• Korean Journal of Cleft Lip And Palate
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• v.8 no.1
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• pp.23-29
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• 2005

The facial cleft and duplicated maxilla are lire congenital anomaly. After Rushton and Walker had reported a unilateral facial cleft with excess tooth and bone formation in 1937, few authors described similar cases. The etiology of this anomaly is not well understood, but considered embryologically as a neurocristopathy. A neurocristopathy is defined as a condition arising from aberrations in early migration, growth and differentiation of neural crest cells. This aberrations result in facial malformation such as facial clefts and loss or duplication of facial structures. We experienced a male newborn baby with bilateral facial cleft and duplicated maxilla. The cleft was surgically corrected when he was 5 months old. The function and appearance of lip are improved. Duplicated maxilla will be surgically removed. We report this case with review of literatures.

• Journal of Chest Surgery
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• v.27 no.8
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• pp.650-655
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• 1994

The author analyzed 99patients with VSD weighting less than 10kg of body weight who underwent surgical correction from 1981 to 1992 at cardiovascular department of Hanyang University hospital. Patients occupied 29.3% of total cases who were underwent surgical corrections for congenital heart diseases during that time. Of the 99 patients, 51 patients were male [52%] and 48 patients[48%] were female. Age ranged from 28 days to 36 months with mean age of 13.6 months. Mean body weight was 7.53kg. According to Kirklin`s anatomical classification, type II defect was most common [61.6%]. Associated anomaly was found in 48 patients [48.5%]. Patent foramen ovale was most commonly associated cardiac anomaly [14.1%] and followed by atrial septal defect [12.1%], patent ductus arteriosus [10.1%]. Cardiac catheterization data were analyzed. The most common range of Qp/Qs, Rp/Rs, Pp/Ps were above 3.0, 0.1 - 0.25, and above 0.75 respectively. Among the indications of surgical correction, there were pulmonary hypertention in 69 patients, congestive heart failure in 44 patients, frequent respiratory infection in 47 patients, growth retardation in 33 patients. The most common surgical approach and method for VSD closure were right atriotomy[48.3%] and dacron patch closure[93.3%]. Complication rate was 13.1% [13 cases], and overall mortality was 17.1% [17 cases]. The cause of death consisted of low cardiac output syndrome[11 cases], acute renal failure[3 cases], sepsis[2 cases] and pulmonary insufficiency[1 case] in order of frequency.

• Clinical and Experimental Reproductive Medicine
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• v.25 no.3
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• pp.323-329
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• 1998

The safety of ICSI as a novel procedure of assisted fertilization may be assessed by the health of the baby born. In order to evaluate the safety of ICSI, perinatal outcome and congenital anomaly of the babies born after ICSI were compared with those of babies born after IVF (control group). We analysed the clinical data from the obstetric and pediatric records, including the information obtained through telephone. The results are as follows; Mean gestational age $({\pm}SEM)$ and birth weight in singleton pregnancy were $38.8{\pm}1.9$ weeks and $3209.7{\pm}501.9gm$ in IVF group, $39.0{\pm}2.2$ weeks and $3289.9{\pm}479.5gm$ in ICSI group, respectively. Mean gestational age and birth weight in twins were $36.8{\pm}2.1$ weeks and $2512.8{\pm}468.0gm$ in IVF group, $36.5{\pm}2.8$ weeks and $2492.7{\pm}537.1gm$ in ICSI group. In IVF group, perinatal mortality rates were 8.5 in singletons and 56.6 in twins; for the ICSI singletons and ICSI twins, the perinatal mortality rates were 11.6 and 49.0, respectively. The incidence of congenital malformations was 3.6% (8/224) in IVF group and 2.1% (4/188) in ICSI group, there was no statistical difference (p>0.05, Fisher's exact test). The incidence of major congenital anomalies was 0.9% (2/224; pulmonary artery hypoplasia, renal cystic dysplasia) in IVF group and 1.1% (2/188; holoprosencephaly, Cri du chat syndrome) in ICSI groups (p>0.05, Fisher's exact test). Similarly, there was no significant difference in incidence of minor congenital anormalies 2.7% (6/224) in IVF group and 1.1% (2/188) in ICSI group respectively (p>0.05, Fisher's exact test). In conclusion, there was no difference in the perinatal outcome and the incidence of congenital anomalies between the babies born after ICSI and those after conventional IVF.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.227-236
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• 2002

Purpose : A study was done to assess the incidence and classification of congenital urinary tract anomalies detected by antenatal ultrasonogram. Methods : We reviewed 558 cases of urinary tract anomaly which were detected by antenatal ultrasonogram and postnatally confirmed between June 1989 and May 2002. We investigated the incidence and classified congenital urinary tract anomalies by review of medical records, antenatal and postnatal radiologic studies retrospectively. Results : In 558 cases of congenital urinary tract anomalies, 292 cases of hydronephrosis were found and the most common. Another anomalies were composed of 65 cases of multicystic dysplastic kidney, 32 cases of hydroureteronephrosis, 31 cases of duplication of kidney, 25 cases of renal agenesis, 21 cases of simple renal cyst, 20 cases of polycystic disease, 13 cases of ureterocele, 11 cases of renal hypoplasia, 10 cases of horseshoe kidney, 9 cases of vesicoureteral reflux, 8 cases of posterior urethral valve, 7 cases of bladder diverticulum, 6 casts of megaureter, 5 cases of ectopia, 2 cases of megacystis, and 1 case of medullary cystic disease. In 82 of the 558 cases, there were two or more combined urinary tract anomalies. Associated diseases other than urinary tract were observed in 13 cases, of which the congenital heart disease was the most common. Conclusion : The congenital urinary tract anomaly is frequently found and diverse during the antenatal ultrasonography. The multicenter study is needed to investigate precise incidence and distribution of each anomalies in general population.

