Abstract
Placental chorioangioma (CA) is a benign placental tumor. No specific treatment is required for asymptomatic cases. We report a female infant born to a mother with giant placental CA. However fetal growth was normal and, fetal hydrops was not detected by ultrasound examination until delivery, she had hydrops, subgaleal hematoma, thrombocytopenia, hemolytic anemia, respiratory distress and circulatory failure after birth. She was successfully treated without any neurological sequelae. At 2 months of age, infantile hemangioma appeared in her lower lip. The present case suggested that giant placental CA might cause postnatal problems and be associated with the development of infantile hemangioma.
Introduction
Chorioangioma (CA) is a benign placental tumor, defined by the abnormal proliferation of vessels moving up from chorionic tissue [1]. Small placental CA tends to remain asymptomatic and rarely complicates the course of pregnancy. On the other hand, ‘giant’ placental CA (some reports > 4 cm in diameter, but the definition of giant tumor is controversial) is a rare neoplasm, with a prevalence ranging from 1:9,000 to 1:50,000 pregnancies. This type of placental CA is associated with pregnancy complications such as premature labor, placental abruption, polyhydramnios, fetal hydrops, intrauterine growth restriction, hepatosplenomegaly, cardiomegaly, congestive heart failure and death [2]. Neonatal consequences have been reported to include severe microangiopathic hemolytic anemia, thrombocytopenia and hydrops [3]. Infantile hemangioma (IH) is the most common tumor of childhood, occurring in 5% – 10% of infants. Several reports have demonstrated that placental hemangioma and IH were pathologically identical [4] and presence of placental CA was closely correlated with that of IH [5].
Here, we report a baby born to a mother with a giant CA, who had circulatory failure, hydrops, hepatosplenomegaly, coagulopathy and hemolytic anemia at birth. At 2 months of age, IH developed in her lower lip.
Case report
We report a female infant, first daughter of a healthy middle-aged couple. The pregnancy was followed up at a local obstetrical hospital. The placental tumor was detected at thirty weeks gestation by ultrasound examination. Polyhydramnios didn’t exist during pregnancy. At the time of transfer to our hospital from the previous doctor, we determined the initial tumor size to be 6.5×5.2 cm and located on the fetal side of the placenta. Vascular flow was rich inside the tumor by power Doppler method (Fig. 1-a). The size of placental tumor was gradually increased, but fetal growth progressed well and fetal hydrops, such as subcutaneous edema, ascites, and pleural effusion was not detected by ultrasound examination until delivery. There were no abnormal signs and reflects two points for each of five categories, so cardiovascular profile score was ten (perfect score). She was delivered by cesarean section at 37 weeks of gestation following an arrest of labor. Although augmentation of labor with oxytocin was attempted at 37 weeks of gestation and vacuum extraction was aborted due to rapidly expanding caput succedaneum, and delivery was by cesarean section due to the arrest in labor. Her birth weight was 2,912 kg (79.5% tile) and Apgar score at 5 minutes after birth was 7 points. She required resuscitation. Patient breathing improved with mask ventilation, but sustained ventilation was necessary, so subsequently admitted to a neonatal intensive care unit (NICU). At birth, she had hydrops, subgaleal hematoma, hepatosplenomegaly, purpura, respiratory distress requiring high flow nasal cannula oxygen therapy for the first 4 days of life and circulatory failure necessitating dopamine and dobutamine (up to doses of 5μg/kg/min for both). Neither dysmorphic feature nor cutaneous hemangioma was observed. Laboratory testing showed platelet count of 68×109/L, hemoglobin concentration of 8.0 g/dl, aspartate aminotransferase (AST) of 120 U/l, lactate dehydrogenase of 2,530 U/l, fibrinogen degradation products of 22.7μg/ml, prothrombin time-international normalized ratio of 3.75, fibrinogen of 99 mg/dl, albumin of 1.2 g/dl, and N-terminal pro-brain natriuretic peptide (NT-proBNP) of 25,282 pg/ml (neonatal normal range:1121– 7740 pg/ml [6]). Hypoalbuminemia and anemia required red blood cell and albumin transfusions. We also diagnosed disseminated intravascular coagulation (DIC) based on purpura on her limbs and coagulation profile, and treated her with fresh frozen plasma transfusions. After treatment, her complete blood count, coagulation tests and electrolyte levels became normal. She was discharged to home at 15 days of life without any respiratory support and with full enteral feedings with breast milk.
