Congenital Nevi

Bruce S. Bauer and Sara R. Dickie

ContentsIntroduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2

Congenital Melanocytic Nevi . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2Etiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4Pathology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5Presentation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5Small and Intermediate Congenital Melanocytic Nevi . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5Large Congenital Melanocytic Nevi . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7History and Physical Examination . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8

Other Congenital Nevi . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8Blue Nevi . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8Mongolian Spots . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9Nevus of Ota/Nevus of Ito . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9Café au Lait Spots . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11Nevus Spilus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11Epidermal Nevi . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11Sebaceous Nevi . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12Spitz Nevi . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13

Management of Small and Intermediate Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13

Management of Large and Giant Congenital Melanocytic Nevi and OtherLarge Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 14

Dermabrasion, Curettage, and Laser Therapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 14

Surgical Excision and Reconstruction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15

B. S. Bauer (*) · S. R. DickieClinical Associate of Surgery, Section of Plastic andReconstructive Surgery, University of Chicago, PritzkerSchool of Medicine, Chicago, IL, USA

Clinical Associate, University of Chicago Hospitals,Director of Plastic and Reconstructive Surgery, IllinoisDermatology Institute, Skokie, IL, USAe-mail: bbauer@surgery.bsd.uchicago.edu;sdickie@illinoisderm.com

© Springer-Verlag Berlin Heidelberg (outside the USA) 2020P. Puri (ed.), Pediatric Surgery,https://doi.org/10.1007/978-3-642-38482-0_162-1

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Tissue Expansion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 23

Conclusion and Future Directions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 25

Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 25

References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 25

AbstractThis chapter provides a review of differenttypes of congenital lesions of the skin includ-ing their natural history, diagnosis, classifica-tion, and treatment options. Most lesions canbe approached surgically through a variety oftechniques. This chapter aims to provide afamiliarity with the diagnosis and help thereader develop a management strategy whichsatisfies both a functional and aesthetic out-come for these patients.

KeywordsCongenital nevi · Blue nevi · Café au laitspots · Malignancy

Introduction

Pigmented lesions are a common diagnosisencountered by the pediatric surgeon. Congenitalnevi represent a group of skin lesions which occurat birth or within the first several years of life andare characterized by ectopic nests of dermal ele-ments. Although most commonly melanocytic innature (such as the congenital melanocytic nevus,Nevi of Ota, Nevi of Ito, nevus spilus, café au laitspots and Mongolian spots), nevi can also origi-nate from epidermal (Nevus sebaceous ofJadassohn) and neural elements. Lesion character-istics vary according to the type of cell involved,location within the skin, and level of cell differ-entiation. Knowledge of the differential diagnosisand natural history of these lesions can help bal-ance the plan of care, so as to address the potentialrisk of malignant degeneration while accountingfor functional and aesthetic concerns encounteredduring excision and reconstruction.

Congenital Melanocytic Nevi

Congenital melanocytic nevi (CMN) are the mostcommon congenital nevi. They are composed ofmelanin producing cells originating from the neu-ral crest which carry a variable amount of pig-ment. Historically, these nevi have been classifiedbased on their expected size at adulthood(Fig. 1a–d); 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 congenitalnevi includes lesions>2% total body surface area(Quaba and Wallace 1986). These classificationgroups are important not only when determiningtreatment options, but when stratifying the poten-tial risk of malignant change. Recently, a morestandardized classification has been introducedand has been adopted by the greater nevus com-munity (Krengel et al. 2013). This consensus-based schema categorizes nevi by their cutaneousfeatures including size, number of satellites, ana-tomic location, color heterogeneity, surface rugos-ity, hypertrichosis, and dermal nodularity.Prospective analysis using this system will hope-fully allow practitioners to risk stratify individualsfor the presence of neurocutaneous melanosis(NCM) and validate observed melanoma risk.

When evaluating a lesion in a baby, body sur-face area changes between infancy and adulthoodcan be used to predict if a lesion falls into the largecategory. As a general rule, a 12 cm lesion on thehead or neck or 7 cm lesion on the body of aninfant will grow to meet the large nevi classifica-tion (Marghoob et al. 1996). Incidence of CMNdecreases with increasing size of the lesion withthe relatively common small lesions occurring in1:100 births, intermediate lesions in 1:1000, largelesions in 1:20,000, and giant lesions in 1:500,000

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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

Congenital Nevi 3

births (Quaba and Wallace 1986; Alper andHolmes 1983; Illig et al. 1985).

Etiology

Embryologically, melanocytes originate fromneural crest cells as melanoblasts. During earlygestation, these melanoblasts migrate to the basallayer of the epidermis at which time they differ-entiate into dendritic melanocytes and associateinto melanosomes that produce pigment for trans-fer to keratinocytes. Congenital melanocytic neviresult from a disturbance in this migration anddifferentiation process resulting in ectopic nestsof immature cells along their course of migration.Because of this, nevus cells often extend deep intothe subcutaneous tissues and can even involvefascia, muscle, periosteum, or the leptomeninges.Congenital melanocytic nevi (CMN) overwhelm-ingly occur in a sporadic pattern with rare reportsof familial cases. There have been several studiesseeking to identify genetic hits underlying CMNpathogenesis, and the results of these studies sug-gest NRAS mutations and, to a lesser extentBRAF mutations, contribute to the development

of CMN (Roh et al. 2015). BRAF or NRAS muta-tions are typically found in small CMN, but theprevalence of NRAS mutations is greater amongmedium to giant CMN (Charbel et al. 2014).

When nevus cells are found within the centralnervous system (leptomeninges, brain, or spinalcolumn), the condition is called neurocutaneousmelanosis (NCM) (Fig. 2a). The clinical presen-tation of NCM can range from asymptomatic toprogressive, severe neurologic deterioration withdevelopmental delay, hydrocephalus, seizures,and death. Large CMN of the posterior midlineand those found in association with greater than20 satellite nevi have the highest risk of NCM andshould be considered for MRI screening(Marghoob et al. 2004) (Fig. 2b).

