Crossed renal ectopia is an unusual congenital anomaly, probably produced by abnormal development of the ureteral bud. The deformity itself produces no symptoms and the clinical presentation generally is for obstruction and infection. The pediatric patients present most often with multiple congenital anomalies, especially of the skeletal system. We report the two cases of crossed renal ectopia with fusion with review of literatures.
Congenital Abnormalities ; Humans ; Ureter

Horseshoe kidney and retrocaval ureter are uncommon congenital anomalies of the genitourinary system that are easily diagnosed by typical imaging features. Both anomalies presenting in one patient is a rare disease characterized by isthmus of horseshoe kidney between the abdominal aorta and inferior vena cava. The clinical diagnosis and treatment of horseshoe kidney with retrocaval ureter remain a challenge. Here, we reported a case of a 44-year-old man with the two anomalies who was preoperatively diagnosed by unenhanced computed tomography scanning immediately after retrograde pyelography. The literatures on such combined anomalies are reviewed and the diagnostic evaluation and surgical management of this rare entity are discussed.
Adult ; Humans ; Kidney ; abnormalities ; surgery ; Male ; Ureter ; abnormalities ; surgery

OBJECTIVETo observe the imaging findings of congenital megaureter in order to enhance the understanding of this disease.

METHODSImage data of 5 patients with congenital megaureter and 2 misdiagnosed patients were analyzed, and image findings of congenital megaureter were summarized.Elscint Prestig 2.0T superconductive magnetic resonance urography (MRU) with conventional sequence (spin-echo, T1WI560 ms/16 ms; fast spin-echo, T2WI 9600 ms/96 ms ) was performed. Raw data were acquired with fastspin-echo sequence from heavy T2-weighted image (9600 ms/120 ms). Post-processing method of MRU was the maximum intensity projection with three-dimensional reconstruction in the workstation. Intravenous pyelography (IVP) was conducted, in which X-rayfilms were taken 7 minutes, 15 minutes, and 30 minutes after injecting contrast agent, exceptthat in 2 patients the films were taken delayed at 60 and 90 minutes .X-ray retrograde pyelography was performed on 2 patients, successful in one butfailed in the other.

RESULTSThe dilated ureter showed hypointensity on T1-weighted images and hyperintensity on T2-weighted images in conventional MRI. The mass wall was intact, uniform in thickness, and showing hypointensity on T1-weighted and T2-weighted images. The MRU images showed a retroperitoneal mass appearing as an elongated tubular cystic structure spreading from kidney to bladder. MRU also revealed dilated calices and renal pelvis, pelviureteric obstruction, and renal duplication. The main signs of congenital megaureter in X-urography was significant dilatation of ureter, or normal renal pelvis with ureter dilatation, hydronephrosis, deformity, and displacement.

CONCLUSIONSMRU with X-urography could visualizethe characteristics of congenital megaureter, including the dilation of renal pelvis and ureter, calculi, urinary tract duplication, and stenosis location. The two techniques can complement each other in disease diagnosis and provide more detailed information for preoperative treatment.

Cystic dysplasia of the testis is a rare benign congenital lesion of the rete testis that is often associated with renal and ureteral abnormalities. Herein, a case of cystic dysplasia of the testis, associated with an ipsilateral hypoplastic type of renal dysplasia and an ureterovesical junction obstruction, is reported in a 7-year-old boy.
Child ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Humans ; Male ; Rete Testis ; Testis* ; Ureter ; Urogenital Abnormalities

Abnormalities, Multiple ; diagnostic imaging ; Adult ; Female ; Humans ; Kidney Calculi ; diagnostic imaging ; Radiography ; Ureter ; abnormalities ; diagnostic imaging

We report a rare case of a left ejaculatory duct that allotropically protrudes towards or invades the left vesicle triangular area with its dead end. The patient simultaneously exhibited multiple congenital malformations of the homolateral urogenital system, such as absence of the left kidney, dysplasia and allotopia of the left seminal vesicle, absence of the left ureterostoma, separation between the left testis and the epididymis tail, and maldevelopment of the left testis. According to all clinical and laboratory evidence, the case represented a new syndrome, which we named Wuyang's syndrome. It involved a rare phenomenon in embryonic development; the dysplastic proximal vas precursor, having intruded into a common mesonephric duct and accidentally encroaching on the ureteric bud position, resulted in the absence or dysplasia of the homolateral urinary tract and ectopic invasion of the bladder by the homolateral seminal tract.
Abnormalities, Multiple ; pathology ; surgery ; Adult ; Ejaculatory Ducts ; abnormalities ; pathology ; surgery ; Humans ; Magnetic Resonance Imaging ; Male ; Syndrome ; Ureter ; abnormalities ; Urinary Bladder ; abnormalities ; pathology ; surgery ; Wolffian Ducts ; abnormalities