• Korean Journal of Veterinary Service
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• v.38 no.3
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• pp.189-193
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• 2015

Epitheliogenesis imperfecta is a congenital disease in which localized or widespread defect areas of squamous epithelium of the skin. Epitheliogenesis imperfecta is rare anomaly of calves, piglets, foals, lambs, puppies, and kittens. Three cases of epitheliogenesis imperfecta in Korean indigenous calves were diagnosed in animal and Plant Quarantine Agency in 2011~2014. In this paper, we tried to report them in detailed and compare with similar cases occurred in other countries.

• Journal of Chest Surgery
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• v.36 no.10
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• pp.759-765
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• 2003

Truncus arteriosus with interrupted aortic arch is a very rare congenital cardiac anomaly that has an unfavorable natural course. We report a successful one-stage repair of truncus arteriosus with interrupted aortic arch through median sternotomy in a 25-day-old neonate weighing 3.1 kg. We reconstructed the aortic arch with direct side-to-end anastomosis between ascending and descending aortas. The right ventricular outflow reconstruction was performed with untreated autologous pericardial conduit without valve following Lecompte maneuver. The patient has been grown-up in good condition (25 ∼ 50 percentile of body weight) and shows the right ventricular outflow tract wide 1 year after the operation.

• Journal of Chest Surgery
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• v.39 no.8 s.265
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• pp.643-647
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• 2006

Congenital tracheomalacia associated esophageal atresia is a rare foregut anomaly. We report a case of 40-day old male infant with tracheomalacia who has undergone repair of esophageal atresia at his age of 1 day. The patient had progressive dyspnea and stridor after repair of esophageal atresia. His 3-dimensional chest computed tomography showed severe stenosis at the middle of trachea. We underwent resection and end-to-end anastomosis under cardiopulmonary bypass. Histologic examination revealed esophageal tissues indicating congenital origin as well as no cartilage.

• Annals of Clinical Neurophysiology
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• v.20 no.2
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• pp.85-88
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• 2018

Septo-optic dysplasia is a congenital anomaly with diverse phenotypes from normal to mixtures of visual abnormality, endocrine dysfunction, psychomotor retardations and epileptic seizures. It is characterized by optic atrophy, pituitary dysfunction and midline structure abnormalities in corpus callosum or septum pellucidum. Diagnosis of septo-optic dysplasia plus is made when cortical malformations accompanied. Here we report a middle-aged woman with septo-optic dysplasia plus having unilateral optic atrophy, agenesis of septum pellucidum and cortical malformations.

• Journal of Korean Neurosurgical Society
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• v.45 no.1
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• pp.43-45
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• 2009

A 75-year-old female presented with subarachnoid hemorrhage. Angiography revealed a partial duplication (fenestration) in the proximal $A_1$ segment and a ruptured aneurysm at the distal end of $A_1$ fenestration. This congenital anomaly accompanying an aneurysm was associated with duplicated ipsilateral middle cerebral artery (MCA). Congenital defect of the arterial wall and hemodynamic factors at the fenestrated $A_1$ are considered to play a significant role in the development of this aneurysm. The present case is peculiar because not only the ruptured $A_1$ aneurysm was related with the anterior and middle cerebral artery duplication but also the location of $A_1$ fenestration and the origin of $A_1$ aneurysm in a fenestration are quite unusual.

• Journal of Chest Surgery
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• v.28 no.4
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• pp.381-386
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• 1995

In our hospital we have seen 20 cases of congenital diaphragmatic anomalies from June 1984 until December 1993. These were classified into 10 cases of diaphragmatic eventration, 8 cases of Bochdalek hernia, 1 case of Morgagni hernia, and 1 case of esophageal hiatal hernia. Diaphragmatic eventration cases were composed of 8 males and 2 females with ages varing from 3 hour to 42 year. They were discovered by symptoms: 5 cases of respiratory insufficiency; 3 cases of frequent respiratory infection; and 2 cases by chance; 6 cases involved the left side, 4 cases involved right side. Emergency operations were done to 4 patients. Among the 10 patients, only one operative mortality occurred; 3 hour old female.Bochdalek hernia cases composed 6 females and 2 males, 5 patients were less than 6 hour old. All patients were operated on an emergency status and three of them expired due to the vicious cycle of pulmonary hypertension and pulmonary vasoconstriction, persistent fetal circulation, hypoxia, and metabolic acidosis. Morgagni hernia was seen in one 69 year old female patient, she had no complaint of symptoms and was incidentally detected. Hernia was repaired through right thoracotomy. She was discharged with healthy appearence. Esophageal hiatal hernia was seen in a 10 month old male patient, his symptoms were persistent vomiting and coughing since birth. Sliding type of esophageal hiatal hernia repair was completed through left thoracotomy.