Although it is difficult to know the true inci-dence of NCM in association with large CMNbecause screening of asymptomatic patients isnot universally performed, a study of an internetregistry found NCM onMRI in approximately 5%of cases of large or giant CMN of which only5–6% became symptomatic (Agero et al. 2005).Bett reported an incidence of 7.5% in a largecohort of at-risk nevus patients with a mortalityrate of 34% in symptomatic patients (Bett 2006).

Fig. 2 (a) AT-1 weighted magnetic resonance image of the brain demonstrates several lesions typical of NCM. (b) LargeCMN of the posterior midline and those with greater than 20 satellite nevi have the highest risk of NCM

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Symptoms are produced by either malignantdegeneration or the benign proliferation of nevuscells within the central nervous system which canblock the circulation of cerebral spinal fluid lead-ing to hydrocephalus and increased intracranialpressure. If NCM is to become symptomatic, thevast majority of cases will do so before age 5 years(Bett 2006).

Pathology

Nevus cells differ from melanocytes histologi-cally as they group together in clusters ratherthan arrange as individual melanosomes. In addi-tion, nevus cells assume a rounded rather thandendritic shape and keep their pigment withintheir cytoplasm instead of transferring it to thesurrounding keratinocytes (Elder and Elenitsas1997).

A higher risk of malignant transformation hasbeen associated with CMN compared to acquirednevi. For this reason, efforts have been made toidentify characteristics specific to CMN. Clini-cally, examination of small CMN with adermatoscope or under loupe magnification willreveal small pigment granules at the peripheralaspect of the lesion which is a specific finding toCMN (Alper et al. 1979) (Fig. 3). Histologicalcharacteristics of CMN show nevus cells withineccrine ducts, follicular epithelium, and/or blood

vessels. However, not all CMN display these find-ings (Rhodes et al. 1986). Only congenital lesionsdemonstrate nevus cells within the deeper subcu-taneous tissues, fascia, nerve sheath, and muscle.

Presentation

CMN can differ greatly in their size and appear-ance. Often obvious at birth, some lesions can bequite faint and seem to appear with time over the1st year of life as they increase in pigmentation.Coloration varies from pale tan to a deep bluishblack and may be uniform or extremely varie-gated. The lesions are often thickened in texturewith increased skin markings compared to sur-rounding normal skin. They often have hairwhich can range from fine light fuzz to coarsethick follicles. When large lesions are present,they may be accompanied by smaller peripheralsatellite lesions of different sizes and numberswhich can appear over the course of a lifetime,but predominantly before age three.

Over time, the surface of CMN may change tobecome verrucous and irregular with darkening ofpigmentation. Lightening of color can also beseen in as many as a third of patients with largeand giant nevi. Erosion or breakdown is notuncommon in the neonatal period, but may alsooccur later. This does not necessarily indicatemalignant change but warrants furtherinvestigation.

Management

The management strategy of CMN can be strati-fied based on size of the lesion and potential riskof malignant change.

Small and Intermediate CongenitalMelanocytic Nevi

Most CMN fall into the small category and canusually be addressed with simple or staged exci-sion (Fig. 4). A large systematic evaluation ofsmall CMN and melanoma risk has yet to be

Fig. 3 Small pigment granules associated with the nevusare specific to CMN. (Image courtesy of Joel Joyce, MD,Dermatology)

Congenital Nevi 5

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

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published, so the actual risk of malignant changein these is unknown and controversial. In a largepopulation-based study of nearly 4000 individualswith CMN, none developed melanoma over10 years (Berg and Lindelof 2003). When basedon patient reported history of a congenital lesion,the lifetime risk of melanoma occurring in smallCMN has been quoted at 4.9% but lowers to 2.6%for lesions determined to be CMN on histologiccriteria (Rhodes and Melski 1982). The risk ofmelanoma arising within small CMN beforepuberty is extremely unlikely (Scalzo et al.1997). For this reason, most defer the removal ofthese lesions until the patient reaches an age atwhich comfortable excision can be performedunder local anesthesia to avoid the associatedrisks of general anesthesia. However, if the lesionfalls in a cosmetically sensitive area or is locatedin an area that will require general anesthesiaregardless of age, consideration should be givento earlier removal to avoid potential psychologicsequella of delaying treatment. From a practicalpoint of view, these procedures are best performedeither before the patient begins toddling or justprior to school entrance in order to avoid potentialcomplications from falls, heightened anxiety, andlack of patient cooperation found at the ages inbetween.

Large Congenital Melanocytic Nevi

Management of large CMN is a more complexinvolved process that again strikes a balancebetween addressing the risk of malignancy withfunctional and aesthetic concerns of the lesion andits reconstruction.

At this time, large CMN are not able to bediagnosed prenatally. The appearance of dark,extensive, hairy lesions of the face, trunk, orextremities is by nature devastating for parentswho have been anxiously awaiting the birth oftheir child. Because of this, the infant with largecongenital melanocytic nevi should be referredearly to a dermatologist and surgeon familiarwith the management of these lesions to allowparents to be counseled about the nature of theselesions, risks of malignancy, and potential for

excision and reconstructive options. If presentedin a compassionate manner, even the news of amultiple-stage reconstruction over many yearscan be well accepted by families. In over30 years of experience, the senior author hasdeveloped treatment plans for the managementof these lesions with the tenet that aesthetic andfunctional outcome are as important as removal ofthe nevus itself.

