• Childhood Kidney Diseases
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• v.22 no.1
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• pp.12-16
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• 2018

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital malformation syndrome that is characterized by a triad of uterine didelphys, blind hemivagina, and ipsilateral renal agenesis. There is a wide variety of phenotypic presentation which is recognized as a spectrum of disease rather than a separate entity. The exact incidence and pathogenesis of HWW syndrome are yet to be investigated. While this disease typically involves adolescent girls who present with abdominal pain or a pelvic mass that is secondary to hematocolpos, nowadays, a majority of potential patients with HWW are being prenatally screened for renal anomalies. Therefore, it is recommended to search for uterovaginal anomalies whenever a multicystic dysplastic kidney or the absence of a kidney is noted in a newborn female, and the role of pediatric nephrologists has become ever more important for early recognition of the disease.

• Journal of Chest Surgery
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• v.19 no.2
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• pp.306-312
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• 1986

The development of extracardiac valved conduits for establishment of RV-PA continuity has permitted repair of previously uncorrectable congenital heart anomalies and has facilitated the repair of other complex lesions. But the distressing problem of neointimal peel formation with eventual conduit obstruction in patients with Dacron valved conduits has led to the need for premature replacement in many patients. Therefore in the absence of pulmonary hypertension, hypoplastic pulmonary arteries, significant right ventricular dysfunction or unrepaired tricuspid regurgitation, preference of a valveless conduit to a valved conduit gives excellent results and may diminish the need for late reoperation. In our Hanyang University Hospital, the Rastelli operation was performed for the repair of pulmonary atresia with a valveless Gore-Tex conduit. The patient was operated on with good result. The CVP after operation was 8-13 mmHg at POD #0 and 4-6 mmHg from POD #3-4. Postoperatively the patient was acyanotic and had improved physical capacity compared with his preoperative status.

• Journal of Chest Surgery
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• v.21 no.5
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• pp.905-910
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• 1988

Anomalous origin of the coronary artery from the pulmonary artery is a rare congenital coronary artery disease and the origin of the left coronary artery from the pulmonary artery represents the commonest form of these unusual lesions. Because of differences in symptomatology, clinical course and prognosis, this malformation has been divided into infant type[Bl-and-White-Garland syndrome] and adult type on the basis of the absence or presence of collateral circulation between the right and left coronary artery. The latter type has been reported relatively few cases. A 21-year-old male was admitted to the Yeungnam University Hospital, due to study of incidentally noticed heart murmur. At that time he was asymptomatic and past medical history was noncontributory. Chest roentgenogram was within normal limit and electrocardiogram was consistent with hypertrophy of left ventricle. Echocardiogram and aortogram demonstrated markedly dilated and tortuous right coronary artery and anomalous origin of the left coronary artery from the pulmonary artery. To prevent arteriosclerosis, progressive myocardial infarction, infection and aneurysmal rupture, Takeuchi operation which establish a two coronary system by transpulmonary arterial reconnection of the anomalous left coronary artery was done. Postoperative course was uneventful.

• Journal of Chest Surgery
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• v.18 no.3
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• pp.428-435
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• 1985

Tetralogy of Fallot associated with a single pulmonary artery is a rare cardiac anomaly. In previously reported cases, left pulmonary arteries were absent except 2 cases. Congenital absence of the pulmonary valve is a rare anomaly too. In the majority of cases, this lesion is associated with TOF. TOF associated with a single pulmonary artery and absent pulmonary valve is a very rare anomaly and only less than 20 cases were reported in the literatures. We have operated on one patient with TOF associated with absent right pulmonary artery and rudimentary pulmonary valve, a variant of absent pulmonary valve, and report this case with review of the literatures.

