Multiple ruptured cerebral aneurysms in a child with Takayasu arteritis

The authors report the case of an 18-month-old girl who presented with a ruptured anterior communicating artery aneurysm, and who was later diagnosed with Takayasu arteritis. Her initial aneurysm was successfully treated with clip application. However, over a 6-month period she had multiple ruptures from new and rapidly recurring aneurysms adjacent to the clips. These aneurysms were treated with repeated craniotomy and clip application and then with endovascular coil placement. Aneurysmal subarachnoid hemorrhage is a rare presentation of Takayasu arteritis. To the authors' knowledge, this is the youngest reported patient with Takayasu arteritis to present with a ruptured cerebral aneurysm.     

Takayasu arteritis and renovascular hypertension in childhood.

Takayasu arteritis with renal artery involvement was the cause of severe persistent hypertension in eight children under 12 years of age. Features of severe hypertension dominated the clinical picture. Unequal or absent pulses were found in three patients. An irregular contour of the descending thoracic aorta on the chest roentgenogram was an early clue to the diagnosis. On aortography both renal arteries were affected in five patients, although a discrepancy in kidney size on excretory urography had suggested a unilateral lesion. Total aortography is mandatory to outline the full extent of the vascular abnormalities. All the patients had strongly positive tuberculin skin tests, and, although mycobacteria were not isolated, all patients received antituberculous as well as antihypertensive therapy. Nephrectomy of the worse kidney is contraindicated if the renal arteries are asymmetrically involved, for fear of later extension of the arteritis. Takayasu arteritis is an important cause of severe persistent hypertension in nonwhite children.     

Takayasu arteritis presenting as cerebral aneurysms in an 18 month old: A case report

Background  

Central nervous system involvement occurs in as many as twenty percent of Takayasu arteritis cases. When central nervous system disease is present, it typically manifests as cerebral ischemia or stroke. There are rare reports of intracranial aneurysms in adults with Takayasu arteritis, but none in children.     

克罗恩病合并多发性大动脉炎1例报告并文献复习

目的提高对儿童克罗恩病(CD)合并多发性大动脉炎(TA)的认识。方法回顾分析1例CD合并TA患儿的临床资料,并复习相关文献。结果 10岁女孩,经肠镜诊断CD,接受英夫利昔单抗(IFX)治疗并随访。3年后患儿于临床缓解时出现头晕、晕厥,经血管影像学诊断为TA。停用IFX,换用糖皮质激素及吗替麦考酚酯治疗,患儿CD及TA均缓解。文献复习发现CD合并TA好发于年轻女性,以CD首发多见。CD合并TA临床表现多样,以全身非特异症状及消化道症状最常见。儿童TA临床表现更不典型,合并CD时易漏诊或误诊。四肢血压、脉搏以及血管杂音检测对TA有提示作用。一旦怀疑TA,应及早完善血管影像学检查诊断。多数CD合并TA患者接受美沙拉嗪、糖皮质激素和免疫抑制剂联合治疗,部分接受生物制剂治疗,超过1/3的患者接受手术治疗。结论 CD合并TA少见。当CD患者炎症指标与临床治疗效果存在差异时,应警惕是否合并其他疾病。     

儿童多发性大动脉炎合并心功能不全5例病例系列报告

目的 总结儿童多发性大动脉炎（TA）合并心功能不全的临床特征。方法 回顾性分析首都儿科研究所附属儿童医院2013年1月至2019年12月确诊的TA合并心功能不全患儿的临床资料。结果 共5例患儿进入本文分析,均为女性,起病年龄2~14岁。以心脏症状起病4例,包括胸闷、心悸、头晕、乏力、多汗。血管杂音5例,脉弱/无脉4例,高血压4例。5例均合并心脏瓣膜受累,3例为多瓣膜受累。二尖瓣关闭不全4例;主动脉瓣关闭不全3例,其中2例伴有升主动脉扩张;三尖瓣关闭不全3例;冠状动脉扩张3例。5例临床分型均为Ⅲ型（混合型）。4例接受糖皮质激素和（或）免疫抑制剂治疗,2例接受TNF-α拮抗剂英夫利昔单抗治疗。1例失访,4例随访6~48个月,患儿症状均有不同程度好转,除已有不可逆心脏损伤外,炎性指标均正常,无新发病灶。1例合并顽固高血压,拟接受外科手术治疗。结论 TA患儿应定期行心脏超声检查以及早发现心脏病变,、及时干预。以心脏症状起病的患儿,除应考虑原发性心脏疾病,还应警惕TA累及心脏的可能。TA合并心功能不全患儿中,主动脉和肾动脉出现血管狭窄者,多合并高血压。     

