reactivity in CD68, CD163, and in (1-AT antibodies. These histological findings led us to diagnose an orbital melanocytoma with partial tumor regression.

From unfractionated embryonic mice liver cells, appreciable amount of spherical bodies containing nestin-positive cells were generated in the presence of neuronal growth factors. Following cultivation on poly-D: -lysine/laminin-coated slips, approximately 70% of the cells expressed neuronal markers, and 16% had long processes. Functional analysis of these long-process-bearing cells with the whole-cell patch clamp method showed an inward current in response to glutamate, GABA, and serotonin as the neuronal characteristics. Furthermore, regenerating liver in adult mice also contained nestin-positive cells to the same extent as fetal liver. Regenerating liver could have potential as a source of neural cells for autologous transplantation.

A 64-year-old man underwent microvascular decompression of the left superior cerebellar artery (SCA) for left trigeminal neuralgia (TN) using a sling of Teflon tape fixed to the tentorium with fibrin glue. The TN disappeared immediately after surgery, but recurred unusually rapidly at 2 weeks later at the same intensity as before. Second surgery revealed the SCA was suspended from the tentorium, but the trigeminal nerve was stretched and displaced superolaterally because of adhesion to the superior petrosal vein. The adhesion was thought to involve the fibrin glue used during the sling retraction procedure. The nerve was meticulously dissected from the adhesion, and the trigeminal nerve was placed in the correct position. The postoperative course was uneventful, and the TN disappeared completely. We recommend that the smallest amount of the fibrin glue possible be used to avoid adhesion to the surrounding neurovascular elements.

A 13-day-old female infant was admitted with hydrocephalus that had been diagnosed on prenatal ultrasound at 33 weeks' gestation. She was delivered by Caesarean section at 34 weeks with an Apgar score of 10. On admission, she weighed 2,103 g. The head circumference was 32.3 cm, and the fontanelle was tense. T(1)- and T(2)-weighted MR images revealed an isointense mass occupying the fourth ventricle with multiple cysts in the vermis. The mass was not enhanced after gadolinium administration. CT showed no definite calcification in the lesion. Preoperatively, vermian tumors, including medulloblastoma, ependymoma, astrocytoma, and hamartomas, were considered in the differential diagnosis. Hamartoma was strongly suspected due to the lack of enhancement on MRI. After a suboccipital midline craniotomy, subtotal resection of a soft grayish tumor with areas of hematoma was carried out. The pathological diagnosis was medulloblastoma. Despite chemotherapy, CSF dissemination resulted in death at 11 months. We report this case of congenital medulloblastoma with atypical MRI findings and discuss the clinical characteristics of this lesion.