Pregnancy‐associated diffuse cavernous hemangioma of the uterus

Abstract

Diffuse cavernous hemangiomatosis of the pregnant uterus is a rare but serious condition. Six cases (1) have been reported in the literature since 1897. This is the first case diagnosed by sonography and magnetic resonance imaging (MRI) and the second one in which pregnancy was terminated by a successful cesarean section. The diagnosis was confirmed by histopathologic examination. Several complications are described. A 26-year-old Caucasian woman, gravida 1, was referred to our department at 18 weeks of gestation, because of suspected hematoma of the uterine wall. The patient had had vaginal spotting at 8 weeks of gestation and one week prior to referral. The general history revealed an undefined disease of the venous system with several aneurysmal malformations in the lower part of the body, but not in the uterus. She had been receiving treatment with low molecular weight heparin (Fragmin P forteTM) because of elevated D-dimers since week 10. The enlarged uterus was already in the epigastric region. Ultrasound examination identified an extensive lesion involving the whole corpus with numerous vascular channels largely replacing the uterine wall, with exception of a small area in the midline of the lower anterior wall (Fig. 1a). Power Doppler revealed slow blood flow in the affected area. The amount of amniotic fluid, placenta, fetal growth, uterine and fetal Doppler flow measurements remained appropriate for gestational age during pregnancy. The MRI findings corroborated the diagnosis (Fig. 1b). Cerebral venous changes and a Klippel-Trenaunay-Weber syndrome were excluded. a. Ultrasound scan of the thickened uterine wall (anterior: 4 cm; lateral: 7.1 cm) containing multiple fluid-filled spaces at 18 weeks of gestation. b. The T1-weighted SE sequence with fatsat after intravenous application of Gd-DPTA (0.1 mmol/kg) shows intensive enrichment of contrast medium. Inside the uterine cavity: the fetus with the umbilical cord. c. Histopathology of the myometrial biopsy: Ectatic and large vessels are densely packed with erythrocytes. Caliber and crowding of the vessels is increased compared to a normal pregnant uterus (H&E, ×200). Premature contractions at 32 weeks of gestation necessitated treatment with tocolytic agents for 4 days. Labor began spontaneously at term, but oxytocin and amniotomy were needed because of a delay of labor. Vacuum extraction from level of the pelvic brim was attempted because of bradytocia and maternal exhaustion, but a deterioration in the fetal heart rate forced a cesarean section. A healthy female infant weighing 3185 g was delivered. Delivery was complicated by atonic bleeding and a pulmonary disturbance, considered to be due to amniotic fluid embolism. The patient’s condition was stabilized by infusion of prostaglandin F2α, oxytocin, packed red cells and fresh frozen plasma (FFP). A representative piece of the uterine wall was sent to histopathological examination. Unusual large and thick-walled vessels with distension of their lumen led to the description of angioleiomyomatous hyperplasia of the uterus, consistent with the diagnosis of cavernous hemangioma (Fig. 1c). The patient was discharged in a stable condition 7 days later. Another six days later massive vaginal bleeding occurred and was stopped again by infusion of prostaglandin F2α, FFP and antithrombin III. Curettage excluded the presence of retained placental material. Her further recovery was uneventful. The diagnosis in the published cases of uterine hemangioma were based on the examination of the resected uterus (2, 4, 5) or sonography alone (1, 3). The reported case is the first one in which the diagnosis was made by sonography and MRI. Only one other pregnancy (1) was terminated by cesarean section. The risks for mother and child are considerable, as shown by the fatal outcome in the first published case (2). However, most of the patients described had an unremarkable pregnancy and the diagnosis was confirmed sonographically by chance (1, 3). Reported symptoms were abdominal pain (1), a tendency to collapse (5), and, as in our case, an enlarged uterus with a mild vaginal bleeding. A delay in labor has been described previously (2), but we also observed severe vaginal bleeding post partum, which might have been due to rupture of congested vessels or an inability of the uterus to contract sufficiently. Hypervascularity and angiomegaly, with a consequent increase in the vascular cross-sectional area, may lead to amniotic fluid embolism, as was presumed to have occurred in our case. Because the risks associated with pregnancy in cases of uterine hemangiomatosis are considerable, close surveillance of affected patients is needed. Vaginal delivery is to be preferred, but if cesarean section is required a vertical incision should be performed.