• Journal of Chest Surgery
• /
• 제16권1호
• /
• pp.3-9
• /
• 1983

Congenital mitral stenosis is a rare cardiac lesion which frequently associated with other congenital anomalies of the heart and great vessels. There are many difficulties in its preoperative diagnosis and choice of adequate treatment. We present two cases of congenital mitral stenosis who have had operated in this hospital at March 1981 and January 1983. One was 13 years old female with isolated, type III mitral stenosis who had mitral valve replacement with Ionescu-Shiley bioprosthetic valve, the other was 2 years and 3 months old female with supramitral ring associated with VSD and PDA who had operation of resection of supramitral ring and repair of associated lesions. Postoperative course of both cases is excellent except one episode of sudden supraventricular tachyar-rhythmia on latter case and it was controlled by medical treatment.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
• /
• 제43권6호
• /
• pp.423-426
• /
• 2017

Congenital auricular deformities may be either deformational or malformational. Malformational anomalies present with a skin or cartilage shortage. Two cases with congenital cleft earlobe were presented. A new surgical technique using a two-layered repair with front and back flaps were introduced. She waited to begin wearing earrings until six months after the surgery to prevent possible scar contracture. The patients were followed up for a period ranging from 3 to 14 months. The earlobe volume deficiency was replaced, and acceptable scar maturation was obtained.

• 대한두개안면성형외과학회지
• /
• 제20권4호
• /
• pp.265-269
• /
• 2019

Congenital hypoplasia of the depressor anguli oris muscle is a rare cause of asymmetrical crying facies in newborns. The clinical manifestations range from mild to severe asymmetry and may persist up to adulthood. In the current case, the patient did not exhibit other congenital anomalies or paralysis of other branches of the facial nerve. This adult patient presented with severe asymmetrical lower lip deformity during full mouth opening since birth. A chromosomal study for the detection of 22q gene deletion yielded negative results. The electromyography findings of the lower lip were insignificant. Depressor labii inferioris muscle resection was not effective, but bidirectional (horizontal and vertical) fascia lata grafting improved the aesthetic appearance of the asymmetrical lower lip. The patient showed improved lower lip symmetry during full mouth opening at 1 year after the surgery. Therefore, the details of this rare case are reported herein.

• Journal of Chest Surgery
• /
• 제19권4호
• /
• pp.644-662
• /
• 1986

Seventy cases of open heart surgery were performed in the department of Thoracic and Cardiovascular Surgery, Pusan Paik Hospital, Inje College, from Oct. 1985 to Oct. 1986. And the results were summarized as follows. 1. Among the 70 cases, there were 48 cases of congenital heart anomalies and 22 cases of acquired rheumatic valvular heart diseases. Age range of the congenital patients was 7 months to 31 years with the mean age of 10 years, and the acquired patients was 18 to 62 years with the mean age of 40 years. 2. The heart-lung machine used for cardiopulmonary bypass was Sarns 7000, 5-head roller pump, and the number and type of oxygenators were 5 of membrane type and 65 of bubble type. For all cases GIK [glucose-insulin-potassium] solution was used as cardioplegic solution for myocardial protection during operation. 3. Among the 48 congenital anomalies, there were 12 cases of ASD group, 29 of VSD group, 3 of ECD, 3 of TOF and one of PDA + MR, and to all of which the appropriate radical operations were applied. 4. Among the 22 acquired valvular diseases, there were 11 cases of mitral valve diseases [MS; 4, MSr; 3, MRs; 4], 3 cases of aortic valve diseases [AR:1, ARs;1, ASr;1], 4 cases of double valve diseases [MRs+TR; 3, MRs+ARs; 1] and 4 cases of triple valve diseases [MSr+ASr+TR; 3, MSr+Ar+TR; 1]. To all the diseased mitral and aortic valves, artificial valve replacement was applied except one [As], in which valve plication was applied. And to all the diseased tricuspid valve, DeVega annuloplasty was applied. 5. The number of replaced artificial valves were 29 in 25 patients [congenital; 3, acquire; 22]. In MVR, 6 of mechanical valves [St. Jude Medical valve; 6] and 15 of tissue valves [Carpentier-Edward valve; 11, lonescu-Shiley valve; 4] were used. In AVR, 6 of mechanical valves [St. Jude Medical valve; 6] and 2 of tissue valves [Carpentier-Edward valve; 2] were used. 6. Postoperative complications were occurred in 12 cases. Among them 11 cases were recovered with intensive cares, but one patient [VSD + Fistula of Valsalva sinus] was expired with low cardiac out put syndrome.

• 대한치과교정학회지
• /
• 제23권3호
• /
• pp.319-326
• /
• 1993

The purpose of this study was to evaluate the frequency of congenital missing teeth and supernumerary teeth in cleft patients. The subjects were divided into bilateral cleft lip and palate(BCLP), unilateral cleft lip and palate(UCLP) and cleft palate alone(CP alone) groups. 97 cleft patients(BCLP 15, UCLP 70, CP alone 12) between 6-20 years old were evaluated. Panorama film, Orthodontic chart and initial intraoral photogram were employed for this research. The obtained results were as follows. 1. The incidence of congenital missing teeth in total cleft samples was $57.7\%$, and the incidence of supernumerary teeth was $26.8\%$. Each incidence was higher than non-cleft. 2. The incidence of congenital missing teeth was the highest in BCLP and the lowest in CP alone. 3. The number of congenital missing teeth per perso was usually one, and the frequency was higher in the maxillary lateral incisors$(67.8\%)$, and maxillary second premolar$(14.9\%)$ than other teeth. 4. Most of tooth number anomalies in cleft patients were found in maxilla, especially adjacent region to the cleft site.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• 제10권2호
• /
• pp.211-214
• /
• 2007

선천성 허리헤르니아는 12번 늑골과 장골 사이의 후복벽 요측부에 발생하는 탈장으로 매우 드문 질환이다. 출생 시부터 우측 측복부에서 덩이가 발견되었고 점차크기가 증가하여 내원한 4개월 된 남아에서 복부 초음파 검사 및 복부 전산화 단층 촬영으로 선천성 상부 허리헤르니아가 진단되었고 일차 봉합술로 완치되었기에 보고하는 바이다.

