Clinical and cytogenetic observations during a six-year period in an adult with Fanconi's anaemia

Summary A male adult patient suffering from Fanconi's anemia is described who was diagnosed 5 years before the onset of clinical symptoms by cytogenetic findings of chromosomeinstability in a lymphocyte culture. Repeated clinical, haematological and biochemical investigations of the untreated patient have been made during the observation period of six years. In the same period of time cytogenetic studies have been carried out which show no correlation in results compared with the clinical or physical findings. Four well defined lymphocyte clones have been discovered. The patient is still under observation of the clinic and the cytogenetic department.This work was supported by the Deutsche Forschungsgemeinschaft.     

DSA血流定量分析软件评价血流导向装置治疗颅内大型动脉瘤的有效性

目的 探讨DSA血流定量分析软件评价血流导向装置治疗颅内大型动脉瘤后的血流特性改变,以分析血流导向装置治疗颅内大型动脉瘤的有效性.方法 回顾性分析2012年8月-2013年4月采用血流导向装置（Tubridge,Microport,上海）治疗的颅内大型动脉瘤患者15例.以相同标准采集所纳入患者治疗前后常规造影图像,并通过DSA血流定量分析软件进行图像后处理,通过生成的时间密度曲线,分析载瘤动脉远端显影延迟时间、动脉瘤瘤体内血流达峰时间、瘤体内血流曲线下面积以及瘤体内血流最大斜率的变化情况.结果 本组患者的支架置入技术成功率为100%,通过DSA分析软件术后即刻分析发现,与术前相比,载瘤动脉远端延迟时间缩短中位数（M）1.031 s（范围0.324~2.143 s）,动脉瘤瘤腔内血流曲线下面积（相对值）以及最大斜率（相对值）分别减少57±15和49±25.结论 Tubridge支架置入前后载瘤动脉远端显影时间延迟、瘤腔内血流曲线下面积及瘤腔内血流最大斜率的下降,证实采用血流定量分析方法评估血流导向装置对于颅内大型动脉瘤的即刻治疗效果是有效的,但其对于远期疗效的评估还有待进一步观察.     

Anastomotic obstruction after magnetic vascular coupling after minimally invasive direct coronary artery bypass grafting

We report a 61-year-old man who required reoperation 8 months after minimally invasive direct coronary artery bypass grafting after magnetic vascular coupling due to a symptomatic subtotal obstruction at the anastomotic site. It was also determined that the patient had been noncompliant in following the prescribed postoperative antiplatelet therapy.     

Objective Assessment of Transcatheter Arterial Chemoembolization Angiographic Endpoints: Preliminary Study of Quantitative Digital Subtraction Angiography

PurposeTo explore the significance of quantitative digital subtraction angiography (DSA; Q-DSA) in the assessment of chemoembolization endpoints.Materials and MethodsTwenty patients with hepatocellular carcinoma treated with chemoembolization were included in the study. All DSA series before and after chemoembolization were postprocessed with Q-DSA. The maximal enhancement and time to peak (TTP) were measured for several homologous anatomic landmarks, including the origin and embolized site of the tumor-feeding artery, parenchyma of the tumor, and ostia of the pre- and postprocedure catheter. The TTP, tumor blood supply time, and maximal enhancement of the time density curve (TDC) were analyzed.ResultsOf the 20 DSA series collected, 18 were successfully postprocessed. The TTPs of the landmarks before and after treatment were 3.60 seconds±1.02 and 3.57 seconds±0.78 at the ostia of the catheter, 3.91 seconds±1.01 and 4.09 seconds±1.14 at the origin site of the tumor-feeding artery, and 4.07 seconds±1.02 and 5.60 seconds±1.56 s the embolized site of the main tumor-feeding artery, respectively. Statistical differences were detected between pre- and postprocedural TTP of the embolized site of the feeding artery (P<.01), as well as between pre- and postprocedural tumor blood supply time (P<.01). The mean maximal TDC enhancements of selected tumor spots were 3.01 units±1.04 and 0.81 units±0.35 before and after the procedure (P<.01), respectively.ConclusionsQ-DSA may provide a feasible quantitative measurement in the assessment of chemoembolization endpoints.     

