• Journal of Chest Surgery
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• v.24 no.8
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• pp.819-823
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• 1991

The congenital cystic adenomatoid malformation of the lung is a rare disease, and is one of the most common congenital lung diseases which require prompt surgical intervention. The prognosis depends on its tissue type, prompt diagnosis and surgical intervention. The lesion consists of enlarged, variable sized multiple cyst with overgrowth of terminal bronchioles, like hamartoma. This disease can be associated with other vascular anomalies or other congenital defect especially in type II lesion We recently experienced one case of congenital cystic adenomatoid malformation The patient was 2 months old infant who showed respiratory distress without associated anomaly. After right upper lobe lobectomy, the patient was recovered uneventfully.

• Journal of Korean Neurosurgical Society
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• v.46 no.6
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• pp.522-527
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• 2009

Objective : Atlantal arch defects are rare. Few cadaveric and imaging studies have been reported on the variations of such anomalies. Our goal in this study was to examine the incidence and review the clinical implications of this anomaly. Methods : A retrospective review of 1,153 neck or cervical spine computed tomography (CT) scans was performed to identify patients with atlantal arch defects. Neck CT scans were performed in 650 patients and cervical spine CT scans were performed in 503 patients. Posterior arch defects of the atlas were grouped in accordance with the classification of Currarino et al. In patients exhibiting this anomaly, special attention was given to defining associated anomalies and neurological findings. Results : Atlantal arch defects were found in 11 (11/1153, 0.95%) of the 1,153 patients. The type A posterior arch defect was found in nine patients and the type B posterior arch defect was found in two patients. No type C, D, or E defects were observed. One patient with a type A posterior arch defect had an anterior atlantal-arch midline cleft (1/1153, 0.087%). Associated cervical spine anomalies observed included one $C_{6-7}$ fusion and two atlantal assimilations. None of the reviewed patients had neurological deficits because of atlantal arch anomalies. Conclusion : Most congenital anomalies of the atlantal arch are found incidentally during investigation of neck mass, neck pain, radiculopathy, and after trauma.

• Journal of Chest Surgery
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• v.39 no.12 s.269
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• pp.931-933
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• 2006

Robotic assisted surgery in adult patient with congenital ventricular septal defect Since December in 2005, we have done minimally invasive surgeries in selected cases of mitral valve diseases, tricuspid valve diseases, atrial septal defects and atrial fibrillations with the $AESOP^{TM}$ robotic arm. We have had a better surgical view and skill, according to accumulation of the experience of this procedure. Recently, we performed robotic assisted surgery in a 47-year-old female with congenital perimembranous ventricular septal defect.

• The Korean Journal of Nuclear Medicine
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• v.15 no.1
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• pp.37-43
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• 1981

Congenital hypothyroidism due to organification defect was first reported by Haddad and Sidbury in 1959. The organification defect is easily proved by perchlorate discharge test. We experienced a patient who had large goiter, growth and mental retardation, and revealed positive reponse to perchlorate discharges test, and the surgical biopsied specimen showed Huerthle cell adenoma, which was probably due to chronic stimulation of thyroid stimulating hormone, or coexisted incidentally. Described here a case of congenital hypothyroidism due to organification defect associated with Huerthle cell adenoma, with review of some literatures.

• Journal of Chest Surgery
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• v.24 no.1
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• pp.83-87
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• 1991

A unilateral pulmonary artery agenesis, which develops occasionally as one of the associated anomalies in congenital cardiovascular defect like as tetralogy of Fallot, is very rare anomaly as an isolated congenital defect. The diagnostic approach for the pulmonary artery agenesis is first suggested by the unique appearance of the involved lung on a routinely checked chest roentgenogram, because most patients are asymptomatic unless pulmonary infection had been superimposed. We have recently experienced a case of left pulmonary artery agenesis, which was diagnosed by perfusion scan, digital subtraction angiogram and then treated by left pneumonectomy in a 9 year-old boy, and presented hereby with the review of relevant literature.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.39
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• pp.5.1-5.9
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• 2017

Many congenital and acquired defects occur in the maxillofacial area. The buccal fat pad flap (BFP) is a simple and reliable flap for the treatment of many of these defects because of its rich blood supply and location, which is close to the location of various intraoral defects. In this article, we have reviewed BFP and the associated anatomical background, surgical techniques, and clinical applications. The surgical procedure is simple and has shown a high success rate in various clinical applications (approximately 90%), including the closure of oroantral fistula, correction of congenital defect, treatment of jaw bone necrosis, and reconstruction of tumor defects. The control of etiologic factors, size of defect, anatomical location of defect, and general condition of patient could influence the prognosis after grafting. In conclusion, BFP is a reliable flap that can be applied to various clinical situations.

