Predictive role of regional thigh tissue oxygen saturation monitoring during cardiopulmonary bypass in lung injury after cardiac surgery.

Acute respiratory distress syndrome (ARDS) is a serious complication following cardiac surgery mainly associated with the use of cardiopulmonary bypass (CPB), which could increase the risk of mortality and morbidity. This study investigated the association of regional oxygen saturation (rSO2) during CPB with postoperative outcomes, including respiratory function. Patients who underwent cardiac surgery with CPB from 2015 to 2019 were included. Near-infrared spectroscopy was used to monitor rSO2 at the forehead, abdomen, and thighs throughout the surgery. Postoperative markers associated with CPB were assessed for correlations with PaO2/FiO2 (P/F) ratios at intensive care unit (ICU) admission. Postoperative lung injury (LI) was defined as moderate or severe ARDS based on the Berlin criteria, and its incidence was 29.9% (20/67). On multiple regression analysis, the following were associated with P/F ratios at ICU admission: vasoactive-inotropic scores at CPB induction (P = 0.03), thigh rSO2 values during CPB (P = 0.04), and body surface area (P < 0.001). A thigh rSO2 of 71% during CPB was significantly predictive of postoperative LI with an area under the curve of 0.71 (P = 0.03), sensitivity of 0.70, and specificity of 0.68. Patients with postoperative LI had longer ventilation time and ICU stays. Thigh rSO2 values during CPB were a potential predictor of postoperative pulmonary outcomes.

An analysis of the demographic history of the risk allele R4810K in RNF213 of moyamoya disease.

BACKGROUND: Ring finger protein 213 (RNF213) is a susceptibility gene of moyamoya disease (MMD). A previous case-control study and a family analysis demonstrated a strong association of the East Asian-specific variant, R4810K (rs112735431), with MMD. Our aim is to uncover evolutionary history of R4810K in East Asian populations. METHODS: The RNF213 locus of 24 MMD patients in Japan were sequenced using targeted-capture sequencing. Based on the sequence data, we conducted population genetic analysis and estimated the age of R4810K using coalescent simulation. RESULTS: The diversity of the RNF213 gene was higher in Africans than non-Africans, which can be explained by bottleneck effect of the out-of-Africa migration. Coalescent simulation showed that the risk variant was born in East Asia 14,500-5100 years ago and came to the Japanese archipelago afterward, probably in the period when the known migration based on archaeological evidences occurred. CONCLUSIONS: Although clinical data show that the symptoms varies, all sequences harboring the risk allele are almost identical with a small number of exceptions, suggesting the MMD phenotypes are unaffected by the variants of this gene and rather would be more affected by environmental factors.

PDGFRA gene rearrangements are frequent genetic events in PDGFRA-amplified glioblastomas.

Gene rearrangement in the form of an intragenic deletion is the primary mechanism of oncogenic mutation of the epidermal growth factor receptor (EGFR) gene in gliomas. However, the incidence of platelet-derived growth factor receptor-α (PDGFRA) gene rearrangement in these tumors is unknown. We investigated the PDGFRA locus in PDGFRA-amplified gliomas and identified two rearrangements, including the first case of a gene fusion between kinase insert domain receptor (KDR) (VEGFRII) and the PDGFRA gene, and six cases of PDGFRA(Δ8, 9), an intragenic deletion rearrangement. The PDGFRA(Δ8, 9) mutant was common, being present in 40% of the glioblastoma multiformes (GBMs) with PDGFRA amplification. Tumors with these two types of PDGFRA rearrangement displayed histologic features of oligodendroglioma, and the gene products of both rearrangements showed constitutively elevated tyrosine kinase activity and transforming potential that was reversed by PDGFR blockade. These results suggest the possibility that these PDGFRA mutants behave as oncogenes in this subset of gliomas, and that the prevalence of such rearrangements may have been considerably underestimated.

Vaginal Delivery under Epidural Analgesia in Pregnant Women with a Diagnosis of Moyamoya Disease.

