• Clinical and Experimental Pediatrics
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• v.45 no.8
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• pp.1038-1042
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• 2002

We present a case of in-utero vesicoamniotic shunting and postnatal vesicostomy in a very low birth weight baby with posterior urethral valve syndrome. He was diagnosed as posterior urethral valve at $24^{+5$ weeks' gestation. Because of severe hydronephrosis and oligohydroamnios, the shunt was established by basket-shaped catheter at $27^{+0}$ weeks' gestation. After shunt, hydronephrosis improved. At $29^{+4}$ weeks' gestation, the shunt catheter escaped from its position and severe urinary ascites and hydronephrosis developed. At 30 weeks' gestation, the baby was born and showed elevated BUN and creatinine. On the 10th day of birth, vesicostomy was done for urinary drainage. Thereafter, the baby has been followed up for 10 months and has had recurrent episodes of urinary infection. We report the case with a brief review of literature.

• Journal of Genetic Medicine
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• v.11 no.2
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• pp.74-78
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• 2014

Sotos syndrome (SS, OMIM 117550) is characterized by prenatal and postnatal overgrowth with multiple congenital anomalies. However, there have been few cases of growth retardation caused by renal failure from infancy. We report a case of dysplasia of the bilateral kidneys with renal failure and poor postnatal growth. A 2-month-old boy visited the emergency room owing to poor oral intake and abdominal distension. He was born at the gestational age of 38 weeks with a birth weight of 4,180 g. After birth, he had feeding difficulty and abdominal distension. Upon physical examination, his height and weight were in less than the 3rd percentile, while his head circumference was in the 50th percentile on the growth curve. He also showed a broad and protruding forehead and high hairline. Blood laboratory tests showed severe azotemia; emergent hemodialysis was needed. Abdominal ultrasonography revealed bilateral renal dysplasia with multiple cysts and diffuse bladder wall thickening. A posterior urethral valve was suggested based on vesicoureterography and abdominal magnetic resonance findings. Results of a colon study to rule out congenital megacolon did not reveal any specific findings. The conventional karyotype of the patient was 46, XY. Array comparative genomic hybridization study revealed a chromosome 5q35 microdeletion including the NSD1 gene, based on which SS was diagnosed. We describe a case of SS presenting with end stage renal disease due to posterior urethral valve. The typical somatic overgrowth of SS in the postnatal period was not observed due to chronic renal failure that started in the neonatal period.

• Journal of Yeungnam Medical Science
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• v.2 no.1
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• pp.249-252
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• 1985

Polyps of the male urethra are relatively uncommon lesions. They usually arise from the prostatic urethra mostly verumontanum or Just lateral to the verumontanum. Posterior urethral polyp often presents with hematuria, hemospermia and sometimes lower urinary tract obstruction. We recently experienced a pedunculated prostatic urethral polyp in a 63-year-old man who complained of gross total hematuria and difficult micturition for several years. On rectal examination the prostate was slightly enlarged with normal consistency. Excretory urogram showed a round filling defect in the right side of the bladder neck measuring about $2{\times}2cm.$ in size. Cystourethroscopy revealed trabeculation of the vesical wall, mild bilateral prostatic hypertrophy and a round cystic mass with a long stalk arising from the prostatic floor 1 cm. Proximal to the verumontanum which caused ball-valve obstruction in the bladder neck. This pedunculated polypoid mass was then resected at its base with resectoscope and was removed transurethrally using Lowsley's grasping forceps. The specimen was proved as fibrous polyp histologically.

• Childhood Kidney Diseases
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• v.5 no.1
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• pp.30-35
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• 2001

Purpose : To evaluate tile frequency of urinary tract anomalies in male neonates less than 3 months old who presented with urinary tract infection(UTI) and to evaluate a appropriate imaging approach after first UTI. Materials and methods : During a period of 5 years from March 1994 to February 1999, 65 male infants less than 3months old(range: 4-92 days, mean: 43 days) with UTI were evaluated. Ultrasound(US) and Voiding cystourethrogram(VCUG) were done in 60 patients. Due to refusal and technological problem, 5 patients were missed. 99mTc-dimercaptosuccinic acid renal scan (99mTc-DMSA renal scan) was recommended to most patients but performed in 40 patients. Renal scan was performed at least 3 months later after urinary tract infection. Results : Urinary tract anomalies were found in 26 of 65 infants. Twenty-six had vesicoureteral reflux(VUR), two had both VUR and double ureter, two had both U and posterior urethral valve. In patients with VUR, eight had renal scar or renal atrophies. In case of renal scar or atrophy, grades of VUR were III or above. Conclusion : We suggest that US and VCUG should be routinely performed in infants(<3months)with first UTI. 99mTc-DMSA renal scan should be performed only when renal parenchymal damage was observed in US and VUR grade III or above in VCUG. (J. Korean Soc Pediatr Nephrol 5 : 30- 5, 2001)

• Clinical and Experimental Pediatrics
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• v.52 no.4
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• pp.464-470
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• 2009