Aortic valve replacement surgery for a case of infantile Takayasu arteritis

Takayasu arteritis is a chronic inflammatory disease of unknown etiology primarily affecting the aorta and its major branches and usually occurring in the second or third decade of life. Here, we report a case of Takayasu arteritis in a 10-month-old patient. The infant presented with signs of congestive heart failure and severe aortic regurgitation. Echocardiography and computed tomography angiography showed an abnormally dilated thoracic and abdominal aorta. The infant was initially treated with prednisolone, followed by commissuroplasty of the aortic valve but neither approach ameliorated the heart failure. The patient was eventually treated with a mechanical aortic valve replacement surgery at the age of 12 months, and her condition stabilized. Although unusual, this case indicates that the diagnosis of Takayasu arteritis should be considered in children with unexplained systemic symptoms, aortic valve regurgitation, and heart failure. Because severe aortic regurgitation may be a fatal complication of Takayasu arteritis, early aortic valve replacement surgery should be considered, even in very young children.     

多发性大动脉炎的诊治与预后

多发性大动脉炎是1908年由日本眼科医生高安首先报道,故又称为"高安氏动脉炎"(Takayasu's arteritis,TA).TA是主动脉及其主要分支的慢性进行性非特异性炎性疾病.病变主要位于主动脉弓及其分支,其次为降主动脉、腹主动脉、肾动脉.主动脉的二级分支(如肺动脉、冠状动脉)也可受累.虽然TA是一种世界性疾病,但以亚洲人群高发,其中以女性发病者较多,儿童中以青春期女孩发病多见.     

多发性大动脉炎的诊治与预后

多发性大动脉炎是1908年由日本眼科医生高安首先报道,故又称为"高安氏动脉炎"(Takayasu's arteritis,TA).TA是主动脉及其主要分支的慢性进行性非特异性炎性疾病.病变主要位于主动脉弓及其分支,其次为降主动脉、腹主动脉、肾动脉.主动脉的二级分支(如肺动脉、冠状动脉)也可受累.虽然TA是一种世界性疾病,但以亚洲人群高发,其中以女性发病者较多,儿童中以青春期女孩发病多见.     

Familial Takayasu arteritis - a pediatric case and a review of the literature

Takayasu arteritis (TA) is a rare chronic inflammatory disease of the aorta and its major branches. It is seen predominantly in females during the second and third decades of life, although it can occur in childhood. The aetiology of TA remains unknown. To date, familial cases of TA have been considered rare; however, a review of the literature suggests that cases are accumulating. We report a case of two sisters affected by severe TA, and review other reported familial cases.     

