Peer-review started: August 31, 2015
First decision: September 28, 2015
Revised: October 19, 2015
Accepted: December 13, 2015
Article in press: December 14, 2015
Published online: February 2, 2016
Processing time: 154 Days and 23 Hours
Infantile hemangioma (IH) is the most common benign tumor seen in infancy. This review provides up-to-date information on the pathophysiology, variations in clinical presentation, and natural history of IH, elaborating on associated anomalies, such as PHACE(S) syndrome and LUMBAR syndrome. Because of the benign and self-limiting characteristics seen in more than 90% of cases of IH, a conservative approach is usually chosen. However, some circumstances, such as ulceration, vision loss, breathing difficulties, or potential disfigurement, will require treatment during the proliferative phase. For decades, treatment of IH has primarily consisted of corticosteroids or surgery. Since 2008, propranolol has become the treatment of first choice. In this article, we bring to light the crucial changes in the treatment of IH over the past years. To date, there is still a lack of data on the possible long-term effects of propranolol treatment in young infants. A theoretical probability of the central nervous system being affected (that is, impairment of short- and long-term memory, psychomotor function, sleep quality, and mood) has recently been suggested. This review highlights research topics concerning these long-term adverse effects. Finally, information is provided on the potential instruments to measure IH severity and activity in clinical trials and/or in clinical practice and the recently developed and first-validated IH-specific quality-of-life questionnaire.
Core tip: The discovery that propranolol is efficacious in the treatment of infantile hemangioma (IH) has led to an upsurge in publications, increasing our knowledge of this subject. In this review, we provide the most up-to-date information on the pathophysiology, variations in clinical presentation, and natural history of IH. We look at possible working mechanisms of several treatments and the current concerns regarding the treatment of first choice, propranolol. Finally, we provide an overview of instruments, measuring IH severity and/or activity and IH-related quality of life.