Exploring the Value of Using Color-Coded Quantitative DSA Evaluation on Bilateral Common Carotid Arteries in Predicting the Reliability of Intra-Ascending Aorta Flat Detector CT–CBV Maps

BACKGROUND AND PURPOSE:Cerebral blood volume, acquired with flat panel detector CT by injecting contrast medium into the ascending aorta, enabled real-time acquisition of brain functional information with remarkable reduction of contrast medium usage comparing to an intravenous injection approach. However, individual vasculature and flow variations cause inhomogeneous contrast medium distribution and unexpected asymmetric perfusion for certain patients even without cerebral circulatory disorders. This work aimed at testing the feasibility of using color-coded quantitative DSA to predict the reliability of flat panel detector CT–based CBV maps by injecting contrast medium into the ascending aorta by exploring the correlation between measurements of color-coded quantitative DSA and the symmetry of CBV maps.MATERIALS AND METHODS:For 12 patients without perfusion-related cerebral abnormities, color-coded quantitative DSA at the aortic arch and flat panel detector CT–based CBV maps by injecting contrast medium into the ascending aorta were acquired. In color-coded quantitative DSA, ROIs were defined on the bilateral common carotid arteries. Time-density curves were extracted, and area under the curve values were calculated. To evaluate brain perfusion symmetry, we defined ROIs on the anterior and middle cerebral artery territories in CBV maps, and quantitative CBV values were extracted.RESULTS:Eight patients demonstrated good perfusion symmetry with relative CBV of 0.96 ± 0.06, and their relative area under the curve was found to be 0.99 ± 0.02. For the other 4 patients, CBV from the left hemisphere was significantly lower than that from the right with relative CBV of 0.81 ± 0.09. This asymmetric perfusion was confirmed by the color-coded quantitative DSA with relative area under the curve values of 0.79 ± 0.03.CONCLUSIONS:This preliminary study showed good correlation between relative area under the curve from color-coded quantitative DSA and relative CBV from CBV maps. Color-coded quantitative DSA potentially helped sort out patients whose vascular anatomy could support reliable CBV acquisitions of flat detector CT by injecting contrast medium into the ascending aorta.

Angiographic suites equipped with flat panel detector CT (FPCT) for clinical imaging practice have been increasingly accepted during neurointerventions. This technique offers significant advantages in patient management because the patient does not have to be transferred between different clinical units for diagnosis and treatment, improving clinical workflow and increasing patient safety.^1–3 Continuous development in imaging technology has led to significant improvement of image acquisition and postprocessing tools in FPCT. Recently, an important brain perfusion parameter, CBV (milliliter/1000 milliliter), can be acquired by using FPCT. It has been suggested that a color-coded CBV map, which displays hemodynamic information in the capillary level of the entire brain parenchyma, provides a measurement of regional CBV variations and information on suspected ischemia, which is of great value for patients with acute ischemic stroke.^4,5 The CBV value measured with this technique has been validated in a number of clinical studies, proving that the CBV maps generated from FPCT correlated well with CT perfusion maps from multisection CT, which were regarded as ground truth.^6–9 This technique offered the possibility of obtaining functional information and assessing the viability of the brain tissue during interventional procedures.¹⁰Advancements in protocols of contrast medium (CM) injection and image acquisition have been further investigated to achieve an optimized whole-brain CBV map acquisition pipeline by using FPCT.^11–14 A number of studies have demonstrated the feasibility of obtaining CBV maps via intravenous CM injection by using FPCT. However, with this injection protocol, the CM will be diluted during the transit, thus only a small portion of the injected CM contributed to the formation of CBV maps. In contrast, intra-arterial administration has become an attractive method to increase the CM delivery and efficiency and may provide a viable approach for reducing the amount of CM used during FPCT CBV imaging. The feasibility and clinical value of intra-ascending aorta CM injection to acquire FPCT CBV maps (IA-FPCT-CBV) have been tested and confirmed; this approach enabled remarkably reduced CM usage relative to the intravenous CM injection protocol (350 mg I/mL; total amount, 96 mL; 20% diluted versus 60–80 mL, nondiluted) by using FPCT. Moreover, the reliability of this intra-arterial injection approach has also been validated against conventional CT perfusion imaging with intravenous CM injection.¹⁵However, nonuniform CM distribution could be observed for a number of patients even without cerebral circulatory disorders, causing unexpected asymmetric perfusion in 2 hemispheres, mainly in the territories of the anterior and middle cerebral arteries.^15,16 A possible explanation for nonuniform distribution could be patients'' individual vasculature variations at the aortic arch, high blood flow speed, and the limited length of the ascending aorta.Currently, color-coded quantitative digital subtraction angiography (CCQ-DSA) could be generated from a conventional DSA sequence at no additional radiation exposure or CM use, which enhanced the recognition of hemodynamic and morphologic findings. This technique provides a means to display temporal information of contrasted blood flow and attenuation for the duration of the DSA acquisition in a single color-coded image. Moreover, this image allowed the creation of a time-density curve (TDC) of a CM bolus in user-defined regions, from which quantitative blood flow information could be also extracted.^17–19This work aimed at exploring the correlation between measurement of CCQ-DSA on the main feeding arteries and symmetry of CBV maps and testing the feasibility of using CCQ-DSA to predict the reliability of IA-FPCT-CBV on a patient-specific basis before the acquisition.     

