10
Giant Congenital Nevi: A 20-Year Experience and an Algorithm for Their Management Arun K. Gosain, M.D., Timothy D. Santoro, M.D., David L. Larson, M.D., and Reudi P. Gingrass, M.D. Milwaukee, Wis. A variety of treatment options exists for the manage- ment of giant congenital nevi. Confusion over appropriate management is compounded because not all giant con- genital nevi are pigmented, and malignant potential varies between different types. The present study sought to de- fine factors in the presentation of giant congenital nevi that could provide an algorithm for their management, with respect to both the extent of resection and subse- quent reconstructive options. A retrospective review of all patients who presented with a congenital nevus of 20 cm 2 or greater since 1980 was performed, distinguishing among nevi involving the head and neck, the torso, and the extremities. Sixty-one pa- tients with giant congenital nevi were evaluated (newborn to age 16 years), of which 60 nevi in 55 patients have been operated on. Giant congenital nevi having malignant potential were pigmented nevi (53 patients) and nevus sebaceus (four patients). Those not having malignant potential were ver- rucous epidermal nevi (three patients) and a woolly hair nevus (one patient). Of the 60 giant congenital nevi op- erated on, expanded flaps were used in 25, expanded full-thickness skin grafts were used in 10, split-thickness or nonexpanded full-thickness skin grafts were used in 13, and serial excision was used in 30. After 1989, operations tended to use multimodality treatment plans, with an in- creased use of expanded full-thickness grafts and imme- diate serial tissue expansion. The use of serial excision, particularly in the extremities, also increased after 1989. Serial excision was the treatment of choice when it could be completed in two procedures or less, which occurred in more than 80 percent of cases using serial excision alone. Expanded flaps were the most common mode of reconstruction in the head and neck region and were used in 49 percent of these procedures. Serial excision was the most common form of treatment in the extremities, used in 50 percent of procedures. Tissue expansion in the extremities was infrequently used to provide an expanded flap (8 percent of procedures), whereas it was frequently used to provide expanded full-thickness skin grafts har- vested from the torso (used in 31 percent of procedures). On the basis of these data, algorithms for the extent of resection and subsequent reconstructive options for giant congenital nevi were developed. Their management should be formulated relative to pigmentation, malignant potential, and anatomic location of the respective lesions. (Plast. Reconstr. Surg. 108: 622, 2001.) The optimal management of giant congeni- tal nevi has been a longstanding surgical chal- lenge, both because of differences pertaining to the unique biologic characteristics belong- ing to the different types of nevi and because of the reconstructive complexity. Arbitrary size criteria defining pigmented giant congenital nevi include those greater than 2 percent body surface area, 1 nevi greater than 20 cm in largest diameter, 2 and nevi that cannot be excised in a single procedure. 3 We chose to study lesions greater than 20 cm 2 in size. Because there is considerable overlap in reconstructive princi- ples after resection of both pigmented and nonpigmented giant congenital nevi, our sur- gical experience for all forms of the lesion is combined to develop a more consistent and clinically useful algorithm. Because the potential for malignant transfor- mation exists within pigmented giant congen- ital nevi, 1 surgical excision remains the stan- dard of care for their treatment. Dermabrasion has been used in the treatment of these le- sions 4 ; however, this technique may not re- move the majority of nevus cells and is not currently recommended as an effective treat- ment for the prevention of potential malignant transformation. 5 Although laser therapy has also been used to treat congenital pigmented nevi 6 and may be useful in lightening unresect- able lesions, the number of nevus cells actually removed by this technique cannot be docu- From the Department of Plastic and Reconstructive Surgery, Medical College of Wisconsin. Received for publication February 7, 2000; revised October 20, 2000. Presented at the 78th Annual Meeting of the American Association of Plastic Surgeons, in Colorado Springs, Colorado, May 4, 1999. 622

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Giant Congenital Nevi: A 20-Year Experienceand an Algorithm for Their ManagementArun K. Gosain, M.D., Timothy D. Santoro, M.D., David L. Larson, M.D., and Reudi P. Gingrass, M.D.Milwaukee, Wis.

