Purely endoscopic subtemporal keyhole anterior transpetrosal approach to access the petrous apex region: surgical techniques and early results.

OBJECTIVE: The anterior transpetrosal approach using a microscope to provide wider access to the petrous apex region has been described for radical resection of lesions of the middle and posterior skull base. The microscopic anterior transpetrosal approach (mATPA) requires a wide craniotomy and meticulous epidural procedures to minimize temporal lobe retraction. Recently, the clinical application of transcranial endoscopic keyhole approaches for minimally invasive surgery has been steadily expanding. In this study, the details of the purely endoscopic subtemporal keyhole ATPA (eATPA) for petrous apex lesions are described and its initial results are reported. METHODS: Between May 2022 and May 2023, the authors performed eATPA in 10 patients with petrous apex lesions, of which 6 were meningiomas, 3 were trigeminal schwannomas, and 1 was epidermoid cyst. The surgical procedure of the purely eATPA is as follows. After a small temporal craniotomy, the endoscopic procedure is started. The anterior rim of the petrous bone and Meckel's cave are exposed via an intradural subtemporal approach. The lesion is removed with additional drilling of Kawase's triangle, cutting the superior petrosal sinus, opening Meckel's cave, and cutting the tentorium. The authors also compared the outcomes of mATPA versus eATPA for consecutive cases of petrous apex lesions. RESULTS: Gross-total resection was achieved in 8 of the 10 patients. The average operative time was 4 hours 13 minutes. There were 3 cases of transient abducens nerve palsy and 1 case of trochlear nerve palsy in the postoperative period. No new-onset motor deficits or CSF leakage was noted in any of these patients. Only 1 patient exhibited postoperative asymptomatic temporal lobe edema. The Karnofsky Performance Scale (KPS) scores remained unchanged or improved for all patients postoperatively. Compared with mATPA, eATPA achieved a similar extent of resection and comparable postoperative KPS scores with a significantly shorter mean operative time, much smaller temporal craniotomy, and thus less mean blood loss during surgery with lower rates of new-onset temporal lobe edema in the postoperative period. CONCLUSIONS: An eATPA allows a direct route to access Meckel's cave and posterior cranial fossa lesions similar to conventional mATPA, with shortening the operative time and reducing the risk of postoperative temporal lobe edema. This eATPA is considered one of the new surgical techniques that can be expected to develop in the future.

Rheumatologic manifestations with elevated levels of IL-6, IL-17A, and IL-23 in a patient with scurvy.

Symptomatic vitamin C deficiency, scurvy, is a relatively rare disease in developed countries, but it has been reported in patients with autism spectrum disorder or developmental delay who tend to have selective diets. Patients with scurvy often demonstrate musculoskeletal manifestations with unknown pathophysiology. Herein, we report a case of scurvy in an 11-year-old boy who presented with iron-deficiency anaemia, systemic osteomyelitis, myositis predominantly in the lower extremities, and right ventricular volume overload with mild pulmonary hypertension and was diagnosed with scurvy. He had a mild developmental disorder and a selective diet, which resulted in severe vitamin C deficiency. He received intravenous and oral vitamin C supplementation, which relieved his arthralgia and muscle pain in a week. Following 4 months of vitamin C supplementation, he demonstrated no abnormal manifestations on laboratory or imaging examination and recovered without sequelae. Inflammatory cytokine and chemokine evaluations demonstrated elevated levels of interleukin (IL)-6, IL-17A, and IL-23, which are associated with T-helper (Th) 17 cell activation. This study is the first to suggest the association between the inflammation seen in scurvy, rheumatic manifestations in the patient, and Th17 cell activation. Further analysis of the association between the inflammation and vitamin C supplementation may contribute to new insights for the comprehension and treatment of other inflammatory diseases, such as rheumatic diseases.

Visualization of Resected Area in Endonasal Endoscopic Approach versus Transcranial Approach for Skull Base Meningiomas by Voxel-Based-Lesion Mapping.

