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.     

A new active vitamin D3 analog, eldecalcitol, prevents the risk of osteoporotic fractures--a randomized, active comparator, double-blind study

Background

Eldecalcitol is an analog of 1,25-dihydroxyvitamin D₃ that improves bone mineral density; however, the effect of eldecalcitol on the risk of fractures is unclear. The objective of this study is to examine whether eldecalcitol is superior to alfacalcidol in preventing osteoporotic fractures. This trial is registered with ClinicalTrials.gov, number NCT00144456.

Methods and results

This 3 year randomized, double-blind, active comparator, superiority trial tested the efficacy of daily oral 0.75 μg eldecalcitol versus 1.0 μg alfacalcidol for prevention of osteoporotic fractures. 1054 osteoporotic patients 46 to 92 years old were randomly assigned 1:1 to receive eldecalcitol (n = 528) or alfacalcidol (n = 526). Patients were stratified by study site and serum 25-hydroxyvitamin D level. Patients with low serum 25-hydroxyvitamin D levels (< 50 nmol/L) were supplemented with 400 IU/day vitamin D₃. Primary end point was incident vertebral fractures. Secondary end points included any non-vertebral fractures and change in bone mineral density and bone turnover markers. Compared with the alfacalcidol group, the incidence of vertebral fractures was lower in eldecalcitol group after 36 months of treatment (13.4 vs. 17.5%; hazard ratio, 0.74; predefined 90% confidence interval [CI], 0.56–0.97). Eldecalcitol reduced turnover markers and increased bone mineral density more strongly than alfacalcidol. Eldecalcitol reduced the incidence of three major non-vertebral fractures, which was due to a marked reduction in wrist fractures by a post-hoc analysis (1.1 vs. 3.6%; hazard ratio, 0.29; 95% CI, 0.11–0.77). Among the adverse events, the incidence of increase in serum and urinary calcium was higher in the eldecalcitol group, without any difference in glomerular filtration rate between the two groups.

Conclusions

Eldecalcitol is more efficacious than alfacalcidol in preventing vertebral and wrist fractures in osteoporotic patients with vitamin D sufficiency, with a safety profile similar to alfacalcidol.     

Clinical outcomes and adverse events following transforaminal interbody fusion for lumbar degenerative spondylolisthesis in elderly patients

Transforaminal lumbar interbody fusion (TLIF) procedure is widely used, but the surgical indications for TLIF in elderly patients remain controversial because of potential risks such as inferior bone quality and higher rate of postoperative complications. Clinical efficacy and operative risk of TLIF in elderly patients are unclear. This study investigated the clinical effect and safety of TLIF for lumbar degenerative spondylolisthesis with radiculopathy or neurogenic claudication in patients aged 70 years or older. The clinical records were retrospectively reviewed of 35 consecutive patients aged 70-86 years (mean 74.8 years) who underwent one or two-level TLIF. The preoperative diagnosis included degenerative spondylolisthesis with segmental instability. Clinical outcomes were assessed by the Japanese Orthopaedic Association score, visual analogue scale, Oswestry Disability Index. Radiological fusion rate was also investigated. Clinical and radiological results were compared with those of 43 younger patients. Clinical outcome measures were significantly improved after operation in the elderly patients, but improvement rates were significantly lower than those of younger patients. Fusion rate was similar in both groups. Overall postoperative complications were increased in aged patients, although the prevalence of complications directly related to surgical technique was not significantly increased. Postoperative complications not related to the surgical procedure were factors affecting poor results. TLIF is acceptable for achieving clinical recovery and lumbar fusion with high radiographic fusion success even in elderly patients, although clinical benefits were limited compared with those of younger patients. Postoperative morbidity was mainly related to general or non-operative site complications.     