A variety of treatment options exists for the manage-ment of giant congenital nevi. Confusion over appropriatemanagement is compounded because not all giant con-genital nevi are pigmented, and malignant potential variesbetween different types. The present study sought to de-fine factors in the presentation of giant congenital nevithat could provide an algorithm for their management,with respect to both the extent of resection and subse-quent reconstructive options.

A retrospective review of all patients who presentedwith a congenital nevus of 20 cm2 or greater since 1980 wasperformed, distinguishing among nevi involving the headand neck, the torso, and the extremities. Sixty-one pa-tients with giant congenital nevi were evaluated (newbornto age 16 years), of which 60 nevi in 55 patients have beenoperated on.

Giant congenital nevi having malignant potential werepigmented nevi (53 patients) and nevus sebaceus (fourpatients). Those not having malignant potential were ver-rucous epidermal nevi (three patients) and a woolly hairnevus (one patient). Of the 60 giant congenital nevi op-erated on, expanded flaps were used in 25, expandedfull-thickness skin grafts were used in 10, split-thickness ornonexpanded full-thickness skin grafts were used in 13,and serial excision was used in 30. After 1989, operationstended to use multimodality treatment plans, with an in-creased use of expanded full-thickness grafts and imme-diate serial tissue expansion. The use of serial excision,particularly in the extremities, also increased after 1989.Serial excision was the treatment of choice when it couldbe completed in two procedures or less, which occurredin more than 80 percent of cases using serial excisionalone. Expanded flaps were the most common mode ofreconstruction in the head and neck region and were usedin 49 percent of these procedures. Serial excision was themost common form of treatment in the extremities, usedin 50 percent of procedures. Tissue expansion in theextremities was infrequently used to provide an expandedflap (8 percent of procedures), whereas it was frequentlyused to provide expanded full-thickness skin grafts har-vested from the torso (used in 31 percent of procedures).

On the basis of these data, algorithms for the extent ofresection and subsequent reconstructive options for giantcongenital nevi were developed. Their management

should be formulated relative to pigmentation, malignantpotential, and anatomic location of the respectivelesions. (Plast. Reconstr. Surg. 108: 622, 2001.)

The optimal management of giant congeni-tal nevi has been a longstanding surgical chal-lenge, both because of differences pertainingto the unique biologic characteristics belong-ing to the different types of nevi and because ofthe reconstructive complexity. Arbitrary sizecriteria defining pigmented giant congenitalnevi include those greater than 2 percent bodysurface area,1 nevi greater than 20 cm in largestdiameter,2 and nevi that cannot be excised in asingle procedure.3 We chose to study lesionsgreater than 20 cm2 in size. Because there isconsiderable overlap in reconstructive princi-ples after resection of both pigmented andnonpigmented giant congenital nevi, our sur-gical experience for all forms of the lesion iscombined to develop a more consistent andclinically useful algorithm.

Because the potential for malignant transfor-mation exists within pigmented giant congen-ital nevi,1 surgical excision remains the stan-dard of care for their treatment. Dermabrasionhas been used in the treatment of these le-sions4; however, this technique may not re-move the majority of nevus cells and is notcurrently recommended as an effective treat-ment for the prevention of potential malignanttransformation.5 Although laser therapy hasalso been used to treat congenital pigmentednevi6 and may be useful in lightening unresect-able lesions, the number of nevus cells actuallyremoved by this technique cannot be docu-

From the Department of Plastic and Reconstructive Surgery, Medical College of Wisconsin. Received for publication February 7, 2000; revisedOctober 20, 2000.

Presented at the 78th Annual Meeting of the American Association of Plastic Surgeons, in Colorado Springs, Colorado, May 4, 1999.

622

mented. For this reason, the present study willfocus only on surgical excision of giant congen-ital nevi, because this is the only technique inwhich complete removal of nevus cells can bedocumented.

The purpose of the present study is to ad-dress the following questions: (1) What are thereasons to operate on pigmented and nonpig-mented lesions, and how extensive a resectionshould be performed for each presentation ofa giant congenital nevus? (2) What reconstruc-tive options should be used after resection of agiant congenital nevus, and when is it appro-priate to combine different reconstructive mo-dalities? (3) Do the optimal reconstructive op-tions vary in different anatomic regions of thebody?