BACKGROUND: Moyamoya disease more commonly occurs in young people and women, so patients with this disease may experience pregnancy and delivery. Cesarean section (CS) is often chosen as the mode of delivery for these patients in Japan. No appropriate mode of delivery has yet been established for pregnant women with moyamoya disease in terms of stroke prevention. We have used vaginal delivery under epidural analgesia (EA) in such patients unless CS has been indicated for the maternal or fetal reasons. This study retrospectively analyzed our patients with moyamoya disease who gave birth to confirm the safety of vaginal delivery under EA. METHODS: Twelve consecutive patients diagnosed with moyamoya disease had 14 deliveries at our hospital between September 2004 and January 2013. The incidences of intrapartum stroke were compared between cases of vaginal delivery under EA and CS cases. RESULTS: Ten vaginal deliveries under EA and 4 elective CSs were performed. No intrapartum stroke was observed during either vaginal delivery under EA or CS. Among the patients who underwent vaginal delivery under EA, 1 parturient who experienced 2 deliveries suffered transient ischemic attack during both postpartum periods. All 14 infants were healthy without sequelae. CONCLUSIONS: Vaginal delivery under EA is an option for patients with moyamoya disease, provided that close cooperation with neurosurgeons, obstetricians, and anesthesiologists is assured.

A nationwide survey of hypertrophic pachymeningitis in Japan.

OBJECTIVES: To clarify the prevalence, frequent causes and distinct features of hypertrophic pachymeningitis (HP) according to background conditions in a nationwide survey in Japan. METHODS: The study began with a preliminary survey to determine the approximate number of HP patients diagnosed from 1 January 2005 to 31 December 2009, and was followed by a questionnaire survey for clinical and laboratory findings. HP was defined as a condition with thickening of the cranial or spinal dura mater with inflammation, evidenced by MRI or histology. RESULTS: Crude HP prevalence was 0.949/100 000 population. The mean age at onset was 58.3±15.8 years. Among 159 cases for whom detailed data were collated, antineutrophil cytoplasmic antibody (ANCA)-related HP was found in 54 cases (34.0%) and IgG4/multifocal fibrosclerosis (MFS)-related HP in 14 cases (8.8%). Seventy cases (44.0%) were classified as 'idiopathic' and 21 (13.2%) as 'others'. ANCA-related HP cases showed a female preponderance, a higher age of onset, and higher frequencies of otological symptoms and elevated systemic inflammatory biomarkers, but lower frequencies of diplopia compared with idiopathic HP. IgG4/MFS-related HP cases showed a marked male predominance; all had cranial HP while none had isolated spinal HP or decreased sensation. CONCLUSIONS: HP is not extremely rare. ANCA-related HP is the most frequent form, followed by IgG4/MFS-related HP. Both forms have unique features, which may help to differentiate background causes.

Investigation of IgG4-positive cell infiltration in biopsy specimens from cases of hypertrophic pachymeningitis.

This study was an immunohistological study of IgG4-positive cell infiltration in 6 cases of hypertrophic pachymeningitis excluding secondary hypertrophic pachymeningitis caused by infectious diseases such as aspergillosis. The cases included 5 males and 1 female, ranging in age from 36 to 82 years (mean, 55 years). A biopsy was performed in all of the cases for diagnostic purposes, revealing fibrous dural hyperplasia with nonspecific inflammatory cell infiltration histologically. Two of the 6 patients had been treated with steroids before the biopsy, which was taken for poor response to steroid treatment. In these two cases, some IgG-positive cell infiltration of the thickened dura was observed; however, most of the cells were IgG4-negative. In the remaining four cases, many IgG- and IgG4-positive cells infiltrated the thickened dura and the IgG4-positive/IgG-positive cell ratio exceeded 40%. One of these patients was finally diagnosed with IgG4-related sclerosing disease, since he was diagnosed subsequently with retroperitoneal fibrosis. There was no evidence of any other lesions associated with IgG4-related sclerosing disease, other than in the dura. It is not rare for IgG4-positive cells to appear in the dura in cases of hypertrophic pachymeningitis; however, no IgG4-related systemic disease is present in these cases. Hypertrophic pachymeningitis with IgG4-positive cells may have some kind of relation to other systemic autoimmune diseases.

Signal intensity changes for the middle cerebral artery on 3-dimensional time-of-flight magnetic resonance angiography indicate acute hemodynamic changes after carotid endarterectomy.

