Plastic Surgical Considerations in Pediatric Dermatologic Abnormalities

Fig. 7.1
Partially obstructing bulky hemangioma of the left upper eyelid. This patient was treated with steroid therapy in 2003, prior to the advent of beta blocker therapy in 2008

In contrast, a capillary vascular malformation (port-wine stain) involving the V1 distribution of the trigeminal nerve is present at birth and enlarges proportionately (Fig. 7.2). During infancy a port-wine stain is usually flat, but hypertrophy can occur as the child matures.


Fig. 7.2
Port-wine stain. This capillary vascular malformation was present at birth and involves the V1 division of the right trigeminal nerve. Such patients require ophthalmologic examination to rule out glaucoma; a CT scan may be warranted if a seizure history suggests Sturge–Weber syndrome

Hemangiomas will generally grow until 6–12 months of age and then undergo a prolonged regression period over an average of 5 years. During involution the vessels shut down, the color lightens, and the mass softens. While many hemangiomas will significantly improve, some will leave behind fibrofatty bulky tissue and/or redundant skin. On the other hand, if the history indicates continued growth beyond the first year of life, one may be dealing with a slowly expanding venous, lymphatic, venolymphatic, or arteriovenous malformation. A plexiform neurofibroma may also present as a slowly expanding mass that fails to involute, and the correct diagnosis may be suggested by a positive family history of neurofibromatosis and other physical stigmata of this condition. If the parents or pediatrician reports a rapidly enlarging and intermittently bleeding “hemangioma” in a child well past the first year of life, it is more likely a pyogenic granuloma rather than a classic infantile hemangioma.

Interestingly, although hemangiomas are far more common than vascular malformations, the majority of vascular anomalies associated with syndromic newborns are not hemangiomas [2]. The majority are macular stains (stork bite) that tend to fade with time. Hemangiomas have been associated with a handful of midline defects (sternal clefting, midabdominal raphe, right-sided coarctation of the aorta, sacral and genitourinary defects), and large plaque-like facial hemangiomas have been associated with Dandy–Walker and other posterior fossa malformations (PHACES syndrome – posterior cranial fossa cerebellar cysts, hemangiomas, arterial abnormalities, cardiac abnormalities and coarctation, eye findings such as microphthalmia and cataract, and sternal clefting) [3].

Physical Examination

The physical examination usually confirms the diagnosis, particularly if the hemangioma is located in the dermis layer of the skin. Superficial hemangiomas display a classic strawberry red pigmentation. However, hemangiomas in the subcutaneous tissue appear bluish and have been mislabeled “cavernous” with the presumption that they contain dilated venules, but microscopic examination reveals proliferating capillaries identical to the dermal type. It is simply the deeper location that renders a bluish hue. In contrast, true venous vascular malformations tend to be softer and more compressible and may significantly expand when the head is held in a dependent position. In the older child, a venous malformation may also contain hard nodules (phleboliths), or they may show rapid painful enlargement and prolonged tenderness if rupture of the fragile ectatic vessels results in a transient hematoma.

Hemangiomas are often mixed lesions that have both a dermal and a subcutaneous component, with potential submuscular extension into the orbit. One will commonly see a central island of protruding bright red tissue atop a mound of bluish-tinted skin. It is the superficial component that is more likely to result in ulceration and permanent dermal scarring. Overdistention of the skin envelope may also result in atrophic skin redundancy, and incomplete resolution of the mass may result in residual fibrofatty tissue that will benefit from later judicious debulking. Purely subcutaneous and intramuscular hemangiomas may also expand the overlying skin, but there is greater potential for normal skin tone and texture following involution if the integrity of the dermis has not been violated. Twenty percent of infants will have more than one hemangioma, which may grow and regress at different rates and may differ in maximum size and degree of involution.

Hemangiomas in the periorbital region may involve either the upper or lower eyelid, the brow, the forehead, the paranasal regions and nasal dorsum, the cheek, or the temple. The orbit itself may be involved. Massive hemangiomas may involve all of these areas simultaneously.

