Design consideration on integration of mechanical intravascular ultrasound and electromagnetic tracking sensor for intravascular reconstruction.

PURPOSE: Considering vessel deformation, endovascular navigation requires intraoperative geometric information. Mechanical intravascular ultrasound (IVUS) with an electromagnetic (EM) sensor can be used to reconstruct blood vessels with thin diameter. However, the integration design should be evaluated based on the factors affecting the reconstruction error. METHODS: The interference between the mechanical IVUS and EM sensor was measured in different relative positions. Two designs of the integrated catheter were evaluated by measuring the reconstruction errors using a rigid vascular phantom. RESULTS: When the distance from the EM sensor to the field generator was 75 mm, the interference from mechanical IVUS to an EM sensor was negligible, with position and rotation errors less than 0.1 mm and 0.6°, respectively. The reconstructed vessel model for proximal IVUS transducer had a smooth surface but an inaccurate shape at large curvature of the vascular phantom. When the distance to the field generator was 175 mm, the error increased significantly. CONCLUSION: Placing the IVUS transducer on the proximal side of the EM sensor is superior in terms of interference reduction but inferior in terms of mechanical stability compared to a distal transducer. The distal side is preferred due to better mechanical stability during catheter manipulation at larger curvature. With this configuration, surface reconstruction errors less than 1.7 mm (with RMS 0.57 mm) were achieved when the distance to the field generator was less than 175 mm.

Diameters of lingual, facial, and maxillary arteries measured according to an objective protocol on 3D computed tomography angiography images.

PURPOSE: Retrograde superselective intra-arterial chemoradiotherapy is a radical treatment for advanced oral cancer. The catheter tip is placed into tumor-feeding arteries-the lingual, facial, or maxillary arteries. The diameter of the tumor-feeding arteries newly bifurcated from the external carotid artery is crucial for determining the requirement of a catheter navigation system. This study aimed to measure the diameter and distribution of the tumor-feeding artery according to an objective protocol using 3D computed tomography angiography images reproducibly. METHODS: Angiographic data of 20 noncatheterized carotid arteriesof 10 randomly selected patients were analyzed. We followed the external carotid artery to the entrance of each feeding artery to determine the center point where the artery diameter was measured. The diameter of the optimum circle measured at the adopted center point was taken as the diameter of each tumor-feeding artery. RESULTS: The diameters (mean ± standard deviation) were 3.5 ± 0.45, 2.9 ± 0.56, and 3.5 ± 0.56 mm for the maxillary, lingual, and facial arteries, respectively. The diameters of the maxillary and facial arteries were similar (p = 0.877), whereas the diameter of the lingual artery was smaller than that of the maxillary and facial arteries (p < 0.001). CONCLUSION: The findings of this study will be beneficial in determining the need of a new catheter navigation system and diameter of catheters to be used in the clinical practice. From the viewpoint of measurement automation and reproducibility, 3DCTA vessel measurement taken according to the proposed protocol was considered to be effective.

Severe RAS-Associated Lymphoproliferative Disease Case with Increasing αß Double-Negative T Cells with Atypical Features.

Autoimmune lymphoproliferative syndrome (ALPS) is a disease of lymphocyte homeostasis caused by FAS-mediated apoptotic pathway dysfunction and is characterized by non-malignant lymphoproliferation with an increased number of TCRαß+CD4-CD8- double-negative T cells (αßDNTs). Conversely, RAS-associated leukoproliferative disease (RALD), which is caused by gain-of-functional somatic variants in KRAS or NRAS, is considered a group of diseases with a similar course. Herein, we present a 7-year-old Japanese female of RALD harboring NRAS variant that aggressively progressed to juvenile myelomonocytic leukemia (JMML) with increased αßDNTs. She eventually underwent hematopoietic cell transplantation due to acute respiratory distress which was caused by pulmonary infiltration of JMML blasts. In general, αßDNTs have been remarkably increased in ALPS; however, FAS pathway gene abnormalities were not observed in this case. This case with RALD had repeated shock/pre-shock episodes as the condition progressed. This shock was thought to be caused by the presence of a high number of αßDNTs. The αßDNTs observed in this case revealed high CCR4, CCR6, and CD45RO expressions, which were similar to Th17. These increased Th17-like αßDNTs have triggered the inflammation, resulting in the pathogenesis of shock, because Th17 secretes pro-inflammatory cytokines such as interleukin (IL)-17A and granulocyte-macrophage colony-stimulating factor. The presence of IL-17A-secreting αßDNTs has been reported in systemic lupus erythematosus (SLE) and Sjögren's syndrome. The present case is complicated with SLE, suggesting the involvement of Th17-like αßDNTs in the disease pathogenesis. Examining the characteristics of αßDNTs in RALD, JMML, and ALPS may reveal the pathologies in these cases.

