Facial haemangioma

Facial haemangioma

Description

A benign vascular lesion of the skin and other tissues, composed of endothelium lined spaces and a fibrous or fibromucinous stroma. Haemangiomas of the skin and subcutaneous tissues occur in 40 -45% of infants and, as such, are among the most common congenital anomalies. Females are affected more often than males (2:1). The three main types of haemangiomas are cavernous, capillary and nevus flammeus. Pathological classification is based on the nature of the cellularity of the stroma and the prominence of endothelial components. Capillary haemangiomas (strawberry marks) are composed of vessels of small diameter, whereas cavernous haemangiomas have thin-walled vessels with diameters that are quite large. Only cavernous haemangiomas would be detected prenatally. They affect 2% of infants. They have a lesser growth rate than capillary haemangiomas, and regression is slow and generally incomplete, unlike their capillary counterparts.

Diagnosis

Haemangiomas can appear to be cystic, solid or mixed. Occasionally they will be accompanied by multiple internal homogeneous echogenic reflections, representing calcified areas. These are small and widely scattered, unlike the gross calcification of a teratoma. The identification of pulsatile cystic areas, or the use of Doppler velocimetry to demonstrate low-resistance blood flow through the cystic areas, will aid diagnosis.

Differential Diagnosis

A goitre will present as a mainly solid mass, with echolucent areas. Cysts of the branchial cleft and thyroglossal duct are embryologic remnants occurring in characteristic locations. The former is found anterolateral to the carotid sheath, and the latter is located in the midline. Both tend to be unilocular. Teratoma has a complex echo pattern, with solid and cystic components. Calcification may be present in both haemangioma and teratoma, however gross calcification is generally restricted to teratoma, and fine scattered calcification to haemangioma. Solid masses of the neck include neuroblastoma, lipoma, salivary gland tumour, mesenchyme sarcoma and neurofibroma. Lymphangiomas are often multiloculated, with linear septations of variable thickness. Solid portions are found within and between the cysts. Encephalocele can be confused with haemangioma, particularly when the position is midline and occipital. A careful search should be made for an underlying bony defect, and the absence of pulsations.

Sonographic Features

Cystic, solid or mixed mass Generally, echogenicity is similar to that of the placenta

Occasionally accompanied by multiple internal homogeneous echogenic reflections, representing calcified areas.

These are small and widely scattered, unlike the gross calcification of a teratoma.

Cystic areas may be pulsatile

Doppler velocimetry should demonstrate low-resistance blood flow through the cystic areas

Associated Syndromes

  • Abdominal raphe
  • Bannayan-Zonana
  • Blue rubber bleb nevus
  • Branchio-oculo-facial
  • Cystic angiomatosis-Seip
  • Kasaback-Merritt
  • Klippel-Trenaunay-Weber
  • Maffucci
  • Maraschio
  • Neurocutaneous
  • Oculo-cerebro-acral
  • Pasyk-Argenta-Erickson
  • Proteus
  • Wegner

References

  1. Der Kaloustian VM In: Human Malformations and Related Anomalies Oxford University Press: New York, p990-7
  2. Meizner I, Bar-Ziv J, Holcberg G, Katz M In utero diagnosis of fetal facial tumour-haemangioma J Clin Ultrasound 13: 435-7
  3. McGahan JP, Schneider JM Fetal neck haemangioendothelioma with secondary hydrops fetalis: sonographic diagnosis J Clin Ultrasound 14: 384-8
  4. Bronshtein M, Bar-Hava I, Blumenfeld Z Early second-trimester sonographic appearance of occipital haemangioma simulating encephalocele Prenat Diagn 12: 695-8
  5. Sherer DM, Perillo AM, Abramowicz JS Fetal haemangioma overlying the temporal occipital suture, initially diagnosed by ultrasonography as an encephalocele J Ultrasound Med 12: 691-3
  6. Lasser D, Preis O, Dor N, Tancer ML Antenatal diagnosis of giant cystic haemangioma by Doppler velocimetry Obstet Gynecol 72: 476-7
  7. Grundy H, Glasmann A. Burlbaw J, Walton S, Danner C Haemangioma presenting as a cystic mass in the fetal neck J Ultrasound Med 4: 147-50
  8. Pennell RG, Baltarowich OH Prenatal sonographic diagnosis of a fetal facial haemangioma J Ultrasound Med 5: 58
  9. Shipp TD, Bromley B, Benacerraf B The ultrasonographic appearance and outcome for fetuses with masses distorting the fetal face J Ultrasound Med 14:673-8