A vegetation in a unique location without exposure to regurgitation or a shunt jet: A case report.

Bacteria can adhere to cardiac endothelium damaged by regurgitation or a shunt jet; however, healthy cardiac endothelium is supposedly resistant to bacterial adhesion. A 22-year-old man presented to our emergency department with fever. Physical examination revealed no obvious cardiac murmur, but there was evidence of splinter hemorrhages and Janeway lesions. Transthoracic echocardiography did not reveal vegetative lesions, but a 15 × 7-mm vegetation was identified on the surface of the left ventricular muscle just below the anterolateral commissure of the mitral valve without regurgitation or a shunt jet by means of transesophageal echocardiography. Surgery was performed on the seventh day, but the patient's postoperative course was unstable. Some complications occurred because the vegetation existed in a unique location. Although the patient continued to have an uncontrollable infection over the subsequent course, he was discharged on the 94th hospital day. We present a case of a vegetation in a unique location without exposure to regurgitation or a shunt jet. This case indicates that vegetative lesions may develop even in the absence of regurgitation and shunt jets. In case of infective endocarditis where a vegetation exists in a unique location, comprehensive testing or strategy are required to treat this condition. .

Patient with terminal 9 Mb deletion of chromosome 9p: refining the critical region for 9p monosomy syndrome with trigonocephaly.

We describe a patient with typical manifestations of 9p monosomy syndrome, including trigonocephaly and sex reversal. Array comparative genomic hybridization (CGH) revealed a 9p terminal deletion of approximately 9 Mb with the breakpoint at 9p23. We compared the deleted segments of 9p associated with reported cases of 9p monosomy syndrome with trigonocephaly. We did not identify a region that was shared by all patients; however, when only pure terminal or interstitial deletions that did not involve material from any other chromosome were compared, we identified a segment from D9S912 to RP11-439I6 of approximately 1 Mb that was deleted in every patient. We propose that this 1-Mb segment might be the critical region for 9p monosomy syndrome with trigonocephaly.

An extremely large coronary aneurysm associated with a quadricuspid aortic valve in an adult patient.

A 68-year-old woman exhibited an increasingly protruding mass on the left heart border on chest X-ray. Transthoracic echocardiography revealed an echo-free mass in the anterior pericardial space. Transesophageal echocardiography revealed blood flow from the proximal left anterior descending coronary into a large coronary artery aneurysm measuring 61 mm × 51 mm in diameter and a quadricuspid aortic valve with a small cusp between the left and right coronary cusps. Coronary angiography demonstrated the presence of a coronary aneurysm connected to the proximal left coronary anterior descending artery. A giant coronary artery aneurysm and pulmonary artery fistulas extending from the left and right coronary arteries were confirmed by surgeons and successfully treated with surgery.

Safety of the paravertebral block in patients ineligible for epidural block undergoing pulmonary resection.

OBJECTIVE: We previously reported the noninferiority of paravertebral block (PVB) to epidural block. In this study, we assessed whether PVB via an intrathoracic approach was also safe for the patients ineligible for epidural block because of, for example, anticoagulation or antiplatelet therapy. METHODS: Patients admitted to our hospital for pulmonary resection between April 2010 and March 2012, and who were ineligible for epidural block for various reasons, were enrolled in this study. A catheter for PVB was inserted in the operative field by the surgeons just before closing the chest. Ropivacaine of 0.2 % was injected at 4 ml/h using an infuser pump for 5 days. Concurrent use of intravenous patient controlled analgesia (IVPCA) for 2 days with PVB was permitted as a post-operative analgesic at the discretion of anesthesiologists. We estimated the post-operative complications in these patients. RESULTS: A total of 35 (15.8 %) consecutive patients were enrolled in this study and successfully completed the study protocol. Thirty-two patients received concurrent IVPCA treatment. Post-operative complications due to PVB were not observed, but other complications included 1 incidence of atrial fibrillation, 1 hypertension, 1 pleural fluid accumulation, 1 respiratory failure requiring mechanical ventilation, and 1 of late chest pain requiring intercostal nerve block. CONCLUSION: This study suggests that PVB is safe in patients ineligible for epidural block and can contribute to their pain relief following pulmonary resection procedure including video-assisted thoracic surgery.

Post-traumatic diaphragmatic herniation of the liver, examined by positron emission tomography: case report.

