Rapidly involuting congenital haemangioma of the liver

Abstract

Rapidly involuting congenital haemangiomas (RICHs) are rare benign vascular tumours of infancy. They are generally asymptomatic, but can present with thrombocytopaenia and coagulopathy. Significant complications including life-threatening bleeding, high-output heart failure and liver failure, though rare, can occur. RICHs generally regress by 12-14 months of age and can be managed clinically with symptomatic treatment, watchful waiting and close monitoring of the size of the haemangioma. Medical management (corticosteroids, propranolol) has not shown to be effective, in contrast to infantile haemangioma which will not regress spontaneously and has been noted to respond to medical therapy. Awareness of this diagnosis is important to prevent unnecessary medical and surgical intervention. Here, we present a case of a full-term infant with RICH who presented with thrombocytopaenia and abnormal coagulation profile. The coagulopathy was treated symptomatically, while the lesion was observed with serial ultrasounds and gradually decreased in size.

Successful Use of Propranolol in a Symptomatic Giant Congenital Hepatic Hemangioma

“…CHHs have multiple managements, ranging from observation 24-26 or use of corticosteroids 16, 25, 27-30 to interferon 28 or vincristine, 27 demonstrating complete or partial involution. In refractory, severe, or noninvoluting cases, invasive procedures such as lobectomies, tumor resection, embolization, and even transplantation occur.…”

Pediatric Primary Hepatic Tumors: Diagnostic Considerations

“…Hepatic hemangiomas can be divided into congenital and infantile forms. Congenital hepatic hemangiomas (CHH) develop prenatally, are fully grown at birth, and are much more rare, comprising only 3% of all infantile hemangiomas [ 3 ]. They may demonstrate three distinct patterns of progression: those which rapidly involute in infancy, known as “rapidly involuting congenital hemangioma” (RICH); those that do not involute, known as “non-involuting congenital hemangioma” (NICH); or those that partially involute, known as “partially involuting congenital hemangioma” (PICH).…”

“…Infantile hepatic hemangiomas (IHH) are the most common benign vascular tumors in infancy, with a prevalence of 4.5% in term neonates, most commonly in white females [ 3 ]. They can be classified into three distinct subtypes: focal hepatic hemangiomas (FHH), multifocal hepatic hemangiomas (MHH), and diffuse hepatic hemangiomas (DHH) [ 4 ].…”

“…FHH may also develop prenatally and be present at birth. In fact, many authors consider FHH to be the same clinical entity as rapidly involuting congenital hemangioma (RICH), as they both stain negative for GLUT-1, unlike other forms of IHH [ 3 ].…”

“…On MRI and CT, this lesion appears as a solitary, spherical tumor with robust and rapid enhancement ( Figure 1 ). Calcification and central cystic changes are rarely seen with IH but are present in RICH and NICH [ 3 , 6 ]. In comparison to RICH, NICH is more likely to demonstrate intratumoral shunting on ultrasound and display prominent drainage veins on MRI.…”