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Congenital Nevi

  • Bruce S. BauerEmail author
  • Sara R. Dickie
Living reference work entry
  • 6 Downloads

Abstract

This chapter provides a review of different types of congenital lesions of the skin including their natural history, diagnosis, classification, and treatment options. Most lesions can be approached surgically through a variety of techniques. This chapter aims to provide a familiarity with the diagnosis and help the reader develop a management strategy which satisfies both a functional and aesthetic outcome for these patients.

Keywords

Congenital nevi Blue nevi Café au lait spots Malignancy 

Introduction

Pigmented lesions are a common diagnosis encountered by the pediatric surgeon. Congenital nevi represent a group of skin lesions which occur at birth or within the first several years of life and are characterized by ectopic nests of dermal elements. Although most commonly melanocytic in nature (such as the congenital melanocytic nevus, Nevi of Ota, Nevi of Ito, nevus spilus, café au lait spots and Mongolian spots), nevi can also originate from epidermal (Nevus sebaceous of Jadassohn) and neural elements. Lesion characteristics vary according to the type of cell involved, location within the skin, and level of cell differentiation. Knowledge of the differential diagnosis and natural history of these lesions can help balance the plan of care, so as to address the potential risk of malignant degeneration while accounting for functional and aesthetic concerns encountered during excision and reconstruction.

Congenital Melanocytic Nevi

Congenital melanocytic nevi (CMN) are the most common congenital nevi. They are composed of melanin producing cells originating from the neural crest which carry a variable amount of pigment. Historically, these nevi have been classified based on their expected size at adulthood (Fig. 1ad); small (<1.5 cm), intermediate (1.5–19.9 cm), large (>20 cm), and giant (>50 cm) (Kopf et al. 1979; Marghoob et al. 1999). Another cited definition of large congenital nevi includes lesions >2% total body surface area (Quaba and Wallace 1986). These classification groups are important not only when determining treatment options, but when stratifying the potential risk of malignant change. Recently, a more standardized classification has been introduced and has been adopted by the greater nevus community (Krengel et al. 2013). This consensus-based schema categorizes nevi by their cutaneous features including size, number of satellites, anatomic location, color heterogeneity, surface rugosity, hypertrichosis, and dermal nodularity. Prospective analysis using this system will hopefully allow practitioners to risk stratify individuals for the presence of neurocutaneous melanosis (NCM) and validate observed melanoma risk.
Fig. 1

Demonstrating classification of nevi by size. (a) Small, <1.5 cm (b) Intermediate, 1.5–19.9 cm (c) Large, >20 cm (d) Giant, >50 cm

When evaluating a lesion in a baby, body surface area changes between infancy and adulthood can be used to predict if a lesion falls into the large category. As a general rule, a 12 cm lesion on the head or neck or 7 cm lesion on the body of an infant will grow to meet the large nevi classification (Marghoob et al. 1996). Incidence of CMN decreases with increasing size of the lesion with the relatively common small lesions occurring in 1:100 births, intermediate lesions in 1:1000, large lesions in 1:20,000, and giant lesions in 1:500,000 births (Quaba and Wallace 1986; Alper and Holmes 1983; Illig et al. 1985).

Etiology

Embryologically, melanocytes originate from neural crest cells as melanoblasts. During early gestation, these melanoblasts migrate to the basal layer of the epidermis at which time they differentiate into dendritic melanocytes and associate into melanosomes that produce pigment for transfer to keratinocytes. Congenital melanocytic nevi result from a disturbance in this migration and differentiation process resulting in ectopic nests of immature cells along their course of migration. Because of this, nevus cells often extend deep into the subcutaneous tissues and can even involve fascia, muscle, periosteum, or the leptomeninges. Congenital melanocytic nevi (CMN) overwhelmingly occur in a sporadic pattern with rare reports of familial cases. There have been several studies seeking to identify genetic hits underlying CMN pathogenesis, and the results of these studies suggest NRAS mutations and, to a lesser extent BRAF mutations, contribute to the development of CMN (Roh et al. 2015). BRAF or NRAS mutations are typically found in small CMN, but the prevalence of NRAS mutations is greater among medium to giant CMN (Charbel et al. 2014).

When nevus cells are found within the central nervous system (leptomeninges, brain, or spinal column), the condition is called neurocutaneous melanosis (NCM) (Fig. 2a). The clinical presentation of NCM can range from asymptomatic to progressive, severe neurologic deterioration with developmental delay, hydrocephalus, seizures, and death. Large CMN of the posterior midline and those found in association with greater than 20 satellite nevi have the highest risk of NCM and should be considered for MRI screening (Marghoob et al. 2004) (Fig. 2b).
Fig. 2

(a) A T-1 weighted magnetic resonance image of the brain demonstrates several lesions typical of NCM. (b) Large CMN of the posterior midline and those with greater than 20 satellite nevi have the highest risk of NCM

Although it is difficult to know the true incidence of NCM in association with large CMN because screening of asymptomatic patients is not universally performed, a study of an internet registry found NCM on MRI in approximately 5% of cases of large or giant CMN of which only 5–6% became symptomatic (Agero et al. 2005). Bett reported an incidence of 7.5% in a large cohort of at-risk nevus patients with a mortality rate of 34% in symptomatic patients (Bett 2006).

Symptoms are produced by either malignant degeneration or the benign proliferation of nevus cells within the central nervous system which can block the circulation of cerebral spinal fluid leading to hydrocephalus and increased intracranial pressure. If NCM is to become symptomatic, the vast majority of cases will do so before age 5 years (Bett 2006).