Flowcharts for the management of biliary tract and ampullary carcinomas

No strategies for the diagnosis and treatment of biliary tract carcinoma have been clearly described. We developed flowcharts for the diagnosis and treatment of biliary tract carcinoma on the basis of the best clinical evidence. Risk factors for bile duct carcinoma are a dilated type of pancreaticobiliary maljunction (PBM) and primary sclerosing cholangitis. A nondilated type of PBM is a risk factor for gallbladder carcinoma. Symptoms that may indicate biliary tract carcinoma are jaundice and pain in the upper right area of the abdomen. The first step of diagnosis is to carry out blood biochemistry tests and ultrasonography (US) of the abdomen. The second step of diagnosis is to find the local extension of the carcinoma by means of computed tomography (CT), magnetic resonance imaging (MRI), magnetic resonance cholangiopancreatography (MRCP), percutaneous transhepatic cholangiography (PTC), and endoscopic retrograde cholangiopancreatography (ERCP). Because resection is the only way to completely cure biliary tract carcinoma, the indications for resection are determined first. In patients with resectable disease, the indications for biliary drainage or portal vein embolization (PVE) are checked. In those with nonresectable disease, biliary stenting, chemotherapy, radiotherapy, and/or best supportive care is selected.     

Characterization of K-ras gene mutations in association with mucinous hypersecretion in intraductal papillary-mucinous neoplasms

Background/Purpose Intraductal papillary-mucinous neoplasms (IPMNs) of the pancreas have a favorable prognosis. However, invasive ductal carcinomas of the pancreas show a rapid progression. The aim of this study was to investigate gene mutations in pure pancreatic juice from IPMN patients and to define these genetic mutations in relation to the histopathological and clinical features of IPMNs. Methods Twenty-two patients with IPMN, 21 patients with ductal carcinoma, and 20 patients with normal pancreas or chronic pancreatitis were recruited for this study. We measured the main pancreatic duct’s largest diameter and the maximum size of a dilated branch was assessed by ultrasonography or endoscopic ultrasonography. Pure pancreatic juice was collected and was investigated for K-ras, p16, and p53 mutations. Results Mutant K-ras gene was detected in 13 of the 22 patients (59.1%) with IPMNs. Different kinds of mutations were detected in the same patient in 4 cases. In the 13 patients with mutant K-ras gene, the diameter of the most dilated part of the main pancreatic duct was 2–8 mm (average, 4.5 mm) and in 7 patients with wild-type K-ras gene, the diameter was 2–5 mm (average, 2.7 mm). There was a significant difference in the diameter of the main pancreatic duct between patients with and without the mutant K-ras gene (P = 0.0323). Conclusions The incidence of K-ras mutation may be associated with the hypersecretion of mucin.     

Cystic Lymphangioma of the Gallbladder: Report of a Case

Abdominal lymphangioma is usually diagnosed within the first 2 years of life and is extremely rare in adults. The most common location of abdominal lymphangioma is the mesentery, but there are sporadic reports of its development in the gallbladder. A 66-year-old woman was found to have a cystic lesion near the gallbladder. Preoperative studies, including endoscopic ultrasonography, computed tomography, and magnetic resonance imaging, showed a tumor with multilocular cystic structure, originating in the gallbladder fossa. The patient underwent exploratory laparotomy, and the mass was resected en bloc with the gallbladder, as there was no evidence of malignancy on intraoperative ultrasonography. Macroscopically, the tumor was a multilocular cystic mass, 6 × 3 × 2 cm in size, with a rough, sponge-like appearance. Histologically, the cystic tumor was diagnosed as a lymphangioma, originating in the gallbladder. To our knowledge, only three other cases of a cystic lymphangioma originating in the gallbladder have been reported in the medical literature of the world.     

