Successful treatment of a complete Susac syndrome in a 16-year-old boy: A case report.

Key Clinical Message: We reported a pediatric case of SuS with a complete diagnosis triad. Although the optimal treatment of SS is unclear, prompt diagnosis and treatment can result in almost a complete recovery. Abstract: Susac's syndrome (SuS) is a rare, autoimmune disorder known as a typical triad of sensorineural hearing impairment, central nervous system involvement, and multiple branch retinal artery occlusions (BRAOs). It is usually misdiagnosed or underdiagnosed because its symptoms may vary at the presentation time. Diagnosis can be established based on neuroimaging, ophthalmic examination, and audiometry, which match the clinical symptoms. SuS is very limited and rare in childhood and can be easily misdiagnosed with multiple sclerosis or acute disseminated encephalomyelitis. We report a 16-year-old boy patient with a completed SuS triad including BRAO in fluorescent angiography (FA), mild to moderate sensory neural hearing loss (SNHL), "Snowball lesions," and "pearl of string" signs in magnetic resonance imaging (MRI). Successful treatment was achieved with methylprednisolone, rituximab, azathioprine, cyclophosphamide, and plasmapheresis. SuS is a rare disorder, which rarely presents with a full triad and all the manifestations may not be present at the onset of the disease, leading to misdiagnosis or underdiagnosis. Our case is exceptional because he was in a pediatric age and presented with a complete triad of SuS which adds to the rarity of this disease. Although optimal treatment of SuS is unclear, our treatment regimen resulted in almost a complete recovery.

Embolization of pseudoaneurysms in the ureteral branch of the renal artery.

BACKGROUND: Although transcatheter arterial embolization for pseudoaneurysms is already well-established, ureteral artery pseudoaneurysm embolization is extremely rare. The present case shows a successful transcatheter arterial embolization for pseudoaneurysms in the ureteral branch of the renal artery due to ureteral invasion from gastric cancer. CASE PRESENTATION: A 57-year-old female presented with gross hematuria after treatments for poorly differentiated gastric adenocarcinoma. A contrast-enhanced computed tomography revealed pseudoaneurysms around the right ureter with a massive hematoma in the right ureter and bladder. The diagnosis was ureteral branch pseudoaneurysms resulting from possible retroperitoneal invasion due to pelvic lymph node metastasis of gastric cancer. Transcatheter arterial embolization was performed using gelatin particles, successfully controlling her hematuria without complications. CONCLUSIONS: Ureteral branch artery embolization, although extremely rare, may be an effective and safe treatment option.

Protection of branches during clipping of low grade internal carotid communicating segment aneurysms via frontolateral keyhole approach / 中华神经医学杂志

Objective:To investigate the clinical experiences and outcomes of protection of branches during clipping of low grade internal carotid communicating segment (C7) aneurysms via frontolateral keyhole approach. Methods:Fifty-two patients with internal carotid C7 aneurysms and Hunt-Hess grading I-III, admitted to our hospital from January 2017 to December 2020, were chosen; 45 patients were with posterior communicating artery (PCoA) aneurysms, 6 patients were with anterior choroidal artery (ACHA) aneurysms, and one patient was with PCoA aneurysm combined with ACHA aneurysm. The relation between origin of the branch arteries and tumor neck was determined during surgery. The relation between shape of the branch arteries and tumor body was investigated in the Spaces II, III and V of the internal carotid artery (indocyanine green fluorescence angiography was used when necessary); the aneurysms were clipped by appropriate aneurysm clip or clips combination; after clipping, microspic or endoscopic exploration and indocyanine green fluorescence angiography were performed, and re-clipping was performed in the misclipped or stenosed branch arteries.Results:During the surgery, the branch arteries of 6 patients (4 from posterior communicating artery, 1 from anterior choroidal artery, and 1 from perforator artery at the bifurcation of internal carotid artery) were misclipped, and therefore, the aneurysm clips were adjusted or replaced and then re-clipped. Aneurysms were successfully clipped in all 52 patients. Brain CT examination within 24 h of surgery showed that focal cerebral infarction in the branch artery feeding area was noted in 5 patients, of which 3 patients were accompanied by contralateral limb muscle strength decline (grading 4, grading 3 and grading 1, respectively) and medication and neurological rehabilitation were given. The other 47 patients had good general condition without special condition. During the follow-up of 9-12 months, the muscle strength of 3 patients with decreased contralateral limb strength recovered to grading 5, 4 and 2, respectively. There were no residual or recurrent aneurysms in all patients. At the last follow-up, 47 patients had Glasgow Outcome scale (GOS) scores of 5, 3 patients had GOS scores of 4, 1 patient had GOS scores of 3, and 1 patient had GOS scores of 2.Conclusion:In the treatment of low-grade internal carotid artery C7 segment aneurysms via frontolateral keyhole approach, multiple protective strategies can be adopted to actively prevent branch artery injury, which can effectively reduce the incidence of postoperative cerebral infarction and improve the prognosis.

