Rapidly involuting congenital haemangioma (RICH) of the liver

Abstract

RICH should be suspected in neonates who present with a solitary liver lesion and normal-for-age serum alpha-fetoprotein. Serial US scans should be used to confirm a progressive shrinkage of the lesion. Corticosteroids and β2-adrenergic antagonists have no proven effect in treating RICH. If the lesion grows, percutaneous needle biopsy is recommended to exclude a malignant tumour and to direct further management. Infants with cardiac failure should be treated medically. Embolization (with or without needle biopsy) should only be performed when this strategy fails.

Rapidly involuting congenital haemangioma of the liver

“…The lesions also differ in their clinical course. IH is typically small or undetectable at birth, grows rapidly in infancy and then gradually involutes 4. In contrast, the less common CHs are fully grown at birth then may shrink rapidly in early infancy and therefore are known as RICH, or do not involute and are therefore known as non-involuting congenital haemangioma (NICH).…”

RICH depuis la lettre

“…First, FranchiAbella and her colleagues [4] show that many, but not all, antenatally diagnosed liver haemangiomas behave like RICH. In a second paper, my colleagues and I demonstrate the imaging features of biopsy-proven hepatic RICH [5]. We also show in a literature review that many of the cases published as solitary hepatic infantile haemangioma (often called haemangioendothelioma) were almost certainly actually RICH.…”

“…Children with symptomatic hepatic RICH should be treated with aggressive supportive therapy, with embolisation reserved for those who develop uncontrollable cardiac failure [5,11]. Corticosteroid therapy has not been shown to be beneficial in RICH, and may cause serious complications [4].…”

Interventional radiology in infancy

“…Both infantile and congenital haemangiomas can occur within the liver; RICH lesions of the liver are usually large, solitary lesions while infantile haemangiomas are typically multifocal or diffuse. These latter lesions tended to be known as infantile haemangioendotheliomas but this confusing terminology has rightly fallen out of favour [11]. Although both types of liver lesion are benign and self-limiting, emergency embolisation may be required in the neonatal period due to massive cardiac overload or to overwhelming mass effect causing splinting of the diaphragm or abdominal compartment syndrome.…”

“…RICHs can be large and extremely hypervascular, and cause significant anxiety when they are found in a newborn child. They can be mistaken for common infantile haemangiomas or tumours and misdiagnosis or mistreatment is common [11]. Paediatric interventional radiologists tend to have considerable experience in the imaging and management of vascular anomalies and should be consulted early in such cases.…”