A, More than 65% of the lower lip was involved. The IH caused substantial tissue expansion. More than 95% of the hemangioma was removed without microstomia. B, The remaining erythema was treated with pulsed-dye laser treatment.
IH shown before (A) and after (B) surgical excision. The tissue expander effect enabled complete removal without ectropion.
A and B, The absence of a tissue expander effect led to medialization of the eyebrows and a “unibrow” effect. C-E, The tissue expander effect is depicted by the arrowheads. Immediately after the operation, the mass is shown to have been resected without any medialization of the eyebrows.
A, Note the tissue expansion. B, The child is shown approximately a year after surgical removal of the IH, which took advantage of the tissue expansion.
A young girl with a large focal IH of the cheek (A) is treated with a 1-stage surgical excision (B) that takes advantage of the tissue expansion effect.
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Brennan TE, Waner M, O TM. The Tissue Expander Effect in Early Surgical Management of Select Focal Infantile Hemangiomas. JAMA Facial Plast Surg. 2017;19(4):282–286. doi:10.1001/jamafacial.2016.1991
Is early surgical management appropriate in select focal infantile hemangiomas?
In this case series of 5 children, tissue expansion effect of select focal infantile hemangiomas permitted primary closure without incurring significant functional or aesthetic deformity. This procedure also shortened the time needed to treat these patients with medication and observation.
Select focal infantile hemangiomas may be treated with surgery, taking advantage of the tissue expansion effect of the lesion itself to close the defect without significant aesthetic or functional deformity.
The current standard of treatment for infantile hemangiomas (IHs) involves initial observation for regression throughout infancy and childhood, with or without medical management with β-blocker medications. Approximately 50% of the lesions respond almost completely to this regimen. However, the remaining 50% of the lesions, especially established focal IHs of the lip, nose, eyelids, forehead, cheek, and scalp, do not regress completely with this regimen or do so leaving a deformity; among these lesions, early surgical management may result in a superior aesthetic and functional outcome.
To identify select focal head and neck lesions of IH that will likely not completely involute with medical management and that are ideal for a 1-stage surgical excision.
Design, Setting, and Participants
In this case series, records of infants and children presenting to a tertiary care vascular anomalies center for management of IHs by the senior author were reviewed. Representative examples of focal IHs of the lips, nose, eyelids, cheek, and glabella demonstrating the tissue expansion effect were selected for presentation. Expert opinion based on more than 20 years of experience of the senior surgeon treating more than 2000 patients with focal IH and long-term clinical follow-up is also provided.
Main Outcomes and Measures
Eradication of the IH while restoring aesthetic form and function to the face.
Five examples of patients with focal IHs of the lip, nose, eyelid, cheek, and glabella demonstrating the tissue expander effect who were successfully treated with surgery are presented. The 5 patients with these lesions ranged in age from 3 months to 5 years old, and all of them were female. One of these patients was treated with β-blockers, and another with steroids, with incomplete response to treatment prior to undergoing surgery. The tissue expander effect of a focal IH on adjacent, unaffected tissue facilitated excision of the lesion and primary closure without distortion of anatomical subunits in all 5 of these cases. Improved cosmesis with either improved or unaffected function was demonstrated.
Conclusions and Relevance
Clinicians should consider early surgical intervention in infants with select focal infantile hemangiomas in lieu of prolonged observation or medical management. The psychological benefit of early removal of these disfiguring lesions has not been quantified, but is subjectively apparent to clinicians and the families of patients. Furthermore, the costs and unknown long-term sequelae of β-blocker medication, which is the current standard of treatment for IHs along with observation for regression, have not yet been quantified but will gain increasing salience in the current medical climate.