• Clinical and Experimental Pediatrics
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• v.48 no.8
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• pp.799-805
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• 2005

Hypothyroidism is a deficiency in thyroid hormone secretion by the thyroid gland and a defect in thyroid hormonal receptor activity. It is categorized by the two major forms in children, the one is congenital hypothyroidism and the other is acquired hypothyroidism. Congenital hypothyroidism is one of the commonest treatable causes of mental retardation and occurs in 1 in 3,000-4,000 infants worldwide. Acquired hypothyroidism is a diseases that have an onset usually after 6 months of age and it may be relate to deceleration in linear growth. The objectives of this article are obtain general and practical concepts of congenital and acquired hypothyroidism during infancy, childhood, and adolescence.

• Journal of Chest Surgery
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• v.19 no.2
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• pp.250-258
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• 1986

From Aug. 1984 to May 1986, 116 cases of cardiovascular surgery including 85 cases of open heart surgery were performed at Korea Veterans Hospital. There were 108 congenital anomalies and 8 acquired valvular heart diseases. Among 108 congenital malformations 92 operations were done for acyanotic group, and 16 operations for cyanotic group. Thirteen cases of open heart surgery for infants or small children under 10kg of body weight were performed, which occupied 15.3 percent of total open heart surgery done in the same period. Common congenital cardiovascular anomalies were ventricular septal defect [40.7%], patent ductus arteriosus [29.6%], tetralogy of Fallot [12.[%], atrial septal defect [11.1%], and pulmonary stenosis [1.9%] in order of frequency. Valve replacement using lonescu-Shiley or Carpentier-Edwards valve was performed for 8 cases of acquired mitral valve disease, and valve replacement using St. Jude valve was done for a case of patent ductus arteriosus with severe mitral insufficiency. There was no mortality in acyanotic congenital malformations and acquired valve lesions, and 5 cases of mortality in cyanotic congenital malformations. Overall mortality was 4.3 percent for total cases and 5.9 percent for 85 cases of open heart surgery.

• Journal of Chest Surgery
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• v.20 no.4
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• pp.659-672
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• 1987

Nitrofen [2,4-dichlorophenyl-P-nitrophenyl ether] is a diphenyl ether herbicide used for pre and post-emergent control of broad leafed weeds. This chemical was known to induce a variety of congenital cardiovascular anomalies with diaphragmatic hernia and hydronephrosis in the rate fetuses. The present study was conducted to produce congenital cardiovascular anomalies in the rat fetuses by oral nitrofen administration at the indicated doses and days of gestation, and to find the characteristics of nitrofen-induced cardiovascular anomalies. All the observed fetuses were removed from the pregnant Sprague-Dawley rats sacrificed on the twenty-first day of gestation. They were preserved in 10 per cent formalin and dissection for examination were carried out under a dissecting microscope using forceps and scissors. Following results and conclusion were based on dissecting microscopic findings on 482 offsprings. 1. The eleventh day of gestation was the most sensitive day for nitrofen induction of congenital cardiovascular anomalies in the rat. This incidence was dose-related in rats exposed on the eleventh day of gestation. 2. Ventricular septal defect was the most common single anomaly that represented more than half of the total cardiovascular anomalies, followed by aortic arch anomalies and tetralogy of Fallot. 3. Cardiac anomalies derived from infundibular maldevelopment such as tetralogy of Fallot and pulmonary atresia with ventricular septal defect were only observed in the eleventh gestation day treated group. 4. Aortic arch anomalies were found in high frequency and the great majority were characteristically anomalous right subclavian artery with left aortic arch. Key words; nitrofen, congenital cardiovascular anomalies.

• Clinical and Experimental Pediatrics
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• v.46 no.3
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• pp.308-311
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• 2003

Congenital chloride diarrhea is a serious autosomal recessive disease, and defect of intestinal electrolyte absorption that involves, specifically, $Cl^-/{HCO_3}^-$ exchange in the distal part of the ileum and colon. The clinical feature is dominated by profuse, watery diarrhea containing high concentrations of chloride(>90 mmol/L) and sodium. The chloride loss results in severe dehydration with a hypochloremic alkalosis. The molecular pathology involves an epithelial $Cl^-/{HCO_3}^-$ exchanger protein. Mucosal ion transport is affected to differing degrees and the severity of the disease may thus vary. Recently, a gene defect on chromosome 7 has been identified. However, there was a deficit in replacement of fluid and electrolyte, abdominal distension remained and the character of stools was watery. We report a case of congenital chloride diarrhea in a premature female who presented with watery diarrhea containing high concentrations of chloride and abdominal distension.