Difficulties of diagnosing idiopathic hypertrophic pachymeningitis in children: Case report and literature review.

Hypertrophic pachymeningitis (HP) is a rare inflammatory disorder characterised by local or diffuse thickening of the cranial and spinal dura mater. HP occurs owing to idiopathic or secondary causes, including autoimmune disease, infection, and trauma. HP has mainly been reported in adults, with few reported cases in children. We encountered an 11-year-old boy with idiopathic HP who presented with chronic inflammation and daily occipital headache. Gadolinium (Gd)-enhanced magnetic resonance imaging (MRI) helped us to diagnose him with HP. He was successfully treated with corticosteroids and azathioprine with no recurrence. We also conducted a literature review of childhood-onset HP and found only 16 cases, including our patient. Seven patients had idiopathic HP, and the remaining nine had secondary HP, including two with rheumatic disease. The most common clinical symptoms were headache (68.8%) and cranial nerve-related symptoms (68.8%). Inflammatory laboratory markers were elevated in 60% of patients with available data. Fifteen cases were diagnosed using Gd-enhanced MRI. The main initial treatment was steroids and/or immunosuppressants, to which 87.5% of patients responded. However, two patients with HP associated with trauma and neuroblastoma (12.5%) died, and seven patients (43.8%) had left cranial nerve-related sequelae. As the prognosis for childhood HP is poor, early diagnosis and treatment are essential. Children with headache, cranial nerve symptoms, and elevated inflammatory marker levels should be suspected of having HP and Gd-enhanced MRI should be considered.

A pilot study on an electromagnetic tracking system using tunneling magnetoresistance (TMR) sensors applicable to a 4F catheter (1.4 mm in diameter).

PURPOSE: Tracking the position and orientation of a 4F catheter ([Formula: see text] 1.4 mm) is required in superselective intra-arterial chemotherapy (SSIAC). Tunneling magnetoresistance (TMR) sensors, which measure magnetic fields, are promising candidates because the size of the TMR sensor can be less than a few tenths of a millimeter. The purpose of this paper is to prove the feasibility of an EMT system utilizing TMR sensors as magnetometers. METHODS: Three 1-axis TMR sensors (0.3 mm × 0.3 mm) were packaged on a flexible printed circuit board (PCB) together with an amplifier chip. The PCB was integrated into a 4F catheter. Six field generator coils driven by alternating current (AC) at different frequencies were used. Magnetic field measurement errors were evaluated to assess the effect of electromotive force (EMF) on TMR-based sensing by changing the coils' driving frequencies. The tracking error was also evaluated. As a result, the feasibility of catheter navigation utilizing the EMT system was demonstrated. RESULTS: There was a positive correlation between the frequency and the magnetic field measurement error using the TMR sensor (R2 = 0.999). With magnetic field frequencies less than 603 Hz, the average position and orientation estimation error were 10.1 mm and 2.3 degree, respectively. Under ideal conditions, the average estimation error values were 0.9 mm and 0.3 degree, respectively. CONCLUSION: The position and orientation errors varied with frequency owing to the induced electromotive force. We should consider the effect of electromotive force on TMR sensor assemblies caused by alternating magnetic fields. An EMT system using TMR sensors was validated, although room for further improvement was identified.

Laryngotracheal separation surgery in a patient with severe Angelman syndrome involving a 19.3 Mb deletion on 15q11.2-q14.

A severe Angelman syndrome (AS) patient with a very large deletion (19.3 Mb) at 15q11.2-q14 required laryngotracheal separation, which is not a common surgery in AS. Comparative genomic hybridization-based microarrays can be useful to confirm deletion size and clinical severity.

Applicability of a single camera-based catheter navigation system using teeth arch as an anatomical landmark for superselective intraarterial infusion in advanced oral cancer treatment.