BACKGROUND: For 3-dimensional time-of-flight magnetic resonance angiography (3D-TOF-MRA), the signal intensity (SI) loss depends on the flow velocity. In this study, we aimed to evaluate whether 3D-TOF-MRA could be used as an alternative to single-photon emission computed tomography (SPECT) for assessing the increase in the regional cerebral blood flow (rCBF) after carotid endarterectomy (CEA). To do this, we compared the SI of the middle cerebral artery (MCA) on magnetic resonance angiography (MRA) and the rCBF on SPECT. METHODS: We enrolled 30 patients with internal carotid artery stenosis. SPECT and MRA were performed before and 3-4 days after CEA. rCBF was assessed using SPECT, and the SI of the MCA was assessed using single-slab 3D-TOF-MRA. Regions of interest were placed in the bilateral middle M1 portions of the MCA on MRA, and their mean SI was measured. The increase ratio of the rCBF on SPECT and the increase ratio of the SI of the MCA on MRA were calculated using the formula: (post-CEA ipsilateral/post-CEA contralateral)/(pre-CEA ipsilateral/pre-CEA contralateral). RESULTS: A significant correlation was observed between the increase ratio of the rCBF on SPECT and the increase ratio of the SI of the MCA on MRA (r=.894, y=.4863+.5184x, P<.001). All values obtained by MRA were greater than or equal to the SPECT values, indicating that MRA tends to overestimate the post-CEA rCBF increase. CONCLUSION: Because MRA identified increased rCBF after CEA, we recommend that patients first be screened using MRA.

Midline Suboccipital Unilateral Trans-Cerebellomedullary Fissure Approach for Clipping of Ruptured VA-PICA Aneurysm: Two-Dimensional Operative Video.

The vertebral artery-posterior inferior cerebellar artery (VA-PICA) aneurysm poses a technical challenge for microsurgical clipping due to its anatomical complexity, which requires dissection of lower cranial nerves. Endovascular treatment is regarded as a feasible first-line therapeutic option for VA-PICA aneurysm because it has an acceptable aneurysm occlusion rate and is less invasive. However, microsurgical clipping remains an effective treatment option. We present the case of a 62-year-old man who presented with subarachnoid hemorrhage (SAH) due to a ruptured VA-PICA aneurysm. Neuroradiologic examination revealed a 2-3 mm medially pointing left VA-PICA aneurysm with acute obstructive hydrocephalus due to massive SAH in the posterior cranial fossa. As the patient had acute obstructive hydrocephalus and a relatively small aneurysm, we selected clipping over endovascular treatment. Because the aneurysm was located close to the midline and anterolateral to the medulla oblongata, we approached it from the midline. A midline suboccipital craniotomy, C1 laminectomy, and drilling of the left condylar fossa were performed; a unilateral cerebellomedullary fissure opening was added; and the aneurysm was clipped. Postoperative neuroradiologic examinations revealed complete obliteration of the aneurysm. As shown in this video, unilateral cerebellomedullary fissure opening combined with adequate removal of the condylar fossa provides a wide operative field in the cerebellomedullary cistern while avoiding strong retraction of the cerebellum. We believe that this technique makes VA-PICA aneurysm clipping safe and successful. Patient consent was obtained to perform the surgery and to publish the surgical video (Video 1).

A proposal for standardized analysis of the results of microvascular decompression for trigeminal neuralgia and hemifacial spasm.

BACKGROUND: The purpose of this study was to evaluate and analyze overall postoperative results from microvascular decompression (MVD) by combining the cure rate of symptoms with the complication rate. A new scoring system for obtaining objective surgical results from MVD for trigeminal neuralgia (TN) and hemifacial spasm (HFS) is proposed to document treatment results using consistent criteria in a standardized manner. METHOD: Surgical results combining complications , if any, were obtained from a questionnaire sent to patients who had undergone surgery for TN or HFS in recent years and had been followed-up for more than 1 year after surgery (TN patients, n = 54; HFS patients, n = 81) When surgical outcome is complete resolution of symptoms, the efficacy of surgery (E) is designated E-0, but when moderate symptoms are still persist postoperatively, the score is designated E-2. When no complications are seen after surgery, the complication score (C) is C-0, while the score is C-2 if troublesome complications remain. In addition, total evaluation of the results (T) is judged by combining the E and C scores. For example, when E is 0, and C is C-2, the total evaluation is scored as T-2, which is diagnosed as fair. FINDINGS: The response rate of the questionnaire was 80.7% (109/135). Overall surgical data were evaluated and analyzed using our new scoring system. Analysis of the collected data revealed an outcome of T-0 was 70% (35/50 patients) and T-1 was 24% (12/50) and T-2 was 6% (3/50) in TN, whereas in HFS, T-0 was 61% (36/59) and T-1 was 27.1% (16/59) and T-2 was 6.8% (4/59) and T-3 was 5.1% (3/59). CONCLUSION: The total results of MVD should be evaluated and analyzed by combining the cure rate of symptoms together with the complication rate. This new scoring system could allow much more objective analysis of the results of following MVD. Adopting this scoring system to objectively judge treatment results for TN and HFS, individual surgeons can compare their own overall surgical results with those of other institutes. Comparative results of MVD can also be provided to patients considering therapy to allow informed decision-making on the basis of good quality evidence.

