{"title":"双侧肾淋巴管扩张症:一种通过微创方法治疗的罕见肾囊性疾病--病例报告","authors":"Navaneethakrishnan Venkatachalam, Anandan Murugesan","doi":"10.1186/s12301-023-00399-7","DOIUrl":null,"url":null,"abstract":"Renal cystic diseases are one of the commonest renal lesions encountered in clinical practice. Although common, most of the cysts are solitary, benign, asymptomatic and seldom clinically significant. But, renal lymphangiectasia is an exception. These are rare lymphatic malformation seen around the kidneys and in the retroperitoneum. It masquerades clinically like ADPKD and renal tumors and radiologically like a complex renal cyst. Although the cyst is benign, it possesses a significant impact on the quality of life. Because of its rarity, the management of this condition has not been well defined in the literature. A clinician must be aware of this rare condition and able to differentiate it from other similar conditions to aid in appropriate management. Hence, we present a case report of a female with bilateral renal lymphangiectasia managed successfully by laparoscopic excision. A 34-year-old hypertensive female came with complaints of bilaterally progressive flank masses for 3 months and breathlessness for 2 weeks. On examination, she had bilateral pitting pedal edema, bilateral palpable renal mass and ascites. She had nephrotic range proteinuria, hypoalbuminemia and normal renal function. Imaging showed 22-cm bilateral peri-renal and hilar multi-loculated cystic lesions, suggestive of bilateral renal lymphangiectasia. Antihypertensives and percutaneous interventions were not successful in relieving her symptoms. Subsequently, she was managed with laparoscopic excision on both sides. After surgery, she had an uneventful postoperative period and good symptomatic relief. No recurrence of the lesion found in follow-up CT imaging after 18 months. Renal lymphangiectasia is a rare yet clinically significant cystic lesion of the kidney. It can be diagnosed confidently by noninvasive imaging modalities. Medical treatment offered for mild symptomatic disease. Patients with severe symptoms need surgical intervention especially if it is not responding to medical management. Minimal invasive approach is feasible and successful in the management of this voluminous disease.","PeriodicalId":7432,"journal":{"name":"African Journal of Urology","volume":"22 1","pages":""},"PeriodicalIF":0.5000,"publicationDate":"2024-01-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Bilateral renal lymphangiectasia: a rare renal cystic disease managed by minimal invasive approach—a case report\",\"authors\":\"Navaneethakrishnan Venkatachalam, Anandan Murugesan\",\"doi\":\"10.1186/s12301-023-00399-7\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Renal cystic diseases are one of the commonest renal lesions encountered in clinical practice. Although common, most of the cysts are solitary, benign, asymptomatic and seldom clinically significant. But, renal lymphangiectasia is an exception. These are rare lymphatic malformation seen around the kidneys and in the retroperitoneum. It masquerades clinically like ADPKD and renal tumors and radiologically like a complex renal cyst. Although the cyst is benign, it possesses a significant impact on the quality of life. Because of its rarity, the management of this condition has not been well defined in the literature. A clinician must be aware of this rare condition and able to differentiate it from other similar conditions to aid in appropriate management. Hence, we present a case report of a female with bilateral renal lymphangiectasia managed successfully by laparoscopic excision. A 34-year-old hypertensive female came with complaints of bilaterally progressive flank masses for 3 months and breathlessness for 2 weeks. On examination, she had bilateral pitting pedal edema, bilateral palpable renal mass and ascites. She had nephrotic range proteinuria, hypoalbuminemia and normal renal function. Imaging showed 22-cm bilateral peri-renal and hilar multi-loculated cystic lesions, suggestive of bilateral renal lymphangiectasia. Antihypertensives and percutaneous interventions were not successful in relieving her symptoms. Subsequently, she was managed with laparoscopic excision on both sides. After surgery, she had an uneventful postoperative period and good symptomatic relief. No recurrence of the lesion found in follow-up CT imaging after 18 months. Renal lymphangiectasia is a rare yet clinically significant cystic lesion of the kidney. It can be diagnosed confidently by noninvasive imaging modalities. Medical treatment offered for mild symptomatic disease. Patients with severe symptoms need surgical intervention especially if it is not responding to medical management. Minimal invasive approach is feasible and successful in the management of this voluminous disease.\",\"PeriodicalId\":7432,\"journal\":{\"name\":\"African Journal of Urology\",\"volume\":\"22 1\",\"pages\":\"\"},\"PeriodicalIF\":0.5000,\"publicationDate\":\"2024-01-03\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"African Journal of Urology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1186/s12301-023-00399-7\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"UROLOGY & NEPHROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"African Journal of Urology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1186/s12301-023-00399-7","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"UROLOGY & NEPHROLOGY","Score":null,"Total":0}
Bilateral renal lymphangiectasia: a rare renal cystic disease managed by minimal invasive approach—a case report
Renal cystic diseases are one of the commonest renal lesions encountered in clinical practice. Although common, most of the cysts are solitary, benign, asymptomatic and seldom clinically significant. But, renal lymphangiectasia is an exception. These are rare lymphatic malformation seen around the kidneys and in the retroperitoneum. It masquerades clinically like ADPKD and renal tumors and radiologically like a complex renal cyst. Although the cyst is benign, it possesses a significant impact on the quality of life. Because of its rarity, the management of this condition has not been well defined in the literature. A clinician must be aware of this rare condition and able to differentiate it from other similar conditions to aid in appropriate management. Hence, we present a case report of a female with bilateral renal lymphangiectasia managed successfully by laparoscopic excision. A 34-year-old hypertensive female came with complaints of bilaterally progressive flank masses for 3 months and breathlessness for 2 weeks. On examination, she had bilateral pitting pedal edema, bilateral palpable renal mass and ascites. She had nephrotic range proteinuria, hypoalbuminemia and normal renal function. Imaging showed 22-cm bilateral peri-renal and hilar multi-loculated cystic lesions, suggestive of bilateral renal lymphangiectasia. Antihypertensives and percutaneous interventions were not successful in relieving her symptoms. Subsequently, she was managed with laparoscopic excision on both sides. After surgery, she had an uneventful postoperative period and good symptomatic relief. No recurrence of the lesion found in follow-up CT imaging after 18 months. Renal lymphangiectasia is a rare yet clinically significant cystic lesion of the kidney. It can be diagnosed confidently by noninvasive imaging modalities. Medical treatment offered for mild symptomatic disease. Patients with severe symptoms need surgical intervention especially if it is not responding to medical management. Minimal invasive approach is feasible and successful in the management of this voluminous disease.