Hemangiomas don’t always resolve, so refer

NEW YORK – Too many parents are incorrectly reassured that their child’s hemangioma will spontaneously resolve, and that no immediate treatment is needed, according to Dr. Francine Blei, medical director of the Vascular Birthmark Institute of New York at Roosevelt Hospital.

"I cannot stress enough the importance of early referral," Dr. Blei said.

Although some hemangiomas will resolve with time, those that require treatment will have the lowest risk for functional defects and the best cosmetic outcome if intervention occurs early, Dr. Blei said at the American Academy of Dermatology summer meeting.

For clinicians with experience managing these lesions, early examination can help separate those at risk of permanent disability or disfigurement from those whose lesions will resolve with an acceptable result, she said.

Infantile hemangiomas are part of an array of vascular anomalies, of which the most serious can involve internal organs and be life-threatening. Based on emerging information regarding the etiology and histopathology of vascular anomalies, the International Society for the Study of Vascular Abnormalities (ISSVA) has updated its classification system several times. Given recent progress, particularly related to the role of genetics in the pathophysiology of these lesions, new terminology is "in the works" and will be a topic of discussion at the ISSVA Workshop on Vascular Anomalies in April 2014 in Melbourne, Dr. Blei said. The two broad categories in the current terminology separate vascular tumors (such as infantile hemangiomas, which occur in up to 10% of infants) from vascular malformations, which are not necessarily congenital.

"The inconsistent terminology has been a problem," noted Dr. Blei, citing a study from the Vascular Anomalies Center at Boston Children’s Hospital. Data from this study showed that only 53% of 5,621 cases reviewed had a correct referral diagnosis (J. Pediatr. Surg. 2011;46:1784-9). Although referral diagnoses were correct for 77.8% of infantile hemangiomas, the rate was only 45.6% for vascular malformations.

For infantile hemangiomas, Dr. Blei offered a series of simple questions for clinicians to ask to help determine the risk of an adverse outcome. Although size and location are important, she emphasized looking for associated symptoms, such as stridor, ptosis, ulceration, and bleeding. While some parents may seek a conservative approach in the hope that the lesions will resolve without an invasive procedure, Dr. Blei said that she is "dogmatic" in regard to urging aggressive intervention when there is a risk of persistent functional impairment.

Other questions useful in understanding the prognosis and guiding the therapy of infantile hemangiomas include whether the lesion has subcutaneous involvement, whether there are multiple lesions, and whether the lesion is focal or segmental. For segmental lesions, a particular concern is whether the hemangioma is a manifestation of a larger syndrome, such as PHACE, a condition that includes cardiac abnormalities, and LUMBAR, which includes spinal and urogenital complications.

The age of the child at the time of evaluation is also relevant, particularly for differentiating infantile from congenital hemangiomas, Dr. Blei noted. Infantile hemangiomas begin proliferation soon after birth and, on average, reach their peak proliferation at 8-10 months of age before involuting slowly. By contrast, congenital hemangiomas are fully developed at birth and, unlike infantile hemangiomas, are negative for the immunohistochemical marker GLUT1. One kind of congenital hemangioma, called RICH (rapidly involuting congenital hemangioma), reaches an advanced stage of spontaneous resolution within the first several months of life. The other major form, called NICH (noninvoluting congenital hemangioma) persists through childhood.

If infantile hemangiomas are identified early, their treatment may be more effective and less invasive, said Dr. Blei. She noted that an adequate response can be achieved with topical beta-blockers in some cases. Oral beta-blockers and other systemic therapies also may allow for a relatively conservative resolution. Pulse dye lasers have a greater likelihood of efficacy if used early rather than after the hemangioma has become bulky, she added.

"If you treat early, you limit the size," Dr. Blei said. If you wait, the opportunity for complete or near-complete resolution may be lost, she added.

Dr. Blei had no financial conflicts to disclose.

NEW YORK – Too many parents are incorrectly reassured that their child’s hemangioma will spontaneously resolve, and that no immediate treatment is needed, according to Dr. Francine Blei, medical director of the Vascular Birthmark Institute of New York at Roosevelt Hospital.

"I cannot stress enough the importance of early referral," Dr. Blei said.

Although some hemangiomas will resolve with time, those that require treatment will have the lowest risk for functional defects and the best cosmetic outcome if intervention occurs early, Dr. Blei said at the American Academy of Dermatology summer meeting.

For clinicians with experience managing these lesions, early examination can help separate those at risk of permanent disability or disfigurement from those whose lesions will resolve with an acceptable result, she said.

Infantile hemangiomas are part of an array of vascular anomalies, of which the most serious can involve internal organs and be life-threatening. Based on emerging information regarding the etiology and histopathology of vascular anomalies, the International Society for the Study of Vascular Abnormalities (ISSVA) has updated its classification system several times. Given recent progress, particularly related to the role of genetics in the pathophysiology of these lesions, new terminology is "in the works" and will be a topic of discussion at the ISSVA Workshop on Vascular Anomalies in April 2014 in Melbourne, Dr. Blei said. The two broad categories in the current terminology separate vascular tumors (such as infantile hemangiomas, which occur in up to 10% of infants) from vascular malformations, which are not necessarily congenital.

"The inconsistent terminology has been a problem," noted Dr. Blei, citing a study from the Vascular Anomalies Center at Boston Children’s Hospital. Data from this study showed that only 53% of 5,621 cases reviewed had a correct referral diagnosis (J. Pediatr. Surg. 2011;46:1784-9). Although referral diagnoses were correct for 77.8% of infantile hemangiomas, the rate was only 45.6% for vascular malformations.