We present a case of vaginal ectopic ureter with ipsilateral partial duplication of the upper ureter (Y-type ureter), ipsilateral hypoplastic pelvic kidney and bicornuate uterus in a 20-year-old woman who presented with mild urinary incontinence since infancy. Ultrasonography, computed tomography and intravenous pyelography examination showed a left kidney with no evidence of a right kidney. Cystourethroscopy showed absence of the right hemitrigone. Magnetic resonance (MR) urography demonstrated the presence of a bicornuate uterus, an ectopic dysplastic right kidney in the pelvic cavity, and a right ureter that terminates in the vaginae fornix. The patient underwent right nephroureterectomy and urinary continence was restored completely. Although congenital malformations of the urinary tract are frequently associated with genital tract abnormalities, to best our knowledge, this is the first report of the coexistence of all of these anomalies in an individual. Our report also highlights the importance of MR urography in the diagnosis of such rare and complex anomalies.
Female ; Humans ; Kidney ; abnormalities ; pathology ; Kidney Diseases ; diagnosis ; pathology ; Magnetic Resonance Imaging ; Ureter ; abnormalities ; pathology ; Urinary Incontinence ; etiology ; Uterus ; abnormalities ; pathology ; Vagina ; abnormalities ; pathology ; Young Adult

Vitamin D-dependent rickets(VDDR) is a rare autosomal disorder, characterized by hypocalcemia, hypophosphatemia, increased alkaline phosphatase, secondary hyperparathyroidism and many other clinical features. Type I VDDR is due to congenital defects of renal 1alpha-hydroxylase, the enzyme responsible for the conversion of 25-(OH)D3 to 1,25-(OH)2D3. Type II VDDR arise from target organ resistance to 1,25-(OH)2D3. Unilateral renal aplasia is generally thought to result from a lack of induction of the metanephric blastema from the ureteral bud, which may be secondary to ureteral bud maldevelopment and/or to a problem with the formation of the mesonephric duct. The incidence of unilateral renal aplasia is approximately 1/500-3,200. Type 1 VDDR associated with unilateral renal aplasia has not been reported yet. Thus we report a case of a 3 month old female infant diagnosed as type 1 VDDR with unilateral aplasia of kidney.
Alkaline Phosphatase ; Congenital Abnormalities ; Female ; Humans ; Hyperparathyroidism, Secondary ; Hypocalcemia ; Hypophosphatemia ; Incidence ; Infant ; Kidney ; Rickets ; Ureter ; Urogenital Abnormalities ; Vitamins ; Wolffian Ducts

Duplication is the most common type of congenital anomaly of the ureter. It occurs more frequently in females and it is often accompanied by an ectopic orifice. Ectopic ureter is usually derived from the upper pole of the ipsilateral kidney and its orifice is attached medial & inferior from the normal opening according to Weigert-Meyer's law. Thus, sometimes the ectopic ureteral opening is made at a distal part of the external sphincter or even out of the urinary system. These conditions may result in urinary incontinence in female patients, so they can be treated early at the mean age of 5 and it's not common that ectopic ureter accompanying complete duplication of the ureter is presented in adults. We report here on a case of ectopic ureter accompanying complete duplication of the ureter in a 44 year old female who presented with leukorrhea.
Adult ; Female* ; Humans ; Jurisprudence ; Kidney ; Leukorrhea ; Middle Aged* ; Ureter* ; Urinary Incontinence ; Urogenital Abnormalities ; Vaginal Discharge*

Crossed renal ectopia with fusion is an unusual congenital anomaly, probably produced by abnormal development of the ureteral bud. The deformity itself produces no symptoms but it presents possible urinary tract infection, obstuction and stone formation, and other anomaly in body. Herein we report two cases of crossed fused renal ectopia complained of abdominal mass in 28 year old female and abdominal pain after trauma in 14 year-old boy.
Abdominal Pain ; Adolescent ; Adult ; Congenital Abnormalities ; Female ; Humans ; Male ; Ureter ; Urinary Tract Infections