• Journal of Chest Surgery
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• v.21 no.1
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• pp.70-81
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• 1988

From January 1962 to March 1987, 97 patients underwent operation for mitral valve disease. Of these patients, 61 [62.9%] required mitral valve replacement. Thirty-six patients [37.1%] had mitral valve repair. The mean age was 26.9*11.6 years [range 5 months to 48 years]. There were 32 [88.9%] cases of rheumatic valve disease, 4 [11.1%] cases of congenital mitral valve disease. Valve dysfunction was classified into three types: type I [normal leaflet motion], 6 patients; type II [prolapsed leaflet], 1 patient; type III [restricted leaflet motion], 29 patients. Twenty-nine patients [80.6%] had pure or predominant stenosis and 7[19.4%] had pure regurgitation. No patient was in NYHA functional class I. Three patients [16.7%] were in functional class II, 15[83.3%] were in functional class II. The techniques used included closed mitral commissurotomy [16 patients], open mitral commissurotomy [13 patients], localized Wooler type annuloplasty [4 patients], suture repair of leaflet defect [3 patients], chordal shortening [1 patient], Carpentier ring annuloplasty [1 patient], and fenestration of fused chordae [1 patient]. There were two perioperative deaths [5.6%], related to left ventricular failure and reoperation. The survivors were followed up for 94 patient-years [mean 4.68*5.54 years]. One late death [1.1*1.1% per patient-year] occurred and was valve related. Reoperation was required in 3 patients, of whom 2 were deaths. There was 1 case [1.1*1.1% per patient-year] of thromboembolism. No patient received anticoagulant after operation. At 10 year, 92*7.4% of the patients were still alive. The actuarial survival rate of patients free of valve-related complication was 79*6.4% at 6 years, 27*12.1% at 11 years. After surgery, 18 patients [88.9%] were in NYHA functional class I or II.

• Journal of Chest Surgery
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• v.29 no.2
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• pp.136-146
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• 1996

Studies on normal human embryos and on malformed human hearts have been two main sources of the information on the developmental cardiology, Recent advances in the biological technology has opened a new era and descriptive embryology is being shifted into dynamic developmental biology. In this review, we discuss the current understanding on the cardiac embryology relevant to clinical practices of pediatric cardiology. Classical cardiac embryology starts with understanding on five segments of a straight heart tube : the sinus venosus, the primitive atria, the embryonic left ventricle, the embryonic right ventricle and the truncus arteriosus. Key steps in the normal morphogenetic process are the complex spiral septation of ventriculoarterial junction and two jumping connections : between the embryonic right atrium and embryonic right ventricle, and between the embryonic left ventricle and the aorta. Only after these two steps are successfully completed, the third fetal stage tak s place, when myocardial growth and remodeling take place There are two outstanding progresses on the cardiac embryology during recent five-year period. One is immunohistochemical mapping of the conduction system in the developing heart and the other is the understanding on the neural crest cell migration followed by molecular detection of the microdeletion of chromosome 22. A balanced progress of classical morphological studies, modern biological technics and advanced clinical medicine is an urgent task for doctors and scientists dealing with children with sick hearts.

• Journal of Chest Surgery
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• v.44 no.2
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• pp.108-114
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• 2011

Background: Homograft cardiac valves and valved-conduits have been available in our institute since 1992. We sought to determine the long-term outcome after right ventricular outflow tract (RVOT) reconstruction using homografts, and risk factors for reoperation were analyzed. Materials and Methods: We retrospectively reviewed 112 patients who had undergone repair using 116 homografts between 1992 and 2008. Median age and body weight at operation were 31.2 months and 12.2 kg, respectively. The diagnoses were pulmonary atresia or stenosis with ventricular septal defect (n=93), congenital aortic valve diseases (n=15), and truncus arteriosus (N=8). Mean follow-up duration was $79.2{\pm}14.8$ months. Results: There were 10 early and 4 late deaths. Overall survival rate was 89.6%, 88.7%, 86.1% at postoperative 1 year, 5 years and 10 years, respectively. Body weight at operation, cardiopulmonary bypass (CPB) time and aortic cross-clamping (ACC) time were identified as risk factors for death. Forty-three reoperations were performed in thirty-nine patients. Freedom from reoperation was 97.0%, 77.8%, 35.0% at postoperative 1 year, 5 years and 10 years respectively. Small-sized graft was identified as a risk factor for reoperation. Conclusion: Although long-term survival after RVOT reconstruction with homografts was excellent, freedom from reoperation was unsatisfactory, especially in patients who had small grafts upon initial repair. Thus, alternative surgical strategies not using small grafts may need to be considered in this subset.