Treatment of Traumatic Intracranial Pseudoaneurysms: A Single-Center Experience.

Background and Purpose: As a rare lesion secondary to brain trauma, traumatic intracranial aneurysms (TICAs) lead to high mortality and morbidity, and multiple treatment modalities have been applied for TICAs. All patients diagnosed with TICAs in our institution from 2010 to 2020 were included in the report, and their clinical features, treatment, and outcomes are described in detail. The purpose of this study is to illustrate the characteristic of different therapeutic methods of TICAs, and focus on the endovascular treatment. Methods: A total of 20 patients were included in this study. The 3 patients who declined treatment all died. Five of the other 17 patients were treated surgically, including clipping, wrapping, and trapping with or without EC-IC high-flow bypass, with only 1 case of parent artery preservation. Twelve patients underwent endovascular treatment, including bare coil embolization (1 case), stent-assisted coiling (2 cases), balloon-assisted coils/Onyx glue embolization (1 case) and covered stents (8 cases), with only 1 case of parent artery sacrifice. Results: 20 patients were included in the present study with 17 males, and the mean of age on 27 years (IQR: 22, 44 years). Eight patients presented with epistaxis, followed by 5 patients with coma, 3 patients with visual defects and 2 patients with CSF leakage. There were 18 TICAs located at the internal carotid artery (ICA); The other 2 TICAs located at pericallosal artery and A1 segment anterior cerebral artery (ACA). One case of diplopia occurred due to sacrifice of the ICA. Occlusion of the ophthalmic artery occurred in 3 patients after placement of a covered stent, with 1 patient suffering an irreversible vision decrease. None of the other patients who underwent the treatment have experienced an aggravation of their symptoms since the treatment; During the imaging follow-up, 1 case of recurrence and 1 case of endoleak occurred in this case series. Conclusions: TICAs are associated with significant morbidity and mortality, and endovascular treatment has emerged as a valuable option, which may be promising to improve the clinical outcomes due to their advantages of preserving the parent artery if occlusion of the side branch artery can be avoided.

Microcatheter-Assisted Protection of the Posterior Inferior Cerebellar Artery During Parent Artery Sacrifice of a Vertebral Artery Dissecting Aneurysm.

Branch vessel occlusion is a major cause of stroke in parent artery sacrifice (PAS) for vertebral artery dissecting aneurysms (VADA). There is now an increasing trend towards preservation of branch vessels during PAS. Stents are commonly employed to achieve this but bring with it the attendant risks of future thrombosis and lifelong antiplatelet use. Although a microcatheter protection technique has been utilised in branch artery protection of wide-necked saccular aneurysms, it has rarely been described in PAS for VADAs. We describe the use of a dual microcatheter technique in the protection and remodelling of the posterior inferior cerebellar artery (PICA) during PAS of the vertebral artery, which also served as a temporary scaffold to support placement of the coils during the embolisation process.

Sequential Pontine Strokes With the "Heart Appearance" Sign.

Most brainstem infarcts are caused by pontine strokes. When a bilateral pontine or brainstem stroke occurs, it can result in an image called the "heart appearance" sign. Sequential pontine strokes have not yet been described to cause the "heart appearance" sign. We report a case of sequential pontine strokes in a 47-year-old man with no relevant medical history. He presented with acute left-sided hemiparesis. Initial brain MRI showed a right-sided pontine infarction. Two weeks later, the patient's left side hemiparesis and dysarthria worsened, and he developed right-sided facial paralysis. The brain MRI showed a left-sided pontine infarction that looked like the "heart appearance" sign. The patient had a good recovery. This report highlights a case of atypical pontine stroke recurrence two weeks apart and discusses branch artery disease as a possible etiologic cause.