We present a case of post-traumatic diaphragmatic herniation of the liver, which mimicked an intrathoracic tumor. After an automobile accident, the patient underwent thoracotomies for hemothorax and lung cancer in the right chest. Seven months later, computed tomography (CT) demonstrated a round tumor in the thorax adjacent to the right diaphragm with a higher density than the liver parenchyma. An intrathoracic tumor including a primary or metastatic lung cancer was suspected. However, positron emission tomography (PET) showed that the uptake of fluorine-18-fluorodeoxyglucose (FDG) was identical to that in the liver, and the tumor appeared to be contiguous with the liver. Thus, we suspected liver herniation. Core needle biopsy revealed liver cells without neoplastic tissue. Upon surgical exploration, herniation of the liver was found and repaired. PET was helpful in providing morphological and functional information leading to accurate diagnosis of liver herniation in this unusual case.

Inflammatory pseudotumor suspected of lung cancer treated by thoracoscopic resection.

We report a relatively rare surgical treatment for two cases of inflammatory pseudotumors of the lung. In case 1, a 52-year-old male with a history of left chest pain was admitted to our hospital for an abnormal nodule with an irregular margin that was detected in the left upper lung field. The nodule, measuring 15 mm in diameter, was larger than the one observed six months earlier, which had been removed by a thoracoscopic resection. In case 2, a 64-year-old female with a history of chronic cough and hemoptysis was admitted to our hospital, and an abnormal nodule with pleural indentation was detected in the lower left lung field. The nodule, measuring 8 mm in diameter, was also removed by a thoracoscopic resection. In both cases, the histologic examination enabled us to diagnose the lesion as an inflammatory pseudotumor. In general, it is very difficult to differentiate inflammatory pseudotumors from malignant tumors of the lung. The best treatment for inflammatory pseudotumors is usually early and complete surgical resection, since it can lead to improved survival. Therefore, we consider thoracoscopy-aided surgery to be less invasive and more useful than other surgical methods in the diagnosis and treatment of inflammatory pseudotumor of the lung.

Treatment of iliac artery rupture during percutaneous transluminal angioplasty: a report of three cases.

We report three cases of iliac artery rupture during percutaneous transluminal angioplasty (PTA). In all three cases, bleeding was temporarily controlled by inflating an angioplasty balloon at the site of bleeding. Two patients underwent subsequent surgical revascularization, and one underwent endovascular stent grafting but ultimately required a surgical bypass. Arterial rupture is a rare but potentially fatal complication of PTA. Although stent grafts for peripheral arteries are not yet covered by Japanese medical insurance, it is a useful treatment for arterial injury during PTA.

Severe Peters Plus syndrome-like phenotype with anterior eye staphyloma and hypoplastic left heart syndrome: proposal of a new syndrome.

Peters Plus syndrome is a very rare autosomal recessive condition characterized by ocular defects (typically Peters anomaly) and other systemic major/minor anomalies. Mutations in the B3GALTL gene encoding beta 1,3-glucosyltransferase have been found in virtually all patients with typical Peters Plus syndrome. We report on a female patient with unusually severe manifestations of Peters Plus syndrome, including anterior eye staphyloma, cleft lip and palate, and hypoplastic left heart syndrome (HLHS). Analysis of the B3GALTL gene revealed no mutation in the patient. To our knowledge, HLHS has not previously been reported in Peters Plus syndrome so far, and anterior staphyloma, a most severe defect of the anterior eye chamber, is also apparently rare in the syndrome. Our patient might represent a new syndrome of severe Peters Plus syndrome-like phenotype with anterior eye staphyloma and HLHS.

[Solitary metachnonous jejunum and duodenum metastasis after surgical resection of lung cancer].

We report a rare case of a 58-year-old man of long-term survival after surgical treatment of solitary metachnonous jejunum and duodenum metastasis from lung cancer. He underwent right upper lobectomy with a diagnosis of lung cancer which was histologically diagnosed as large cell carcinoma (pT4-MONO, stage IIIB). One month after the operation, he suffered from ileus caused by metastasis in the jejunum. Partial resection of the jejunum and postoperative chemotherapy were performed. Two years after the 2nd surgery, another metastatic tumor was found in the duodenum, and pancreatoduodenectomy was performed. The postoperative course of the patient was uneventful without recurrence 6 years after surgical resection of lung cancer.

