• Childhood Kidney Diseases
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• 제7권2호
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• pp.189-196
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• 2003

목적 : 중복 요관은 부검상 전인구의 0.9%에서 발견되는 비교적 흔한 비뇨기계 기형이다. 과거에는 발열, 복통, 혈뇨 등을 단서로 하여 발견되었으나, 최근에는 산전초음파에 의하여 조기 진단되고 있다. 저자들은 최근 5년간 본원에서 진단된 환아들의 임상 양상 및 산전초음파에 의한 조기 진단의 유용성에 대해 고찰하였다. 방법 : 1998년부터 2003년 7월까지 본원에 입원하여 진단된 18세 미만의 55례의 중복 요관 환아들을 대상으로 후향적 고찰을 시행하였다. 결과 : 진단 당시 평균 연령은 2.2세였고, 추적 기간의 평균은 14개월이었으며, 남녀비는 1:2.7이었다. 완전 중복 요관은 49례(89%)였다. 발열을 주소로 내원한 경우는 15례(27%)였고, 산전 진찰로 진단된 경우는 26례(47%)였다. 방광요관역류가 동반된 경우는 14례(28%)였고, 수신증은43례(88%)에서 동반되었다. 산전 진찰을 통해 진단된 군과 생후에 진단된 군의 비교시, DMSA 신주사상 결손의 비율은 각각 70.8%와 67.7%였으며, 환측 신장의 동위원소 섭취율은 각각 48.2%와 45.5%로 두 군간에 통계학적인 차이는 없었다. 결론 : 과거와 달리 최근 5년간 산전초음파로 중복 요관이 진단되는 비율은 47%로 증가하고 있으며, 이에 따라 진단시 연령이 낮아져(2.2세) 경과 추적이 조기에 시작될 수 있었다. 그 외의 임상적인 양상은 과거와 유사하였다. 산전초음파로 진단된 경우와 생후에 진단된 경우를 비교할 때, 두 군간에 신손상의 차이에 대한 통계적학적 유의성은 발견하지 못하였다. 산전 초음파가 조기 진단과 이에 따른 추적 관찰을 통해 치료시기를 적절히 결정하는 데에 도움이 될 것인지 알아보기 위하여 향후 광범위한 다기관 연구가 필요하다.

• Journal of Yeungnam Medical Science
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• 제38권3호
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• pp.240-244
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• 2021

Imperforate anus is an anomaly caused by a defect in the development of the hindgut during early pregnancy. It is a relatively common congenital malformation and is more common in males. Although there are cases of a solitary imperforate anus, the condition is more commonly found as a part of a wider spectrum of other congenital anomalies. Although urgent reconstructive anorectal surgery is not necessary, immediate evaluation is important and urgent decompressive surgery may be required. Moreover, as there are often other anomalies that can affect management, prenatal diagnosis can help in optimizing perinatal care and prepare parents through prenatal counseling. In the past, imperforate anus was diagnosed by prenatal ultrasonography based on indirect signs such as bowel dilatation or intraluminal calcified meconium. Currently, it is diagnosed by directly checking the perineum with prenatal ultrasonography. Despite advances in ultrasound technology, accurate prenatal diagnosis is impossible in most cases and imperforate anus is detected after birth. Here, we present two cases of imperforate anus in female fetuses that were not diagnosed prenatally.

• Clinical and Experimental Pediatrics
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• 제49권8호
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• pp.870-874
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• 2006

