Pulmonary manifestations in cerebrotendinous xanthomatosis.

We investigated five cases with cerebrotendinous xanthomatosis (CTX) with particular reference to biochemical and pathological pulmonary disorders. To date, few reports discuss the pathophysiology of pulmonary disorders of CTX patients. This study is the first investigation of such pulmonary disorders. All 5 patients had no pulmonary symptoms and no disturbances on radiological studies and pulmonary function tests. However, in bronchoalveolar lavage (BAL) fluids, many cells with cruciform reflexes, which is characteristic of intracellular sterol accumulation, were found under phase contrast microscopy. Biochemically, cholestanol was found to be increased in the BAL fluid as well as in serum. Pathological findings of transbronchial lung biopsy (TBLB) samples disclosed foamy macrophages and small granulomas in alveolar septa. In conclusion, the lung was apparently involved in CTX, and the lesions were characterized with the accumulation of foamy and giant cells with a high concentration of cholestanol, which likely results in the formation of foreign body granulomas.

[Syphilitic thoracic aortic aneurysm with destruction of vertebral body, producing numbness of lower extremities and paraplegia].

Numbness and paraplegia are uncommon complaints in patient with thoracic aortic aneurysm (TAA). The patient was a 64-year-old man. He suffered numbness and gait disturbance (paraplegia). The blood examination showed no positive findings except a Wassermann was positive. Roentgen examination of the chest showed two abnormal shadows like tumors. The CT and MRI revealed destruction of the vertebral bodies and TAAs adjacent to the spinal cord. After the graft replacement was performed, numbness and paraplegia disappeared. This suggests that in our patient the TAAs destruct the vertebral body and produce pressure on the spinal cord, causing numbness and paraplegia. We experienced a rare case of the syphilitic TAA producing bone destruction, numbness and paraplegia.

[Two case reports of recurrent mediastinitis with chronic mediastinal fistula successfully treated with muscle flap re-transposition].

We experienced two cases of recurrent poststernotomy mediastinitis with chronic mediastinal fistula. Both cases had already received muscle flaps for post operative mediastinitis. However, chronic mediastinal fistula appeared after nine months in the first case, and eleven months in the second case. We removed the infected tissue and the predgets, which were used on the ascending aorta. Then closed the wound by the muscle flap closure. The chronic fistula were closed, and the functional and cosmetic results were excellent.

[An operative case of aortic valve regurgitation due to blunt trauma to the chest].

While a 70-year-old man was riding a motorcycle, he was hit by car on his chest on October 19, 1990. Medical check-up at the emergency room of another hospital was negative. However, he fell in to dyspnea on the night of next day which progressed to develop signs of orthopnea. He revisited the hospital where he was found to have aortic regurgitation and subsequent congestive heart failure after medical examination including echocardiography and was referred to our hospital 7 days after the accident. He was operated upon the following day. On opening the pericardium, about 100 ml of reddish black fluid was accumulated. The appearance of the heart was normal except for black discoloration of the epicardial fat pad at the base of the aorta. Opening the ascending aorta transversely, the right coronary cusp was found to be perforated. The aortic valve was considered preferable to replace than to repair. The aortic valve was replaced with a St. Jude Medical valve. His recovery was uneventful and he has been well thereafter.

[A case of surgical repair for combined septal perforation and cardiac rupture after myocardial infarction].

The successful surgical repair of the combination of septal perforation and cardiac rupture after myocardial infarction was underwent on a 73-year-old man. Complete correction could be performed under the fibrillated heart state employing cardiopulmonary bypass, resulted in the early postsurgical cardiac performance.

[Results of surgical repair by muscle flap closure for poststernotomy mediastinitis].

Between 1984 and 1991, 30 patients developed poststernotomy mediastinitis after cardiac or mediastinal operations. Sixteen of these patients were treated with debridement and open drainage or primary closure following mediastinal irrigation (drainage group). Fourteen patients were treated with debridement, and delayed or primary wound closure by the technique of pectoral or rectus abdominus muscle flap mobilization (muscle flap group). The purpose of this study was to compare the results of the different treatment of poststernotomy mediastinitis. Although the pre- and perioperative profile, complications and risk factors for mediastinal infection were the same, the mortality of muscle flap group was significantly lower than that of drainage group (Fisher exact probability test, p < 0.05). Pulmonary function of the patients who received muscle flap mobilization did not decrease from the function before surgery, despite the defect of sternal bony stabilization. We conclude that muscle flap mobilization may be a superior method as the treatment for poststernotomy mediastinitis after cardiac operations, and may not decrease respiratory function of the patients.

