Nephrectomy in a Patient with Medullary Sponge Kidney Associated with Severe Nephrocalcinosis and Nephrolithiasis: A Rare Condition  

作　　者：Landry Oriole Mbouché Achille Aurèle Mbassi Junior Mekeme Mekeme Axel Stéphane Nwaha Makon Pierre Joseph Fouda Fru Fobuzshi Angwafo III Landry Oriole Mbouché;Achille Aurèle Mbassi;Junior Mekeme Mekeme;Axel Stéphane Nwaha Makon;Pierre Joseph Fouda;Fru Fobuzshi Angwafo III(Department of Surgery and Specialities, Faculty of Medicine and Biomedical Sciences, Yaoundé, Cameroon;Department of Uro-Andrology, Yaoundé Central Hospital, Yaoundé, Cameroon;Department of Surgery, Higher Institute of Medical Technology, Yaoundé, Cameroon)

机构地区：[1]Department of Surgery and Specialities, Faculty of Medicine and Biomedical Sciences, Yaoundé, Cameroon [2]Department of Uro-Andrology, Yaoundé Central Hospital, Yaoundé, Cameroon [3]Department of Surgery, Higher Institute of Medical Technology, Yaoundé, Cameroon

出　　处：《Open Journal of Urology》2021年第9期332-341,共10页泌尿学期刊（英文）

摘　　要：<strong>Background:</strong> Medullary sponge kidney (MSK) is a disturbance of renal development characterized by cystic dilation and diffuse precalyceal duct ectasia. The disease affects both genders in equal proportions and is generally diagnosed in adulthood, as a result of recurrent calcium nephrolithiasis and nephrocalcinosis. The most frequently encountered manifestations being renal colic, microscopic or macroscopic hematuria, and fever. The intravenous pyelogram is standard for diagnosis and metabolic workup is required to identify the underlying cause. The main goal of treatment is to prevent recurrence and disease progression. Though considered a benign condition, a nephrectomy may often be required in patients presenting late with irreversible complications and end-stage renal disease.<strong> Aim:</strong> To highlight and discuss the presentation and management of a rare case of nephrocalcinosis and nephrolithiasis secondary to the medullary sponge kidney. <strong>Case presentation:</strong> We report herein the case of a 56-year-old male with long-standing hematuria in whom a diagnosis of medullary sponge kidney disease was made and he underwent a left total nephrectomy. The postoperative course was uneventful. <strong>Conclusion:</strong> Nephrocalcinosis and nephrolithiasis are complications of MSK and can result in irreversible renal damage. A high index of suspicion is necessary for patients presenting with renal colic, recurrent urinary tract infections, or hematuria for prompt diagnosis and management.<strong>Background:</strong> Medullary sponge kidney (MSK) is a disturbance of renal development characterized by cystic dilation and diffuse precalyceal duct ectasia. The disease affects both genders in equal proportions and is generally diagnosed in adulthood, as a result of recurrent calcium nephrolithiasis and nephrocalcinosis. The most frequently encountered manifestations being renal colic, microscopic or macroscopic hematuria, and fever. The intravenous pyelogram is standard for diagnosis and metabolic workup is required to identify the underlying cause. The main goal of treatment is to prevent recurrence and disease progression. Though considered a benign condition, a nephrectomy may often be required in patients presenting late with irreversible complications and end-stage renal disease.<strong> Aim:</strong> To highlight and discuss the presentation and management of a rare case of nephrocalcinosis and nephrolithiasis secondary to the medullary sponge kidney. <strong>Case presentation:</strong> We report herein the case of a 56-year-old male with long-standing hematuria in whom a diagnosis of medullary sponge kidney disease was made and he underwent a left total nephrectomy. The postoperative course was uneventful. <strong>Conclusion:</strong> Nephrocalcinosis and nephrolithiasis are complications of MSK and can result in irreversible renal damage. A high index of suspicion is necessary for patients presenting with renal colic, recurrent urinary tract infections, or hematuria for prompt diagnosis and management.

关 键 词：Medullary Sponge Kidney NEPHROLITHIASIS NEPHROCALCINOSIS NEPHRECTOMY 

分 类 号：R69[医药卫生—泌尿科学]