Mobius syndrome is generally considered to be a static disorder of congenital origin, and is manifested as unilateral or bilateral facial weakness and lateral gaze limitation. In most instances the syndrome occurs sporadically, but rarely familial cases have been reported. We report a family of three members with Mobius syndrome; a 7-year-old girl, a 6-year-old boy, and their 29-year-old mother. Each patient revealed facial diplegia, and unilateral or bilateral lateral rectus palsy. Brain MRI scans showed normal and there were no definite brainstem dysfunctions on electrophysiologic studies.
Abducens Nerve Diseases ; Adult ; Brain ; Brain Stem ; Child ; Female ; Humans ; Magnetic Resonance Imaging ; Male ; Mobius Syndrome* ; Mothers

Mobius syndrome is generally considered to be a static disorder of congenital origin, and is manifested as unilateral or bilateral facial weakness and lateral gaze limitation. In most instances the syndrome occurs sporadically, but rarely familial cases have been reported. We report a family of three members with Mobius syndrome; a 7-year-old girl, a 6-year-old boy, and their 29-year-old mother. Each patient revealed facial diplegia, and unilateral or bilateral lateral rectus palsy. Brain MRI scans showed normal and there were no definite brainstem dysfunctions on electrophysiologic studies.
Abducens Nerve Diseases ; Adult ; Brain ; Brain Stem ; Child ; Female ; Humans ; Magnetic Resonance Imaging ; Male ; Mobius Syndrome* ; Mothers

This study was desinged for compare the efficacy of ephedrine infusion that of crystalloid administration in reducing the incidence of hypotension during spinal anesthesia. Sixty ASA 1 and 2 patients scheduled for transurethral prostatic resection and lower extremity surgery under spinal anesthesia were ramdomly allocated to receive either 15 ml/kg crystalloid (crystalloid group) or an ephedrine infusion (infusion group). Spinal anesthesia was performed using 12-14 mg of 0.4% tetracaine. Analgesic sensory level was T10 in both groups, Thirty patients in the crystalloid group received crystalloid solution (Ringer'lactate solution, 15 ml/kg) within 20 min prior to induction of spinal anesthesia. Thirty patients in the infusion group immediately after induction spinal anesthesia received ephedrine infusion at a rate 5 mg/ min for the first 2 min and then 1 mg/min for the next 18 min. Cardiovascular responses (systolic blood pressure and heart rate) and complications (hypotension, nausea and vomiting) after induction of spinal anesthesia were compared and results were as follows; 1) The incidence of hypotension was ll/30 (36%) in the crystalloid group and 3/30 (10%) in the infusion group (p<0.001). 2) Systolic blood pressure after induction of spinal anesthesia in the crystalloid group significantly decreased from 5 min to 20 min as compared to the infusion group (p<0.05). But, systolic blood pressure did not decrease siginificantly until 9 min after spinal anesthesia in the infusion group. 3) The mean heart rate after induction of spinal anesthesia in the crystalloid group significantly increased at 1 min as compared to infusion group; thereafter, there was no significant difference between the two groups. 4) The incidence of nausea and vomiting was 1/30 in the crystalloid group and 0/30 in the infusion group; The difference between the two groups was not significant. 5) Reactive hypertension or tachycardia did not occur in either group. Considering above results, we conclude that a prophylactic ephedrine infusion is effective for minimizing and managing hypotension associated with spinal anesthesia.
Anesthesia, Spinal* ; Blood Pressure ; Ephedrine* ; Heart ; Heart Rate ; Humans ; Hypertension ; Hypotension* ; Incidence ; Lower Extremity ; Nausea ; Tachycardia ; Tetracaine ; Vomiting

Congenital coronary arteriovenous fistula is a rare condition which is an abnormal communication of the coronary artery with the right ventricle, right atrium, left atrium or left ventricle. Coronary artery aneurysm is an uncommon disease which is defined as coronary dilatation which exceeds the diameter of normal adjacent segments or the diameter of the patient's largest coronary vessel by 1.5 times. In young ages, symptoms are unusual, but significant symptoms and complications such as congestive heart failure, subacute bacterial endocarditis, coronary steal syndrome, aneurysm formation, rupture, and pulmonary hypertension may appear among the older age group. We report a case of giant aneurysm of a congenital coronary arteriovenous fistula between left co-ronary artery and right ventricular outflow tract with significant left to right shunt confirmed in a 84-year old female with a brief review of literature.
Aged, 80 and over ; Aneurysm* ; Arteries ; Arteriovenous Fistula* ; Coronary Aneurysm ; Coronary Vessels* ; Dilatation ; Endocarditis, Subacute Bacterial ; Female ; Heart Atria ; Heart Failure ; Heart Ventricles ; Humans ; Hypertension, Pulmonary ; Rupture