An immediate concern of the family and doc-tors involved with the care of these patients is thepotential risk of malignancy (Lim et al. 2019;Mosa et al. 2019). Historical review of the litera-ture reports a range anywhere from 2% to 31% forthe rate of melanoma occurring in patients withlarge CMN (Kopf et al. 1979; Marghoob et al.1999; Quaba and Wallace 1986; Alper andHolmes 1983; Sandsmark et al. 1993; Bittencourtet al. 2000). This variance is explained in part bydifferences in study design and patients (with anincreasing number undergoing surgical interven-tions) and can lead to confusion in how to counselpatient families. In a large systematic review of14 studies observing over 6500 patients, Krengeland co-workers identified a 2.5% risk of mela-noma in LCMN (>20 cm) and 3.1% risk inGCMN (>40 cm) (Krengel et al. 2006). Thisprojected a lower than expected lifetime risk;however, this also was a culmination of manyheterogeneic studies. However, current thinkingis that the risk is lower than originally predicted,with risk of malignant transformation increasingwith the increasing nevus size and distribution.The timing of melanoma diagnosis in patientswith large CMN has also been studied with atrend toward development of malignancy earlyin life. It has been reported that 50% of largeCMN that develop malignancy do so in the first3 years of life, with 60% by childhood and 70% bypuberty (Marghoob et al. 1996). Although the trueincidence of malignant melanoma arising inuntreated large CMN is unlikely to be clearlydefined, these numbers encourage early removal.

Congenital Nevi 7

History and Physical Examination

On initial history, any changes in the appearanceof the nevus or satellite lesions should be elicitedalong with any family history of melanoma. Doc-umentation of developmental milestones andpresence of any neurologic symptoms should benoted. Serial examination of the lesion(s) shouldbe undertaken every 3–6months depending on thecharacter and variability in the lesion’s appear-ance. Often patches of darker color or raised nod-ules develop within a large CMN that mayrepresent neural nevus. This is a form of intrader-mal nevus with melanocytes that appear histolog-ically similar to Schwann cells and contain nerveorganelles such as Meissner’s and Pacinian cor-puscles. The patches may also represent localareas of proliferation that do not necessarilybehave in an aggressive manner. Certainly, biopsyof any suspicious raised, ulcerating, or atypicalareas should be used to exclude malignancy(Fig. 5). Histologic findings of low mitotic rate,lack of necrosis, evidence of maturation in the cellpopulation, and lack of high-grade nuclear atypiaare clues to a benign course. The best descriptionof these areas is melanocytic tumor of uncertainbehavior, and these unusual areas are bestaddressed earlier in the course of reconstruction.

In the case of large nevi in an axial locationespecially of the posterior midline scalp or back or

when numerous satellite lesions (>20) are pre-sent, MRI is recommended to document the pres-ence of NCM. Asymptomatic patients can beidentified specifically by T1 shortening on theMRI scan. For increased sensitivity, the MRIshould be obtained prior to age 4 months, becauseas the patient grows increasing myelinization ofthe CNS can potentially obscure visualization ofnevus cells (Barkovich et al. 1994).

It is important to convey that the finding ofNCM on MRI does not necessarily imply thefuture development of neurologic symptoms. Itdoes, however, indicate a risk for later develop-ment of benign or malignant melanotic tumorswithin the CNS. In the cases of symptomaticNCM, the associated poor prognosis shoulddeter the surgeon from aggressive managementof the cutaneous lesion. At this point, the lowincidence of symptom development reported inasymptomatic patients with a positive screen forNCM would certainly caution against applyingthat same philosophy to this patient population.Further study will help to fully ascertain the truepredicted course of disease in asymptomatic,scan-positive patients and help guide both surgi-cal planning and the role of serial scanning. At thistime, if the initial MRI is positive, we proceedwith reconstruction as planned and feel that fur-ther scans are unnecessary unless neurologicsymptoms develop.

Other Congenital Nevi

Blue Nevi

Blue nevi are smooth, bluish-black lesions thatcan be present at birth but more likely appearduring childhood and puberty. They occur morefrequently in females and are usually found on thehead or extremities. Two variants exist: commonand cellular. The common blue nevus is a rela-tively small (<1 cm), sharply demarcated, dome-shaped benign lesion (Fig. 6a). Histologically, it iscomprised of intradermal and possibly subcutane-ous dendritic melanocytes with normal overlyingepithelium. The cellular blue nevus is larger(1–3 cm) with less regular borders (Fig. 6b) and

Fig. 5 Suspicious raised, ulcerating or atypical areasshould be biopsied to identify or exclude malignancy.(Puri P, Hollwarth ME 2006 © Berlin, Heidelberg,New York: Springer Verlag 2006)

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is frequently found in the lumbosacral area. Thelesion tends to be wider at the surface than thebase and is comprised of spindle-shaped melano-cytes in aggregates mixed within dendritic mela-nocytes. Unlike the common form, malignant

transformation has been reported within the cel-lular variant, and therefore excision isrecommended.

Mongolian Spots

Mongolian spots are blue-gray macules usuallyoverlying the lumbosacral area of otherwisehealthy infants (Fig. 7). They are more commonin Asian and darker-skinned individuals. Mongo-lian spots are most often present at birth but mayappear within the 1st weeks of life and usuallyregress spontaneously by age 3–4 years of age.Lesions are made up of widely scattered dendriticmelanocytes within the lower two thirds of thedermis (Carpo et al. 1999). No treatment of thesebenign lesions is necessary; however, laser can beeffective for management of a persistent lesion.

Nevus of Ota/Nevus of Ito

The nevus of Ota is a blue-gray facial discolor-ation characterized by speckled or mottled

Fig. 6 (a) Common blue nevus; (b) Cellular blue nevus. (Puri P, Hollwarth ME 2006 © Berlin, Heidelberg, New York:Springer Verlag 2006)

Fig. 7 Mongolian spot of the lumbosacral region. (Puri P,Hollwarth ME 2006 © Berlin, Heidelberg, New York:Springer Verlag 2006)

Congenital Nevi 9

coalescing macules appearing at birth or in child-hood in the V1-V2 trigeminal nerve distribution(Fig. 8a). Unlike Mongolian spots, these lesionsdo not regress and can become hyperpigmented inpuberty. The lesion may extend to involve themucosal membranes of the nose and mouth aswell as sclera, conjunctiva and retina, and ocularpathology and glaucoma have been associated.Nevus of Ota displays a female predominanceand is found most commonly in Asian andIndian populations. In 10% of cases, the nevus isbilateral and associated with extensive Mongolianspots.