Multimodal Imaging in Susac Syndrome: A Case Report and Literature Review.

Susac syndrome (SS) is a rare microangiopathy that involves arterioles of the brain, retina, and cochlea. Diagnosis is extremely difficult because of the rarity of the disease and because the signs and symptoms often occur at different times. Multidisciplinary approaches and multimodal images are mandatory for diagnosis and prompt therapy. In this report, we describe a case of SS and the application of multimodal retinal imaging to evaluate the ophthalmologic changes and to confirm diagnosis. Early diagnosis and therapy based on the associations of steroids and immunosuppressants are necessary to limit the sequelae of the disease.

The Mini-Cross Prefenestration for Endovascular Repair of Aortic Arch Pathologies.

Objective: To examine the feasibility, integrity, efficacy, and safety of endovascular repair of the aortic arch pathologies with the mini-cross prefenestration (MCPF) on stent grafts. Methods: First, to prove the feasibility of the MCPF, an in-vitro prefenestration experiment was conducted. Second, to examine the integrity of the MCPF stent grafts, a fatigue test was conducted. Then, the membranes and metal structures of stent grafts were examined by light microscopy and scanning electron microscopy (SEM). Third, a clinical experiment was conducted to investigate the efficacy and safety of this novel technique (ClinicalTrials.gov Identifier: NCT04544579). Results: All the 12 branch stents were successfully implanted and flared in vitro. After the fatigue test stimulating a 5-year cardiac cycle, no obvious disintegration or fracture was found in light microscopy or SEM. From December 2017 to February 2020, 26 patients with left subclavian arteries and/or left common carotid arteries involved received the novel technique. The endovascular repair with the MCPF was successfully performed on all the 26 (100%) patients. Eighteen (69.2%) patients underwent the reconstruction of the left subclavian artery (LSCA) only. The fenestrations of both the LSCA and left common carotid artery (LCCA) were conducted in 8 (30.8%) patients. Median operative time was 120 [interquartile range (IQR), 95-137.5] min and median revascularization time of the LSCA and LCCA was 30.5 (IQR, 22.8-42.0) s and 20.0 (IQR, 18.0-32.0) s separately. During the median follow-up duration of 38.9 (range, 18.8-44.2) months, one case needed an open surgery because of retrograde type A aortic dissection 3 months after implantation and no other complications or mortality occurred. The maximum aortic diameters were significantly decreased in patients with thoracic aortic dissection and thoracic aortic aneurysm (p < 0.05). Conclusion: The existing evidence demonstrated the safety, rapid branch artery revascularization, and positive aortic remodeling of the novel technique. Long-term observation is warranted to prove the durability.

Laser fenestration of aortic arch stent grafts for endovascular treatment of retrograde type A dissection.

OBJECTIVES: Retrograde type A dissection (RTAD) is a rare but life-threatening event following thoracic endovascular aortic repair (TEVAR), and its total endovascular treatment is a huge challenge. This research aimed to evaluate the safety, effectiveness, technical success, and medical outcomes of in situ laser fenestration of aortic arch stent grafts during TEVAR of RTAD. METHODS: We retrospectively reviewed the clinical data of 15 patients with RTAD who received in situ laser fenestration of aortic arch stent grafts during TEVAR between Mar 2016 and Dec 2019. All patients were subjected to intraoperative extracorporeal brain perfusion. The preoperative, intraoperative and postoperative medical data were collected and analyzed. RESULTS: The mean age of the 15 patients was 64 ± 8 years, 8 of whom were male. The immediate overall technical success rates, including aortic stent deployment were 100% and primary fenestration success was achieved in 13 (86.7%) patients. The mean postoperative length of stay was 10 ± 4 days. Stroke occurred in 1 case. No in-hospital/30-day death nor permanent paraplegia/paresis was observed. The mean follow-up time was 13 ± 5 months. Two type Ia endoleaks were found, but no late occlusion and migration of the supra-aortic branch arteries stents during the follow-up were observed. CONCLUSIONS: The in situ laser fenestration of aortic arch stent grafts during TEVAR of RTAD is a potential total endovascular therapy of RTAD for patients unsuitable for direct surgical repair.

