Aberrant right renal perfusion from right internal mammary artery
来自右侧乳内动脉的右肾灌注异常
1.肾脏通过侧枝供血，可能灌注不足，造成肾血管性高血压。2.肾动脉变异较大3.Alport 综合征 ：AS(Alport syndrome ,AS) 是一种遗传性疾病，其特征是进行性肾小球病、高调感音神经性听力损失和眼部异常引起的肾病，可以合并脑动脉瘤等血管病变。4.肌纤维发育不良 （FMD）：需要终身监测 FMD 的其他表现，包括颈动脉狭窄和颅内动脉瘤。 |
29 岁男性的病例，其中右肾的上三分之二由右乳内动脉和侧支网络供应。此外，上左侧 RA 有近端狭窄伴远端动脉瘤。肾素取样证实肾血管性高血压。他成功接受了主动脉至右上 RA 搭桥手术和左上 RA 动脉瘤的一期修复。该病例增加了对 RA 异常的现有知识，并强调了综合评估替代肾血供对肾血管性高血压有效手术治疗的重要性。
Fig 1 Three-dimensional reconstruction of computed tomography angiography demonstrating right internal mammary artery (RIMA) collateral supplying the right kidney via the superior right renal artery (RRA) as well as an aneurysm of the superior left RA (LRA).
图 1计算机断层扫描血管造影的三维重建显示右乳内动脉 （RIMA） 侧支通过右肾上动脉 （RRA） 以及左上 RA （LRA） 的动脉瘤供应右肾。
contrast-enhanced abdominal aortography revealed delayed filling of the right kidney (Fig 2, A). The arch angiogram demonstrated that the RIMA supplied the upper two-thirds of the right kidney. Selective catheterization of the RIMA confirmed filling of the right renal parenchyma via collateral flow from the right inferior phrenic artery (IPA) at the level of the diaphragm, which anastomosed with the superior RRA (Fig 2, B). Superior LRA catheterization demonstrated severe RAS with a distal aneurysm (Fig 2, C). Subsequent renal vein sampling revealed the right renal vein to systemic renin ratio of 11.3, diagnostic of RVH.
增强腹部主动脉造影显示右肾充盈延迟（图 2、A）。主动脉弓血管造影显示 RIMA 供应右肾的上三分之二。RIMA 的选择性导管插入术证实了右侧肾实质通过膈肌水平的侧支填充右肾实质，该横膈膜水平与上 RRA 吻合（图 2、B）。LRA 上级导管插入术显示严重的 RAS （ RA stenosis (RAS) ，肾动脉狭窄）伴远端动脉瘤 （图 2、C）。随后的肾静脉取样显示右肾静脉与全身肾素比值为 11.3，诊断为 RVH肾血管性高血压 （RVH）。
（A） 主动脉图显示双侧肾脏充盈不对称。
Fig 2 (A) Aortogram demonstrating asymmetric filling of bilateral kidneys.
RRA, right renal artery.
(B) Angiogram demonstrating filling of the upper two-thirds of the right renal parenchyma via a collateral network originating from the right internal mammary artery (RIMA) and right inferior phrenic artery (IPA).
（B） 血管造影显示通过源自右乳内动脉 （RIMA） 和右下膈动脉 （IPA） 的侧支网络充盈右肾实质的上三分之二。RRA, right renal artery.
(C) Angiogram demonstrating superior left RA (LRA) aneurysm. （C） 血管造影显示左上 RA （LRA） 动脉瘤。RRA，右肾动脉。
治疗计划：
Given the patient's RVH, young age, and unique anatomy of renal perfusion, open repair via a midline approach was decided to access the bilateral RAs. Although the renin level was sampled only from the right side, given the severity of the superior LRA stenosis, it was thought that the patient’s RVH was caused by bilateral RA stenoses. Although it would have been possible to coil and aneurysm and stent the superior LRA, open reconstruction was chosen to maximize the preservation of the left renal parenchyma for the following reasons: (1) his young age, (2) the small diameter of left superior RA size (<4 mm), which is associated with poorer outcomes with endovascular interventions, (3) the diagnosis of AS, which can result in renal dysfunction, and (4) the planned open reconstruction of the RRA.
