• Journal of Yeungnam Medical Science
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• v.13 no.2
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• pp.367-371
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• 1996

Median raphe cyst is known as congenital lesion of the perineum and genitalia, but its etiology is unclear. Most investigators believe that the median raphe cyst represent defects in the embryologic developement of the male genitalia. Simple surgical excision is effective in most cases. We report our experience with two cases of median raphe cyst without specific symptoms. Even though median raphe cyst is asymptomatic, surgical therapy is worth applicable because it relieve a patient from cosmetic and psychotic problem.

• Clinical and Experimental Pediatrics
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• v.46 no.4
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• pp.389-392
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• 2003

Netherton's syndrome is an unusual disorder which consists of triad of ichtyosiform dermatosis, multiple defects of hair shaft and an atopic diathesis. The finding of bamboo hair is pathognomic in Netherton's syndrome and the ichthyosiform dermatosis may consist of either ichtyosis linearis circumflexa or congenital ichthyosiform erythroderma. Often, variability in the clinical features leads to a delay in diagnosis in many cases. We report a case of Netherton's syndrome diagnosed in the neonatal period. The patient presented with severe ichthyosis and confirmed microscopically distinctive bamboo hair.

• Journal of Chest Surgery
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• v.11 no.1
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• pp.69-74
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• 1978

Coarctation of the aorta is rare condition among the congenital cardiovascular defects in Korea. We experienced one case of coarctation of the aorta [postductal type], which was successfully corrected with Dacron graft. This 20 year old man was admitted to our hospital for evaluation of hypertension in the upper extremities and frequent occipital headache of 2 month duration. On physical examination, grade II systolic murmur was heard over the apex and mid back, but thrill was not palpable. Blood pressures were measured at both extremities, its values were 190/70mmHg. in the arms and 120/60mmHg in the legs. Routine chest roentgenogram showed the dilated left subclavian artery high on the left mediastinal border and associated with left ventricular prominence, but notchings of lower border of rib was not seen. Electrocardiogram also showed left ventricular hypertrophy. Final Pre-operative diagnosis was made by the aortic catheterization and aortography, which showed the typical configuration of postductal type of coarctation with poststenotic dilatation of aorta. Under the moderate hypothermia by surface cooling, coarcation was resected 3.5cm in length and then Dacron graft was inserted. After prosthetic correction, blood pressures were recorded 120/60mmHg in arms and 160/120mmHg in legs. The post-operative course was uneventful.

• Journal of Chest Surgery
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• v.49 no.2
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• pp.112-114
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• 2016

The half-turned truncal switch (HTTS) operation has been reported as an alternative to the Rastelli or $r{\acute{e}}paration$ $\grave{a}$ $l^{\prime}{\acute{e}}tage$ ventriculaire procedures. HTTS prevents left ventricular outflow tract (LVOT) obstruction in patients with complete transposition of the great arteries (TGA) with a ventricular septal defect (VSD) and pulmonary stenosis (PS), or in those with a Taussig-Bing anomaly with PS. The advantages of the HTTS procedure are avoidance of late LVOT or right ventricular outflow tract (RVOT) obstruction, and of overstretching of the pulmonary artery. We report the case of a patient who underwent HTTS for TGA with VSD and PS, in whom there was no LVOT obstruction and only mild aortic regurgitation and mild RVOT obstruction, including observations at 12-year follow-up. Our experience with long-term follow-up of HTTS supports a solution for late complications after the Rastelli procedure.

• Journal of Chest Surgery
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• v.45 no.6
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• pp.415-417
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• 2012

The development of Morgagni hernias is related to malformations of the embryologic septum transversum after failure of the sternal and costal fibrotendinous elements of the diaphragm to fuse. The overall incidence of Morgagni hernias among all congenital diaphragmatic defects is 3% to 4%. Inguinal hernias are the most common hernias in males and females (25% and 2%, respectively). An inguinal hernia is defined as a protrusion of the abdominal cavity contents through the inguinal canal. The combination of Morgagni and inguinal hernias is rare, and treatment using laparoscopic surgery has not been previously reported. This case presents a one-stage laparoscopic repair of Morgagni and inguinal hernias in a 2-month-old male.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.44 no.3
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• pp.128-135
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• 2018