Development of visual acuity under hyperbaric oxygen treatment (HBO) in non arteritic retinal branch artery occlusion.

PURPOSE: Nonperfusion of retinal tissue due to arterial occlusion leads inevitably to mostly irreversible retinal damage. Until today no evidence-based treatment exists. Inhalation of 100% oxygen at high atmospheric pressure causes an increased solubility of oxygen in the blood that helps the retinal tissue to survive through diffusion in case of an artery occlusion till vascular recanalization occurs. Hence the purpose of this study is to compare the visual outcome in patients with retinal branch artery obstruction treated with hyperbaric oxygen versus patients treated with hemodilution only. METHODS: Non-randomized, monocentric, retrospective study. Patients with diagnosis of non-arteritic retinal branch artery occlusion (BRAO) treated with hyperbaric oxygen therapy between 1997 and 2017. Exclusion criteria were central retinal artery occlusion, presence of a cilioretinal artery and arteritic cases. The control group was matched based on visual acuity (VA) at admission, age, and delay between symptoms and beginning of clinical care. RESULTS: The control group and the matching oxygen group contained 14 patients each. Initial VA in the matched HBO group was 0.18 ± 0.19 and 0.23 ± 0.19 in the control group (p = 0.57). Final VA at discharge was 0.69 ± 0.29 in the matched oxygen group and 0.32 ± 0.23 in the control group (p = 0.0009). HBO-treated patients had a significant visual increase compared with the control group. The most common comorbidities were arterial hypertension and vascular sclerosis. CONCLUSION: HBO treatment appears to have a beneficial effect on visual outcome in patients with retinal branch artery occlusion. HBO treatment could be a rescue therapy at an early stage of BRAO, especially to bridge the time of a potential reperfusion. However, further, prospective, randomized clinical trials are required to verify this assumption.

Clinical value of ultrasonographic superb micro‐vascular imaging assessment for abdominal branch arteries in Stanford B aortic dissection / 中华超声影像学杂志

To investigate the clinical diagnostic value of ultrasonographic superb micro‐vascular imaging ( SM I) for abdominal branch arteries′involvement in Stanford B aortic dissection ( AD ) . Methods A total of 34 AD patients underwent conventional ultrasound ,SMI and CTA . The characteristics of the ultrasonographic images of branch artery involvement including coeliac artery ,superior mesenteric artery ,left and right renal artery were analyzed . T he diagnostic performance of conventional ultrasound and SM I were compared and CT A was used as the reference standard . Results Among the 34 patients with AD ,according to the ultrasonographic images′characteristics ,136 branch arteries were categorized into four classes :Class Ⅰ (81/136) ,branch artery perfusion supplied from the aortic true lumen ; Class Ⅱ ( 27/136) , dissection extending into the branch ; Class Ⅲ ( 16/136 ) ,branch artery perfusion supplied from the aortic false lumen ; Class Ⅳ ( 12/136) ,branch artery perfusion supplied from the aortic true and false lumens . T he diagnostic accordance rate of the conventional ultrasound and SM I were 76 .5% ( 104/136 ) and 92 .6%( 126/136) and had significant differences ( P ＜0 .001) . Conclusions SMI can be used to evaluate branch artery involvement in AD patients w hich will guide the practice of pre‐operation and post‐operation .

Investigation of the hemodynamics of a juxtarenal aortic aneurysm with intervention by dual-stents strategy.

