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JYMS : Journal of Yeungnam Medical Science

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15 "Congenital"
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Original articles
Outcomes after repair of complete atrioventricular canal with a modified single-patch technique: a retrospective study
George Samanidis, Konstantinos Kostopanagiotou, Meletios Kanakis, Georgios Kourelis, Kyriaki Kolovou, Georgios Vagenakis, Dimitrios Bobos, Nicholas Giannopoulos
J Yeungnam Med Sci. 2023;40(2):187-192.   Published online February 1, 2023
DOI: https://doi.org/10.12701/jyms.2022.00759
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AbstractAbstract PDF
Background
This study aimed to present the short- and midterm outcomes after complete atrioventricular canal defect (CAVC) repair using a single-patch technique.
Methods
This study included 30 children who underwent surgical correction of the CAVC using a single-patch technique.
Results
The median age of the patients was 5.7 months (interquartile range [IQR], 5.0–7.5 months), and 23 patients (76.7%) had type A CAVC. Fourteen patients (46.7%) were female and 17 (56.7%) had been diagnosed with Down syndrome. The in-hospital mortality rate was 0%. No deaths were observed during a median follow-up of 4 years (IQR, 3.5–5.0 years). Patients without Down syndrome were associated with late moderate mitral regurgitation (MR) (p=0.02). Late MR less than moderate degree was observed in 96.6%, 78.5%, and 50% of patients after 2, 4, and 5 years of follow-up, respectively, while late tricuspid valve regurgitation less than moderate degree was observed in 96.7%, 85.9%, and 59.0% of patients after 2, 4, and 6 years of follow-up, respectively. After a median follow-up of 4 years, only one patient had required surgical repair of a left ventricular outflow tract obstruction, which occurred 26 months after the first operation. Multivariable logistic regression analysis adjusted for the type of CAVC, sex, Down syndrome, age, and weight revealed that the absence of Down syndrome was a risk factor for late moderate MR (MR-2) (odds ratio, 0.05; 95% confidence interval, 0.006–0.50; p=0.01).
Conclusion
A single-patch technique for CAVC surgical repair is a safe method with acceptable short- and midterm results.
Rates and subsequent clinical course of fetal congenital anomalies detected by prenatal targeted ultrasonography of 137 cases over 5 years in a single institute: a retrospective observational study
Haewon Choi, Hyo-Shin Kim, Joon Sakong
J Yeungnam Med Sci. 2023;40(3):268-275.   Published online November 2, 2022
DOI: https://doi.org/10.12701/jyms.2022.00514
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  • 67 Download
AbstractAbstract PDF
Background
With the establishment of international guidelines and changes in insurance policies in Korea, the role of targeted ultrasonography has increased. This study aimed to identify the rates and clinical course of anomalies detected using prenatal targeted ultrasonography.
Methods
This study was a retrospective analysis of all pregnancies with targeted ultrasonography performed in a single secondary medical center over 5 years.
Results
Fetal anomalies were detected by targeted ultrasonography in 137 of the 8,147 cases (1.7%). The rates of anomalies were significantly higher in female fetuses (2.0% vs. 1.3%). In cases of female fetuses, the rate of anomalies was significantly higher in the advanced maternal age group (2.4% vs. 1.2%). In cases of male fetuses, the rate of anomalies was significantly higher in nulliparous (2.4% vs. 1.5%) and twin (5.7% vs. 1.9%) pregnancies. Pulmonary anomalies were significantly more common in the multiparity group (17.6% vs. 5.8%). Among the 137 cases, 17.5% terminated the pregnancy, 16.8% were diagnosed as normal after birth, and 42.3% were diagnosed with anomalies after birth or required follow-up.
Conclusion
Through the first study on the rates and clinical course of anomalies detected by targeted ultrasonography at a single secondary center in Korea, we found that artificial abortions were performed at a high rate, even for relatively mild anomalies or anomalies with good prognosis. We suggest the necessity of a nationwide study to establish clinical guidelines based on actual incidences or prognoses.
Incidence of congenital hypothyroidism by gestational age: a retrospective observational study
Ha Young Jo, Eun Hye Yang, Young Mi Kim, Soo-Han Choi, Kyung Hee Park, Hye Won Yoo, Su Jeong Park, Min Jung Kwak
J Yeungnam Med Sci. 2023;40(1):30-36.   Published online April 12, 2022
DOI: https://doi.org/10.12701/jyms.2022.00059
  • 3,568 View
  • 151 Download
  • 2 Web of Science
  • 2 Crossref
AbstractAbstract PDF
Background
Congenital hypothyroidism (CH) is the leading cause of preventable physical and intellectual disabilities. This study aimed to assess the incidence and clinical characteristics of CH in newborns.