鉴于患者的 RVH、年轻和独特的肾灌注解剖结构，决定通过中线入路进行开放修复以进入双侧 RAs。虽然肾素水平仅从右侧取样，但考虑到 LRA 上狭窄的严重程度，认为患者的 RVH 是由双侧 RA 狭窄引起的。
尽管可以对上 LRA 进行弹簧圈和动脉瘤并支架置入，但选择开放重建以最大限度地保留左肾实质，原因如下：（1） 他年轻，（2） 左上 RA 大小的小直径（<4 mm），这与血管内介入治疗的不良结果相关， （3） 可导致肾功能不全的 AS 诊断，以及 （4） 计划对 RRA 进行开放重建。
Upon exposure, the superior RRA, the larger of the two RRAs, was found to be proximally occluded and reconstituted via a large right IPA (Fig 3, A).
The superior LRA aneurysm was noted (Fig 3, B). Aortosuperior RRA bypass grafting was performed using a 6-mm woven polyester graft (Fig 3, C). A prosthetic graft was used in this patient owing to the lack of adequate great saphenous vein. The superior LRA aneurysm was resected and LRA reimplanted onto the aorta (Fig 3, C). The patient tolerated the procedure well with an uneventful postoperative course, being discharged home on postoperative day 5 with normal renal function.
Tissue samples from bilateral RAs demonstrated disordered intimal hyperplasia, suggestive of a variant of intimal type of fibromuscular dysplasia (FMD).
暴露后，发现上 RRA（两个 RRA 中较大的一个）在近端闭塞并通过一个大的右侧 IPA 重建（图 3、A）。
注意到 LRA 上动脉瘤 （图 3， B）。主动脉上 RRA 旁路移植术使用 6 毫米编织聚酯移植物进行（图 3、C）。由于缺乏足够的大隐静脉，该患者使用了假体移植物。切除 LRA 上动脉瘤，并将 LRA 重新植入主动脉（图 3、C）。患者对手术的耐受性良好，术后过程顺利，术后第 5 天出院回家，肾功能正常。
来自双侧 RA 的组织样本显示无序的内膜增生，提示内膜型纤维肌发育不良 （FMD） 的变体。
图 3术中图像显示 （A） 右上肾动脉 （RRA） 起源闭塞，右侧膈下动脉 （IPA） 侧支支占较大。（B） 左上 RA （LRA） 动脉瘤。（C） 使用 6 mm 编织聚酯移植物和 LRA 一期吻合术的主动脉到 RRA 旁路术。
RA anomaly is common. During the development of renal vasculature, multiple mesonephric arteries begin to regress around the 8th week of gestation, leaving a single branch that becomes the main RA.1 Supernumerary RAs arise from the persistence of these arteries during development, often originating from the abdominal aorta or iliac arteries.2 The prevalence in general population ranges from 20% to 40%, with 10% being bilateral.2-5 Collateral flow to the RA can also arise from the lumbar, intercostal, ureteral, adrenal, and gonadal arteries.6,7 The right IPA, typically originating from the aorta, can also arise from the celiac axis, hepatic artery, left gastric artery, or RRA.8,9 In this patient, the angiographic findings indicated that perfusion of the superior RRA occurred via the RIMA and IPA network. Occlusion of the proximal superior RRA with reconstitution via the aforementioned collateral network is the most salient finding of this report (Fig 3, A). The collateral perfusion of the right upper pole resulted in hypoperfusion, activating the renin-angiotensin-aldosterone axis, which consequently resulted in RVH. The right renal vein to systemic renin ratio of >1.5 confirmed the hemodynamic significance of the aberrant RRA anatomy, thereby warranting surgical intervention.10
RA 异常很常见。在肾脉管系统的发育过程中，多条中肾动脉在妊娠第 8 周左右开始退化，留下一个分支成为主要的 RA。 1 多发性 RA 是由于这些动脉在发育过程中的持续存在而引起的，通常起源于腹主动脉或髂动脉。 2 一般人群的患病率为 20%-40%，其中 10% 为双侧。 2-5 流向 RA 的侧支血也可来自腰动脉、肋间动脉、输尿管动脉、肾上腺动脉和性腺动脉。 6 7 右侧 IPA 通常起源于主动脉，也可以起源于腹腔轴、肝动脉、左胃动脉或 RRA。 8 9 在该患者中，血管造影结果表明上 RRA 的灌注是通过 RIMA 和 IPA 网络发生的。通过上述侧支网络重建近端上 RRA 的闭塞是本报告最突出的发现（图 3、A）。
右上极的侧支灌注导致灌注不足，激活肾素-血管紧张素-醛固酮轴，从而导致 RVH。右肾静脉与全身肾素比值为 >1.5 证实了异常 RRA 解剖结构的血流动力学意义，因此需要手术干预。 10
Often associated with middle-aged women, FMD can also occur in younger individuals and in men. FMD, particularly in younger patients, is a well-known cause of RAS and RVH.12 The presence of at least one focal or a multifocal arterial lesion is required to establish the diagnosis.12 Although the pathology report was not consistent with classic FMD, bilateral RA focal stenosis and occlusion may represent a variant of FMD, and histopathological analysis was suggestive of an intimal (focal) type of FMD. Consequently, this patient requires life-long surveillance of other manifestations of FMD, including carotid stenosis and intracranial aneurysm.
