Phocomelia: The Clinical Manifestation, Classification and Surgical Treatment of Korean Patients with Phocomelia / 대한정형외과학회잡지

PURPOSE: Phocomelia is an extremely rare congenital anomaly of the upper extremity. There have been no clinical reports about phocomlia in Korea except for five birth reports. We present here the clinical features, classifications and surgical treatments of our phocomelia cases. MATERIALS AND METHODS: From January 1993 to August 2007, seven patients were diagnosed as having phocomelia in 9 upper extremities at our clinic. Surgical treatments were performed for five patients on their hand anomalies. We retrospectively reviewed the medical records and radiographs of our cases, and we tried to classify them by the previously suggested systems. We evaluated the functional improvement and measured the VAS scale for parental satisfaction with the operative outcomes. RESULTS: We could not find any problems during the fetal periods or any hereditary features. The bilaterally-affected patients also had deformities of the lower extremity, while the unilaterally-affected patients did not. We couldn't classify our cases according to the Frantz and O'Rahilly system. We found that the classifications suggested by Tytherleigh-Strong and Hooper (2003) and Goldfarb et al. (2005) could be promising alternatives for classification. One upper extremity was classified as type A, one as type B, and 7 as type C by Tytherleigh-Strong and Hooper's system. Using the Goldfarb's system, two upper extremities were classified as proximal radial longitudinal dysplasia, and seven were classified as proximal ulnar longitudinal dysplasia. Three patients who underwent pollicization showed opposition and tip pinch. Two patients who underwent syndactyly division could do lateral pinch. The VAS scale for parental satisfaction with the functional improvement averaged 8.2 postoperatively. CONCLUSION: The bilateral cases had different clinical features from unilateral ones. Phocomelia could not simply be classified by the Frantz and O'Rahilly system, and it may not be a true transverse intercalary deficiency. We could gain functional improvement after operations on the hand anomalies.

Serotonin(5-HT): can it be a cause in overactive bladder

PURPOSE: The purpose of this study is to investigate in vitro the effects of serotonin on the rat detrusor. In particular, this study examines what drugs inhibit the serotonin-induced detrusor contractions. MATERIALS AND METHODS: Isometric tension changes of isolated rat bladder muscle strips were recorded in an organ bath using a force transducer. Acute effects of serotonin (0.0001-0.01mM) were assessed on resting tension. Electrical field stimulation (EFS), bethanechol (0.0001-0.01mM), ATP (1-3mM) or KCl(63.5-254mM)-induced contractions using application in organ bath were compared with serotonin-induced contractions. In order to examine the action mechanism of serotonin-induced stimulation, EFS, bethanechol, ATP or KCl-induced contraction under serotonin (0.001mM) was assessed and serotonin (0.001 to 0.1mM) was cumulatively added to the organ bath following pre-incubation with propranolol, ketanserine, tropisetron, propiverine, sodium nitroprusside or doxazocin. RESULTS: There are two phases to the serotonin-induced responsean initial transient contraction and a prolonged tonic phase. Serotonin produced a reversible and dose-dependent contraction of the detrusor strips. Responses to bethanechol significantly increased with a concentration of 0.001mM serotonin (p<0.05). There was no effect on the responses to ATP, KCl, or EFS under 0.001mM serotonin. The 5-HT2 receptor is mainly responsible for serotonin-induced contractions of the detrusor (p<0.05), while the 5-HT1 receptor is partially responsible. Doxazocin and propiverine each significantly suppressed the responses to serotonin, while sodium nitroprusside and tropisetron each had no effect (p<0.05). CONCLUSIONS: Because the 5-HT2 antagonist blocked the effect of serotonin-induced bladder contractions and the stimulation of the adrenoreceptors, the 5-HT2 antagonist seems to improve lower urinary tract symptoms.

