Presence of intra-aneurysmal thrombi with delayed coil migration after embolization of an aneurysmal subarachnoid hemorrhage: illustrative case.

BACKGROUND: Coil migration is a rare, but notable complication of endovascular treatment. Risk factors include communicating segment aneurysms, aneurysmal shape, and technical factors. Although cerebral blood flow obstruction caused by early coil migration requires urgent coil removal, delayed coil migration is often asymptomatic, making it difficult to determine a treatment strategy. OBSERVATIONS: A 47-year-old woman was referred to the institute with acute-onset headache. She was diagnosed with subarachnoid hemorrhage due to rupture of the right internal carotid artery-posterior communicating artery aneurysm and underwent endovascular coil embolization. Following the procedure, the patient showed no obvious complications; however, 14 days later, images showed coil migration to the distal side, leading to surgical removal. Right frontotemporal craniotomy was performed, and the remaining coil was removed. The aneurysm was clipped again, and blood flow was confirmed. The patient was discharged 12 days after the craniotomy with transient oculomotor nerve palsy. At the 15-month follow-up, there was no aneurysm recurrence and the oculomotor nerve palsy showed improvement. LESSONS: Retrieval of the migrated coil by craniotomy is an effective remedial measure; however, intraoperative complications are common. Early detection, established protocols, and prompt treatment decisions are important for preventing undesirable outcomes.

Disappearance of a moyamoya-related distal anterior cerebral artery aneurysm after target bypass revascularization: illustrative case.

BACKGROUND: Aneurysm formation is a complication of moyamoya disease (MMD). Distal anterior cerebral artery (ACA) aneurysms account for approximately 1% of MMD-related aneurysms. We report a case of target bypass for adult patients with MMD who presented with intracranial hemorrhage due to rupture of a distal ACA aneurysm, whose disappearance was confirmed postoperatively. OBSERVATIONS: A 45-year-old woman presented with sudden-onset headache and loss of consciousness. Head computed tomography showed hemorrhage in the genu of the corpus callosum with intraventricular extension. Digital subtraction angiography (DSA) revealed Suzuki stage III MMD and a left A3 segment aneurysm. Superficial temporal artery (STA)-middle cerebral artery (MCA) bypass and STA-ACA target bypass were performed to reduce hemodynamic stress on the left ACA. DSA 6 months after surgery showed patency of both bypasses and disappearance of the aneurysm. At the 20-month follow-up, the patient was asymptomatic and neurologically intact. LESSONS: Bypass revascularization may be an effective treatment to reduce hemodynamic stress and eliminate MMD-related aneurysms.

Repeated coil embolization of traumatic basilar artery pseudoaneurysm accompanied by bilateral traumatic internal carotid artery stenosis following severe head injury in a pediatric patient: A case report and literature review.

Background: Few cases of pediatric traumatic intracranial aneurysms (pTICAs) in the posterior circulation involving the basilar artery (BA) following severe head trauma have been reported. Here, we describe a pediatric case of traumatic BA pseudoaneurysm accompanied by bilateral traumatic internal carotid artery (ICA) stenosis following blunt head trauma. Case Description: A 16-year-old boy presented to our emergency department after being hit by a car. The patient was initially diagnosed with multiple skull base fractures underlying traumatic subarachnoid hemorrhage and left acute epidural hematoma. Seven days following emergency craniectomy, magnetic resonance imaging revealed bilateral ICA stenosis, BA stenosis, and BA pseudoaneurysm. We decided to perform coil embolization, resulting in body filling and a volume embolization ratio of 15.7%. Twenty-eight days after coil embolization, digital subtraction angiography revealed aneurysmal rupture. We performed repeated coil embolization, resulting in body filling and a volume embolization ratio of 20.9%. Conclusion: We reported a pediatric case of traumatic BA pseudoaneurysm accompanied by bilateral traumatic ICA stenosis following a severe head injury treated with repeated coil embolization. Considering the risk of further brain injury due to high incidence of rupture, early vascular survey and appropriate treatment may be the most important prognostic factors in pTICAs.

Precision Mapping of Thalamic Deep Brain Stimulation Lead Positions Associated With the Microlesion Effect in Tourette Syndrome.