Treatment of Supratentorial Spontaneous Intracerebral Hemorrhage Using Image-Guided Minimally Invasive Surgery: Initial Experiences of a Flat Detector CT–Based Puncture Planning and Navigation System in the Angiographic Suite

BACKGROUND AND PURPOSE:The intracerebral hemorrhage drainage through minimally invasive approach is emerging as an alternative for traditional craniotomy, due to its improved survival rate and reduced complication rate. In this study, we investigated the feasibility and safety of a flat detector CT–based puncture planning and navigation system for minimally invasive hematoma drainage on patients with intracerebral hemorrhage.MATERIALS AND METHODS:The minimally invasive hematoma drainage was performed on 21 hypertensive patients with intracerebral hemorrhage in the angiographic suite with the guidance of a flat detector CT–based puncture planning and navigation system. This system is integrated in the angiographic machine, and was used for 1) planning the needle path based on a preprocedural flat detector CT scan, 2) advancing the catheter with real-time fluoroscopic guidance, and 3) confirming the procedure outcome based on an immediate postprocedural flat detector CT. The surgery efficiency, accuracy, and the treatment outcome were measured and compared with the published data.RESULTS:All procedures were successfully completed with the catheter placed 4 ± 1 mm from the planned position. The average surgery time was 40 ± 7 minutes. The volume of the hematoma was reduced to 28 ± 4% of the original volume. The Glasgow Coma Scale score was significantly improved from 10 ± 1 at the admission to 14 ± 1 at the discharge. The Extended Glasgow Coma Scale score also improved from 5 ± 1 at the discharge to 6 ± 1 at the 6-month follow-up. No major complication, rebleeding, and mortality were observed in this study.CONCLUSIONS:This flat detector CT–based needle guidance system provided a feasible, convenient, and safe way to perform the puncture and drainage of brain hematoma in the angiographic suite.

Spontaneous intracerebral hemorrhage (ICH) affects more than 2 million people each year around the world.¹ Timely medical intervention is critical for ICH, as it is a medical emergency with a mortality rate range from 35% to 52% within 1 month.² However, the current treatments for patients with ICH continue to be controversial, with several large-scale randomized trials reporting no significant benefit of early surgery, primarily craniotomy, over conservative treatment.^3–5 In recent years, the surgical removal of hematoma through burr-holes is emerging as a minimally invasive alternative for conventional craniotomy because of its improved complication rate and survival rate.⁶ These new techniques usually involve stereotactic positioning and intraprocedural image guidance, such as endoscopic, traditional CT, intraoperative CT, and sonography-guided aspiration.^6–8 These techniques have not yet been widely used because of expensive clinical costs such as the demand for specialized equipment, difficulties scheduling procedures on the diagnostic machines (eg, CT scan suite), and a lack of confirmed clinical benefit from large-scale clinical trials. Until now, the implementation of minimally invasive surgery for treating intracranial hemorrhage remains investigational, with only 2 ongoing large-scale randomized trials (Minimally Invasive Surgery and Thrombolytic Evacuation in ICH [MISTIE] and Clot Lysis Evaluating Accelerated Resolution III [CLEAR III]).^9,10 MISTIE and CLEAR III are currently phase III trials aimed at comparing minimally invasive surgery combined with a thrombolysis agent with the best critical management alone for treating ICH, and with minimally invasive surgery combined with placebo for treating intraventricular hemorrhage, respectively. Both of their early results indicated that the catheter location is one of the most important criteria for good surgery outcome, and careful planning and accurate execution is necessary for optimal catheter placement.¹⁰In this study, we attempted an affordable clinical solution for image-guided hematoma drainage that could be performed in the traditional angiographic suite without the need for specialized equipment. We implemented an innovative puncture planning and navigation system that is integrated into the angiographic machine for hematoma evacuation. This guided evacuation procedure based on an intraprocedural flat detector CT (FDCT), in which a 3D CT-like image was reconstructed from a rotational scan¹¹using a C-arm angiographic machine. Commercial software (syngo iGuide needle guidance; Siemens, Erlangen, Germany) then facilitates the needle path planning that allows physicians to choose optimum entry and target points of the puncture, and automatically overlays the path onto the fluoroscopic images during the procedure for real-time guidance.The feasibilities of this puncture planning and guidance system had been previously demonstrated in selective cervical nerve root block,¹² percutaneous kidney puncture,¹³ and hepatic tumor ablation¹³ based on ex vivo animal models and real patients. Its application in the brain was only recently demonstrated on 6 patients with cerebrovascular ischemia for external ventricular drainage.¹⁴ In this study, we further expanded this technique on a more complicated brain procedure, intracranial hemorrhage evacuation in 21 hypertensive patients with ICH. Unlike the external ventricular drainage, the location of hemorrhage in patients with ICH could vary from patient to patient, and could not be performed freehand without any image guidance. Moreover, we assessed the feasibility and the safety of this technique in terms of procedure length, puncture accuracy, drainage volume, and treatment outcome at the discharge and 6-month follow-up. This is also the first attempt to evaluate the short-term treatment outcome of FDCT-guided hemorrhage drainage using Glasgow Coma Scale (GCS) and Extended Glasgow Coma Scale (GOSE) scores.     