• Korean Journal of Cleft Lip And Palate
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• v.15 no.2
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• pp.61-68
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• 2012

Cleft lip and palate is the most common congenital facial malformation and has a significant developmental, physical, and psychological impact on those with the deformity and their families. When treating the patients with unilateral cleft lip, many surgeons adopt the rotation advancement flap method originally developed by Millard, or the triangular flap technique developed by Tennison, Randall or the modifications of these techniques. Among these, Millard's rotation advancement flap method has its advantage in designing the flap using the patient's anatomic landmarks. For performing this rotation advancement technique, skillful operation is needed to obtain esthetically satisfactory results. Vomer flap sometimes is used to repair anterior hard palate in complete cleft lip and palate patients. Vomerine tissue is readily available in the vicinity of the palatal defect and elevation of the vomerine flap is relatively simple procedure. In this article, we will introduce the comprehensive vomer flap technique conjunction with primary lip closure and review the comparative studies of the outcome of simultaneous repair of cleft lip and cleft hard palate with Millard's rotation advancement method and vomer flap.

• Advances in pediatric surgery
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• v.6 no.1
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• pp.1-9
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• 2000

Despite of advances in perinatal management and treatment modalities congenital diaphragmatic hernia(CDH) remains a frustrating problem. Although the sheep has proven to be a reliable experimental model for the production of intrauterine CDH, the rabbit may have some advantages. These include lower cost, smaller body size, year-round availability, high number of fetuses per pregnancy, and short gestational period. To evaluate the feasibility of the rabbit model of CDH, twenty-seven pregnant New Zealand rabbits were utilized. Hysterotomy and an operative procedure for creating a diaphragmatic defect on gestational day 24 or 25, in two fetuses of each pregnant rabbit were performed. In one fetus of one cornu of the uterus, the left fetal diaphragm was excised through an open thoracotomy(DH group). In another fetus in the other cornu, CDH was created and the trachea clipped(Surgiclip, USSC, Norwalk, Conn., USA) (TL group). Delivery was by Cesarean section on 30 days of gestation. Among twenty- seven pregnant rabbits, 12 in the DH group and eight in the TL group were born alive. The most common herniated organ was the left lobe of the liver. In thee DH group, the lungs were hypoplastic with decreased lung weight/body weight ratio, reduced numbers of alveoli, thicker media of the pulmonary arteries, and immature alveoli. In TL group, the alveoli were more mature and did not differ from the control animals. In conclusion, (1) pulmonary hypoplasia develops in the fetal rabbit diaphragmatic hernia model and (2) simultaneous tracheal ligation prevents pulmonary hypoplasia.

• Journal of the korean academy of Pediatric Dentistry
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• v.42 no.2
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• pp.180-187
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• 2015

Incontinentia pigmenti, also called Bloch-Sulzberger syndrome, is a rare X-linked inherited dominant disorder that affects females, but causes spontaneous abortion of prenatal males. Incontinentia pigmenti is a systemic disease with clinical features similar to ectodermal dysplasia, including congenitally missing teeth. The pathogenesis is related to gene mutations in NF-kappa-B essential modulator on chromosome Xq28. Incontinentia pigmenti is caused by a defect in the developmental stage of organs originating from the ectoderm or mesoderm and involves the skin, eyes, hair, teeth and central nervous system. This report discusses the management of three cases of 3 to 5 years old females with incontinentia pigmenti and accompanying multiple missing teeth. The cases had sparse hair, and showed oligodontia and anomalous crowns with supplementary cusps in the posterior teeth and conical anterior teeth. Removable space maintainers were applied, achieving improved esthetics, recovery of mastication and increased self-esteem in the patients.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.27 no.2
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• pp.183-187
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• 2005