BACKGROUND: The microlesion effect refers to the improvement of clinical symptoms after deep brain stimulation (DBS) lead placement and is suggested to indicate optimal lead placement. Very few studies have reported its implications in neuropsychiatric disorders. OBJECTIVE: To evaluate the magnitude of the microlesion effect in Tourette syndrome and the relationship between the microlesion effect and the anatomic location of implanted DBS leads. METHODS: Six male patients were included. Their median age at surgery and follow-up period were 25 years (range, 18-47) and 12 months (range, 6-24), respectively. All patients were videotaped pre- and postoperatively, and tic frequencies were counted. We also analyzed the precision of lead placement and evaluated the normative connectome associated with the microlesion area. RESULTS: The microlesion effect was observed as an improvement in tic symptoms in all patients, and the long-term clinical outcomes were favorable. The median motor tic frequency was 20.2 tics/min (range, 9.7-60) at baseline and decreased to 3.2 tics/min (1.2-11.3) in patients on postoperative day 1 ( P = .043) and to 5.7 tics/min (range, 1.9-16.6) in patients on postoperative day 7 ( P = .028). Phonic tic tended to improve immediately after surgery although the changes were not significant. Image analyses revealed that the precise position of the electrode was directed toward the anteromedial centromedian nucleus. Normative connectome analysis demonstrated connections between improvement-related areas and wide areas of the prefrontal cortex. CONCLUSION: This study shows that the microlesion effect may seem as an immediate improvement after optimal DBS lead placement in patients with Tourette syndrome.

Case report: Radiofrequency thalamotomy as palliative care for Holmes tremor in a patient with terminal cancer and cardiac pacemaker.

Background: Herein, we present a case report of a patient with Holmes tremor due to thalamic infarction with end-stage pancreatic cancer who underwent successful computed tomography (CT)-guided ventralis intermedius nucleus (Vim) thalamotomy as palliative care. Case Description: A 78-year-old man with gradually worsening involuntary movements on the left side of his body 2 years after a right thalamic infarction was referred to our institute. He had a history of chronic atrial fibrillation for which he was implanted with a cardiac pacemaker not compatible with magnetic resonance imaging. He also received adjuvant therapy for pancreatic cancer. As the involuntary movements interfered with his daily life, the patient elected for neurosurgical treatment despite having terminal cancer. Although the prognosis for pancreatic cancer was considered to be more than 6 months at the time of surgery, we performed CT-guided Vim thalamotomy under local anesthesia without pulse generator implantation considering the patient's general condition. The involuntary movements of the left side of the body reduced following surgery, thus improving his quality of life (QOL). However, 6 months after thalamotomy, the patient died of pancreatic cancer. Conclusion: Thalamotomy significantly reduced the involuntary movements immediately after the procedure. Therefore, thalamotomy can be performed under local anesthesia without the use of any device and may contribute to the improvement of QOL in terminal patients.

Pain Relief Following Selective Tibial Neurotomy for Spastic Equinus Foot Secondary to Stroke and Traumatic Brain Injury.

OBJECTIVE: Selective tibial neurotomy (STN) has been indicated for spastic equinus foot (SEF); however, the impact of this procedure on quality of life and activities of daily living has not been evaluated in detail. This study aimed to evaluate the surgical outcomes of STN and its effect on SEF accompanied by pain. METHODS: We evaluated 26 patients (mean age: 59.6 ± 15.2 years; 14 men and 12 women) who underwent STN for SEF, 10 of whom complained of spontaneous pain preoperatively. We used the following scales for clinical evaluation: the Modified Ashworth Scale, Medical Research Council (MRC), 10-m walking test, Functional Independence Measure, and numeric rating scale for pain. These scales were evaluated preoperatively and postoperatively. Differences in clinical characteristics were compared between the 10 patients with pain and the 16 patients without pain. RESULTS: Significant differences were observed in all evaluation scale scores except for the Functional Independence Measure, and no serious adverse events were reported. Pain intensity was significantly improved from 6.4 ± 2.0 to 2.7 ± 2.3 (P < 0.05). An analysis showed that the preoperative mean Medical Research Council score of ankle movement was significantly lower in patients with pain but recovered to the same level postoperatively at the 6-month follow-up. CONCLUSIONS: Our study showed significant improvements in spasticity and its associated symptoms, and STN effectively addressed spastic pain and motor weakness. Among various treatment modalities, STN may be positively indicated for patients with spastic pain in the lower leg.

