• Journal of Korean Neurosurgical Society
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• v.58 no.5
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• pp.454-461
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• 2015

Objective : In this study, we aimed to investigate the underlying ethiological factors in chiari malformation (CM) type-I (CMI) via performing volumetric and morphometric length-angle measurements. Methods : A total of 66 individuals [33 patients (20-65 years) with CMI and 33 control subjects] were included in this study. In sagittal MR images, tonsillar herniation length and concurrent anomalies were evaluated. Supratentorial, infratentorial, and total intracranial volumes were measured using Cavalieri method. Various cranial distances and angles were used to evaluate the platybasia and posterior cranial fossa (PCF) development. Results : Tonsillar herniation length was measured $9.09{\pm}3.39mm$ below foramen magnum in CM group. Tonsillar herniation/concurrent syringomyelia, concavity/defect of clivus, herniation of bulbus and fourth ventricle, basilar invagination and craniovertebral junction abnormality rates were 30.3, 27, 18, 2, 3, and 3 percent, respectively. Absence of cisterna magna was encountered in 87.9% of the patients. Total, IT and ST volumes and distance between Chamberlain line and tip of dens axis, Klaus index, clivus length, distance between internal occipital protuberance and opisthion were significantly decreased in patient group. Also in patient group, it was found that Welcher basal angle/Boogard angle increased and tentorial slope angle decreased. Conclusion : Mean cranial volume and length-angle measurement values significantly decreased and there was a congenital abnormality association in nearly 81.5 percent of the CM cases. As a result, it was concluded that CM ethiology can be attributed to multifactorial causes. Moreover, congenital defects can also give rise to this condition.

• Clinical and Experimental Reproductive Medicine
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• v.30 no.2
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• pp.179-184
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• 2003

The Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome refers to a condition which presents as normal female secondary sex characteristics, normal external genitalia, congenital absence of the internal vagina, usually a rudimentary uterus in the form of bilateral noncanaliculated muscular buds, and normal tubes and ovaries with normal cytogenetic and endocrine evaluation, frequent association of renal, skeletal and other congenital anomalies. However, rarely, whole uterus or a segment of uterus may be present, but lacking a conduit to the introitus. If a partial endometial cavity is present in a segment of uterus, cyclic abdominal pain may be a complaint and furthermore endometiosis can be developed. Recently, we experienced a case of MRKH syndrome with the segments of uterus accompanying endometriosis in young woman. We present this case with a brief review of literatures.

• Journal of Veterinary Clinics
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• v.11 no.1
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• pp.437-470
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• 1994

These studies were performed to establish more effective surgical method for correcting congenital atresia coli in calves. Distal colon fistulation, proximal colon fistulation and colon anastomosis which could be applied for surgical repair of intestinal obstruction were carried out in goatlings experimentally and in calves with atresia coli clinically. After treatment of the animals clinical signs, blood component values, body weight gain, survival and necropsy findings were observed. In goatlings, clinical signs after colon fistulation were diarrhea, anorexia and vitality loss, and those of proximal colon fistulation group were severer than those of distal colon fistulation group. Surviviability after operation was 9~16 weeks in distal colon fistulation group, 2~8 weeks in proximal colon fistulation group, and 2-3 days in colocolic anastomosis group, respectively. There were no alterations in blood component values among experimental groups. Weight gain rates were 54.6% in distal colon fistulation group and 42.9% in proximal colon fistulation group compared with those of control. Necropsy findings observed in experimental groups were distension of intestine and excessive fluid in abomasum and intestine. Two calves with atresia coli died 1 day and 6 days after operation but one with colon fistulation survived more than two months. Preoperative clinical signs in calves with atresia coli were abdominal distension, progressive anorexia, no defecation and postoperative clinical signs wert diarrhea and periodic abdominal distension. After operation there were no alterations in blood component values between the calf with atresia coli and control calf. Weight gain rate of calf with atresia coli was similar to that of control but revealed the tendency to decrease from the 2nd month after operation. Necropsy findings observed in two calves with atresia coli were intestinal distension. intraluminal excessive fluid, blind atretic sac and absence of intrarectal content. It was concluded that proximal or distal colon fistulation could be available for surgical correction of congenital atresla coli and prognosis of distal colon fistulation was better than of proximal colon fistulation, but that extensive colocolic anastomosis could not be compatible with life in ruminants.

• Journal of Chest Surgery
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• v.30 no.1
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• pp.108-111
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• 1997

Congenital unilateral agenesis of pulmonary artery is a rare anomaly and it usually occurs in association with other cardiac anomaly such as tetralogy of Fallot. Since most patients affected by this defect without associated congenital cardiac anomaly or pulmonary Infection are asymptomatic, the clinical diagnosis of this anomal is first recognized by a characteristic pattern in chest roentgenogram taken as a routine checking; the findings on chest film consists of cardiac and mediastinal displacement, absence of the pulmonary arterial shadow, smaller hemithorax, and elevationof the hemidiaphragm, all on the affected side. We experienced rlght pulmonary artery agenesis in a 48 year-old male, who complained of massive hemoptysis, and it was diagnosed by digital subtraction pulmonary arteriogram and perfusin scan, and treated by right middle and lower lobe bi-lobectomy, and we report this case with the review of relevant literatures.