OBJECTIVE: To study the feasibility of using two stents (a combination of multilayer stent [MS] and stent graft [SG]) in the treatment of a juxtarenal aortic aneurysm that involves a significant branch artery and to determine the advantages and disadvantages of using SGs upstream and downstream from the aneurysm so as to provide some theoretical guidance for preoperative clinical decision-making in the future. METHODS: Four ideal geometric models were established for numerical computation: case 1 refers to an aneurysm without the use of stents, case 2 represents the implantation of two MSs in an aneurysm, and case 3 (SG + MS) and case 4 (MS + SG) both involve the treatment of an aneurysm by using a combination of SG and MG. RESULTS: The aneurysm pressure is slightly lower and there are more vortices when the SG is implanted (case 3 and case 4). In particular, for case 4, additional vortices appear in the sac and the area of the low-wall shear stress is larger on the aneurysm compared with those of the other three cases. However, the pressure becomes uneven, and a peak pressure region is observed on the wall of the aneurysm, and therefore, the aneurysmal wall will become buckled. In addition, the flux of the renal artery in the four cases is greater than that in the normal case. CONCLUSION: The arrangements in cases 3 and 4 can effectively isolate the aneurysm from circulation, but clinically, it is necessary to avoid such a high-risk situation wherein the SG is positioned downstream of the aneurysm (case 4), even though this leads to improved isolation.

A flap based on the plantar digital artery arch branch to improve appearance of reconstructed fingers: Anatomical and clinical application.

AIM: To investigate blood supply features of the flap based on the plantar digital artery arch and arch branch artery, and the treatment of outcomes of reconstructed fingers by the plantar digital artery arch branch island flap. METHODS: Eight fresh foot specimens were employed with red emulsion infusion and microdissection. The vascular organization was observed in the second toe, such as initiation site, the course, and the number of the plantar digital artery arch branch. There were 15 fingers of 13 patients (8 males and 5 females) with finger defects accompanied by toe transfer, using the plantar digital artery arch branch flap inserted in the neck of the second toe to correct the appearance defect caused by a narrow "neck" and a bulbous tip. RESULTS: The intact plantar digital arches were identified in all specimens. The plantar digital artery arch had 5 branches. The range of external diameter of the arch branch was 0.4-0.6 mm. All the plantar digital artery arch branch island flaps and the reconstructed fingers survived. These cases were conducted with a follow-up period for 3-18 months (average, 9 months). All the plantar digital artery arch branch island flaps and reconstructed fingers demonstrated a satisfactory appearance and favorable sense function. The reconstructed finger-tip characteristic was good, with no obvious scar hyperplasia. The range of flexion and extension of reconstructed fingers was favorable as well. CONCLUSIONS: The plantar digital artery arch and arch branch artery possess regular vasa vasorum and abundant vascularity. A flap based on the plantar digital artery arch branch is an ideal selection for plastic surgery of reconstructed fingers.

Stenosis length of middle cerebral artery and branch atheromatous disease associated infarct.

BACKGROUND: We analyzed the relationship between the characteristics of branch atheromatous disease associated infarct (BAI) and stenosis length (SL) of relevant middle cerebral artery (MCA) M1 segment. METHODS: Ninety-five patients with BAI were recruited from 1024 consecutive acute ischemic stroke patients. Among them, 59 patients (62.11%) had concurrent relative stenosis of MCA M1 segment. The neurologic deficit severity at admission was assessed by National Institutes of Health Stroke Scale (NIHSS) and the infarct size by infarct lesion thickness and maximum diameter. The SL and the distance from the carotid terminal segment to stenosis (DT) were measured on coronary projection on 3D TOF MRA. The correlations between SL and NIHSS, SL and infarct lesion maximum diameter were analyzed. RESULTS: There was no difference between BAI patients with or without M1 stenosis in image markers for infarct etiological subtype. SL was significant difference in patients with NIHSS >3 vs. ≤ 3 (p = 0.032) at admission. Further analysis showed that SL correlated with NIHSS at admission (rs = 0.613, p = 0.000) and maximum diameter (rs = 0.621, p = 0.000) significantly. CONCLUSION: SL is a significant moderate predictor for infarct lesion diameter and neurologic deficit severity.

In Situ Laser Fenestration Is a Feasible Method for Revascularization of Aortic Arch During Thoracic Endovascular Aortic Repair.