The coronal approach has been used for over a century by neurosurgeons to access to the anterior cranium. Indications for the coronal approach expanded from use in the correction of congenital skeletal anomalies to applications in acute maxillofacial trauma and secondary deformity correction, oncologic surgery and reconstruction, and esthetic surgery. Complications were such as injury to frontal branch of the facial nerve, motor nerve paralysis, hematoma under flap, trismus, ptosis, epiphora, infection and anterior temporal depression. $Medpor^{(R)}$ is made up of dense polyethylene connected in porous structures. It is easily shapable without collapsing the pores due to it's hardness and tissue growth takes place at the porosities. Based on these advantages, $Medpor^{(R)}$ has been used in augmentation and restoration in craniofacial defect. A temporal depression after the coronal approach for treatment of Le Fort III fracture was successfully reconstruction with $Medpor^{(R)}$ and we report this case with review of literature.

• The Journal of the Korean bone and joint tumor society
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• v.8 no.2
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• pp.63-67
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• 2002

Myositis ossificans progressiva, also known as 'fibrodysplasia ossificans progressiva' is a rare disorder, most probably inherited as a mendelian dominant trait with irregular penetrance. It is characterized by congenital malformations of the great toes and progressive edema, calcification and ossification of the fasciae, aponeurosis, ligaments, tendons, and connective tissue in interstitial tissues of skeletal muscle. The basic defect is in the connective tissue, whereas the skeletal muscle remains fundamentally normal. We report two cases of a brother and sister whose the disorder is involved in a same family.

• Journal of Chest Surgery
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• v.31 no.9
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• pp.915-918
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• 1998

The chest wall deformity associated with Poland's syndrome is a very rare anomaly which consists of congenital unilateral absence of the sternal head of the pectoralis major muscle and various abnormalities of the upper extremity. Other clinical features associated with Poland's syndrome include deficiency or absence of the breast and nipple, deficiency of subcutaneous fat and axillary hair, and abnormalities of costal cartilages and anterior ends of ribs. The origin remains uncertain, but is considered not to be hereditary. Poland's syndrome may pose a serious psychologic and cosmetic problem, early recognition and surgical correction may prove beneficial. A 37 year old patient with Poland's syndrome was encountered and underwent satisfactory surgical correction.

• Journal of Chest Surgery
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• v.14 no.4
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• pp.381-387
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• 1981

Fourteen Infants with congenital cardiac anomalies underwent primary surgical Intervention within the first 12 months of life. There were eight patients with ventricular septal defect, two with total anomalous pulmonary venous return [TAPVR], and the remainders with tetralogy of Fallot, transposition of great arteries [d-TGA], Taussing-Bing malformation, and coronary A-V fistula. The age of the patients ranged from 5 to 12 months, with a mean age of 9.9 months. The mean weight was 6.7 Kg [3.8 to 9.5 KS]. Congestive heart failure persisting despite intensive medical treatment was present In 8 patients [56%], and was the most common indication for operation. Early operation was necessary in 5 of these patients [35%], because of failure to thrive and recurrent pulmonary infection. In one patient with TOF, frequent hypoxic spell prompted the necessity for early operation. In cases of VSD, TAP. VR, TOF, and coronary A-V fistula, Intracardiac repair was done with conventional cardiopulmonary bypass, chemical cold cardioplegia, and topical myocardial cooling. Deep hypothermic circulatory arrest with surface induced cooling, followed by core cooling and core rewarming, was employed .for better exposure in the cases of d-TGA and Taussing-Bing malformation. The results were however, not satisfactory. The overall mortality was 28 per cent. There were no deaths in the eight patients with VSD. The one with coronary A-V fistula survived. The other 5 cases all expired either on the table or immediately after operation. The non-fatal post-operative complications included low cardiac output, respiratory insufficiency, bleeding, and temporary A-V block. The causes of death were prolonged circulatory arrest time in d-TGA, complete A-V block and low cardiac output in TOF and Taussing-Bing malformation and prolonged bypass time and Inadequate correction in TAPVR.