Clinical Evaluation of Female Patients with Acute Urinary Retention

PURPOSE: While acute urinary retention(AUR) secondary to bladder outlet obstruction in the male occurs commonly and rarely causes difficulty with diagnosis and treatment but AUR in female is an uncommon findings. This study was performed to evaluate AUR in possible causes by urodynamic study and analyzed the results of treatment. MATERIAL AND METHODS: We have retrospectively tried to evaluate the possible causes of AUR in 41 female patients. Urine analysis, urine culture, urodynamic study and selectively cystoscopy were performed in 41patients. We analysed the urodynamic feature respectively and classified into areflexia type, hyperreflexia type, detrusor hyperreflexia with impaired contractility(DHIC) type and normal finding. RESULTS: The mean age of patients was 57.9 years. Major causes of AUR were CNS lesion(13 cases, 31.7%), diabetes mellitus(6 cases, 15.3%) and spinal lesion(4 cases, 10.2%). Urethral catheterization was employed as initial treatment in 32 cases(78.0%). Catheter was removed after mean 5.2 days and selective urodynamic study was done in 36 cases. Urodynamically, there were hyperreflexia type 16 cases(44.4%), areflexic type 9 cases(25%), DHIC type 6 case(16.7%), normal finding 5 cases(13.9%). The treatments of areflexic type included intermittent catheterization(IC) in 4 cases (44.4%), urethral catheterization in 3 cases (33.3%), medical therapy in 2 cases(22.2%). The treatments of hyperreflexia type included IC in 4 cases(25%), urethral catheterization in 1 case(6.3%) and medical therapy in 11 cases(68.8%). The treatments of DHIC type included IC in 2 cases(5.5%), urethral catheterization in 1 case(2.8%), medical therapy in 2 cases(5.5%) and ileal conduit in 1 case(2.8%). CONCLUSIONS: Causes of female AUR were commonly considered the functional urinary retention and rarely organic urinary retention, but there were various causes. Our study indicated the need to consider the gender and urodynamic finding on the evaluation and treatment of AUR.

Urodynamic Evaluation of Patients with Intracranial Lesions according to Lesion Site

PURPOSE: Typical urodynamic findings in patients with intracranial lesion is detrusor hyperreflexia with coordinated sphincter activity. However the findings of urodynamic study may be different from this typical findings, because neurological net effect on voiding function should be variable according to the degree and site of intracranial lesions, the presence of underlying disease and adequacy of initial management of voiding problems from onset of accident. This study was performed to evaluate the type of voiding dysfunction on the anatomic lesion of brain mainly. MATERIAL AND METHODS: In this study, we have retrospectively tried to evaluate the urodynamic findings in 108 patients who had intracranial lesion. Computerized tomography or magnetic resonance imaging was performed to localize the intracranial lesions. RESULTS: Mean patient age was 59.4 years(range 10 to 87). In 108 cases, cerebral infarct was in 47 cases, 29 cases of intracranial hemorrhage, 8 cases of Parkinsonism and 24 cases of other diseases. Patient were grouped according to the presenting voiding complaints, such as 46 cases of irritative symptom and 62 cases of obstructive symptom. Underlying diseases such as diabetic mellitus were 21 cases, which showed hyperreflexia or areflexia. In urodynamic study, detrusor hyperreflexia was noted in 64% of patients, detrusor hyperreflexia with impaired contractility in 14.8% and detrusor areflexia in 18.5%. Patients with detrusor sphincter dyssynergia(DSD) were 14 cases, but only 2 cases had true DSD. In 108 cases according to brain lesion, lesion sites were on basal ganglia in 23 cases and on basal ganglia and thalamus in the 22 cases. Urodynamic study revealed 14 cases of detrusor hyperreflexia in patients with basal ganglia lesion and 14 cases of detrusor hyperreflexia in patients with basal ganglia and thalamus lesion. The involvement of frontal lobe or diffuse lesion showed detrusor areflexia and others showed detrusor hyperreflexia mainly. Most of treatment method was medical therapy(63.9%). CONCLUSION: Presenting symptoms did not predict the urodynamic findings and treatment. Underlying diseases may exacerbate the symptom of the intracranial lesion induced voiding dysfunction. Our study indicates the need to consider bladder outlet obstruction as a cause of urinary retention in old men who suffered from a stroke. On the basis of this study, it is assumed that the effect of the basal ganglia on micturition is inhibitory in nature. Evaluation of underlying diseases and intracranial lesion seems to be helpful in making the protocol of treatment of voiding dysfunction with intracranial lesion.

Cardiac Metastasis of Malignant Melanoma: A Case Report / 대한흉부외과학회지

We report a case of a resection of very large intracavitary metastatic malanoma causing obstruction of the right ventricular inflow and outflow tract of the heart. A 49-year-old woman with dyspnea and generalized edema was seen. Echocardiography reveal an intra cavitary mass occupying the entire right ventricle and pericardial effusion. The lesion was palliatively resected using a cardiopulmonary bypass and was confirmed as a malignant melanoma. The patient is alive and improved symptomatically 30days after the operation.