FMD 通常与中年女性有关，也可能发生在年轻人和男性中。FMD，尤其是在年轻患者中，是 RAS 和 RVH 的一个众所周知的原因。 12 需要存在至少一个局灶性或多灶性动脉病变才能确定诊断。 12 虽然病理报告与经典的 FMD 不一致，但双侧 RA 局灶性狭窄和闭塞可能代表 FMD 的一种变体，并且组织病理学分析提示内膜（局灶性）类型的 FMD。因此，该患者需要终身监测 FMD 的其他表现，包括颈动脉狭窄和颅内动脉瘤。
AS is a genetic disorder characterized by nephropathy resulting from progressive glomerulopathy, high-tone sensorineural hearing loss, and ocular abnormalities. It is caused by pathogenic variants in genes that encode collagen type IV, including COL4A3, COL4A4, and COL4A5.13 There are three types of AS: X-linked, autosomal recessive, and autosomal dominant. Autosomal-dominant AS, which our patient has, is rare and results from pathogenic variants in the COL4A3 or COL4A4 genes. Although AS is primarily known for its impact on the glomerular basement membrane leading to chronic kidney disease, it can also be associated with vascular abnormalities, particularly in X-linked AS caused by pathogenic variants in COL4A5. Although there is no known association between AS and RAS, several case reports describe aneurysms in patients with the severe form of AS (ie, X-linked), including intracranial aneurysm, RA pseudoaneurysm, aortic root dilatation, aortic aneurysms, and small vessel aneurysms.14-18 Although this patient's hematuria can be attributed to AS, it is unclear whether the LRA aneurysm is related to AS or represents poststenotic aneurysmal dilatation.
AS(Alport syndrome ,AS--Alport 综合征 ) 是一种遗传性疾病，其特征是进行性肾小球病、高调感音神经性听力损失和眼部异常引起的肾病。它是由编码 IV 型胶原蛋白的基因中的致病性变异引起的，包括 COL4A3、COL4A4 和 COL4A5。 13
AS 分为三种类型：X 连锁、常染色体隐性和常染色体显性遗传。我们的患者患有常染色体显性遗传性 AS，这种情况很少见，是由 COL4A3 或 COL4A4 基因的致病性变异引起的。虽然 AS 主要以其对导致慢性肾病的肾小球基底膜的影响而闻名，但它也可能与血管异常有关，尤其是在由 COL4A5 致病性变异引起的 X 连锁 AS 中。虽然 AS 和 RAS 之间没有已知的关联，但一些病例报告描述了严重 AS 患者（即 X 连锁）患者的动脉瘤，包括颅内动脉瘤、RA 假性动脉瘤、主动脉根部扩张、主动脉瘤和小血管动脉瘤。 14-18 虽然该患者的血尿可归因于 AS，但尚不清楚 LRA 动脉瘤是否与 AS 相关或代表狭窄后动脉瘤扩张。
Conclusions 结论
This case report describes an extremely rare RA anatomy and perfusion pathway involving the RIMA and right IPA network, adding to the existing knowledge of complexity and variability of the RA anomalies. The need for comprehensive evaluations for alternate blood supplies to the kidneys is underscored. This case illustrates the importance of a detailed anatomical assessment in patients with RVH and that successful outcomes can be obtained through appropriate surgical interventions.
本病例报告描述了一种极其罕见的 RA 解剖结构和灌注通路，涉及 RIMA 和右侧 IPA 网络，增加了对 RA 异常复杂性和可变性的现有认识。强调需要对肾脏的替代血液供应进行综合评估。本病例说明了对 RVH 患者进行详细解剖评估的重要性，并且可以通过适当的手术干预获得成功的结果。