METHODS

A 20-year retrospective review was performedfor all giant congenital nevi evaluated at theMedical College of Wisconsin from January of1980 through May of 1999. Giant congenitalnevi were defined as all nevi with a surface areagreater than 20 cm2. A separate assessment wasmade for the head and neck, torso, and ex-tremities. Lesions of the head and neck con-sisted of all lesions above the clavicles. Lesionsof the torso consisted of all lesions involvingthe chest, abdomen, back, and buttocks. Le-sions of the extremities consisted of all lesionsdistal to the axilla in the upper extremities ordistal to the groin in the lower extremities.

Single lesions extending into more than oneof the above-defined regions were classified asdistinct nevi, provided the component withinthe given region was greater than 20 cm2. Anevus extending from the back to the arms thatinvolved greater than 20 cm2 in each of the twobody zones was classified as two giant congen-ital nevi for the purpose of this study. Althoughmultiple satellite nevi were present in severalpatients, the index lesion was still required tobe greater than 20 cm2 to be considered a giantcongenital nevus.

RESULTS

Of the 61 patients who were evaluated, 60nevi in 55 patients were operated on. On pre-sentation, there were 60 pigmented giant con-genital nevi in 53 patients (Table I). Among all61 patients at initial presentation, five patientseach had two lesions and two patients hadthree. One patient had a giant nevus involvingtwo different body regions. Age range of the

patients was from newborn to 16 years. Nofocus of malignancy or cytoatypia was identi-fied in any patient. The deep margin of resec-tion was positive for nevus cells in 14 resectedspecimens, all of which were pigmented le-sions. Four of the 14 patients (29 percent) withpositive deep margins later developed recur-rent visible pigmentation in the reconstructedarea. No patient presented with symptoms sug-gestive of central nervous system involvement.Magnetic resonance imaging of the brain andspinal cord was performed in five patients,each with extensive pigmented giant congeni-tal nevi involving at least 50 percent body sur-face area. In one patient, an asymptomatic fo-cus of leptomeningeal melanosis was foundwithin the cerebellum for which no specifictreatment was recommended.

Reconstructive techniques consisted of ex-panded skin flaps, expanded skin for full-thickness skin grafts, nonexpanded full-thickness skin grafts, split-thickness skin grafts,and serial excision. Expanded flaps were usedin 25 nevi, expanded full-thickness grafts wereused in 10 nevi, split-thickness or nonex-panded full-thickness skin grafts were used in13 nevi, and serial excision was used in 30 nevi.Nine nevi were operated on before 1990,equally distributed among three surgeons. Fif-ty-one nevi were operated on after 1989, 49 ofwhich were operated on by one surgeon(A.K.G.). The use of split-thickness skin graftsdecreased from 22 percent before 1990 to 6percent after 1990, whereas the use of nonex-panded full-thickness grafts increased fromnone before 1990 to 10 percent after 1989.This change reflects the evolving preferencefor the use of full-thickness grafts over split-thickness grafts toward the latter portion ofthis series.

The relationship between treatment modal-ity and its use in single versus multiple modalitytreatment is shown in Table II. All five recon-structive options were commonly used in both

TABLE IDistribution of Giant Congenital Nevi

No. ofPatients

No. ofNevi

Nevi thought to have malignant potentialPigmented 53 60Nevus sebaceus 4 4

Nevi without known malignant potentialWoolly hair 1 1Inflammatory linear verrucous epidermal 3 5

Vol. 108, No. 3 / MANAGEMENT OF GIANT CONGENITAL NEVI 623

single and multiple modality treatment. Eachof the five reconstructive options constitutedone component of a multimodality treatmentplan at least 40 percent of the time. Whensingle modality treatment was used, multipleprocedures were often required. The numberof procedures needed to complete reconstruc-tion when single modality treatment was usedis shown in Table III. Serial expansion wasrequired in six (33 percent) of the 18 nevi inwhich expanded flaps alone were used to re-construct the defect, two of which requiredfour successive stages of serial expansion forgiant congenital nevi of the scalp. More than80 percent of nevi treated with serial excisionalone could be completed in two proceduresor less. Three successive procedures were re-quired in 19 percent of nevi treated with serialexcision alone. The latter cases involved theknee or elbow, where increased morbiditymight have resulted from the use of alternativereconstructive options.

