Unusual complications of placental chorioangioma: consumption coagulopathy and hypertension in a preterm newborn

Abstract

We report a case of a preterm neonate born to a mother with giant placental chorioangioma. The baby had microangiopathic haemolytic anaemia, thrombocytopenia and cardiac failure at birth. In addition, she had a disseminated intravascular coagulation-like picture and had bleeding from multiple sites, which was treated with transfusion of multiple blood products. She also developed transient hypertension and required antihypertensive drugs for 3 weeks. The baby was successfully managed and discharged home, though with signs of neurosensory impairment.

Background

Giant placental chorioangiomas are often associated with various maternal, fetal and neonatal complications.¹ The fetal–neonatal complications reported include non-immune hydrops, microangiopathic haemolytic anaemia, thrombocytopenia and cardiac failure. The mortality rate is high and very few successfully treated cases have been reported.^{2 3} We report a case of a preterm infant who presented with unusual complications of consumption coagulopathy and hypertension, secondary to giant chorioangioma. The baby was successfully treated and discharged home, though with signs of neurosensory impairment.

Case presentation

A 27-year-old woman with an obstetric score of G4 P1 L1 A2 was diagnosed as having placental chorioangioma at 27 weeks of gestation and referred to our hospital for further management. Ultrasound showed an 11×10×9 cm tumour (figures 1 and 2), polyhydramnios with a single liquor pocket of 10 cm, fetal hydrops with subcutaneous oedema, bilateral pleural effusion, ascites and echogenic bowel. The baby was delivered by elective caesarean section at 31 weeks of gestation.

Figure 1.

Antenatal ultrasound (grey scale) showing a giant chorioangioma (11×10×9 cm).

Figure 2.

Antenatal ultrasound—colour Doppler showing intratumour vascularity.

At delivery, the weight of the placenta (with the tumour) was 1350 g. On gross examination, the tumour had a tan-brown cut surface, admixed with areas of suspected infarction (figure 3). On microscopic examination, the tumour was composed of sheets and lobules of closely packed small sized capillaries lined by benign endothelial cells (figure 4), with areas of haemorrhage and infarction. Histopathology of the placenta was normal.

Figure 3.

Gross examination of the tumour showing tan-brown cut surface with areas of suspected infarction.

Figure 4.

Histopathology of the tumour—H&E stain showing tumour composed of sheets and lobules of closely packed small sized capillaries lined by benign endothelial cells supported by inconspicuous, loose stroma; (A) ×4 magnification, (B) ×10 magnification, (C) ×20 magnification, (D) ×40 magnification.

The baby girl had a birth weight of 1840 g and APGAR scores of 6 and 9 at 1 and 5 min, respectively. At birth, she had pallor, petechiae, hepatosplenomegaly and subcutaneous oedema. She had hyperdynamic precordium and tachypnoea, and required oxygen through a nasal catheter at birth. At 14 h of life, there was worsening of respiratory distress and increasing oxygen requirement, hence she was intubated and ventilated along with inotropic support. Echocardiography showed severe myocardial dysfunction with mitral and tricuspid regurgitation (gradient of 70–80 mm/Hg). There was neither pleural nor pericardial effusion nor ascites.

The baby's haemoglobin on day 1 was 11.5 g/dL and reticulocyte count 12.8%, for which she received a packed cell transfusion. She had bleeding at multiple sites such as continuous bleeding from the umbilical cord after umbilical venous catheterisation, bleeding from the endotracheal tube and development of new petechiae and bruises. The coagulation profile on day 1 showed a platelet count of 30 000/μL, prothrombin time of 20 s, activated partial thromboplastin time of 62 s, serum fibrinogen of 104 mg/dL and thrombin time of 30 s. D-Dimer was elevated (3955 ng/mL). Peripheral smear showed anisopoikilocytosis with numerous fragmented red cells suggestive of microangiopathic haemolytic anaemia (figure 5). The infant required multiple transfusions with fresh frozen plasma, cryoprecipitate, platelet rich concentrate and packed red cells, in the first 3 days of life. Sepsis work up and direct Coomb's test were negative. All the coagulation parameters normalised on day 4 of life. She required ventilator support for 3 days, followed by continuous positive airway pressure (CPAP) for 2 days.

Figure 5.

Peripheral smear of the baby showing anisopoikilocytosis with numerous fragmented red cells.

From day 3 of life, the baby started having hypertension with systolic blood pressure >99th centile. Inotropes were tapered and stopped. She had never required an umbilical arterial catheter. Her creatinine on day 3 of life was 1.3 mg/dL, but repeat values showed a decreasing trend. Her urine output and serum electrolytes were normal. Renal ultrasound with arterial and venous Doppler was normal. She was started on oral nifedipine followed by oral hydralazine, with which systolic blood pressure was maintained below the 95th centile. Antihypertensive drugs were given for 2 weeks, then tapered over 1 week and stopped, following which there was no recurrence of hypertension. On day 7 of life, the baby developed culture-positive sepsis without meningitis, treated with appropriate antibiotics for 14 days.

