Stereotactic radiosurgery, including gamma knife radiosurgery (GKS), can in rare cases result in de novo cavernous malformations (CMs). Here, we present a case of de novo CM induced by GKS following treatment of a cerebellar arteriovenous malformation (AVM). A 48-year-old woman was diagnosed with left unilateral Moyamoya disease. Conventional cerebral angiography also revealed an AVM in the left cerebellum. The patient underwent GKS using a 50% isodose of 15 Gy at the margin of the left cerebellar AVM. Magnetic resonance imaging (MRI) taken 3 years after GKS revealed small chronic hemorrhages with perilesional edema in the left cerebellum. Five years later, the lesions became aggravated, but were asymptomatic. Eight years following GKS, the patient was admitted complaining of headache and dizziness. Brain MRI revealed a 1.3cm hemosiderin deposit with an inner hyperintense nodular portion that was enhanced in the left cerebellum. An open craniotomy was performed and the mass was removed, from which pathological findings were compatible with those for CM. The patient recovered to the prehemorrhagic state. This case shows that De novo CMs can rarely develop after radiosurgery. Most CMs have been reported to develop following radiosurgery for brain tumors. As shown in this patient, CMs can also develop after radiosurgery for cerebellar AVM in adults.
Radiosurgery

Epikeratophakia is a newly developed refractive corneal surgery for the correction of aphakic vision. Two aphakic patients have received hyperopic epikeratophakia grafts. Three months postoperatively, two patients had visual acuities of 0.6 without spectacle overcorrection. Epikeratophakia appears to be effective for the correction of adult aphakia.
Adult* ; Aphakia* ; Epikeratophakia* ; Humans ; Transplants ; Visual Acuity

No abstract available.
Fetal Weight* ; Humans ; Infant* ; Infant, Low Birth Weight* ; Infant, Newborn ; Parturition* ; Weight Gain*

BACKGROUND: Multidrug-resistant tuberculosis(MDR-TB) in patients is mainly caused by acquired drug resistance, However, a small proportion of MDR-TB is caused by initial drug resistance(IDR), which may be somewhat different from acquired drug resistance. This study analyzed the clinical characteristics of IDR in MDR-TB patients to use the results as basic data in managing the disease. METHODS: A retrospective study of 30 IDR cases in MDR-TB patients from Jan. 1995 to Dec. 1998 was perormed. In order to analyzed the clinical charcteristics, the age, sex, family history, duration of negative conversion, number of resistant drugs, treatment regimens, duration of treatment, extent of disease and cavitary lesion on the chest X-ray was examined. In order too analyzed the level of improvement, the extent of the disease and cavitary lesion on the chest X-ray, tested by Wilcoxon signed rank sum test, and the disease free interval rate of 1-year and 4-year was examined using the Kaplan-Meier method. RESULTS: The mean age of the patients was 46.6 years and the sex ratio 1:1. Six(20%) patients had a family history. The mean negative conversin of the sputum AFB stain was 2.6 months. The number of resistant drugs was 7.6 and the number of used drugs 3.6. Twenty-three(67%) patients were treated for less than 12months and 28(93%) patients were treated with first-line drugs. The extent of the disease and the cavitary lesion on the chest X-ray improved after treatment(p<0.05). Among 13 patients who were followed up for 22.6 months, 2(15%) patients relapsed and the disease free interval rate of 1-year and 4-year was 85%. CONCLUSION: It is recommended that the duration of treatment of IDR in MDR-TB with first-line drugs be 9-12 months even if the extent of disease and cavitary lesion on the chest X-ray improves.
Drug Resistance* ; Humans ; Retrospective Studies ; Sex Ratio ; Sputum ; Thorax ; Tuberculosis, Multidrug-Resistant