• Journal of Chest Surgery
• /
• 제11권4호
• /
• pp.523-528
• /
• 1978

Recent advances in the surgical treatment of congenital disorders of the heart have necessitated an accurate preoperative diagnosis. Right heart catheterization has become widely accepted as a research tool and diagnostic test to detect the heart diseases, especially in the congenital heart anomalies. Right heart catheterizations were carried out in 50 cases of congenital heart diseases at Department of Thoracic and Cardiovascular Surgery, , Kyungpook National University Hospital, during the period of June, 1975 through September 1978. In age distribution, 27cases were below 10 years of age, 18 cases between 11 and 20, and 5 cases above 20 male to female ratio was 2.8:1. The distribution of congenital heart diseases was VSD [42%], TOF [36%], PDA [10%], ASD [8%], and PS[4%]. Of these, 44 cases [88%], were compatible with the clinical impressions that were made preliminarily before cardiac catheterization, and all the cases except 1 case of VSD was correlated well with the postoperative diagnosis. The right heart catheterization is considered to be reliable and accurate toll in the preoperative diagnosis of congenital heart diseases. These procedures caused complications such as left side hemiplegia [lcase], occlusion of the femoral artery [lcase], and transient ventricular tachycardia [1case], and so the complication rate of right heart catheterization was 6% [3 cases]. None of patients who have undergone right heart catheterization was died.

• Journal of Chest Surgery
• /
• 제19권1호
• /
• pp.116-121
• /
• 1986

Patients over 15 years of age who have undergone a surgical correction of a congenital cardiac malformation during period of January 1958 through January 1986 have been reviewed. During this period there were 3957 congenital cardiac lesions consisting of 2712 acyanotic and 1245 cyanotic cases. Among them, a total of 725 adults [18.3%] with a variety of congenital heart lesions, 548 acyanotic group and 177 cyanotic group were operated on. 372 patients were male and 252 patients were female. There were 280 patients under 20 years of age, 206 between 20-24 years, 102 between 25-29 years, 48 between 30-34 and 89 over 35 years. The most common defects were atrial septal defect which accounted for 207 cases [28.6%] and other common anomalies were VSD [140 cases, 19.3%], TOF [136 cases, 18.6%], PDA [120 cases, 16.6%] and PS [33 cases, 4.6%] in order of incidence. Overall operative mortality for this series was 3.6% [1.8% of a cyanotic group and 9.0% of cyanotic group] compared with 2.8% of total cases of congenital heart disease [acyanotic group 1.1% and cyanotic group 6.5%]. This reviewed series reveals the incidence of operable congenital heart defects appearing in any adult life and demonstrates that surgical repair can be accomplished with a satisfactory low mortality rate.

• Journal of Yeungnam Medical Science
• /
• 제39권2호
• /
• pp.161-167
• /
• 2022

Congenital web formations are extremely rare anomalies of the extrahepatic biliary tree. We herein report a case of common bile duct septum combined with multiple intrahepatic bile duct strictures in a 74-year-old female patient who was successfully treated with radiological intervention. The patient initially visited the hospital because of upper abdominal pain. Imaging studies revealed multifocal strictures with dilatation in both intra- and extrahepatic ducts; the final clinical diagnosis was congenital common bile duct web combined with multiple intrahepatic duct strictures. Surgical treatment was not indicated because multiple biliary strictures were untreatable, and the disease was clinically diagnosed as benign. The multiple strictures were extensively dilated twice through bilateral percutaneous transhepatic biliary drainage (PTBD) for 2 months. After 1 month of observation, PTBD catheters were successfully removed. The patient is doing well at 6 months after completion of the radiological intervention, with the maintenance of normal liver function. Congenital web of the bile duct is very rare, and its treatment may vary depending on the patterns of biliary stenosis. In cases where surgical intervention is not indicated for congenital web and its associated disease, radiological intervention with balloon dilatation can be a viable therapeutic option.

• Childhood Kidney Diseases
• /
• 제22권1호
• /
• pp.12-16
• /
• 2018

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital malformation syndrome that is characterized by a triad of uterine didelphys, blind hemivagina, and ipsilateral renal agenesis. There is a wide variety of phenotypic presentation which is recognized as a spectrum of disease rather than a separate entity. The exact incidence and pathogenesis of HWW syndrome are yet to be investigated. While this disease typically involves adolescent girls who present with abdominal pain or a pelvic mass that is secondary to hematocolpos, nowadays, a majority of potential patients with HWW are being prenatally screened for renal anomalies. Therefore, it is recommended to search for uterovaginal anomalies whenever a multicystic dysplastic kidney or the absence of a kidney is noted in a newborn female, and the role of pediatric nephrologists has become ever more important for early recognition of the disease.