Ultrasound cranium at 6 weeks of age showed grade 2 cystic periventricular leukomalacia. Ophthalmic evaluation showed no retinopathy of prematurity. Auditory brainstem response showed absence of replicable wave V even at 90 decibels. The baby was discharged on day 54 of life on direct breast feeds; she weighed 1880 g. At discharge, the baby had hypertonia with an incomplete Moro reflex. Repeat hearing assessment and intervention was planned after a month. She was started on an early stimulation programme and is under regular follow-up.

Discussion

Chorioangioma is a hamartoma resulting from excessive proliferation of blood vessels in the chorionic villi of the placenta. The reported incidence is as high as 0.6%, by histopathological examination of the placenta.⁴ However, less than 10% of the tumours were described as being >4 cm in size.⁴ The incidence was higher in female fetuses and in pregnancies at high altitude.^{4 5}

Small chorioangiomas do not produce any symptom and are usually an incidental diagnosis. A tumour >4 cm in size is called a giant chorioangioma and associated with maternal, fetal and neonatal complications.¹ The maternal complications include preeclampsia, polyhydramnios and maternal mirror syndrome with polyhydramnios increasing the risk of preterm labour and placental abruption.¹

The fetus develops hydrops due to two major pathophysiological mechanisms: chorioangioma is a vascular tumour containing blood vessels in direct continuity with the fetal circulation and hence acts as an arteriovenous malformation and causes high-output cardiac failure. Also, the hamartomatous small capillary-type vessels inside the tumour cause breakdown of the red cells traversing the vessels resulting in microangiopathic haemolytic anaemia. The fetus may suffer growth restriction due to chronic in utero hypoxia.⁶ There is also a risk of sudden fetal demise, probably due to rupture of the delicate vessels within the tumour resulting in massive fetomaternal haemorrhage.^{7 8}

Ultrasonography is the most commonly used method for diagnosis. Colour Doppler, demonstrating blood vessels inside the tumour that communicate with the fetal circulation, helps to differentiate chorioangioma from other tumours such as teratoma, partial hydatidiform mole and placental haematoma. Colour Doppler also helps in determining the prognosis—the less vascular the tumour, the better the prognosis, due to decreased fetotumoural shunting.^{9 10} In our case, the colour Doppler showed a highly vascular tumour, which was hence causing significant complications.

There are reports of spontaneous improvement of fetal hydrops; probably because increase in tumour size with advancing gestation causes relative ischaemia and necrosis of the tumour, and thrombotic degeneration of the blood vessels, which in turn result in reduced fetotumoural shunting. In our case, there was improvement in the severity of hydrops in the form of resolution of pleural effusion and ascites from 27 to 31 weeks gestation, which might have been due to the same mechanism, as the tumour showed areas of haemorrhage and infarction.^{9 11 12}

The reported neonatal complications are microangiopathic haemolytic anaemia, thrombocytopenia and myocardial dysfunction causing congestive cardiac failure.^{2 3} This baby had all these complications and, in addition, had a disseminated intravascular coagulation-like picture. This is probably due to formation of multiple microthrombi within the tumour mass resulting in consumption coagulopathy in utero, a condition similar to Kasabach-Merritt syndrome. With the replacement of platelets and clotting factors, the coagulopathy resolved in 72 h.

Hypertension as a neonatal complication of placental chorioangioma has not so far been reported. The chronic haemodynamic compromise in utero might have reset the renin-angiotensin and adrenergic system in order to maintain fetal circulation in the presence of low-pressure tumour circulation. Once the low-pressure tumour circulation was removed and myocardial dysfunction improved with supportive treatment, these overactive compensatory mechanisms might have resulted in hypertension. This diminished over a period of time, resulting in resolution of the hypertension. The transient nature of the hypertension and the absence of recurrence after stopping antihypertensive drugs make other aetiologies unlikely.

In our patient, although all the complications related to chorioangioma were successfully treated, the baby developed hearing loss and signs of evolving neuromotor impairment probably because of the chronic in utero hypoxia secondary to chorioangioma and the complicated postnatal course.

Learning points.

• Giant placental chorioangioma can cause a disseminated intravascular coagulation-like picture soon after birth as a result of consumption coagulopathy in utero due to formation of microthrombi within the tumour vessels.

• Giant chorioangioma may cause transient hypertension in the newborn period, probably due to persistence of the in utero compensatory mechanisms toward tumour-induced cardiac failure.

• The extent of complications is proportional to the vascularity of the tumour. The increase in tumour size with advancing gestation may cause ischaemic necrosis of the tumour and thrombotic obliteration of the blood vessels within the tumour. This may result in decrease in fetotumoural shunting and improvement in hydrops.