Myocardial fat overgrowth in Proteus syndrome.

Proteus syndrome (PS) is a rare, mosaic disorder with asymmetric and distorting overgrowth of the skeletal system, skin, and adipose tissues. Cardiac abnormalities are rare in this syndrome and only two prior cases have been reported. Many patients with PS followed at our institution underwent transthoracic echocardiograms for preoperative evaluation or as work-up for associated pulmonary disease. Some were noted to have prominent, focal echodense areas in the myocardium. We further investigated cardiac findings in a cohort of children and adult patients with PS. Patients with abnormal echocardiograms were referred for cardiac magnetic resonance imaging, Holter monitoring, and exercise treadmill testing. Twenty children and adults with PS, age 24 months to 50 years old, underwent transthoracic echocardiograms. Seven patients (35%) had focal bright echodense areas within the myocardium suggesting fatty infiltration. The majority of patients had significant involvement of the interventricular septum. The cardiac characteristics of all patients with fatty infiltration on transthoracic echocardiograms were compared to Proteus patients without these findings. There were no significant differences in chamber sizes, mass, systolic or diastolic function. No increased risk of conduction defects or arrhythmias was found. This study shows that abnormal fat overgrowth is a common finding in the myocardium in patients with Proteus syndrome; however, it is not associated with functional derangements or arrhythmias. Further evaluation of a larger number of Proteus patients is needed in order to determine the frequency and prognosis of cardiac involvement. Published 2014. This article is a U.S. Government work and is in the public domain in the USA.

Panniculitis and arthritis as the presenting manifestation of chronic pancreatitis.

Although rare, panniculitis and polyarthritis can be the presenting manifestations of pancreatitis. Early recognition of this triad is critical because of the high mortality rate from pancreatic disease when the diagnosis is delayed. We report a patient with chronic alcohol intake whose sole presentation was severe polyarthritis and skin lesions secondary to chronic pancreatitis associated with extra pancreatic pseudocyst. Both arthritis and skin lesions disappeared after partial pancreatectomy and pancreatic stent insertion for a pancreatic duct stricture.

Fractured occluder disc: a previously unrecognized complication of the Starr-Edwards disc prosthesis.

Fracture of the occluder disc of a low-profile Starr-Edwards prosthesis is a hitherto unrecognized complication. We describe a patient who presented with right heart failure and severe pulmonary hypertension 27 years after mitral valve replacement with a model 6520 caged-disc prosthesis. At surgery, there was a longitudinal split in the occluder disc, and organized thrombus was lodged between the split segments. This case offers a unique opportunity to study the long-term effects of wear on the polyethylene poppet and Stellite cage.