Hydrosyringomyelia associated with a Chiari I malformation in children and adolescents

The clinical presentation, radiological features, and results of surgical treatment were analyzed in 17 cases of hydrosyringomyelia associated with a Chiari malformation, in children and adolescents younger than 20 years of age. The initial symptoms were a skeletal abnormality (71%), such as scoliosis (11 patients) or pes cavus (1 patient), pain or numbness (24%), and motor weakness (6%). Frequently seen signs on admission were sensory deficit (100%), scoliosis (85%), muscle weakness (64%), muscle atrophy (35%), and lower cranial nerve palsy (35%). The characteristic neurological findings were unilateral sensory and motor deficits (65%) with decreased or absent deep tendon reflexes on the same side. The localization of the syrinx on the axial section varied according to the level, even in the same patient. In 11 patients with unilateral sensory disturbances or unilateral sensory and motor deficits, the syrinx was located in the region corresponding to the posterolateral portion on the same side as that of sensory disturbance at the cervical or thoracic level. On the other hand, in 6 patients with bilateral sensory and motor deficits, the syrinx was located in the central portion and extended into the posterolateral portion of the more affected side. A syringosubarachnoid shunt was placed in 16 patients, foramen magnum decompression without closure of the obex was performed in 1 patient, ventriculoperitoneal shunt in 1 patient, terminal syringostomy in 1 patient, and foramen magnum decompression with terminal syringostomy in 1 patient. In 15 of 17 patients (88%), the neurological symptoms improved after an average follow-up of 4 years and 1 month. We think that as a surgical treatment, placement of a syringosubarachnoid shunt is effective.     

The use of the bio-pump with an interposed soft reservoir in replacement of descending thoracic aortic aneurysms

The centrifugal pump (bio-pump) as a temporary shunt during the operation of the descending thoracic aortic aneurysms was used as effective means of providing adequate circulation to the lower body. But in the acute excessive bleeding, estimates of the flow to the abdominal viscera as well as spinal cord are variable. We employed bio-pump with an interposed soft reservoir under low dose systemic heparinization in 2 cases of the descending thoracic aortic aneurysms. Both cases had been maintained adequate flow and perfusion pressure in the time of acute excessive hemorrhage, and there were no evidence of the organ failures due to microembolism. Furthermore, postoperative hemorrhage by using low dose heparin seemed to be unrisky. Compared with the previous method, bio-pump with an interposed soft reservoir provides adequate circulation to the lower body even if acute excessive bleeding occurred.     

Clinical evaluation of a new oral cephem, cefdinir, in children

Cefdinir (CFDN, FK482) was evaluated for its safety, efficacy and pharmacokinetics in 28 children. CFDN was effective in 100% of 22 evaluable cases with respiratory, middle ear, urinary or soft tissue infections. From the clinical response, adverse effects and the pharmacokinetic results, daily dose of 9-18 mg/kg, administered in 3 divided portions is suggested. Increase of dose will be associated with increase of gastrointestinal side effects. The data suggest that CFDN is safe and effective when used in children with infections caused by susceptible bacteria including Staphylococcus aureus.     

Diagnosis and treatment of thoracic outlet syndrome

Patients who develop symptoms of thoracic outlet syndrome (TOS) have a predisposing anatomic abnormality. In most patients with TOS, the symptoms are caused by entrapment of the brachial plexus and they do not arise from compression of the subclavian artery, as was previously thought. The tests advocated for diagnosing this common syndrome (i.e., evaluating the positional compression of the artery when the arms are raised, the neck is turned, or the shoulders are braced) cannot accurately diagnose this syndrome. There are two reasons for this. The symptoms of TOS are not related to the compression of the artery in the outlet in 98% of patients, and 75% of normal individuals without symptoms show diminished radial pulse on various provocation tests. We employed four timed provocation tests (minute tests) to diagnose TOS: the timed Morley test, timed Wright test, timed Eden test, and elevated arm stress exercise, all of which are very sensitive. In normal individuals without symptoms, 20% experience transitional symptoms such as slight pain and tiredness, on these tests indicating a subclinical state. TOS is treated by keeping the thoracic outlet wide, this being done either conservatively or surgically. In 1993 and 1994, we conservatively treated 418 of 422 patients with TOS by means of active exercise, a brace, and by block therapy. These measures did not reduce the symptoms in 23 of these patients, so surgical treatment was indicated. In the remaining 4 of the 422 patients, conservative treatment was not indicated and surgery was performed directly. All the patients showed significant clinical improvement of varying degree. Presented at the 69th Annual Meeting of the Japanese Orthopaedic Association, Tokyo, April 12, 1996     

Ultrastructural and immunohistochemical study of Ewing's sarcoma and related tumors: Morphological neural markers suggesting neural histogenesis in 32 small round-cell sarcomas