婴幼儿多发性大动脉炎14例临床特点分析

目的通过分析总结14例婴幼儿多发性大动脉炎(TA)的临床特点,并结合文献复习总结,以提高对该病的认识及诊疗水平。方法回顾性分析2016年7月至2019年5月在首都儿科研究所附属儿童医院住院的TA婴幼儿的临床资料及随访情况,并结合文献,分析总结该病的临床特点。结果14例患儿年龄为1个月23 d^28个月,男6例,女8例。临床表现中最常见的是发热[10例(71.4%)],高血压9例(64.3%),脉弱或无脉5例(35.7%)。按照病变血管部位的临床分型,广泛型11例(78.5%),头臂动脉型3例(21.4%),本组患儿无胸腹主动脉及单纯肺动脉型。14例TA患儿中,12例有颈总动脉、颈动脉、锁骨下动脉及冠状动脉及其分支(前降支、回旋支)受累(85.7%);11例肾动脉受累(78.6%);9例腋动脉受累(64.2%);8例腹主动脉受累(57.1%);6例降主动脉受累(42.9%);6例胸主动脉受累(42.9%);6例肠系膜上动脉受累(42.9%);5例股动脉受累(35.7%);5例肺动脉受累(35.7%);4例肱动脉受累(28.6%)。14例患儿中,误诊11例,诊断不清3例,误诊时间为18 d^2个月。误诊病例中,8例误诊为不典型川崎病。14例患儿中,7例治疗后大部分病变血管范围逐渐减少,受累较轻血管甚至可以完全恢复正常血管状态。4例患儿血管影像学检查较前无明显加重或好转。9例出现高血压患儿应用降压药血压能控制在正常范围,但不能停用降压药。5例查体发现脉弱或无脉患儿均未改善。14例患儿中7例生长发育同正常同龄儿,7例落后于正常同龄儿身高体质量第25百分位。14例患儿随访2~22个月,均规律治疗,未出现复发。结论3岁以内TA累及血管较多,病情严重,误诊率高,经过治疗病情很快能够控制,但容易遗留血管病变,部分患儿预后不佳。     

Reduced aortic elastic properties in a child with Takayasu arteritis: case report and literature review

Takayasu arteritis (TA) is a chronic inflammatory vasculitis of the aorta and its major branches with a very low incidence in Europe and North America. Our objective was to determine the elastic properties of the affected ascending and descending aortic walls non-invasively in a 14-year-old Iraqi girl with a 3-year history of fever, fatigue, malaise and diffuse pain. Ultrasound and magnetic resonance angiography showed marked thickening of the aortic wall, dilatation of the aortic arch, and decreased luminal diameters of the abdominal aorta and both subclavian arteries, consistent with TA. Ascending and descending aortic elastic properties such as distensibility and stiffness index were markedly reduced compared to a group of healthy controls (n=39): ascending aortic distensibility was 20 kPa^-1×10^–3 versus 63±23 kPa^-1×10^–3 in controls, and the ascending aortic stiffness index 9.6 versus 3.5±1.3 in controls. Although the patients general condition improved rapidly on oral prednisolone and azathioprine and inflammatory parameters normalised within 3 weeks, the aortic elastic parameters did not change during the first 2 weeks of anti-inflammatory treatment. Unfortunately, no further follow-up was possible. Conclusion: In patients with Takayasu arteritis, non-invasive quantification of reduced aortic elastic properties can help to assess aortic involvement, and possibly to follow disease activity and vascular response to therapy.D. Baumgartner and M. Sailer-Höck contributed equally to this work.     

Arteritis of the large vessels originating at the aorta (Takayasu arteritis) in childhood (author's transl)]

A 9-month-old female child with extensive inflammatory lesions in the large arterial trunks originating at the aorta (Takayasu arteritis) was reported. Severe cerebral, intestinal and coronary circulatory disturbances as well as insufficient blood supply in both arms, particularly the left arm, developed as a result of the stenoses and obliterations. Clinical course, postmortem findings and the results of EKG, EEG and carotid angiography were discussed. The onset in infancy and the very rapid progression of the disease were unusual.     

Clinical characteristics,interdisciplinary treatment and follow-up of 14 children with Takayasu arteritis

Background  

Pediatric patients with Takayasu arteritis were studied by analyzing clinical presentation, diagnostic images, response to multimodal therapy, and long-term outcome.     

Sonographic evaluation of renal artery aneurysm in childhood

We report a child presenting with renovascular hypertension and sonographic evidence of a renal artery aneurysm (RAA). The diagnosis of RAA was made sonographically by demonstrating vascular flow in an aneurysmal segment adjacent to but continuous with the right renal artery and externally compressing the inferior vena cava. Comparison of the sonographic studies and an abdominal angiogram illustrate the sensitivity of sonography in diagnosing this condition. We suggest that with renal doppler sonography, RAA may be diagnosed less invasively and possibly with greater frequency yet believe that the gold standard of angiography is necessary prior to surgical intervention.