Level of Evidence
Infantile hemangiomas (IHs) are the most common tumors of infancy. Girls are more commonly affected than boys, and 65% of IHs involve the head and neck.1,2 Infantile hemangiomas are also more common among fair-skinned infants. Facial IHs have been noted to occur more commonly at regions of embryologic fusion.3 Infantile hemangiomas may be focal or segmental, the latter of which may be associated with PHACES (posterior fossa brain malformations, hemangiomas [large segmental], arterial anomalies, cardiac/heart anomalies, ear/eye/endocrine abnormalities, sternal cleft/supraumbilical raphe) syndrome. Airway lesions may also be found in either a focal or segmental distribution and are particularly associated with V3 segmental IHs.
Infantile hemangiomas have a characteristic clinical course marked by early rapid proliferation in the first few months of life, followed by a quiescent period, and then a period of slower spontaneous involution beginning at approximately 9 months of age. Many lesions disappear with no treatment, whereas some respond to medical management alone and do not require surgery. Other lesions will only partially involute spontaneously or with medical management, and surgery will be used to address any residual cosmetic or functional deformity.
An objective or goal of this article is to emphasize that surgical treatment should play a primary role in the management of select lesions.4 These lesions include those that affect vital functions (breathing, eating, and vision), focal lesions resistant to medical therapy, ulcerated and bleeding lesions, and select large, cosmetically deforming lesions, such as those involving the lips, nose, eyelid, cheek, and glabella, that are likely to respond poorly to β-blocker (eg, propranolol) therapy. Delaying surgical intervention in favor of prolonged medical management for these patients is often not helpful and may negatively affect the psychosocial well-being of a child and his or her family.
Surgery should therefore be considered the primary treatment modality among focal IHs of the lips, nose, eyelids, forehead, glabella, cheeks, and scalp that have responded poorly to β-blocker treatment. At least 50% of these focal IHs treated with propranolol during infancy and childhood eventually require surgery and/or laser treatment owing to either partial involution on medical management or secondary deformities.5,6
In the expanded state of a IH before involution, there is often redundant skin and tissue that is useful to a surgeon when removing the lesion. In these instances, complete or near complete removal can be accomplished without distortion of the anatomical site. This effect is lost during involution or as a result of partial response to medical management. For this reason, it is beneficial to treat these large focal lesions surgically at an earlier stage.
Five representative cases were selected as examples. We selected 1 example of a patient with an IH from each of the following sites: lip, nose, eyelid, cheek, and glabella. The forehead and scalp are also sites that demonstrate the tissue expansion effect; however, because of figure limitations in this review, we do not show examples of those sites. The selected patients included infants and children with focal IHs whose lesions were removed surgically and defects closed primarily with good cosmetic and functional results. All patients were treated by the same primary surgeons (M.W. and T.M.O.) at a tertiary care referral center. Successful treatment is defined here as removal of the IH while restoring aesthetic form and function to the face. This study was approved by the institutional review board of Lenox Hill Hospital at Northwell Health System (previously Northshore LIJ Health System). The parents or guardians of all patients gave written informed consent.
The 5 patients included in this case series ranged in age from 3 months to 5 years at the time of surgery, and 3 were female. All the patients received treatment with β-blockers with incomplete response to treatment.
Figure 1A depicts a large IH that involves more than 65% of the lower lip. This lesion did not regress with medical management and continued to grow. The child underwent 4 months of treatment with propranolol prior to undergoing surgery at the age of 5.5 months. The IH itself caused substantial expansion of the lip, allowing for removal of greater than 95% of the IH and primary closure without resultant microstomia. Figure 1B depicts the small amount of residual lesion that remained and was subsequently treated with pulsed dye laser.
Figure 2 depicts a young girl with an IH of the lower eyelid that did not respond to medical management with propranolol. The tissue expansion effect of the focal IH resulted in enough expansion to allow more than the customary amount of resection. A wedge excision was performed without incurring significant eyelid shortening or ectropion, and a favorable cosmetic result was achieved. Early treatment allows reversal of astigmatism and any permanent detrimental effects to the vision that can occur owing to pressure from the mass. Ideally, eyelid lesions that produce unilateral astigmatism should be removed before age 9 months.