BACKGROUND: We aimed to evaluate the resected area of endonasal endoscopic approach (EEA) and transcranial approach (TCA) for skull base meningiomas (SBMs) using voxel-based-lesion mapping and visualized the appropriate tumor location in each approach. METHODS: We retrospectively examined 182 patients with SBMs who underwent tumor resection in our hospital between 2014 and 2019. Pre- and post-operative SBMs were manually delineated on MRI to create the voxels-of-interest (VOIpre and VOIpost) and were registered onto the normalized brain (normalized VOIpre and normalized VOIpost). The resected map was created by subtracting normalized VOIpost from the normalized VOIpre divided by the number of cases. The resected maps of TCA and EEA were compared by subtracting them. RESULTS: Twenty patients underwent EEA and 135 patients underwent TCA. The tumor resected map demonstrated that the resected area of EEA frequently accumulated on the central skull base, while that of TCA accumulated near the central skull base. The border of both approaches matched the circle that connects neural foramens at the skull base. CONCLUSIONS: The resected area of SBMs by EEA and TCA was well visualized by voxel-based-lesion mapping. The circle connecting the neural foramens was the border of EEA and TCA.

Endoscopic Contralateral Interhemispheric Transfalcine Keyhole Approach for Large Falcine Meningiomas.

OBJECTIVE: Falcine meningioma is usually approached ipsilaterally, and the technique for tumor removal has traditionally been performed under microscopy. We report a surgical procedure for large falcine meningioma by an endoscopic contralateral interhemispheric transfalcine keyhole approach. METHODS: The study period was from September 2019 to March 2021. Study participants were patients with World Health Organization grade I meningioma showing falx attachment, excluding neurofibromatosis, who underwent initial surgery at our institution. The surgical procedure begins with a small contralateral craniotomy of about 3 cm, followed by insertion of an endoscope. The tumor attachment to the falx is excised, exposing the tumor. Internal decompression is performed, and the lesion is dissected from the surrounding brain before removal through the falx. RESULTS: An endoscopic contralateral interhemispheric transfalcine keyhole approach was used to resect 4 cases of large falcine meningioma. The mean operation time was 265 minutes (range: 216-294 minutes), achieving Simpson grade I removal in all cases. No evidence of cerebral infarction, cerebral edema, or new neurological complaints related to impaired venous return was seen using this surgical method. CONCLUSIONS: In the case of falcine meningioma, the endoscopic keyhole contralateral technique allows detachment of the tumor from the falx and safe manipulation in a minor field of view. In addition, because the craniotomy is smaller and the operation time is shorter, this procedure offers a less-invasive approach for the patient. This technique is thus, in our opinion, quite advantageous.

Two-stage posterior decompression and fusion for tuberculous spondylitis after intravesical bacillus Calmette-Guerin instillation.

Intravesical bacillus Calmette-Guerin (BCG) instillation is an effective treatment for nonmuscle invasive superficial bladder cancer. BCG induces a massive influx of inflammatory cells and production of cytokines in the bladder mucosa and lumen that leads to an immune response against tumor cells, acting as an immunotherapy. Cystitis, bladder ulceration, and bladder contracture are known local genitourinary complications, whereas systemic complications such as miliary pulmonary tuberculosis, mycotic aneurysms, tuberculous spondylodiscitis, and granulomatous hepatitis are very rare. A case of tuberculous spondylodiscitis at the T8 and T9 levels following intravesical BCG instillation for bladder carcinoma is reported. The patient initially underwent decompressive laminectomy for spastic paraparesis. After reporting improvement for few weeks, the patient again presented with similar complaints and was found to have an increased kyphotic deformity, for which he underwent fixation surgery.

Osaka Sliding Knot Seals Dural Defect Simply in Extended Endoscopic Endonasal Approach.