• Proceedings of the KOR-BRONCHOESO Conference
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• 1983.05a
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• pp.18.3-19
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• 1983

Choanal atresia, described first by Johann Roedere (1755) is an obstruction between the nasal cavity and nasopharyngeal vault, and the diagnosis and treatment were developed because of severity of the disease. Embryologically, incomplete development of olfactory pit, or failure of nasobuccal membrane to rupture, or persistent remaining of buccopharyngeal membrane, etc, all forms the congenital choanal atresia. And the acquired type was the result from syphilis or diphtheria with a resultant stenosing cicatrix and after the inexpert surgery and the trauma. Multiple abnormalities may be present particularly affecting the head, the heart and the alimentary system in the congenital type. The operative technique employed would depend upon the type of obstruction(whether membranous or bony), the age of the patient, and the presence or absence of any associated pathologic condition. Since Emmert (1853) first tried blind puncture of the atretic plate with the trocar, other surgical techniques have been introduced over the years for the correction of choanal atresia, which were the transnasal, transpalatal, transantral and the transseptal approach. Among them, transpalatal approach was proved to be a popular technique, that it provides a direct route, thus permitting an exact reconstruction and low restenosis rate. Recently, we have experienced two cases of choanal atresia and treated successfully with transpalatal approach, so authors report these cases with a review of the literature.

• 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 the korean academy of Pediatric Dentistry
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• v.34 no.4
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• pp.658-665
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• 2007

Oligodontia is defined as the congenital absence of six or more teeth in dentition, excluding the third molars. The prevalence of congenital missing teeth is about 1.6 to 9.6% of population and the prevalence of oligidontia is about 0.08 to 1.1%. The mandibular second premolar is the most frequently absent after the third molar, followed by the maxillary lateral incisor and upper second premolar. Females seem to be affected slightly more than males. Oligodontia may occur either in isolation, or as a part of a syndrome such as ectodermal dysplasia. Different causes are possible for oligodontia: physical obstruction or distruction of the dental lamina, space limitation, functional abnormalities of the dental epithelium, failure of induction of the underlying mesenchyme, chemotherapy, radiotherapy or genetic factor. Because oligodontia would result in esthetic and functional problems, such as facial asymmetry or occlusal disharmony, early diagnosis from clinical and radiographic examination was necessary. And appropriate treatment plan should be followed. This case report was about oral conditions and treatment of the oligodontia patients who have no specific systemic disease.

• Journal of the korean academy of Pediatric Dentistry
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• v.28 no.3
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• pp.369-373
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• 2001

The congenital missing of mandibular second premolars is among the common dental anomaly in children. When a second premolar is diagnosed as congenitally missed, we should consider many factors influencing the treatment plan such as patient's age, states of roots of 2nd primary molar, degree of crowding, skeletal growth pattern, facial profile, procumbency of the incisor and lower facial height, etc. The mineralization of the second premolars begins in the majority of cases at the age of $2\sim2\frac{1}{2}$ years, but this period varies more widely than those for other permanent teeth. Also, mandibular second premolars show the greatest variations in differentiation and calcification. For this reason, aplasia of this group of teeth cannot be diagnosed at early age and with the same degree of certainty. From the clinical studies with 2 cases and some literature review on late development of second premolars, it could be summarized as follows : 1. The 2 cases showed marked delay in the development of mandibular second premolars. 2. After the crypt formation, the speed of calcification seemed nearly normal, suggesting that the delay was due to differentiation rather than calcification. 3. When one is encountered with similar conditions, it would be desirable to consider the possibility of delayed tooth development.

• Neonatal Medicine
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• v.16 no.1
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• pp.85-88
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• 2009

The amniotic band syndrome is an uncommon congenital fetal abnormality, presumably due to fetal entanglement in strands of ruptured amniotic sac. The defects caused by this syndrome vary from simple limb defects to major visceral and craniofacial defects. We cared for a newborn infant with this syndrome, who showed constriction rings of the right leg and right axilla, right club foot, thoracic scoliosis, polydactyly, absence of the right thumb and aplasia cutis of the scalp. We report this case with a brief review of the literature.

• Journal of Chest Surgery
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• v.48 no.6
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• pp.415-418
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• 2015

Right aortic arch with isolation of the left subclavian artery is a rare anomaly. The incidence of bilateral ductus arteriosus is sporadic, and a right aortic arch with isolation of the left subclavian artery in association with bilateral ductus arteriosus is therefore extremely rare. Since the symptoms and signs of isolation of the left subclavian artery can include the absence or underdevelopment of the left arm, subclavian steal syndrome, or pulmonary artery steal syndrome, the proper therapeutic approach is controversial. We report a case in which surgical reconstruction was used to treat isolation of the left subclavian artery with right aortic arch in association with bilateral ductus arteriosus and a ventricular septal defect.