Congenital dislocation of the patella

Purpose Congenital dislocation of the patella is permanent and manually irreducible, and it manifests immediately after birth with flexion contracture of the knee, genu valgus, external tibial torsion and foot deformity. We retrospectively reviewed the results of operative treatment of seven knees in six patients with congenital dislocation of the patella. Methods The age of the six patients at diagnosis ranged from 8 days to 3.6 years, with an average of 1.3 years, and their age at the time of operation ranged from 0.6 to 3.9 years, with an average of 2.1 years. Serial casting and/or a brace was attempted before surgery in five of seven knees, leading to improvement in the flexion contracture of the knee. All knees were treated operatively in combination with lateral release, medial plication, V-Y lengthening of the quadriceps, medial transfer of the lateral patellar tendon and posterior release of the knee. Results Although these deformities were noticed at birth in all seven knees, diagnosis was delayed in three knees due to the low suspicion of the disease and invisible patellae on radiographs. Ultrasonography confirmed the diagnosis of dislocation. The patella was centered in the groove of the femoral condyle after surgery in all knees, but subluxation of the knee with flexion was observed in one knee in which the operation was performed at 3.9 years. Genu valgus and external tibial torsion improved after surgery in all knees. The operated knee was mobile in all cases, with less than 10° flexion contracture of the knee. Flexion contracture did not increase in any of the knees. Conclusion Congenital dislocation of the patella should be suspected in every patient with knee flexion contracture, genu valgus, external tibial torsion, foot deformity and delayed walking. Successful results were obtained when the operation was performed in younger children. Other procedures, such as the semitendinosus tenodesis or tendon transfer, might have to be combined to achieve better stability with flexion in older children. None of the authors received financial support for this study.     

Acute disappearance of ruptured aneurysm located near the origin of the superior cerebellar artery - case report -

A 63-year-old woman presented with a ruptured aneurysm which apparently spontaneously thrombosed. She was admitted after sudden onset of severe headache. Computed tomography (CT) demonstrated subarachnoid hemorrhage (SAH) in the pontine and interpeduncular cisterns. Initial three-dimensional CT (3D-CT) angiography revealed an aneurysm (diameter, 9 mm) near the origin of the left superior cerebellar artery. However, angiography 3 hours later failed to show the aneurysm. Total thrombosis was thought to have occurred in the aneurysm. The patient returned home with no deficit 1 month after admission. T(1)- and T(2)-weighted magnetic resonance imaging 75 days after the SAH demonstrated the thrombosed aneurysm as an isointense mass lesion. 3D-CT angiography showed no recanalization of the aneurysm 9 months after the SAH.     

Operative reconstruction of the severe sequelae of infantile septic arthritis of the hip

We retrospectively reviewed the results of operative reconstruction of 21 hips in 21 patients with severe sequelae due to infantile septic arthritis of the hip. Eleven hips were classified as Choi type IIIA, 4 as type IIIB, 1 as type IVA, and 5 as type IVB sequelae. The average age at the time of the first surgery was 4.2 years, and the average follow-up period was 8.9 years. Successful results were obtained in 8 of the 11 type IIIA hips by means of a combination of open reduction, femoral varus osteotomy, and pelvic osteotomy. However, only 2 of the 4 type IIIB hips treated by femoral valgus osteotomy and/or bone grafting and 2 of the 6 types IVA and IVB hips treated by greater trochanter arthroplasty had successful results.     

Antegrade selective cerebral perfusion with mild hypothermic systemic circulatory arrest during thoracic aortic surgery

Objective Antegrade selective cerebral perfusion (ASCP) and retrograde cerebral perfusion (RCP) have proven to be reliable methods of brain protection during aortic surgery. These techniques are usually accompanied by systemic circulatory arrest with moderate hypothermia (24–28°C) or deep hypothermia (18–24°C). However, hypothermia can lead to various problems. The present study therefore reports results for thoracic aorta replacement using ASCP with mild hypothermic systemic arrest (28–32°C).

Design Between 1995 and 2003, 68 consecutive patients underwent repair of the ascending aorta and/or aortic arch. Mild hypothermic ASCP was utilized in 31 cases, moderate hypothermic ASCP in 20, and deep hypothermic RCP in 17. Various parameters were compared between the mild hypothermic ASCP, moderate hypothermic ASCP, and RCP.

Results Hospital mortality was 10.3%, with no significant differences observed between any groups. Permanent neurological dysfunction was 8.8%, and no significant differences were observed between any groups. Mild hypothermic ASCP displayed significantly decreased transfusion volume, intubation time, and ICU stay.

Conclusions Use of ASCP with mild hypothermic systemic circulatory arrest during aortic surgery resulted in acceptable hospital mortality and neurological outcomes. ASCP with mild hypothermic arrest allows decreased transfusion volume and reduced duration of intubation and ICU stay.