Particular attention should be given to determining whether the hemangioma already obstructs vision or if continued growth will result in potential obstruction of the visual field. Extensive involvement of the head may also be associated with subglottic involvement, and any evidence of stridor or respiratory compromise warrants immediate otorhinolaryngology evaluation. Infants with large plaque-like facial hemangiomas and evidence of macrocephaly, enlarging head circumference, or unusual ophthalmologic findings (choroidal hemangioma, cryptophthalmos, microphthalmus, exophthalmos, and cataract) may have associated Dandy–Walker malformations (hypoplasia or absence of the cerebellar vermis and posterior fossa cysts) (Fig. 7.3). These patients may also display apnea, vomiting, posturing, developmental delay, and hemiparesis. The diagnosis can be confirmed with a computed tomography (CT) or a magnetic resonance imaging (MRI) scan [4].


Fig. 7.3
An infant with extensive facial hemangioma and PHACES syndrome that included coarctation of the aorta. She was treated with systemic steroid therapy to suppress the hemangiomas because of threatened visual obstruction

Lab and Imaging Studies

The majority of hemangiomas will not require radiologic studies for confirmation, but the extent of periorbital hemangiomas is best determined with MRI and magnetic resonance angiography (MRA). Gadolinium-enhanced MRI scanning demonstrates progressive and total filling of intraorbital vascular lesions. Distortion of the globe and orbital walls from mass effect can be appreciated. This will also help to distinguish a hemangioma from a venous or lymphatic malformation or a high-flow arteriovenous malformation. While contrast-enhanced CT scans will also show the extent of hemangiomas, the ability of MR scanning to demonstrate flow characteristics and the lack of radiation exposure makes it preferable to CT scanning in the vast majority of patients.

Arteriography is not necessary, although it has been described in the management of problematic hepatic hemangiomas associated with high-output cardiac failure, where embolization is a potential treatment option. Plane films are generally not useful. Hemangiomas rarely show bony distortion, although there may be some orbital enlargement from the expanding mass. In contrast, vascular malformations may show skeletal overgrowth or, in high-flow situations, bone destruction. The presence of a phlebolith would favor a venous or venolymphatic malformation instead of a hemangioma as the proper diagnosis. Ultrasound has been utilized to monitor hepatic hemangiomas and to scan the body for other vascular anomalies.

In the rare event that a biopsy is performed, typical infantile hemangiomas will demonstrate high immunoreactivity for glucose transporter protein 1 [5]. Tissue that is GLUT1 positive is considered diagnostic of classic hemangiomas, whereas the less common rapidly involuting congenital hemangioma (RICH) and non-involuting congenital hemangioma (NICH) are GLUT1 negative.

Clinical Course

The stimulus for angiogenesis usually persists until 6–12 months of age, but may extend to 18 months. As the hemangioma reaches the slow involutional phase, a plateau in growth is noted, the thrombosis and sclerosis of the tiny capillary vessels results in patchy gray pigmentation, and tissue turgor begins to diminish.

The average hemangioma takes about 5 years to fully involute, although some may take several years more. It is estimated that about 70% of hemangiomas will regress satisfactorily enough so as to need no intervention [6]. However, that still leaves at least 30% of patients who will have significant residual deformity (Fig. 7.4).


Fig. 7.4
Bulky pedunculated hemangioma that is composed of redundant skin and fibrofatty tissue. Surgical debulking was recommended to remove the obstructing soft tissue mass

Additionally, there are certain regions of the body where the risk of unacceptable scarring and residual deformity requiring reconstructive surgery is more likely, particularly when the lips, cheeks, and periorbital regions are involved. Additionally, since obstruction of the visual axis by an enlarging hemangioma for as little as 1 week during the first year of life can result in deprivation amblyopia, periorbital hemangiomas must be regarded as a potential ophthalmologic emergency (Fig. 7.5). There is a linear corelation between the duration of complete eye obstruction and the loss of visual acuity. Refractive errors can result from the mass effect of an adjacent hemangioma on the globe or cornea (anisometropic amblyopia), and hemangiomas involving the extraocular muscles can result in strabismus due to distortion and scarring of the muscles despite later involution. Retrobulbar hemangiomas can also compress the optic nerve.


