A case report of anesthesia for a child with Hajdu-Cheney syndrome.

Hajdu-Cheney syndrome is an extremely rare disorder characterized by progressive skeletal acro-osteolysis, which results in extremity fractures and scoliosis often requiring surgical treatment from childhood. A unique facial structure and deformity of the cervical spine is associated with a difficult airway. We report here a 10-year-old girl with Hajdu-Cheney syndrome who developed progressive basilar impression and medullary compression for which foramen magnum decompression was performed. After slow induction of anesthesia, we were able to perform fiberoptic orotracheal intubation via a VBM bronchoscope airway. This case report contributes to the accumulation of knowledge about anesthesia for this rare syndrome.

[Carotid artery stenting].

Carotid artery stenting (CAS) is used as a less invasive substitute for carotid endarterectomy (CEA) and is the treatment of choice for patients with severe carotid artery stenosis in whom CEA might accompany a high risk of complications. Although evidence showing that CAS is more effective than CEA is scarce, CAS has been widely used in Japan in the past 5 years. The development of carotid sinus reflection, cerebral hyperperfusion syndrome, and myocardial ischemia must be carefully monitored to reduce the primary procedural causes of mortality and morbidity. Strict perioperative management by anesthesiologists may improve the treatment outcome of CAS. In future, if CAS is proven superior to CEA, there is a possibility that CAS would become the first-line treatment for carotid artery stenosis.

[Langerhans cell histiocytosis of the rib in adult male].

A 57-year-old man was admitted to our hospital with an abnormal shadow on chest X-ray film. Computed tomography (CT) scanning demonstrated a low-density, destructive mass on the right 8th rib. The maximum standardized uptake value of the tumor measured by positron emission tomography (PET) was 2.9, indicating malignancy. Wide resection of the tumor, including the right 8th rib and the 7th to 8th intercostal muscle, was performed. Chest wall reconstruction was achieved with Composix Mesh. The histologic findings revealed proliferation of histiocytes and eosinophil infiltration. No malignant cells were detected in the tumor. Histiocytes stained for S-100 protein and CD1a, compatible with a diagnosis of Langerhans cell histiocytosis (LCH). LCH in the ribs is very rare and difficult to diagnose using CT or PET. Tumor biopsy or resection is needed to diagnose LCH.

[Two successful cardiovascular surgeries in Jehovah's witnesses].

Anesthesia for Jehovah's witnesses is sometimes problematic, especially when they have an open heart surgery. We could successfully manage two Jehovah's witnesses who underwent mitral valve replacement and thoracic aneurysm repair without transfusion. Prior to surgery, it is crucial for a operation to carefully assess the patient's cardiovascular reserve, estimated hemorrhage volume, permissive range of hemorrhage, alternative methods of blood transfusion, and risk of death.

[Combined surgery of off-pump coronary bypass graft with splenectomy in a patient with refractory immune thrombocytopenic purpura].

Coronary artery bypass graft surgery in patients with immune thrombocytopenic purpura (ITP) refractory to preoperative medical therapy accompanies an increased risk of bleeding perioperatively. In the ITP patient without responding to intravenous immunoglobulin and corticosteroids, we performed combined off-pump coronary artery bypass grafting with splenectomy to minimize the risk of intraoperative bleeding and cardiac ischemia. Using platelets infusion, we successfully managed the patient without major bleeding and ischemic events.

Sarcomatoid adenocarcinoma of the lung: clinicopathological, immunohistochemical and molecular analyses.

BACKGROUND: Biphasic lung cancers with admixtures of adenocarcinoma and sarcomatoid components but lacking true mesenchymal differentiation (sarcomatoid adenocarcinomas) are rare, and extensive studies of their clinicopathological characteristics and histogenesis have not been performed. MATERIALS AND METHODS: Six surgically resected sarcomatoid adenocarcinomas were compared clinicopathologically with 317 standard adenocarcinomas, and studied immunohistochemically and by loss of heterozygosity (LOH) analysis focusing on each component. RESULTS: In comparison with standard adenocarcinomas, the sarcomatoid adenocarcinomas occurred in older patients, were more likely to be associated with smoking and were more acinar than papillary, with a worse five-year prognosis. Immunohistochemically, sarcomatoid components were positive for epithelial markers in three cases. In one case the carcinoma showed retention of heterozygosity and the sarcomatoid components allelic loss, while all other cases showed retention or LOH in both components. CONCLUSION: The sarcomatoid adenocarcinoma exhibits different clinicopathological characteristics from standard adenocarcinomas. The present immunohistochemical and LOH analyses provided support for the idea that both components are derived from one precursor cell, and that progression may be by way of adenocarcinoma to sarcomatoid differentiation.

Cambio morfológico del cáncer rectocolónico con aumento de tamaño en cada histogénesis: análisis histopatológico y estadístico de 5.000 carcinomas que miden menos de 2 cm. de diámetro máximo / Morfological changes in colorectal cancer size increase in histogenesis: statistical and histopathological analysis on 5.000 carcinomas with 2cm maximun diameter

La histogénesis del cáncer colorectal tiene aspectos controversiales. Su origen como carcinoma preferentemente de novo en contraposición a aquellos originados en adenomas (secuencia adenoma carcinoma) es motivo de permanente análisis. Interesa evaluar las características de un gran número de cánceres colorectales de menos de 2 cm de diámetro máximo respecto a su histogénesis, relación entre tipo macroscópico y tamaño y los cambios histológicos al aumentar de tamaño. Material y método: El grupo de estudio Japonés multicéntrico para afecciones intestinales Shirakabe Forum, que reúne a 26 hospitales del Japón ha recolectado 5.000 carcinomas colorectales que miden menos de 2 cm. de diámetro. Los diagnósticos histológicos fueron revisados por el grupo de patólogos de cada hospital de acuerdo a una pauta común. Se aplicó la clasificación según la Sociedad Japonesa para el estudio del cáncer colorectal temprano y avanzado. Resultados. En carcinoma de novo constituía el 60 por ciento del total de los cánceres y el 80 por ciento de los menores de 5mm. Lo cual se relacionaría con su histogénesis. En cánceres de tipo pediculado el cáncer de novo representa el 30 por ciento y el originado de adenoma el 70 por ciento, por el contrario, los carcinomas de tipo de depresión con o sin protusión en su gran mayoría son de novo 96 por ciento. Las depresiones en las lesiones de menos de 2 cm, deben interpretarse como carcinomas de novo al sugerir mucosectomía versus colectomía. 82 por ciento de los cánceres de novo muestran protrusión plana o depresión con o sin potrusión. Son pediculados solo en el 18 por ciento. El 48 por ciento de los cánceres originados en el adenoma son pediculados y solo el 2 por ciento son deprimidos. La mayoría de los cánceres de novo al crecer se presentan como depresión con o sin protrusión. La mayoría de los originados en adenoma no se transforman al crecer en tipo de depresión con o sin protrusión, el 60 por ciento crece a pólipo pediculado. 9 por ciento de los microcarcinomas comprometen la submocosa. En los menores de 2 cm. de novo, el 30 por ciento compromete la submocosa y el 10 por ciento la muscular de la mucosa. El carcinoma de novo comienza a comprometer la submucosa al medir, más de 0,6 cm. de diámetro máximo.