Figure 3A and B show a patient with a large focal infantile hemangioma of the glabella with significant ulceration. This patient’s lesion did not respond to medical management and was causing significant pain and bleeding. The child was treated at an outside institution with steroids for a month prior to undergoing excision at the age of 6 months. However, in this patient there was no significant expansion of the tissue adjacent to the mass in the glabellar region. The mass was removed, and the absence of a tissue expander effect necessarily led to medialization of the eyebrows creating somewhat of a “unibrow.”
Figure 3C-E shows another child who also had an IH of the glabella. Unlike the patient in Figure 3A and B, this child’s lesion produced a significant tissue expansion effect on the adjacent tissue in the glabella uninvolved by the mass. After removal of the lesion, this expanded tissue was easily reapproximated and closed without tension.
Figure 4 illustrates a child with an IH of the nasal dorsum, tip, alae, and sidewalls that incompletely responded to a course of steroid treatment. The lesion caused tissue expansion of the adjacent, unaffected tissue. A modified subunit approach was used to remove the IH, and primary closure was achieved with the help of the tissue expansion effect.7 The patient experienced improvement in her appearance, and her nasal obstruction was also alleviated by the surgery.
Figure 5 demonstrates the tissue expansion effect of a large focal IH of the cheek in a young girl. She was treated with a 1-stage surgical excision that involved only resection of involved skin and subcutaneous tissue. The IH extended to the deep fascial layer that covered the muscles of facial expression. All but a small rim of the cutaneous component of this IH was resected, and primary closure was achieved without distorting the contour of the cheek or creating tension on the adjacent lip, ala, or eyelid.
We demonstrate several cases of focal IH that were successfully treated by surgical excision. In particular, patients with focal IHs of the lips, nose, eyelid, forehead, and glabella whose lesions have not regressed with β-blocker therapy during the proliferative phase (first year of life) may benefit from early surgical intervention. These patients, if treated early, may be saved months of observation and/or treatment with propranolol, as well as years of living with a cosmetic and/or functional deformity, by opting for early surgical intervention.
Surgical intervention in infants and young children is technically easier to perform as a result of the tissue expansion effect of focal IHs. As the IH proliferates, it stretches the overlying skin and subcutaneous tissue. Infantile hemangiomas do not infiltrate adjacent tissue. Instead, they displace the surrounding tissues and condense a soft tissue plane around them. This plane facilitates surgical dissection and, provided the surgeon remains in this tissue plane during dissection, blood loss will be kept to a minimum. Stretching the overlying skin allows primary closure without undue skin tension or distortion of anatomy and an ideal wound healing environment in children. Infants and children younger than 3 years form less type II collagen than do older children and adults.8 Taking advantage of this biological effect will minimize fibrosis and result in a superior aesthetic result.
The tissue expander effect results from proliferation and expansion of the hemangioma. This in turn stretches the overlying skin and displaces adjacent tissue. This effect is not always evident but, if present, is only seen while the hemangioma is in an expanded state. It is lost during the late involution phase or after a partial response to propranolol because, as the lesion shrinks, the amount of expanded skin decreases. At this stage, skin involved with hemangioma may need to be left in place to achieve primary closure and not distort the anatomy.
It is worth reiterating that focal IHs, especially those that are treated at a later stage in their growth cycle and have grown to form a large mass, tend to respond less well to medical management.5 In addition to this, a subset of patients with focal IHs of the lip, nose, eyelids, forehead, cheek, glabella, or scalp may first present for treatment after the proliferative phase of the IH has ended. β-Blocker therapy at this late stage of a focal IH is not likely to succeed. In these cases, one may remove the lesion, taking advantage of the tissue expansion effect of the lesion itself on adjacent normal tissue to treat the lesions without leaving a significant cosmetic or functional deformity. Of course, one may also observe the child between the ages of 1 and 5 to 7 years to assess the degree of regression of the lesion before considering surgical intervention. In cases with no disfigurement, anatomical distortion, or functional impairment, this monitoring is an option. However, more often than not, even a fully regressed lesion may leave a mass of fibrofatty tissue or atrophic skin that still appears cosmetically disturbing on these areas of the face.