An extended endoscopic endonasal approach (EEA) has become standard for parasellar and midline skull base lesions. However, postoperative leakage of cerebrospinal fluid (CSF) can result from dural defects after lesion removal. We present a simple and effective technique, the Osaka sliding knot, to prevent CSF leakage. Between November 2018 and March 2021, a total of 41 patients underwent reconstruction of skull base defects with intraoperative high-flow CSF leaks after extended EEA by using this closure technique, of whom only 1 patient experienced postoperative CSF leakage. This technically simple and efficient method seals the dural defect to prevent CSF leakage after surgeries using an extended EEA.

Pediatric giant cell reparative granuloma of the lower clivus: A case report and review of the literature.

Giant cell reparative granuloma (GCRG) is a benign nonneoplastic granulomatous lesion and is rare in the cranial bone. We present a pediatric case of this lesion arising from the condyle and lower clivus. A 9-year-old girl presented with slowly progressive hoarseness and dysphagia. She showed left glossopharyngeal, vagus, and hypoglossal nerve palsy. An osteolytic lesion around the lower clivus and condyle joint was accompanied by deformation of the craniovertebral junction. An endoscopic endonasal approach was used to decompress the cranial nerve and confirm the pathological finding. The lesion around the condyle was not resected to preserve occipito-cervical stability. The residual lesion has been observed carefully for 6 months, and regrowth has not occurred. GCRG is a rare granulomatous lesion in the cranial bone. This case is the first report of a pediatric clival GCRG. Treating pediatric GCRG may be helpful.

Minimal anterior and posterior combined transpetrosal approach for large petroclival meningiomas.

OBJECTIVE: Petroclival meningiomas (PCMs) remain difficult to remove, and radical tumor resection continues to pose a relatively high risk of neurological morbidity in patients with these lesions because of the proximity of the tumor to neurovascular structures. The anterior and posterior combined (APC) transpetrosal approach allows resection of a large petroclival lesion with minimal retraction of the temporal lobe. However, this approach is thought to be complex and time-consuming. The authors simplified this approach by minimizing the petrosectomy and used this method for large PCMs. This retrospective study describes the surgical technique and surgical outcomes of large PCMs. METHODS: Between 2014 and 2019, 23 patients (19 women and 4 men) with benign (WHO grade I) PCMs were treated using the minimal APC (MAPC) transpetrosal approach. The mean age at surgery was 54.0 years (range 37-74 years). The mean tumor diameter was 40.3 mm (range 30-74 mm). The surgical technique consisted of a temporo-suboccipital craniotomy and minimal drilling of the petrous ridge. After opening Meckel's cave and removing the lesion at the prepontine cistern, drilling of the petrous apex with superior mobilization of the trigeminal nerve was performed through the subdural space for further tumor resection around the petrous apex. Finally, the tumor was removed as much as possible. RESULTS: The mean preoperative and postoperative tumor volumes were 26.8 and 1.3 cm3, respectively. The mean extent of resection was 95.4% (range 62%-100%). Postoperative impairments included facial numbness in 7 patients, trochlear nerve palsy in 3 patients, mild oculomotor nerve palsy in 2 patients, and transient abducens nerve palsy in 1 patient. Preoperative Karnofsky Performance Status was improved in 13 patients, remained stable in 9 patients, and deteriorated in 1 patient. CONCLUSIONS: The MAPC transpetrosal approach provides sufficiently wide exposure of petroclival lesions. Maximal resection via the MAPC transpetrosal approach is a suitable surgical option for the treatment of large PCMs.

Adult-Onset Mixed Germ Cell Tumor Composed Mainly of Yolk Sac Tumor Around the Pineal Gland: A Case Report and Review of the Literature.