Methods
We retrospectively reviewed the medical records of all newborns delivered at the Pusan National University Hospital between January 2011 and March 2021. The incidence of CH was compared according to gestational age, birth weight, and small for gestational age (SGA). The patients aged ≥3 years who could not maintain normal thyroid function and required levothyroxine treatment were diagnosed with permanent CH. Logistic regression analysis was performed to compare CH risks.
Results
Of 3,722 newborns, 40 were diagnosed with CH (1.07%). Gestational age and birth weight were significantly associated with CH incidence. The odds ratios (ORs) of CH in infants delivered at 32–37, 28–31, and <28 weeks were 2.568 (95% confidence interval [CI], 1.141–5.778), 5.917 (95% CI, 2.264–15.464), and 7.441 (95% CI, 2.617–21.159) times higher, respectively, than those delivered at term. The ORs of CH in infants weighing 1,500–2,499 g, 1,000–1,499 g, and <1,000 g were 4.664 (95% CI, 1.928–11.279), 11.076 (95% CI, 4.089–29.999), and 12.544 (95% CI, 4.350–36.176) times greater, respectively, than those in infants weighing ≥2,500 g. The OR of CH was 6.795 (95% CI, 3.553–13.692) times greater in SGA than in non-SGA infants.
Conclusion
The CH incidence in South Korea has increased significantly compared with that in the past. Gestational age, birth weight, and SGA were significantly associated with CH incidence.

Citations

Citations to this article as recorded by  
  • History of Neonatal Screening of Congenital Hypothyroidism in Portugal
    Maria José Costeira, Patrício Costa, Susana Roque, Ivone Carvalho, Laura Vilarinho, Joana Almeida Palha
    International Journal of Neonatal Screening.2024; 10(1): 16.     CrossRef
  • The prevalence of hypothyroxinemia in premature newborns
    Renata Stawerska, Marzena Nowak-Bednarek, Tomasz Talar, Marzena Kolasa-Kicińska, Anna Łupińska, Maciej Hilczer, Ewa Gulczyńska, Andrzej Lewiński
    Frontiers in Endocrinology.2022;[Epub]     CrossRef
Case report
Congenital web of the common bile duct combined with multiple intrahepatic duct stricture: a case report of successful radiological intervention
Hanseul Lim, Shin Hwang, Gi-Young Ko, Hyejin Han
J Yeungnam Med Sci. 2022;39(2):161-167.   Published online July 8, 2021
DOI: https://doi.org/10.12701/yujm.2021.01179
  • 4,993 View
  • 59 Download
AbstractAbstract PDF
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.
Case Reports
Spontaneous migration of a congenital intratympanic membrane cholesteatoma
Tae Hoon Kim, Kyu-Yup Lee, Da Jung Jung
Yeungnam Univ J Med. 2018;35(2):244-247.   Published online December 31, 2018
DOI: https://doi.org/10.12701/yujm.2018.35.2.244
  • 4,674 View
  • 65 Download
  • 2 Crossref
AbstractAbstract PDF
Congenital intratympanic membrane cholesteatoma (ITMC) is a rare type of congenital cholesteatoma located within the tympanic membrane. This lesion tends to increase in size over time. The development of ITMC can cause several complications such as hearing impairment, dizziness, facial palsy, and intracranial complications, similar to any other cholesteatoma. The treatment of congenital cholesteatoma requires the removal of the lesion through surgery, because disease progression induces bony destruction of the nearby tissue. Most patients presenting with this cholesteatoma type are also treated with primary surgical removal. However, we recently experienced a case of an ITMC that showed a natural transition to an external auditory canal cholesteatoma.

Citations

Citations to this article as recorded by  
  • Intratympanic membrane cholesteatoma after traumatic tympanic membrane perforation: a case report
    Junhui Jeong, Hyun Seung Choi
    Journal of Medical Case Reports.2023;[Epub]     CrossRef
  • A Case of Intratympanic Membrane Cholesteatoma Moving to the External Auditory Canal
    Shinya Hirahara, Yutaka Hanamure, Minoru Takaki, Mizuo Umakoshi, Tamon Hayashi
    Practica Oto-Rhino-Laryngologica.2021; 114(10): 759.     CrossRef
An adult asymptomatic pulmonary artery sling.
