Isaacs' syndrome associated with myasthenia gravis, showing remission after cytoreductive surgery of pleural recurrence of thymoma

We report a patient with Isaacs' syndrome associated with myasthenia gravis and pleural recurrence of thymoma, who showed severe limb pain attributed to hyperexcitability of sensory nerves. Myokymia and severe pain were successfully treated with cytoreductive surgery and intraoperative hyperthermic intrathoracic perfusion chemotherapy, but neither pharmacotherapy nor plasma exchange showed obvious clinical effects. Pleural thymoma in our patient may have caused Isaacs' syndrome, probably by unconfirmed humoral immune mechanisms. Cytoreductive treatment for recurrent thymoma should be actively considered as a potent therapeutic option in refractory patients with disabling neuromyotonia symptoms.     

Gender differences in 3D morphology and bony impingement of human hips

For the proper diagnosis or treatment of hip joint disorders caused by anatomical abnormalities, the normal hip joint morphology must be studied to understand its influence on the maximum range of motion (ROM) until bony impingement by focusing on gender differences. Acetabular and femoral morphologies were analyzed from 3D CT images of 106 normal hip joints from elderly men (n = 36 joints) and women (n = 70 joints), and measurements of ROM until bony impingement were made in four directions (flexion, extension, and external and internal rotation at 90° flexion) using surface models of the pelvis and femur reconstructed from the CT data. Gender differences were found not only in joint orientation, including anteversion and inclination of the acetabulum and femoral neck anteversion, but also in the shape around the joint, including the acetabular rim and the femoral neck. This ROM study also showed gender differences in all four standard directions. In conclusion, significant gender differences were observed in the acetabular and femoral morphology, which led to significant gender differences in ROM until bony impingement. © 2010 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 29:333–339, 2011     

Adenocarcinoma of the minor duodenal papilla: report of a case

We report a case of adenocarcinoma of the minor duodenal papilla, a rare type of duodenal neoplasm. A 76-year-old man with a history of surgery for rectal cancer and gastric cancer was referred to us after a follow-up upper gastrointestinal endoscopy revealed an abnormal elevation in the minor duodenal papilla. The pathological diagnosis of a biopsy specimen was adenocarcinoma. Preoperative examination of other organs revealed a tumor in the ascending colon, which was also identified as adenocarcinoma. We performed synchronous pancreatoduodenectomy and ileocecal resection with lymph node dissection. Histopathological examination of the resected specimen revealed that the papilla tumor arose from the duodenal mucosa and infiltrated the submucosa of the duodenal wall, but not the pancreatic parenchyma. Based on these findings, we diagnosed primary adenocarcinoma of the minor duodenal papilla. To our knowledge, this is only the sixth such case reported in the English-language literature, and we review all six cases after this case report.     

Severe parkinsonism following endoscopic third ventriculostomy for non-communicating hydrocephalus--case report

A 23-year-old male with non-communicating hydrocephalus caused by intraventricular hemorrhage from an arteriovenous malformation was treated by endoscopic third ventriculostomy (ETV) after repeated ventricular cerebrospinal fluid drainage and removal of ventriculoperitoneal shunt due to infection. Postoperatively, the patient developed robust rigidity and akinesia with mutism, suggesting secondary parkinsonism. These symptoms showed marked improvement after administration of levodopa, and were not aggravated after discontinuation. The pathophysiology of this case might involve dysfunction of the dopaminergic system caused by repeated compression and release of the paraventricular white matter. Careful perioperative management not leading to excessive dilation of the the ventricles should be considered for ETV for non-communicating hydrocephalus to avoid this unexpected complication.     

Noonan syndrome with occipito-atlantal dislocation and upper cervical cord compression due to C1 dysplasia and basilar invagination

A 11-year-old female with Noonan syndrome presented with occipito-atlantal dislocation and upper cervical cord compression due to C1 dysplasia and basilar invagination. Computed tomography (CT) of the cervical spine showed dysplasia of the C1 posterior arch and bilateral dislocation of the occipito-atlantal joints. Dynamic lateral radiography revealed no instability at the occipito-atlantal joints. CT also demonstrated basilar invagination. The tip of the odontoid process extended above the Chamberlain line by 9 mm and the McGregor line by 10 mm. Whole spinal radiography showed no scoliosis. C1 laminectomy was performed with instrumented occipito-C2 fixation. The postoperative course was uneventful, and magnetic resonance imaging revealed sufficient decompression of the upper cervical cord at 2 months after surgery. CT demonstrated solid bony fusion between the occipital bone and C2 at 8 months after surgery. Cervical neuraxial malformations are rare in patients with Noonan syndrome.     