Pathology

Nevus cells differ from melanocytes histologically as they group together in clusters rather than arrange as individual melanosomes. In addition, nevus cells assume a rounded rather than dendritic shape and keep their pigment within their cytoplasm instead of transferring it to the surrounding keratinocytes (Elder and Elenitsas 1997).

A higher risk of malignant transformation has been associated with CMN compared to acquired nevi. For this reason, efforts have been made to identify characteristics specific to CMN. Clinically, examination of small CMN with a dermatoscope or under loupe magnification will reveal small pigment granules at the peripheral aspect of the lesion which is a specific finding to CMN (Alper et al. 1979) (Fig. 3). Histological characteristics of CMN show nevus cells within eccrine ducts, follicular epithelium, and/or blood vessels. However, not all CMN display these findings (Rhodes et al. 1986). Only congenital lesions demonstrate nevus cells within the deeper subcutaneous tissues, fascia, nerve sheath, and muscle.
Fig. 3

Small pigment granules associated with the nevus are specific to CMN. (Image courtesy of Joel Joyce, MD, Dermatology)

Presentation

CMN can differ greatly in their size and appearance. Often obvious at birth, some lesions can be quite faint and seem to appear with time over the 1st year of life as they increase in pigmentation. Coloration varies from pale tan to a deep bluish black and may be uniform or extremely variegated. The lesions are often thickened in texture with increased skin markings compared to surrounding normal skin. They often have hair which can range from fine light fuzz to coarse thick follicles. When large lesions are present, they may be accompanied by smaller peripheral satellite lesions of different sizes and numbers which can appear over the course of a lifetime, but predominantly before age three.

Over time, the surface of CMN may change to become verrucous and irregular with darkening of pigmentation. Lightening of color can also be seen in as many as a third of patients with large and giant nevi. Erosion or breakdown is not uncommon in the neonatal period, but may also occur later. This does not necessarily indicate malignant change but warrants further investigation.

Management

The management strategy of CMN can be stratified based on size of the lesion and potential risk of malignant change.

Small and Intermediate Congenital Melanocytic Nevi

Most CMN fall into the small category and can usually be addressed with simple or staged excision (Fig. 4). A large systematic evaluation of small CMN and melanoma risk has yet to be published, so the actual risk of malignant change in these is unknown and controversial. In a large population-based study of nearly 4000 individuals with CMN, none developed melanoma over 10 years (Berg and Lindelof 2003). When based on patient reported history of a congenital lesion, the lifetime risk of melanoma occurring in small CMN has been quoted at 4.9% but lowers to 2.6% for lesions determined to be CMN on histologic criteria (Rhodes and Melski 1982). The risk of melanoma arising within small CMN before puberty is extremely unlikely (Scalzo et al. 1997). For this reason, most defer the removal of these lesions until the patient reaches an age at which comfortable excision can be performed under local anesthesia to avoid the associated risks of general anesthesia. However, if the lesion falls in a cosmetically sensitive area or is located in an area that will require general anesthesia regardless of age, consideration should be given to earlier removal to avoid potential psychologic sequella of delaying treatment. From a practical point of view, these procedures are best performed either before the patient begins toddling or just prior to school entrance in order to avoid potential complications from falls, heightened anxiety, and lack of patient cooperation found at the ages in between.
Fig. 4

Staged excision of an intermediate sized nevus of the cheek (a) preoperative photo; (b) 6 months after first stage excision; (c) 6 months following second stage excision; (d) 4 months following completion excision

Large Congenital Melanocytic Nevi

Management of large CMN is a more complex involved process that again strikes a balance between addressing the risk of malignancy with functional and aesthetic concerns of the lesion and its reconstruction.

At this time, large CMN are not able to be diagnosed prenatally. The appearance of dark, extensive, hairy lesions of the face, trunk, or extremities is by nature devastating for parents who have been anxiously awaiting the birth of their child. Because of this, the infant with large congenital melanocytic nevi should be referred early to a dermatologist and surgeon familiar with the management of these lesions to allow parents to be counseled about the nature of these lesions, risks of malignancy, and potential for excision and reconstructive options. If presented in a compassionate manner, even the news of a multiple-stage reconstruction over many years can be well accepted by families. In over 30 years of experience, the senior author has developed treatment plans for the management of these lesions with the tenet that aesthetic and functional outcome are as important as removal of the nevus itself.

An immediate concern of the family and doctors involved with the care of these patients is the potential risk of malignancy (Lim et al. 2019; Mosa et al. 2019). Historical review of the literature reports a range anywhere from 2% to 31% for the rate of melanoma occurring in patients with large CMN (Kopf et al. 1979; Marghoob et al. 1999; Quaba and Wallace 1986; Alper and Holmes 1983; Sandsmark et al. 1993; Bittencourt et al. 2000). This variance is explained in part by differences in study design and patients (with an increasing number undergoing surgical interventions) and can lead to confusion in how to counsel patient families. In a large systematic review of 14 studies observing over 6500 patients, Krengel and co-workers identified a 2.5% risk of melanoma in LCMN (>20 cm) and 3.1% risk in GCMN (>40 cm) (Krengel et al. 2006). This projected a lower than expected lifetime risk; however, this also was a culmination of many heterogeneic studies. However, current thinking is that the risk is lower than originally predicted, with risk of malignant transformation increasing with the increasing nevus size and distribution. The timing of melanoma diagnosis in patients with large CMN has also been studied with a trend toward development of malignancy early in life. It has been reported that 50% of large CMN that develop malignancy do so in the first 3 years of life, with 60% by childhood and 70% by puberty (Marghoob et al. 1996). Although the true incidence of malignant melanoma arising in untreated large CMN is unlikely to be clearly defined, these numbers encourage early removal.

