Couvelaire uterus (also known as uteroplacental apoplexy) is a life-threatening condition in which loosening of the placenta (abruptio placentae) causes bleeding that penetrates into the uterine myometrium forcing its way into the peritoneal cavity. This condition makes the uterus very tense and rigid. Symptoms and signs
Patients can have pain secondary to uterine contractions, uterine tetany or localized uterine tenderness. Signs can also be due to abruptio placentae including uterine hypertonus, fetal distress, fetal death, and rarely, hypovolemic shock (shock secondary to severe blood loss). The uterus may adopt a bluish/purplish, mottled appearance due to extravasation of blood into uterine muscle. Pathophysiology
Couvelaire uterus is a phenomenon where the retroplacental blood may penetrate through the thickness of the wall of the uterus into the peritoneal cavity. This may occur after abruptio placentae. The hemorrhage that gets into the decidua basalis ultimately splits the decidua, and the haematoma may remain within the decidua or may extravasate into the myometrium (the muscular wall of the uterus). The myometrium becomes weakened and may rupture due to the increase in intrauterine pressure associated with uterine contractions. This may lead to a life-threatening obstetric emergency requiring urgent delivery of the fetus. Prevention
The occurrence of Couvelaire uterus can be prevented by prevention of abruptio placentae. This includes proper management of hypertensive states of pregnancy; treatment of maternal diseases like diabetes mellitus, and other collagen disease complicating pregnancy; prevention of trauma during pregnancy; mothers should also avoid smoking or consumption of alcohol during pregnancy.
In those with severe liver disease liver transplantation may be an option. Avoiding smoking and getting vaccinated for influenza, pneumococcus, and hepatitis is also recommended.People with lung disease due to A1AD may receive intravenous infusions of alpha-1 antitrypsin, derived from donated human plasma. This augmentation therapy is thought to arrest the course of the disease and halt any further damage to the lungs. Long-term studies of the effectiveness of A1AT replacement therapy are not available. It is currently recommended that patients begin augmentation therapy only after the onset of emphysema symptoms. As of 2015 there were four IV augmentation therapy manufacturers in the United States, Canada, and several European countries. IV therapies are the standard mode of augmentation therapy delivery.Liver disease due to A1AD does not include any specific treatment, beyond routine care for chronic liver disease. However, the presence of cirrhosis affects treatment is several ways. Individuals with cirrhosis and portal hypertension should avoid contact sports to minimize the risk of splenic injury. All people with A1AD and cirrhosis should be screened for esophageal varices, and should avoid all alcohol consumption. Nonsteroidal antiinflammatory drugs (NSAIDs) should also be avoided, as these medications may worsen liver disease in general, and may particularly accelerate the liver injury associated with A1AD. Augmentation therapy is not appropriate for people with liver disease. If progressive liver failure or decompensated cirrhosis develop, then liver transplantation may be necessary. Epidemiology
People of Northern European and Iberian ancestry are at the highest risk for A1AD.