Mitral Annular Calcification in Marfan Syndrome: A 38-Year Follow-Up Observation.

Background Mitral annular calcification (MAC) associated with Marfan syndrome is rare in comparison with that frequently found in elderlies with valvular disease. Case Presentation A 17-year-old woman with Marfan syndrome underwent mitral valve replacement for severe mitral regurgitation. Preoperative examination showed mitral valve prolapse and a dense C -shaped MAC. We evaluated MAC and adjacent area, respectively, in postoperative 18 and 38 years, and neither progression nor expansion of calcification was observed. Conclusion MAC associated with Marfan syndrome is more likely to be caused by stresses due to valve prolapse than by connective tissue disorder.

[Total Arch Replacement with Frozen Elephant Trunk for Treatment of Traumatic Aortic Injury with Stanford Type A Aortic Dissection].

An 83-year-old woman was injured in a traffic accident. Enhanced computed tomography (CT) showed aortic injury on the isthmus, Stanford type A aortic dissection and intracranial hemorrhage. Neurological deficit was not noted. We immediately started to lower the blood pressure, and her hemodynamic status remained stable. Although intracranial bleeding had not worsened on the next day, a false lumen of the aortic arch was newly enhanced on CT. Emergency total arch replacement with frozen elephant trunk was performed under deep hypothermia and selective cerebral perfusion. Intraoperative angiography showed no endoleak at the descending aorta. Postoperative course was uneventful, and the patient was discharged on the 21st postoperative day. This procedure is useful for aortic injury involving the ascending aorta and the arch.

[Coronary Artery Bypass Grafting complicated with sternocostoclavicular hyperostosis;Report of a Case].

The patient was 72-year-old man, who had old myocardial infarction, diabetes mellitus, dyslipidemia, hypertension and chronic obstructive pulmonary disease. He was complicated with congestive heart failure, and multi-vessel coronary artery disease and moderate aortic valve stenosis and regurgitation were diagnosed. We performed coronary artery bypass grafting(CABG) and aortic valve replacement. Intraoperative findings showed severe adhesions and tissue fibrosis around sternum. It was very difficult to dissect adhesions around left internal thoracic artery (LITA). LITA was injured, and great saphenous vein was anastomosed to left anterior descending coronary artery. Sternocostoclavicular hyperostosis was diagnosed on computed tomography( CT) findings such as remarkably thickened ster-num and adhesion of sternoclavicular joint. Postoperative course was complicated by osteomyelysis and necrosis of left side skin incision, for which omentopexy was needed. Sternocostoclavicular hyperostosis is rare disease, but we should recognize in preoperative evaluation.

A Case of Ruptured Immunoglobulin G4-Related Periaortitis.

An 80-year-old man had high serum immunoglobulin G4 (IgG4) concentration and fibrous thickening of the soft tissue mass surrounding the region from the abdominal aorta to the bilateral iliac arteries, suggestive of IgG4-related periaortitis. He presented to our emergency department with sudden-onset abdominal pain and lumbago. Computed tomography revealed a ruptured abdominal aorta. He was a poor candidate for open surgery due to his hostile abdomen. Therefore, endovascular aneurysm repair was performed. No consensus about the surgical indication for IgG4-related arterial disease has been reached yet. We believe that a novel indicator is needed for this disease.

[Cardiac Surgery in Two Patients with Parkinson's Disease who were Using Deep Brain Stimulation Devices].

For the treatment of Parkinson's disease, deep brain stimulation( DBS) devices are implanted for the control of motor symptoms including tremor. We performed cardiac surgery in 2 patients with Parkinson's disease who were using DBS devices. Coronary artery bypass was performed in one patient, and closure of ventricular septal perforation after acute myocardial infarction was performed in the other. There is a risk of injury and electromagnetic interference of DBS devices. No device failure or aggravation of Parkinson's symptom was observed in these cases. In many cases of cardiac surgery, various devices are concomitantly used, and the potential interference with the devices should be carefully examined in perioperative management.

