Rapidly involuting congenital haemangioma: diagnostic and therapeutic approach regarding two case reports

Abstract

Two unrelated neonates were born with a large purplish congenital mass of the thigh and forearm. Both showed signs of heart dysfunction, and one of them had anaemia and thrombocytopenia. The imaging assessment of the lesions showed well-defined subcutaneous solid masses with an exuberant vascular component. Both were kept under surveillance and maintenance therapy. A progressive dimensional reduction of the lesions supported the diagnosis of rapidly involuting congenital haemangioma (RICH). RICH is a rare vascular tumour that presents as a congenital purplish bulky mass. The diagnosis depends on the clinical evaluation of the lesion and the imaging characterisation of its solid components and vascular network. RICH may be complicated by high-output heart failure, anaemia and thrombocytopenia. Despite its exuberant presentation, it undergoes involution in the first year of life; therefore, early invasive therapies should be avoided. It is essential to detect any dimensional increase, suggesting more aggressive diagnoses, such as kaposiform haemangioendothelioma.

Keywords: congenital disorders; haemangioma; paediatric oncology.

Figure 1

(A,B) Clinical pictures of the reported lesions ((A) inferior limb of the female patient; (B) the forearm of the male patient) show large purplish and greyish lesions of the thigh and forearm, respectively, with a smooth surface and visible superficial veins (A). Note the desquamative aspect of the skin of the inferior limb (A).

Figure 2

(A,B) CT angiography images of the right thigh of the female patient which were acquired on the 19th day of life ((A) axial view; (B) three-dimensional reconstruction). CT imaging showed a large exophytic lesion localised in the anterior compartment of the thigh, predominantly located in the subcutaneous tissue, with diffuse contrast enhancement, reflecting solid constitution and hypervascularisation. The three-dimensional reconstruction (B) shows the exuberant vascular network that supplies the lesion.

Figure 3

(A,B) CT angiography images of the right thigh of the female patient which were acquired on the 19th day of life (simple coronal view), showing the calibre discrepancy between the right (A, arrow) and the left (B, arrow) primary iliac arteries, as a likely result of the arterial steal phenomenon in the right thigh.

Figure 4

(A–C) MRI of the forearm of the male patient which were acquired on the 10th day of life ((A) axial T2; (B) coronal T1; (C) coronal fat suppressed proton density), showing a homogeneous intermediate signal lesion in both ponderations, suggesting a solid component. Note the important vascular component, with multiple tortuous and dilated intralesional vessels extending superiorly through the arm. There is a predominance of rapid-flow vessels in the internal strand of the arm, with low luminal signal (asterisks) and slow-flow vessels in the external strand of the arm, with high luminal signals (arrows).

Figure 5

(A–D) Clinical pictures of the forearm of the male patient acquired at the time of birth and sequentially throughout the follow-up time ((A) initial presentation; (B) 3 months of follow-up; (C) 6 months of follow-up; (D) 9 months of follow-up), demonstrating marked dimensional involution, with progressively lighter colour and skin retraction. Note that the lesion is almost not discernible at 9 months of follow-up.