TEACHINGS / LECTURES / CASES

Prenatal Diagnosis of Congenital Hepatic Haemangioma

L Govender

We report on a prenatally detected case of congenital hepatic haemangioma. A 21-year-old, para 2 was referred at 38 weeks gestation with sonographic findings, suspicious of arterio-venous malformations or a haemangioma of the fetal liver.  Our sonar findings revealed a hypoechoic mass with well defined but irregular edges in the left lobe of the fetal liver (Fig 1). Colour and spectral Doppler demonstrated a vascular mass with both arterial and venous signals. A soft tissue component noted within the vascular mass suggested that a diagnosis of a haemangioma was likely. There was no sonographic evidence of fetal hydrops and the rest of the fetal anatomy appeared normal. A multi-disciplinary team, (neonatologists, paediatric surgeons, and feto-maternal specialists) decided on an elective caesarean delivery and felt that chromosomal analysis, haematological evaluation and other imaging modalities were not justified at 38 weeks gestation.

A day prior to her planned delivery, the amniotic membranes spontaneously ruptured and a healthy baby weighing 3kg was delivered by emergency caesarean section. At birth there was no evidence of cardiac failure but the liver was palpably enlarged.  Cutaneous haemangiomas were noted on the upper lips and the right flank. Ultrasound of the baby’s liver showed a heterogenous echo-pattern with multiple hypoechoeic lesions in the left lobe. MRI and CT scans confirmed the findings of a multifocal haemangiomaendothelioma.

Fetal echocardiographic findings showed a mildly dilated heart, confirmed by X-ray. The baby was not anaemic and there was no evidence of coagulopathy or cardiac failure. At one-month review, the baby developed new haemangiomas under feet and on the hands.  There was no evidence of cardiac failure and repeat ultrasound showed multiple hepatic haemangiomas. Conservative management and monthly reviews were planned.

DISCUSSION

Fetal vascular anomalies are characterized by their capacity for progression as proliferate vascular tumours and include haemangiomas, haemangioendothelioma, angiosarcomas and developmental abnormalities of lymphatics, capillaries, and venous and arterial vessels. These anomalies may be found in any anatomical region and have varying prognoses. The characteristics of each lesion are distinct in time course, location and presentation. Whereas haemangiomas may develop prenatally or postnatally (usually in the first few months of life), vascular malformations are present before birth and persist for life [Chaft & Blei, 2003].

Recent improvements in ultrasound imaging, the use of fetal MRI [Meizner, 2000] as well as chromosomal studies, utilising amniocentesis [Al-Nemri et al., 2000] and / or cordocentesis have improved prenatal diagnosis.

Congenital hepatic haemangiomas are the most common tumour of infancy. They are usually benign and characterised by a stage of rapid endothelial cell proliferation followed by gradual involution. Hepatic haemangiomas are of mesenchymal origin and usually solitary, but the actual aetiology remains unknown [Al-Nemri et al., 2000].  They are composed of masses of arterial and venous connections within the liver. As a result of high flow low resistance shunt, this malformation can result in high output cardiac failure and hydrops. Large hepatic haemangiomas and diffuse haemangiomatosis may cause severe perinatal complications, particularly high output cardiac failure and / or Kasabach-Merritt sequence with severe consumption of platelets and clotting factors and haemolytic anaemia (Gembruch et al., 2002).  If untreated, there maybe up to 90% mortality.

Prenatal diagnosis of vascular lesions therefore provides the opportunity for antenatal intervention, parental counselling and planning delivery to optimise postnatal care.  With very large and diffuse haemangiomas, checking fetal haemoglobin, platelets and coagulation profile, enable prompt prenatal detection and correction of any abnormalities by intrauterine blood or platelet transfusion, especially just before delivery. This will significantly reduce perinatal morbidity and mortality. Morris et al. [1999] have reported that the compromised preterm fetus can also be treated in-utero by maternal corticosteroid administration, hence avoiding early emergency delivery.

Although our patient presented very late for any further prenatal evaluation, fortunately there was no sonographic evidence of cardiac failure or anaemia and the prenatal diagnosis

of hepatic haemangioma, made our delivery plans and postnatal management vigilant. 

Fig 1. Transverse section of fetal abdomen showing a hypoechoic mass (white arrow) with well defined but irregular edges in the left lobe of the liver (Post = posterior , L = left, R= right)

REFERENCES

Al-Nemri AR, Kilani RA, Salih MA, Al-Ajlan AA. (2000) Embryonal rhabdomyosarcoma and chromosomal breakage in newborn infant with possible Dubowitz Syndrome. Am J Med Genet 92, 107-110.

Chaft J and Blei F. (2003) Prenatal diagnoses of vascular anomalies: Update and literature review. Lymphat Res Biol 1, 309-312.

Gembruch U, Baschat AA, Gloeckner-Hoffman K, Gortner L, Germer U. (2002) Prenatal diagnoses and management of fetuses with liver haemangiomata. Ultrasound Obstet Gynaecol 19, 454- 460. 

Meizner I. (2000) Perinatal Oncology- role of prenatal ultrasound diagnosis. Ultrasound Obstet Gynecol 16, 507-509.

Morris J, Abbott J, Burrows P, Levine D. (1999) Antenatal diagnosis of fetal hepatic haemangioma treated with maternal corticosteroids Obstet Gynecol 94, 813-815.