For infantile hemangiomas, Dr. Blei offered a series of simple questions for clinicians to ask to help determine the risk of an adverse outcome. Although size and location are important, she emphasized looking for associated symptoms, such as stridor, ptosis, ulceration, and bleeding. While some parents may seek a conservative approach in the hope that the lesions will resolve without an invasive procedure, Dr. Blei said that she is "dogmatic" in regard to urging aggressive intervention when there is a risk of persistent functional impairment.

Other questions useful in understanding the prognosis and guiding the therapy of infantile hemangiomas include whether the lesion has subcutaneous involvement, whether there are multiple lesions, and whether the lesion is focal or segmental. For segmental lesions, a particular concern is whether the hemangioma is a manifestation of a larger syndrome, such as PHACE, a condition that includes cardiac abnormalities, and LUMBAR, which includes spinal and urogenital complications.

The age of the child at the time of evaluation is also relevant, particularly for differentiating infantile from congenital hemangiomas, Dr. Blei noted. Infantile hemangiomas begin proliferation soon after birth and, on average, reach their peak proliferation at 8-10 months of age before involuting slowly. By contrast, congenital hemangiomas are fully developed at birth and, unlike infantile hemangiomas, are negative for the immunohistochemical marker GLUT1. One kind of congenital hemangioma, called RICH (rapidly involuting congenital hemangioma), reaches an advanced stage of spontaneous resolution within the first several months of life. The other major form, called NICH (noninvoluting congenital hemangioma) persists through childhood.

If infantile hemangiomas are identified early, their treatment may be more effective and less invasive, said Dr. Blei. She noted that an adequate response can be achieved with topical beta-blockers in some cases. Oral beta-blockers and other systemic therapies also may allow for a relatively conservative resolution. Pulse dye lasers have a greater likelihood of efficacy if used early rather than after the hemangioma has become bulky, she added.

"If you treat early, you limit the size," Dr. Blei said. If you wait, the opportunity for complete or near-complete resolution may be lost, she added.

Dr. Blei had no financial conflicts to disclose.

NEW YORK – Too many parents are incorrectly reassured that their child’s hemangioma will spontaneously resolve, and that no immediate treatment is needed, according to Dr. Francine Blei, medical director of the Vascular Birthmark Institute of New York at Roosevelt Hospital.

"I cannot stress enough the importance of early referral," Dr. Blei said.

Although some hemangiomas will resolve with time, those that require treatment will have the lowest risk for functional defects and the best cosmetic outcome if intervention occurs early, Dr. Blei said at the American Academy of Dermatology summer meeting.

For clinicians with experience managing these lesions, early examination can help separate those at risk of permanent disability or disfigurement from those whose lesions will resolve with an acceptable result, she said.

Infantile hemangiomas are part of an array of vascular anomalies, of which the most serious can involve internal organs and be life-threatening. Based on emerging information regarding the etiology and histopathology of vascular anomalies, the International Society for the Study of Vascular Abnormalities (ISSVA) has updated its classification system several times. Given recent progress, particularly related to the role of genetics in the pathophysiology of these lesions, new terminology is "in the works" and will be a topic of discussion at the ISSVA Workshop on Vascular Anomalies in April 2014 in Melbourne, Dr. Blei said. The two broad categories in the current terminology separate vascular tumors (such as infantile hemangiomas, which occur in up to 10% of infants) from vascular malformations, which are not necessarily congenital.

"The inconsistent terminology has been a problem," noted Dr. Blei, citing a study from the Vascular Anomalies Center at Boston Children’s Hospital. Data from this study showed that only 53% of 5,621 cases reviewed had a correct referral diagnosis (J. Pediatr. Surg. 2011;46:1784-9). Although referral diagnoses were correct for 77.8% of infantile hemangiomas, the rate was only 45.6% for vascular malformations.

For infantile hemangiomas, Dr. Blei offered a series of simple questions for clinicians to ask to help determine the risk of an adverse outcome. Although size and location are important, she emphasized looking for associated symptoms, such as stridor, ptosis, ulceration, and bleeding. While some parents may seek a conservative approach in the hope that the lesions will resolve without an invasive procedure, Dr. Blei said that she is "dogmatic" in regard to urging aggressive intervention when there is a risk of persistent functional impairment.

Other questions useful in understanding the prognosis and guiding the therapy of infantile hemangiomas include whether the lesion has subcutaneous involvement, whether there are multiple lesions, and whether the lesion is focal or segmental. For segmental lesions, a particular concern is whether the hemangioma is a manifestation of a larger syndrome, such as PHACE, a condition that includes cardiac abnormalities, and LUMBAR, which includes spinal and urogenital complications.

The age of the child at the time of evaluation is also relevant, particularly for differentiating infantile from congenital hemangiomas, Dr. Blei noted. Infantile hemangiomas begin proliferation soon after birth and, on average, reach their peak proliferation at 8-10 months of age before involuting slowly. By contrast, congenital hemangiomas are fully developed at birth and, unlike infantile hemangiomas, are negative for the immunohistochemical marker GLUT1. One kind of congenital hemangioma, called RICH (rapidly involuting congenital hemangioma), reaches an advanced stage of spontaneous resolution within the first several months of life. The other major form, called NICH (noninvoluting congenital hemangioma) persists through childhood.

If infantile hemangiomas are identified early, their treatment may be more effective and less invasive, said Dr. Blei. She noted that an adequate response can be achieved with topical beta-blockers in some cases. Oral beta-blockers and other systemic therapies also may allow for a relatively conservative resolution. Pulse dye lasers have a greater likelihood of efficacy if used early rather than after the hemangioma has become bulky, she added.

"If you treat early, you limit the size," Dr. Blei said. If you wait, the opportunity for complete or near-complete resolution may be lost, she added.

Dr. Blei had no financial conflicts to disclose.