Axillosubclavian vein thrombosis, also referred to as effort thrombosis or Paget–Schroetter syndrome, is the most common presentation of the venous form of thoracic outlet syndrome. With timely diagnostic and therapeutic measures, thrombosis of the axillary or subclavian veins can be treated with minimal long-term morbidity. Case reports and single-center reviews have greatly contributed to our...

A 47-year-old woman diagnosed with Marfan syndrome underwent valve-sparing aortic root replacement for aortic regurgitation and annulo-aortic ectasia. Her cardiac function was normal. Preoperative coronary angiography did not demonstrate any stenosis. The David reimplantation procedure with a 28-mm Valsalva graft was performed. Both coronary orifices were reconstructed in a button fashion with ...

abstract horseshoe lung is a congenital pulmonary malformation that is usually associated with scimitar syndrome. this malformation consists of fusion of both pulmonary lobes from the posterobasal segments. the fusion appears in the retrocardiac area, in front of the esophagus and thoracic aorta. pleural separation of pulmonary lobes distinguishes pseudohorseshoe appearance from a true horsesho...

We present the case of a patient that progressively developed xerophthalmia, xerostomia, cutaneous xerosis and exocrine pancreatic insufficiency 3 months after metamizole-induced toxic epidermal necrolysis. Though the association of Sjögren's syndrome and exocrine pancreatic impairment is well established, the Sjögren-like syndrome after drug-induced toxic epidermal necrolysis in association wi...

Shwachman Diamond Syndrome is a rare congenital disorder characterized by pancreatic insufficiency, bone marrow dysfunction and skeletal abnormalities. No specific test is available for a definitive diagnosis for SDS. In the presence of clinical features of SDS, pancreatic insufficiency should be tested for or lipomatosis radiologically demonstrated. New directions might be found in genetic ana...

Sir, Klinefelter’s syndrome, originally described by Klinefelter et al. in 1942 (1), is the most frequent major abnormality of sexual differentiation in men with two or more X-chromosomes. Its prevalence has been estimated at one male birth in 500. The prevalence of leg ulcers as a result of a venous insufficiency in patients with Klinefelter’s syndrome is about 13% (2). The pathogenesis of thi...

IMAGe syndrome is an exceedingly rare condition first described in 1999. Components of the syndrome are intrauterine growth retardation (IUGR), metaphyseal dysplasia, congenital adrenal hypoplasia and genital anomalies. Cases generally present with life-threatening adrenal insufficiency in the neonatal period. Herein, we describe a patient with pronounced IUGR diagnosed with severe hyperpigment...

Prader-Willi syndrome (PW) is a rare genetic disorder with multi-organ system involvement. These patients present many perioperative challenges including sleep-related breathing disorders, morbid obesity, thick salivary secretions, mental retardation, and difficult intravenous access. PW has been suggested to be associated with central adrenal insufficiency. We report a novel case of persistent...

A 64-year-old nulliparous woman presented with clinical signs of thyroid and adrenocortical insufficiency. Subsequent hormonal investigations demonstrated a failure of all anterior pituitary functions. Pneumotomo-encephalography revealed a large arachnoid herniation, leading to the diagnosis of primary empty sella turcica syndrome with secondary panhypopituitarism. This unusual observation emph...