Fig. 7.5
Right periorbital hemangioma with both a dermal and subcutaneous component, with potential obstruction of the right eye

The plastic surgeon who deals with hemangiomas should have a close working relationship with a multidisciplinary team of specialists familiar with all vascular anomalies. In addition to an interventional radiologist, dermatologist, and nursing coordinator, other team members may include a hematologist–oncologist, an orthopedic surgeon, and, when the periorbital region is involved, an oculoplastic surgeon and/or ophthalmologist.

Nonoperative Treatment of Periorbital Hemangiomas

While the plastic surgeon may choose to primarily manage the patient, any infant with a hemangioma that already does or may eventually obstruct even part of the visual field should be immediately evaluated by an ophthalmologist or oculoplastic surgeon. Thomson et al. [7] reviewed 51 patients with eyelid hemangiomas. Of these, 17 patients who had normal vision had only partial eyelid obstruction (smaller or more localized hemangiomas) that involuted at an earlier age (2.8 years) compared with patients with ultimately compromised vision. These favorable hemangiomas occupied only one-third of the lid margin, did not extend beyond the region of the eyelid, and/or involved only the lower lid.

Seven patients with isolated anisometropic (astigmatic) amblyopia had larger and bulkier lesions that took longer to resolve (4 years). Fifteen patients with deprivation amblyopia had large hemangiomas extending beyond the eyelid region that completely obstructed the eye for variable periods and, on average, took 4.5 years to resolve. They recommended measures to splint the affected eye open while patching the dominant eye. Lesions that were refractory to conservative measures were candidates for surgical excision if frequent ophthalmologic examinations showed developing amblyopia.

At our institution, the diagnosis of amblyopia secondary to an enlarging hemangioma in an infant is suggested by an abnormal Teller preferential looking test. A consultation between the pediatric ophthalmologist or oculoplastic surgeon and the plastic surgeon determines the need for a patching schedule for the contralateral side, and strong consideration is given for the immediate institution of suppressive antiangiogenic therapy.

Prior to 2008, intralesional or systemic steroids were the treatment of choice for problematic hemangiomas [811]. Oral steroid therapy was highly effective in up to 90% of patients, and regression was often observed within a week of steroid institution. However, the common side effects of steroids which included irritability, growth suppression, gastrointestinal upset, and hypertension prompted ophthalmologists to try intralesional injection with a combination of a rapid-acting steroid such a betamethasone and a long-acting steroid such as triamcinolone.

Intralesional steroid injection was promoted for the treatment of periorbital hemangiomas as early as 1978 by Mazzola [12], who treated 11 patients, with rapid benefit noted in 7 patients after 1 or 2 injections. Kushner was a strong proponent of intralesional steroid therapy in the ophthalmologic literature [13], citing the ease of administration, speed of action, paucity of side effects, and repeatability of injections. Using both a rapid-acting (betamethasone) and a long-acting (triamcinolone) steroid combination, visible change (blanching) was noted in as early as 1–2 days and dramatic results by 1 week. Doses ranged from 40 to 80 mg triamcinolone and 6 to 12 mg betamethasone per injection, delivered through 27-gauge needles. Most patients received two injections a month apart. In a later report, Kushner [14] limited his injections to 40 mg triamcinolone and 6 mg betamethasone because of potential complications of higher doses in small infants.

The safety of intralesional therapy for periorbital hemangiomas has been questioned [15, 16], with concerns noted regarding the use of general anesthesia during injection, the development of cholestrin plaques at the site of injection, and potential hematoma development and localized necrosis and scarring of the skin where concentrations of steroid might be heavy. Fat atrophy can also occur, with subsequent soft tissue contour abnormalities. Kushner himself noted the theoretical possibility of retinal artery occlusion with injections into retrobulbar or nasal mucosal hemangiomas. Furthermore, steroid injection into a vascular mass probably does not totally avoid systemic dosing, and the beneficial effect may not be due entirely to local effects.