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

Both these lesions represent field defects ofdermal melanocytosis, histologically character-ized by elongated dendritic melanocytes scattered

within the collagen bundles mostly in the upperthird of reticular dermis. They may contain raisedareas within the lesion which are indistinguish-able from blue nevus.

Although considered benign, there have been afew reported cases of malignant change, espe-cially in areas similar to cellular blue nevus, andmay require biopsy to distinguish from melanoma(Carpo et al. 1999). Historically, cryotherapy andnonselective destruction with CO2 laser was usedwith mixed esthetic results. Current laser technol-ogy allows the surgeon to take advantage of selec-tive photothermolysis to direct laser energy todestroy the melanocytes without damaging thesurrounding tissues with excellent cosmeticresults. Multiple treatments with a Q-switchedruby laser, Q-switched alexandrite laser, orQ-switched Nd:YAG laser are effective to fadethe lesion (Carpo et al. 1999). For Nevus of Ota,an ophthalmologist should be consulted due to therisk of ocular pathology.

Fig. 8 (a) Nevus of Ota; (b) Nevus of Ito. (Puri P, Hollwarth ME 2006 © Berlin, Heidelberg, New York: Springer Verlag2006)

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Café au Lait Spots

Café au lait spots are benign sharply demarcatedmacules of light tan to brown regular pigmenta-tion (Fig. 9). They can present in normal individ-uals or when multiple can be associated withsyndromes such as neurofibromatosis. Histologi-cally, lesions are caused by increased pigment inmacromelanosomes within the keratinocytes inthe basal layer. Laser ablation can be used tosuccessfully treat lesions that are of cosmetic con-cern, but recurrence is common (Alster 1995). Forlarger lesions that do not respond to laser therapyand are in cosmetically sensitive areas, these canbe treated much the same as large melanocyticnevi with either serial excision or tissueexpansion.

Nevus Spilus

Nevus spilus are light tan to brown macules thatresemble café au lait spots, but with areas of

darker speckling within it (Fig. 10). The darkerareas may not be apparent at birth and this isfrequently a cause of misdiagnosis. On histology,there are both areas of increased pigment withinthe keratinocytes of the basal layer as well as anincreased number of melanocytes. The speckedareas represent a mixture of findings from freck-ling, to congenital melanocytic nevi, to blue neviand can develop within the lesion from infancythrough adolescence. Suspicious areas within thelesion should be excised for biopsy as thenevocellular areas do carry somemalignant poten-tial. If the entire lesion is in a cosmetically sensi-tive area, it can be removed surgically.

Epidermal Nevi

Epidermal nevi are hamartomas originating fromectodermal precursors. Histologically theyinclude clusters of cells with either sebaceous

Fig. 9 Café au Lait spot. (Puri P, Hollwarth ME 2006 ©Berlin, Heidelberg, New York: Springer Verlag 2006)

Fig. 10 Nevus Spilus

Congenital Nevi 11

(see next section), apocrine, eccrine, follicular, orkeratinocytic components. Up to a third of thesepatients have involvement of other organ systemsand the nevus represents part of a syndromicentity (Rogers et al. 1989). Linear sebaceousnevus, linear nevus comedonicus, and inflamma-tory linear verrucous epidermal nevus (ILVEN)are all represented in this category (Fig. 11).Symptomatic lesions may be inflammatory, ery-thematous, or pruritic especially in the case ofILVEN and indications to excise and reconstructthese nevi include issues related to discomfort andhygiene. Typically, these lesions do not penetrateto the depths of melanocytic nevi and there havebeen many reports of successful treatment withlaser therapy (Boyce and Alster 2002; Paradelaet al. 2007; Ratz et al. 1986).

Extensive linear nevi are often seen in the headand neck and present some unique challenges tothe reconstructive surgeon. For the larger lesionsinvolving scalp and face, tissue expansion can beapplied in a similar fashion which will be

described for large CMN. Narrow linear lesionscan be excised in stages, often timed so a partialexcision is performed at the same time a tissueexpander is placed elsewhere and further excisiondone when the expander is removed for distantflap reconstruction. It is not uncommon to seelinear nevi involving the lower lip, chin, and adja-cent neck. Given the linear orientation, it is impor-tant to design the reconstruction to minimizetension on the repair while breaking up the scarline to avoid scar hypertrophy and contracture.The senior author’s surgical approach has beendescribed in detail in a previous publication(Margulis et al. 2003).

Sebaceous Nevi

Sebaceous nevi are hamartomas of sebaceousglands and represent a type of epidermal nevuspresent at birth as a waxy, hairless, yellow-orangeplaque usually on the scalp, head, or neck

Fig. 11 (a) ILVEN –Inflammatory linear verrucous epidermal nevus; (b) Linear sebaceous nevus

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(Fig. 12a). Over time, they can become morenodular, verrucous, and itchy most pronouncedat puberty. Early reports showed a 15–20% life-time risk of malignant transformation into basalcell carcinoma (Michalowski 1962; Mehreganand Pinkus 1965). However, more recent seriesreport the number to be much lower (0.8–6.5%)(Cribier et al. 2000; Jones et al. 1970). This dis-crepancy is grounds for controversy, but theirremoval is still recommended.

Spitz Nevi

Although not usually congenital, Spitz nevi areanother commonly encountered pediatric skinlesion. They present as pink, raised, firm lesionsthat on occasion can be darkly pigmented(Fig. 13). At times, they may be confused withpyogenic granulomas because of their appearanceand rapid growth at onset. Originally termed

benign juvenile melanoma, these lesions displaya bizarre pathology beneath the microscope andcan be confused with malignancy if the patholo-gist is not provided with the history of the lesionand patient’s age. In fact, these lesions are benign,but do grow rapidly and tend to recur aggressivelyif not completely excised. Because of that, exci-sion with a generous border of normal tissue willdecrease the chance of recurrence.

