PURPOSE The aim of this study was to clarify the radiologic and clinical characteristics of multiple unilateral subcapsular cortical hemorrhagic cystic disease of the kidney. METHOD Fourteen patients with unique and characteristic multiple hemorrhagic subcapsular cortical cysts of the kidney, not categorized in any existing renal cystic diseases, were retrospectively reviewed. The clinical information including age, sex, symptom, family history of renal or renal cystic disease, and laboratory data were collected. CT and MRI findings including distribution, number and size of cysts, and CT attenuation and signal intensity on T1- and T2-weighted MRI of cysts were analyzed. RESULTS All patients except one were young and none had a family history of renal or renal cystic disease. Common clinical symptoms were flank or abdominal pain and hematuria. In all cases, only the left kidney was involved at initial presentation. Cysts were small (median cyst size, 4-15 mm), numerous, and distributed mainly along the subcapsular cortex of the kidney. Cysts were hyper-attenuated on unenhanced CT, extremely hypointense on T2-weighted MRI, and mildly hyperintense on T1-weighted MRI. All patients except one had normal renal function. Imaging follow-up revealed stable or mildly progressive disease in seven patients. Two patients developed several hemorrhagic subcapsular cortical cysts in the right kidney at follow-up. Three of five patients with a renal pathology specimen showed concurrent IgA nephropathy. CONCLUSION We have identified a unique renal cystic disease with multiple unilateral subcapsular cortical hemorrhagic cystic disease of the kidney that has a characteristic manifestation both radiologically and clinically. KEY POINTS • Multiple unilateral subcapsular cortical hemorrhagic cystic disease of the kidney is a unique non-familial renal cystic disease with a characteristic manifestation both radiologically and clinically. • Most cases of multiple unilateral subcapsular cortical hemorrhagic cystic disease of the kidney are stable or slowly progressive, and do not require invasive intervention.

中文翻译：

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肾脏多发性单侧囊下皮层出血性囊性疾病：CT和MRI表现及临床特征。

目的本研究的目的是阐明肾脏多发性单侧囊下皮层皮质出血性囊性疾病的放射学和临床特征。方法回顾性分析了14例具有独特性和特征性的多发性出血性肾功能不全的肾皮质囊性囊肿的患者。收集临床信息，包括年龄，性别，症状，肾脏或肾脏囊性疾病的家族史以及实验室数据。分析了CT和MRI表现，包括囊肿的分布，数量和大小，以及T1和T2加权囊肿MRI的CT衰减和信号强度。结果除一名患者外，所有患者均为年轻患者，均无肾脏或肾脏囊性疾病家族史。常见的临床症状是胁腹或腹痛和血尿。在所有情况下，最初出现时仅累及左肾。囊肿小（囊肿中位大小为4-15 mm），数量众多，主要分布在肾脏的囊下皮质。在未增强的CT上，囊肿高度衰减，在T2加权MRI上囊肿极低，而在T1加权MRI上囊肿轻度。除一名患者外，所有患者的肾功能均正常。影像学随访显示有7名患者稳定或轻度进行性疾病。两名患者在随访时在右肾中出现了多个出血性包膜下皮层囊肿。五分之三的肾脏病理标本患者显示并发IgA肾病。结论我们已经确定了一种独特的肾脏囊性疾病，其多发性肾脏单侧囊下皮质出血性囊性疾病在放射学和临床上均具有特征性表现。要点•肾脏多发性单侧囊下皮层出血性囊性疾病是一种独特的非家族性肾囊性疾病，在放射学和临床上均具有特征性表现。•大多数多发性肾脏单侧囊下皮层出血性囊性疾病是稳定的或缓慢进展的，不需要侵入性干预。