목 적 : 선천성 수신증은 산전 초음파검사로 발견되는 태아기형 중 가장 흔하며 초음파검사의 발달로 인해 발견되는 빈도가 늘어나고 있다. 본 연구는 산전 진단되는 선천성 수신증의 빈도 및 임상적 특징들을 알아보고 주요 원인인 신우요관 협착증의 생후 경과에 대해 조사하고자 하였다. 방 법 : 본 연구는 1994년 1월부터 2003년 6월말까지 전남대학교 병원 산부인과에서 출생한 총 환아 9,076명을 대상으로 산전 진단된 선천성 수신증의 빈도 및 생후 3일째 호전 빈도를 조사하였다. 또한 상기 기간 동안 소아과에서 산전 진단된 선천성 수신증으로 진료를 받았던 228명(284신단위)을 대상으로 수신증의 임상 특성을 알아보았다. 이들 중 신우 요관 이행부 폐색(222신단위)에 대하여서는 자연 회복군(121신단위)과 수술군(25신단위)에서 초기 수신증의 크기를 비교하였으며, 자연 회복군에서 시간에 따른 신우 크기의 자연 소실 빈도를 조사하였다. 결 과 : 상기 9년 동안 출생했던 환아 총 9,076명중 231명의 환아(2.54%, 293신단위)가 선천성 수신증으로 산전 진단되었으며, 이들 중 생후 3일경 시행한 초음파 검사에서 수신증이 자연호전되었던 경우는 19신단위(6.5%)였다. 상기 기간 동안 소아과진료를 받았던 산전 진단된 선천성 수신증 환아 228명은 일측성 172명(75.4%, 좌측 119명 및 우측 53명), 양측성 56명(24.6%)으로 총 284신단위였으며, 남아(71.9%)가 여아(28.1%)에 비해 많았고, 그 원인으로는 신우요관 이행부 폐색이 222신단위(78.1%)로 가장 흔하였다. 신우요관 이행부 폐색의 경우 자연 회복군(121신단위)에서 초기 신우 전후 직경이 $7.8{\pm}6.28mm$으로 수술군(25신단위)의 $26.8{\pm}12.14mm$보다 통계적으로 유의하게 작았으며, 자연 회복된 경우 1년 이내에 81%에서 자연 호전되었다. 결 론 : 선천성 수신증은 좌측신장이 우측에 비하여, 남아가 여아에 비하여 많았다. 원인으로는 신우요관 이행부 폐색이 가장 흔하며, 신우요관 이행부 폐색의 경우 생후 3일째 시행한 초음파검사상의 신우전후직경의 크기는 수신증의 예후를 알수 있는 주요한 예측인자가 되며 자연 회복의 경우 12개월 내 81%에서 자연 호전되는 것으로 보아 생후 1년 내에는 면밀한 추적관찰이 필요할 것으로 사료된다.

• Childhood Kidney Diseases
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• 제20권1호
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• pp.29-32
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• 2016

Purpose: Congenital anomalies of the kidneys and urinary tract (CAKUT) are the most common anomalies identified in newborns. This study aims to demonstrate the prevalence of CAKUT including hydronephrosis diagnosed by antenatal and postnatal ultrasound over a five-year period. Methods: The records of births between May $1^{st}$, 2009 and April $30^{th}$, 2014 at our hospital were collected. The number of infants who underwent renal ultrasound after birth for the detection of CAKUT was counted. The incidence of each type of CAKUT such as hydronephrosis, size abnormality, horseshoe kidney, and Multicystic dysplastic kidney (MCDK) was retrospectively evaluated for antenatal screening and postnatal follow-up examination. Results: During the study period, 33,276 infants were born and 521 neonates underwent postnatal renal ultrasound. 183 cases of CAKUT were detected prenatally and 140 postnatally using ultrasonographic examinations at the following time: (i) 3-7 days postnatally in 123 newborns (87.9%), (ii) during 1-3 months in 11 newborns (7.9%), and (iii) later than 3 months in 6 newborns (4.3%). Among diagnosed CAKUT, hydronephrosis was the most common anomaly with 113 newborns diagnosed prenatally and 46 postnatally. Duplex kidney was the second most common anomaly followed by horseshoe kidney, simple cysts in the kidney and so on. Conclusion: The detection of CAKUT is an important part of the prenatal ultrasound. This study analyzed the prevalence of CAKUT detected on prenatal screening and compared the results to those detected postnatally. Prenatal ultrasound screening fulfills the needs of postnatal examinations and therefore, both antenatal and postnatal sonographic investigations are of vital importance for diagnosis of renal and urinary tract anomalies.

• Advances in pediatric surgery
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• 제10권2호
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• pp.87-91
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• 2004

As prenatal ultrasonography becomes popular, the number of prenatal diagnosis of congenital surgical diseases is also increasing. To evaluate the impact of antenatal ultrasonography on outcome the mortality rate in neonatal surgical emergencies was studied. The authors retrospectively reviewed 281 patients (congenital diaphragmatic hernia: 44, tracheoesophageal fistula: 78, intestinal atresia: 98, omphalocele: 28 and gastroschisis: 33 who had been managed at Seoul National University Childrens Hospital, from January 1991 to December 2000. The patients were divided into two groups; group A (1991 to 1995; 139 patients) and group B (1996 to 2000; 142 patients). These two groups were subdivided into prenatally diagnosed subgroup and postnatally diagnosed subgroup. We analyzed the changes of prenatal diagnosis rate, total mortality rate, and mortality rate of subgroups. Prenatal diagnosis rate was increased significantly in group B (Group A: 24.5 % and Group B: 45.1 %). Total mortality rate of group A was 21.6 %, and that of group B was 10.6 %, showing a significant decrease in group B. However, in both group A and B, when compared antenatally diagnosed subgroup with postnatally diagnosed subgroup, the mortality rate was lower in postnatally diagnosed subgroups but statistically not significant. The authors conclude that although prenatal diagnosis rate has been increased, prenatal diagnosis itself has not resulted in significant improvement in outcome.