Management of Smalland Intermediate Lesions

As stated earlier, many small lesions can beremoved at a time during adolescence when thepatient can tolerate a local anesthesia. Most ofthese can be directly excised and the skin closedin layers. For some small lesions and intermediatelesions, excision should be approached in a stagedfashion. Goals of serial excision should be com-plete excision of the lesion in two to three surger-ies without distortion of surrounding features orrestrictive scar bands. If either of the

Fig. 12 Typical sebaceous nevus of the scalp

Fig. 13 Spitz nevus of the nose. (Puri P, Hollwarth ME2006 © Berlin, Heidelberg, New York: Springer Verlag2006)

Congenital Nevi 13

aforementioned is not possible, or is questionable,tissue expansion should be considered. By stagingan excision, the scar will typically be shorter thana single stage excision just by nature of reducingthe size of the dog-ears. In the author’s experi-ence, a staged excision can be used to reorient ascar that may not be as feasible in a single stage.This is especially true for nevi of the face that areadjacent to the eyelids, nasal ala, or lips or mod-erate sized nevi of the trunk near the breast or thegenitalia. Time between stages should be calcu-lated based on expected scar maturation and notbe performed during the proliferative phase ofwound healing. This occurs around 3–4 monthspostexcision, but may be longer depending on theindividual patient, location, wound complica-tions, and scar management.

Management of Large and GiantCongenital Melanocytic Neviand Other Large Lesions

Treatment for large and giant CMN remains con-troversial to some, as they feel the risk of malig-nant degeneration within these lesions is too lowto warrant the extensive number of surgeries andpotential scar burden associated with removal.Others feel the potential for noncutaneous mela-noma to develop in extra-cutaneous nevus cellsnot amenable to surgical removal (such as inNCM) represents a remaining risk that negatesthe effectiveness of cutaneous excision for reduc-tion of melanoma risk (Kadonaga and Frieden1991). However, the significant deformity andassociated psychological impact caused by theselesions often tip the scales toward intervention.Management plans should always strive to bal-ance removal of the nevus with a functional andaesthetic reconstruction, and lesions that cannotbe effectively addressed in this manner should beconsidered for conservative management usingserial observation by an experienceddermatologist.

Treatment options are most often surgical withexcision and reconstruction using adjacent orexpanded skin or skin grafts/substitutes, but par-tial excision techniques can also be used.

Dermabrasion, Curettage, and LaserTherapy

Partial thickness excisions for CMN includingdermabrasion, curettage, Versa Jet, and laser ther-apy have been reported as effective methods ofreducing the cell burden to improve appearance ofthe nevus without completely excising it. Theoriginal theory behind these techniques relied onthe belief that in infancy nevus cells lay in asuperficial location within the upper reticular der-mis and epidermis and gradually descended todeeper layers within the 1st weeks of life. How-ever, this theory has not been substantiated(Marghoob 2013). Dermabrasion works to abradeaway the surface cells, whereas curettage sepa-rates the cells at the natural cleavage planebetween the superficial and deep dermis. Thesemethods do nothing to address deeper nevus cellswithin the deeper dermis or subcutaneous tissues.Therefore, the number of nevus cells within alesion is reduced but not eliminated. Initially,lesions may be lighter, but may darken with timeas described in long term study (Magalon et al.1998). In addition, any associated hypertrichosisis not affected, as hair follicles are found muchdeeper extending into the subcutaneous fat. Sub-sequent problems arise in these patients relating topruritus and scarring secondary to the disruptedhair follicles with trapped and ingrown hair.Finally, malignant potential remains for the left-over nevus cells and scarring from these interven-tions may make follow up examination difficult.At least one case has been reported of melanomaarising within a large trunk nevus following derm-abrasion (Dragieva et al. 2006). Due to thesedownsides, many practitioners have largely aban-doned split thickness excision as a method oftreatment.

Laser offers another potential treatment strat-egy attractive to both the patient and doctor as asimplified method to reduce the pigmentation of alesion without scarring. Laser therapy is wellsuited for the treatment of lesions characterizedby superficial dermal pigmentation such as theNevi of Ota and Ito, persistent Mongolian spots,café au lait macules, and some elements of nevusspilus due to its minimal thickness, location of

14 B. S. Bauer and S. R. Dickie

pigment within the dermis, and low potential formalignancy. Effective treatment relies on properwavelength and pulse-width selection to allowpenetration of the skin and photoselective destruc-tion of melanin while preserving the remainingelements to avoid scarring (Carpo et al. 1999;Alster 1995). Serial treatments are required. Scar-ring can occur with aggressive treatment orimproper setting selection. Hypo- and hyper-pigmentation have also been reported which maybe temporary or permanent. Exposure to sunlightin the perioperative period can cause significantburning, scarring, and hyperpigmentation.

Unfortunately, because CMN display nevuscells within all layers of the skin as well as withinthe deeper structures, it is unrealistic to think that alaser could effectively penetrate to the depthsnecessary to eliminate all pigment-producingcells without significant secondary damage andscarring. In addition, laser treatment vaporizesthe specimen, therefore eliminating histologicevaluation of the lesion to determine its nature.Finally, the impact of the exposure of nevus cellsto the radiant energy associated with laser therapyis unknown and may not be apparent for manyyears into the future.

Although dermabrasion, curettage, and laseroffer a relatively simple approach to improvingthe appearance of CMN, they present many draw-backs. In addition, scarring from these therapiesmay complicate ultimate excisional treatmentoptions in the future. Finally, the delay of moredefinitive reconstruction may have its own psy-chological effects on a child. Although limited useof these therapies may be useful in certain areas ofreconstruction (such as lightening of a thin lesionin the cosmetic and functionally sensitive eyelidarea), thoughtful consideration is warranted priorto widespread implementation of these modalitiesin a treatment algorithm for large CMN.