The Sympathetic Skin Responses after Thoracic Sympathicotomy for Patients with Palmar Hyperhidrosis / 대한흉부외과학회지

BACKGROUND: Thoracic sympathicotomy has been used safely and successfully to manage palmar hyperhidrosis. The preoperative and postoperative recording of Sympathetic Skin Responses(SSR) was performed for objective evaluation and follow-up of thoracic sympathicotomy in hyperhidrosis patients, and also for ascertaining the clinical usefullness of SSR. MATERIAL AND METHOD: The recording of SSR was performed on 15 patients suffering from palmar hyperhidrosis with Medelec Sapphire Plus electromyogragh before and after thoracic sympathicotomy. Eletrical stimuli on the right median nerve was made in patients in supine position and results were recorded on right and left palms with soles at the same time by 4 channels. Skin temperatures were also monitored simultaneously. T2,3 sympathicotomy was performed with VATS in every patients. SSR was done in 2 patients one month later. RESULT: Clinically, all patients had symptomatic improvement with satisfaction. Postoperative complication was small amount of residual pneumothorax in 5 patients but it was absorbed sponteneously. There was no recurrence during follow-up period and ten patients(66%) complained compensatory hyperhidrosis. After operation, SSR change was shown in every 15 patients. Abolition of SSR on both palms was achieved in 12 patients(80%) and on both soles in 6 patients. In the other 3 patients, the latencies were significantly delayed and the amplitudes were significantly reduced at both palms and soles. In two patients who were examined at one month later after operation, similar results with postoperative SSRs were shown. The skin temperature on preoperative both palm and sole were lower than normal temperature, and those on postoperative both palm and sole were increased. Those had statistical significance(p<0.05), and the temperature on the palm was increased higher that than on the sole. CONCLUSION: After thoracic sympathicotomy was performed on palmar hyperhidrosis patients, an increment of skin temperatures and SSR changes were achieved at both palms and soles of all patients. Palmar SSRs were completely abolished in 12 patients(80%), and similar results of postoperative SSRs were achieved. The recording of SSR may be useful to easily and objectively assess the completeness of sympathicotomy and the follow-up of recurrence in hyperhidrosis patients.

Surgical Management of Giant Aneurysm of Intracranial Artery Under Circulatory Arrest: one case report / 대한흉부외과학회지

Complete circulatory arrest with profound hypothermia has been an indispensable adjunct to the safe management of selected giant intracranial aneurysms. For the conduct of cardiopulmonary bypass, there are usually two kinds of methods, open and closed chest methods. We could manage one case of huge intracranial aneurysm that was successfully operated under circulatory arrest using the closed chest method, especially with percutaneous insertion of arterial and venous cannulas for cardiopulmonary bypass.

Clinical Result of the Patent Ductus Arteriosus in the Premature Infants / 대한흉부외과학회지

BACKGROUND: This study is to suggest the optimal method as a treatment for the patent ductus arteriosus in the premature infants. MATERIAL AND METHOD: Between April 1994 and April 1997, 45 premature infants with evidence of a hemodynamically significant patent ductus arteriosus associated with cardiopulmonary compromise underwent indomethacin therapy, surgical treatment, or both. Thirty-nine infants received indomethacin and twelve infants among them were surgically ligated because of indomethacin failure(5) or complications(7). Six infants, who weighed less than 1,500 gm at birth, were referred for primary surgical ligation because of contraindication to indomethacin therapy. RESULT: The failure rate of indomethacin therapy was 43%(17/39) and the complications(13/39, 33%) to the indomethacin were associated with a high morbidity and mortality. Among the infants who underwent ligation, there were no failures and complications related to the operation. This data suggests that in the premature neonate with a hemodynamically significant PDA, (1) indomethacin therapy is associated with a high failure rate and significant complications, (2) surgical duct closure is associated with minimal morbidity. CONCLUSION: Although the results of this study cannot suggest the optimal management for PDA in premature infants, primary surgical ligation may be considered. However, long-term studies will be needed to confirm this later.

Lecompte Procedure in Complex Congenital Heart Diseases / 대한흉부외과학회지

BACKGROUND: This study is to evaluate the effectiveness and application of Lecompte procedure as a treatment for various complex cardiac anomalies with pulmonary outflow tract obstruction. Methods: Between July 1988 and December 1997, 44 patients underwent Lecompte procedure in Seoul National University Children's Hospital. The male to female ratio was 24 to 20 and the mean age was 29.2 months (range, 3 to 83). Of these patients, 28 (63.6%) had transposition of great arteries with ventricular septal defect and pulmonary stenosis (or pulmonary atresia), 14 (31.8%) had double outlet right ventricle with pulmonary stenosis (or pulmonary atresia), and so on. The principles of the technique are 1) extension of the ventricular septal defect or conal resection, 2) construction of a intracardiac tunnel connecting the left ventricle to the aorta, and 3) direct connection, without a prosthetic conduit, of the pulmonary trunk to the right ventricle. RESULTS: There were 3 in-hospital deaths and their causes were sustained hypoxia, myocardial failure, and sepsis, respectively. There was 1 late death due to sepsis. Reoperations were performed in 6 patients who had pulmonary outflow tract obstructions (4 cases), residual muscular ventricular septal defect (1 case), and recurrent septic vegetation (1 case). The cumulative survival rates by the Kaplan-Meier method were 92.7%, 92.7%, and 92.7% at 1, 2, and over 4 years. The reoperation free survival rates were 92.7%, 92.7%, and 70.2% at 1, 3, and over 5 years. Among the risk factors for the operative death, aortic cross clamping time had statistical significance (p0.05). CONCLUSIONS: Our review suggests that Lecompte procedure is an effective treatment modality for various complex cardiac anomalies with pulmonary outflow tract obstruction. Repair in early age is possible and the rates of mortality and morbidity are also acceptable.