The relationship between the anatomic zonein which the nevus was located and the selectedtreatment modality is shown in Table IV. Ex-panded flaps were the most common mode ofreconstruction in the extremities. Expandedflaps and serial excision were more equallydistributed in the torso. Serial excision was themost common form of treatment in the ex-tremities, and expanded flaps were used infre-

quently. The primary use of tissue expansionfor extremity reconstruction was to provide ex-panded full-thickness skin grafts. Representa-tive cases demonstrating principles of recon-struction in each of the anatomic zones arepictured in Figures 1 through 7.

Head and Neck

Single modality treatment of a giant congen-ital nevus of the scalp is shown in Figure 1. A4-year-old girl presented with a woolly hair ne-vus occupying two-thirds of the occipitoparietalscalp (Fig. 1, above, left). Serial tissue expansionwas performed, with new tissue expandersplaced immediately after advancement of thepreviously expanded scalp flaps. After serialexpansion in two stages, the remaining nevusoccupied approximately one-third of the occip-itoparietal scalp (Fig. 1, above, right). After fourstages, only a negligible amount of woolly hairnevus remained (Fig. 1, below).

Combined modality treatment was often re-quired for lesions of the head and neck involv-ing different anatomic structures. This is illus-trated by a 13-year-old girl who presented witha giant congenital nevus sebaceus of the lefttemporoparietal scalp, postauricular sulcus,and auricle (Fig. 2, above, left). Tissue expan-sion was used to resurface the temporoparietalscalp in one stage (Fig. 2, above, right). Nonex-panded full-thickness skin grafts harvestedfrom bilateral groins were used to resurface thepostauricular sulcus and involved portion ofthe auricle (Fig. 2, below).

Treatment of complex lesions of the headand neck, particularly those involving the eye-lids, often required multiple modalities. This isillustrated by an 8.5-month-old girl who pre-sented with a giant congenital nevus involving

TABLE IIRelationship between the Selected Treatment Modality

and Combination Treatment

SingleModality

(%)

MultipleModalities

(%)

Expanded flaps (n � 25) 48 52FTSG (expanded) (n � 10) 50 50FTSG (nonexpanded) (n � 8) 25 75STSG (n � 5) 60 40Serial excision (n � 30) 53 47

FTSG, full-thickness skin grafts; STSG, split-thickness skin grafts.

TABLE IIINumber of Procedures Needed to Complete Coverage in

Single Modality Treatment

One(%)

Two(%)

Three(%)

Four orMore (%)

Expanded flaps (n � 18) 67 22 0 11FTSG (expanded) (n � 6) 100 0 0 0FTSG (nonexpanded) (n � 4) 100 0 0 0STSG (n � 2) 50 0 50 0Serial excision (n � 21) 24 57 19 0

FTSG, full-thickness skin grafts; STSG, split-thickness skin grafts.

TABLE IVRelationship between Nevus Location and Treatment

Modality

Head and Neck Torso Extremities

No. of nevi 23 15 22No. of procedures 35 17 26No. of modalities per nevus

Single (%) 70 80 73Multiple (%) 30 20 27

Expanded flaps* (%) 49 35 8FTSG (expanded)* (%) 3 6 31FTSG (nonexpanded)* (%) 20 0 4STSG* (%) 0 18 8Serial excision* (%) 29 41 50

FTSG, full-thickness skin grafts; STSG, split-thickness skin grafts.* Percent distribution is reported relative to the number of procedures

performed in the specified location.

624 PLASTIC AND RECONSTRUCTIVE SURGERY, September 1, 2001

the scalp, forehead, upper and lower eyelids,and cheek (Fig. 3). Tissue expanders wereplaced in the scalp and the forehead, andeach of these areas was reconstructed withexpanded flaps (Fig. 3, above, right). Serialexcision was used to reduce the nevus involv-ing the right cheek. The residual nevus in-volving the right upper eyelid and its nasalextension was resected and reconstructedwith a full-thickness skin graft obtained fromthe groin (Fig. 3, below, left). A tissue ex-pander was then placed in the cheek, and theresulting flap was used to reconstruct theright lower eyelid and temporal extension ofthe nevus (Fig. 3, below, right).