History and Physical Examination

On initial history, any changes in the appearance of the nevus or satellite lesions should be elicited along with any family history of melanoma. Documentation of developmental milestones and presence of any neurologic symptoms should be noted. Serial examination of the lesion(s) should be undertaken every 3–6 months depending on the character and variability in the lesion’s appearance. Often patches of darker color or raised nodules develop within a large CMN that may represent neural nevus. This is a form of intradermal nevus with melanocytes that appear histologically similar to Schwann cells and contain nerve organelles such as Meissner’s and Pacinian corpuscles. The patches may also represent local areas of proliferation that do not necessarily behave in an aggressive manner. Certainly, biopsy of any suspicious raised, ulcerating, or atypical areas should be used to exclude malignancy (Fig. 5). Histologic findings of low mitotic rate, lack of necrosis, evidence of maturation in the cell population, and lack of high-grade nuclear atypia are clues to a benign course. The best description of these areas is melanocytic tumor of uncertain behavior, and these unusual areas are best addressed earlier in the course of reconstruction.
Fig. 5

Suspicious raised, ulcerating or atypical areas should be biopsied to identify or exclude malignancy. (Puri P, Hollwarth ME 2006 © Berlin, Heidelberg, New York: Springer Verlag 2006)

In the case of large nevi in an axial location especially of the posterior midline scalp or back or when numerous satellite lesions (>20) are present, MRI is recommended to document the presence of NCM. Asymptomatic patients can be identified specifically by T1 shortening on the MRI scan. For increased sensitivity, the MRI should be obtained prior to age 4 months, because as the patient grows increasing myelinization of the CNS can potentially obscure visualization of nevus cells (Barkovich et al. 1994).

It is important to convey that the finding of NCM on MRI does not necessarily imply the future development of neurologic symptoms. It does, however, indicate a risk for later development of benign or malignant melanotic tumors within the CNS. In the cases of symptomatic NCM, the associated poor prognosis should deter the surgeon from aggressive management of the cutaneous lesion. At this point, the low incidence of symptom development reported in asymptomatic patients with a positive screen for NCM would certainly caution against applying that same philosophy to this patient population. Further study will help to fully ascertain the true predicted course of disease in asymptomatic, scan-positive patients and help guide both surgical planning and the role of serial scanning. At this time, if the initial MRI is positive, we proceed with reconstruction as planned and feel that further scans are unnecessary unless neurologic symptoms develop.

Other Congenital Nevi

Blue Nevi

Blue nevi are smooth, bluish-black lesions that can be present at birth but more likely appear during childhood and puberty. They occur more frequently in females and are usually found on the head or extremities. Two variants exist: common and cellular. The common blue nevus is a relatively small (<1 cm), sharply demarcated, dome-shaped benign lesion (Fig. 6a). Histologically, it is comprised of intradermal and possibly subcutaneous dendritic melanocytes with normal overlying epithelium. The cellular blue nevus is larger (1–3 cm) with less regular borders (Fig. 6b) and is frequently found in the lumbosacral area. The lesion tends to be wider at the surface than the base and is comprised of spindle-shaped melanocytes in aggregates mixed within dendritic melanocytes. Unlike the common form, malignant transformation has been reported within the cellular variant, and therefore excision is recommended.
Fig. 6

(a) Common blue nevus; (b) Cellular blue nevus. (Puri P, Hollwarth ME 2006 © Berlin, Heidelberg, New York: Springer Verlag 2006)

Mongolian Spots

Mongolian spots are blue-gray macules usually overlying the lumbosacral area of otherwise healthy infants (Fig. 7). They are more common in Asian and darker-skinned individuals. Mongolian spots are most often present at birth but may appear within the 1st weeks of life and usually regress spontaneously by age 3–4 years of age. Lesions are made up of widely scattered dendritic melanocytes within the lower two thirds of the dermis (Carpo et al. 1999). No treatment of these benign lesions is necessary; however, laser can be effective for management of a persistent lesion.
Fig. 7

Mongolian spot of the lumbosacral region. (Puri P, Hollwarth ME 2006 © Berlin, Heidelberg, New York: Springer Verlag 2006)

Nevus of Ota/Nevus of Ito

The nevus of Ota is a blue-gray facial discoloration characterized by speckled or mottled coalescing macules appearing at birth or in childhood in the V1-V2 trigeminal nerve distribution (Fig. 8a). Unlike Mongolian spots, these lesions do not regress and can become hyperpigmented in puberty. The lesion may extend to involve the mucosal membranes of the nose and mouth as well as sclera, conjunctiva and retina, and ocular pathology and glaucoma have been associated. Nevus of Ota displays a female predominance and is found most commonly in Asian and Indian populations. In 10% of cases, the nevus is bilateral and associated with extensive Mongolian spots.
Fig. 8

(a) Nevus of Ota; (b) Nevus of Ito. (Puri P, Hollwarth ME 2006 © Berlin, Heidelberg, New York: Springer Verlag 2006)

The nevus of Ito is a blue-gray macular lesion similar to nevus of Ota that affects the shoulder (scapula, deltoid, supraclavicular) area and is sometimes associated with sensory changes (Fig. 8b). It is rarer than nevus of Ota, and more common in Asians.

Both these lesions represent field defects of dermal melanocytosis, histologically characterized by elongated dendritic melanocytes scattered within the collagen bundles mostly in the upper third of reticular dermis. They may contain raised areas within the lesion which are indistinguishable from blue nevus.

