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Disease summary: Schwartz--Jampel syndrome (SJS) is a rare disorder that is characterised by myotonia and skeletal abnormalities. SJS is caused by mutations in the HSPG2 gene encoding the protein perlecan. Myotonia results in a fixed facial expression with blepharophimosis, microstomia, pursed lips and mask-like faces. The typical skeletal and other abnormalities include short stature, kyphoscoliosis, joint contractures, and micrognathia. Tracheal intubation is difficult in these patients because they have micrognathia, cervical kyphoscoliosis, and a limited mouth opening. [ABSTRACT FROM AUTHOR]

Introduction Atrial fibrillation (AF) is the most common perioperative cardiac arrhythmia. Its incidence increases with age, affecting 0.7 % of patients aged 55-59 years, increasing to 17.8 % in those [...], We present a patient with atrial fibrillation (AF) in whom a left atrial (LA) thrombus might have formed during laparotomy despite bridging anticoagulation therapy. No evidence of thrombus was detected by transesophageal echocardiography (TEE) at the start of surgery; however, a thrombus measuring 13 x 10 mm was found in the LA appendage by the end of the procedure, suggesting that thrombus might develop intraoperatively in patients with AF even when bridging anticoagulation is properly established. Intraoperative TEE can assist in detecting intracardiac thrombus in patients with AF regardless of their anticoagulation status and provides a tool for intervention to prevent systemic embolization. Keywords Atrial fibrillation * Thrombus * Bridging anticoagulation therapy

Introduction Postanesthetic shivering occurs in approximately 40% of unwarmed patients recovering from general anesthesia and is associated with substantial adrenergic activation and discomfort [1]. Shivering can increase tissue oxygen consumption [...], Background Postanesthetic shivering can be triggered by surgical stress and several aspects of anesthetic management and is frequently preceded by a decrease in peripheral blood flow due to thermoregulatory vasoconstriction. As perfusion index correlates with peripheral blood flow, we examined whether perioperative perfusion index, measured using pulse oximetry, might be correlated with post-anesthetic shivering. Methods Twenty-eight patients presenting for elective abdominal surgery were enrolled. Core (esophagus) and peripheral (finger) temperatures and perfusion index were recorded in the perioperative periods. Correlations between perfusion index and peripheral temperature and core-to-peripheral temperature gradient were then explored. Plasma levels of epinephrine and norepinephrine were also measured. The extent of shivering was graded after emergence from anesthesia. Results Perfusion index declined before emergence from anesthesia in patients who then developed post-anesthetic shivering. This coincided with the time at which the difference between core and peripheral temperature became dissociated and peripheral temperature declined. Perioperative perfusion index was correlated with peripheral temperature and peripheral-core temperature gradient. Perfusion index at closure of the peritoneum predicted postanesthetic shivering and was significantly correlated with the extent of shivering. Plasma levels of both epinephrine and norepinephrine were significantly elevated after shivering events. Conclusions Perfusion index was significantly lower in patients with postanesthetic shivering before emergence from anesthesia, indicating that measurement of perfusion index during and before the end of anesthesia might be a useful means of predicting postanesthetic shivering. Keywords Postanesthetic shivering * Perfusion index * Core-to-peripheral temperature gradient