(a) Transabdominal ultrasound tomography: Normal placenta and solid tumor mass with clear boundary on placenta’s fetus side. (b) Macroscopic appearance of the placenta after normal vaginal delivery. A 12 cm diameter, mass on the fetal surface of the placenta near its boundary is shown. (c), (d) Lesional cells displayed characteristic vascular invasion, replacing vessel walls, and tumor cells have vast areas of necrosis (hematoxylin and eosin staining, original magnification 40x, 200x.).
In a macroscopic assessment of the placenta, a single well-circumscribed mass of 10 cm diameter, was found along the fetal surface of the placenta (Fig. 1-b). Microscopic examination showed histological features of angiomatous type of CA (Fig. 1-c, d).
At 2 months of age, IH appeared in her lower lip (Fig. 2-a). As it gradually increased, she experienced feeding difficulties. After oral administration of propranolol was initiated at 3 months of age, IH ameliorated markedly (Fig. 2-b). Follow-up up to the present time have shown normal psychomotor development and growth.
(a) Infantile hemangioma before treatment. (b) Infantile hemangioma after treatment.
The present case poses two significant clinical issues. First, in spite of no remarkable abnormality detected in the fetus before delivery, giant placental CA might cause postnatal problems. Even if ultrasound examinations are normal besides giant placental CA during pregnancy, a skilled neonatal resuscitation team should be prepared. Well known pregnancy complications include polyhydramnios, however, it did not exist our case. Polyhydramnios is a well-known complication, and the occurrence frequency is around 27% [7]. It is thought that chorioangiomas may act as peripheral AV shunts leading to an increase in fetal cardiac output, cardiac hypertrophy, high output congestive cardiac failure and fetal hydrops. Some reports [8] says that it is in equilibrium with the placenta with low vascular resistance, maintaining the peripheral circulation of the whole body and it is difficult to become heart failure in the fetal period. After birth, the vascular resistance of the whole body may be increased due to the loss of the placental circulation, which is likely to exhibit high output heart failure. In this case there were seven weeks between placental CA diagnosis and birth with no follow-up evaluations, there should be an argument provided for more frequent monitoring.
On the other hand, delivery is considerable reasonable with regard to the fetus in the case of giant placental hemangioma. Haiyan et al. [9] reported that transvaginal delivery is considered reasonable as far as fetal growth and viability is fine in the case of giant placental hemangioma. In our case, although fetal distress was not seen during labor, subgaleal hematoma after vacuum extraction and subsequent DIC was seen in the new born infant. Fetal anemia should be examined by middle cerebral artery peak systolic velocity (MCA-PSV) using color Doppler analysis before vacuum extraction. If fetal anemia is suspected after color Doppler analysis, vacuum extraction should be avoided to prevent subgaleal hematoma of a new born infant.
Second, two major pathophysiological mechanisms of fetal hydrops due to placental CA have been postulated. One is that CA acts as arteriovenous malformation and causes high out-put cardiac failure. The other is that hamartomatous in small capillary-type vessels in the giant CA can cause hemolytic anemia, similar to Kasabach-Merritt syndrome, resulting in chronic in utero hypoxia and fetal growth restriction [10].
Microangiopathic hemolytic anemia with thrombocytopenia, nucleated erythrocyte and consumption coagulopathy were present in our case. Although there are no clear guidelines in Japan regarding delivery methods in the case of placental CA, it may be safer to avoid vacuum labor because of the possibility of coagulopathy and the risk of subgaleal hematoma after birth. Giant placental CA might be associated with the development of IH.
In the present case, IH appeared in the lower lip of a newborn after discharge, and gradually became enlarged. She required treatment at 3 months of age. Several reports have suggested that placental CA was associated with the development of IH [5]. North PE [11] reported that the erythrocyte type glucose transporter protein (GLUT1), was highly expressed in IH as well as placental CA. Furthermore, several reports have demonstrated that placental hemangioma and IH were pathologically identical [4]. Further studies are needed to reveal the relationship between the two tumors.
In conclusion, special attention is needed for giant placental CA, because it might cause postnatal problems and be associated with the development of IH. Further studies are warranted to examine this association.
Disclosure
None declared.