Torso

The torso was the most common location forsuccessful single modality treatment of giant

nevi. A 1-year-old girl presented with a pig-mented giant congenital nevus of the back anda second giant nevus involving the left buttock(Fig. 4). Separate single modality treatmentwas used for each giant congenital nevus, usingtwo-stage serial tissue expansion for the backand two-stage serial excision for the buttock.The completed reconstruction is shown in Fig-ure 4 (right).

ExtremitiesSerial excision was the preferred treatment

modality for lesions at or just proximal to theknee or elbow. A 10-year-old girl presentedwith a pigmented giant congenital nevus of thedistal right thigh just proximal to the knee(Fig. 5, left). The lesion was serially excised inthree stages, spacing each stage at least 6months apart (Fig. 5, right).

FIG. 1. Single modality treatment of a giant woolly hair nevus of the scalp with expanded flaps. (Above, left) Preoperativeappearance. (Above, right) Appearance after two-stage immediate serial expansion. (Below) Appearance after four-stage immediateserial expansion.

Vol. 108, No. 3 / MANAGEMENT OF GIANT CONGENITAL NEVI 625

Similar lesions distal to the knee or elboware difficult to treat using serial excisionalone because of reduced skin complianceand reduced diameter of the distal extremity.These areas are best treated with expandedfull-thickness skin grafts. A 9-month-old boypresented with a circumferential pigmentedgiant congenital nevus of the right forearmand hand (Fig. 6). The lower abdominal skinwas expanded, and a single expanded full-thickness skin graft was harvested to recon-struct the volar surface of the forearm andhand as one unit. This reconstruction is pic-tured 16 months postoperatively (Fig. 6, cen-ter, left). During a second procedure, the dor-sal digits and web spaces were resurfacedusing expanded full-thickness grafts. Becauseof limitations in expanded full-thicknessskin, a split-thickness graft was used to resur-face the dorsal surface of the wrist and fore-arm. Twelve months later, a marked differ-ence in aesthetic outcome could be seenbetween the full-thickness grafts covering the

digits and the split-thickness grafts coveringthe distal forearm (Fig. 6, center, right). Overthe next 3 years, there was progressive con-tracture of the expanded full-thickness graftreconstruction of the web spaces of the hand.This was corrected using dorsal rectangularflaps, raised from the initial full-thicknessgrafts, to deepen the second and third webspaces. Additional full-thickness grafts wereplaced on the borders of the respective dig-its. The first web space was deepened using aZ-plasty (Fig. 6, below).

A 4-year-old girl who presented with in-flammatory linear verrucous epidermal neviinvolving the scalp, vulva, and both the up-per and lower extremities is shown in Figure7. The right forearm and ring and indexfingers and the left index finger were themost symptomatic regions of the upper ex-tremities, and the dorsal ankle was the mostsymptomatic lower extremity region. Theseintensely pruritic regions were surgically ex-cised, with no effort made to obtain clearmargins. The digits were resurfaced with afull-thickness skin graft harvested from thegroin, and the symptomatic regions of thedorsal ankle, scalp, and vulva were eachtreated with serial excision in two stages.These procedures resulted in symptomaticrelief of the involved areas.

DISCUSSION

What Are the Reasons to Operate on Pigmented andNonpigmented Lesions, and How Extensive a Resec-tion Should Be Performed for Each Presentation of aGiant Congenital Nevus?

On the basis of our experience, an algorithmillustrating principles for the extent of resec-tion of pigmented and nonpigmented giantcongenital nevi is shown in Figure 8. Both pig-mented and nonpigmented nevi can presentwith aesthetic disfigurement, pruritus, and al-tered skin integrity, and may have malignantpotential. Significant aesthetic and psychoso-cial concerns were present with all four types ofgiant congenital nevi encountered, irrespectiveof pigmentation. The potential for malignanttransformation exists only with pigmented le-sions and sebaceous nevi, and only in the lattertwo nevi is there a need to clear the margins ofresection. Excoriation with resultant alterationin skin integrity severely diminishes the quality

FIG. 2. Treatment of a giant congenital nevus sebaceususing combined modality treatment with tissue expansionand a nonexpanded full-thickness skin graft. (Above, left) Pre-operative appearance. (Above, right) Appearance after one-stage reconstruction of the temporoparietal scalp with ex-panded flaps. (Below) Final appearance after nonexpandedfull-thickness skin graft to the postauricular sulcus andauricle.