Favorable management of symptomatic cerebellar hemangioblastoma presenting with obstructive hydrocephalus during pregnancy: A case report and literature review.

Background: Cerebellar hemangioblastoma is a highly vascular benign tumor and the growth rate of hemangioblastomas is believed to often accelerate during pregnancy; however, the reason for this rapid increase in size remains poorly understood. There are several case reports of symptomatic hemangioblastoma during pregnancy; however, the favorable management strategy has not been well established. Case Description: A 35-year-old woman, gravida 2 para 1, with no significant medical history presented with vertigo and difficulty walking at around 11 weeks of pregnancy and was referred to our institute at 30 weeks of gestation because of worsening symptoms. Brain magnetic resonance imaging revealed a 5.6 cm cystic lesion with a mural nodule in the right cerebellar hemisphere and the lesion blocked cerebrospinal fluid drainage from the fourth ventricle and brainstem, resulting in obstructive hydrocephalus. After obtaining the patient's consent, a multidisciplinary team consisting of obstetricians and neurosurgeons decided to perform resection of the intracranial lesion following delivery of the fetus by emergency cesarean section in view of the symptoms of increased intracranial pressure. The patient's general condition was confirmed to be stable postoperatively and she was discharged on the 16th day of her hospitalization without any neurological deficits or fetal complications. Conclusion: Urgent tumor resection combined with cesarean section can be planned once fetal lung maturity is confirmed. Most cases of symptomatic hemangioblastoma during pregnancy have an uneventful gestational course and a favorable outcome for both mother and child.

Snowfall reduces the risk of chronic subdural hematoma onset: Analysis of an administrative database in Japan.

BACKGROUND: Chronic subdural hematoma (CSDH) is a frequently observed disease in neurosurgical practice. Although first snowfall has been considered to increase the onset of CSDH, few reports have assessed the relationship between snowfall and CSDH. In this study, we aimed to investigate the relationship between CSDH onset and first snowfall events. METHODS: This retrospective study was based on the Japanese Diagnosis Procedure Combination inpatient database from July 1, 2010, to March 31, 2016, and on the global observation of snowfall events in Japan obtained from the Japan Meteorological Agency. We used a binomial approximation to evaluate the average number of CSDH patients after first snowfall events compared with that before first snowfall events. We calculated the odds ratio of CSDH onset on the first snowfall. RESULTS: We identified 8526 CSDH patients from the database. A total of 5573 (65.4%) were observed before first snowfall events, and 2953 (34.6%) after first snowfall events. The first snowfall of winter was significantly associated with a reduction in the occurrence of CSDH (odds ratio, 0.53; 95% confidence interval; 0.51-0.55). In subgroup analysis, only the first snowfall in October was not associated with reduction in the occurrence of CSDH. CONCLUSION: First snowfall events did not affect the onset of CSDH in Japan.

Case report: Favorable outcomes of spinal cord stimulation in complex regional pain syndrome Type II consistent with thermography findings.

BACKGROUND: Complex regional pain syndrome (CRPS) is a chronic pain disorder that develops as a consequence of trauma to one or more limbs. Despite the availability of multiple modalities to diagnose CRPS, a gold standard technique for definitive diagnosis is lacking. Moreover, there are limited reports describing the use of spinal cord stimulation (SCS) to treat CRPS Type II, given the low prevalence of this condition. Herein, we present the case of a patient with CRPS Type II with novel thermography findings who underwent SCS for pain management after an Achilles tendon repair surgery. CASE DESCRIPTION: A 38-year-old woman was referred to our institute because of chronic left leg pain after Achilles tendon rupture repair surgery. Her case was diagnosed as CRPS Type II based on the International Association for the Study of Pain diagnostic criteria. After an epidural block, thermography showed a significant increase in the body surface temperature of the foot on the observed side. She was subsequently treated with SCS, following which her pain ameliorated. She reported no pain flare-ups or new neurological deficits over 2 years of postoperative follow-up assessments. CONCLUSION: SCS could be a useful surgical treatment for medication refractory CRPS Type II as supported by our thermography findings. We may refine surgical indication for permanent implantation of SCS with the presented method.

