• Journal of the korean academy of Pediatric Dentistry
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• v.33 no.1
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• pp.122-130
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• 2006

Congenital Missing Teeth(hypodontia, oligodontia) is the developmental absence of one or more teeth. It has been reported as being the most common anomaly of dental development in human, relatively common in the permanent dentition. In a recent review, Vastardis has quoted incidence ranges of $1.6%{\sim}9.6%$ in the permanent dentition. Brook has quoted a prevalence of $3.5%{\sim}6.5%$ in most populations, with severe hypodontia, defined as the absence of six or more teeth, having a prevalence of $0.3{\sim}0.4%$. The most commonly affected teeth are third molars, followed by maxillary lateral incisor, and second premolars. The etiology is unknown, several hypotheses include trauma, nutritional deficiency, infection, metabolic abnormalities, systemic disease and genetic influence. The multiple congenital missing is commonly associated with specific syndrome or severe systemic abnormalities such as cleft lip & palate and Down's syndrome. These cases present that children have multiple congenital missing teeth in the permanent dentition, without any systemic disease. Management of this condition must be considered orthodontic and prosthodontic treatment comprehensively. In these cases, children were treated by space maintainer or orthodontic appliance and follow-up checked.

• Journal of Yeungnam Medical Science
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• v.7 no.2
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• pp.39-48
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• 1990

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 asociated congenital anomalies were found in six patients. 10. Postoperative complications occurred in five cases(31%).

• Advances in pediatric surgery
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• v.17 no.2
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• pp.162-169
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• 2011

Branchial cleft anomalies are the second most common head and neck congenital lesions seen in children. Amongst the branchial cleft malformations, second cleft lesions account for 95 % of the branchial anomalies. This article analyzes all the cases of second branchial cleft anomalies operated on at Seoul National University Hospital from September 1995 to February 2011. We analyzed sex, age, symptom and sign, accompanied anomaly, diagnosis, treatment, pathologic report and outcome via retrospective review of medical records. In this series, we had 61 patients (27 female and 34 male). The mean age at the time of operation was 38 months. 31 lesions were on the right, 20 were on the left and 10 were bilateral. The most frequent chief complaints at presentation were non-tender mass and cervical opening without any discharge. According to anatomic type, 29 patients had branchial cleft sinuses, 14 had cysts, 14 had fistulas and 4 had skin tags. Complete excision was attempted if possible and antibiotics challenged when infection was suspected. Complete excision was achieved in 96.7 % of cases. Incision and drainage was done in 2 cases due to severe inflammation, and both recurred. Postoperative complications included wound infection in 2 cases. Microscopic examonation revealed squamous epithelium in 90.2 % and squamous metaplasia in one case in the branchial cleft cyst wall. In summary, second branchial anomaly is found more frequently on right side of neck. Fistulas are diagnosed earlier than cystic forms. Most cases could be diagnosed by physical examination. The definitive treatment is complete excision and sufficient antibiotics coverage for cases with inflammation. After drainage of infected lesions, follow up excision after 1 year might be beneficial for preventing recurrence.

• Journal of Chest Surgery
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• v.38 no.9 s.254
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• pp.633-636
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• 2005

Sixteen-day-old baby with severe Ebstein anomaly underwent emergency operation to relieve progressive hypoxia and congestive heart failure. Operative findings showed huge right atrium and atrialized right ventricle (aRV) with very small functional RV by distal displacemcent of tricuspid valve mechanism. We elected to perform modified Starness operation because biventricular repair was deemed unattainable. After pulmonary and tricuspid valves were primarily closed, aRV was obliterated with multiple sutures from RV apex to the base. Then a PTFE (Gore-Tex, USA) vascular graft was interposed between innominate artery and main pulmonary artery for systemic to pulmonary shunt. The patient was discharged uneventfully, and received bi-directional cavopulmonary shunt 6 months later.

• Journal of Chest Surgery
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• v.43 no.5
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• pp.518-521
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• 2010

A one-day-old baby was transferred for cyanosis and heart murmur. Echocardiographic evaluation revealed that he had severe neonatal Ebstein's anomaly (Carpentier type C), pulmonary atresia, and pulmonary circulation via patent ductus arteriosus. Because the wall of the atrialized right ventricle was very thin, showed decreased contractility, and the small right ventricle showed pulmonary atresia, we decided that a two-ventricular repair was impossible. When the patient was one-month-old, he underwent right atrium reduction-plasty, a right ventricular exclusion procedure (including atrialized right ventricle resection and functional right ventricle plication), and right modified Blalock-Taussig shunt. He was discharged without specific problems. He received a bidirectional cavopulmonary shunt successfully at 4 months later.