Although considered benign, there have been a few reported cases of malignant change, especially in areas similar to cellular blue nevus, and may require biopsy to distinguish from melanoma (Carpo et al. 1999). Historically, cryotherapy and nonselective destruction with CO2 laser was used with mixed esthetic results. Current laser technology allows the surgeon to take advantage of selective photothermolysis to direct laser energy to destroy the melanocytes without damaging the surrounding tissues with excellent cosmetic results. Multiple treatments with a Q-switched ruby laser, Q-switched alexandrite laser, or Q-switched Nd:YAG laser are effective to fade the lesion (Carpo et al. 1999). For Nevus of Ota, an ophthalmologist should be consulted due to the risk of ocular pathology.

Café au Lait Spots

Café au lait spots are benign sharply demarcated macules of light tan to brown regular pigmentation (Fig. 9). They can present in normal individuals or when multiple can be associated with syndromes such as neurofibromatosis. Histologically, lesions are caused by increased pigment in macromelanosomes within the keratinocytes in the basal layer. Laser ablation can be used to successfully treat lesions that are of cosmetic concern, but recurrence is common (Alster 1995). For larger lesions that do not respond to laser therapy and are in cosmetically sensitive areas, these can be treated much the same as large melanocytic nevi with either serial excision or tissue expansion.
Fig. 9

Café au Lait spot. (Puri P, Hollwarth ME 2006 © Berlin, Heidelberg, New York: Springer Verlag 2006)

Nevus Spilus

Nevus spilus are light tan to brown macules that resemble café au lait spots, but with areas of darker speckling within it (Fig. 10). The darker areas may not be apparent at birth and this is frequently a cause of misdiagnosis. On histology, there are both areas of increased pigment within the keratinocytes of the basal layer as well as an increased number of melanocytes. The specked areas represent a mixture of findings from freckling, to congenital melanocytic nevi, to blue nevi and can develop within the lesion from infancy through adolescence. Suspicious areas within the lesion should be excised for biopsy as the nevocellular areas do carry some malignant potential. If the entire lesion is in a cosmetically sensitive area, it can be removed surgically.
Fig. 10

Nevus Spilus

Epidermal Nevi

Epidermal nevi are hamartomas originating from ectodermal precursors. Histologically they include clusters of cells with either sebaceous (see next section), apocrine, eccrine, follicular, or keratinocytic components. Up to a third of these patients have involvement of other organ systems and the nevus represents part of a syndromic entity (Rogers et al. 1989). Linear sebaceous nevus, linear nevus comedonicus, and inflammatory linear verrucous epidermal nevus (ILVEN) are all represented in this category (Fig. 11). Symptomatic lesions may be inflammatory, erythematous, or pruritic especially in the case of ILVEN and indications to excise and reconstruct these nevi include issues related to discomfort and hygiene. Typically, these lesions do not penetrate to the depths of melanocytic nevi and there have been many reports of successful treatment with laser therapy (Boyce and Alster 2002; Paradela et al. 2007; Ratz et al. 1986).
Fig. 11

(a) ILVEN –Inflammatory linear verrucous epidermal nevus; (b) Linear sebaceous nevus

Extensive linear nevi are often seen in the head and neck and present some unique challenges to the reconstructive surgeon. For the larger lesions involving scalp and face, tissue expansion can be applied in a similar fashion which will be described for large CMN. Narrow linear lesions can be excised in stages, often timed so a partial excision is performed at the same time a tissue expander is placed elsewhere and further excision done when the expander is removed for distant flap reconstruction. It is not uncommon to see linear nevi involving the lower lip, chin, and adjacent neck. Given the linear orientation, it is important to design the reconstruction to minimize tension on the repair while breaking up the scar line to avoid scar hypertrophy and contracture. The senior author’s surgical approach has been described in detail in a previous publication (Margulis et al. 2003).

Sebaceous Nevi

Sebaceous nevi are hamartomas of sebaceous glands and represent a type of epidermal nevus present at birth as a waxy, hairless, yellow-orange plaque usually on the scalp, head, or neck (Fig. 12a). Over time, they can become more nodular, verrucous, and itchy most pronounced at puberty. Early reports showed a 15–20% lifetime risk of malignant transformation into basal cell carcinoma (Michalowski 1962; Mehregan and Pinkus 1965). However, more recent series report the number to be much lower (0.8–6.5%) (Cribier et al. 2000; Jones et al. 1970). This discrepancy is grounds for controversy, but their removal is still recommended.
Fig. 12

Typical sebaceous nevus of the scalp

Spitz Nevi

Although not usually congenital, Spitz nevi are another commonly encountered pediatric skin lesion. They present as pink, raised, firm lesions that on occasion can be darkly pigmented (Fig. 13). At times, they may be confused with pyogenic granulomas because of their appearance and rapid growth at onset. Originally termed benign juvenile melanoma, these lesions display a bizarre pathology beneath the microscope and can be confused with malignancy if the pathologist is not provided with the history of the lesion and patient’s age. In fact, these lesions are benign, but do grow rapidly and tend to recur aggressively if not completely excised. Because of that, excision with a generous border of normal tissue will decrease the chance of recurrence.
Fig. 13

Spitz nevus of the nose. (Puri P, Hollwarth ME 2006 © Berlin, Heidelberg, New York: Springer Verlag 2006)

Management of Small and Intermediate Lesions

As stated earlier, many small lesions can be removed at a time during adolescence when the patient can tolerate a local anesthesia. Most of these can be directly excised and the skin closed in layers. For some small lesions and intermediate lesions, excision should be approached in a staged fashion. Goals of serial excision should be complete excision of the lesion in two to three surgeries without distortion of surrounding features or restrictive scar bands. If either of the aforementioned is not possible, or is questionable, tissue expansion should be considered. By staging an excision, the scar will typically be shorter than a single stage excision just by nature of reducing the size of the dog-ears. In the author’s experience, a staged excision can be used to reorient a scar that may not be as feasible in a single stage. This is especially true for nevi of the face that are adjacent to the eyelids, nasal ala, or lips or moderate sized nevi of the trunk near the breast or the genitalia. Time between stages should be calculated based on expected scar maturation and not be performed during the proliferative phase of wound healing. This occurs around 3–4 months postexcision, but may be longer depending on the individual patient, location, wound complications, and scar management.