626 PLASTIC AND RECONSTRUCTIVE SURGERY, September 1, 2001

of life in patients with inflammatory linear ver-rucous epidermal nevi. Pruritus and skinbreakdown are the primary reasons to excise

the latter lesions, and in patients with wide-spread epidermal nevi, surgical treatmentshould be limited to symptomatic areas.

FIG. 4. Single modality treatment for two separate pigmented giant congenital nevi of thetorso. (Left) Preoperative appearance of nevus on the back. (Center) Appearance after first oftwo-stage immediate serial expansion for nevus of the back, and preoperative appearance of thenevus involving the left buttock. (Right) Final appearance after two-stage immediate serialexpansion for nevus of the back, and two-stage serial excision for nevus of the left buttock.

FIG. 3. Combined modality treatment of a pigmented giant congenital nevus using expanded flaps, a full-thickness skin graft,and serial excision. (Above, left) Preoperative appearance. (Above, right) Appearance after reconstruction of the scalp and foreheadwith expanded flaps, and serial excision of the cheek. (Below, left) Intraoperative appearance after placement of a nonexpandedfull-thickness graft to the right upper eyelid and nasal extension of the nevus. (Below, right) Final postoperative appearance afterreconstruction of the right cheek and temporal extension of the nevus with an expanded flap.

Vol. 108, No. 3 / MANAGEMENT OF GIANT CONGENITAL NEVI 627

The risk of malignant transformation in pig-mented giant congenital nevi remains contro-versial. Quaba and Wallace1 calculated the riskof melanoma to be 8.52 percent during thefirst 15 years of life. A longitudinal study of alarge population of patients with pigmentedgiant congenital nevi reported a lifetime inci-dence of malignant transformation of 4 to 6percent.7 Other authors have reported the in-cidence of malignant transformation to be be-tween 2 percent and 31 percent.8 Although therate of malignant transformation as a functionof lesion size is not known, it is assumed thatthis risk within a given nevus is decreased pro-portionate to the fraction of the lesion re-moved or destroyed. The efficacy of dermabra-sion and laser treatment in removing ordestroying nevus cells has never been estab-lished.4–6 There are no longitudinal studiesdocumenting decreased rates of malignanttransformation after laser or dermabrasiontreatment. In fact, lightening of pigmented gi-ant congenital nevi using these techniques maymake it more difficult to monitor the resultantlesion for signs of malignant transformation,because alteration in pigmentation of the le-sion can no longer be followed reliably. Surgi-cal excision remains the treatment of choicefor the removal of pigmented lesions, becausethis technique has documented efficacy in re-moval of nevus cells.5

The extent of resection of giant congenitalnevi is closely linked to available reconstructiveoptions. Because the same surgeon is usuallyresponsible for both of these components of

the patient’s surgical care, the algorithms guid-ing each of these aspects of the proceduremust be interdependent. Because pigmented gi-ant congenital nevi have malignant potentialbut are most commonly benign on presenta-

FIG. 6. Combined modality treatment of a pigmented gi-ant congenital nevus involving the right upper extremity us-ing expanded full-thickness and split-thickness skin grafts.(Above) Preoperative appearance. (Center, left) Appearanceafter resurfacing the volar surface of the involved extremitywith a single expanded full-thickness graft. (Center, right) Ap-pearance after resurfacing the dorsal surface of the involvedextremity with a single expanded full-thickness graft from themetacarpal heads to the distal phalanges and split-thicknessgrafts to the proximal surface of the involved extremity. (Be-low) Appearance 3 years later after deepening of the first,second, and third web spaces of the left hand.

FIG. 5. Single modality treatment for a pigmented giantcongenital nevus proximal to the right knee using serial ex-cision. (Left) Preoperative appearance. (Right) Postoperativeappearance after three-stage serial excision.

628 PLASTIC AND RECONSTRUCTIVE SURGERY, September 1, 2001

tion, an incomplete resection may be indicatedin certain situations. An extensive resectionand reconstruction resulting in mutilation of-the involved body part, to remove a minimalamount of residual nevus, cannot be justifiedon the basis of current oncologic data. Theabove principle is illustrated in Figure 6, center,right. In this case, small distal foci of pigmen-tation were not resected to preserve theeponychial folds of the fingers and digital sen-sory function. In some instances, nevus cells

infiltrate deeply into muscle, bone, or carti-lage. In such situations, the patient and familyshould be educated regarding the morbidity offurther resection versus continued surveillancefor signs of malignant transformation.