Superselective intraarterial infusion chemoradiotherapy is a modality of oral cancer therapy in which the artery feeding the tumor is catheterized. 3D information about the carotid artery is required to enable the surgeon to judge whether to advance, retract, or rotate the catheter. For this purpose, we proposed and conducted a model experiment to assess a new method of catheterization that applies a tracking system using registration with a monocular camera using the maxillary arch as the anatomical landmark. In this method, the preoperative 3D computer tomography angiographic image of the carotid artery that the catheter will be passed through is overlaid on the 2D video image. The mean TRE was 0.96 ± 0.36 mm and 0.88 ± 0.31 mm and 1.12 ± 0.46 mm when images were registered with the anterior and posterior teeth as the landmarks, respectively; the difference was not significant (p = 0.21). This tracking system that enables markerless registration simply by taking images of the maxillary anterior teeth with a single camera was convenient and effective for catheterization. In this study, we propose the new application of this tracking system and a novel method of catheterization for superselective intraarterial infusion chemoradiotherapy for oral cancer. In retrograde superselective intraarterial catheterization, a catheter is inserted into a tumor-feeding artery originating from the external carotid artery (ECA) (the lingual artery [LA], facial artery [FA], or maxillary artery [MA]). Because the maxillary dentition is located near the external carotid artery, we focused on real-time markerless registration using maxillary dentition fixed to the skull.

Prognostic factors and treatment outcomes of advanced maxillary gingival squamous cell carcinoma treated by intra-arterial infusion chemotherapy concurrent with radiotherapy.

BACKGROUND: The aim of this study was to evaluate the prognostic factors and treatment outcomes of advanced maxillary gingival squamous cell carcinoma (SCC) treated with intra-arterial infusion chemotherapy concurrent with radiotherapy. METHODS: A total of 46 patients were reviewed retrospectively in this study. The treatment schedule comprised intra-arterial chemotherapy (total, 60 mg/m2 docetaxel and 150 mg/m2 cisplatin) and three-dimensional computed tomography based, daily conventional radiotherapy (total, 60 Gy/30 fr) for 6 weeks. RESULTS: The median follow-up period was 40 months (range, 3-110 months). The 3-year overall survival and locoregional control rates for all patients were 64.3% and 84.3%, respectively. The OS rate of the patients with N0-1 was significantly higher than that of the patients with N ≥ 2 (P < .05). No grade 5 toxicities were observed. CONCLUSIONS: Intra-arterial infusion chemotherapy concurrent with radiotherapy was effective for advanced maxillary gingival SCC.

Daily Cisplatin and Weekly Docetaxel versus Weekly Cisplatin Intra-Arterial Chemoradiotherapy for Late T2-3 Tongue Cancer: A Pilot and Feasibility Trial.

Background and objectives: The aim of present study was to compare the treatment results of daily cisplatin (CDDP), weekly docetaxel (DOC) intra-arterial infusion chemotherapy combined with radiotherapy (DIACRT) regimen and weekly CDDP intra-arterial infusion chemotherapy combined with radiotherapy (WIACRT) for patients with tongue cancer. Materials and Methods: Between January 2007 and December 2016, a total of 11 patients treated with WIACRT and 45 patients treated with DIACRT were enrolled in the present study. In the DIACRT group, 25 patients had late T2, and 20 patients had T3. A total of nine patients had late T2 and two had T3 in WIACRT (p = NS). In DIACRT, the treatment schedule consisted of intra-arterial chemotherapy (DOC, total 60 mg/m²; CDDP, total 150 mg/m²) and daily concurrent radiotherapy (RT) (total, 60 Gy). In WIACRT, the treatment schedule consisted of intra-arterial chemotherapy (CDDP, total 360 mg/m²) and daily concurrent RT (total, 60 Gy). Results: The median follow-up periods for DIACRT and WIACRT were 61 and 66 months, respectively. The five-year local control (LC) and overall survival (OS) rate were 94.5% and 89.6% for the DIACRT group, and 60.6% and 63.6% for the WIACRT group, respectively. The LC rate and OS of the DIACRT group were significantly higher than those of the WIACRT group. As regards toxicities, no treatment-related deaths were observed during the follow-up periods in both groups. Conclusions: DIACRT was found to be feasible and effective for patients with tongue cancer and could become a new treatment modality.

Childhood-onset inflammatory bowel diseases associated with mutation of Wiskott-Aldrich syndrome protein gene.

AIM: To screen primary immunodeficiency, Wiskott-Aldrich syndrome (WAS), and chronic granulomatous disease (CGD) among children with inflammatory bowel disease (IBD). METHODS: This was a single-center retrospective study. Eighteen children with IBD were investigated. We analyzed their expression of Wiskott-Aldrich syndrome protein (WASP) in lymphocytes and superoxide generation in phagocytes using flow cytometry. When the expression of WASP or superoxide generation was low or absent, we performed genetic analysis to determine the cause of this. RESULTS: Eighteen patients were classified as having ulcerative colitis (n = 10), Crohn's disease (n = 5), or IBD-unclassified (n = 3). In total, three patients revealed low expression of WASP associated with a WAS gene c.1378 C>T p.Pro460Ser mutation, which has previously been reported as a pathogenic mutation in WAS and X-linked thrombocytopenia. However, with respect to the major symptoms of WAS, none of these three patients showed either thrombocytopenia or increased susceptibility to infection, but one patient showed generalized eczema. No CGD patients were discovered in this study. CONCLUSION: Despite the lack of typical clinical manifestations of WAS, low expression of WASP could be associated with the pathogenesis of a subtype of IBD patients.