Preliminary clinical experience with the Ventrica automatic distal anastomosis system in coronary surgery

The surgical treatment of coronary lesions is based on bypassing the anatomical lesions with autologous vascular grafts. This procedure has traditionally been "invasive", requiring a micro-surgery technique, institution of extra-corporeal circulation, as well as temporary cardiac arrest with a cardioplegic solution. Recently, an automatic distal anastomosis procedure has been developed (Ventrica, Medtronic Inc.), based on a magnetic coupling with two implanted intravascular magnets, allowing easy connection between the graft and the coronary artery. The immediately obvious advantages are the time saved, ease of use, reproducibility and reliability. The learning curve is fast. Furthermore, the use of this automatic process does not compromise a manual anastomosis in case of implantation failure. The immediate post-operative results, as well as angiography immediately and at 6 months are all satisfactory. This technique is applicable for multiple revascularisations, all types of autologous grafts, terminal or sequential bypasses, as well as nearly all types of coronaries. The contribution to beating heart, closed thorax coronary surgery seems equally promising.     

Application of color-coded digital subtraction angiography in treatment of indirect carotid-cavernous fistulas: Initial experience

BackgroundParametric-colored digital subtraction angiography using Tmax is almost a routine angiographic imaging procedure, currently. The current feasibility study is aimed to using the imaging to monitor treatment effects while embolizing indirect carotid-cavernous fistulas (CCF).MethodsTen patients with CCFs receiving embolization and 40 patients with normal circulation time were recruited. Their color-coded DSAs were used to define the Tmax of selected intravascular ROIs. A total of 19 ROIs in the internal carotid artery (ICA) (cervical segment of ICA in AP view (I0), cavernous segment of ICA in AP view (I1), supraclinoid segment of ICA in AP view (I2) and cervical segment of ICA in lateral view (I0′), cavernous portion of ICA in lateral view (IA), supraclinoid portion of ICA in lateral view (IB)), ACA (first segment of anterior cerebral artery, second segment of anterior cerebral artery (A1, A2)), middle cerebral vein (MCA) first segment of MCA ((M1), second segment of MCA (M2)), frontal vein (FV), parietal vein (PV), superior sagittal sinus (SSS), sigmoid sinus (SS), internal jugular vein (JV), fistula, superior ophthalmic vein (SOV), inferior petrosal vein (IPS), and MCV were selected. Relative Tmax was defined as the Tmax at selected ROIs minus Tmax at I0 or I0′. An intergroup comparison between the normal and treatment groups and pre- and post-treatment comparison of the peri-therapeutic rTmax for the treatment group were performed.ResultsrTmax's for the normal group were as follows: Anterior-posterior view: I1: 0.16, I2: 0.32, A1: 0.31, M1: 0.35, SSS: 6.16, SS: 6.56, and MCV: 3.86 seconds. Lateral view: IA: 0.05, IB: 0.20, A2: 0.53, M2: 0.95, FV: 4.84, PV: 5.12, IPS: 4.62, JV: 6.81, and MCV: 3.86 seconds. Before embolization, rTmax of the IPS, SS, and JV for the treatment group were shortened (p < 0.05). No rTmaxs for any arterial ROIs in the fistula group were significantly different. After embolization, the rTmaxs for all venous ROIs returned to normal except for two which were partially obliterated.ConclusionThis postprocessing method does not require extra radiation exposure and contrast media. It facilitates real-time hemodyamic monitoring and may help determining the endpoint of embolization, which increases patient safety.     