BACKGROUND: Mixed germ cell tumors (MGCTs) usually occur in children. In the present report, we discuss an extremely rare case of adult-onset MGCT composed mainly of yolk sac tumor (YST) around the pineal gland. CASE DESCRIPTION: A 54-year-old Japanese man presented with disturbance of consciousness, Parinaud's syndrome, and gait disturbance. Magnetic resonance imaging revealed a pineal mass lesion, and subtotal resection of the tumor was achieved. The histologic diagnosis was MGCT, consisting mainly of YST. Although he underwent 5 courses of chemotherapy and craniospinal irradiation after surgery, tumor dissemination could not be controlled, and he died 10 months postoperatively. CONCLUSION: The present case highlights the need for clinicians to include YST in the differential diagnosis of acute progressive lesions around the pineal region, even in adult patients.

Surgical implementation and efficacy of endoscopic endonasal extradural posterior clinoidectomy.

OBJECTIVEThe endoscopic endonasal approach (EEA) for skull base tumors has become an important topic in recent years, but its use, merits, and demerits are still being debated. Herein, the authors describe the nuances and efficacy of the endoscopic endonasal extradural posterior clinoidectomy for maximal tumor exposure.METHODSThe surgical technique included extradural posterior clinoidectomy following lateral retraction of the paraclival internal carotid artery and extradural pituitary transposition. In cases with prominent posterior clinoid process, a midline sellar dura cut was added to facilitate extradural exposure. Forty-four consecutive patients, in whom this technique was performed between 2016 and 2018 at Osaka City University Hospital, were reviewed. The pathology included 19 craniopharyngiomas, 7 chordomas, 6 meningiomas, 6 pituitary adenomas, 4 chondrosarcomas, and 2 miscellaneous. Utilization and effectiveness of this approach were further demonstrated with neuroimaging.RESULTSExtradural posterior clinoidectomies were successfully applied in all patients without permanent neurovascular injury and with better maneuverability and greater resection rate of the tumors. Four patients experienced transient postoperative abducens nerve paresis, and 1 patient experienced transient postoperative oculomotor nerve paresis; however, the patients with deficits recovered within 3 months. On radiological examination, the surgical field was 2.2 times wider in cases with bilateral posterior clinoidectomy than in cases without posterior clinoidectomy.CONCLUSIONSThe extended EEA with extradural posterior clinoidectomy creates an extra working space and allows adequate accessibility with safe surgical maneuverability to remove tumors that extend behind the posterior clinoid and dorsum sellae.

Safe maximal resection of primary cavernous sinus meningiomas via a minimal anterior and posterior combined transpetrosal approach.

OBJECTIVE Meningiomas arising from the cavernous sinus (CS) continue to be a significant technical challenge, and resection continues to carry a relatively higher risk of neurological morbidity in patients with these lesions because of the tumor's proximity to neurovascular structures. The authors report the surgical outcomes of 9 patients with primary CS meningiomas (CSMs) that were surgically treated using a minimal anterior and posterior combined (MAPC) transpetrosal approach, and they emphasize the usefulness of the approach. METHODS This retrospective study included 9 patients who underwent surgery for CSM treatment between 2015 and 2016 via the MAPC transpetrosal approach. Two patients were men and 7 were women, with a mean age of 58.5 years (39-72 years). Five patients (55.5%) had undergone previous treatment. The surgical technique consisted of a temporo-occipito-suboccipital craniotomy and exposure of the posterolateral part of the CS via the presigmoidal MAPC approach. After opening Meckel's cave and identifying the 3rd-5th cranial nerves in the prepontine cistern, Parkinson's triangle and supratrochlear triangles were opened. Finally, the tumor occupying the posterolateral part of the CS was removed. RESULTS All lesions were safely and maximally removed, with preservation of external ocular movements and preoperative Karnofsky Performance Scale scores. The mean extent of resection was 77.0% (range 58.7%-95.4%). Six patients underwent adjuvant therapy in the form of stereotactic radiosurgery (SRS) or stereotactic radiotherapy (SRT) during the follow-up period; none of these patients experienced recurrence. CONCLUSION The authors conclude that the MAPC transpetrosal approach could be superior to other approaches for CSMs, as it provides direct visual access to the posterolateral portion of the CS. In their experience, this approach is an alternative and better option for safe maximal resection of CSMs.

