Intradural, extramedullary spinal Ewing's sarcoma in childhood.

We describe an 11 year old girl with progressive paraparesis from a spinal tumour. Magnetic resonance imaging showed an intradural, extramedullary mass extending from the C7 level to T1. Neither osteolytic nor osteosclerotic changes were seen in the vertebral bodies. Extraskeletal Ewing's sarcoma was diagnosed histopathologically.     

Subepicardial aneurysm detected during diagnosis of acute cardiac failure

A 55-year-old female developed dyspnea at night and visited our hospital. A diagnosis of acute cardiac failure was made and the patient was immediately admitted. A false ventricular aneurysm with blood flow was found in the lateral wall of the left ventricle. Past history of myocardial infarction was not clear. Repeated cardiac failure may have resulted from decreased cardiac output by increased blood flow in the aneurysm due to its expansion. After patch closure was performed, the symptoms improved.     

Case management of hydrocephalus associated with the progression of childhood brain stem gliomas

OBJECT: Most patients diagnosed with brain stem glioma become bedridden because of deteriorating brain stem function. Many brain stem glioma patients develop hydrocephalus. Both of these outcomes greatly detract from the quality of life of these patients. We have analyzed the occurrence of hydrocephalus in diffuse brain stem gliomas in children, and we discuss the management of advanced cases. METHODS: Eighteen patients diagnosed with brain stem glioma while under 15 years of age, including 1 with dissemination, were studied retrospectively. The average overall survival was 11.8 +/- 6.5 months (mean +/- SD). Hydrocephalus occurred in 16 (88.9%) of the 18 cases. The patients diagnosed with hydrocephalus all exhibited a rapid decline in consciousness. The average time to onset of hydrocephalus after tumor diagnosis was 5.1 +/- 3.3 months. Twelve of the 16 patients with hydrocephalus were treated with cerebrospinal fluid (CSF) diversion, by means of a Torkildsen shunt, a ventriculoperitoneal shunt, or third ventriculostomy. The level of consciousness and patient performance status improved after CSF diversion except in 2 patients who had received Torkildsen shunts. The patients treated for hydrocephalus survived significantly longer than those patients who did not undergo any intervention for hydrocephalus. CSF diversion may be a therapeutic intervention that significantly improves the quality of life and survival of patients. CONCLUSION: Our results suggest that patients diagnosed with brain stem glioma should be closely monitored for signs of hydrocephalus and be examined by neuroimaging rapidly when indicated. Our results also suggest that once hydrocephalus is diagnosed CSF diversion should be performed promptly.     

General anesthesia for patients with Brugada syndrome. A report of six cases

PURPOSE: To review six cases of Brugada syndrome presenting for insertion of a cardioverter-defibrillator under general anesthesia. CLINICAL FEATURES: All patients had a history of syncope, ST segment elevation in the right precordial lead of the electrocardiogram (ECG) which became prominent after a pilsicainide challenge test. Routine monitors, right precordial lead of the ECG and an external defibrillator were installed prior to anesthesia. We administered propofol/midazolam for induction, and propofol/sevoflurane combined with fentanyl for maintenance of anesthesia. Atropine and ephedrine were administered to decrease vagal tone. No ECG change or arrhythmia was observed perioperatively. After the successful implantation of the defibrillator, all patients were discharged without any adverse event. CONCLUSION: By avoiding agents or conditions that may exacerbate Brugada syndrome during anesthesia, we were able to manage the patients uneventfully for implantation of a cardioverter-defibrillator.     

In vivo effect of etoposide on the function of bone marrow-derived macrophages

Proliferative and colony-forming abilities of bone marrow cells to M-CSF and the function of the bone marrow-derived macrophages were examined 2, 7, 14 and 21 days after intraperitoneal injection of etoposide. Only two days after administration of etoposide (50 mg/kg of body weight), the number of bone marrow cells per femur was significantly decreased, but completely recovered by day 7. In contrast, proliferative and colony-forming responses of the bone marrow cells to M-CSF was increased after 2 days, but there-after returned to the normal. The abilities of bone marrow-derived macrophages to adhere to plastics and to produce monokines (IL-1 and TNF) were the same as those of controls before and after treatment with etoposide. These data suggest that activation of macrophage progenitors in bone marrow may be useful in combination with chemotherapy for treatment of malignant diseases.     

