• Clinical and Experimental Pediatrics
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• v.52 no.2
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• pp.261-264
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• 2009

Duplication cysts are rare congenital malformations, that may be detected anywhere along the alimentary tract, and they may communicate with the intestinal tract. Cystic duplication of the cecum is especially rare. About 80% of these cases are detected in the first 2 years of life as a result of an acute intestinal obstruction, which manifests as vomiting, recurrent abdominal pain, recurrent gastrointestinal bleeding and constipation. We report a case of intestinal obstruction secondary to a duplication cyst of the cecum in a neonate. The patient underwent surgery and was diagnosed subsequently, and is presently healthy.

• Advances in pediatric surgery
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• v.7 no.1
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• pp.7-14
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• 2001

Congenital anomalies of the head and neck region such as preauricular sinus and skin tag, thyroglossal duct cyst, branchial anomaly, cystic hygroma and dermoid cyst are common in pediatric population. It is important for pediatricians and pediatric surgeons to be familiar with the embryology and the anatomical characteristic of these lesions in order to diagnose and treat them properly. Three hundred and nineteen patients with congenital head and neck anomalies treated at Hanyang University Hospital between 1980 and 1999 were reviewed to determine the relative frequency of the anomalies and to analyze the method of management. Eight-four (25.1 %) of 335 lesions were preauricular sinus and skin tag, 81 (24.2 %) were thyroglossal duct cyst, 81 (24.2 %) branchial anomaly, 58 (17.3 %) cystic hygroma and 31 were (9.2 %) dermoid cyst. The male-to-female ratio was 1.4:1. Thyroglossal duct cyst most commonly present at 3-5years, however branchial anomalies commonly are diagnosed in children younger than 1 year. Preauricular sinus showed familial tendency in three patients and was bilateral is 33.8 %. Most head and neck anomalies in children have specific clinical and anatomical characterics. A careful history and physical examination is very useful for diagnosis and proper management. Experienced pediatric surgeons should do the initial surgery since the recurrence rate after incomplete surgical excision can be high.

• Proceedings of the KOR-BRONCHOESO Conference
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• 1987.05a
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• pp.13.1-13
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• 1987

저자들은 1984년 12월 17일부터 1987년 2월 26일까지 경희대학교 의과대학 부속병원 이비인후과 및 흉부외과에서 경험한 9례의 기관협착증환자를 대상으로 임상적 고찰을 하여 다음과 같은 결과를 얻었다. 1) 연령 및 성별분포는 여자 3례, 남자 6례이었고, 10세이하 3례, 10대 2례, 20대 2례, 40대 이상이 2례 이었다. 2) 원인은 지속적 기관내삽입관에 의한 경우가 4례이었고 1례는 선천성 혈관기형인 double aortic arch에 의해 기관이 눌리어 기도협착증상이 있었던 경우이었다. 상기관절개술에 의한 경우가 2례, 경부외상 1례, 기관내 종양 2례이었다. 3) 협착부위의 길이는 1.5cm에서 2cm까지가 4례로 가장 많았고 3cm이내가 2례, 4cm이내 1례, 6cm이내 1례이었다. 4) 치료는 보존적인 방법으로 내시경하에서 육아조직 및 반흔조직을 laser를 이용하여 제거한 후 silastic stent 혹은 Montgomery T-tube 삽입후 4주에서 6주후 제거하여 치료한 경우가 2례이었고 협착부위절제 및 단단문합술을 시행한 경우가 6례이었다. 1례에서는 aortic arch division을 시행하였다. 5) 예후는 9례중 단단문합술을 시행한 6례 그리고 보존적 방법으로 laser를 이용한 육아조직의 제거 및 지지물삽입을 시행한 3례에서 모두 현재까지 재발은 보이지 않고 있다. 6) 기관협착의 길이가 1.5cm에서 4cm까지의 경우에는 supralaryngeal release procedure없이 단단문합술을 시행하였고 협착길이가 6cm인 1례에서는 supralaryngeal release를 하여 tension없이 문합술을 시행할 수 있었다.

• Archives of Plastic Surgery
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• v.34 no.4
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• pp.498-500
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• 2007

Purpose: The oral teratoma is found approximately in live birth at the rate from 1 : 35,000 to 1 : 200,000. In a review of literature 16 cases of midline teratoma with cleft palate were reported. We report a case of congenital palatal teratoma with cleft palate in a 1-year-old girl. Methods: A 1-year-old girl was admitted our institution for the closure of cleft palate. On the intraoperative findings there was $4{\times}1{\times}0.5cm$ sized hairy soft mass at the midline and complete cleft palate. We did incisional biopsy intraoperatively and its pathology revealed heterotopic brain tissue. The excision of remaining mass and palatoplasty with Sommerlad's method were performed. The final pathology of the mass was mature cystic teratoma. Results: After the operation there were neither recurrence nor oronasal regurgitation. Conclusion: We report for one patient with congenital palatal teratoma associated with cleft palate and obtained an excellent result.