Clinical applications of the C-arm cone-beam CT-based 3D needle guidance system in performing percutaneous transthoracic needle biopsy of pulmonary lesions

PURPOSE

This study explored the value of flat detector C-arm CT-guidance system in performing percutaneous transthoracic needle biopsy (PTNB) for lung lesions in clinical practice.

METHODS

A total of 110 patients with solid lung lesions were enrolled to undergo PTNB procedures. The mean diameter of lesions was 4.63 cm (range, 0.6–15cm). The needle path was carefully planned and calculated on the C-arm CT system, which acquired three-dimensional CT-like cross-sectional images. The PTNB procedures were performed under needle guidance with fluoroscopic feedbacks.

RESULTS

Histopathologic tissue was successfully obtained from 108 patients with a puncture success rate of 98.2% (108/110). The diagnostic accuracy rate was found to be 96.3% (104/108). There was only one case of pneumothorax (0.9%) requiring therapy. The rates of mild pneumothorax and hemoptysis were low (12.0% and 6.5%, respectively). In addition, procedural time could be limited with this technique, which helped to reduce X-ray exposure.

CONCLUSION

Our study shows that C-arm CT-based needle guidance enables reliable and efficient needle positioning and progression by providing real-time intraoperative guidance.Lung cancer is the commonest cause of a pulmonary mass, which can be identified through chest X-ray or computed tomography (CT) examinations (1, 2). Accurate identification of histopathological cell type is crucial to determine the right treatment method and reduce the morbidity and mortality rates. Image-guided percutaneous transthoracic needle biopsy (PTNB) is a widely accepted technique for characterization of pulmonary mass that is not reachable by broncoscopy. CT has been considered as the technique of choice for guiding PTNB procedures as it enables detailed visualization of the lesion and surrounding tissue structures. This technique offers good in-plane resolution. However, the view is limited to the plane that the needle is inserted, causing double-oblique approach with an angulated needle trajectory which is difficult to perform, time consuming (25–27 minutes), and heavily relies on the experience of the clinicians (3). The introduction of CT fluoroscopy (CTF) enables real-time visualization of the needle advancement. Thus, the challenges correlated with displacement and disappearance of small lesions from scan planes due to respiratory movement in conventional CT-guided PTNB can be overcome, leading to a reduced procedural time (12–24 minutes) (4). Nevertheless, CTF may result in higher radiation dose to clinicians (5–9). Besides, according to previous studies, lesion size is a determining factor in diagnostic accuracy of CT- or CTF-guided PTNB, which yields 75%–90% diagnostic accuracy, with correlated rates of 15%–20% for pneumothorax and 2%–3% for pneumothorax requiring drainage (10–14). In addition, due to the limited size of the gantry, CT- or CTF-guided PTNB procedures in obese patients can be technically challenging or impossible.Nowadays, flat detector-equipped angiographic C-arm cone-beam CT (CBCT) systems can be used to acquire CT-like cross-sectional images directly within the interventional radiology suite (15–17). The CBCT systems offer real-time visualization of PTNB procedure and more flexibility in the orientation of the detector system around the patient compared to traditional CT systems. Thus, CBCT could provide image guidance for PTNB procedures, combining the advantages of CT and fluoroscopic guidance, as it is proved to be valuable for evaluating pulmonary lesions even smaller than 2 cm. This technique has been shown to yield 90%– 98% diagnostic accuracy, 94%–97% sensitivity, and 75%–100% specificity. The incidence of complications was 20%–39%, the associated procedural time ranged 11.9–18.1 minutes and exposure dose ranged 170–275 mGy (3, 18–24). Along with the development of CBCT, a novel technique has recently emerged for PTNB guidance. It combines advanced virtual needle path planning based on user interactions using three-dimensional (3D) CBCT images with real-time fluoroscopic guidance (21, 25). This technique offers high spatial resolution of less than 1 mm, as well as contrast resolution of 10 HU, which is adequate for lung imaging, as lung inherently has a high contrast (soft tissue against air). In addition, CBCT allows good access to the patient without any patient transfer or movement, increasing the effectiveness and efficacy of clinical workflow.The purpose of this study is to explore the value of using a CBCT-based 3D needle guidance system in performing PTNB for pulmonary lesions in the interventional radiology suite.