Purpose : This study assessed the incidence and outcome of congenital anomalies of the kidney and urinary tract (CAKUT) detected by prenatal ultrasonography Methods : There were 906 cases of CAKUT detected by prenatal ultrasonography and postnatally confirmed at the Asan Medical Center from October 1989 to October 2007. We investigated the incidence and outcome of these cases by reviewing medical records retrospectively. Results : The order of incidence was hydronephrosis, multicystic dysplastic kidney (MCDK), duplex kidney, vesico-ureteral reflux (VUR), single kidney, hydroureteronephrosis, ectopic kidney, polycystic kidney, ureterocele, and posterior urethral valve (PUV). There were 520 cases (57.4%) of hydronephrosis, and 20% of these needed an operation due to significant obstruction. MCDK was associated with other CAKUT in 25.4% of all cases. Approximately 57.9% of duplex kidney cases needed surgical treatment due to ureterocele and VUR. VUR had a male: female ratio of 10:1. Two out of seven cases of autosomal recessive polycystic kidney had progressed to chronic renal failure. Patients with PUV were relatively uncommon, and one out of nine cases progressed to end-stage renal disease. Conclusion : CAKUTs detected by prenatal ultrasonography were composed of various anomalies, and almost all of them had a good outcome without any intervention. However, in some cases, recurrent urinary tract infection or renal failure occurred, especially in bilateral cases. For further management, a long-term multicenter study is needed to investigate the precise incidence and outcome of each anomaly in the general population.

• Childhood Kidney Diseases
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• v.3 no.2
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• pp.180-186
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• 1999

Purpose : It has been well known that urinary tract infection(UTI) in infants and children is frequently associated with vesicoureteral reflux(VUR). However, the publishied papers dealing with congenital anomalies associated with UTI emphasized the importance of VUR only. The aim of our study was to evaluate the type, incidence and spectrum of urologic anomalies associated with UTI. Methods : Medical records of clinical, bacteriologic and radiologic study were assessed retrospectively in 65 infants or children with documented UTI who were admitted to the Department of Pediatrics, Samsung Seoul Hospital from March 1996 to February 1998. Results : Spectrum of anomalies were associated with UTI as follows: VUR(n=23), both ectopic kidney(n=1), ureterovesical junction(UVJ) obstruction(n=1), multicystic dysplastic kidney(n=1), ureteropelvic junction(UPJ) obstruction with hydronephrosis(n=1), hutch diverticulum(n=1), UPJ stenosis(n=1), posterior urethral valve(n=1), urachal remnant(n=1) and bladder diverticula(n=1). Congenital urinary anomalies other than VUR were detected in 9 children among 65 patients with UTI(13.8%). 4 children among 9 congenital urinary anomalies other than VUR were combined with VUR. Sex distribution with congenital urinary anomalies other than VUR was more prevalent in male than female (7 males : 2 females). Age distribution at the time of UTI was less than 5 years in most patient (under 1 year in 1 patient, 1-2 year in 5 patients, 3-5 year in 1 patient, and above 5 year in 2 patients). And age distribution at the time of UTl associated with VUR was less than 5 years in most patient (under 1 year in 6 patients, 1-2 year in 8 patients, 3-5 year in 5 patients, and above 5 year in 4 patients), too Conclusion : Because congenital urinary anomalies other than VUR are seen in as high as 13.8% of patients, more careful evaluation of all possible congenital urinary anomalies as well as VUR is mandatory in pediatric patient with UTI.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.227-236
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• 2002

Purpose : A study was done to assess the incidence and classification of congenital urinary tract anomalies detected by antenatal ultrasonogram. Methods : We reviewed 558 cases of urinary tract anomaly which were detected by antenatal ultrasonogram and postnatally confirmed between June 1989 and May 2002. We investigated the incidence and classified congenital urinary tract anomalies by review of medical records, antenatal and postnatal radiologic studies retrospectively. Results : In 558 cases of congenital urinary tract anomalies, 292 cases of hydronephrosis were found and the most common. Another anomalies were composed of 65 cases of multicystic dysplastic kidney, 32 cases of hydroureteronephrosis, 31 cases of duplication of kidney, 25 cases of renal agenesis, 21 cases of simple renal cyst, 20 cases of polycystic disease, 13 cases of ureterocele, 11 cases of renal hypoplasia, 10 cases of horseshoe kidney, 9 cases of vesicoureteral reflux, 8 cases of posterior urethral valve, 7 cases of bladder diverticulum, 6 casts of megaureter, 5 cases of ectopia, 2 cases of megacystis, and 1 case of medullary cystic disease. In 82 of the 558 cases, there were two or more combined urinary tract anomalies. Associated diseases other than urinary tract were observed in 13 cases, of which the congenital heart disease was the most common. Conclusion : The congenital urinary tract anomaly is frequently found and diverse during the antenatal ultrasonography. The multicenter study is needed to investigate precise incidence and distribution of each anomalies in general population.