• Journal of Chest Surgery
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• v.19 no.1
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• pp.116-121
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• 1986

Patients over 15 years of age who have undergone a surgical correction of a congenital cardiac malformation during period of January 1958 through January 1986 have been reviewed. During this period there were 3957 congenital cardiac lesions consisting of 2712 acyanotic and 1245 cyanotic cases. Among them, a total of 725 adults [18.3%] with a variety of congenital heart lesions, 548 acyanotic group and 177 cyanotic group were operated on. 372 patients were male and 252 patients were female. There were 280 patients under 20 years of age, 206 between 20-24 years, 102 between 25-29 years, 48 between 30-34 and 89 over 35 years. The most common defects were atrial septal defect which accounted for 207 cases [28.6%] and other common anomalies were VSD [140 cases, 19.3%], TOF [136 cases, 18.6%], PDA [120 cases, 16.6%] and PS [33 cases, 4.6%] in order of incidence. Overall operative mortality for this series was 3.6% [1.8% of a cyanotic group and 9.0% of cyanotic group] compared with 2.8% of total cases of congenital heart disease [acyanotic group 1.1% and cyanotic group 6.5%]. This reviewed series reveals the incidence of operable congenital heart defects appearing in any adult life and demonstrates that surgical repair can be accomplished with a satisfactory low mortality rate.

• Journal of the Korean Society of Radiology
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• v.82 no.1
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• pp.207-211
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• 2021

Bronchogenic cysts are rare congenital anomalies that are most frequently found in the mediastinum along the tracheobronchial tree, especially in the posterior aspect of the superior mediastinum. Bronchogenic cysts have also been reported in intrapulmonary, intrapericardial, abdominal, and retroperitoneal locations. Herein, we report a case of a retroperitoneal bronchogenic cyst in the presacral space. The patient was diagnosed based on a post-operative histopathological examination.

• 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.

• Childhood Kidney Diseases
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• v.12 no.2
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• pp.262-266
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• 2008

Crossed renal ectopia is a congenital malformation in which both kidneys lie on the same side of the spine, usually side by side longitudinally. More often on the right side. Fusion of the two renal units is eight times more common than nonfusion. Although crossed renal ectopia is uncommon, this unusual entity must be considered in an infant when cystic mass in the abdomen or pelvis paticularly if no kidney can be found on the opposite side. In many cases of crossed fused ectopia with multicystic dysplastic kidney(MCDK), the diagnosis can be strongly suspected from the sonogram, and no other studies may be necessary. However, both intravenous urography and isotope renography is useful to assess the function of the crossed kidney. Crossed renal ectopia and MCDKs are associated with a greater incidence of ureteropelvic junction obstruction and reflux. So, screening voiding cystourethrography should be performed. Very few studies of MCDK in the setting of crossed fused ectopia have been reported. We have experienced a 3-year-old boy with crossed fused renal ectopia with multicystic dysplasia.

• Journal of the korean academy of Pediatric Dentistry
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• v.27 no.3
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• pp.388-393
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• 2000

Cleft lip and palate is one of the most common congenital defects in oro-maxillo-facial region. Because most patients undergo surgical repair in early life, the sagittal jaw relationships used to be deteriorated gradually from palate surgery up to adulthood. Also, the maxillary lateral incisor may be absent or atypical-shaped in the cleft site and may not erupt or erupt ectopically, so multidisciplinary dental cares are needed for cleft lip and palate patients. The effects of the cleft lip and alveolus seem to be limited to that part of the dentofacial complex that surrounds the cleft area. In the maxillary arch, the anterior part of the non cleft segment has a tendency to be rotated forward. On the other hand, the cleft segment has a tendency to rotated slightly medially ; hence, the tendency for canines to be edge-to-edge and sometimes in crossbite. Lip and alveolus surgery adequetely correct these problems, with little untoward effect on the skeletal maxillary-mandible relationships. In this report, the patient has a repaired lip and cleft alveolus on the left side with congenital missing on '62, '22, oronasal fistula, and skeletal class III malocclusion which is not affected by lip surgery. Dental treatments for this patient including orthodontic(space supervision, functional regulator in mixed dentition, fixed therapy in permanent dentition) and prosthodontic(removable obturator with key and keyway attachment and Konus crown) therapy were performed to improve the patient's functions and esthetics.

• Journal of Genetic Medicine
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• v.8 no.1
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• pp.71-75
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• 2011

A male infant was diagnosed with obstetric brachial plexus injury, congenital muscular torticollis and cleft palate 17 days after birth. His mother presented with gestational diabetes and premature rupture of membranes. Although it is possible that these three disorders arose independently, it is very likely that all three have the same etiologic cause, and we propose that a possible mechanism for this concurrence is related to maternal gestational diabetes. Maternal hyperglycemia mostly affects fetal structures deriving from the neural crest, including the palatine bone, and may have caused the cleft palate observed in this case. Gestational diabetes is also associated with increased frequency of large for gestational age infants and, by extension, with increased risk of birth injuries such as obstetric brachial plexus injury or congenital muscular torticollis associated with large for gestational age infants. Since the children of mothers with gestational diabetes are at increased risk for congenital defects such as cleft palate as well as being large for gestational age, precautions indicated for each respective disorder must be taken during prenatal testing and during birth. However, further studies of more cases are required to evaluate whether the concurrence of obstetric brachial plexus injury, congenital muscular torticollis and cleft palate in this case are complications specifically associated with gestational diabetes or just a simple coincidence.