The purpose of this study was to validate the hypothesis of neural histogenesis of Ewing's sarcoma of bone and related tumors by light microscopic, electron microscopic, and immunohistochemical analysis. We studied 32 round-cell sarcomas (19 cases of Ewing's sarcoma of bone, 3 extraskeletal Ewing's sarcomas, 5 peripheral primitive neuroectodermal tumors (PNET) and 5 cases of unclassified small round-cell type of neurogenic sarcoma (NS). Immunoreactivity for MIC2 was observed in all cases of Ewing's sarcoma and PNET, and in 1 cases of NS. Positive immunoreactivity for neural markers (NSE, synaptophysin, S-100) was found frequently in some tumors. Ultrastructurally, some specific features of neural differentiation, such as a fragmented basal lamina and neurosecretory granule-like particles, were found even in typical cases of Ewing's sarcoma of bone, which presented without a rosette arrangement and were almost negative for neural immuno-markers, but positive for MIC2. These ultrastructural neural features were observed less frequently in Ewing's sarcoma of bone than in PNET and NS. However, no significant correlation was demonstrated between the immunoreactivity for neural markers and the ultrastructural and histological neural features. These results support the hypothesis of a neural origin of Ewing's sarcoma and related neoplasms, and suggest that some overlapping entity could persist in PNET and Ewing's sarcoma and that this entity could be seen in histological and immunohistochemical studies of both tumors.     

Multiple neurinoma of the spinal cord: case report

The patient, a 72-year-old female, was admitted with an 11-year history of progressive dysesthesia in the left leg. Neurological findings on admission revealed weakness in the bilateral legs, hyperreflexia of left leg, hypalgesia and hypesthesia under the Th 8 level, and urinary incontinence. Plain lumber X-ray showed enlargement of the intervertebral foramen of L 1/2. Myelography disclosed block age at the level of Th 9 and filling defect at the level of Th 10/11 and L1/2. CT myelography revealed a cord swelling and partially exophytic tumor from Th 8 to Th 9, another tumor located posterior-laterally at the level of Th 11, and another tumor located extra and intradural at the level of L1/2. Laminectomy was performed from Th 8 to L 3. A tumor of the Th 9 was located intramedullary, another tumor of the Th 11 was located intradural extramedullary, and another tumor of the L1/2 was located in the epidural space (so-called dumb-bell type tumor). These tumors were removed completely except the extra-canal part of L1/2 tumor. Histopathological examination revealed typical Antoni type A schwannoma in all tumors. This case was considered multiple neurinomas of the spine in which tumors were located in three separate anatomical sites, intramedullary, intradural extramedullary, and epidural sites. This patient did not show café-au-lait spot, and neurofibroma in her body. The authors considered the patient might be a case of central neurofibromatosis. The authors stressed that multiple neurinomas of the spine is not rare, so careful study of the whole spine is necessary including its intramedullar space.     

Effects of glycyrrhetinic acid on aminonucleoside nephrosis in rats

The effects of glycyrrhetinic acid (GA), an aglycon of glycyrrhizin extracted from the roots of Glycyrrhizae radix, on puromycin aminonucleoside (PA) nephrosis were studied in rats. Urine protein excretion in female rats (130g–150g) receiving PA (50 mg/kg) alone was significantly elevated on the 2nd day after injection of PA and reached a peak on the 14th day. Urinary protein on the 14th day was reduced to 74% in animals treated with GA (20 mg/kg) starting on the 2nd day after injection of PA. The increase in serum cholesterol and the decrease in serum protein were also suppressed by GA. Observation by electron microscopy revealed that the degree of abnormality in glomerular epithelial cells, i.e. loss or fusion of foot processes, was lower in the rats treated with GA after PA injection than in the rat treated with PA alone. Moreover, pretreatment with GA did not suppress urinary protein excretion but when it was given at the same time as PA and after PA a significant decrease in urinary protein excretion was observed. Correspondence to: H. Abe at the above address     

Late results after total correction of tetralogy of Fallot: over 10 year follow-up

Late results after total correction of tetralogy of Fallot over ten year follow up of 84 patients were discussed. No late death was experienced and 95.1% of these patients are now in NYHA class I. Reoperations were performed in 7 cases (8.3%) in which six had had residual shunt and one had had sick sinus syndrome and had VVI pacemaker implantation. Ten cases had over 90% of ratio of right ventricular pressure to systemic pressure in systole in their cardiac catheterization about 40 days after correction. In spite of this high pressure ratio, all cases are now in NYHA class I. We conclude that there is a possibility of enabling to preserve pulmonary valve function by adopting smaller criteria than the Pacifico's criteria.     

Granular cell tumors of the breast: A report of two cases

Granular cell tumors (GCT) are rare neoplasms, and only 173 cases of benign GCT of the breast have been documented. We report herein the cases of two patients with this tumor and discuss the methods of diagnosis and treatment. The first patient was a 60-year-old woman who presented with a firm ill-defined mass in her left breast. Mammography showed a dense shadow with spicula and skin thickness, and ultrasonography revealed a hypoechoic mass with an irregular border. Radical mastectomy was performed under the wrong preoperative diagnosis of breast cancer. The second patient was a 31-year-old woman who presented with an elastic-hard mass in her left breast. Mammography showed a well-demarcated dense mass, and ultrasonography revealed a well-defined hypoechoic mass with a large depth-width ratio. Fine-needle aspiration cytology (FNAC) showed a large number of histiocytic cells with abundant granular cytoplasm. An excisional biopsy was performed, and histological examination confirmed a diagnosis of GCT. GCT is benign, but often misdiagnosed as breast cancer both clinically and radiologically. Therefore, histological examination is essential for making a correct diagnosis, while FNAC is also useful. Local resection is still the treatment of choice, and surgeons should do their utmost to avoid performing needless radical mastectomy.