Reconstruction of the middle hepatic vein tributaries draining segments V and VIII of a right liver graft by using the recipient’s own middle hepatic vein and vascular closure staples

A right liver graft lacking the middle hepatic vein can result in congestion of the anterior segment. We describe a method of reconstructing the middle hepatic vein tributaries by using the recipient’s own middle hepatic vein with vascular closure staples. During a living donor right liver transplantation, the middle hepatic vein tributaries draining segments V (V5) and VIII (V8) of the right lobe graft were reconstructed using the recipient’s own middle hepatic vein and secured with vascular closure staples. Computed tomography showed good venous outflow from the middle hepatic vein and no congestion or atrophy of the anterior segment of the right liver grafts. Thus, using the recipient’s own middle hepatic vein is a suitable option for reconstructing the middle hepatic vein tributaries (V8 and V5) in right-liver living donor transplantation and the application of vascular closure staples helps to accomplish this.     

Hydrodynamic evaluation of axillary artery perfusion for normal and diseased aorta

Axillary artery perfusion is an attractive alternative to reduce the frequency of atheroembolism in extensive atherosclerotic aorta and aortic aneurysms. This study was conducted to evaluate the flow dynamics of axillary artery perfusion. Transparent glass models of a normal aortic arch and an aortic arch aneurysm were used to evaluate hydrodynamic properties. Streamline analysis and distribution of the shear stress was evaluated using a particle image velocity method. In the normal aortic arch model, rapid flow of 80 cm/s from the right axillary artery ran out from the brachiocephalic artery and grazed the lesser curvature of the aortic arch. There was secondary reversed flow in the ascending aorta. Flow from left axillary perfusion went straight to the descending aorta. In the aortic arch aneurysm model, flow from both axillary arteries hit the lesser curvature of the aortic arch and went into the ascending aorta with vortical flow. Distribution of shear stress was high along the jet from the ostium of the brachiocephalic artery and left subclavian artery. Flow in the aortic arch and the ascending aorta was unexpectedly rapid. Special care must be taken when the patient has frail atheroma around arch vessels or the lesser curvature of the aortic arch during axillary artery perfusion.     

Long-Term Outcomes of Immunosuppressed Renal Transplant Recipients with Malignancies

This study analyzes ten cases of malignancy in a cohort of 183 renal transplant recipients, examining surgical management, postoperative immunosuppressive therapy, and long-term outcome. One of these ten patients, who had malignant lymphoma of the jejunum, died of the neoplasm, but the other nine patients did not show any signs of tumor recurrence after removal. All of these nine patients, except for one who had transplant renal cell carcinoma (RCC), received the same dose of immunosuppressive agents after surgery for the malignant disease. Seven patients were still alive at the time of this report, six of whom had good transplant renal function. The findings of this study indicate that even if immunosuppressive agents predispose to the development of cancer, it is not necessary to reduce their dose after removal of the tumor. Received: April 17, 2000 / Accepted: November 20, 2000     

Successful Surgical Treatment of Renal Cell Carcinoma in a Transplanted Kidney from a Cadaveric Donor: Report of a Case

Posttransplant renal cell carcinoma (RCC) usually arises in the native kidneys of renal transplant recipients rather than in the transplanted kidney. This report describes a case of RCC that developed in the transplanted cadaveric kidney in a 37-year-old male recipient 9 months after transplantation. An en bloc radical transplant nephrectomy was performed, and he has subsequently remained stable on hemodialysis for 3 years without any sign of recurrence. Received: March 27, 2000 / Accepted: September 26, 2000     

Clinical significance and prognostic value of apoptosis related proteins in superficial esophageal squamous cell carcinoma

Background: The purpose of the present study was to examine the expression of cell cycle regulators [p53, p21^WAF1/CIP1 (p21), and Rb] and apoptosis related proteins Bax and Bcl-X_L and to evaluate the relationship between their expressions and clinicopathological findings in patients with superficial squamous cell carcinomas of the esophagus.Methods: We immunohistochemically investigated the expression of p53, p21, Rb, Bax, and Bcl-X_L in 79 patients with superficial esophageal carcinoma.Results: p21 overexpression was found in mucosal carcinoma (P = 0.05) and a high Bcl-X_L score was observed for submucosal carcinoma (P = 0.03). The patients with high Bcl-X_L score had more frequent lymphatic invasion and lymph node metastasis than did those with low Bcl-X_L score (P < 0.05). Univariate analysis revealed significantly shorter survival in patients with high Bcl-X_L expression than in those with low Bcl-X_L expression, but Bcl-X_L expression was not identified as an independent prognostic factor by multivariate analysis.Conclusions: Because Bcl-Xl expression correlated well with depth of tumor invasion, lymphatic invasion, and lymph node metastasis, examination of Bcl-X_L expression will help to estimate the properties in superficial squamous cell carcinoma of the esophagus.