Prenatal diagnosis of a giant fetal hepatic hemangioma: a case report

Case report

We report the antenatal diagnosis of a giant fetal hepatic hemangioma (HH) with an uncomplicated fetal and postnatal evolution, demonstrating that absence of fetal treatment is an option, even in large tumors.

A 26-year-old woman, primigravida, underwent routine fetal ultrasound at 33 weeks’ gestation (WG) and 5 days, which revealed an isolated 57×38 mm diameter well circumscribed heterogeneous mass of the right fetal hepatic lobe. Its structure was mostly hypoechogenic (Figure 1), with marked peripheral and central Doppler blood flow (Figure 2). The feeding vessel showed an arterial flow pattern on color Doppler and the mass was drained into dilated, tortuous right sus-hepatic veins. The image was compatible with a fetal HH. No other fetal anatomic abnormalities were detected. Biometry was appropriate for gestational age and amniotic fluid volume and the placenta were normal. Umbilical fetal arterial Doppler flow was normal. There was a moderate cardiomegaly and dilated right ventricle, but without evidence of atrioventricular regurgitation or peripheral signs of heart failure. Pregnancy evolution was uneventful, with twice weekly fetal ultrasound, including echocardiography, as well as cardiotocogram (CTG). At 36 WG and 5 days, the fetal ultrasound showed a stable image size with no associated complications. The delivery was fixed at 37 WG for fetal protection to limit the eventuality of a complication linked to the abnormal intrahepatic vascular tumor such as the onset of heart failure. A cesarean delivery was decided to decrease the risk of body dystocia and to prevent labor-induced fetal heart rate anomalies especially with the presence of a moderate cardiomegaly and labor-induced rupture of the tumor.

Figure 1:

Prenatal imaging: an ultrasound transverse section showing the abdominal mass.

Figure 2:

Prenatal imaging: color Doppler image confirmed the marked peripheral and central blood flow.

The patient received two intramuscular doses of betamethasone in the 48 h before the elective cesarean section.

A classic cesarean procedure was done with a Pfannenstiel incision in the skin and a transverse hysterotomy. There were no fetal body dystocia at the time of the cesarean. There were no pre- and postoperative complications during the procedure.

The woman gave birth to a 3130 g female neonate, with 5 min Apgar score of 10. The newborn was transferred to the neonatal intensive care unit. Her physical examination revealed a hepatomegaly, no skin lesions, and especially no hemangiomas or petechiae. There were no signs of peripheral heart failure or visible thoraco-abdominal circulation. Postnatal abdominal ultrasound found a 44×43 mm heterogeneous lesion of the right fetal hepatic lobe, with marked peripheral and central Doppler blood flow (Figure 3), compatible with the diagnosis of an HH.

Figure 3:

Postnatal imaging confirmed a highly vascular intrahepatic tumor.

Postnatal cardiac ultrasound confirmed the absence of signs of heart failure. The newborn’s initial blood results showed slightly elevated liver enzymes [ALT 258U/L (N<45), γ-GT 93U/L (N<50), ALKP 235U/L (N<126)] which came back to normal ranges 6 weeks postnatally. Blood cell count and clotting were normal at birth and 6 weeks postnatally. Regular ultrasound follow-up was performed at 6 weeks, 6 months, and 1 year postnatally, showing a reduction in tumor size to 32×29 mm 6 months postnatally (Figure 4), and to 25×13 mm at 1 year. The spontaneous regression of this lesion led us to do an ultrasound follow-up every 6 months.

Figure 4:

Postnatal imaging showing regression of tumor size at 6 month follow-up.

Discussion

Hepatic hemangioma is a rare benign tumor representing, however, the most common vascular tumor of the liver in neonates and the third most common tumor of the liver in childhood [1, 2]. Neonatal mortality rate is high, reported as high as 70–90% [3]. The typical natural evolution is biphasic with a rapid proliferative phase within the first months, and later with a slow resolution phase over a period of years. Although the differential diagnosis include hepatoblastoma, mesenchymal hamartoma, and metastatic neuroblastoma, recognition of the classic sonographic features of HH [4] (Table 1) has made biopsy confirmation of the diagnosis mostly unnecessary, limiting the risks of major hemorrhage. As for most ultrasound abnormalities, the diagnosis challenge is associated to other malformations, which is the case for 50% of HH. These are cutaneous hemangiomas or rarely a more generalized hemangiomatosis involving the central nervous system, respiratory and gastrointestinal tract, and possibly placenta.

Small HH are usually asymptomatic, requiring no treatment, whereas giant HH, defined as >40 mm in diameter, are usually responsible of complications [5], and need aggressive management. When large enough, the tumor can act as an arterio-venous shunt and cause consumptive thrombocytopenic coagulopathy (Kasabach-Merritt syndrome) [6], anemia, and high-output congestive heart failure leading to fetal hydrops. It can also be responsible of hemorrhage due to tumor rupture. Aggressive management of large and evolutive lesions may include medical therapy such as corticosteroids or agents with strong antiangiogenic effect, irradiation, selective embolization, or surgical procedures [3, 7].

Antenatal management for HH depends on the associated complications and gestational age. At term, delivery is indicated. If the fetus has reached an advanced gestation (>30 WG) and the lesion is symptomatic (presence of signs of heart failure), then early delivery may be indicated [8]. Before 30 WG, if the fetus is symptomatic, a first-line medical treatment is indicated, using corticosteroids administered either to the mother or direct injection into the umbilical cord [9]. Postnatally, no treatment is indicated for asymptomatic tumors and conservative management with ultrasound follow-up to document that expected regression is required. In the present case, the infant was managed expectantly because of the HH stability in size prenatally and the lack of symptoms postnatally.

Our case, with few other cases of asymptomatic giant HH reported in the literature [10, 11], support the fact that complications of these lesions are not related only to its size and that other prognosis factors have to be determined.

Since prognostic factors for giant HH are not clearly established and due to lack of guidelines for antenatal follow-up, we suggest at least weekly fetal ultrasound, associated with echocardiography and CTG, to assess signs of heart failure prior to development of hydrops.