Percutaneous transluminal angioplasty for suspected vertebral artery stump syndrome.

Vertebral artery stump syndrome is a rare disease associated with a posterior circulation stroke after vertebral artery origin occlusion. However, few reports have addressed its management. We herein present a case involving a patient with vertebral artery stump syndrome who underwent successful intravascular intervention. We also present a literature review of previous cases of this rare disease. The present case involved a 91-year-old man with acute onset of vertigo and disturbance of consciousness. Diffusion-weighted imaging showed an acute ischaemic stroke in the bilateral cerebellar hemispheres. Magnetic resonance angiography revealed left vertebral artery origin occlusion. Angiography detected a nearly occluded left vertebral artery site, with distal antegrade collateral flow via the deep cervical artery at the C6 level. We observed intravascular stasis at the proximal end of the left vertebral artery via the collateral flow. We performed percutaneous transluminal angioplasty towards the occluded left vertebral artery site. The flow from the left vertebral artery was significantly improved. This is the first report of percutaneous transluminal angioplasty performed for vertebral artery stump syndrome. Although vertebral artery stump syndrome has a high risk of recurrence and a poor prognosis, endovascular intervention showed a better outcome than pharmacotherapy in our patient. However, such cases are rare and further investigations are needed.

Effects of vedolizumab in Japanese patients with Crohn's disease: a prospective, multicenter, randomized, placebo-controlled Phase 3 trial with exploratory analyses.

BACKGROUND: Vedolizumab is a gut-selective humanized antibody that binds the α4ß7 integrin. We evaluated efficacy and safety of vedolizumab in Japanese patients with moderate-to-severe Crohn's disease (CD). METHODS: In this Phase 3, double-blind study (NCT02038920), 157 patients were randomized to receive intravenous vedolizumab 300 mg (n = 79) or placebo (n = 78) at Weeks 0, 2, and 6 (induction phase). Patients with CD activity index (CDAI)-70 response at Week 10 were randomized to receive vedolizumab 300 mg (n = 12) or placebo (n = 12) at Week 14, then every 8 weeks until Week 54 (maintenance phase). Primary endpoints were ≥ 100-point reduction in CDAI (CDAI-100 response) at Week 10 for induction, and clinical remission (CR: CDAI ≤ 150) at Week 60 for maintenance. RESULTS: At Week 10, 26.6% of patients who received vedolizumab and 16.7% who received placebo achieved CDAI-100 response (odds ratio [OR] [95% confidence interval (CI)] 1.80 [0.82-3.96]; p = 0.145). At Week 60, 41.7% of vedolizumab-treated patients and 16.7% of placebo-treated patients achieved CR (OR [95% CI] 3.57 [0.53-23.95]; p = 0.178). The incidence of adverse events was similar in both treatment groups in both induction and maintenance phases. In patients without prior anti-TNFα exposure or with inadequate response to anti-TNFα, vedolizumab showed improved outcomes over placebo in the induction phase. Age might be a possible predictive factor of CR for future research. CONCLUSION: Vedolizumab showed a numerically greater efficacy versus placebo as induction therapy, but the difference was not statistically significant. Vedolizumab also showed a numerically greater efficacy in maintenance therapy, and was well tolerated.

Correction: Vedolizumab in Japanese patients with ulcerative colitis: A Phase 3, randomized, double-blind, placebo-controlled study.

[This corrects the article DOI: 10.1371/journal.pone.0212989.].

Vedolizumab in Japanese patients with ulcerative colitis: A Phase 3, randomized, double-blind, placebo-controlled study.