Improvement of heart failure after intracardiac operation for tetralogy of Fallot by transcatheter embolization of major aortopulmonary collateral arteries

A two-year-and-ten-month-old female with tetralogy of Fallot with major aortopulmonary collateral arteries (MAPCAs) developed a persistent heart failure after an intracardiac repair. Angiograms revealed four MAPCAs originating from the upper portion of the descending thoracic aorta and draining into the left pulmonary artery, one MAPCA from the inferior phrenic artery into bilateral pulmonary arteries, and remaining one from the left subclavian artery into left pulmonary artery. Three of these MAPCAs were occluded by transcatheter embolization by the use of the steal coil. The heart failure was improved dramatically by this treatment.     

Combined medical and surgical treatment of 74 cases of acute type III aortic dissection]

In the early period up to 1986, our treatment of acute type III dissection was anti-hypertension drug therapy as a rule, and Collins operations were performed in two cases of progressive hemothorax. Among 21 patients receiving medical therapy, five died of rupture, and three operated cases died of multiple organ ischemia, and then the hospital mortality was 40%. Since 1987, we have selected hypotensive treatment of strictly maintaining blood pressure less than 120mmHg for the completely thrombosed type of the dissected lumen, and the emergency operation of ringed intraluminal graft insertion (RIG operation) for the blood-flow type and aneurysm formation type of the dissected lumen, diagnosed by the emergency cine-angiography. As the result, among 51 cases having hypotensive therapy, one died of respiratory failure. In the 23 operated cases, in which RIG operation and/or arterial reconstruction was performed, four died of multiple organ ischemia. The hospital mortality was 8%, which was significantly improved compared with that of the early period.     

Surgical outcome of stentless aortic valve replacement for calcified aortic stenosis

We have conducted aortic valve replacement (AVR) using a stentless bioprosthesis (Medtronic Freestyle valve) on 10 patients with calcified aortic stenosis since March 2004. There were 64-84 years of age and 75 ± 5.5 years old on average, and included 4 males. Implantation was conducted by a modified subcoronary method in all the patients. The preoperative New York Heart Association class was class II in 80% of the patients. The preoperative left ventricular mass and the left ventricular mass index (LVMI) were 193.1-524.1 g and 144.1-299.5 g/m2 and, on average 328.4 ± 104.7 g and 217.3 ± 55.7 g/m2, respectively. The annulus dimension was 18-24 mm and, on average, 20.3 ± 1.7 mm. The size of implanted valve was 19-25 mm and, on average, 21 ± 2.2 mm. The maximum pressure gradient of the aortic valve remained at 14.2-46.5 mmHg, 25.2 ± 10.2 mmHg on average, 1 or 2 months after surgery, but the LVMI significantly improved to 153.2 ± 33.9 g/m2 (p = 0.018). The hospitalization period were 24.7 ± 16.9 days for all the patients and 19.3 ± 5.1 days for patients undergoing the AVR alone. These results show that LVMI is significantly reduced by using a stentless bioprosthesis in the early phase after surgery, and early discharge from hospital can be expected by concurrently using minimally invasive cardiac surgery.     

Compartment Syndrome of Bilateral Lower Extremities Following Laparoscopic Surgery of Rectal Cancer in Lithotomy Position: Report of a Case

A 67-year-old man underwent laparoscopic surgery for rectal cancer in the lithotomy position. After surgery he complained of bilateral lower limb pain, swollen legs, and sensory disturbance. The serum creatine kinase value was 46 662 U/l. Venography demonstrated compression from outside without any obstruction. The T2 image of magnetic resonance imaging (MRI) showed a massive swollen muscle and a partial high-intensity area in the bilateral lower limbs. The posterior compartment pressures of lower legs were high (gastrocnemius muscle: 30 mmHg [right] and 44 mmHg [left]). Compartment syndrome (superficial posterior compartment) was thus diagnosed. He underwent a fasciotomy using the single dorsal approach and the administration of a large amount of fluid. He recovered well without any motor or sensory deficits. Compartment syndrome is rare, occurring only once in every 3500 cases, but it is a severe complication of surgery in the lithotomy position. Several risk factors have been pointed out: including prolonged operation, hardness of the operating table, obesity, dehydration, and hypothermia. To prevent compartment syndrome, appropriate positioning during surgery is therefore essential. To make a timely diagnosis and identify the precise location of muscle edema, the T2 image of MRI is useful.