Monitoring of intraoperative femoral oxygenation predicts acute kidney injury after pediatric cardiac surgery.

Cardiopulmonary bypass-associated acute kidney injury (CPB-AKI) is a pediatric cardiac surgery postoperative complication that is associated with a longer duration of mechanical ventilation and length of hospital stay. Identifying an early predictor of CPB-AKI is critical. Near infrared spectroscopy (NIRS), which can provide real-time monitoring of regional tissue oxygen saturation (rSO2) during CPB, may predict CPB-AKI in an early phase of surgical treatment. This study analyzed clinical data from 87 children who underwent an elective surgical repair of ventricular septal defect (VSD) from January 2013 to March 2019. NIRS sensors were placed on the patients' forehead, abdomen, and thighs. The pediatric modified risk, injury, failure, loss, and end-stage (p-RIFLE) score was determined for each patient postoperatively. The incidence of AKI based on the p-RIFLE classification was 11.5% at the end of surgery, 23.0% at 24 h after surgery, and 5.7% at 48 h after surgery. The AKI incidence rate was highest at 24 h after surgery. Multiple regression analysis revealed that femoral oxygenation (rSO2) during CPB, CPB time, oxygen delivery index (DO2i), and lactate at the end of CPB were independent risk factors for AKI. Receiver-operating characteristic curve analysis indicated that femoral oxygenation of 74% or less predicted AKI development within 24 h after surgery. In conclusion, rSO2 measured at the thigh during CPB is highly predictive of CPB-AKI.

Linear fractures occult on skull radiographs: a pitfall at radiological screening for mild head injury.

BACKGROUND: Skull radiography is widely used to screen for fractures in patients with mild head injury. However, the clear depiction of a fracture requires a gap in the skull separated by the fracture that is wide enough to allow the passage of x-rays. We studied atypical linear fractures that were not visualized clearly, because a specific anatomical configuration hampered the passage of x-rays. METHODS: We retrospectively evaluated 278 patients with mild head injuries who had undergone routine skull radiography (anteroposterior and lateral views) and head computed tomography (CT). We found that some patients negative for linear fracture on skull radiographs were positive on bone window CT scans. RESULTS: Of the 278 patients aged between 2 months and 66 years, 8 (2.9%) manifested a linear fracture on CT scans that presented as a cross section of the fracture oblique to the direction of the x-rays. Four of the 8 developed acute epidural hematoma; 2 of these patients underwent craniotomy. CONCLUSIONS: Radiographic study returned false-negative results, because x-rays were absorbed by the double-layered skull along fractures whose cross section was oblique to the direction of the x-rays. The evaluation of head injury by radiography only may miss these fractures and their undetected presence may result in sequelae such as intracranial hematoma.

Effectiveness of interferon-beta therapy for recurrent glioblastoma: a case report.

AIMS AND BACKGROUND: Glioblastoma has a poor prognosis, with few therapeutic options if it recurs. We report a case in which we were able to inhibit the growth of a recurrent glioblastoma by weekly single-dose administration of interferon-beta. CASE REPORT: A patient with recurrent glioblastoma after radiation and chemotherapy was treated with nimustine and interferon-beta. After 2 cycles of nimustine, the patient's leukocyte, neutrophil, and platelet counts showed grade 4 toxicity according to the National Cancer Institute's Common Toxicity Criteria. The patient was treated with a weekly single dose of interferon-beta at 6 x 10(6) IU. The tumor showed no remarkable changes after 18 months, and the patient's Karnofsky performance status remained at 50%. CONCLUSIONS: The administration of interferon-beta produced long-term control in one case of glioblastoma and may be an effective therapy.