[Recurrent thymic carcinoid tumor--report of a case and review of the literature].

A case is presented of a 48-year-old male patient with thymic carcinoid tumor who was operated on twice and followed for 12 years. The original tumor was removed in 1979, and a recurrent tumor was removed 4 years later. Multiple metastases to the lungs, pleura and lymph nodes of the neck were found in 1988; chemotherapy (BAPP) and radiation therapy were not effective, and there was subsequent subcutaneous invasion of the anterior chest. We also discuss the 153 cases of thymic carcinoid tumor reported in the Japanese literature in the Niigata Carcinoid Registry.

[A case of tricuspid pouch associated with ventricular septal defect and functional left ventricular-right atrial communication].

We reported a 60-year-old woman with tricuspid pouch associated with ventricular septal defect and subsequent left ventricular-right atrial communication. Preoperative angiographic findings suggested the presence of membranous septal aneurysm, ventricular and atrial septal defects, and tricuspid insufficiency. However, at operation, besides perimembranous inlet type ventricular septal defect, a pouch, 1.5 cm in diameter was found in the adjacent part to the septal leaflet within the anterior one of the tricuspid valve. The ventricular septal defect was closed with a patch. From these findings, it is speculated that the tricuspid pouch was formed by the effect of jet stream through the ventricular septal defect.

[A rare case of airway foreign bodies complicated with flail chest following traffic accident].

A 30-year-old man was taken to the emergency room of this hospital because of chest injury following traffic accident. On arrival he was found to be dyspneic due to multiple rib fractures (flail chest) and tension pneumothorax on the left, and was intubated immediately concomitantly with a chest drainage. During the management with the respirator, he was also noticed to have continuous bleeding from the airway. The X-ray film of the chest showed a suspicion of foreign materials on the right hilar region and subsequent bronchoscopic examination revealed the presence of several fragments of a broken windshield in the bronchial trees. Nine fragments were removed bronchoscopically. Tracheal tube was extubated on the 14th day and he was discharged on the 28th day without any sequela.

Prediction of preserved flow to the infarct area based on admission electrocardiogram in anterior wall acute myocardial infarction.

To determine whether preserved flow to the infarct area could be predicted from the admission electrocardiogram and to define the effect of preserved flow on the late results after reperfusion, 20 anterior myocardial infarction patients who were successfully reperfused were studied. Patients were divided into 3 groups: (1) no-flow group (8 patients), with an occluded infarct-related artery and no easily visible collaterals; (2) intact collateral group (6 patients); and (3) subtotal obstruction group (6 patients). From the admission electrocardiogram, the sum of ST-segment elevation (sigma ST), the sum of R-wave amplitude (sigma R) in leads V1 through V6 and the ratio of these (sigma R/sigma ST) were measured. There was no significant difference in sigma R among the 3 groups. The no-flow group had significantly lower sigma R/sigma ST and higher sigma ST than the intact collateral group or subtotal obstruction group. All patients (6 of 6) with subtotal obstruction and all except 1 patient (5 of 6) with intact collateral showed sigma R/sigma ST greater than 2.5 or sigma ST less than 2.0 mV. All patients (8 of 8) with no flow showed sigma R/sigma ST less than or equal to 2.5 and all except 1 patient with no flow (7 of 8) showed sigma ST greater than or equal to 2.0 mV. The regional wall motion was assessed by the radial method at 4 weeks. The mean percentage systolic shortening in the anterior and apical regions was significantly correlated with sigma R/sigma ST (r = 0.75, p less than 0.001) and sigma ST (r = -0.65, p less than 0.005).(ABSTRACT TRUNCATED AT 250 WORDS)

Malignant spindle cell tumor of the pericardium. Evidence of sarcomatous mesothelioma with aberrant antigen expression.