Familial dysautonomia, first described by Riley and co-workers in 1949, is a congenital, heritable syndrome. It is transmitted by a recessive autosomal gene which is generally limited to persons of Jewish extraction; however, rare occurrences among non-Jewish Caucasians and in a black girl have been reported. An eight-year old Korean boy was admitted to Severance Hospital with bulbar conjunctival injection OU for 1 month and visual disturbance OS for 10 days. Examination revealed exfoliated epithelium in an area of about 3mm in diameter in the center of the left cornea, multiple punctate erosions and edema of the corneal epithelium. Yet, he experienced no discomfort, blepharospasm, nor photophobia. He had decreased corneal sensation, decreased lacrimation by Schirmer test and miosis was induced biJaterally by 0.25% pilocarpine (equivalent to 2.5% methacholine). The patient had decreased deep tendon reflexes and postural hypotension, and showed emotional lability, indifference to pain, and abnormal temperature control. He also had marked anorexia, swallowing difficulty, cyclic vomiting, abdominal pain, headache, intermittent vascular hypertension and one episode of convulsive seizure. He was positive to histamine intradermal injection test and had abnormal EEG. EMG was suggestive of some form of neuropathy. He was treated with toplca 0.5% chloramphenicol solution and 10% dextran solution alternatively q.2 hrs., terramycin ophthalmic ointment q.h.s. and bilateral patching; he also received 50,000 units of vitamin A for 10 days. He showed marked improvement of his ocular symptoms in 6 days. The above patient shows many of the essential features of the familial dysautonomia syndrome as outlined by Riley; however, he also lacks some of the most important features. Therefore, we feel that the patient has a case of some other type of autonomic dysfunction simulating Riley-Day svndrome.
Abdominal Pain ; Anorexia ; Blepharospasm ; Chloramphenicol ; Cornea ; Deglutition ; Dextrans ; Dysautonomia, Familial* ; Edema ; Electroencephalography ; Epithelium ; Epithelium, Corneal ; Female ; Headache ; Histamine ; Humans ; Hypertension ; Hypotension, Orthostatic ; Injections ; Injections, Intradermal ; Male ; Miosis ; Oxytetracycline ; Photophobia ; Pilocarpine ; Primary Dysautonomias* ; Reflex, Stretch ; Seizures ; Sensation ; Vitamin A ; Vomiting

Canavan disease(CD) is a rare autosomal recessive leukodystrophy caused by the deficiency of aspartoacylase and the accumulation in brain of N-acetylaspartate(NAA). CD has been reported mainly Ashkenazi Jews but also occurs in other ethnic groups. Usually it presents as early as the third month of life with megalencephaly, hypotonia later progressing to hypertonia, psychomotor and mental retardation, blindness, occasionally deafness and seizure. Diagnosis is based on the clinical feature, N-acetylaspartic aciduria, radiologic and pathologic findings. Histologically, the affected white matter shows extensive vacuolation and demyelination. There is no treatment for CD and the only prevention is through genetic counselling and prenatal diagnosis. We experienced a case of Canavan disease that was presented with hypotonia and developmental delay. Diagnosis was confirmed histologically. Radiologic findings are extensive high signal throughout the white matter on T2-weighted MRI and increased NAA peak and decreased choline peak of the white matter on MR spectroscopy.
Blindness ; Brain ; Canavan Disease* ; Choline ; Deafness ; Demyelinating Diseases ; Diagnosis ; Ethnic Groups ; Humans ; Intellectual Disability ; Jews ; Magnetic Resonance Imaging ; Magnetic Resonance Spectroscopy ; Muscle Hypotonia ; Prenatal Diagnosis ; Seizures

Chest wall osteoradionecrosis, one of the most serious complications of radiation therapy for breast cancer treatment, is usually treated by wide debridement followed by coverage with a well-vascularized flap. However, the extent of radiation-induced injury and limits in performing wide resection of the injured bones often present challenges in treatment. Herein, we present our experience treating chest wall osteoradionecrosis with a contralateral breast Y-V flap in an 81-year-old woman. She was diagnosed with chest wall osteoradionecrosis and had grade 3 ptotic breasts. Redundant contralateral breast tissue was used for reconstruction to cover the wound. The flap was elevated in the subfascial plane after an inverted-T incision was made in the lower pole and inframammary fold of the contralateral breast, while preserving the perforators of the left lateral thoracic artery. The flap was spread using the Y-V advancement fashion to cover the wound. The patient was discharged 2 weeks after surgery. At 19 months postoperation, there were no complications or recurrence. The patient was satisfied with the short recovery time and surgical results. The contralateral breast Y-V flap allows simple and quick reconstruction, potentially expanding the available treatment options and therefore increasing flexibility in choosing a treatment plan for patients.