In the examples involving the lower lip, excision of the IH was performed and the lip was closed without creating significant microstomia. It is generally taught that a wedge resection of the lower lip should never be more than 30% of the lower lip to prevent microstomia. Hemangiomas of the lower lip invariably cause soft tissue expansion that stretches the adjacent lip, thereby allowing for a larger proportion of lip resection. We have often resected up to 50% of the lip in these cases. If the lesion were to involve a greater proportion of the lower lip, a wedge excision may be performed, leaving behind some hemangioma to facilitate closure without significant lip shortening. If the IH is still proliferating at the time of surgery, propranolol may be used to prevent further proliferation. In many cases, medical therapy is enough to treat the lesion without any appreciable cosmetic deformity of the lip. Later on in childhood, if a small amount of residual IH or atrophic/scarred skin is apparent, it may be treated with a minor surgical or laser procedure for cosmetic improvement.
In the example involving the eyelid, primary wound closure was achieved without distorting the upper or lower eyelid or causing ectropion. Eyelid function and closure were maintained owing to the tissue expansion effect created by the proliferating IH.
Concerning cheek IHs, the tissue expander effect will often enable surgical resection of most of the cutaneous component of the lesion while still preserving a normal contour of the cheek. This advantage will be lost if surgery is delayed and the IH partially involutes. In the case presented, we were able to resect the entire deep component and leave a small rim of cutaneous lesion to prevent too much shortening of the cheek. This small area was subsequently treated with a pulsed dye laser.
Early surgical intervention saves patients and their families multiple visits to physicians and prolonged exposure to a medication whose long-term effects in infants and children are still being studied. A typical course of β-blocker treatment for a child with an IH may last 6 months. Many parents object to the idea of having their child receive long-term medication. Although the cardiovascular safety profile of propranolol therapy has been validated in several studies,9,10 there are other adverse effects of propranolol that have not been examined in children. Studies in mice have suggested that propranolol may interfere with memory acquisition.11 Propranolol has been shown to block the consolidation and reconsolidation of emotional memories among healthy adults and shows promise as a therapy for adults with posttraumatic stress disorder.12 To our knowledge, there are no studies to date investigating the effect, if any, on memory acquisition among infants and young children exposed to 6 or more months of propranolol therapy for the treatment of IHs. Furthermore, one of the more common adverse effects of propranolol is sleep disturbance, which suggests that there is a central effect. These reasons favor early surgical intervention for patients with focal IHs in whom medical management has been shown to be less effective than among patients with segmental IHs.
Patients and their families experience great relief after a cosmetically deforming facial lesion is removed.2,13-16 Before treatment, many children are reluctant to socialize because they are ridiculed by peers who are confused and afraid of their disfiguring lesions. Many children with IHs are home schooled for this reason. Patients are aware of their different appearance at a very young age. After surgery, many parents will relate the complete turnaround their child experiences as he or she reenters his or her social world. Interaction and function at school are improved and their lives are significantly improved. Although the psychological effect of a large, disfiguring IH has not been fully evaluated, most psychologists agree that early intervention is preferable. For all of these reasons, we believe that early surgical intervention for a subset of patients with focal IHs is warranted.
Corresponding Author: Milton Waner, MD, FCS(SA), Vascular Birthmark Institute of New York, 210 E 64 St, Seventh Floor, New York, NY 10065 (email@example.com).
Accepted for Publication: November 4, 2015.
Published Online: February 2, 2017. doi:10.1001/jamafacial.2016.1991
Author Contributions: Drs Waner and O had full access to all the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis.
Study concept and design: All authors.
Acquisition, analysis, or interpretation of data: O.
Drafting of the manuscript: All authors.
Critical revision of the manuscript for important intellectual content: Waner, O.
Study supervision: Waner, O.
Conflict of Interest Disclosures: None reported.
Additional Contributions: We thank the patients’ families for granting permission to publish this information.