A case of malignant spindle cell tumor occurring in the pericardium is presented. The tumor arose from the pericardium of a 51-year-old Japanese woman with no history of exposure to asbestos. The tumor extended into the pericardial and left pleural cavities. The primary and metastatic tumors consisted of fusiform cells with frequent mitoses. Ultrastructurally, the tumor cells possessed a discontinuous external lamina, cytoplasmic processes, microfilaments and desmosomal intercellular junctions. Immunohistochemical examination showed that most tumor cells were positive for Leu 7, and several for S-100 and glial fibrillary acidic protein. Unexpectedly, most of the tumor cells also expressed keratin. These findings favor a diagnosis of sarcomatous mesothelioma with aberrant antigenic expression or heterogeneous differentiation of neoplastic cells.

[The effect of body respirator on the desaturation during the night in Duchenne muscular dystrophy].

Recently body respirator (BR) has been used to control respiratory failure in patients with late stage Duchenne muscular dystrophy (DMD). We examined the effect of BR using a pulse oximeter. Arterial oxygen saturation (SaO2) for the night (21:00-7:00) was monitored in 15 DMD patients (5 cases without BR, 3 cases with BR partially for the night and 6 cases with BR all night long) and the desaturation (SaO2 less than 90%) time was followed three times (Jan. '87, Nov. '87, Apr. '88) in each patient. Desaturation time did not increase in 4 cases without BR. But in one case without BR it increased so much that we decided to put the patient on BR. In 3 cases with BR partially for the night, desaturation was well controlled when they used BR. No marked increase of desaturation was found in 4 cases with BR all night long. 2 of these cases were changed from cuirass type BR to jacket type BR and were getting on satisfactorily. Thoracic cage expansion of jacket type was larger than that of cuirass type, and it was found that jacket type was valuable. Also, we investigated the cause of desaturation by recording SaO2, nasal flow, thoracic cage motion and abdominal motion at the same time by making use of a polygraphy. The result showed that the main cause of desaturation was the resistance of thoracic cage motion against BR. And we think research and development is needed.

[Clinical and myopathological findings in polymyositis].

The present report analyzed a clinical and myopathological findings of 60 patients defined for the diagnosis of polymyositis. Patients were classified in four groups according to the system Walton and Adams. Forty five per cent were classified as Group I, 28.3 per cent as Group II, 15.0 per cent as Group III and 11.7 per cent as Group IV. Seven patients were associated with a malignant neoplasm and all of them were over fifty. The primary growth was carcinoma of the lung in 2, of the prostate, ovarium, stomach in one case each, and in one case a chronic myelogenous leukemia and in one case a malignant lymphoma. The female to male ratio was 2:1. Though cases were seen in all age groups, the largest number was in the sixth decade. The presenting symptoms and signs were essentially similar to those reported before. Proximal lower and upper limb weakness was the most frequent symptoms (91.7 and 83.3 per cent respectively). Other characteristic symptoms were skin changes, muscle pain, arthralgia and Raynaud's phenomenon. The CK was measured at the time of presentation in 58 of the 60 cases. Normal values were found in 18.3 per cent of cases. In general, muscle biopsy was performed in the first stage of the disease. A diagnostically abnormal biopsy was 55 cases (92 per cent), but 8 per cent of cases had normal biopsy. The characteristic changes in the biopsies from 60 cases were muscle fiber necrosis, inflammatory infiltration, fibrosis, basophilia and increase of internal nuclei. We could not get a significant difference between the two groups. However all of the cases of Group IV had muscle fiber necrosis, inflammatory infiltration and fibrosis.

Extraskeletal myxoid chondrosarcoma arising from the retroperitoneum.

A case of extraskeletal myxoid chondrosarcoma is presented. The tumor occurred in the retroperitoneum and systemic metastases were found at autopsy. The primary and metastatic tumors were soft and strikingly myxoid on gross appearance. Microscopic observation revealed undifferentiated malignant tumor having large amounts of myxoid substance and a small amount of well-differentiated chondrosarcoma element in the primary lesions. The authors obtained an immunohistochemical result that the tumor cells showed positivity for alpha-1-antitrypsin and alpha-1-antichymotrypsin. Regarding S-100 protein, the well-differentiated chondrosarcoma element revealed intense positivity, whereas the poorly differentiated myxoid areas were not positive except for a few tumor cells. This is the first case, to our knowledge, of extraskeletal myxoid chondrosarcoma arising from the retroperitoneum, and immunohistologic findings suggest that alpha-1-antitrypsin and alpha-1-antichymotrypsin may be available markers in poorly differentiated chondrosarcomas showing a negative reaction for S-100 protein.