Utility of intraoperative fluorescent diagnosis of residual hemangioblastoma using 5-aminolevulinic acid.

Hemangioblastoma is a benign tumor of the cerebellum, and treatment involves surgical excision, both as the initial treatment and also in case of recurrence. Recurrence of hemangioblastoma can be local due to incomplete resection or can be distant and separate from the tumor resection region. Local recurrence can largely be avoided by verifying for any residual tumor intraoperatively before closure. In this study, we used intraoperative fluorescent diagnosis using 5-aminolevulinic acid (5-ALA) to verify the presence of a residual tumor during surgical resection. Nine patients with hemangioblastoma were given 1 g of 5-ALA orally before surgery, and a laser beam of 405 nm was focused on the tumor during resective surgery. Fluorescence of protoporphyrin IX (PPIX) was observed in the core of tumor in all the cases. Fluorescence of PPIX was observed in the peritumoral cyst wall in two patients after tumor resection, and in both of them fluorescent parts of PPIX were resected and histological examination showed tumor cells. Usually, there are no tumor cells in the peritumoral cyst of a hemangioblastoma, yet hemangioblastomas may sometimes recur from an unresected cyst wall. It is thus necessary to excise an infiltrating cyst of tumor cells to prevent recurrence. Intraoperative fluorescent diagnosis using 5-ALA is a useful method to discern whether tumor cells are present in the peritumoral cyst wall of a hemangioblastoma.

[A case of open head injury caused by electric saw].

A rare case of traumatic injury caused by an electric saw is herein described. A 75-year-old male patient was admitted to our hospital as an emergency patient. At the time of the patient's admission, he was fully conscious with no neurological deficits. Computed tomography revealed an intracerebral hematoma in the right frontal lobe, traumatic subarachnoid hemorrhage and pneumocephalus. Emergency surgery was performed at 4 hours 30 minutes after the injury. After a right frontal craniectomy, the intracerebral hematoma was completely evacuated. We applied sufficient debridement. The patient had neither intracranial infection nor vascular injury. We believe that the early operation for the sake of safety was therefore effective for this patient.

[Dural scoring: an assistance technique for primary dural closure at craniotomy].

OBJECTIVE: At craniotomy the dura shrinks due to the drying effect of illumination and air exposure, rendering primary closure of this tissue difficult. We have developed a new technique, "dural scoring", that facilitates primary dural closure. PATIENTS AND METHODS: We used this technique in adults who underwent craniotomy when we encountered difficulties with primary dural closure of openings less than 5 mm in width. We placed scores along the edge of the opening on the surface of the dura (periosteal dura), taking care not to perforate the deep layer (meningeal dura). The dura relieved of tension expanded enough to be closed with sutures. RESULTS: This technique was successful in achieving primary dural closure in 53 patients who primarily underwent small supratentorial craniotomies. There were no procedure-related complications, e. g. cerebrospinal fluid leakages. CONCLUSIONS: Dural scoring is simple, requires no special instrumentation, and facilitates primary dural closure.

[Effect of Chai-ling-tang (Sairei-to) for methotrexate concentration degradation delay by methotrexate high-dose therapy].

When methotrexate (MTX) salvage chemotherapy is performed for primary brain malignant lymphoma, use of leucovonrin rescue must often be extended due to delays in the degradation of blood concentration. We examined whether delay in MTX blood concentration degradation could be prevented by chai-ling-tang (Sairei-to) which has diuretic action. In the five cases examined were MTX blood concentration 72 hours after MTX administration was more than 1 x 10(-7) M. A single dose of 3 g of chai-ling-tang was administered three times on the day the MTX salvage chemotherapy was subsequently performed. MTX blood concentration at 72 hours post MTX administration and subsequent chai-ling-tang administration was less than 1 x 10(-7) M in all five cases. In addition, urea nitrogen and creatinine levels in serum increased and creatinine clearance decreased following MTX administration, however these changes induced by MTX administration were reduced by chai-ling-tang administration. Chai-ling-tang was effective in preventing an MTX deferent delay in MTX high-dose therapy by improving renal blood flow.