Bilyk et al. [17] provide a comprehensive overview of treatment options for periorbital hemangiomas of infancy, again listing the risks and benefits of intralesional versus systemic steroid therapy. Other treatment options of historical interest include cryotherapy, which can cause skin scarring and hypopigmentation; surgical ligation of the feeding arteries, which has equivocal results on involution; radiation, which can cause damage to the lens, retina, periorbital bone, and overlying skin; sclerosing agents, which can cause significant destruction and scarring of local tissues; and embolization, which risks infarction of critical local tissues. While embolization has been reported, retinal artery occlusion and blindness is a significant risk. Embolization appears to have a more appropriate role in the treatment of high-output cardiac failure associated with visceral hemangiomas or as an adjunct to excision of high-flow arteriovenous malformations.

Interferon [1820] is also of mostly historical interest, having received a great deal of enthusiasm in the 1990s as an alternative to steroid therapy, but its many significant side effects including neurologic sequelae restricted its use to the most serious life- and vision-threatening situations where steroids were ineffective. With the advent of beta blocker therapy in 2008, both steroids and interferon have taken a back seat in the treatment of problematic hemangiomas.

Beta Blocker Therapy for Infantile Hemangiomas

Leauté-Labréze et al. reported 11 patients treated with propranolol who showed significant hemangioma regression [21]. The mechanism of action may be related to vasoconstriction, suppression of vascular endothelial growth factor and basic fibroblast growth factor, and stimulation of capillary endothelial cell apoptosis. At the Children’s Hospital of Philadelphia, propranolol has replaced steroids as first-line therapy for problematic hemangiomas, including those involving the periorbital area and airway. Initially, because of the potential cardiovascular, pulmonary, and hypoglycemic effects of beta blocker therapy, the propranolol protocol required hospital admission for blood pressure, heart rate, and glucose monitoring, while children were gradually administered therapeutic levels. Both inpatient and outpatient propranolol administration were directed by our dermatology colleagues. Currently, the dermatology protocol follows the FDA guideline that children of 45 weeks gestation or older are candidates for outpatient propranolol therapy if they have a normal EKG. Blood pressure and heart rate are monitored for 1–2 h during an initial partial dose administration and again the following week when the full dose is administered. Propranolol appears to have fewer adverse side effects and may even be more effective than steroids in suppressing hemangiomas and causing them to involute more rapidly (Figs. 7.6 and 7.7).


Fig. 7.6
(a, b) Periorbital and forehead hemangioma treated with propranolol with excellent suppression and regression within 4 months (Courtesy James Treat, MD)


Fig. 7.7
(a, b) Lateral eyelid hemangioma treated with propranolol with excellent regression (Courtesy James Treat, MD)

A review of 100 patients with periocular hemangiomas treated with propranolol revealed it was the first-line treatment in the majority, with improvement in 96% of patients and minimal side effects. The most common dose was 2 mg/kg/day [22].

Topical beta blocker in the form of timolol maleate gel, 0.25% applied twice daily, may also be effective and is easily applied by parents [23].

Laser Therapy for Periorbital Hemangiomas

Historically, a variety of vascular lesion lasers have been used for the treatment of undesirable cutaneous blood vessels [2429]. All operate on the principle that blood (oxyhemoglobin) absorbs certain wavelengths of light preferentially, thereby allowing selective or semiselective destruction of blood vessels by heating the blood inside. These have included argon (wavelength 488,515 nm; blue-green light), KTP (wavelength 532 nm, green light), pulsed yellow dye (current wavelength 595 nm, yellow light), tunable yellow dye (wavelength 577–630 nm, usually tuned to 585 nm), and copper vapor (wavelength 578 nm, yellow light) lasers. The pulsed yellow dye laser delivers a high-energy pulse of laser light that causes rapid expansion and rupture of the blood vessels, resulting in purpura. All of the other lasers cause vascular coagulation and blanching. None of the lasers can penetrate more than 1–2 mm into the skin, and therefore treatment is limited to thin cutaneous lesions. Neodymium:YAG lasers (wavelength 1064) will penetrate 5–6 mm, but the light is not specifically absorbed by blood. It will cause nonspecific deep thermal coagulation, and therefore has potential in treating highly vascular lesions, but at the risk of significant thermal damage to surrounding tissue.