Ascending to Abdominal Aorta Extraanatomic Bypass for Descending Aortic Coarctation: A Reconstruction Technique without Laparotomy or Left Thoracotomy.

The descending aortic coarctation is often difficult to anatomically reconstruct. We report two cases of ascending aorta to abdominal aorta bypass without laparotomy or thoracotomy. This approach enabled us to avoid anastomosis close to the inflammatory lesion and left thoracotomy causing bleeding from the collateral vessels, and to allow concomitant cardiac procedures to be performed. The graft contact with the intestines can be preventable by the retroperitoneal approach. This technique is useful for the selective patients.

Association between metabolic syndrome and male overactive bladder in a Japanese population based on three different sets of criteria for metabolic syndrome and the Overactive Bladder Symptom Score.

OBJECTIVE: To investigate whether metabolic syndrome is a risk factor for overactive bladder (OAB) defined by the Overactive Bladder Symptom Score (OABSS). METHODS: A digital rectal examination of the prostate and an OABSS questionnaire were conducted in 1031 men who visited our hospital for metabolic screening from April 2009 to March 2010. The OABSS includes scores for daytime frequency, nighttime frequency, urgency, and urgency incontinence. Relationships of OAB (defined as OABSS ≥3 with an urgency score ≥2) with metabolic syndrome diagnosed by Japan Society for the Study of Obesity (JASSO), National Cholesterol Education Program-Adult Treatment Panel III (NCEP-ATP III), and International Diabetes Federation (IDF) criteria were examined. The severity of OAB symptoms was compared among younger, middle-aged, and elderly men (<50, 50-64, and ≥65 years old, respectively) with and without metabolic syndrome. RESULTS: Diagnoses of metabolic syndrome were made in 16.4%, 16.9%, and 12.0% of the men using JASSO, NCEP-ATP III, and IDF criteria, respectively. Regardless of the presence of metabolic syndrome, aging was significantly associated with increased rates of moderate or severe OABSS findings and OAB defined by the OABSS. In middle-aged men, metabolic syndrome had a significant negative association with OAB rate. In elderly men, metabolic syndrome had a significant negative association with the total OABSS. CONCLUSION: A relationship between age and OAB was observed, but metabolic syndrome did not show a clear association with OAB. Our results suggest that OAB is associated with aging regardless of the presence of metabolic syndrome.

Comparison of medically versus surgically treated acute type a aortic dissection in patients <80 years old versus patients ≥80 years old.

Although recent progress in emergency surgery has resulted in an increase in the indication for older patients with acute type A aortic dissection (AAD), some patients remain who cannot undergo surgical treatment and little is known about the prognosis of patients with AAD who receive medical treatment, especially in elderly patients. Of the 82 patients with AAD who were admitted to our institution, 48 received medical therapy only. We retrospectively reviewed their clinical data and analyzed the prognostic value of the clinical characteristics in both younger and older patients. The in-hospital and 1-year mortality were significantly lower in the patients who underwent surgical treatment than in those who received medical treatment (6% vs 65%, p <0.001; 8% vs 73%, p <0.001, respectively). Of the patients with medical treatment, the in-hospital and 1-year mortality rate in the younger (<80 years old, n = 27) and older (≥80 years old, n = 21) groups was 70% and 80% and 57% and 65%, respectively. For the younger group, the presence of an open false lumen was significantly associated with in-hospital mortality (89% vs 50%, p = 0.044). In contrast, in the older group, a lower serum albumin level (3.4 ± 0.3 vs 4.0 ± 0.5 g/dl, p = 0.010) and the incidence of an open false lumen (83% vs 33%, p = 0.032) were significantly associated with in-hospital mortality. In conclusion, in addition to an open false lumen as a risk factor, a lower serum albumin level is an important prognostic factor in older patients with AAD.

Association between metabolic syndrome and male lower urinary tract symptoms in Japanese subjects using three sets of criteria for metabolic syndrome and International Prostate Symptom Score.