Contralateral Minimum Anterior and Posterior Combined Petrosal Approach for Retrochiasmatic Craniopharyngiomas: An Alternative Technique.

Retrochiasmatic craniopharyngiomas (RC) are a challenge for the neurosurgeon to treat surgically, restrained by their location in the interpeduncular fossa, surrounded by vital neurovascular structures, narrow corridor and poor visibility. Many approaches are possible and elucidated in the literature, which the surgeon chooses, based on multiple factors, such as the size of tumor, calcification, laterality, preoperative neurological deficits and the endocrine function status, recurrence, postradiotherapy status, or significant superior and/or posterior extension. 1 2 We describe a contralateral minimum anterior and posterior (CL-MAPC) petrosal approach for a case of recurrent RC, in a 37-year-old female patient operated before using a pterional approach, now presented with left homonymous hemianopia and panhypopituitarism ( Fig. 1 ). We preferred a contralateral approach to protect the ipsilateral optic tract (OT) from retraction injury, which formed an obstacle to the tumor from ipsilateral side. Apart from various benefits described by the author previously for RC, using MAPC petrosal approach, the CL-MAPC offers a safe corridor, protecting the ipsilateral OT, visualization of tumor origin usually posterior to chiasm, wider corridor if PCoM could be sacrificed, as it was done in this case, and pituitary stalk identification, with a probability of its functional preservation, unlike a necessity of pituitary transposition in EEA, though the endocrine outcome is poor after a radical resection irrespective of the approach chosen. 1 3 4 There was complete excision of the tumor with preservation of visual function postoperatively. We recommend the use of CL-MAPC as an alternative to EEA in some specific indications when the tumor is large, calcified, obscuring OT on the ipsilateral side and with significant lateral extension, which may be limiting factors in EEA ( Fig. 2 ). The link to the video can be found at: https://youtu.be/gWCJmh4_evs .

Genetic and prenatal findings in two Japanese patients with Schinzel-Giedion syndrome.

We report two Japanese patients with Schinzel-Giedion syndrome. When polyhydramnios is observed, additional fetal findings such as overlapping fingers, hydrocephalus, hydronephrosis, and very characteristic facial appearance comprising high, prominent forehead, hypertelorism, and depressed nasal root may suggest Schinzel-Giedion syndrome.

Cholangiocyte senescence caused by lysophosphatidylcholine as a potential implication in carcinogenesis.

BACKGROUND: The incidence of biliary tract cancer in patients with pancreaticobiliary maljunction or intrahepatic cholelithiasis is markedly high with undefined mechanism. In these diseases, biliary lysophosphatidylcholine (LPC) level is reportedly increased. This study investigated the influence of LPC on cholangiocytes focusing on cellular senescence and its potential contribution to carcinogenesis. METHODS: Cultured MMNK-1, an immortalized human cholangiocyte was treated with LPC in vitro and its effect was evaluated. RESULTS: Lysophosphatidylcholine demonstrated cytotoxicity with generation of intracellular reactive oxygen species. Accordingly, LPC provoked oxidative DNA injury, whereas the gene expressions of DNA repair enzyme (OGG1, MUTYH, MTH1) remained unchanged. Interestingly, LPC caused global DNA hypomethylation, which is frequently observed in cancer tissues. Microarray analysis identified differentially regulated genes in response to LPC, which included the components of senescence-associated secretory phenotype (SASP) including interleukin-8 (IL-8), IL-6, transforming growth factor-ß and plasminogen activator inhibitor-1. Significant induction of these genes was further confirmed by quantitative real-time polymerase chain reaction. In addition to upregulation of p21 gene expression, senescence-associated beta-galactosidase activity, a widely used marker of cellular senescence was significantly induced by the treatment of LPC. CONCLUSIONS: Based on these data, cholangiocyte senescence and SASP caused by LPC are potential pathogenic mechanisms in the development of biliary tract cancer.