• Neonatal Medicine
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• v.16 no.1
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• pp.81-84
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• 2009

Septo-optic dysplasia is a rare congenital disorder characterized by the absence of the septum pallucidum, hypoplasia of the optic chiasma and nerves, and various types of hypothalamic-pituitary dysfunction. Schizencephaly is an abnormal cleft in the brain, lined with gray matter which may communicate with the ventricular system. Septo-optic dysplasia with schizencephaly is associated with endocrinologic disorders, visual impairment, mental retardation, and seizures. We report a case of septo-optic dysplasia with schizencephaly which was diagnosed in the early neonatal period.

• Journal of Korean Neurosurgical Society
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• v.55 no.6
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• pp.375-378
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• 2014

Hydranencephaly is a rare congenital disease defined as an absence of cerebral hemispheres with an intact thalamus, brain stem, and cerebellum. Generally, patients with hydranencephaly require cerebro-spinal fluid diversion due to progressive hydrocephalus. An excellent view of the choroid plexus and advancement of the neurosurgical endoscope were possible, leading to easy coagulation of the choroid plexus to balance cerebro-spinal fluid production without the use of a shunt device. We present this rare case and good treatment outcome from endoscopic coagulation of the choroid plexus.

• The korean journal of orthodontics
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• v.42 no.2
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• pp.94-98
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• 2012

In this report, we present the case of a girl with delayed odontogenesis of a lower second premolar for which she was followed up for 8.5 years. Congenital absence of permanent mandibular second premolars was observed at the initial radiographic examination at 8 years and 1 month. One year later, during the treatment period, an unexpected odontogenesis of a right second premolar was diagnosed on follow-up radiography. The original treatment plan was revised and a new plan was successfully implemented. This unusual case showed that the orthodontist's clinical philosophy must be flexible because unexpected situations can arise, especially when treating growing patients.

• The Journal of the Korean dental association
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• v.11 no.5
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• pp.313-316
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• 1973

970 children visited attached hospital, S.N.U, were taken orthopantomograph. Of those children, there were 14 patients who have primary fused teeth. The author observed those cases and got following results. 1) Incidence of primary fused teeth was higher in female. 2) In the order of frequency, fused teeth of mandibular right primary lateral incisor and canine were 9 and that of mandibular left primary lateral incisor and canine were 4, and there was only one case in maxillary. 3) 13 cases showed the congenital absence of lateral incisor of the corresponding permanent teeth. 4) Incidence of fused teeth was 1.44%.

• ALGAE
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• v.28 no.2
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• pp.149-160
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• 2013

Six species of the genus Symphyocladia are currently recognized worldwide, all of which are reported to grow on the Korean coast. We described a new species Symphyocladia glabra sp. nov. based on morphological and molecular evidence. The new species is characterized by mostly an erect and broad thallus, the parallel arrangement of numerous apical cells, completely fused congenital cells, a corticated basal portion of faint midrib with six to eight pericentral cells, rhizoids cutting off from pericentral cells, and absent vegetative trichoblasts. The new species has morphological similarity to S. marchantioides and S. jejuinsula. However, S. glabra sp. nov. is distinguished from S. marchantioides by the corticated lower portion of the thallus and the absence of vegetative trichoblasts, and S. jejuinsula by number of pericentral cells and the fact that the thallus does not taper upward. The phylogeny of rbcL sequences indicated that S. glabra sp. nov. is definitely a separate entity within the genus Symphyocladia.

• Journal of Korean Neurosurgical Society
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• v.55 no.3
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• pp.156-159
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• 2014

Coarctation and occlusion of the aorta is a rare condition that typically presents with hypertension or cardiac failure. However, neuropathy or myelopathy may be the presenting features of the condition when an intraspinal subarachnoid hemorrhage has compressed the spinal cord causing ischemia. We report two cases of middle-aged males who developed acute non-traumatic paraplegia. Undiagnosed congenital abnormalities, such as aortic coarctation and occlusion, should be considered for patients presenting with nontraumatic paraplegia in the absence of other identifiable causes. Our cases suggest that spinal cord ischemia resulting from acute spinal subarachnoid hemorrhage and can cause paraplegia, and that clinicians must carefully examine patients presenting with nontraumatic paraplegia because misdiagnosis can delay initiation of the appropriate treatment.