Surgical resection and reconstruction for primary malignant sternal tumor.

We report a very rare and successful surgery for primary malignant tumor of the sternum. A 73-year-old male, previously healthy, was admitted to our hospital because a chest computed tomography scan detected an abnormal shadow that suggested a sternal tumor destroying part of the sternum body. Aspiration needle biopsy demonstrated a primary sternal chondrosarcoma measuring 3 x 4 cm in diameter. The sternum below the second intercostal space was resected along with a 1-cm width of cartilage below the third rib on each side. Sternal reconstruction was performed with Composix mesh, titanium mesh, and Marlex mesh, using a right pectoralis major muscle flap translation. The patient was extubated just after surgery, and the postoperative course was uneventful. This procedure may be useful for repairing the detect after wide sternotomy.

Cryptic 17q22 deletion in a boy with a t(10;17)(p15.3;q22) translocation, multiple synostosis syndrome 1, and hypogonadotropic hypogonadism.

We report on a boy who had multiple synostosis syndrome 1, an autosomal dominant disorder characterized by progressive symphalangism, multiple joint fusions, conductive deafness, and mild facial dysmorphism. In addition the boy developed delay of puberty, bone age, and closure of the epiphyseal lines of long bones with tall stature. These findings and decreased plasma LH and FSH levels at age 19 years were compatible with hypogonadotropic hypogonadism. G-banded chromosomes showed a balanced translocation t(10;17)(p15.3;q22). Chromosomal FISH analysis, using a series of BAC clones surrounding the translocation breakpoints, detected a 2.2-3.9 Mb deletion at 17q22. The deletion encompassed NOG, a gene responsible for multiple synostosis syndrome 1. It was assumed that a gene for pituitary secretion of gonoadotropic hormones was deleted at the 17q22 segment.

Surgical resection of primary liposarcoma of the anterior mediastinum.

We report on the rare and surgical treatment of a case of primary mediastinal liposarcoma. A 64-year-old male complained of hoarseness for one month and was admitted to our hospital because of an abnormal shadow, which was postulated to be an anterior mediastinal tumor on a chest computed tomography (CT) scan. Horizontal T1-weighted magnetic resonance imaging (MRI) showed an anterior mediastinal round mass with a signal intensity similar to that of subcutaneous fat, which was 6.5 cm in diameter. An operation similar to extended thymectomy was perfomed through a median sternotomy. Histological examination of the resected specimen revealed that the tumor was composed of well-differentiated liposarcoma and pleomorphic malignant fibrous histiocytoma and the tumor was diagnosed as a de-differentiated liposarcoma. We discuss it with reference to a collective review of the Japanese literature for surgical cases of primary liposarcoma of the mediastinum.

Degree of fusiform dilatation of the proximal descending aorta in type B acute aortic dissection can predict late aortic events.

OBJECTIVE: Predicting the risk factors for late aortic events in patients with type B acute aortic dissection without complications may help to determine a therapeutic strategy for this disorder. We investigated whether late aortic events in type B acute aortic dissection can be predicted accurately by an index that expresses the degree of fusiform dilatation of the proximal descending aorta during the acute phase; this index can be calculated as follows: (maximum diameter of the proximal descending aorta)/(diameter of the distal aortic arch + diameter of the descending aorta at the pulmonary artery level). METHODS: Patients with type B acute aortic dissection without complications (n = 141) were retrospectively analyzed to determine the predictors of late aortic events; these include aortic dilatation, rupture, refractory pain, organ ischemia, rapid aortic enlargement, and rapid enlargement of ulcer-like projections. RESULTS: The fusiform index in patients with late aortic events (0.59) was higher than that in patients without late aortic events (0.53, P < .01). Patients with a higher fusiform index exhibited aortic dilatation earlier than those with a lower fusiform index. By multivariate analysis, we conclude that the predominant independent predictors of late aortic events were a maximum aortic diameter of 40 mm or more, a patent false lumen, and a fusiform index of 0.64 or more (hazard ratios, 3.18, 2.64, and 2.73, respectively). The values of actuarial freedom from aortic events for patients with all 3 predictors at 1, 5, and 10 years were 22%, 17%, and 8%, respectively, whereas the values in those without these predictors were 97%, 94%, and 90%, respectively. CONCLUSIONS: The degree of fusiform dilatation of the proximal descending aorta, a patent false lumen, and a large aortic diameter can be predominant predictors of late aortic events in patients with type B acute aortic dissection. Patients with these predictors should be recommended to undergo early interventions (surgery or stent-graft implantation) or at least be closely followed up during the chronic phase before such events develop.