Surgical Excision and Reconstruction

The benefits of surgical excision of large and giantCMN include complete removal of involved skinand subcutaneous tissue. Although some havefocused on skin grafts, skin substitutes, or

cultured epithelium for reconstruction, when pos-sible, use of tissue expansion allows the mostfunctional and aesthetic result by allowingreplacement with full thickness normal tissue.Goals of treatment include complete excision atan early age, minimization of scarring, and func-tional impact with a low requirement for futureprocedures. Early excision is emphasized for fourreasons. First, the greatest risk for malignancy isreported in childhood, most notably in the first3 years of life. Second, the elasticity and healingcapacity of the skin is better the younger thepatient. Third, patients operated on in infancytolerate surgery better both physically and emo-tionally than their older counterparts. Finally, bycompleting reconstruction early, the psychologi-cal impact of the lesions and surgical interventionscan be reduced.

In 1988, the senior author presented a coordi-nated approach to the management of theselesions in 78 patients (Bauer and Vicari 1988).This report outlined the spectrum of treatmentoptions from skin graft to tissue expansion andassessed the effectiveness of excision and recon-struction with each technique in each body region.Since then, further experience with over300 patients has allowed further developmentand refinement of approach (Bauer and Margulis2004;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 man-agement of each of large CMN is beyond thescope of this chapter, the following is a summaryof the author’s current approach.

Tissue Expansion

Several types of tissue expanders exist. The morecommonly used include the round and rectangulartypes; crescent-shaped (and croissant) prostheses,originally developed to minimize dog-ears at thedonor site, have fallen out of favor as it has beenrecognized that the added tissue gained with rect-angular expanders may increase the choices pos-sible for flap design (i.e., use of transpositionflaps). This author prefers using rectangularexpanders in almost all cases. Expander volumes

Congenital Nevi 15

have a wide range and vary according to theanatomic site. Rectangular expanders of70–1000 cc are standard, but smaller and largercustom expanders are available. Saline is deliv-ered via a remote valve port connected to thedevice by silicone tubing placed in a separatesubcutaneous pocket. Remote, internal ports arepreferred in the author’s practice to allow saferhome expansion and avoid the risk of perforationby a misplaced needle accessing an integratedport. Risks of the remote port include migrationof the port, flipping of the port, tube obstruction,kinking, or separation. To avoid these, meticulousattention is paid to the tubing length and courseand ensuring a reliable port connection. As well,dissection of the port pocket is kept to a minimumand the port placed over firm supporting tissue. Ifneeded, the port can be secured with suture. Onthe extremities larger ports are used to help avoidmigration and turnover. The expander is typicallypositioned directly adjacent to or partially belowthe nevus itself. Donor skin needs to be free ofinfection, trauma, or previous scars (or havemature, stable scars) to prevent flap breakdownor extrusion. In the majority of cases, the expandercan be placed through an incision within theboarder of the nevus.

Design of the flap is not a frivolous undertak-ing. Historically, expansion was used to generateadvancement flaps only. Experience has demon-strated that expanded transposition and rotationflaps are frequently preferable, allowing greaterversatility in flap design and more effective use ofthe expanded tissue. The capsule of the expandedflap also provides an improved blood supply to thetissue allowing for greater advancement and rangeof motion on a smaller pedicle than would beallowed in a nonexpanded flap. In applying thisprinciple, it is important to avoid scoring of thecapsule so that the added vascularity provided bythe 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 thefree margin of advancing tissue as possible. Thiswill improve the blood flow to this segment of theflap. Inset of the expanded flap is done in layeredfashion ensuring that sutures capture a portion ofthe capsule. By preventing capsular recoil both

the arterial inflow and the venous outflow of theflap are preserved.

ScalpScalp reconstruction is complicated by its rela-tively inflexible quality and the unique aestheticsof maintaining hair quality and direction. Becauseof this, tissue expansion remains the workhorsefor scalp reconstruction during excision of largeCMN. Scalp lesions are best reconstructed instages, with placement of one or more tissueexpanders in a subgaleal plane beneath the normalscalp skin. Flaps are designed with considerationof the major blood supply to the scalp (superficialtemporal, postauricular, occipital, and contribu-tions from the supraorbital vessels). Followingadequate expansion (generally from 10 to12 weeks), the expanders are removed, lesionsexcised, and the defect closed using both advance-ment and transposition flaps designed to preservehair direction and hairline (Bauer and Margulis2004). Flaps should be carried out and checked forviability before excision of the nevus. The seniorauthor has found that by planning transpositionflaps, the number of expansions needed isreduced. Serial expansion of the scalp typicallyleads to scarring of the galea to the periosteum. Ifthis occurs, expander placement below the peri-osteum can be performed in the subsequentstages. In these cases it is often difficult to providesignificant prefill of the expander at the time ofsurgery. Therefore, the expansion process maytake slightly longer than the first or second roundof expansion. Defects in the temporal hairline areoften best treated with a large transposition flapfrom the occipital scalp. This provides favorablehair direction for both the scars and the hair alongthe sideburn.

Expansion of the infant scalp can safely beginat 6 months of age. Careful attention should begiven to the fontanel during expander placementand reconstruction (Fig. 14). Although temporarycranial molding is common at this age, this doesnot persist and spontaneously corrects within1–4 months following expander removal. Inolder children and adults, the capsule can form a“wash-tub” deformity at its margins along thecranium. At the time of reconstruction, the free

16 B. S. Bauer and S. R. Dickie

edge of this can be safely excised as the flap isinset. The posterior edge which is attached to theflap capsule and carries the pedicle should be leftintact. This ridge resolves once the expander hasbeen removed.