The pulsed yellow dye laser has become well accepted as the laser of choice for pediatric port-wine stains, because of its low risk of scarring, favorable results, and rapidity and ease of delivery [30]. It may be very effective in aborting small flat hemangiomas, but in many cases, rebound growth may warrant repeated laser treatments until the hemangioma undergoes permanent regression. In such cases, interim oral propranolol or topical application of timolol may help to suppress rebound growth.

In general, thick hemangiomas do not appear to respond well to laser because of the limited depth of penetration. They may lose their bright red surface pigmentation without decreasing in size and may still require surgical debulking in the case of an obstructing hemangioma.

The pulsed dye laser is best used for early flat hemangiomas or for those that have regressed but have left persistent vascular pigmentation in the dermis (Fig. 7.8).


Fig. 7.8
In addition to treating early hemangiomas, the pulsed yellow dye laser is useful for residual vascular pigmentation after hemangiomas have involuted. In this case, redundant skin and scar tissue from prior ulceration will be excised, while the peripheral hemangioma is treated with the laser. Note the protective corneal shield

Operative Therapy for Periorbital Hemangiomas

Small hemangiomas can be excised or debulked using standard surgical techniques. If the surgeon has the luxury of being able to stay away from the hemangioma, the dissection is through normal tissue planes, and bleeding is minimal. Usually there are 1–2 feeding vessels, and vascular control and hemostasis are straightforward. Ideally, incisions are placed inconspicuously or along natural expression lines, but this is not always possible depending on the size and location of the hemangioma (Fig. 7.9).


Fig. 7.9
(a) Redundant lower eyelid skin and fibrofatty subcutaneous tissue after hemangioma regression. (b) In this case, it was not possible to remove all involved skin without placing excessive traction on the lower lid

Certain problematic periorbital hemangiomas, however, are not amenable to complete excision, and debulking becomes necessary to prevent or relieve visual obstruction. In such cases, incisions must be made within the hypervascular tissue, and hemostasis may be difficult. Electric cautery, particularly the bipolar cautery, is extremely useful to shrink and seal off engorged capillary vessels, and the bipolar forceps can even be used to dissect through the hemangioma by pinching the tissue with the tips of the forceps. Standard cautery tends to be less hemostatic, but may be useful for making initial incisions. Vaporization with a CO2 laser can also prove useful, but the laser does not coagulate as well as other lasers that target oxyhemoglobin. After removing the necessary bulk of the hemangioma, residual raw edges must be meticulously cauterized or oversewn to avoid a hematoma. Suture closure with a tapered needle is preferable to a cutting needle, to avoid further bleeding.

Regressed hemangiomas that have left redundant skin and fibrofatty tissue as well as persistent vascular pigmentation may be treated by a combination of excision and pulsed yellow dye laser photocoagulation (Fig. 7.10).


Fig. 7.10
Upper lid hemangiomas often leave redundant skin due to their tissue expansive effect. Excess skin and soft tissue can be debulked using a blepharoplasty-type pattern

Other Vascular Lesions

Pyogenic Granulomas

Pyogenic granulomas are neither pyogenic nor are they granulomas, but this misnomer appears to be forever entrenched in our medical terminology. More accurately termed lobular capillary hemangiomas , these are truly proliferative vascular lesions that may occur throughout childhood and adulthood. The history of a rapidly enlarging and engorged vascular lesion with periodic bleeding, in a patient that is usually beyond infancy, is almost diagnostic. By the time the child presents to a plastic or oculoplastic surgeon, he/she has usually been to the emergency room for troublesome bleeding, and one usually observes a Band-Aid in place and some localized black staining secondary to prior attempts at silver nitrate cauterization (Fig. 7.11).


Fig. 7.11
(a) Pyogenic granuloma . A typical pyogenic granuloma exhibiting crusting and silver nitrate staining in the area of ulceration and bleeding. There is also adhesive residue on the skin, the “positive Band-Aid sign.” (b) Pyogenic granulomas that have not ulcerated have a fragile epidermal layer overlying a red nodule of proliferating capillaries

Patrice et al. [31] reviewed 178 cases of pyogenic granulomas, of which two-thirds were located on the head and neck, including 9% involving the lips or eyelids. A thin and easily traumatized epidermal covering over a protruding and often pedunculated vascular mass characterizes these lesions, which, in fact, are neither pyogenic nor granulomas. Microscopically, there are lobules of proliferating capillaries, of which the pericyte rather than the endothelial cell is the major hyperplastic cellular component. The etiology is unclear, but may be associated with minor trauma, an insect bite, localized viral infections, underlying port-wine stains (capillary vascular malformations), or other underlying dermatologic disorders that may result in an angiogenic stimulus.