The Potential and Limits of Hematopoietic Stem Cell Transplantation for the Treatment of Autosomal Dominant Hyper-IgE Syndrome.

PURPOSE: Autosomal dominant hyper-IgE syndrome (AD-HIES) is included among primary immunodeficiencies, and results from heterozygous mutations in the signal transduction and activator of transcription 3 (STAT3) gene. AD-HIES leads to impaired Th17 cell differentiation and IL-17 production, and is associated with increased susceptibility to bacteria and fungi. It was reported that several patients with AD-HIES were treated with hematopoietic stem cell transplantation (HSCT). The efficacy of HSCT in treating AD-HIES is variable. This study aims to evaluate the long-term clinical and immunological efficacy of HSCT for AD-HIES. METHODS: We have followed for more than 8 years two patients with AD-HIES who were treated with HSCT. Their ability of IL-17 production was evaluated by flow cytometry. RESULTS: Both patients indicated the normal ability of IL-17 production and their serum IgE levels decreased after HSCT. On the other hand, they suffered from pulmonary complications of AD-HIES such as pneumatoceles and bronchiectasis even after HSCT; however, the frequency of infections was decreased. CONCLUSIONS: Although the dysfunction of STAT3 in non-hematological tissues such as the lungs could not be corrected by HSCT, AD-HIES patients with risk factors for pulmonary complications may benefit from immunological correction by HSCT before severe pulmonary complications occur. Future studies should investigate risk factors for pulmonary complications in AD-HIES patients.

Pathological crying and emotional vasovagal syncope as symptoms of a dorsally exophytic medullary tumor.

A 3-year-old boy with a dorsally exophytic tumor arising from the rostral medulla presented with a chief complaint of a change in his emotional behavior, most notably anxiety and paroxysmal crying often followed by syncope. Magnetic resonance imaging revealed that the tumor pushed on the dorsal surface of the medulla and displaced the medulla anteriorly, and also displaced the cerebellar vermis upward and slightly posteriorly. Tissue from a partial resection was diagnosed as a pilocytic astrocytoma. The symptoms did not improved after surgery, but did improve clinically after chemotherapy with vincristine and carboplatin, at which time MR showed a reduction in tumor size. We diagnosed the paroxysmal crying as 'pathological crying' and the syncope with increased anxiety as 'emotional vasovagal syncope'. This case stresses the importance of recognition of this rare presentation as an indication of a medullary tumor.

Vessel bifurcation localization based on intraoperative three-dimensional ultrasound and catheter path for image-guided catheter intervention of oral cancers.

We present a method to localize intraoperative target vessel bifurcations under bones for ultrasound (US) image-guided catheter interventions. A catheter path is recorded to acquire skeletons for the target vessel bifurcations that cannot be imaged by intraoperative US. The catheter path is combined with the centerlines of the three-dimensional (3D) US image to construct a preliminary skeleton. Based on the preliminary skeleton, the orientations of target vessels are determined by registration with the preoperative image and the bifurcations were localized by computing the vessel length. An accurate intraoperative vessel skeleton is obtained for correcting the preoperative image to compensate for vessel deformation. A reality check of the proposed method was performed in a phantom experiment. Reasonable results were obtained. The in vivo experiment verified the clinical workflow of the proposed method in an in vivo environment. The accuracy of the centerline length of the vessel for localizing the target artery bifurcation was 2.4mm. These results suggest that the proposed method can allow the catheter tip to stop at the target artery bifurcations and enter into the target arteries. This method can be applied for virtual reality-enhanced image-guided catheter intervention of oral cancers.

Analysis of carotid artery deformation in different head and neck positions for maxillofacial catheter navigation in advanced oral cancer treatment.