BACKGROUND: Vedolizumab safety and efficacy have been established in many populations all over the world, but have never been studied in Japan. We report results from a Phase 3, randomized, double-blind, placebo-controlled study of vedolizumab in Japanese patients with active ulcerative colitis (UC). METHODS: Patients with moderate-to-severe UC were enrolled into Cohort 1 (double-blinded) or Cohort 2 (open-label) in the induction phase. Cohort 1 was randomized 2:1 to receive 300 mg vedolizumab or placebo, while Cohort 2 received vedolizumab 300 mg only, at Weeks 0, 2, and 6. Patients from Cohorts 1 and 2 showing a clinical response to vedolizumab at Week 10 were randomized 1:1 to receive vedolizumab or placebo (double-blinded) at Week 14 and then every 8 weeks up to Week 54 as the maintenance phase. The primary endpoint was clinical response at Week 10, for the induction phase, and clinical remission at Week 60, for the maintenance phase. RESULTS: A total of 292 patients were enrolled into the induction phase (246 in Cohort 1, 46 in Cohort 2); 83 patients achieved response to vedolizumab and were subsequently enrolled into the maintenance phase. Clinical response rates at Week 10 were 39.6% (65/164) and 32.9% (27/82) in the vedolizumab and placebo groups in Cohort 1, respectively (adjusted odds ratio [AOR] = 1.37, 95% CI 0.779-2.399; p = 0.2722). In the maintenance phase, clinical remission rate at Week 60 was significantly higher in the vedolizumab group, at 56.1% (23/41), versus 31.0% (13/42) for placebo (AOR = 2.88, 95% CI 1.168-7.108; p = 0.0210). Most adverse events were mild to moderate in intensity, and no deaths occurred during the study period. CONCLUSIONS: Vedolizumab showed numerically greater efficacy compared with placebo as induction therapy, but the difference was not statistically significant. Vedolizumab was significantly superior to placebo as maintenance therapy in Japanese patients with UC. Vedolizumab has favourable safety and tolerability in these patients. TRIAL REGISTRATION: ClinicalTrials.gov: NCT02039505.

A Phase 1, Multiple-Dose Study of Vedolizumab in Japanese Patients With Ulcerative Colitis.

Although previous studies have shown that patients with ulcerative colitis may benefit from treatment with vedolizumab, a humanized monoclonal antibody targeting the α4 ß7 integrin heterodimer, no data exist in Japanese populations. The aim of this phase 1, open-label, multicenter study was to assess the pharmacokinetics, pharmacodynamics, efficacy, and safety of vedolizumab in Japanese patients with ulcerative colitis. Adult patients with confirmed ulcerative colitis received either 150 mg (step 1) or 300 mg (step 2) of intravenous (IV) vedolizumab on days 1, 15, and 43 of the study protocol. Pharmacokinetic, pharmacodynamic, safety, and efficacy parameters were all assessed through study end (day 239). Nine patients were enrolled in this study (150 mg, n = 3; 300 mg, n = 6). Patients who received vedolizumab IV 300 mg had approximately twice the drug exposure of those receiving vedolizumab IV 150 mg (day 1 AUCday14 744 vs 408 µg·d/mL) and a longer-lasting maximal saturation of α4 ß7 integrin (155 vs 99 days). The number of treatment-emergent adverse events, all of which were mild or moderate in intensity, was similar between the 150-mg (15 events) and 300-mg (20 events) groups. The 2 patients (150 mg group) not in clinical remission by partial Mayo score at the start of the study met the criteria for clinical remission on days 15 and 155 of the study, respectively. In conclusion, in Japanese patients with ulcerative colitis, vedolizumab showed similar pharmacokinetic and pharmacodynamic results to those seen in non-Japanese patients. Vedolizumab was well tolerated and demonstrated clinical activity consistent with previous studies.

Extracranial glioblastoma diagnosed by examination of pleural effusion using the cell block technique: case report.

Metastatic glioblastoma is a rare condition, and several studies have reported the involvement of multiple organs including the lymph nodes, liver, and lung. The lung and pleura are reportedly the most frequent sites of metastasis, and diagnosis using less invasive tools such as cytological analysis with fine needle aspiration biopsy is challenging. Cytological analysis of fluid specimens tends to be negative because of the small number of cells obtained, whereas the cell block technique reportedly has higher sensitivity because of a decrease in cellular dispersion. Herein, the authors describe a patient with a history of diffuse astrocytoma who developed intractable, progressive accumulation of pleural fluid. Initial cytological analysis of the pleural effusion obtained by thoracocentesis was negative, but reanalysis using the cell block technique revealed the presence of glioblastoma cells. This is the first report to suggest the effectiveness of the cell block technique in the diagnosis of extracranial glioblastoma using pleural effusion. In patients with a history of glioma, the presence of extremely intractable pleural effusion warrants cytological analysis of the fluid using this technique in order to initiate appropriate chemotherapy.