ForeheadLarge CMN of up to 75% of the forehead can bemanaged with serial tissue expansion(Fig. 15a–d). This requires careful planning tominimize distortion of the eyebrow position andmaintain 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 bethe superior margin of the dog-ear. This allows agenerous segment of expanded tissue to be trans-posed to the fronto-temporal region of the fore-head and the temple. Nevi extending into thetemporal area must be treated by expansion ofboth scalp and forehead to reconstruct the tempo-ral hairline (Unlu et al. 2002). Bilateral expansionof normal forehead is often successful for mid-forehead lesions. In cases of brow elevation, the

abnormal brow can be returned to its preoperativeposition by interposing nonhair-bearing foreheadskin. Most importantly, don’t overextend the flap,re-expand it.

Face and NeckLarge CMN of the face present some of thegreatest reconstructive challenges. By dividingthe face into aesthetic units, tissue expansion ofavailable adjacent areas of normal skin in the neckand forehead can provide skin of excellent qualitymatch while allowing scars to lie at the junction ofaesthetic units and render them less obvious.When advancing tissue from the neck into thecheek area, a transposition design can helpimprove flap movement while aligning the scarsoptimally. Whether transposed with the flap basedlaterally or medially, the transposition minimizesthe risk of downward drift of the flap andectropion of the lower eyelid (Bauer and Margulis2004). Lesions of the nose are best resurfaced byan expanded forehead flap carried on a supra-trochlear, superior orbital, or superficial temporal

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)

Congenital Nevi 17

Fig. 15 (a) Preoperative photo of a patient with a giantCMN of the scalp, forehead, brow, and cheek. (b) Patientfollowing first round of expansion and reconstruction.Expanded flaps from the scalp, forehead, and neck were

used. (c, d) The same patient following second round ofexpansion and reconstruction. Scars fall within the bordersof aesthetic subunits

18 B. S. Bauer and S. R. Dickie

artery pedicle. In children, the typical wider scarleft in adult paramedian donor sites can be quitedisfiguring. This can be minimized bypre-expanding the flap. If forehead is not avail-able, an expanded supraclavicular full thicknessskin graft provides a suitable option. The peri-orbital area is also best addressed with expandedfull-thickness skin grafts to allow preservation ofthe thin nature of the tissue while decreasing inci-dence of ectropion (Margulis et al. 2009). Forlarge facial nevi involving the eyelids, the recon-struction of the lids is the final phase after the restof the nevus has been excised and the surroundingsubunits reconstructed. This allows placement ofgrafts along stable scar margins. Any ectropion orlid distortion that has been caused by the nevus orprior reconstruction can be addressed at this pointas well with either medial or lateral canthoplasty.

EarLesions on the ear are addressed with excision andfull thickness skin grafting (Fig. 16a–c) Forlesions involving the entire ear, the senior authorhas developed a staged approach (Adler et al.2009a). The subunits of the ear can be identifiedas stable or unstable. Stable subunits are thosewhose cartilage is relatively firm and inflexible.

These can be excised and grafted during infancy.The concha and scaphal subunits fall into hiscategory. Full thickness grafts are taken from thecontralateral post-auricular skin. More flexibleparts of the ear, such as the helical rim, are atgreater risk of distortion by grafting. These areaddressed at 6–7 years of age, when the ear hasachieved nearly adult size and the cartilage ismore robust. The postauricular surface on theeffected ear can be replaced with full thicknessskin from the groin at any age. The ear lobe pre-sents a unique challenge due to its thin, soft, andpliable nature. When possible, the earlobe isreconstructed with normal skin from the neck. Inthe case of giant CMN which covers the entirescalp and neck adjacent to the ear, these can bereconstructed with an expanded supraclavicularartery flap. If this is undertaken, the lobe is createdat the same time or subsequent to the flaptransposition.

TrunkThe often extensive involvement of giant CMN ofthe trunk with relative lack of normal donor skinpresents a daunting challenge to the reconstruc-tive surgeon, leading many to resort to split thick-ness skin graft based reconstruction (Fig. 17a, b).

Fig. 16 (a) Preoperative photo of a large CMN of the earand peri-auricular tissue. (b) Patient following reconstruc-tion with expanded neck flap for the peri-auricular tissueand earlobe. Concha was reconstructed with full thickness

skin graft from the contralateral ear. (c) Third stage, patientunderwent full thickness skin graft of the scapha. She willreturn for the final stage of skin grafting of the helix in1–2 years

Congenital Nevi 19

The inferior functional and aesthetic resultsachieved call into question whether excisionshould have been undertaken at all in favor ofconservative observation. With better expandedflap design carried out in series, or use ofexpanded distant flaps with microvascular trans-fer, superior trunk reconstruction can be achieved.

The posterior trunk is the most common loca-tion of giant CMN often extending anteriorly in adermatome distribution. These lesions are bestreconstructed with serial tissue expansion andsubsequent transposition flap closure (Fig. 18).By utilizing flap transposition over simpleadvancement, excision and reconstruction ofbathing trunk and large thorax nevi previouslyfelt to be treatable only with skin grafting hasbecome possible (Bauer and Margulis 2004).Anterior trunk lesions can be treated with anabdominoplasty technique with or without expan-sion depending on lesion size. When adjacent

donor sites are unavailable for expansion, exci-sion and reconstruction of shoulder, upper back,and posterior neck nevi can be accomplishedusing microvascular transfer of a free expandedtransverse rectus abdominus myocutaneous(TRAM) flap. (Fig. 19a–c). One must keep inmind the gender of the patient during reconstruc-tion of the trunk. When approaching a female witha large or giant CMN of the trunk, every effort ismade to avoid the nipple areola and the futureposition of the breast when placing expandersand transposing tissue. For males, the nipple andareola can be moved with the flaps with the antic-ipation that free nipple grafting will be performedfollowing the reconstruction of the trunk. Withincreased use of expanded flaps over skin graftingfor trunk reconstruction, late contour deformitiesseen at junction points between grafted andungrafted areas can be significantly reduced.These modified techniques have resulted in

Fig. 17 (a) Split thickness skin graft used for reconstruction of the back when lack of suitable donor sites are available forexpansion. (b) Boarder areas can then be expanded to compete the reconstruction

20 B. S. Bauer and S. R. Dickie

aesthetic benefits far beyond what could beaccomplished with alternative treatments.