Infantile lobar emphysema is an uncommon disease affecting newborns and infants with varying degree of respiratory distress, lobar overaeration, mediastinal shift and herniation. Although the etiology of the condition is most commonly idiopathic, there is a clear association with congenital heart disease, particularly in the presence of pulmonary hypertension due to left to right shunt. Sites of predilection are the left main bronchus, the left upper and right middle bronchi. This report describes a two-week-old boy who had right middle lobe emphysema with large ventricular septal defect. At first, patch closure of perimembranous ventricular septal defect was performed. Postoperatively, the patient required continuing assisted ventilation and the lobar emphysema was not improve. One week following the initial operation, right middle lobectomy was successfully performed and the patient was weaned from artificial ventilator on the 5th postoperative day. The patient was discharged with good general condition on the 45th postoperative day.
Bronchi ; Emphysema* ; Heart Defects, Congenital ; Heart Septal Defects, Ventricular* ; Humans ; Hypertension, Pulmonary ; Infant ; Infant, Newborn ; Male ; Ventilation ; Ventilators, Mechanical

BACKGROUND: Mitral valve replacement with chordal preservation in patients with mitral regurgitation has been proved to be beneficial for left ventricular function and for reduction of postoperative complication. However, in patients with mitral stenosis, the effectiveness of the technique is controversial. It is not easy to insert prosthetic valve without left ventricular outflow tract obstruction and prosthetic valve leaflet motion hinderance. MATERIAL AND METHOD: Five patients with mitral stenosis and seven patients with mitral stenoinsufficiency underwent mitral valve replacement with preservation of mitral subvalvular apparatus. Thickened and calcified leaflets are made thin by peeling off the thickened and calcified part. Commissurotomy was done and anterior leaflet was incised 2 mm apart from the annulus and then divided into two segments. Anterolateral and posteromedial segments including strut chordae, were reattached to mitral commissural area, respectively. RESULT: There was no evidence of prosthetic valve dysfunction, paravalvular leakage, left ventricular outflow tract obstruction, complications and operative or late deaths. CONCLUSION: We conclude that mitral vlave replacement with chordal preservation was safe and effective technique for the patients with mitral stenotic disease.
Humans ; Mitral Valve Insufficiency ; Mitral Valve Stenosis ; Mitral Valve* ; Postoperative Complications ; Ventricular Function, Left

BACKGROUND: Mitral valve replacement(MVR) with chordal preservation in patients with mitral vlavular disease has been proven to be beneficial for left ventricular function and for reduction of postoperative complication. MATERIAL AND METHOD: From January 1995 to July 1996, the early postoperative results of mitral valve replacement were compared between 20 patients who underwent chordae resection(classic MVR group) and 10 patients who underwent chordae preservation(preservation MVR group) in the Department of Thoracic and Cardiovascular Surgery, Chunbuk National University Hospital. RESULT: There was no significant difference between the two groups in age, sex, NYHA functional class, cardiothoracic ratio, echocardiographic finding, cardiopulmonary bypass time and aortic cross clamping time. The difference between preoperative and postoperative cardiothoracic ratio after 3 months was not statistically significant. At echocardiographic left ventricular evaluation, ejection fraction and fractional shortening decreased slightly in the preservation group then preoperative value (p=0.47, p=0.12), however, decreased significantly in the classic MVR group(p=0.03, p=0.04), and were statistically significant between the two groups(p=0.03, p=0.02). CONCLUSION: We conclude that MVR with chorda preservation seems to have a beneficial effect on postoperative left ventricular performance in mitral valve disease than the classic MVR.
Cardiopulmonary Bypass ; Constriction ; Echocardiography ; Humans ; Mitral Valve* ; Postoperative Complications ; Ventricular Function, Left