BACKGROUND: Reconstruction of the aortic major branches during thoracic endovascular aortic repair is complicated because of the complex anatomic configuration and variation of the aortic arch. In situ laser fenestration has shown great potential for the revascularization of aortic branches. This study aims to evaluate the feasibility, effectiveness, and safety of in situ laser fenestration on the three branches of the aortic arch during thoracic endovascular aortic repair. METHODS AND RESULTS: Before clinical application, the polytetrafluoroethylene and Dacron grafts were fenestrated by an 810-nm laser system ex vivo, which did not damage the bare metal portion of the endografts and created a clean fenestration while maintaining the integrity of the endografts. In vivo, 6 anesthetized female swine survived after this operation, including stent-graft implantation in the aortic arches, laser fenestration, and conduit implantation through the innominate arteries and the left carotid arteries. Based on the animal experiments, in situ laser fenestration during thoracic endovascular aortic repair was successively performed on 24 patients (aged 33-86 years) with aortic artery diseases (dissection type A: n=4, type B: n=7, aneurysm: n=2, mural thrombus: n=7). Fenestration of 3 aortic branches was performed in 2 (8.3%) patients. Both the left carotid artery and the left subclavian artery were fenestrated in 6 (25%) patients. Only left subclavian artery fenestration surgery was done in 16 (66.7%) patients. Among these patients, 1 fenestration was abandoned secondary to an acute takeoff of the innominate artery in a type III aortic arch. The average operative time was 137±15 minutes. The technical success rate was 95.8% (n=23). No fenestration-related complications or neurological morbidity occurred after this operation. During a mean postoperative 10-month follow-up (range: 2-17 months), 1 patient died of severe pneumonia, and all the left subclavian artery and carotid artery stents were patent with no fenestration-related endoleaks upon computed tomography angiography images. CONCLUSIONS: In situ laser fenestration is a feasible, effective, rapid, repeatable, and safe option for the reconstruction of aortic arch during thoracic endovascular aortic repair, which might be available to revascularize the 3 branches. However, follow-up periods should be extended to evaluate the robustness of this technique.

Stenosis of the branches of the neopulmonary artery after the arterial switch operation: A cardiac magnetic resonance imaging study.

BACKGROUND: The neonatal arterial switch operation (ASO) is now the standard of care for children born with transposition of the great arteries. Stenosis of the neopulmonary artery on long-term follow up is a known complication. METHODS: We performed a retrospective analysis of eleven patients who underwent a cardiac magnetic resonance imaging (MRI) due to echocardiographic evidence suggestive of stenosis of the neopulmonary artery or its branches (mean estimated Doppler gradient 48 mmHg, min 30 mmHg, max 70 mmHg). A comprehensive evaluation of anatomy and perfusion was done by cardiac MRI. RESULTS: The branches of the neopulmonary artery (neo PA) showed decreased caliber in three patients unilaterally and in two patients, bilaterally. Magnetic resonance (MR) perfusion studies showed concomitant decreased flow, with discrepancy between the two lungs of 35/65% or worse, only in the three patients with unilateral obstruction, by two different MR perfusion methods. CONCLUSIONS: Cardiac MR can be used as a comprehensive non-invasive imaging technique to diagnose stenosis of the branches of the neopulmonary after the ASO, allowing evaluation of anatomy and function of the neoPA, its branches, and the differential perfusion to each lung, thus facilitating clinical decision making.

Clinical application of branch artery of the sural medial gastrocnemius muscle flap combine with sural nerve nutritional vessel axial / 中华显微外科杂志

Objective To investigate the clinical efficiency of branch artery of the sural medial gastrocnemius muscle flap combine with sural nerve nutritional vessel axial.Methods From February 2006to March 2010,according to the position and size of the soft tissue defects,the muscle flap combined with branch artery of the sural medial gastrocnemius and sural nerve nutritional vessel axial were desingned and obtained to repair 7 cases of sofi tissue defects of the upper-region of the tibial.The flap size ranged from 8.0 cm × 8.0 cm to 12.0 cm × 10.0 cm.The vessel pedicle of branches ranged from 1.8-3.0 cm.The distribution of the vessel pedicle of branches ranged from 10.0-17.0 cm on the distant popliteal fossa,and ranged from 2.0-5.0 cm on the back of medial line.Results The outline and function were satisfactory during 6-15 months follow-up.Conclusion The blood supply of this flap is reliable without sacrifice of major arteries.Flap elevation is easy.It can transfer to a long distance and can repair large skin defects.It is very useful in repairing upper-region of the tibial large soft tissue defect.