• Advances in pediatric surgery
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• 제7권1호
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• pp.68-72
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• 2001

Prenatally diagnosed neuroblastomas have been reported in increasing numbers over the past several years. The vast majority are in favorable stages of the disease (stage I, II, IV-S). The authors experienced one case of stage IV-S neuroblastoma of the adrenal gland with liver metastasis, which regressed spontaneously after removal by adrenalectomy. This patient was noticed to have an abdominal mass at prenatal ultrasonography performed at 36weeks of gestation. This tumor was a neuroblastoma of the left adrenal gland with multiple liver metastases. Left adrenalectomy and liver biopsy were performed at 3 months of age. Thirty-eight months after surgery, an MRI demonstrated that the hepatic metastatic lesions had completely regressed without chemotherapy or radiation.

• Advances in pediatric surgery
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• 제2권2호
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• pp.138-142
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• 1996

Pulmonary sequestration is a complex anomaly involving the pulmonary parenchymal tissue and its vasculature. It presents as a cystic mass of nonfunctional lung tissue without communication with the tracheobronchial system. Usually, it receives blood supply from anomalous systemic vessels. Therefore, preoperative diagnosis of the pulmonary sequestration is difficult, especially when it is located in the abdomen and combined with congenital cystic adenomatoid malformation(CCAM). We encountered such a mass(CCAM type 2) detected prenatally by ultrasonography. It was a kidney bean shaped, pinkish mass straddling the thorax and abdomen on the right side. Because of the sonographic appearance, neuroblastoma was diagnosed preoperatively. The mass was completely extirpated without difficulty.

• Advances in pediatric surgery
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• 제14권1호
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• pp.75-82
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• 2008

Neuroblastoma arises from the primitive neural crest cells, and is a common malignancy in childhood. The clinical features are characterized by biological heterogeneity. Neuronal degeneration and differentiation occur in some patients. However treatment in the high risk group accounting for approximately half, has not been satisfactory despite a multimodal approach. Therefore, effective treatment is determined by the risk group of prognostic factors, such as age at diagnosis, stage of disease, pathological finding and N-myc amplification. Neuroblastoma can be diagnosed prenatally, which suggests its origin during the normal embryogenesis. Recent knowledge of molecular biology, such as Trk genes, and the concept of cancer stem cells have given us some improved understanding on this disease. Currently, targeted therapies based on the molecular biology of neuroblastoma are under investigation and increasing survival rate and decreasing late complications could be appreciated.

• Childhood Kidney Diseases
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• 제22권1호
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• pp.28-31
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• 2018

Ureteropelvic junction obstruction is one of the common causes of hydronephrosis in infancy and childhood. Most cases of ureteropelvic junction obstruction are diagnosed prenatally and are usually asymptomatic. Although less common, older children can experience ureteropelvic junction obstruction that presents with symptoms including flank or abdominal pain. Here, we present the case of a nine-year-old healthy girl who had repeated flank pain and abdominal symptoms, with mild left hydronephrosis, for several months. Computed tomography that was performed during the period of acute flank pain revealed aggravated hydronephrosis on her left kidney, which was secondary to an ureteropelvic junction obstruction. She underwent laparoscopic pyeloplasty, and a crossing vessel that passed the ureteropelvic junction was identified. In addition, we reviewed the current literature of this rare entity.

• Clinical and Experimental Pediatrics
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• 제54권3호
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• pp.133-136
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• 2011

Fetus-in-fetu (FIF) is a rare congenital condition in which a fetiform mass is detected in the host abdomen and also in other sites such as the intracranium, thorax, head, and neck. This condition has been rarely reported in the literature. Herein, we report the case of a fetus presenting with abdominal cystic mass and ascites and prenatally diagnosed as meconium pseudocyst. Explorative laparotomy revealed an irregular fetiform mass in the retroperitoneum within a fluid-filled cyst. The mass contained intestinal tract, liver, pancreas, and finger. Fetal abdominal cystic mass has been identified in a broad spectrum of diseases. However, as in our case, FIF is often overlooked during differential diagnosis. FIF should also be differentiated from other conditions associated with fetal abdominal masses.