Han Hee Chung, Ju Yeol Baek, Won Yik Lee, Ji Hye Jang, Min Young Jeong, Gi Hyeon Woo, Seong Il Park, Il Kyu Kim
Yeungnam Univ J Med. 2014;31(2):109-112.   Published online December 31, 2014
DOI: https://doi.org/10.12701/yujm.2014.31.2.109
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AbstractAbstract PDF
A pulmonary artery sling is a very rare congenital abnormality in which the left pulmonary artery rises from the posterior surface of the right pulmonary artery and then passes between the trachea and the esophagus, causing tracheal compression. It is associated with tracheo-bronchial abnormalities (50%) and cardiovascular abnormalities (30%). It may produce respiratory symptoms through the airway compression of the abnormal left pulmonary artery and congenital abnormalities associated with it. Because most (90%) pulmonary artery sling patients present symptoms during infancy, their condition is often diagnosed in the first year of life. However, a pulmonary artery sling is occasionally found in adults. It is usually asymptomatic and found incidentally. This is a very rare case of an asymptomatic pulmonary artery sling in an adult. A 38-year-old man presented symptoms of mild exertional dyspnea. His spiral computed tomography showed a pulmonary artery sling. He was discharged without specific treatment because his symptoms improved without specific treatment and might not have been associated with a pulmonary artery sling. We report an adult case of an asymptomatic pulmonary artery sling diagnosed via spiral computed tomography, accompanied by a literature review.
A Premature Newborn with Congenital Syphilis.
In Ok Hwang, Eun Sil Lee
Yeungnam Univ J Med. 2007;24(2):333-338.   Published online December 31, 2007
DOI: https://doi.org/10.12701/yujm.2007.24.2.333
  • 1,519 View
  • 8 Download
AbstractAbstract PDF
A preterm newborn affected by congenital syphilis, born to mother not treated during pregnancy is described. The clinical picture was characterized by respiratory distress, cutaneous manifestations, massive hepatosplenomegaly, severe anemia, thrombocytopenia, disseminated intravascular coagulation syndrome and hypoalbuminemia. The patient was treated with daily injections of 190,500 units of crystalline penicillin G for 14 days. Premature infants with these symptoms and signs should be evaluated for congenital syphilis.
A Case of Vascular Anomaly in Swine: Infrahepatic Caudal/Inferior Vena Cava Interruption with Azygos/Hemiazygos Continuation
Won Kyu Park, Kil Ho Cho
Yeungnam Univ J Med. 2007;24(2 Suppl):S719-724.   Published online December 31, 2007
DOI: https://doi.org/10.12701/yujm.2007.24.2S.S719
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AbstractAbstract PDF
Absence of caudal/inferior vena cava (CVC/IVC) with azygos/hemiazygos continuation is an uncommon vascular anomaly. To the best of the investigators knowledge, this is the first report of absence of CVC/IVC with azygos/hemiazygos continuation in the swine in the world. In this case, absence of CVC/IVC was confirmed by venography and necropsy. The recognition of this congenital venous anomaly (CVC/IVC interruption with azygos/hemiazygos continuation) is important for interventional radiologist and cardiologist.
Two Cases of Congenital Vaginal Absence
Sung Chul Park, Hyun Jin Shin, Hyo Hyun Kim, Tae Hyung Lee, Sung Ho Lee, Doo Jin Lee
Yeungnam Univ J Med. 2007;24(2 Suppl):S710-718.   Published online December 31, 2007
DOI: https://doi.org/10.12701/yujm.2007.24.2S.S710
  • 1,187 View
  • 1 Download
AbstractAbstract PDF
Congenital absence of vagina(Mayer-Rokitansky-Küster-Hauser syndrome) is found between 1 in 4,000 and 20,000 female births, and is frequently associated with various urinary tract and skeletal anomalies. As the patients have normal ovarian functions, they appear as normal female phenotype and have normal female karyotype 46, XX. Inability of coitus is one of the main problems they have, so the goal of treatment is to create a vagina adequate to allow sexual activity. Both surgical and nonsurgical approaches have been utilized. The authors experienced two cases of congenital absence of vagina successfully treated by surgical and nonsurgical method.