Nevus sebaceus is associated with a 10 to 20percent rate of malignant transformation afterpuberty.9 Although the resultant cutaneousmalignancy is most often basal cell carcinomaor occasionally squamous cell carcinoma, othercarcinomas such as apocrine and adnexal tu-mors can occur.10 Because of the increased riskof malignant transformation and the difficultyinvolved with following these lesions clinically,incomplete excision by pathologic evaluationwarrants repeated excision until deep and lat-eral margins of resection are free of nevus cells.Guidelines for resection of giant congenitalnevi without malignant potential are indicatedon the basis of presentation of the nevus. In-flammatory linear verrucous epidermal nevi(Fig. 7) are intensely symptomatic lesions.11

The treatment strategy should focus on themost severely affected areas of skin, with morecomplete excision directed toward the aes-thetic needs of the individual patient. Woollyhair nevi of the scalp (Fig. 1) do not possess

FIG. 7. Preoperative appearance of a patient with diffuseinflammatory linear verrucous epidermal nevi involving theright knee (left) and the palmar surface of both upper ex-tremities (right).

FIG. 8. Giant congenital nevi algorithm: extent of resection. SQ, subcutaneous tissue.

Vol. 108, No. 3 / MANAGEMENT OF GIANT CONGENITAL NEVI 629

malignant potential, and the extent of excisionis dictated by aesthetic considerations.12

What Reconstructive Options Should Be Used afterResection of Giant Congenital Nevi, and When Is ItAppropriate to Combine Reconstructive Techniques?Do Optimal Reconstructive Methods Vary in DifferentAnatomic Regions?

On the basis of our experience, a proposedalgorithm for the reconstruction of giant con-genital nevi is shown in Figure 9. Reconstruc-

tive options include tissue-expanded flaps orgrafts, serial excision, and either full-thicknessor split-thickness skin grafts. Serial excision isthe preferred treatment modality for lesionsthat can be excised in two or fewer procedureswithout distorting adjacent structures. Becauseincreased morbidity is often associated with theuse of tissue expanders or skin grafts at or justproximal to the knee or elbow joints, serialexcision is also preferred for use in these areas.If more than three procedures are required to

FIG. 9. Giant congenital nevi algorithm: reconstructive options. *Consider immediate serialexpansion if lesion cannot be resurfaced in one stage. **Consider supplementing with split-thickness skin graft in less-visible areas if expanded full-thickness skin graft alone cannot coverdefect.

630 PLASTIC AND RECONSTRUCTIVE SURGERY, September 1, 2001

complete the resection, the former optionsshould be considered.

Tissue expansion is recommended for le-sions involving the head, neck, or torso, as thereconstructive outcome, particularly in thehead and neck, is far superior to that obtainedwith skin grafting. For large lesions requiringmore than one set of tissue expanders, a sub-sequent set of expanders can be inserted at thetime of the initial flap advancement. Althougha waiting period of 2 weeks is usual, we havedelayed filling the serially placed expanders foras long as 8 to 12 weeks in an effort to avoidsubluxation. Even if a filling delay is necessary,serial expansion is preferable, as it avoids theneed for a repeated operation for insertion oftissue expanders.

Skin grafts are recommended for lesions in-volving the extremities distal to the knee or el-bow joint. Tissue expansion in this context hasgreater morbidity with less potential gain becauseof the restricting circumference of skin envelope.Skin grafts are also preferred for reconstructionof the eyelids or ears. In these areas, the use ofexpanded flaps or serial excision results in distor-tion of involved structures. A full-thickness skingraft is always preferable to a split-thickness graft,because of both an improved aesthetic outcomeand less subsequent graft contracture. An ex-panded full-thickness graft should be consideredif donor skin limitations are problematic. Thebest outcome is often achieved through the useof multimodality therapy, particularly for lesionsinvolving the face or those involving contiguousanatomic regions. No biologic skin substituteswere used in the present algorithm, because skinsubstitutes are currently associated with a lesssatisfactory aesthetic outcome than that achievedwith skin grafts. Dermal allografts may be usefulin creating a thicker neodermis when a split-thickness graft is required. Microsurgical tech-niques may be useful if reconstruction of the faceas one aesthetic unit, as reported by Siebert andLongaker,13 is required.