Surgical treatment of infected intralobar pulmonary sequestration: a collective review of patients older than 50 years reported in the literature.

We report on the rare and surgical treatment of a senile patient of infected intralobar pulmonary sequestration. A 56-year-old male who had complained of headache, vomiting, cough, sputum production, and high fever was admitted to our hospital. Chest computed tomography (CT) showed an infected intralobar pulmonary sequestration as an 8x6 cm cystic mass with multiple air-fluid cavities in the left lower basal segment and severe pneumonia in the left upper and lower lobes around the mass. A 3-D CT showed an aberrant artery entering the consolidation from the descending aorta. A standard lower lobectomy was performed with a ligation of the aberrant artery with a diameter of 1 cm supplying the posterior segment of the left lower lobe. A histological examination of the lung revealed acute and chronic broncho-bronchiolitis with cystic dilatation consistent with intralobar pulmonary sequestration. We discuss the characters of senile patients compared with juvenile patients, with reference to a collective review of patients older than 50 reported in the literature.

Intact kinase homology domain of natriuretic peptide receptor-B is essential for skeletal development.

CONTEXT: Natriuretic peptide receptor-B (NPR-B, GC-B in rodents; gene name NPR2) is a guanylyl cyclase-coupled receptor that mediates the effect of C-type natriuretic peptide. Homozygous mutations in human NPR-B cause acromesomelic dysplasia, type Maroteaux (OMIM 602875), an autosomal recessive skeletal dysplasia. NPR-B has an intracellular kinase homology domain (KHD), which has no kinase activity, and its functional significance in vivo is currently unknown. OBJECTIVE: We examined the functional significance of a novel NPR-B KHD mutation in humans. PATIENTS AND METHODS: A 28-yr-old Japanese male presented with marked short stature (118.5 cm, -9.3 sd). His limbs showed marked shortening in the middle and distal segments. His parents had relatively short stature with height z-scores of -2.75 and -0.98 (his father and mother, respectively). Direct sequencing of coding region of the NPR2 gene of the family was performed. The mutant receptor activity was investigated by saturation binding assay and cGMP measurement. Additionally, interaction between the mutant and wild type allele was investigated by the titration experiments. RESULTS: We identified a novel missense mutation L658F in KHD of NPR-B in homozygous and heterozygous states in the patient and his parents, respectively. The mutation conferred normal binding affinity for C-type natriuretic peptide but no discernible ligand-induced cGMP production. Furthermore, L658F mutant impaired wild-type NPR-B-mediated cGMP production in a dose-dependent manner, suggesting that short stature found in L658F heterozygote can be caused by its dominant-negative effect. CONCLUSIONS: This study provides the first evidence that intact KHD of NPR-B is essential for skeletal development.

Spontaneous rupture of the ascending thoracic aorta resulting in a mimicking pseudoaneurysm.

We report on the rare and successful surgical treatment of a case of spontaneous rupture of the ascending thoracic aorta resulting in a mimicking pseudoaneurysm. A 72-year-old male who had complained of sudden onset of severe chest pain was admitted to our hospital. Initially, acute type A closing aortic dissection was suspected because computed tomography (CT) showed a small ulcer-like projection (ULP) in the posterior aspect of the ascending aortic wall, but it also revealed no intimal flap, false lumen or aortic aneurysm. CT and magnetic resonance imaging (MRI) indicated a change in the radiographic aspect of the ULP and revealed a mimicking saccular-type pseudoaneurysm and gradual increasing size of the pseudoaneurysm. Surgery was performed after considering the risk of pseudoaneurysmal rupture. We replaced the ascending aorta and diagnosed it as a spontaneous aortic rupture by histological examination of the rupture site after failing to observe an aneurysm or dissection. We discuss these results with reference to the literature, including our pathological and radiographical findings.

Role of video-assisted thoracic surgery for the diagnosis of indeterminate pulmonary nodule.

