Khanduri Sachin, Chaudhary Mriganki, Sabharwal Tushar, Goyal Aakshit, Katyal Gaurav
Radiodiagnosis, Era's Lucknow Medical College and Hospital.
Cureus. 2017 Apr 22;9(4):e1187. doi: 10.7759/cureus.1187.
Localized cystic disease of the kidney is a rare, non-familial condition. Its imaging and clinical features are unique and need to be differentiated from autosomal dominant polycystic kidney disease and focal cystic masses such as multicystic nephroma and cystic renal cell carcinoma. It is always restricted to one kidney and is characterized by multiple cysts of varying sizes separated by residual normal renal tissue.
This study reports 12 cases of localized cystic disease of the kidney based on imaging findings and clinical histories. The modalities of choice were ultrasonography followed by contrast-enhanced computed tomography. Eight out of 12 patients were men and the average age of presentation was 46 years. The screening of family members and relatives was done to rule out the differentials.
Localized cystic disease of kidney was diagnosed in all the patients and it presented in two different forms. In three patients, multiple cysts involved whole of the kidney, resulting in thinned-out residual renal parenchyma. In the rest nine patients it remained localised to a particular segment of the kidney. No cysts were observed in the contralateral kidney in seven patients, and one or two simple cysts were observed in five. Clinical presentations included only flank pain in six patients, flank pain with palpable abdominal mass in four patients, two patients presented as asymptomatic cases with diagnosis as an incidental finding and one patient with hematuria. Eight patients underwent imaging and two underwent clinical follow-up for a period of two years showing stability of the disease. One patient underwent nephrectomy for suspected renal neoplasm.
Localized cystic disease of the kidney is a unilateral, rare and stable disease that has two different forms of presentations. Its imaging findings should be clearly understood so as to not classify it as a separate disease and avoid unnecessary surgery. It rarely leads to hypertension or polycythemia, and until then no definitive management is required. It can be followed up using imaging techniques and requires nephrectomy only when the suspicion of malignancy is strong.
肾脏局限性囊性疾病是一种罕见的非家族性疾病。其影像学和临床特征独特，需要与常染色体显性遗传性多囊肾病以及局灶性囊性肿块（如多囊性肾瘤和囊性肾细胞癌）相鉴别。它总是局限于一侧肾脏，其特征是大小不一的多个囊肿被残余的正常肾组织分隔开。
本研究基于影像学表现和临床病史报告了12例肾脏局限性囊性疾病。首选的检查方法是超声检查，随后是增强计算机断层扫描。12例患者中有8例为男性，平均发病年龄为46岁。对家庭成员和亲属进行筛查以排除鉴别诊断。
所有患者均被诊断为肾脏局限性囊性疾病，其表现为两种不同形式。3例患者中，多个囊肿累及整个肾脏，导致残余肾实质变薄。其余9例患者中，病变局限于肾脏的特定节段。7例患者对侧肾脏未观察到囊肿，5例观察到一两个单纯性囊肿。临床表现包括6例患者仅有胁腹痛，4例患者胁腹痛伴有可触及的腹部肿块，2例患者表现为无症状病例，诊断为偶然发现，1例患者有血尿。8例患者接受了影像学检查，2例患者进行了为期两年的临床随访，疾病表现稳定。1例患者因疑似肾肿瘤接受了肾切除术。
肾脏局限性囊性疾病是一种单侧、罕见且稳定的疾病，有两种不同的表现形式。应清楚了解其影像学表现，以免将其误诊为其他疾病并避免不必要的手术。它很少导致高血压或红细胞增多症，在此之前无需进行确定性治疗。可以使用影像学技术进行随访，只有在高度怀疑恶性肿瘤时才需要进行肾切除术。