While some pyogenic granulomas may spontaneously disappear, they are usually problematic enough because of their annoyingly frequent bleeding episodes to warrant interventional therapy. Since the vessels usually penetrate into the deep dermis, shave excision alone or superficial laser or cautery may not be sufficient to obliterate the dermal component. Direct surgical excision is probably the most expeditious and the safest way to remove eyelid pyogenic granulomas. Shave excision followed immediately by cauterization or laser photocoagulation of the base has a high rate of success in other areas, but may risk thermal injury of the delicate eyelid tissues. Removed tissue should always be sent for pathologic confirmation. Protective eyeshields should always be used if a laser is involved.

Capillary Vascular Malformations (Port-Wine Stains)

Capillary vascular malformations occur in 3 out of every 1000 births. Most commonly called port-wine stains, they are composed of dilated ectatic dermal capillaries. Unlike hemangiomas, they are present at birth and grow proportionately with the child. Many appear to roughly follow a dermatome distribution, which supports the theory that improper neuronal influence during development leads to a lack of sympathetic vasomotor control. Unlike macular stains, which are commonly located in the glabellar, central forehead, and nape of the neck areas, capillary vascular malformations do not lighten with time. In fact, it may be difficult to clinically distinguish a macular stain from a port-wine stain, but a history of gradual spontaneous fading favors the former diagnosis. In contrast, many pale childhood port-wine stains will gradually darken with time and develop some bulky nodularity as the small capillaries and venules gradually dilate in adulthood.

Parents should be advised that these malformations will not disappear with time. They can be reassured that they are nonhereditary in nature and that the pattern of vascular pigmentation will not spread to uninvolved areas.

Capillary vascular malformations involving the V1 distribution of the trigeminal nerve have special significance for the physician, since these patients may have associated neuro-ocular symptoms. Sturge–Weber syndrome is characterized by a facial capillary vascular malformation involving at least a portion of the V1 cutaneous area, with ipsilateral leptomeningeal vascular anomalies, and possible ipsilateral choroidal vascular lesions with glaucoma. The neurologic symptoms may include childhood seizures, hemiparesis or hemiplegia, gyriform intracranial calcifications, and cerebral atrophy [32]. All of these tissues are derivatives of the neurectodermal germ layer, suggesting a morphogenetic error arising in a limited part of the cephalic neural crest. Patients with capillary vascular malformations involving the V1 distribution should have repeated ophthalmologic examinations to rule out glaucoma and a CT or an MRI scan to rule out ocular–CNS involvement.

Unlike capillary vascular malformations on the trunk and extremities, facial port-wine stains are highly visible. Although cover makeup can help to camouflage the vascular pigmentation, it requires daily application and tends to give the skin a thick opaque texture. Hypertrophic malformations with a nodular surface cannot be easily hidden by makeup.

Laser photocoagulation is now considered to be the standard of care for pediatric and adult capillary vascular malformations (Fig. 7.12). Previous modalities such as staged excision, excision with skin grafting, and tattoo pigmentation are no longer appropriate for pediatric capillary vascular malformations. Surgical treatments will leave permanent surgical scars and are best reserved for adolescent or adult port-wine stains that have developed significant hypertrophy and excessive soft tissue redundancy that is unresponsive to laser therapy. It is unusual to see hypertrophic periocular skin in children, unless a particularly dark port-wine stain is present, suggesting that there is a dense network of dilated capillaries and venules that gradually engorges with time. The goal of serial laser treatments is to not only lighten the vascular pigmentation but also prevent future hypertrophy and soft tissue distortion.
Dec 19, 2017 | Posted by in OPHTHALMOLOGY | Comments Off on Plastic Surgical Considerations in Pediatric Dermatologic Abnormalities

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