Prediction of recovery from supplementary motor area syndrome after brain tumor surgery: preoperative diffusion tensor tractography analysis and postoperative neurological clinical course.

OBJECTIVE Previous studies have suggested a correlation between interhemispheric sensorimotor networks and recovery from supplementary motor area (SMA) syndrome. In the present study, the authors examined the hypothesis that interhemispheric connectivity of the primary motor cortex in one hemisphere with the contralateral SMA may be important in the recovery from SMA syndrome. Further, they posited that motor cortical fiber connectivity with the SMA is related to the severity of SMA syndrome. METHODS Patients referred to the authors' neurological surgery department were retrospectively analyzed for this study. All patients with tumors involving the unilateral SMA region, without involvement of the primary motor area, and diagnosed with SMA syndrome in the postoperative period were eligible for inclusion. Preoperative diffusion tensor imaging tractography (DTT) was used to examine the number of fiber tracts (NFidx) connecting the contralateral SMA to the ipsilateral primary motor area via the corpus callosum. Complete neurological examination had been performed in all patients in the pre- and postoperative periods. All patients were divided into two groups: those who recovered from SMA syndrome in ≤ 7 days (early recovery group) and those who recovered in ≥ 8 days (late recovery group). Differences between the two groups were assessed using the Student t-test and the chi-square test. RESULTS Eleven patients (10 men, 1 woman) were included in the study. All patients showed transient postoperative motor deficits because of SMA syndrome. Tractography data revealed NFidx from the contralateral SMA to the ipsilateral primary motor area via the corpus callosum. The mean tumor volume (early 27.87 vs late 50.91 cm3, p = 0.028) and mean NFidx (early 8923.16 vs late 4726.4, p = 0.002) were significantly different between the two groups. Fisher exact test showed a significant difference in the days of recovery from SMA syndrome between patients with an NFidx > 8000 and those with an NFidx < 8000. CONCLUSIONS Diffusion tensor imaging tractography may be useful for predicting the speed of recovery from SMA syndrome. To the authors' knowledge, this is the first DTT study to identify interhemispheric connectivity of the SMA in patients with brain tumors.

[Chronic Low Back Pain Associated with Gluteus Medius Muscle:A Case Report].

The clinical features and etiology of low back pain(LBP)and buttock pain(BuP)has been poorly understood. We report a case of long-term BuP that was successfully treated with gluteus medius muscle(GMeM)decompression under local anesthesia. A 71-year-old man was referred to our hospital because of long-term BuP and claudication. Left BuP that radiated to the left thigh was observed. The pain was mostly triggered by palpation at the middle of the iliac crest and greater trochanter. Lumbar and pelvic radiograms showed no significant lesions. Lumbar magnetic resonance imaging revealed a mild lumbar spinal canal stenosis at the L4/L5 segment. Based on the evidence of a trigger point and pain relieved after GMeM block injection, we made a diagnosis of GMeM pain. Although several GMeM block injections relieved his pain, the analgesic effect was transient and the claudication remained. Then, we decided to perform GMeM decompression. We made a 5-cm-long skin incision across the trigger point on the buttock. After confirming a wide exposure of the gluteal aponeurosis over the GMeM, we cut and opened it for sufficient GMeM decompression, and the GMeM expansion was confirmed. After surgery, his symptoms immediately improved. No evidence of recurrence was observed 6 months after his treatment. For the treatment of LBP and BuP, GMeM pain would be considered a causative factor. We report that it can be treated with a less invasive surgical technique, which would contribute to good clinical outcome.

Hypertensive Cerebral Hemorrhage in a Patient with Turner Syndrome Caused by Deletion in the Short Arm of the X Chromosome.

