Kasabach–Merritt syndrome in two successive pregnancies

Abstract

A 20‐year‐old Indian Hindu woman, a primigravida at 8 months of pregnancy, presented with symptoms of progressively worsening dyspnea and anasarca of 1 month duration. Physical examination showed signs of severe congestive cardiac failure. She had multiple hemangiomas, 5 cm in size, located over the right temporal region, right side of the chin, right side of the nose, left upper part of the abdominal wall, and anterior aspect of the left leg ( Fig. 1). These hemangiomas had been present since birth and had enlarged considerably during pregnancy. The clinical diagnosis was pregnancy—34 weeks—with multiple hemangiomas and congestive cardiac failure. 1 Hemangiomas Laboratory investigations revealed hemoglobin (Hb) 11 g/dL, total leucocyte count 7000/mm3, differential count P 60 L 40, random blood sugar 110 mg/dL, blood urea (BU) 34 mg/dL, creatinine 0.9 mg/dL, and serum electrolytes: Na, 130 mEq/L; K, 4 mEq/L; Cl, 110 mEq/L. Electrocardiogram (ECG) was normal. Chest X‐ray showed cardiomegaly with congestion. Echocardiogram (ECHO) showed enlarged right atrium and right ventricle. The patient was refractory to antifailure therapy and went into labor at the end of 35 weeks of pregnancy and delivered a 1.3 kg male baby. The patient developed post partum hemorrhage and was treated with blood transfusion. During labor, the patient developed purpuric eruption and epistaxis. At that time, the bleeding time was more than 15 min and the platelet count was 46,000. At this juncture, a diagnosis of Kasabach–Merritt syndrome was considered. Skin biopsy revealed hemangiomatous changes. After delivery, the patient’s edema and dyspnea decreased considerably and the eruption disappeared. The hemangiomas regressed in size. A platelet count performed 3 days after delivery was 120,000/mm3. The bleeding time was 7 min, and a chest X‐ray showed the reduction of heart size to normal. ECHO repeated 1 week after delivery showed normal‐sized chambers. Follow‐up as an outpatient showed the patient returning to normalcy. She had a similar presentation during her second pregnancy, 1 year later.

Kasabach–merritt syndrome: pathogenesis and management

“…Not all congenital haemangiomata resolve; although the majority of classic strawberry haemangioma of infancy resolve completely, approximately 20–40% of children are left with residual skin changes including disfiguring scars. Episodes of acute DIC have been reported in pregnant women with congenital haemangiomata (Lee & Kirk, 1967; Neubert et al , 1995) and in one woman during two successive pregnancies (Singh & Rajendran, 1998). The hormonal alterations and increase in blood volume in pregnancy may affect pre‐existing lesions, triggering episodes of acute DIC.…”

Excessive Bleeding due to Consumptive Coagulopathy after Delivery of an Intrauterine Fetal Death in a Patient known with Multiple Vascular Malformations; Mimicking the Kasabach-Merritt Syndrome

“…In 1967 the first case of this coagulation disorder occurring during pregnancy was reported. This case and a few other cases, which were described in the subsequent years, showed Localized Intravascular Coagulation (LIC) due to KMS with consumptive coagulopathy after a normal pregnancy and a normal at term delivery [6,7]. In one patient with KMS, LIC occurred after a cesarean delivery, performed because of a large complex of varicosities extending along the vaginal wall and the labia majora which partially obstructed the introitus [8].…”

Treatment of Kasabach–Merritt syndrome: a stepwise regimen of prednisolone, dipyridamole, and interferon

“…Biopsy in one other patient indicated cavernous hemangioma. Although most cases of KMS started in infancy, KMS has been reported in all ages, 1–10 including adult cases with Klippel–Trenaunay–Weber syndrome in pregnancy, 11,12 and hemangioma following transurethral resection of a bladder tumor 13 . Therefore, we believed that KMS can be associated with a variety of vascular tumor 1,7 …”