OBJECTIVES: To investigate whether the metabolic syndrome is a risk factor for lower urinary tract symptoms (LUTS), as defined by the International Prostate Symptom Score (IPSS). METHODS: A total of 900 men underwent digital rectal examination of the prostate and completed an IPSS questionnaire. These men had visited our hospital for metabolic screening from April 2008 to March 2009. The IPSS includes scores for 3 questions on voiding symptoms, 3 on storage symptoms, and 1 on postmicturition symptoms. The relationships of the LUTS (determined from the IPSS subscores) with the metabolic syndrome diagnosed using the Japan Society for the Study of Obesity, 2005 National Cholesterol Education Program-Adult Treatment Panel III, and 2005 International Diabetes Federation criteria were examined. The severity of LUTS was compared among the younger, middle-age, and older men (<50, 50-64, and ≥65 years old, respectively) with and without the metabolic syndrome. RESULTS: A diagnosis of the metabolic syndrome was made in 16.7%, 16.6%, and 11.7% of the men using the Japan Society for the Study of Obesity, 2005 National Cholesterol Education Program-Adult Treatment Panel III, and 2005 International Diabetes Federation criteria, respectively. Regardless of the presence of the metabolic syndrome, aging was significantly associated with an increased rate of moderate or severe LUTS, except for postmicturition symptoms. In the middle-age men, the metabolic syndrome had a significant negative correlation with storage symptoms (odds ratio 0.258-0.426). In the younger and older men, LUTS was observed equally in those with and without the metabolic syndrome. CONCLUSIONS: A relationship between age and LUTS was observed; however, the metabolic syndrome did not show a clear association with LUTS. Our results suggest that LUTS is associated with aging, regardless of the presence of the metabolic syndrome.

Facilitation of expulsion of ureteral stones by addition of α1-blockers to conservative therapy.

OBJECTIVE: An antispasmodic agent and a medicine that facilitates stone expulsion are given commonly as conservative therapy for ureteral stones in Japan. The goal of this study was to compare the efficacy of the addition of various α(1)-blockers to the conservative therapy for spontaneous passage of ureteral stones. MATERIAL AND METHODS: The subjects were 132 patients with stones from the upper to the lower ureter who were randomly placed into one of four groups and followed for 1 month to assess spontaneous passage of stones. The control group received daily doses of 240 mg flopropione as an antispasmodic agent and 1350 mg extract of Quercus salicina Blume/Quercus stenophylla Makino as a medicine that facilitates stone expulsion. The other three groups received this therapy and daily doses of 30 mg urapidil, 0.2 mg tamsulosin or 50 mg naftopidil, respectively. The characteristics of the stones and stone expulsion were evaluated by urinalysis, a kidney, ureter and bladder (KUB) X-ray, ultrasound and computed tomography. RESULTS: All patients completed the study and there were no major side-effects. There was no difference in age, stone position or stone size among the groups. Multivariate analysis using a Cox proportional hazards model indicated that the probability of stone expulsion for 1 month was increased 2.38 times (95% confidence interval 1.23-4.61) by naftopidil compared with control therapy alone (p = 0.01). CONCLUSION: Naftopidil in combination with an antispasmodic agent and a medicine that facilitates stone expulsion produces a significantly increased rate of ureteral stone expulsion.

Primary Ewing's sarcoma/primitive neuroectodermal tumor of the kidney that responded to low-dose chemotherapy with ifosfamide, etoposide, and doxorubicin.

A primitive neuroectodermal tumor (PNET) is a small round cell tumor that arises from the nerve crest. This tumor usually occurs in the central nervous system or soft tissue, but it can occur in the kidney in rare cases. Herein we report a case with severe multiple liver metastases after surgery for right renal PNET. The patient was a 21-year-old man with a chief complaint of right abdominal pain. Hemorrhage in a right renal malignant tumor was diagnosed, and radical nephrectomy was performed. Histopathology showed bare nuclear round atypical cells with a scarce cytoplasm proliferating like a seat and nest. Some of the cells formed a rosette structure and the tumor cells were positive for CD99, leading to diagnosis of PNET. Severe multiple liver metastases occurred 6 months after surgery, and six courses of chemotherapy with ifosfamide, etoposide and doxorubicin were performed. After this treatment, residual tumor was removed, but the tumor cells were absent histologically.

