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. .

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.

[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.

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.

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.

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.

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.

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.

Deletion involving the TWIST locus and the HOXA cluster: a contiguous gene syndrome on 7p?

Deletion of TWIST on 7p21 leads to Saethre-Chotzen syndrome, whereas deletion of the HOXA cluster on 7p15.2 leads to hand-foot-genital syndrome. We report here a patient with 46,XY,del(7)(p15.2p21) who had craniosynostosis, maxillary hypoplasia, prominent ear crus, rectoperineal fistula, and hypoplastic fifth fingers. Using fluorescence in situ hybridization, we demonstrated the deletion to encompass the TWIST locus and the HOXA cluster. We suggest that many, if not all, of the features of this patient could be accounted for by combined haploinsufficiency of the TWIST and HOXA cluster. Hence, the patient's phenotype may define a new contiguous gene syndrome on 7p.

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.

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.

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.

Surgical treatment of methicillin-resistant Staphylococcus aureus infection following infrainguinal arterial reconstruction.

A 64-year-old man was referred to our hospital with Methicillin-resistant Staphylococcus aureus (MRSA) infection following infrainguinal arterial reconstruction. As repeated MRSA sepsis occurred, we decided to remove the infected graft with distal revascularization via circuitous graft tunneling to avoid serious infections and allow limb salvage. An iliofemoro bypass was performed via an extra-anatomical bypass, from just below the iliac crest into the musculus quadriceps femoris using an 8 mm-ringed polyester gelatin polypropylene tube graft, with complete debridement of a groin infection. Postoperative 3-dimentional CT angiography revealed that the prostheses was patent and the patient had an uneventful postoperative course. We concluded that this extra-anatomical bypass was a safe procedure and an excellent option for patients with an infected vascular prosthetic graft in the groin after previous revascularization, like in our case with no available autogeneous vein grafts.

Chronic expanding hematoma in the pericardial cavity after cardiac surgery.

We report the successful surgical treatment of a rare case of chronic expanding hematoma in the pericardial cavity that developed into a very large mass over a long period. The patient, who had a history of cardiac surgery for a double-outlet right ventricle 14 years previously and for tricuspid regurgitation 8 years ago, noticed a slowly growing mass near the left atrium and ventricle 5 years ago. The mass, which confirmed a diagnosis of chronic expanding hematoma, was resected by left thoracotomy.

Numerical Simulation of Nonpulsatile Left Ventricular Bypass.

A computer simulation was carried out to investigate the influence of nonpulsatile left ventricular assistance on hemodynamics. A simulation circuit was constructed to represent the circulatory system. A source of current was added to denote the nonpulsatile blood pump. The left and right ventricles were replaced by variable compliances. Left heart failure was simulated by decreasing the amount of compliance change of the left ventricle. We introduced a pulsatility indicator (PI) to clarify the pulsatility characteristics in the hemodynamics; this PI was defined as the ratio of the pulse pressure (PP) to the mean aortic pressure (AoP). When nonpulsatile bypass flow increased, the mean AoP, tension time index (TTI), and diastolic pressure time index (DPTI) increased, and cardiac output, PP, and PI decreased. When assisted flow increased with the constant total flow rate, the mean AoP and DPTI changed little; the PP, TTI, and PI decreased, and the endocardial viability rate increased. The PI would be helpful in evaluating the effect of pulsatility.

Surgical treatment of virulent descending necrotizing mediastinitis.

We report a 58-year-old male treated with surgical drainage by mediansternotomy using a pedicled omental flap for descending necrotizing mediastinitis (DNM). The patient recovered from DNM after five months of mechanical respiratory support. In deciding upon the most appropriate surgical approach for mediastinal drainage, the level of infection is a good landmark and should be investigated by CT scan. We also review the 43 cases of successful surgical treatment of DNM reported since 1989 in Japan, including our own patient, who were diagnosed with DNM by CT scan according to the classification proposed by Endo et al., and discuss the most appropriate surgical approach for mediastinitis based on the literature. In the treatment of DNM localized to the upper mediastinal space above the carina, a transcervical approach may be appropriate. In diffuse DNM extending into the lower anterior mediastinum, a mediansternotomy or a thoracotomy may be useful, and in diffuse DNM extending into both the anterior and posterior lower mediastinum, a thoracotomy may be the best approach for debridement of the lower posterior mediastinum, in addition to early complete debridement of the entire cervical area.

Primary malignant lymphoma arising in the pleura without preceding long-standing pyothorax.

We report a very rare case of primary malignant lymphoma arising in the pleura with no history of persistent pyothorax. A 72-year-old male was hospitalized with dyspnea on effort and chest CT demonstrated a mass along the right chest wall. Right thoracotomy with complete en bloc resection of the pleural tumor was performed. Immunohistochemical examination of the pleural tumor showed that the histology was marginal zone B-cell malignant lymphoma. We considered that this tumor had originated from the soft tissue in the chest wall based on radiographical and surgical findings. As diagnosis and treatment of pleural malignant lymphoma seems to have been difficult in most cases reported in the literature, it is thought that early active and accurate biopsy with large-bore needles, or, if possible, surgical incision for early diagnosis and aggressive surgery to achieve complete resection combined with radiation therapy and/or chemotherapy would be very important for a good prognosis.

A tracheostomy case of aortic valve regurgitation successfully treated by a lower mini-sternotomy technique.

The performance of cardiac surgical procedures via median sternotomy in a patient with a tracheostomy can result in difficult problems, such as mediastinitis, stoma necrosis or inadequate operative exposure. We present a case of successful treatment for aortic valve regurgitation in a patient with a tracheostomy using a T-shaped sternotomy instead of a usual median sternotomy. This approach permitted adequate surgical exposure for cardiopulmonary bypass and aortic valve replacement. After surgery, the patient had a good clinical course without any complications and he was discharged on the 65th postoperative day. We consider this procedure to be applicable in consideration of the favorable results obtained.

Surgical repair of a common atrium in an adult.

We report a rare successful surgical repair of a common atrium (CA) with mild tricuspid valve (TV) regurgitation due to valvular annulus enlargement in a 39-year-old man, who had a complete atrial septum defect (ASD) without the characteristic of an endocardial cushion defect. The left-to-right shunt ratio was 85 percent and the Qp/Qs was 6.7 due to the CA. Left ventriculogram revealed no evidence of typical goose-neck deformity and no mitral valve regurgitation. The operation consisted of making a new atrial septum with an autologous pericardial patch and tricuspid annuloplasty (DeVega) using extracorporeal circulation. There was no evidence of a cleft on the anterior leaflet of the mitral valve or the septal leaflet of the TV. The postoperative echocardiogram showed no residual shunt flow through a new atrial septum and no TV regurgitation, and atrioventricular (AV) dissociation did not occur. We consider this procedure to be widely applicable in consideration of the favorable results obtained after surgical treatment.