• 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.

• Neonatal Medicine
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• v.18 no.2
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• pp.359-364
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• 2011

Purpose: Aplasia cutis congenita (ACC) is a rare condition characterized by a localized absence of skin, and it can be associated with other congenital anomalies. This study was done to evaluate clinical course and outcome of ACC in neonates. Methods: Based on the medical records, we retrospectively reviewed 8 neonates diagnosed with ACC at Cheil General Hospital and Women's Health Care Center, Kwandong university College of Medicine from January 2004 to December 2010. We classified ACC by Frieden's classification and analyzed the patient's demographic data, clinical course and outcome. Results: Among 8 patients with ACC, 5 patients were classified to group 1; scalp ACC without anomalies and 3 patients to group 7; ACC localized to extremities without blistering. Defect size was from 0.3 cm to 1.5 cm, limited in the superficial skin or subcutaneous tissue without associated anomalies. Defects were healed under conservative treatment with mild scar formation within four months. Conclusion: ACC could be diagnosed easily through physical examination in neonates. All cases showed good clinical outcome without surgical treatment. However because of small numbers and small sized defects of cases, further study including lesions of large size is needed.

• 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.

• The Journal of the Korean bone and joint tumor society
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• v.14 no.1
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• pp.56-61
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• 2008

More than half of the reported cases of congenital arteriovenous malformations involve the extremities. However, these are predominantly arteriovenous malformations of soft tissues. There are few studies on intraosseous arteriovenous malformations. The clinical features of vascular malformations are not well defined, but are likely to be extremely diverse. So, it is not easy to diagnose exactly and treat intraosseous arteriovenous malformations. The authors noted intraosseous arteriovenous malformation of tibia in a child and had a good result by the use of ethanol embolization. Therefore we include those results along with the literature review.

• Journal of Chest Surgery
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• v.29 no.2
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• pp.223-226
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• 1996

Pulmonary sequestration and congenital cystic adenomatoid malformation are two infrequent congenital pulmonary diseases and the combination of these two entities is rare. We had experienced a 3 week old male patient with a pulmonary mass, who had been sufyerring from tachypnea and chest wall retraction after birth. The pulmonary mass was suspected as a congenital cystic adenomat id malformation by chest CT. And therefore, we performed urgent operation via standard thoracotomy incision. Upon a thoracotomy, there was an extrapleural mass with anomalous blood supply near the posterior diaphragm and multiple cystic lesions in right lower lobe. The two anomalous arteries arising from the thoracic aorta and one vein draining into the azygos vein were ligated. and then the extrapleural mass was removed and a right lower lobectomy was performed. Final histologic diagnosis was congenital cystic adenomatoid malformation associated with extralobar pulmonary sequestration. The patient was discharged without any problem.

• Proceedings of the KTCVS Conference
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• 1995.10a
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• pp.67-67
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• 1995

• 대한예방의학회:학술대회논문집
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• 2002.10a
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• pp.307-308
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• 2002

• Journal of Chest Surgery
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• v.37 no.6
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• pp.536-538
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• 2004

There are still some controversies regarding one stage repair of pectus deformity with congenital heart disease due to probable complications after extensive resection of deformed cartilages. We performed Nuss operation with simul-taneous intracardiac repair in 8 year old patient with pectus excavatum and partial atrioventricular septal defect. The result of operation was satisfactory without prolongation of operation time, bleeding or instability of chest wall.

• Clinical and Experimental Pediatrics
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• v.51 no.6
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• pp.660-664
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• 2008

The term tracheal bronchus refers to an abnormal bronchus that comes directly off of the lateral wall of the trachea (above the carina) and supplies ventilation to the upper lobe. Tracheal bronchi occur almost exclusively on the right trachea and are associated with other congenital anomalies. In addition, tracheai bronchus may be related to other inflammatory conditions with persistent wheezing, such as recurrent pneumonia, chronic bronchitis and bronchiectasis, which is a result of the relatively poor local drainage of the involved bronchi. An infant with recurrent wheezing is likely to be a challenge for a clinician in the evaluation of the etiology of airway obstruction and in the differential diagnosis of wheezy breathing. The authors report a case of an 8-month-old female infant with a ventricular septal defect, who presented with stridor and recurrent respiratory infection and finally was finally diagnosed with a tracheal bronchus using computed tomography and a bronchoscopy. Therefore, tracheal bronchus should be included in the differential diagnosis of any child who presents with chronic or recurrent respiratory tract symptoms such as coughing, wheezing, stridor and recurrent respiratory infection, particularly in children with other congenital deformities.

• Korean Journal of Animal Reproduction
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• v.23 no.1
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• pp.63-68
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• 1999

A 4-year old Korean-native cow of third parturition delivered an deformed calf with congenital anasarca and hypoplasia of the extremity at Muan County in Chonnam Province. At parturition, the dam showed amniotic fluid approximately two times more than that at the normal and also occurred severe dystocia. The anomaly died before parturition was 82 cm long and weighed 25 kg. It was difficult to recognize the neck and face of the anomaly due to severe edema and the stretched tongue through mouth was found. The shape of the extremity was recognizable and the systemic hypoplasia of the body with marked edema was found. At necropsy, there was a large amount of serous fluid both in pleural and peritoneal cavity. Also the fluid was contained some of yellowish granular materials and diffusely was fulfilled in all subcutaneous tissue. There was a partial opening in diaphragm which was resulted in the protrusion of approximately half of liver toward peritoneal cavity. There were two kidneys (15${\times}$21 and 13${\times}$18 cm) on the left which were enlarged and relatively larger than the right one (13${\times}$9 cm). In addition, there were found slight to moderate hepatomegaly and splenomegaly with slight congestion, and diffuse edema of intestinal serosa. Lung (32${\times}$49 mm) with marked hypoplasia was smaller than the size of heart (56${\times}$45mm). Serological test to the dam showed relatively high antibody titer (＞64) to Akabane virus which might be involved in the outbreak of the deformed Korean-native calf as one of many causative agents.