Vaginal metastasis of urothelial carcinoma found incidentally during transurethral resection of a bladder tumor.

A 71-year-old woman visited our hospital for routine follow-up cystoscopy and urine cytologic examination. During the preceding 3 years she had undergone left nephroureterectomy for a tumor of the left renal pelvis and had undergone transurethral resection three times for tumors of the urinary bladder and urethra. A small flare region on the posterior wall of the bladder was found with regular cystoscopy, and urine cytologic examination was positive for malignant cells. Computed tomography showed no distant metastasis and no right upper urinary tract mass. Transurethral resection was performed for the small flare region in May 2006. A papillary tumor of the vaginal wall was found incidentally during transurethral resection, and, therefore, transvaginal resection of the tumor was performed at the same time. Histologic examination of the excised genital lesion showed a G3 pT2 urothelial carcinoma, and the patient has been under observation since completion of external radiotherapy for the vaginal metastatic nodule.

Outcome of surgical treatment in patients with acute type B aortic dissection.

BACKGROUND: Surgery for acute type B aortic dissection is associated with significant mortality and morbidity. The purpose of this study was to assess the clinical outcome of surgical management of complicated acute type B aortic dissection. METHODS: During the last 5 years, 112 patients were admitted for acute type B aortic dissection. Of these patients, 24 consecutive patients were enrolled who underwent surgical management during the acute or subacute stage. The mean age was 66.7 +/- 9.1 years; 8 patients were female. Indications for surgery were rupture in 10 patients, impending rupture in 7, and malperfusion in 7. Fifteen patients were transferred from another hospital. The overall clinical outcome including morbidity, aorta-related events, and death were retrospectively assessed. RESULTS: The mean duration from the time of onset to surgery was 7.1 +/- 9.0 days. Graft replacement of the aorta included the total aortic arch with cerebral perfusion in 6 patients, and replacement of the distal aortic arch or descending aorta with left heart bypass in 12. The remaining 6 patients underwent peripheral bypass for ischemia. Significant complications occurred in 7 patients (24.8%). The operative mortality rate was 8.3% (2 of 24); 5.6% (1 of 18) with central operation and 16.7% (1 of 6) with peripheral operation. The 5-year survival rate was 82.6 +/- 7.9% and freedom from aorta-related events at 1 and 5 years were 95.2% +/- 4.7% and 68.0% +/- 16.6%, respectively. CONCLUSIONS: Surgical management of patients with complicated acute type B dissection has an acceptable perioperative risk and survival. This study suggests earlier surgery with left heart bypass may be beneficial for appropriate patients.

A case of seminal vesicle cyst associated with ipsilateral renal agenesis diagnosed during an investigation of perineal pain.

A 38-year-old man was referred to our hospital with perineal and micturition pain. Transrectal ultrasound (TRUS) revealed a cystic mass in the outer prostate. Pelvic cyst and left renal agenesis were confirmed by magnetic resonance imaging (MRI) and computerized tomography (CT), and we diagnosed seminal vesicle cyst. The symptoms have since subsided, the possibility of sterility has been ruled out, and the patient is showing good improvement.

A case of asymptomatic cystitis glandularis found incidentally with ultrasonography at a private clinic.

A 46-year-old man was transferred to our hospital because of a bladder mass. The mass could not be distinguished from a primary bladder tumor or a tumor invading from another organ with computed tomography, magnetic resonance, or cystoscopic examination. Transurethral resection of the mass was performed, and the pathological diagnosis was typical cystitis glandularis. The patient has been followed up with cytologic examination and ultrasonography, and after 10 months there has been no new growth of the mass or malignant change.

