Of aortic valve and bleeding: Heyde's syndrome.

An 80-year-old woman with severe aortic stenosis presented with relapsing enterorrhagia and severe anemia. A video capsule pan-endoscopy showed multiple sites of complex mucosal angiodysplasia in the jejunum. Direct hemostatic treatment of accessible angiodysplasia was done with argon plasma coagulation, and the patient was urgently referred for trans-catheter aortic valve replacement (TAVR). At follow-up 1 month and 3 months later, she was doing well with no further episodes of bleeding. Heyde's syndrome is referred to as the association of aortic stenosis, gastrointestinal angiodysplasia, bleeding, and anemia. It is an acquired type2A von Willebrand syndrome caused by the proteolysis and loss of the largest polymers of vWF due to the high shear forces generated through the stenotic aortic valve. The qualitative and quantitative vWF defects play a central role in the angiogenesis and development of gastrointestinal angiodysplasia The vWF abnormalities are closely associated with the hemodynamic severity of the aortic valve stenosis. Valve replacement is the pivotal strategy to achieve the long-term resolution of bleeding recurrences. TAVR is a valuable option particularly in high-risk patients for whom surgical valve replacement is not feasible.

Kasabach-Merritt syndrome arising from a vascular fistula.

A 58-year-old woman presented with gum bleeding, hematuria, and cutaneous ecchymoses. Left hip replacement had been performed five years prior. The overall findings of our work-up were consistent with ongoing DIC triggered by the presence of an arterio-venous left femoral fistula. The patient was treated successfully with fresh frozen plasma, the fistula was surgically repaired and a stent was placed. On the second day, bleeding had resolved and laboratory values reverted to normal. This uncommon scenario is reminiscent of the Kasabach-Merritt syndrome and well illustrates that patients with an arterio-venous fistula can sometimes present with atypical features. The Kasabach-Merritt syndrome is reported in pediatric and adult patients with giant hemangiomas and angiosarcomas. Adult cases are described also in association with hematomas and large vascular aneurysms. The underlying pathophysiology is the sequestration and consumption of platelets and clotting factors with uncontrolled formation of microthrombi within the vascular lesion. DIC and a microangiopathic hemolytic anemia can subsequently develop. Mechanistic pathways of the Kasabach-Merritt syndrome in the context of a vascular fistula are shared with the more common causes of the syndrome. We speculate that the endothelial dysfunction and injury caused by the flow shear were the pivotal triggers of the aberrant trapping of platelets, the consumptive coagulopathy, and the formation of microthrombi within the fistula. Mortality rate can be as high as up to 40%. The Kasabach-Merritt syndrome could represent the only clinical feature of an otherwise occult vascular fistula. Emergency physicians should be aware of this condition.

Granulomatous peritonitis after laparoscopic cholecystectomy.

BACKGROUND: Granulomatous peritonitis may indicate a number of infectious, malignant, and idiopathic inflammatory conditions. It is a very rare postoperative complication, which is thought to reflect a delayed cellmediated response to cornstarch from surgical glove powder in susceptible individuals. This mechanism, however, is much more likely to occur with open abdominal surgery when compared with the laparoscopic technique. METHODS: We report a case of sterile granulomatous peritonitis in an 80-y-old female after a laparoscopic cholecystectomy. Management was conservative, and no relapse was observed after over 1-y of follow-up. DISCUSSION: We propose that peritoneal exposure to bile acids during the laparoscopic removal of the gallbladder was the trigger of granulomatous peritonitis in this patient. Severe complications, such as peritoneal adhesions, intestinal obstruction, and fistula formation, were observed, but no fatalities were reported. CONCLUSION: We should be aware of this rare cause of peritonitis in the surgical setting.