BACKGROUND: Infants are very different from children and adults in the point of anatomy, physiology, pharmacology and psychology. The mortality rate of infant anesthesia is higher than that of child and adult. So that this study is to analyze the infant's operation and to recognize the tendency and problems of infant's anesthesia and also to improve the outcome. METHODS: A retrospective analysis was performed on 1436 infants who had operations from april 1987 to february 1995 according to age, sex, department, disease, operation time, postoperative complications, mortality rate and anesthetic method. RESULTS: Total number of operation tend to increase annually. The male to female ratio was 67.5% to 32.5%. The distribution of patients by department was 60.6% of general surgery, 21.0% of plastic surgery and small percentage of other department. The distribution of disease was the following : inguinal hernia - the most common; cleft lip and palate, intussusception and others. The postoperative complications developed in 98 of 1436. The most common complication was the respiratory problems, the others were sepsis, electrolyte unbalance, gastrointestinal and CNS problems. The total mortality rate was 3.6%. The emergency operation case was 17.9%. CONCLUSIONS: The most common distribution of age was neonate and the most commm department was general surgery and the most common disease was inguinal hernia. The most common postoperative complication was respiratory problems and the second was infection and sepsis. The mortality rate in preterm infant, neonate, emergency operation and long duration operation was higher than total motality rate.
Adult ; Anesthesia* ; Child ; Cleft Lip ; Emergencies ; Female ; Hernia ; Hernia, Inguinal ; Humans ; Infant ; Infant, Newborn ; Infant, Premature ; Intussusception ; Male ; Mortality ; Palate ; Pharmacology ; Physiology ; Postoperative Complications ; Psychology ; Retrospective Studies* ; Sepsis ; Surgery, Plastic

BACKGROUND: Infants are very different from children and adults in the point of anatomy, physiology, pharmacology and psychology. The mortality rate of infant anesthesia is higher than that of child and adult. So that this study is to analyze the infant's operation and to recognize the tendency and problems of infant's anesthesia and also to improve the outcome. METHODS: A retrospective analysis was performed on 1436 infants who had operations from april 1987 to february 1995 according to age, sex, department, disease, operation time, postoperative complications, mortality rate and anesthetic method. RESULTS: Total number of operation tend to increase annually. The male to female ratio was 67.5% to 32.5%. The distribution of patients by department was 60.6% of general surgery, 21.0% of plastic surgery and small percentage of other department. The distribution of disease was the following : inguinal hernia - the most common; cleft lip and palate, intussusception and others. The postoperative complications developed in 98 of 1436. The most common complication was the respiratory problems, the others were sepsis, electrolyte unbalance, gastrointestinal and CNS problems. The total mortality rate was 3.6%. The emergency operation case was 17.9%. CONCLUSIONS: The most common distribution of age was neonate and the most commm department was general surgery and the most common disease was inguinal hernia. The most common postoperative complication was respiratory problems and the second was infection and sepsis. The mortality rate in preterm infant, neonate, emergency operation and long duration operation was higher than total motality rate.
Adult ; Anesthesia* ; Child ; Cleft Lip ; Emergencies ; Female ; Hernia ; Hernia, Inguinal ; Humans ; Infant ; Infant, Newborn ; Infant, Premature ; Intussusception ; Male ; Mortality ; Palate ; Pharmacology ; Physiology ; Postoperative Complications ; Psychology ; Retrospective Studies* ; Sepsis ; Surgery, Plastic

The pathophysiology, clinical presentation and prognosis of left ventricular obstruction present an important cardiological problem. Various anatomical and functional abnormality can cause this phenomenon. Rarely, left ventricular outflow obstruction can result after mitral valve surgery. We experienced a case of left ventricular outflow obdtruction 13 years after mitral valve replavement. The diagnosis was made using two-dimensinal Doppler echocardiography and confirmed by cardiac catheterization. The pressure gradient across the left ventricular outflow obstruction was 96mmHg. A second mitral valve replacement was performed. Because severe fibrosis, pannis around the prosthetic mitral valve and a subaortic web were detected during the operation, the subaortic web was removes.
Cardiac Catheterization ; Cardiac Catheters ; Diagnosis ; Echocardiography, Doppler ; Fibrosis ; Mitral Valve* ; Prognosis ; Ventricular Outflow Obstruction*