Postoperative Arrhythmias after Open Heart Surgery in Adults / 대한흉부외과학회지

BACKGROUND: We prospectively investigated types, incidences, and risk factors for arrhythmias after open heart surgery in adults. MATERIALS AND METHODS: From June 1994 to May 1995, we performed 302 cases of adult cardiac surgery at our department. This study group consisted of 150 men and 152 women, with a mean age of 43.9+/-28.0 (range 16 to 75)years. We included all the patients irrespective of their operative types or disease entities. RESULTS: The overall incidence of arrhythmias after open heart surgery in adults was 58.3%. The incidence of postoperative arrhythmias for redo-valvular heart surgery was 77.8%, and those for simple valvular procedure, coronary artery bypass surgery, aortic surgery, and congenital heart disease were 70.8%, 45.3%, 40.0%, and 29.5%, respectively. Eight out of twelve risk factors showed statistical significance for the development of postoperative arrhythmias. They were preoperative history of arrhythmias, antiarrhythmic drug medication, previous cardiac surgery, larger left ventricular end-diastolic, end-systolic dimension, left atrial dimension on preoperative echocardiogram, longer cardiopulmonary bypass time and aortic cross clamping time. Univariated analyses for age and types of cardioplegic solution did not show statistical significance. CONCLUSIONS: Prospective study on postoperative arrhythmias occurrence, treatment and prevention of is warrauted to draw more clear conclusion.

Malignant Thrombi of Inferior Vena Cava from Renal Cell Carcinoma: 4 cases reports / 대한흉부외과학회지

Renal cell carcinoma involves the inferior vena cava (IVC) in approximately 5-10% of the patients. Presently surgical extirpation is the only form of therapy that can result in cure. Circulatory arrest with cardiopulmonary bypass is an operative technique that recently has been used to assist in resection of tumors that extend into the vena cava above the level of hepatic veins. We performed removal of tumor thrombi of IVC in 4 patients. All of them who had the renal cell carcinormas with infrahepatic vena caval extension were performed by standard surgical technique without cardiopulmonary bypass. But in one patient, inferior vena caval thrombectomy was done using circulatory arrest because of the recurred extension of the tumor thrombi within the vena cava above the insertion of the hepatic vein. All patients were recovered without any significant problems.

Teratoma of the Pleura: A Case Report / 대한흉부외과학회지

Intrathoracic teratoma is mainly found on the anterior mediastinum. For teratoma of the pleura, one case was described. We have presented what we believe to be the first report of a teraroma of the pleura, which was mainly composed of neuroglial cells and was accompanied with lymph node metastasis.

Changes of Plasma Creatinine Kinase-BB after Total Circulatory Arrest / 대한흉부외과학회지

BACKGROUND: Although profound hypothermia with total circulatory arrest (TCA) is a valuable maneuver in cardiac surgery, its applications have been limited due to serious complications, especially cerebral damage. In this study, the possible role of creatinine kinase-BB (CK-BB), an index enzyme of ischemic cerebral damage, was assayed as a parameter for the assessment of the cerebral complications after TCA. Hemoglobin (Hb), ionized calcium (Ca++), and blood glucose levels were also assessed as clinical parameters involved in cerebral damage. MATERIALS AND METHODS: Among patients with congenital heart disease, 18 patients who had been operated on with TCA were randomly selected and divided into two groups: 6 with acyanotic and 12 with cyanotic heart disease. Arterial blood from each patient was collected before and after TCA at scheduled times (15 min., 30 min, 1, 2, 4, 8, and 12hr). The levels of CK-BB, Hb, Ca++, and blood glucose were assessed in each sample. RESULTS: As a whole, correlation between CK-BB level and blood sampling time after TCA was not statistically significant. Also, the difference in the level of CK-BB after TCA was not significant between the acyanotic and cyanotic groups. The levels of Hb and CK-BB correlated significantly. CONCLUSIONS: The results, which showed no correlation between the alterations in CK-BB level and the TCA duration, suggest that the single assay of the CK-BB level is not a representative measurement for the assessment of cerebral damage after TCA. Also, the cyanotic congenital heart disease group is not more vulnerable to cerebral damage induced by TCA.