PURPOSE: A study was undertaken to evaluate the validity and efficacy of video-assisted thoracic surgery (VATS) for the diagnosis of indeterminate pulmonary nodules. MATERIALS AND METHODS: Between April 2001 and November 2004, 57 adult patients (31 males, 26 females) with a clinical diagnosis of pulmonary nodules by preoperative chest computed tomographic (CT) scanning were included in this study. RESULTS: A definitive tissue diagnosis was obtained in all 57 patients. The mean age of the patients was 66+/-9.8 yrs. The mean size of the tumor was 1.76+/-0.67 cm. 38 patients (67%) had malignant disease. Twenty eight patients (49%) had primary lung cancer and 10 patients (18%) had metastatic lung cancer from an extrathoracic primary neoplasm. The rate of malignancy was 65% in the tumor sizes equal to or less than 2 cm. Among primary lung cancer, most patients had primary adenocarcinoma (24 cases)(86%). Pathologic staging showed T1N0 (stage IA) in 18 cases (69%), T2N0 (stage IB) in 4 cases (15%), T2N1 (stage IIB) in 1 case (4%), and T1N2 (stage IIIA) in 3 cases (12%). In 10 patients (18%) who required preoperative placement of a localization hookwire with a string near the nodule, the rate of malignancy was 70%. In 7 patients with positive positron emission tomography (PET) imaging with F-18 fluorodeoxyglucose (FDG), the rate of malignancy was 57% and proved to be falsely positive in 3 cases (43%). There was no operative mortality and no significant morbidity in all cases. CONCLUSION: We proposed that VATS, which is safe and offers virtually 100% sensitivity and specificity, should be performed for most small (<3 cm in diameter) and all indeterminate pulmonary lesions on the basis of these results.

Surgical treatment of chronic constrictive pericarditis using an ultrasonic scalpel.

A 61-year-old male had complained of cough and dyspnea on effort, with right pleural effusion. Computed tomography demonstrated a calcific pericardium surrounding the entire heart, with thickening of 10 mm. Cardiac catheterization showed no coronary disease, but a dip-and-plateau of the pressure curve of both ventricles. We diagnosed congestive heart failure due to chronic constrictive pericarditis, and performed a subtotal pericardiectomy, using an Ultrasonic Scalpel through a median sternotomy combined with anterior left thoracotomy at the level of the fifth rib, without cardiopulmonary bypass (CPB). After the subtotal pericardiectomy, his postoperative recovery was uneventful and his functional status improved. We consider that our surgical technique is a useful method for treatment of chronic constrictive pericarditis, and advocate the use of the Ultrasonic Scalpel for a safe and easy pericardiectomy.

Refining chromosomal region critical for Down syndrome-related heart defects with a case of cryptic 21q22.2 duplication.

We report here a patient with features of Down syndrome and tetralogy of Fallot who had a 21q22 duplication. The extent of the duplication was defined using fluorescent hybridization probes that map to the critical region on chromosome 21. Included within the interval was the cell adhesion molecule DSCAM but not the collagen COL6A1. The present case provides further support to the concept that there exists Down syndrome-associated congenital heart disease gene(s) on chromosome 21q22 and that over-expression of DSCAM may contribute to the cardiac defects of Down syndrome.

Primary lung cancer arising from the wall of a giant bulla.

We report a 58-year-old man who underwent surgical treatment of primary lung cancer arising from the wall of a giant bulla. Chest roentgenography and computed tomography revealed multiple emphysematous bullae in the bilateral upper lobes, and a right upper giant bulla with a mass measuring 6 cm arising on the bulla wall. Right upper lobectomy was performed, the postoperative pathological diagnosis was large cell carcinoma arising from the wall of a giant bulla. Although the postoperative course was uneventful and he was discharged, he underwent partial resection of the jejunum for recurrence of carcinoma in the jejunum, and postoperative chemotherapy, and he was alive 20 months after that operation. In general, patients with both pulmonary bullous disease and primary lung cancer have a very poor prognosis, because they receive treatment when the tumor is at an advanced stage. On the basis of our review of the literature, we recommend that middle-age male patients with a giant bulla who smoke should have annual chest roentgenography and/or chest computed tomography to screen for lung cancer arising in or close to the bullous disease, and that a giant bulla should be resected in patients older than 50 years because of the high incidence of coexisting cancer and bulla, to improve the prognosis of this disease.