Objectives: The role of alloplastic materials in maxillofacial reconstruction is still controversial. Determining the utility of porous, high-density, polyethylene implants as a highly stable and flexible, porous alloplast, with properties such as rapid vascularization and tissue ingrowth, is crucial in cases of maxillofacial deformities and aesthetic surgery. Materials and Methods: Thirty high-density porous polyethylene implants were implanted in 16 patients that had been referred to a private office over a three-year period. These implants were used for correcting congenital deformities, posttraumatic defects and improving the aesthetic in nasal, paranasal, malar, chin, mandibular angle, body and orbital areas. Results: The outcomes of the cases in this study showed good aesthetic and functional results. The majority of patients had no signs of discomfort, rejection or exposure. Two implants suffered complications: a complicated malar implant was managed by antibiotic therapy, and an infected mandibular angle implant was removed despite antibiotic therapy. Conclusion: Based on the results, the Medpor implant seems to be an excellent biomaterial for correcting various facial deformities. Advantages include its versatility and relatively ideal pore size that allows for excellent soft tissue ingrowth and coverage. It is strong, flexible and easy to shape.

• Korean Journal of Cleft Lip And Palate
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• v.14 no.1_2
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• pp.37-44
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• 2011

Amniotic fluid is a complex and biological reservoir that provides mechanical cushioning and has many nutrients required for fetal growth and development. During our main research works about the fetal surgery of congenital facial defects, we reviewed several recent articles about the effectiveness and composition of amniotic fluid. Among these review processes, amniotic fluid, as the convenient medium to store sking grafts, was focused especially for its growth factors and rich nutrients, and we summarized some experimental investigations of skin grafts stored in amniotic fluid in rats. We reviewed mainly the article, "Turhan-haktanir N. et al. Histological assessment of skin grafts in amniotic fluid and saline. J Plast Surg Hand Surg 2010;44:226-30."

• Biomedical Science Letters
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• v.21 no.1
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• pp.15-22
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• 2015

Dexamethasone, a synthetic glucocorticoid (GC), is clinically administered to woman at risk for premature labor to induce fetal lung maturation. However, exposure to repeated or excess GCs leads to intrauterine growth restriction (IUGR) and subsequently increases risk of psychiatric and cardio-metabolic diseases in later life through fetal programming mechanisms. GCs are key mediators of stress responses, therefore, maternal nutrient restriction or psychological stress during pregnancy also causes negative impacts on birth and neurodevelopment outcome of fetuses, and other congenital defects, such as craniofacial and skeletal abnormalities. In this study, to examine the effect of prenatal stress on fetal skeletal development, dexamethasone (1 mg/kg [DEX1] or 10 mg/kg [DEX10] maternal body weight per day) was administered intraperitoneally at gestational day 7.5~9.5 and the skeletons were prepared from embryos at day 18.5. Seven out of eighteen (39%) embryos treated with DEX10 showed axial skeletal abnormalities in either the T13 or L1 vertebrae. In addition, examination of the sternum revealed that xiphoid process, the protrusive triangular part of the lower end of the sternum, was bent more outward or inward in DEX group embryos. In conclusion, our findings suggest a possible link to the understanding of the effect of uterine environment to the fetal skeletal features.

• Archives of Plastic Surgery
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• v.35 no.1
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• pp.96-99
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• 2008

Purpose: A meningoencephalocele is a congenital malformation involving herniation of the meninges and cerebral tissue through a defect in the skull. For the patient with frontoethmoidal meningoencephalocele with hypertelorism, the removal of the meningoencephalocele without correction of the combined hypertelorism is not enough for getting a good cosmetic appearance. Correction of the hypertelorism is needed for cosmetic problem. We experienced a case of simultaneous correction of frontoethmoidal meningoencephalocele with hypertelorism. Methods: The meningoencephalocele was removed and the hypertelorism was corrected by central segment technique. The bone defects were filled with autogenous bone dusts. And the nose was reconstructed by a calvarial bone graft. Results: The patient had a good cosmetic appearance without any neurological complications without serious complications. Conclusion: We experienced a case of simultaneous correction of frontoethmoidal meningoencephalocele with hypertelorism. And a brief review of related literatures is given.

• Neonatal Medicine
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• v.17 no.1
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• pp.127-131
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• 2010

Supernumerary derivative (22) syndrome is a rare genomic syndrome. It is characterized by severe mental retardation, microcephaly, failure to thrive, preauricular tag or sinus, ear abnormalities, cleft and/or high-arched palate, micrognathia, kidney abnormalities, congenital heart defects, and genital abnormalities in males. In 99% of the cases, one of the parents is a balanced carrier of a translocation between chromosome 11 and chromosome 22. To date, there have been about 100 case reports of supernumerary derivative (22) syndrome. In most of the cases, supernumerary derivative (22) syndrome was the result of 3:1 meiotic segregation in the maternal 11;22 translocation carrier. We now report a case of 47,XX, + der(22)t(11;22)(q23;q11.2) resulting from 3:1 meiotic segregation of the paternal translocation carrier.