Management of Large and Giant Congenital Melanocytic Nevi and Other Large Lesions

Treatment for large and giant CMN remains controversial to some, as they feel the risk of malignant degeneration within these lesions is too low to warrant the extensive number of surgeries and potential scar burden associated with removal. Others feel the potential for noncutaneous melanoma to develop in extra-cutaneous nevus cells not amenable to surgical removal (such as in NCM) represents a remaining risk that negates the effectiveness of cutaneous excision for reduction of melanoma risk (Kadonaga and Frieden 1991). However, the significant deformity and associated psychological impact caused by these lesions often tip the scales toward intervention. Management plans should always strive to balance removal of the nevus with a functional and aesthetic reconstruction, and lesions that cannot be effectively addressed in this manner should be considered for conservative management using serial observation by an experienced dermatologist.

Treatment options are most often surgical with excision and reconstruction using adjacent or expanded skin or skin grafts/substitutes, but partial excision techniques can also be used.

Dermabrasion, Curettage, and Laser Therapy

Partial thickness excisions for CMN including dermabrasion, curettage, Versa Jet, and laser therapy have been reported as effective methods of reducing the cell burden to improve appearance of the nevus without completely excising it. The original theory behind these techniques relied on the belief that in infancy nevus cells lay in a superficial location within the upper reticular dermis and epidermis and gradually descended to deeper layers within the 1st weeks of life. However, this theory has not been substantiated (Marghoob 2013). Dermabrasion works to abrade away the surface cells, whereas curettage separates the cells at the natural cleavage plane between the superficial and deep dermis. These methods do nothing to address deeper nevus cells within the deeper dermis or subcutaneous tissues. Therefore, the number of nevus cells within a lesion is reduced but not eliminated. Initially, lesions may be lighter, but may darken with time as described in long term study (Magalon et al. 1998). In addition, any associated hypertrichosis is not affected, as hair follicles are found much deeper extending into the subcutaneous fat. Subsequent problems arise in these patients relating to pruritus and scarring secondary to the disrupted hair follicles with trapped and ingrown hair. Finally, malignant potential remains for the leftover nevus cells and scarring from these interventions may make follow up examination difficult. At least one case has been reported of melanoma arising within a large trunk nevus following dermabrasion (Dragieva et al. 2006). Due to these downsides, many practitioners have largely abandoned split thickness excision as a method of treatment.

Laser offers another potential treatment strategy attractive to both the patient and doctor as a simplified method to reduce the pigmentation of a lesion without scarring. Laser therapy is well suited for the treatment of lesions characterized by superficial dermal pigmentation such as the Nevi of Ota and Ito, persistent Mongolian spots, café au lait macules, and some elements of nevus spilus due to its minimal thickness, location of pigment within the dermis, and low potential for malignancy. Effective treatment relies on proper wavelength and pulse-width selection to allow penetration of the skin and photoselective destruction of melanin while preserving the remaining elements to avoid scarring (Carpo et al. 1999; Alster 1995). Serial treatments are required. Scarring can occur with aggressive treatment or improper setting selection. Hypo- and hyperpigmentation have also been reported which may be temporary or permanent. Exposure to sunlight in the perioperative period can cause significant burning, scarring, and hyperpigmentation.

Unfortunately, because CMN display nevus cells within all layers of the skin as well as within the deeper structures, it is unrealistic to think that a laser could effectively penetrate to the depths necessary to eliminate all pigment-producing cells without significant secondary damage and scarring. In addition, laser treatment vaporizes the specimen, therefore eliminating histologic evaluation of the lesion to determine its nature. Finally, the impact of the exposure of nevus cells to the radiant energy associated with laser therapy is unknown and may not be apparent for many years into the future.

Although dermabrasion, curettage, and laser offer a relatively simple approach to improving the appearance of CMN, they present many drawbacks. In addition, scarring from these therapies may complicate ultimate excisional treatment options in the future. Finally, the delay of more definitive reconstruction may have its own psychological effects on a child. Although limited use of these therapies may be useful in certain areas of reconstruction (such as lightening of a thin lesion in the cosmetic and functionally sensitive eyelid area), thoughtful consideration is warranted prior to widespread implementation of these modalities in a treatment algorithm for large CMN.

Surgical Excision and Reconstruction

The benefits of surgical excision of large and giant CMN include complete removal of involved skin and subcutaneous tissue. Although some have focused on skin grafts, skin substitutes, or cultured epithelium for reconstruction, when possible, use of tissue expansion allows the most functional and aesthetic result by allowing replacement with full thickness normal tissue. Goals of treatment include complete excision at an early age, minimization of scarring, and functional impact with a low requirement for future procedures. Early excision is emphasized for four reasons. First, the greatest risk for malignancy is reported in childhood, most notably in the first 3 years of life. Second, the elasticity and healing capacity of the skin is better the younger the patient. Third, patients operated on in infancy tolerate surgery better both physically and emotionally than their older counterparts. Finally, by completing reconstruction early, the psychological impact of the lesions and surgical interventions can be reduced.