Brachial plexopathy due to massive swelling of the neck associated with craniotomy in the park bench position.

BACKGROUND: During prolonged neurosurgical procedures, anesthetized patients are at risk for position-related complications. We report a rare combination of neck swelling and brachial plexopathy as operative position-related complications. CASE DESCRIPTION: This 56-year-old woman was placed in the left park bench position for removal of a tentorial meningioma in the right posterior fossa. At 2 hours after the 10-hour procedure, her left neck began to swell with progression during the next 10 hours to involve the face on the same side and the face and neck on the opposite side. Computed tomography showed swelling of the muscles and deep soft tissue primarily on the left. No brain edema was observed. She was conservatively treated with orotracheal intubation, placed in the head-up position, and received anticoagulants. Her swelling subsided by the 20th postoperative day; however, she manifested weakness in the proximal muscles of the left upper extremity. Magnetic resonance imaging revealed swelling of the brachial plexus on the left; electrophysiologic studies were compatible with damage to the upper trunk of the brachial plexus. She was discharged 2 months after surgery with improved weakness. CONCLUSION: Possible pathologic mechanisms are kinking of the jugular vein due to extremely flexed neck position during surgery and associated delayed swelling of the neck and brachial plexus. The cerebral venous return may have been maintained by anastomosis between the internal jugular and the vertebral venous system. To prevent such complications, we must take great care of the anesthetized patients when placed in the forced neck position.

Spinal cord tethering by aberrant nerve root in myelomeningocele: case report.

INTRODUCTION: A 2,796-gram male baby was born at 40 weeks gestation by vaginal delivery. Soon after, he was admitted at our department for treatment of a dorsal appendage. RESULTS AND DISCUSSION: Magnetic resonance (MR) imaging showed a low-position spinal cord, skin-covered myelomeningocele, syringomyelia, and a tethered cord. Resection of the caudal appendage and tethering tissue was performed 22 days after birth under electrophysiological monitoring. Histopathological examination of the caudal appendage that was covered with skin revealed the presence of spinal cord tissue and peripheral nerve tissue. The string-like tethering tissue that extended from the spinal cord to the caudalis was a nerve root, and there was fiberization of the caudalis. The syringomyelia was improved at postoperative MR imaging. To our knowledge, there is no report of a similar case in the literature. We report rare combination of myelomeningocele and spinal cord tethering by aberrant nerve root with thoracic syringomyelia.

Shortening of ventricular shunt catheter associated with cranial growth: effect of the frontal and parieto-occipital access route on long-term shunt patency.

OBJECT: The authors present the difference of shortening the ventricular shunt catheter associated with growth of the cranium between the frontal and parieto-occipital access, a key for long patency of the shunt implanted in children. MATERIALS AND METHOD: Our retrospective study included 28 children. In group A (n=9), the catheter was inserted through a frontal burr hole and in group B (n=19), through a parieto-occipital burr hole. To compare changes that occurred in the interval between the time of insertion and follow-up in the length of the ventricular catheter in the cranium and to assess displacement of the burr used for catheter entry. RESULTS: The results show that ventricular catheter shortening and burr-hole displacement were more pronounced in group A. CONCLUSIONS: This study documents that insertion of the ventricular catheter via the frontal route in children resulted in a higher incidence of shortening due to greater displacement of the burr hole adjacent to the coronal suture. Therefore, we recommend that the parieto-occipital route be used to maintain long-term shunt function.

Subarachnoid haemorrhage due to a lateral spinal artery aneurysm misdiagnosed as a posterior inferior cerebellar artery aneurysm: case report and literature review.

CLINICAL DESCRIPTION: A 61 year old man presenting with subarachnoid haemorrhage had a distal posterior inferior cerebellar artery (PICA) aneurysm diagnosed on the initial cerebral angiogram. However, a PICA aneurysm was not found during surgery. A follow-up, super-selective vertebral artery (VA) performed demonstrated a saccular aneurysm located on the lateral spinal artery (LSA). A right sub-occipital craniotomy was performed and the aneurysm arising was identified and obliterated with a small titanium clip. CONCLUSION: We describe the first example of an LSA aneurysm without occlusion or severe stenosis of the VA and PICA. This extremely rare lesion illustrates how knowledge of the angiographic features and super-selective cerebral angiography aids the precise diagnosis and the prevention of surgical complications.