• Tuberculosis and Respiratory Diseases
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• v.72 no.6
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• pp.501-506
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• 2012

Congenital cystic adenomatoid malformation (CCAM) is an uncommon, nonhereditary anomaly caused by arrest of lung. Patients with CCAM may present with respiratory distress as newborns, or may remain asymptomatic until later in life. CCAM type I is rarely found in association with bronchial atresia (BA) in adults; we present such a case. Case: A 54-year-old female presented with chronic cough and blood-tinged sputum. Physical examination and laboratory tests were unremarkable. Chest radiographs and a CT scan of the chest showed multiple large air-filled cysts consistent with a CCAM in the right lower lobe, and an oval-shaped opacity in the distal right middle lobal bronchus. Based on the radiologic findings, right middle lobectomy and a medial basal segmentectomy of the right lower lobe were performed via a thoracotomy. These lesions were consistent with Stocker's Type I CCAM and BA in the different lobes.

• Journal of Chest Surgery
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• v.26 no.4
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• pp.282-293
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• 1993

From Sep. 1985 to Dec. 1992, total 1000 cases of open heart surgery [OHS] were performed in the department of Thoracic & Cardiovascular Surgery, Pusan Paik Hospital, College Of Medicine, Inje University.Among the total 1,000 cases of OHS, there were 823 cases with congenital heart diseases [CHD] and 177 cases with acquired heart diseases [AHD]. The age distribution was 9 days [4.0 kg] to 49 years in CHD and 11 to 64 years in AHD, In the 823 cases of CHD, there were 763 acyanotic cases and 60 cyanotic cases. The CHD cases consisted of 520 VSD [63.2 %], 177 ASD [21.5 %], 60 TOF[7.3 %], 27 PS [3.3 %], 17 ECD [2.1%], 7 Valsalva sinus rupture [0.9 %], 4 TGA [0.5 %], 3 Ebstein`s anomaly [0.4%], 3 DORV[0.4%], and others. The corrective operations were applied for congenital heart disease with the result of 2. 8 % hospital mortality. In the 177 AHD, 168 cases were valvular heart diseases, 7 cases were cardiac tumors and one LA thrombus and one annuloaortic ectasia. In the 168 valvular heart diseases, there were 115 single valve replacement cases [16 AVR, 99 MVR], 20 cases of double valve replacement[AVR & MVR], 15 cases of MVR with TVA, and 10 cases of AVR, MVR with TVA. The total implanted prosthetic valves were 199`. In MVR, 66 of St. Jude Medical valves, 78 ofCarpentier-Edward valves, and 5 of Ionescu-Shiley valves were used. In AVR, 38 of St. Jude Medical valves and 12 of Carpentier-Edward valves were used.The hospital operation mortality rate of congenital acyanotic, cyanotic, and acquired heart diseases were 1.6%, 18.3 % and 3.4% respectively. The overall mortality rate was 2.9 % [29/1000].

• Journal of Korean Neurosurgical Society
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• v.41 no.5
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• pp.343-346
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• 2007

Hypoplasia of the internal carotid artery is a rare congenital anomaly. Agenesis, aplasia, and hypoplasia of the internal carotid artery [ICA] are frequently associated with cerebral aneurysms in the circle of Willis. Authors report two cases with congenital hypoplasia of the ICA accompanying with the aneurysms. Transfemoral cerebral angiography [TFCA] in one patient identified nonvisualization of the left ICA. Bilateral anterior cerebral artery [ACA] and middle cerebral artery [MCA] were supplied from the right ICA accompanying with two aneurysms at anterior communicating artery [AcoA] and A1 portion of the left ACA. TFCA in another patient demonstrated hypoplastic left ICA and left ACA filled from the right ICA accompanying with AcoA aneurysm. Left MCA was filled from basilar artery via posterior communicating artery [PcoA]. Skull base computed tomography [CT] in two patients showed hypoplastic carotid canal. Authors performed direct aneurysmal neck clipping. Follow up CT angiography [CTA] at one year after surgery did not show regrowth or new development of the aneurysm. In patients with hypoplastic ICA, neurosurgeons should be aware of the possibility of development of the aneurysms, presumably because of hemodynamic process. Direct aneurysmal neck clipping is a good treatment modality. After operation, regular CTA, magnetic resonance angiography [MRA] or TFCA is needed to find progressive lesion and to prevent cerebrovascular attack [CVA].

• Tuberculosis and Respiratory Diseases
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• v.66 no.5
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• pp.385-389
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• 2009

Congenital cystic adenomatoid malformation (CCAM), which is classified into five types according to size and bronchial invasion, is a rare type of developmental anomaly of the lung. CCAM is occasionally accompanied by malignancy, such as bronchioloalveolar carcinoma (BAC) or rhabdomyosarcoma. As defined by the WHO, atypical adenomatous hyperplasia (AAH) is a non-invasive spread of atypical epithelial cells in single rows along the alveolar wall, within a lesion that is usually less than 5 mm in diameter. AAH was also regarded as a pre-invasive neoplasia, especially associated with BAC and adenocarcinoma. We report a case of type II CCAM with AAH in adults, with a review of the references.

• Journal of Chest Surgery
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• v.38 no.11 s.256
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• pp.780-782
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• 2005

The quadricuspid aortic valve is a rare congenital cardiac morphology. In regard to the hemodynamics of the quadricuspid aortic valve, the regurgitation is most common, the regurgitation accompanying the stenosis or pure stenosis are rare. We report hear a case with quadricuspid aortic valve disease which has been known to be extremely rare.