In 1988, the senior author presented a coordinated approach to the management of these lesions in 78 patients (Bauer and Vicari 1988). This report outlined the spectrum of treatment options from skin graft to tissue expansion and assessed the effectiveness of excision and reconstruction with each technique in each body region. Since then, further experience with over 300 patients has allowed further development and refinement of approach (Bauer and Margulis 2004; Margulis et al. 2004, 2009; Unlu et al. 2002; Kryger and Bauer 2008; Alkureishi et al. 2018). Although a full discussion of the nuances of management of each of large CMN is beyond the scope of this chapter, the following is a summary of the author’s current approach.

Tissue Expansion

Several types of tissue expanders exist. The more commonly used include the round and rectangular types; crescent-shaped (and croissant) prostheses, originally developed to minimize dog-ears at the donor site, have fallen out of favor as it has been recognized that the added tissue gained with rectangular expanders may increase the choices possible for flap design (i.e., use of transposition flaps). This author prefers using rectangular expanders in almost all cases. Expander volumes have a wide range and vary according to the anatomic site. Rectangular expanders of 70–1000 cc are standard, but smaller and larger custom expanders are available. Saline is delivered via a remote valve port connected to the device by silicone tubing placed in a separate subcutaneous pocket. Remote, internal ports are preferred in the author’s practice to allow safer home expansion and avoid the risk of perforation by a misplaced needle accessing an integrated port. Risks of the remote port include migration of the port, flipping of the port, tube obstruction, kinking, or separation. To avoid these, meticulous attention is paid to the tubing length and course and ensuring a reliable port connection. As well, dissection of the port pocket is kept to a minimum and the port placed over firm supporting tissue. If needed, the port can be secured with suture. On the extremities larger ports are used to help avoid migration and turnover. The expander is typically positioned directly adjacent to or partially below the nevus itself. Donor skin needs to be free of infection, trauma, or previous scars (or have mature, stable scars) to prevent flap breakdown or extrusion. In the majority of cases, the expander can be placed through an incision within the boarder of the nevus.

Design of the flap is not a frivolous undertaking. Historically, expansion was used to generate advancement flaps only. Experience has demonstrated that expanded transposition and rotation flaps are frequently preferable, allowing greater versatility in flap design and more effective use of the expanded tissue. The capsule of the expanded flap also provides an improved blood supply to the tissue allowing for greater advancement and range of motion on a smaller pedicle than would be allowed in a nonexpanded flap. In applying this principle, it is important to avoid scoring of the capsule so that the added vascularity provided by the capsule is not compromised. In the same vein, when using back cuts to help transpose the tissue, it is important to maintain as much capsule on the free margin of advancing tissue as possible. This will improve the blood flow to this segment of the flap. Inset of the expanded flap is done in layered fashion ensuring that sutures capture a portion of the capsule. By preventing capsular recoil both the arterial inflow and the venous outflow of the flap are preserved.

Scalp

Scalp reconstruction is complicated by its relatively inflexible quality and the unique aesthetics of maintaining hair quality and direction. Because of this, tissue expansion remains the workhorse for scalp reconstruction during excision of large CMN. Scalp lesions are best reconstructed in stages, with placement of one or more tissue expanders in a subgaleal plane beneath the normal scalp skin. Flaps are designed with consideration of the major blood supply to the scalp (superficial temporal, postauricular, occipital, and contributions from the supraorbital vessels). Following adequate expansion (generally from 10 to 12 weeks), the expanders are removed, lesions excised, and the defect closed using both advancement and transposition flaps designed to preserve hair direction and hairline (Bauer and Margulis 2004). Flaps should be carried out and checked for viability before excision of the nevus. The senior author has found that by planning transposition flaps, the number of expansions needed is reduced. Serial expansion of the scalp typically leads to scarring of the galea to the periosteum. If this occurs, expander placement below the periosteum can be performed in the subsequent stages. In these cases it is often difficult to provide significant prefill of the expander at the time of surgery. Therefore, the expansion process may take slightly longer than the first or second round of expansion. Defects in the temporal hairline are often best treated with a large transposition flap from the occipital scalp. This provides favorable hair direction for both the scars and the hair along the sideburn.

Expansion of the infant scalp can safely begin at 6 months of age. Careful attention should be given to the fontanel during expander placement and reconstruction (Fig. 14). Although temporary cranial molding is common at this age, this does not persist and spontaneously corrects within 1–4 months following expander removal. In older children and adults, the capsule can form a “wash-tub” deformity at its margins along the cranium. At the time of reconstruction, the free edge of this can be safely excised as the flap is inset. The posterior edge which is attached to the flap capsule and carries the pedicle should be left intact. This ridge resolves once the expander has been removed.
Fig. 14

(a) Scalp expander in a 6 month old patient; (b) CT scan showing lack of significant cranial molding. (Puri P, Hollwarth ME 2006 © Berlin, Heidelberg, New York: Springer Verlag 2006)

Forehead

Large CMN of up to 75% of the forehead can be managed with serial tissue expansion (Fig. 15ad). This requires careful planning to minimize distortion of the eyebrow position and maintain a normal distance from brow to hairline. By using the expanded tissue overlying the brow, a long back-cut can be made along what would be the superior margin of the dog-ear. This allows a generous segment of expanded tissue to be transposed to the fronto-temporal region of the forehead and the temple. Nevi extending into the temporal area must be treated by expansion of both scalp and forehead to reconstruct the temporal hairline (Unlu et al. 2002). Bilateral expansion of normal forehead is often successful for midforehead lesions. In cases of brow elevation, the abnormal brow can be returned to its preoperative position by interposing nonhair-bearing forehead skin. Most importantly, don’t overextend the flap, re-expand it.
Fig. 15