ExtremitiesLarge CMN of the extremities continue to presenta considerable challenge due to the limitations oflocal expansion techniques in these areas and therelatively poor aesthetic outcome experiencedwith skin grafting. The author’s initial approachutilized both split-thickness and expanded full-thickness skin grafts for most lesions, but thelong-term soft-tissue contour defect and pigmentabnormalities in the grafted skin have led to theuse of alternative approaches (Margulis et al.2004). In upper extremity lesions, use of transpo-sition flaps from the upper back and shoulder haseffectively eliminated contour defects to the prox-imal upper extremity. In addition, three-stage,expanded pedicled flaps from the abdomen andflank can be designed to provide complete cover-age of a circumferential nevus from elbow to wrist

with excellent contour and acceptable scarringachieved at both donor and recipient sites(Margulis et al. 2004) (Fig. 20a–f). A similarpedicled approach has been successful in provid-ing coverage for lower leg lesions (Fig. 21a–f). Anipsilateral expanded thigh flap in the young infantcan be transposed to the lower leg when lowerextremity flexibility is at its peak and provideexcellent results (Kryger and Bauer 2008).

With experience, expansion of the extremityskin is feasible. One must assess the lesion andunderstand the limitations in the basic geometryof the limb (flap movement is easier around thecircumference than in an axial direction). Carefulplanning, choice of expander size and placement,and a slower expansion schedule leads to lowerrisk of extrusion and flap failure. Placement ofexpanders is easier above the knee than below.When possible, more than one expander should beused. For large extremity lesions, using expansionas an adjunct to reduce the overall size of the

Fig. 18 (a, b) Preoperative photos of a giant CMN of thetrunk. (c, d) The patient at the completion of expansionprior to excision of the nevus. (e, f) After first stage

reconstruction. (g, h) Following second round of expanderreconstruction

Congenital Nevi 21

defect to then be covered by a free flap is anotheruseful approach.

Expanded free flaps offer another alternativefor larger lesions of the extremities with improvedfunctional and aesthetic outcomes. These proveespecially useful for coverage of the knee, lowerleg, and ankle. Pre-expansion of the flap or thetissue adjacent to the donor site reduces the donorsite morbidity in pediatric patients, often timesavoiding the need for a skin graft.

For the dorsum of the hands and feet, the use ofexpanded full thickness skin grafts is the bestoption, where subcutaneous tissue is often mini-mal and a full thickness graft provides a suitablereplacement. For the fingers and toes, serial exci-sion has been found to reduce the bulky and oftenverrucous nature of the nevus in these areas.Although the pigmented skin remains, there isoften a compensatory lightening of the tissuesfollowing partial excision (Fig. 22a–c).

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, Heidel-berg, New York: Springer Verlag 2006)

22 B. S. Bauer and S. R. Dickie

Complications

Complications of surgical excision of large CMNwith expanded flap reconstruction are uncommondespite the complex nature of these procedures.During the expansion process, the primary risksinclude expander infection, expander exposure orextrusion, flap compromise, and incisional dehis-cence. The incidence of expander infection hasbeen reported at 5%, which can be due to inocu-lation during expander insertion or repeatedexpansions, after exposure of the expander or byhematogenous seeding from a distant infection(Adler et al. 2009b). These infections can usuallybe managed conservatively by antibiotic adminis-tration allowing completion of expansion and

successful reconstruction. A low threshold forplacing the patient on oral antibiotics duringhealing problems or illness occurring duringexpansion may help minimize risk. It is also vitalto maintain a close communication with thepatient caregivers to ensure there is a free flowof information and prompt reporting of any signsof the aforementioned complications. Well-trained ancillary staff, nursing, and on-call per-sonnel can have a dramatic influence on the suc-cess of tissue expansion.

Technical considerations for the use ofexpanded flap reconstruction rely on the under-standing of the unique characteristics provided byexpansion. Vascularity of a flap through the delayphenomenon makes flap ischemia rare.

Fig. 20 (a) Large CMN of arm and elbow. (b, c) Pedicledexpanded abdominal flap used for reconstruction. (d) Post-operative photo following division of pedicle. (e, f) Final

result following secondary advancement of flap andremoval of remaining nevus

Congenital Nevi 23

Preservation of the capsule with judiciouscapsulotomy and avoidance of excessive tensionduring closure can prevent this potentially

devastating complication. Venous congestionand wound dehiscence can be improved by ensur-ing the capsule is advanced along with the flap and

Fig. 21 (a) Large CMN of lower leg. (b–d) Pedicled expanded posterior thigh flap used for reconstruction. (e, f) Finalresult at 3 years. (Puri P, Hollwarth ME 2006 © Berlin, Heidelberg, New York: Springer Verlag 2006)

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

24 B. S. Bauer and S. R. Dickie

secured using deeper sutures thereby preventingits recoil during the final closure. Finally, meticu-lous closure and postoperative scar managementhelp achieve optimal results. It is important todiscuss scarring with patient families. Whilescars can be quite extensive for large and giantCMN, if they run in favorable directions to avoidtension, even long or wide scars can fade verynicely.

Conclusion and Future Directions

The treatment of congenital large and giant nevipresents a continuing challenge to all individualsinvolved with these patients. The ability to presentan organized discussion of current views on riskof malignant change to parents, patients, referringphysicians, and other allied health care workers iscritical. Treatment strategies should take into con-sideration the varied opinions regarding malig-nant risk, emphasize the benefits of earlyexcision on lowering that risk, and most impor-tantly, provide a means of dealing with these oftendevastating lesions in a manner that optimizes thefunctional and aesthetic outcome and minimizesthe need for major reconstruction in later life.

Cross-References

▶Anatomy of the Infant and Child▶Anesthesia and Pain Management▶Ethical Considerations▶ Surgical Safety

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