• Childhood Kidney Diseases
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• v.9 no.1
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• pp.69-75
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• 2005

Purpose : The most important management of congenital hydronephrosis consists of the early diagnosis and evaluation of the pathologic abnormalities of congenital hydronephrosis. This study was conducted to investigate the different causes of hydronephrosis and its clinical outcome. Methods : 54 live neonates who were hospitalized and diagnosed with congenital hydronephrosis at Chungnam National University Hospital from Aug. 1998 to Aug. 2003 were retrospectively analyzed. Results : Hydronephrosis(renal pelvic AP diameter $gt;5 mm) was postnatally detected in 54 cases(2.1%) among 2,539 neonates who were hospitalized from Aug. 1998 to Aug. 2003. There were three times more males than females. Additional imaging studies revealed that ureteropelvic junction obstruction was the most common postnatal diagnosis(48.7%), followed by multicystic dysplastic kidney, vesicoureteral reflux and duplication kidney with ureterocele. Spontaneous regression of hydronephrosis was revealed in 25 renal units(75.8% ) of mild hydronephrosis, 14 renal units of moderate hydronephrosis and 1 renal unit of severe hydronephrosis. Operative correction were carried out in 14 renal units(70%) of severe hydronephrosls. Conclusion : The most common established cause of congenital hydronephrosis in this study was ureteropelvic junction obstruction. There are many cases of spontaneous regression in mild to moderate congenital hydronephrosis. Urinary tract infections occur in many neonates with hydronephrosis. Therefore, early detection and evaluation of congenital hydronephrosis and continuous follow-up at regular intervals are necessary for conservation of renal function. (J Korean Soc Pediatr Nephrol 2005;9:69-75)

• Childhood Kidney Diseases
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• v.23 no.2
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• pp.116-120
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• 2019

Bilateral renal obstruction is a rare critical condition, requiring a prompt diagnostic approach and treatment to restore the renal function. The most commonly observed obstructive uropathy in children is congenital malformation, such as posterior urethral valves and bilateral ureteropelvic junction obstruction. Malignant pelvic masses obstructing the ureter are widely reported in adults but are rarely observed in children. The treatment of ureteral obstruction related to pelvic malignancy is a therapeutic challenge with a median survival duration of 3-7 months in adults; however, pediatric patients with pelvic malignancy leading to ureteral obstruction had better outcomes, with a reported 5-year mortality rate of 20%, than the adult patients. Here, we report a rare case of bilateral ureteral obstruction associated with pelvic rhabdomyosarcoma presenting with acute kidney injury treated by ureteral diversion with double J stent, and concommittent emergency hemodialysis, leading to restoration of good renal function. We suggest that bilateral ureteral obstruction should be released as soon as possible using surgical or interventional approach to minimize the obstruction period, and subsequential chemotherapy may contribute to improvement of survival and recovery of renal function.

• The Korean Journal of Nuclear Medicine
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• v.33 no.1
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• pp.57-64
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• 1999

Purpose: The purpose of this study was to evaluate the accuracy of Tc-99m DTPA diuretic renal scans in children with dilated upper urinary tract. Materials and Methods: We reviewed diuretic renal scans of 14 pediatric patients (age range: 3 days to 4 years) with unilateral hydronephrosis diagnosed by ultrasonography. Diuretic renal scan was done using Tc-99m DTPA and standardized protocol. In 3 neonates, diuretic renal scans were performed within 1 week and 3-7 months after birth. Results: Six patients required Pyeloplasty and eight were managed conservatively. All 6 patients requiring Pyeloplasty were diagnosed as having ureteropelvic junction obstruction in the diuretic renal scan. In these 6 patients, post-operative renal scans at 3-12 months after surgery were converted to nonobstructive pattern in 5 and a nonfunctioning pattern in 1. In 3 patients who underwent diuretic renal scan within 1 week after birth, nonobstructive patterns of initial scan were converted to obstructive patterns in the follow-up scan. However, all patients with nonobstructive diuretic renal scans performed after the neonatal period did well on serial ultrasonography and showed favorable clinical outcome without progression to obstruction. Conclusion: Tc-99m DTPA diuretic renal scan with standardized protocol is useful in assessing suspected ureteropelvic junction obstruction in children as an initial diagnostic or post-operative follow-up modality. Nonobstructive or indeterminate scan results in the neonatal period requires follow-up scan to monitor development of the obstructive pattern.

• Childhood Kidney Diseases
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• v.21 no.2
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• pp.147-151
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• 2017

Giant hydronephrosis (GH) is a rare urological entity and usually presents with more than a liter of fluid in the collecting system. It may mimic a progressive and benign abdominal cystic tumor. We report a case of GH in an 11-year-old obese boy who presented with abdominal distension and dyspnea on exercise. Hydronephrosis was caused by ureteropelvic junction obstruction, with 2,300 mL of fluid in the collecting system. Diagnostic and therapeutic features of this case are discussed, with reference to current literature.

