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Medullary sponge kidney is a benign asymptomatic developmental anomaly of the kidney mostly seen in adult females. Presentation in childhood is uncommon. Urinary tract infection, nephrolithiasis, hematuria and hypercalciuria are the common complications. We report a eleven-year-old female child who presented with recurrent urinary tract infection and nephrolithiasis and was found to have bilateral medullary sponge kidney.
"Medullary sponge kidney" applies to pathologically dilated collecting tubules within one or more renal pyramids of one or both kidneys, almost always diagnosed radiographically, of uncertain etiology, and presenting a clinical spectrum varying from an asymptomatic, incidental finding to severely complicating calcareous-infective disease, renal insufficiency, and death. Recognition of the characteristic urographic pattern affords the patient presenting clinically with hematuria, ureteral colic, urinary tract infection, or nephrocalcinosis a prompt diagnosis with a frequently benign prognosis, and usually averts more extensive or invasive investigations.
Medullary sponge kidney is described and discussed with special reference to the radiological picture; its status among cystic diseases of the kidney and its differential diagnosis are examined in detail, as are its relations with nephrocalcinosis and distal tubular acidosis of the kidney. X-ray of numerous cases are given.
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A case of simultaneous adult polycystic kidney disease and medullary sponge kidney is reported. Such an occurrence is rare but suggests that these two conditions share a common pathogenesis.
A 22-year-old woman with hemoglobin SC who was hematologically asymptomatic, developed gross hematuria associated with urinary tract infection, without any urological antecedents. Investigations revealed a unilateral hematuria due to papillary necrosis on the left kidney. Medullary sponge kidney was also discovered by radiologic investigations. Papillary cysts could play a role in the occurrence of papillary necrosis.
Medullary sponge kidney is reported in six children aged 2-18 years. One child was asymptomatic; the others had hematuria or a urine-concentrating defect. Renal function and size were otherwise normal, as was liver function. The diagnosis was made at excretory urography according to criteria established in adults. Sonography revealed hyperechogenic pyramids, at first at the periphery, later generalized. Computed tomography proved this to be calcium. Medullary sponge kidney is rare but exists in children. Sonography is very sensitive to the pyramidal nephrocalcinosis that complicates this disease and explains the frequent presenting symptom of hematuria in these children.
Medullary sponge kidney (MSK), parathyroid adenoma, renal cell carcinoma, and renal-leak hypercalciuria coincided in 1 female patient. Renal-leak hypercalciuria was not corrected by removal of a parathyroid adenoma. Since the patient had renal tubular acidosis (RTA), alkali treatment was conducted and resulted in the correction of hypercalciuria. Renal cell carcinoma eventually developed and MSK was confirmed histologically. This case suggests that MSK and primary hyperparathyroidism occurred independently.
Thirteen patients with medullary sponge kidney underwent a short ammonium chloride loading test to investigate their renal acidification capacity. All but 1 presented with a history of recurrent renal calculi and showed bilateral widespread renal medullary calcification on X-ray examination. Nine patients had some form of renal acidification defect; 8 had the distal type of renal tubular acidosis, 2 the complete and 6 the incomplete form. One patient had proximal renal tubular acidosis. These findings, which suggest that renal acidification defects play an important role in the pathogenesis of renal calculi in medullary sponge kidney, have considerable therapeutic implications.
The diagnosis of medullary sponge kidney ( MSK ) is usually made as an incidental finding on pyelogram. The essential abnormality of this condition is a " paintbrush " appearance of the calyces due to the presence of contrast within dilated collecting ducts. Since the radiologic features are similar to those of papillary necrosis, a history of analgesic abuse may make the differential diagnosis difficult. We describe a patient whose urogram strongly suggested MSK but who also had a history of analgesic abuse. Special studies demonstrating completely normal renal function and proven MSK in the patient's mother allowed the diagnosis of medullary sponge kidney. We reviewed the similarities and differences between MSK and papillary necrosis to identify features of diagnostic significance.
The prevalence of medullary sponge kidney in patients with nephrolithiasis and the issue of whether or not medullary sponge kidney has a role in the pathogenesis of renal stones are controversial. We studied the excretory urograms of 280 patients with nephrolithiasis and 280 patients without either nephrolithiasis or a history of renal stones to determine the frequencies of medullary sponge kidney in the two groups. The criterion for the diagnosis of medullary sponge kidney was the presence of a minimum of three linear or round collections of contrast material within one renal papilla. In the patients with nephrolithiasis, we also looked for biochemical evidence of metabolic causes of renal stones. The frequency of medullary sponge kidney was 12% in patients with nephrolithiasis compared with 1% in patients without nephrolithiasis. The statistical difference was highly significant (chi square = 27.1; p less than .001). Metabolic disorders accounting for the lithiasis were detected in 93% of the patients with stones without medullary sponge kidney. Such evidence was present in 60% of patients with stones and medullary sponge kidney. The statistical difference was significant (chi square = 25.8; p less than .001). Our results suggest that medullary sponge kidney is a cause of nephrolithiasis.
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Medullary sponge kidney is a developmental disorder characterized by ectatic and cystic malformation of the collecting ducts and tubules. Clinical manifestations include urinary tract infections, renal stones, and hematuria. It can be associated with other developmental disorders. A case of medullary sponge kidney associated with congenital hemihypertrophy, complicated by nephrocalcinosis and nephrolithiasis, is reported here.
Seventeen patients with documented medullary sponge kidney and nephrolithiasis underwent metabolic evaluation. These patients constituted 3.6% of our stone-forming population. Fifteen (88%) of 17 were hypercalciuric. The most common abnormality was absorptive hypercalciuria, occurring in 59%. Only three patients (18%) had renal hypercalciuria. None of the patients had primary hyperparathyroidism. Hyperuricosuria occurred together with hypercalciuria in five patients. In two patients, the precise cause of hypercalciuria could not be determined, and in another two patients, no metabolic abnormality could be detected. Thus, the patients with medullary sponge kidney and renal stones had the same spectrum of metabolic abnormalities as the overall population of stone formers. While these patients may theoretically have a greater anatomic propensity to form stones because of their anatomic abnormality, they should be evaluated and treated appropriately for any metabolic defect.
A family is presented in which four and possibly five members of a sibship of seven are affected with polycystic kidney with congenital hepatic fibrosis. The excretory urogram in this disease may be indistinguishable from that of medullary sponge kidneys. Therefore, it is suggested that family studies be undertaken when a diagnosis of medullary sponge kidney is made.
Recognizing the changes of medullary sponge kidney (MSK) on computed tomographic (CT) scans may be of value in both diagnosis and in differentiating this from other disease states. The appearance on CT of MSK has not previously been well described. A case is reported that demonstrates ectatic renal collecting tubules visualized by CT, and techniques are suggested to optimize visualization of these changes.