Turner syndrome is a chromosomal disorder usually caused by complete deletion of an X chromosome, with deletion in the short arm of the X chromosome being a rare cause of the condition. Patients with Turner syndrome commonly develop hypertension, and associated vascular complications such as aortic dissection or cerebral hemorrhage have been reported. Cerebral hemorrhage in Turner syndrome is a rare complication, and only a few reports have been published. In these reports, all patients have XO karyotypes or a mosaic type as the cause of Turner syndrome, while no other Turner syndrome types have been documented. In this report, we present for the first time a patient with Turner syndrome caused by deletion in the short arm of the X chromosome who experienced hypertensive hemorrhage as a late complication.

Spinal Cavernous Angioma Associated with Klippel-Trenaunay-Weber Syndrome: Case Report and Literature Review.

BACKGROUND: Klippel-Trenaunay-Weber syndrome (KTWS) is a rare congenital vascular disorder characterized by the classic triad of cutaneous nevi, venous varicosities, and osseous and soft tissue hypertrophy of the affected limb. Various vascular anomalies of the central nervous system have also been described in patients with KTWS. The English language literature to date contains 6 reports of associations between KTWS and spinal cord cavernous angioma (CA), but management of these patients has not been well described. CASE DESCRIPTION: A 23-year-old woman was admitted to our institution with acute onset of leg weakness accompanied by upper back pain. Thoracic magnetic resonance imaging of the spinal cord showed a heterogeneous mass with a slit component at the T1-2 level. The patient underwent left hemilaminectomy followed by removal of the tumor, and her neurologic symptoms improved postoperatively. Pathologic examination showed the spinal lesion was characterized by hemosiderin deposition and thin-walled vascular channels surrounded by fibrous tissue. CONCLUSIONS: This is the first report to provide a detailed pathologic description of the features of spinal CA in a patient with KTWS. Assessment of the clinical features and management of CA associated with KTWS are discussed. This syndrome is rare, and further experience in the treatment of these patients is needed. However, considering that the pathologic findings of spinal CA in patients with KTWS include the typical features of CA, the management of CA in patients with KTWS may be identical to management of isolated CA.

Vonoprazan prevents low-dose aspirin-associated ulcer recurrence: randomised phase 3 study.

OBJECTIVE: Compare efficacy and safety of vonoprazan and lansoprazole for secondary prevention of low-dose aspirin (LDA)-associated peptic ulcers in a 24-week study and long-term extension therapy in separate study. DESIGN: Double-blind, randomised, non-inferiority study; single-blind extension study at 104 Japanese sites, including 621 patients (439 in extension) with a history of peptic ulcers who required long-term LDA therapy. Randomised (1:1:1, computer generated) patients received lansoprazole 15 mg (n=217), vonoprazan 10 mg (n=202) or vonoprazan 20 mg (n=202) once daily for 24 weeks (double blind) and ≤2 years (extension). The following measurements were made: 24-week (primary outcome; double blind) and 12-week peptic ulcer recurrence rate, 24-week GI bleeding rate, cumulative incidences of peptic ulcer recurrence and GI bleeding, treatment-emergent adverse events, laboratory results, serum gastrin and pepsinogen I/II concentrations. RESULTS: The 24-week peptic ulcer recurrence rate was 2.8%, 0.5% and 1.5% in the lansoprazole 15 mg, vonoprazan 10 mg and vonoprazan 20 mg groups, respectively. Vonoprazan was non-inferior (Farrington and Manning test: margin 8.7%, significance level 2.5%) to lansoprazole. In the post hoc analyses of the extension study, peptic ulcer recurrence rates were significantly lower with vonoprazan 10 mg (log-rank test, P=0.039), but not vonoprazan 20 mg (P=0.260), compared with lansoprazole 15 mg. GI bleeding rates were higher with lansoprazole compared with two doses of vonoprazan in both 24-week study and extension study. CONCLUSION: Vonoprazan (10 and 20 mg) was as effective as lansoprazole (15 mg) in preventing peptic ulcer recurrence during LDA therapy, had a similar long-term safety profile and was well tolerated. TRIAL REGISTRATION NUMBERS: NCT01452763; NCT01456247.