Original Articles
Myocardial Protective Effect of Histidine-Tryptophan-Ketoglutarate Solution and Cold Blood Cardioplegic Solution in Pediatric Cardiac Surgery.
Tae Eun Jung, Dong Hyup Lee, Sung Sae Han
Yeungnam Univ J Med. 2006;23(1):19-25.   Published online June 30, 2006
DOI: https://doi.org/10.12701/yujm.2006.23.1.19
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AbstractAbstract PDF
BACKGROUND
There is limited data on comparisons between the effect of histidine-tryptophan-ketoglutarate (HTK) solution and cold blood cardioplegic (CBC) solution in pediatric cardiac surgery. The purpose of this study was to compare the myocardial protective effect of HTK solution and CBC solution in patients undergoing pediatric cardiac surgery. MATERILAS AND METHODS: We selected 49 patients with ventricular septal defect and atrial septal defect. HTK solution was used in 21 patients and CBC solution was used in 28 patents. HTK solution was given as a single dose, whereas CBC solution was used in the usual multi-dose method. The incidence of EKG change and concentration of Troponin T and CK-MB were compared for the evaluation of myocardial damage. RESULTS: There were no significant differences in the incidence of ST, T segment change by EKG and serial cardiac enzyme levels between two groups. CONCLUSION: These results suggested that the myocardial protective effect of HTK solution was similar to CBC solution in simple pediatric cardiac surgery.
Statistical analysis of patients referred to pediatric cardiologic clinic for diagosis of heart disease.
Kwang Hae Choi, Young Hwan Lee
Yeungnam Univ J Med. 2000;17(1):49-54.   Published online June 30, 2000
DOI: https://doi.org/10.12701/yujm.2000.17.1.49
  • 1,728 View
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  • 1 Crossref
AbstractAbstract PDF
BACKGROUND
Echocardiography is rapidly established itself as the primary diagnostic technique for investigation of children with heart disease, and referrals are increasing to the pediatric cardiology clinic for investigation. However, because there is a lack of analysis data on the patients referred to pediatric cardiology clinics, we have proceeded to compare and analyze their characteristics to provide basic data base. METHODS: From Oct. 1, 1998 to Jul. 10, 1999, total 443 cases referred to the pediatric cardiology clinic of Yeungnam University Hospital were studied retrospectively by medical records, chest X-ray, EKG and echocardiography, etc. RESULTS: The results were as follows. 1. The proportion of male was 61.0%(261 cases) and that of female was 39.0%(167 cases). The ratio of male to female was 1.6:1. The proportion infants less than 1 year-old was 62.6%(268 cases) of all patients. 2. Cardiac murmur was present in 248 cases(57.9%), which was the most common case of referral to the pediatric cardiology clinic. The impression at referral was more congenital heart disease(70.6%) than acquired heart disease(17.8%) and arrhythmia(11.6%). 3. The final diagnosis was as follows : congenital heart disease was present in 212 cases(49.5%), acquired heart disease, 59 cases(13.9%); arrhythmia, 13 cases(3.0%); normal heart, 144 cases(33.6%). CONCLUSION: Among the patients referred to pediatric cardiology clinic, 33.6%(144 cases) had normal hearts and why these patient were referred may be possibly due to more dependence on echocardiography than on auscultation instruction. Threfore, clinical and auscultatory skill should be emphasized to minimize dependence on expensive echocardiography for evaluation of pediatric heart disease.

Citations

Citations to this article as recorded by  
  • Evaluation and diagnostic approach for heart murmurs in children
    Hee Joung Choi
    Journal of the Korean Medical Association.2020; 63(7): 398.     CrossRef
Clinical Study of Congenital Duodenal Obstruction.