• Clinical and Experimental Pediatrics
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• v.55 no.3
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• pp.107-110
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• 2012

Partial trisomy 3p results from either unbalanced translocation or $de$ $novo$ duplication. Common clinical features consist of dysmorphic facial features, congenital heart defects, psychomotor and mental retardation, abnormal muscle tone, and hypoplastic genitalia. In this paper, we report a case of partial trisomy 3p with rare clinical manifestations. A full-term, female newborn was transferred to our clinic. She had cleft lip-plate, dysgenesis of the corpus callosum, patent ductus arteriosus, pulmonary hypertension, and severe right-sided hydronephrosis, associated with ureteropelvic junction obstruction. Cytogenetic investigation revealed partial trisomy 3p; 46,XX,der(4)t(3;4)(p21.1;p16). The karyotype of her father showed a balanced translocation, t(3;4)(p21.1;p16). Therefore, the size of duplication can be an important factor.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.178-187
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• 2002

Purpose : The detection of hydronephrosis(HN) with antenatal ultrasonography was first reported in the 1970s. Prenatal HN is diagnosed with an incidence of 1:100 to 1:500 on antenatal screening. Recently, the purpose of antenatal screening has changed from simple detection to selection for specific diagnosis-based management. this study is to evaluate the usefulness of antenatal sonography for HN and to investigate the differential causes of HN and their clinical outcomes. Patients and methods : 11,783 live neonates with prenatal ultrasonographic examination at Ajou University School of Medicine, from Sep. 1994 to Aug. 2001 were analyzed. Results and conclusion : Hydronephrosis (>10 mm) was detected in 119 (1.0%) cases antenatally and among these, 91 were proved to have HN postnatally Males were three times more affected than females. Additional imaging studies revealed that ureteropelvic junction obstruction was the most common postnatal diagnosis (47%), followed by multicystic dysplastic kidney, vesicoureteral junction obstruction and vesicoureteral reflux. During 20 months' follow-up(3 to 72 months), 58(48%) renal units showed spontaneous resolution and surgical interventions were necessary in 10 (7.4%) of postnatally confirmed hydronephrotic renal units.

• Childhood Kidney Diseases
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• v.12 no.2
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• pp.262-266
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• 2008

Crossed renal ectopia is a congenital malformation in which both kidneys lie on the same side of the spine, usually side by side longitudinally. More often on the right side. Fusion of the two renal units is eight times more common than nonfusion. Although crossed renal ectopia is uncommon, this unusual entity must be considered in an infant when cystic mass in the abdomen or pelvis paticularly if no kidney can be found on the opposite side. In many cases of crossed fused ectopia with multicystic dysplastic kidney(MCDK), the diagnosis can be strongly suspected from the sonogram, and no other studies may be necessary. However, both intravenous urography and isotope renography is useful to assess the function of the crossed kidney. Crossed renal ectopia and MCDKs are associated with a greater incidence of ureteropelvic junction obstruction and reflux. So, screening voiding cystourethrography should be performed. Very few studies of MCDK in the setting of crossed fused ectopia have been reported. We have experienced a 3-year-old boy with crossed fused renal ectopia with multicystic dysplasia.

• Clinical and Experimental Pediatrics
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• v.45 no.10
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• pp.1213-1218
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• 2002

Purpose : Hydronephrosis constitutes a great portion of fetal anomalies screened by prenatal sonogram. The present authors made an attempt to access its natural courses through follow up neonatal hydronephrosis diagnosed by prenatal sonogram. Methods : The study was composed of 23 neonates(36 renal units) who were diagnosed with hydronephrosis through prenatal sonogram screening and confirmed 3-7 days after birth with sonographic evaluation at Kyung Hee University Hospital. The neonates were closely monitored for 12-24 months with renal sonogram, diuretic renogram, intravenous pyelography(IVP) and voiding cystoureterography(VCUG). Results : The underlying diseases were composed of 16 cases(44%) of functional abnormalities, 14 cases(39%) of ureteropelvic junction(UPJ) obstruction, three cases(8%) of vesicoureteral reflux (VUR) and on case each of multicystic dysplastic kidney, UPJ obstruction with ureteral stenosis and ureterovesical junction(UVJ) obstruction with VUR. The degree of hydronephrosis was divided into three classes according to its severity. In 30 renal units with UPJ obstruction and functional abnormalities, 26(87%) showed mild hydronephrosis, while four(13%) were moderate. During the follow up period, six cases(20%) showed natural resolution of hydronephrosis, 15 cases(50%) showed improvement while two cases(7%) were aggravated with improvement only after surgery of the underlying disease. The cases which showed natural resolution were all mild hydronephrosis at diagnosis and the cases which underwent surgery due to continuous aggravation were mild one case and moderate one case. Conclusion : Those with cases of mild hydronephrosis show rapid natural improvement. On the other hand, in some cases, follow up monitoring reveal aggravation of the situation, emphasizing the necessity for thorough follow up for a long period of time.

• Childhood Kidney Diseases
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• v.3 no.2
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• pp.203-208
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• 1999

Purpose: The information on the change of the relative renal function after operation is essential to set the initial treatment plan in unilateral hydronephrosis. So we analyzed the preoperative and postoperative relative renal function, and observed the functional changes by operation and reliability of the various factors in those changes. Materials and Methods: A retrospective trial was done of 81 patients, 35 ureteropelvic junction(UPJ) obstruction and 46 vesicoureteral reflux(VUR), among 393 patients undergoing operations at our institution from March. 1992 to February. 1997. The patients who had infravesical abnormalities, abnormal contralateral kidney and insufficient research data were excluded. We determined the relative renal functions using DTPA and MAG3 scan in UPJ obstruction and DMSA scan in VUR. The mean observation period after operation was 2.01(0.25-4) years. Results: A. UPJ Obstruction 1. The relative renal function was improved significantly after operation(P=0.0007). 2. The kidneys which have preoperative functions between 20% and 40% improved significantly in relative renal function comparing to the kidneys of the other functions(P=0.0046). B. VUR 1. The renal functions didn't improve significantly after operation(P>0.05). 2. There was no significant factor affecting the prognosis in renal functions. Conclusions: 1. The kidneys with UPJ Obstruction show the difference in functional improvement after operation according to the degree of the preoperative renal function, but more investigations about the exact value are needed. 2. In VUR there was no difference between preoperative and postoperative functions, the theoretical basis on objectives of the operation could be the symptoms or factors except the operation.

• 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.