BACKGROUND: To improve the accuracy of catheter navigation, it is important to develop a method to predict shifts of carotid artery (CA) bifurcations caused by intraoperative deformation. An important factor affecting the accuracy of electromagnetic maxillofacial catheter navigation systems is CA deformations. We aimed to assess CA deformation in different head and neck positions. METHODS: Using two sets of computed tomography angiography (CTA) images of six patients, displacements of the skull (maxillofacial segments), C1-C4 cervical vertebrae, mandible (mandibular segment), and CA along with its branches were analyzed. Segmented rigid bones around CA were considered the main causes of CA deformation. After superimposition of maxillofacial segments, C1-C4 and mandible segments were superimposed separately for displacement measurements. Five bifurcation points (vA-vE) were assessed after extracting the CA centerline. A new standardized coordinate system, regardless of patient-specific scanning positions, was employed. It was created using the principal axes of inertia of the maxillofacial bone segments of patients. Position and orientation parameters were transferred to this coordinate system. CA deformation in different head and neck positions was assessed. RESULTS: Absolute shifts in the center of gravity in the bone models for different segments were C1, 1.02 ± 0.9; C2, 2.18 ± 1.81; C3, 4.25 ± 3.85; C4, 5.90 ± 5.14; and mandible, 1.75 ± 2.76 mm. Shifts of CA bifurcations were vA, 5.52 ± 4.12; vB, 4.02 ± 3.27; vC, 4.39 ± 2.42; vD, 4.48 ± 1.88; and vE, 2.47 ± 1.32. Displacements, position changes, and orientation changes of C1-C4 segments as well as the displacements of all CA bifurcation points were similar in individual patients. CONCLUSIONS: CA deformation was objectively proven as an important factor contributing to errors in maxillofacial navigation. Our study results suggest that small movements of the bones around CA can result in small CA deformations. Although patients' faces were not fixed properly during CT scanning, C1-C4 and vA-vE displacements were similar in individual patients. We proposed a novel method for accumulation of the displacement data, and this study indicated the importance of surrounding bone displacements in predicting CA bifurcation.

Intravascular catheter navigation using path planning and virtual visual feedback for oral cancer treatment.

BACKGROUND: It is tedious and difficult to position a flexible catheter in a target vessel branch within complicated-shaped vessels owing to the lack of real-time visual feedback. Digital subtraction angiography and fluoroscopic guidance are currently used for catheter placement. METHODS: The proposed method employs an electromagnetic (EM) tracking system to track a sensor-attached catheter. Vessel centerlines are extracted from the CT angiography image, based on which a navigational information tree is built to facilitate catheter manipulation. A virtual endoscopy technique is adopted to generate virtual intravascular video as visual feedback. Unscented Kalman filtering based image registration is performed to align the EM tracker frame with the anatomical atlas and to envision the target registration error. RESULTS: Preliminary experimental results showed the feasibility and effectiveness of the new method, with navigation accuracy of 1.80 ± 0.85 mm. CONCLUSIONS: The proposed method can provide continuous virtual visual feedback to facilitate catheter placement and has the potential for clinical use, with significant reduction in X-ray radiation exposure and doses of contrast agents.

Right ventricular failure due to primary right ventricle osteosarcoma.

A 68-year-old woman with symptoms of dyspnea and peripheral edema was referred to our hospital. Chest computed tomography (CT) scans revealed a huge mass occupying the pulmonary trunk and invading the right main pulmonary artery, with metastatic nodules in the left main and left lower pulmonary artery. She was given a diagnosis of pulmonary thromboembolism and was anticoagulated to no effect, which suggested a neoplasm. Palliative resection of the tumor was carried out even though she was in serious condition with right ventricular failure, liver congestion, renal dysfunction, and coagulopathy disorder. The histopathology, postoperative systemic CT scan, and bone scintigram provided a definitive diagnosis of a primary right ventricle osteosarcoma. Primary cardiac osteosarcoma has a poor prognosis, and this patient was at an advanced stage with pulmonary metastases. Surgical intervention should offer these patients significant palliation to relieve the clinical symptoms due to obstruction.

A case of tuberculous endocarditis with acute aortic valve insufficiency and annular subvalvular left ventricular aneurysm.

Tuberculous endocarditis is an exceptionally rare disease, and its surgical operation has been reported in only one case. This is a successful surgical report of acute aortic insufficiency caused by tuberculous endocarditis associated with annular subvalvular left ventricular aneurysm (ASLVA) beneath the aortic valve. This patient had acute left ventricular failure and cardiorespiratory arrest and suffered from multiple organ dysfunction and coagulopathy disorder. Urgent surgery was performed to replace the aortic valve with the approximation of ASLVA. Histopathological findings of the excised aortic cusps gave the diagnosis of tuberculous endocarditis, and antituberculous drug administration started on the first postoperative day. Postoperative recovery has been uneventful without relapse of tuberculosis for 7 postoperative years.