Urethral stent (Angiomed-Memotherm) implantation in high-risk patients with urinary retention.

Transurethral resection of the prostate (TUR-P) has been established as the golden standard for the treatment of urinary retention in patients with benign prostatic hyperplasia (BPH). However, TUR-P is not performed on patients with certain high-risk complications. We have obtained favorable results using urethral stent (Angiomed-Memotherm) implantation to treat high-risk urinary retention patients. Here, we review the results obtained on 15 patients treated using this procedure. Two patients experienced recurrent urinary retention; in one patient, the stent had to be removed due to stone formation; in the remaining 12 patients, urination was favorable after stent implantation. Also, urethral stent implantation was found to be useful in 4 of the 7 patients with neurogenic bladder (rather than BPH) as the underlying disease. The present technique is convenient and noninvasive, and we strongly believe that it can improve the patient's quality of life (QOL) by facilitating urination in high-risk patients who would otherwise require urethral catheterization.

Diagnosing the location of carcinoma in situ (CIS) of the urinary bladder using pirarubicin hydrochloride.

INTRODUCTION: No established technique for locating solitary carcinoma in situ (CIS) of the urinary bladder or CIS accompanying bladder cancer has been determined. Here we investigated whether the location of CIS of the urinary bladder can be macroscopically ascertained by instilling pirarubicin hydrochloride (THP) into the urinary bladder. PATIENTS AND METHODS: We dissolved 50 mg of THP in 50 ml of distilled water, and instilled the resulting solution into the urinary bladder. After 5 min, the urinary bladder is examined using a cystoscope. The study group consisted of 30 subjects (23 men and 7 women). RESULTS: THP uptake was seen in 19 flat (nontumorous) areas of the bladder mucosa in 13 patients. Of these, 11 lesions in 6 patients were confirmed to be CIS. THP uptake was also seen in flat malignant lesions such as bladder cancer invasion into the prostatic urethra, and in benign lesions such as chronic cystitis and urothelial hyperplasia. CONCLUSIONS: The present method can be useful to find easily and macroscopically the location of flat malignant lesions such as CIS.

New ultrasonic cystofiberscope for staging bladder tumors.

We report on a new ultrasonic cystofiberscope for staging bladder tumors. The endoscope makes it possible to view endoscopic and ultrasound images of bladder tumors at any location, either alternately in full screen or simultaneously on the same monitor, with higher resolution. The cystofiberscope is useful for assessing the depth of bladder tumor invasion accurately.

Identification of the layers of the bladder wall on high-frequency endoluminal ultrasonography by a needle puncture experiment.

The purpose of this study was to identify the layers of the bladder wall on high-frequency endoluminal ultrasonography (ELUS). We performed a needle puncture experiment using five normal pig bladders. The histologic layer structure was compared with the ELUS images obtained using a 30-MHz miniature transducer. The bladder wall was depicted as five layers by ELUS and these corresponded to the histologic layers. The first layer (hyperechoic) was a margin echo that included the epithelium and the upper part of the lamina propria. The second layer (hypoechoic) corresponded to the lamina propria and the third layer (hyperechoic) was a margin echo that included the upper part of the muscle layer. The fourth layer (hypoechoic) corresponded to the muscle layer, and the fifth layer (hyperechoic) was a margin echo that included the upper part of the adventitia.

Transitional cell carcinoma of the bladder in four patients on maintenance hemodialysis.

We report four patients on maintenance hemodialysis (HD) with transitional cell carcinoma (TCC) of the bladder. Three patients underwent transurethral resection (TUR) of their tumors, which were grade 2 or 3, stage pT1 TCC. Among them, one patient underwent repeat TUR for recurrent superficial TCC. The remaining one patient underwent total cystectomy for grade 3, stage pT4 TCC and squamous cell carcinoma of the bladder. Subsequently, he died suddenly without evidence of local recurrence or systemic metastasis. We discuss the relationship between the duration of HD and the tumor grade and stage of primary bladder TCC in maintenance HD patients.