• Advances in pediatric surgery
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• v.12 no.1
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• pp.115-125
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• 2006

Postoperative management of anorectal malformation consists of colostomy management and then management after definitive surgery. Colostomy management requires attention to certain details, i. e. complete decompression of the distal limb to avoid secondary megarectosigmoid and prevention and treatment of urinary tract infections in patients with rectourethral fistula. Management after definitive surgery requires the care of catheters placed in the rectourethral fistula or cloacal defect. Prevention and treatment of various complications after definitive operation, i. e. wound infection, anal stenosis or stricture, anal mucosal prolapse, and management of functional disorders, i.e. constipation, fecal incontinence and urinary incontinence are also necessary. In this review, recent trends for the prevention and treatment of the above mentioned problems after operation for anorectal malformation are presented.

• Advances in pediatric surgery
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• v.12 no.1
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• pp.107-114
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• 2006

Major advances in the management of anorectal malformations have been achieved during the last 20 years. Alberto Pena introduced the posterior sagittal anorectoplasty (PSARP) in 1982. He divided all the sphincteric muscles at the exact posterior midline and fully exposed the crucial anatomy. He was able to manage the associated fistula under direct vision with minimal urinary tract injury. The rectum could be placed in the middle of the sphincteric muscle complex. Even with PSARP by Alberto Pena himself, only 37.5% of all cases were considered totally continent. Anorectal malformation is still acontinuing challenge for the pediatric surgeons.

• Childhood Kidney Diseases
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• v.3 no.1
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• pp.88-94
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• 1999

Purpose : Malformation of urinary tract is among the most common of all congenital anomalies and can progress to irreversible renal damage before diagnosis due to difficulty of early diagnosis. Present study was undertaken to determine the clinical characteristics of urinary tract anomaly and to find out the most appropriate diagnostic and therapeutic measures for children with these anomalies. Methods : During the past 10 years from 1987 to 1998, review of medical records revealed 65 children with congenital anomaly of urinary tract and the following results were obtained. Results : The most common anomalies were ureteropelvic junction obstruction occuring in 26 cases ($36\%$), followed by ureteral duplication in 11 cases, renal agenesis in 10 cases and ureterovesical function obstruction in 7 cases. Complex anomaly of urinary tract was found in 8 cases and anomaly of other systems such as congenital heart disease was detected in 11 cases. The most frequent age group at the time of diagnosis was below 1 year of age constituting 39 cases ($60\%$) and male preponderance was noted as male to female ratio being 2.25:1. Presenting symptoms were urinary tract infection in 25 cases, followed by hematuria, abdominal mass, abdominal pain and voiding difficulty, etc, and in 11 cases, the anomaly was picked up by routine prenatal ultrasonography. Azotemia was noted in 9 cases and the underlying anomaly was obstructive uropathy in 4 out of these 9 cases. Surgical correction was undertaken in 38 cases (most frequently in cases of obstructive uropathy) and in 2 out off cases with obstructive uropathy in whom surgical correction was done, azotemia disappeared during follow up period of 1-5years. No new cases of deteriorating renal function appeared during follow-up period. Conclusion : In spite of high incidence of congenital malformation of urinary tract, early diagnosis is still hampered by nonspecific symptoms and signs. Therefore, in patients with symptoms such as urinary tract infection, abdominal pain and voiding problems, etc, it Is advisable to take various diagnostic tests promptly to pick up any urinary tract anomaly and to apply proper therapy in order to avoid progression to irreversible renal damage. In this regard, prenatal ultrasonography should be utilized more widely as a routine procedure to detect any urinary tract anomalies before birth.

• Archives of Plastic Surgery
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• v.46 no.4
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• pp.381-385
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• 2019

Hypospadias is a congenital malformation of the male genitalia. The reconstructive objectives are to obtain voiding with laminar flow and satisfactory sexual function. Several urethroplasty techniques have been described, but for perineal or revisional cases no single technique has shown robust success. In this study, we describe the expanded use of intestinal flaps for urethral reconstruction and report a peculiar request from a patient to undergo peno-urethral separation after successful hypospadias repair with a free ileum flap. A 51-year-old male patient with perineal hypospadias underwent several urethral reconstructive procedures with poor outcomes. A free ileum flap was attempted as a substitute for the urethra. Following successful reconstruction, separation of the neo-urethra (ileum) from the penile body was performed to address the patient's sexual expectations. A free ileum flap proved to be a reliable urethral substitute in perineal hypospadias reconstruction, with a successful outcome. The peno-urethral separation with the creation of an "accessory penis," however peculiar, optimized the results in terms of both sexual and urinary function. Anatomical restoration of the urethra and patient-reported expectations are the key to successful hypospadias reconstructive procedures. Sexual function outcomes and the patient's perception of success should not be underestimated, even when urinary function has been restored.