Young Soo Huh, Myeung Kook Lim, Sung Kyu Park
Yeungnam Univ J Med. 1998;15(1):67-74.   Published online June 30, 1998
DOI: https://doi.org/10.12701/yujm.1998.15.1.67
  • 1,778 View
  • 1 Download
AbstractAbstract PDF
Successful management of duodenal obstruction in newborn infant implies not only satisfactory nutrition but also achivement of normal growth. To aid early diagnosis and management, we evaluated the diagnostic methods, operative interventions and clinical characteristics of thirty-nine infants with congenital duodenal obstructions. In the 11-year period from July 1986 through June 1997, thirty-nine patients with congenital duodenal obstruction (23 males and 16 females) were treated and reviewed at the Department of Pediatric Surgery, Yeungnam University Hospital. The ratio of male to female was 1.4:1, and 29 cases(74.1%) among total 39 patients were newborn. There were 5 premature patients and 16 patients of small for gestational age. The most common causes of the congenital duodenal obstruction was malrotation (26 cases, 66.7%) and followed by annular pancreas (9 cases, 23.1%), type 1 atresia (3 cases, 7.7%) and wind-sock anomaly (1 case, 2.6%). Common symptoms were vomiting, abdominal distention, jaundice. Plain abdominal X-ray study combined with upper gastrointestinal series was the most commonly used diagnostic method. The operative procedures were performed by same pediatric surgeon utilizing Ladd's procedure in 26, duodenoduodenostomy in 8, duodenojejunostomy in 4, excision of wind-sock membrane in 1. A total of 15 associated congenital anomalies were found in 9 patients. Postoperative complications occurred in 13 (33.3%). Overall mortality was 2.6%(1/39). Bilious vomiting and plain abdominal radiologic study were most useful for the diagnosis of congenital duodenal obstruction. Early diagnosis and operative intervention were important to prevent complications such as sepsis and peritonitis.
Case Report
The treatment of congenital cutis aplasia.
Young Ha Kim, Gyu Ho Cha, Jae Ho Jung, Kyung Ho Lee, Jung Hyun Seul
Yeungnam Univ J Med. 1992;9(2):422-426.   Published online December 31, 1992
DOI: https://doi.org/10.12701/yujm.1992.9.2.422
  • 1,683 View
  • 1 Download
AbstractAbstract PDF
One case of congenital cutis aplasia is presented. The defect involved includes full-thickness skin defect of scalp and cranium. The patient was treated with debridement of dirty necrosed crust which covered exposed dura mater and with double opposing rotation flap including pericranium for bone regeneration. The donor site was covered with skin graft from right thigh. During operation, the superficial temporal artery was found to be short and weak. And after operation, the margin of flap were congested and finally necrotized. The necrotic wound was treated with conservative management. The vascular impairment is thought to be main course of congenital cutis aplasia. So we conclude that the treatment of choice is conservative management or careful flap surgery for coverage of defect area.
Original Article
A Clinical Study of Congenital Duodenal Obstruction.
Young Soo Huh, Bo Yang Suh, Koing Bo Kwun
Yeungnam Univ J Med. 1990;7(2):39-48.   Published online December 31, 1990
DOI: https://doi.org/10.12701/yujm.1990.7.2.39
  • 1,861 View
  • 1 Download
AbstractAbstract PDF
Congenital duodenal obstruction in the newborn infant may be due to a variety of causes. Duodenal obstruction often presents with bilious vomiting and upper abdominal distention. Diagnosis is usually established on plain x-ray of the abdomen by the classic finding of the double-bubble. In the period July 1986 to June 1990, 16 patients with congenital duodenal obstruction were operated and the following results were obtained. 1. Sixteen patients were comprised of 11 males and 5 females, the ratio of male and female was 2.2:1. 2. Thirteen patients (81%) had been admitted to our hospital during one month of life. 3. Congenital duodenal obstruction was in 16 cases; malrotation in eight (50%), annular pancreas in six (38%), type 1 atresia in one (6%), and wind-sock anomaly in one (6%). 4. There were two premature patients and six patients of small for gestational age. 5. Overall, bilious vomiting, occurring in three fourths, was the single most frequent presenting complaint. 6. Polyhydramnios occurred in two of the patients. 7. Diagnosis was possible with clinical symptom and simple abdomen. 8. The operative procedures performed were; duodenoduodenostomy in five, duodenojejunostomy in two, excision of wind-sock membrane in one, and Ladd's procedure in eight. 9. A total of ten associated congenital anomalies were found in six patients. 10. Postoperative complications occurred in five cases (31%).
Case Report
Congenital Left Ventricular Diverticulum.
Jong Young Kim, Jung Ho Kim, Jin Gon Jun
Yeungnam Univ J Med. 1990;7(2):181-187.   Published online December 31, 1990
DOI: https://doi.org/10.12701/yujm.1990.7.2.181
  • 1,595 View
  • 1 Download
AbstractAbstract PDF
Congenital diverticulosis of the left ventricle is an extremely are rare maldevelopment. We report a 9 year old girl with probable isolated left ventricular diverticulum in whom the diagnosis was made by cross sectional echocardiography and by angiography.

JYMS : Journal of Yeungnam Medical Science
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