(a) Preoperative photo of a patient with a giant CMN of the scalp, forehead, brow, and cheek. (b) Patient following first round of expansion and reconstruction. Expanded flaps from the scalp, forehead, and neck were used. (c, d) The same patient following second round of expansion and reconstruction. Scars fall within the borders of aesthetic subunits

Face and Neck

Large CMN of the face present some of the greatest reconstructive challenges. By dividing the face into aesthetic units, tissue expansion of available adjacent areas of normal skin in the neck and forehead can provide skin of excellent quality match while allowing scars to lie at the junction of aesthetic units and render them less obvious. When advancing tissue from the neck into the cheek area, a transposition design can help improve flap movement while aligning the scars optimally. Whether transposed with the flap based laterally or medially, the transposition minimizes the risk of downward drift of the flap and ectropion of the lower eyelid (Bauer and Margulis 2004). Lesions of the nose are best resurfaced by an expanded forehead flap carried on a supratrochlear, superior orbital, or superficial temporal artery pedicle. In children, the typical wider scar left in adult paramedian donor sites can be quite disfiguring. This can be minimized by pre-expanding the flap. If forehead is not available, an expanded supraclavicular full thickness skin graft provides a suitable option. The periorbital area is also best addressed with expanded full-thickness skin grafts to allow preservation of the thin nature of the tissue while decreasing incidence of ectropion (Margulis et al. 2009). For large facial nevi involving the eyelids, the reconstruction of the lids is the final phase after the rest of the nevus has been excised and the surrounding subunits reconstructed. This allows placement of grafts along stable scar margins. Any ectropion or lid distortion that has been caused by the nevus or prior reconstruction can be addressed at this point as well with either medial or lateral canthoplasty.

Ear

Lesions on the ear are addressed with excision and full thickness skin grafting (Fig. 16ac) For lesions involving the entire ear, the senior author has developed a staged approach (Adler et al. 2009a). The subunits of the ear can be identified as stable or unstable. Stable subunits are those whose cartilage is relatively firm and inflexible. These can be excised and grafted during infancy. The concha and scaphal subunits fall into his category. Full thickness grafts are taken from the contralateral post-auricular skin. More flexible parts of the ear, such as the helical rim, are at greater risk of distortion by grafting. These are addressed at 6–7 years of age, when the ear has achieved nearly adult size and the cartilage is more robust. The postauricular surface on the effected ear can be replaced with full thickness skin from the groin at any age. The ear lobe presents a unique challenge due to its thin, soft, and pliable nature. When possible, the earlobe is reconstructed with normal skin from the neck. In the case of giant CMN which covers the entire scalp and neck adjacent to the ear, these can be reconstructed with an expanded supraclavicular artery flap. If this is undertaken, the lobe is created at the same time or subsequent to the flap transposition.
Fig. 16

(a) Preoperative photo of a large CMN of the ear and peri-auricular tissue. (b) Patient following reconstruction with expanded neck flap for the peri-auricular tissue and earlobe. Concha was reconstructed with full thickness skin graft from the contralateral ear. (c) Third stage, patient underwent full thickness skin graft of the scapha. She will return for the final stage of skin grafting of the helix in 1–2 years

Trunk

The often extensive involvement of giant CMN of the trunk with relative lack of normal donor skin presents a daunting challenge to the reconstructive surgeon, leading many to resort to split thickness skin graft based reconstruction (Fig. 17a, b). The inferior functional and aesthetic results achieved call into question whether excision should have been undertaken at all in favor of conservative observation. With better expanded flap design carried out in series, or use of expanded distant flaps with microvascular transfer, superior trunk reconstruction can be achieved.
Fig. 17

(a) Split thickness skin graft used for reconstruction of the back when lack of suitable donor sites are available for expansion. (b) Boarder areas can then be expanded to compete the reconstruction

The posterior trunk is the most common location of giant CMN often extending anteriorly in a dermatome distribution. These lesions are best reconstructed with serial tissue expansion and subsequent transposition flap closure (Fig. 18). By utilizing flap transposition over simple advancement, excision and reconstruction of bathing trunk and large thorax nevi previously felt to be treatable only with skin grafting has become possible (Bauer and Margulis 2004). Anterior trunk lesions can be treated with an abdominoplasty technique with or without expansion depending on lesion size. When adjacent donor sites are unavailable for expansion, excision and reconstruction of shoulder, upper back, and posterior neck nevi can be accomplished using microvascular transfer of a free expanded transverse rectus abdominus myocutaneous (TRAM) flap. (Fig. 19ac). One must keep in mind the gender of the patient during reconstruction of the trunk. When approaching a female with a large or giant CMN of the trunk, every effort is made to avoid the nipple areola and the future position of the breast when placing expanders and transposing tissue. For males, the nipple and areola can be moved with the flaps with the anticipation that free nipple grafting will be performed following the reconstruction of the trunk. With increased use of expanded flaps over skin grafting for trunk reconstruction, late contour deformities seen at junction points between grafted and ungrafted areas can be significantly reduced. These modified techniques have resulted in aesthetic benefits far beyond what could be accomplished with alternative treatments.
Fig. 18

(a, b) Preoperative photos of a giant CMN of the trunk. (c, d) The patient at the completion of expansion prior to excision of the nevus. (e, f) After first stage reconstruction. (g, h) Following second round of expander reconstruction