BACKGROUND: Left ventricular hypertrophy is major cardiovascular risk factor for sudden death, acute myocardial infarction and congestive heart failure. Antihypertensive treatment able to normalize blood pressure and regression of left ventricular mass would also favorabley affect coronary flow reserve and cardiovascular mortality. OBJECT: This study was designed to explore changes of left venrtricular mass, echocardiographic datas such as interventricular septal thickness in diastole, posterior wall thickness in diastole, left ventricular end diastolic dimension, relative wall thickness, mainmorphologic change of LV, and diastolic function after antihypertensive treatment. METHODS: From May 1988 to Agust 1997, in 41 patients(14 men, 27 women) with estaiblished essential hypertension aged 35 to 78(mean 56+/-13) year were studied. We obtained the basal echocardiography and follow up echocardiography after treatment. RESULT: The results were as followings: 1) Baseline blood pressure was 157/92mmHg and fell to 137/81mmHg(p<0.001), and LV mass were reduced from 133.9g/mg2 to 132.9g/m2 without statistical significance. 2) Most of the patients(48.8%) were remained increased LV mass and only 12% of the patients were revert to normal LV mass. 3) Most of the patients remained same LV morphology after antihypertensive treatment. 4) Normalization of LV diastolic dysfunction was not observed after antihypertensive treatment. CONCLUSION: Most of the patients were remained increased LV mass, same morphology, and relaxation abnormality of LV after antihypertensive treatments. For analysis of our result, follow up studies are needed about regression of LV mass, remodeling of LV, diastolic function after antihypertensive treatment.
Blood Pressure ; Death, Sudden ; Diastole ; Echocardiography ; Follow-Up Studies ; Heart Failure ; Humans ; Hypertension ; Hypertrophy, Left Ventricular ; Male ; Mortality ; Myocardial Infarction ; Relaxation ; Risk Factors

Owing to the complexity of the deviated structures and the septal deformities accompanying the external nasal deformities, the complete correction of the deviated nose is regarded as a difficult one. The approach to the nasal bone and osteotomy in classic corrective rhinoplasty is almost blind technique, where the results depends on the feeling by surgeon's hand. To overcome these drawback, endoscopic-assisted corrective rhinoplasty and septoplasty performed for 8 cases of deviated nose between January 1996 and May 1997. Average follow-up period was 10 months. All patients were evaluated by symmetrical nasal pyramid, recurrence of the bony deflection and septal deviation. The postoperative courses were satisfactory in most of cases with few complications. It appears that endoscopic control during corrective rhinoplasty and septoplasty is a big step toward obtaining better results in bony and cartilage resection with extreme precision under monitor control and magnification. This technique is not an open approach but permits one to see more of the nasal skeleton and bony septum what is causing deformity, and the immediate effect of the corrective measures used. The use of endoscope in corrective rhinoplasty will provide expanded field of vision, direct manipulation of lesions, and better aesthetic an6 functional results.
Cartilage ; Congenital Abnormalities ; Endoscopes ; Follow-Up Studies ; Hand ; Humans ; Nasal Bone ; Nose* ; Osteotomy ; Recurrence ; Rhinoplasty ; Skeleton

Owing to the complexity of the deviated structures and the septal deformities accompanying the external nasal deformities, the complete correction of the deviated nose is regarded as a difficult one. The approach to the nasal bone and osteotomy in classic corrective rhinoplasty is almost blind technique, where the results depends on the feeling by surgeon's hand. To overcome these drawback, endoscopic-assisted corrective rhinoplasty and septoplasty performed for 8 cases of deviated nose between January 1996 and May 1997. Average follow-up period was 10 months. All patients were evaluated by symmetrical nasal pyramid, recurrence of the bony deflection and septal deviation. The postoperative courses were satisfactory in most of cases with few complications. It appears that endoscopic control during corrective rhinoplasty and septoplasty is a big step toward obtaining better results in bony and cartilage resection with extreme precision under monitor control and magnification. This technique is not an open approach but permits one to see more of the nasal skeleton and bony septum what is causing deformity, and the immediate effect of the corrective measures used. The use of endoscope in corrective rhinoplasty will provide expanded field of vision, direct manipulation of lesions, and better aesthetic an6 functional results.
Cartilage ; Congenital Abnormalities ; Endoscopes ; Follow-Up Studies ; Hand ; Humans ; Nasal Bone ; Nose* ; Osteotomy ; Recurrence ; Rhinoplasty ; Skeleton