• Advances in pediatric surgery
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• 제12권1호
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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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• 제12권1호
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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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• 제3권1호
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• pp.88-94
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• 1999

목 적 : 요로 기형은 선천성 기형중 가장 높은 빈도를 차지하며 조기 진단에 어려움이 많아 발견당시 이미 비가역적인 신손상을 초래할 수 있다. 이에 본 연구는 소아에서 요로계의 선천성 기형의 임상상을 조사하고 이에 대한 적절한 진단과 치료 방법을 알아보고자 시행되었다. 방 법 : 조사 대상은 1987년부터 1998년까지 10년간 경북대학교병원 소아과에서 선천성 요로기형으로 진단된 65명의 환아로 하였고 후천적으로 발생한 수신증과 방광요관 역류 등은 제외시켰다. 이들 환아에 대한 임상상과 치료 및 경과를 임상 기록을 토대로 후향적 조사를 하였다. 결 과 : 요로계 기형의 빈도는 신우요관 이행부 협착이 26례로 가장 많았으며 그외 중복 요로계 11례, 일측성 신무형성 10례, 방광요관 이행부 협착 7례 등의 순이었다. 이중 8례에서 한가지 이상의 요로계 기형이 동반된 복합 기형을 나타내었으며 11례에서는 선천성 심장병과 같은 타 장기의 기형이 동반되어 있었다. 진단 당시의 연령 분포는 1세 이하가 39명($60\%$)으로 가장 많았고 남녀 비는 2.25:1로 남아가 많았다. 내원시 주 증상은 요로 감염이 25례로서 가장 많았고, 그외 혈뇨, 복부 종괴, 패혈증, 복통, 배뇨 이상 등이 있었다. 산전 초음파검사로 발견된 경우가 11례였고 입원시 고질소 혈증을 보인 경우는 모두 9례로서 이중 4례는 신우요관 이행부 협착, 방광요관 이행부 협착 및 중복 요로계등의 요로계 폐쇄질환이었다. 수술적 교정은 총 73례의 기형중 38례에서 시행되었다. 고질소 혈증을 보인 폐쇄성 요로 기형 4례중 1년에서 5년까지의 추적관찰 기간중 2례에서 고질소 혈증이 소실되었으며 같은 추적관찰 기간중 새로이 신기능 저하를 일으킨 례는 없었다. 결 론 : 요로의 선천성 기형은 높은 빈도로 발생함에도 불구하고 특이적인 증상을 나타내지 않아 조기 진단에 어려움이 많다. 그러므로 요로 감염, 복통, 배뇨 곤란 등의 증상이 있는 환아에서는 요로 기형의 존재 여부를 반드시 확인하여야 하며 특히 산전 초음파검사의 광범위한 이용으로 요로 기형의 존재 여부를 조기에 진단하고 이에 대한 적절한 조처를 행함으로서 신장의 비가역적 손상을 예방할 수 있을 것으로 생각한다.

• Archives of Plastic Surgery
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• 제46권4호
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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.

• 한국임상수의학회지
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• 제29권4호
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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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• 제18권2호
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• pp.116-122
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• 2014

신장 농양은 요로감염에 의한 신장 감염의 가장 심한 상태로 신주위로 파열되어 신장주위농양으로 진행할 수 있으며, 신장 흉터의 후유증을 남길 수 있다. 대개 항생제 치료가 지연되거나 적절한 항생제를 선택하지 못했을 때 발생할 수 있으며, 소아에서는 해부학적 요로계 기형을 동반하기도 한다. 저자들은 방광요관역류가 동반된 대장균에 의한 요로감염 환아에서 발열 초기에 민감한 항생제로 치료하였음에도 불구하고, 좌측 요근과 신피막 주위의 침윤을 동반한 신장 농양으로 진행하여 장기간의 항생제와 함께 경피적 흡인으로 치료된 1례와 해부학적 기형 없이 2주간 발열이 있었던 환아에서 포도알균에 의한 비장과 횡경막을 침범한 다격벽의 신장 농양이 확인되어 항생제 치료와 함께 도관삽입 후 배농하여 치료하였으나 이후 추적관찰에서 신장 흉터가 발생하였던 1례를 경험하였기에 이를 보고 하는 바이다.

• Perinatology
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• 제29권4호
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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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• 제19권2호
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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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• 제23권2호
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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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• 제40권6호
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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.