Despite advances made in the treatment ofpatients with giant congenital nevi, inadequatedonor tissue for reconstruction may rendercomplete removal impossible. Complex lesionssuch as “bathing trunk nevi” involving the per-ineum, genitals, and perirectal area present acontinued surgical challenge. Resection ofthese problematic nevi should be limited toareas in which reconstruction can reasonablybe expected to provide a better aesthetic andfunctional outcome than the original lesion.

Leaving residual nevus in the genital and per-ineal regions results in a more predictable re-constructive outcome. Adjunctive treatmentsfor surgically unresectable portions of complexpigmented giant congenital nevi, such as phe-nol chemical peel, have been reported.14 Giantcongenital nevi are among the most challeng-ing problems faced by plastic surgeons. Athoughtful reconstructive approach to thesepatients may help to improve their treatmentoutcome in future years.

Arun K. Gosain, M.D.Department of Plastic and Reconstructive SurgeryMedical College of Wisconsin9200 West Wisconsin AvenueMilwaukee, Wis. [email protected]

REFERENCES

1. Quaba, A. A., and Wallace, A. F. The incidence of ma-lignant melanoma (0 to 15 years of age) arising in“large” congenital nevocellular nevi. Plast. Reconstr.Surg. 78: 174, 1986.

2. Kopf, A. W., Bart, R. S., and Hennessey, P. Congenitalnevocytic nevi and malignant melanomas. J. Am. Acad.Dermatol. 1: 123, 1979.

3. Pilney, F. T., Broadbent, T. R., and Woolf, R. M. Giantpigmented nevi of the face: Surgical management.Plast. Reconstr. Surg. 40: 469, 1967.

4. Rompel, R., Möser, M., and Petres, J. Dermabrasion ofcongenital nevocellular nevi: Experience in 215 pa-tients. Dermatology 194: 261, 1997.

5. Zitelli, J. A., Grant, M. G., Abell, E., et al. Histologicpatterns of congenital nevocytic nevi and implicationsfor treatment. J. Am. Acad. Dermatol. 11: 402, 1984.

6. Grevelink, J. M., Van Leeuwen, R. L., Anderson, R. R., etal. Clinical and histological responses of congenitalmelanocytic nevi after single treatment withQ-switched lasers. Arch. Dermatol. 133: 349, 1997.

7. Lorentzen, M., Pers, M., and Bretteville-Jensen, G. Theincidence of malignant transformation in giant pig-mented nevi. Scand. J. Plast. Reconstr. Surg. 11: 163, 1977.

8. Mark, G. J., Mihm, M. C., Liteplo, M. G., et al. Con-genital melanocytic nevi of the small and garmenttype: Clinical, histologic, and ultrastructural studies.Hum. Pathol. 4: 395, 1973.

9. Mehregan, A. H., and Pinkus, H. Life history of or-ganoid nevi. Arch. Dermatol. 91: 574, 1965.

10. Domingo, J., and Helwig, E. B. Malignant neoplasmsassociated with nevus sebaceus of Jadassohn. J. Am.Acad. Dermatol. 1: 545, 1979.

11. Morag, C., and Metzker, A. Inflammatory linear verru-cous epidermal nevus: Report of seven new cases andreview of the literature. Pediatr. Dermatol. 3: 15, 1985.

12. Reda, A. M., Rogers, R. S., III, and Peters, M. S. Woollyhair nevus. J. Am. Acad. Dermatol. 22: 377, 1990.

13. Siebert, J. W., and Longaker, M. T. Salvage reconstruc-tion of an extensive facial deformity due to congenitalgiant hairy nevus. Plast. Reconstr. Surg. 102: 2414, 1998.

14. Hopkins, J. D., Smith, A. W., and Jackson, I. T. Adjunc-tive treatment of congenital pigmented nevi with phe-nol chemical peel. Plast. Reconstr. Surg. 105: 1, 2000.

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