Fig. 19

(a) Large CMN of posterior neck and upper back. (b) Reconstruction with expanded free TRAM flap. (c) Final result following re-expansion of the TRAM and Z-plasty. (Puri P, Hollwarth ME 2006 © Berlin, Heidelberg, New York: Springer Verlag 2006)

Extremities

Large CMN of the extremities continue to present a considerable challenge due to the limitations of local expansion techniques in these areas and the relatively poor aesthetic outcome experienced with skin grafting. The author’s initial approach utilized both split-thickness and expanded full-thickness skin grafts for most lesions, but the long-term soft-tissue contour defect and pigment abnormalities in the grafted skin have led to the use of alternative approaches (Margulis et al. 2004). In upper extremity lesions, use of transposition flaps from the upper back and shoulder has effectively eliminated contour defects to the proximal upper extremity. In addition, three-stage, expanded pedicled flaps from the abdomen and flank can be designed to provide complete coverage of a circumferential nevus from elbow to wrist with excellent contour and acceptable scarring achieved at both donor and recipient sites (Margulis et al. 2004) (Fig. 20af). A similar pedicled approach has been successful in providing coverage for lower leg lesions (Fig. 21af). An ipsilateral expanded thigh flap in the young infant can be transposed to the lower leg when lower extremity flexibility is at its peak and provide excellent results (Kryger and Bauer 2008).
Fig. 20

(a) Large CMN of arm and elbow. (b, c) Pedicled expanded abdominal flap used for reconstruction. (d) Postoperative photo following division of pedicle. (e, f) Final result following secondary advancement of flap and removal of remaining nevus

Fig. 21

(a) Large CMN of lower leg. (b–d) Pedicled expanded posterior thigh flap used for reconstruction. (e, f) Final result at 3 years. (Puri P, Hollwarth ME 2006 © Berlin, Heidelberg, New York: Springer Verlag 2006)

With experience, expansion of the extremity skin is feasible. One must assess the lesion and understand the limitations in the basic geometry of the limb (flap movement is easier around the circumference than in an axial direction). Careful planning, choice of expander size and placement, and a slower expansion schedule leads to lower risk of extrusion and flap failure. Placement of expanders is easier above the knee than below. When possible, more than one expander should be used. For large extremity lesions, using expansion as an adjunct to reduce the overall size of the defect to then be covered by a free flap is another useful approach.

Expanded free flaps offer another alternative for larger lesions of the extremities with improved functional and aesthetic outcomes. These prove especially useful for coverage of the knee, lower leg, and ankle. Pre-expansion of the flap or the tissue adjacent to the donor site reduces the donor site morbidity in pediatric patients, often times avoiding the need for a skin graft.

For the dorsum of the hands and feet, the use of expanded full thickness skin grafts is the best option, where subcutaneous tissue is often minimal and a full thickness graft provides a suitable replacement. For the fingers and toes, serial excision has been found to reduce the bulky and often verrucous nature of the nevus in these areas. Although the pigmented skin remains, there is often a compensatory lightening of the tissues following partial excision (Fig. 22ac).
Fig. 22

(a) Large CMN of hand and fingers. (b) Result following full thickness skin graft to the dorsum of the hand. (c) Final result following first stage serial excision of fingers

Complications

Complications of surgical excision of large CMN with expanded flap reconstruction are uncommon despite the complex nature of these procedures. During the expansion process, the primary risks include expander infection, expander exposure or extrusion, flap compromise, and incisional dehiscence. The incidence of expander infection has been reported at 5%, which can be due to inoculation during expander insertion or repeated expansions, after exposure of the expander or by hematogenous seeding from a distant infection (Adler et al. 2009b). These infections can usually be managed conservatively by antibiotic administration allowing completion of expansion and successful reconstruction. A low threshold for placing the patient on oral antibiotics during healing problems or illness occurring during expansion may help minimize risk. It is also vital to maintain a close communication with the patient caregivers to ensure there is a free flow of information and prompt reporting of any signs of the aforementioned complications. Well-trained ancillary staff, nursing, and on-call personnel can have a dramatic influence on the success of tissue expansion.

Technical considerations for the use of expanded flap reconstruction rely on the understanding of the unique characteristics provided by expansion. Vascularity of a flap through the delay phenomenon makes flap ischemia rare. Preservation of the capsule with judicious capsulotomy and avoidance of excessive tension during closure can prevent this potentially devastating complication. Venous congestion and wound dehiscence can be improved by ensuring the capsule is advanced along with the flap and secured using deeper sutures thereby preventing its recoil during the final closure. Finally, meticulous closure and postoperative scar management help achieve optimal results. It is important to discuss scarring with patient families. While scars can be quite extensive for large and giant CMN, if they run in favorable directions to avoid tension, even long or wide scars can fade very nicely.

Conclusion and Future Directions

The treatment of congenital large and giant nevi presents a continuing challenge to all individuals involved with these patients. The ability to present an organized discussion of current views on risk of malignant change to parents, patients, referring physicians, and other allied health care workers is critical. Treatment strategies should take into consideration the varied opinions regarding malignant risk, emphasize the benefits of early excision on lowering that risk, and most importantly, provide a means of dealing with these often devastating lesions in a manner that optimizes the functional and aesthetic outcome and minimizes the need for major reconstruction in later life.

Cross-References

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Copyright information

© Springer-Verlag Berlin Heidelberg (outside the USA) 2020

Authors and Affiliations

  1. 1.Clinical Associate of Surgery, Section of Plastic and Reconstructive SurgeryUniversity of Chicago, Pritzker School of MedicineChicagoUSA
  2. 2.Clinical Associate, University of Chicago Hospitals, Director of Plastic and Reconstructive SurgeryIllinois Dermatology InstituteSkokieUSA

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