• Journal of Veterinary Clinics
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• v.29 no.4
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• pp.352-355
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• 2012

Hypospadias is a rare congenital malformation of the urethra reported in cattle. The urethral lumen of male indigenous Korean calf is open along the ventral aspect of the penis in the perineal region. Renal abscess and renal stone formation causing urinary tact infection has not been reported in hypospadia calves. The objective of this study was investigation for renal abscess and renal stone formation through autopsy. Histopathological examination and laboratory tests were performed. At autopsy, the pustules were formed on the right renal cortex, and the renal medulla abscess were formed on right and left part of the renal pelvis. Histopathological finding, this case was diagnosed as severe acute suppurative and necrotizing pyelonephritis, and severe chronic interstitial nephritis with fibrosis and moderate multifocal acute cystitis with edema. Milky exudate of the kidney has been identified as Actinomyces meyeri using the VITEK-2 system for identification of bacteria, and the stone has been identified as carbonate apatite using FT-IR system for quantification analysis. This case report describe the hypospadias complicated with urinary tract infection due to carbonate apatite stones and Actinomyces meyeri.

• Childhood Kidney Diseases
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• v.18 no.2
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• pp.116-122
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• 2014

Renal and perinephric abscesses are a rare but potentially fatal complication of urinary tract infection (UTI). Diagnosing renal and perinephric abscesses has been known to be difficult. These abscesses may occur when the appropriate antibiotic treatment for a UTI is delayed, or in cases with a congenital malformation of the urinary system, especially in children. In the present report, we describe 2 cases of renal abscesses with extra-capsular invasion in children with febrile UTI. A 4-month-old male infant with vesicoureteral reflux developed a renal abscess that infiltrated the perinephric area and the left psoas muscle, despite early antibiotic treatment. A 9-year-old boy with prolonged fever also showed a multi-loculated renal abscess that infiltrated the spleen and diaphragm. Both patients were successfully treated with appropriate antibiotics and percutaneous drainage.

• Perinatology
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• v.29 no.4
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• pp.185-188
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• 2018

Jarcho-Levin syndrome is a congenital disorder characterized by several vertebral and costal anomalies. Other abnormalities have also been described, including neural tube defects, Arnold-Chiari malformation, renal/urinary tract abnormalities, hydrocephalus, hydroureteronephrosis, and meningomyelocele. We describe a spondylocostal dysplasia form of Jarcho-Levin syndrome that was prenatally diagnosed at 11 weeks of gestation and surviving. Although the patient had sporadic-type Jarcho-Levin syndrome, with normal karyotype and no family history of disease, the assessment of inheritance patterns and genetic counseling for the parents was important to inform them about the potential risks.

• Childhood Kidney Diseases
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• v.19 no.2
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• pp.154-158
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• 2015

Purpose: This study aimed to evaluate the status of renal function and the presence of urinary abnormalities in early adult patients with Turner syndrome (TS). Methods: Sixty-three girls with TS, who are attending pediatric endocrine clinics in Busan Paik Hosp., were studied. Urine and blood chemistry tests were performed in every visiting times. Renal ultrasonography was performed in all patients at the initial diagnosis, and intravenous pyelography, DMSA renal scan and renal CT were also performed, if necessary. Results: Of the 63 patients, the karyotype showed 45,X in 32 (50.8%), mosaicism in 22 (34.9%) and structural aberration in 9 (14.3%). The renal function at the latest visit was shown as normal in all patients. Nephrotic syndrome had developed in one patient. Hematuria was observed in seven patients. Renal anomalies were observed in 20 of the 63 TS (31.7%). Of the 32 TS patients with 45,X karyotype, 13 (40.6%) had renal anomalies, while these were found in 7 (22.6%) of 31 TS patients with mosaicism/structural aberration. But there was no significant statistical difference between two karyotype groups. Conclusion: Based on this study, most of the patients with TS do not have any significant problems related to renal function until early adulthood, regardless of renal malformation or hematuria.

• 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.

• Archives of Plastic Surgery
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• v.40 no.6
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• pp.766-772
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• 2013

Background Hypospadias is the most common congenital malformation of the male urinary tract, wherein the urethral opening is located proximal to the normal site. Tubularized incised-plate urethroplasty reconstruction, and its efficacy, remains controversial due to the high recurrence rate. This study aimed to evaluate the results of dartos fascia-reinforced flap surgery in hypospadias patients with previous operative complications. Methods Nine patients (mean age, 12 years) who had previous one- or two-stage repair with TIP urethroplasty and suffered from urethrocutaneous fistulas or fissures as complications, underwent dartos fascia-reinforced flap surgery in our clinic between January 2010 and December 2012. The mean postoperative follow-up period was 6 months. Results Among the 9 patients, 7 had complete correction. In all the patients, the maximum uroflow angle was > $45^{\circ}$. No patient complained of an unnatural urinating position. The patients and parents gave very high satisfaction scores (4.7 points) with regard to the appearance of the outer genitalia. Conclusion The dartos fascia-reinforced flap could be a useful and reliable option for complications of hypospadias repair or fissure with the prepuce preserved, given that histological aspects vary and that the possibility of recurrence in the glanular region is high.