THE TREATMENT OF GIANT HAIRY PIGMENTED N~EVI OF THE FACE

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1 THE TREATMENT OF GIANT HAIRY PIGMENTED N~EVI OF THE FACE By JOHN MARQUIS CONVERSE, M.D., F.A.C.S., CARY L. GuY, M.D., F.A.C.S., and ANDRIES MOLENAAR, M.D. From the Institute of Reconstructive Plastic Surgery, New York University Medical Center, 560 First Avenue, New York, New York THE term " giant na:vus " is generally applied to a lesion covering most of the trunk, the so-called bathing-trunk na:vus, or to a large na:vus occupying a major surface area of an extremity (Conway, 1939; Williams, I954). We submit that, from a surgical standpoint, a n~evus covering the major portion of the face, although representing less than I per cent. of the body surface, should also be considered as a giant na:vus (Figs. I and 2). Pigmented nmvi occur in both sexes and usually are congenital. They may be large, deeply pigmented, covered with coarse dark hair, or they may be small, inconspicuous growths at birth and attain these features as the child develops. A hereditary tendency has been suggested especially in those cases in which the pigmented lesions are particularly numerous (Figi, I943). It has also been observed that many of the fiat, smooth, hairless, pigmented mevi common over the trunk appear after birth (Figi, 1943). There are reports of malignant changes in giant nmvi of the bathing-trunk type as well as in the isolated facial type. A review of the literature on malignant change in giant pigmented na:vi was recently published by Greeley, Middleton and Curtin (1965). These authors concluded that treatment should not be considered for cosmetic reasons alone, but also to forestall malignant degeneration. Malignant changes in localised pigmented hairy mevi of the face have been reported. Truax and Page (1953) reported the case of a 2½-year-old girl with a nmvus covered with hair which differed from the surrounding normal hair. No growth of this lesion, which was present at birth, was observed. The parents requested that it be removed for cosmetic reasons. An excisional biopsy was performed and the pathologist reported a benign pigmented na:vus. Five months later a recurrent pigmented lesion was noted which grew rapidly to a size of about 2 cm. This lesion was excised ; the pathology report now was a malignant melanoma. Wide excision of the area with bilateral posterior triangle neck dissection was performed. The patient had recurrent masses in the right posterior neck and radical resection of the area was done. The child died within one year after the first operation. McWhorter and Woolner (1954) reported a case of an 8-year-old boy with a large hairy pigmented mole on the right cheek and auricle present since birth. Carbon dioxide snow was applied at the age of 4 years. A small retromandibular mass was noted three years later. At the age of 8 years the growing mass was excised and the pathology report revealed that it was a malignant melanoma. Excision of the lesion and a radical neck dissection was done. The child died shortly following this procedure with generalised metastases. It could be concluded that besides the cosmetic indications for removal of a hairy na:vus of the face, the possibility of malignant change in these hairy nmvi even prior to puberty should be borne in mind. Methods of Treatment.--Various methods of treatment have been advocated. The ideal is complete excision of the lesion and primary closure : in an extensive lesion this cannot be accomplished. 302

GIANT HAIRY PIGMENTED N~VI OF THE FACE 303 A C D FIG. I A, Giant hairy nmvus of the face covering approximately one-half of the facial area in an 8-month-old baby girl.

C, Appearance after further advancement-rotation of the cervical skin. D, Final result. The patient is now i6 years of age.

2 GIANT HAIRY PIGMENTED N~VI OF THE FACE 303 A C D FIG. I A, Giant hairy nmvus of the face covering approximately one-half of the facial area in an 8-month-old baby girl. B, Appearance after split-thickness grafting of the forehead and of the upper eyelid and rotation-advancement o the cervical skin in two stages. C, Appearance after further advancement-rotation of the cervical skin. D, Final result. The patient is now i6 years of age. The nose has been resurfaced by a median forehead flap, the lower eyelid by split-thickness graft and the eyebrow has been excised. The patient uses an eyebrow pencil to simulate the eyebrow. Gillies treated two patients with hairy n~evi of one side of the face- in which he employed a forehead flap to cover the defect of the cheek ; he repaired the forehead defect by means of a tubed pedicleflap from the abdomen. In another case he employed a forehead flap as a bipedicle visor flap to replace the skin of the centre of a little girl's face after excision of the nmx~us. The nmvus was also resected from the upper lip and the defect covered by means of a split thickness graft (Gillies and Millard, I957). Conway and Jerome (1954) treated two patients with hairy n~evi of the face by means

Block and Conway (1963) described the case of a xo-year-old girl with a hairy nmvus covering 6o per cent.

3 304 BRITISH JOURNAL OF PLASTIC SURGERY A of excision and subsequent application of split thickness skin grafts. Serial excisions of the skin graft with advancement of local flaps were planned for a" later stage. Block and Conway (1963) described the case of a xo-year-old girl with a hairy nmvus covering 6o per cent. of the body surface ; only the central portion of the face, portions of the thighs, legs and buttocks were unaffected. Stage excisions were undertaken in the face followed by split-thickness skin grafting; full-thickness skin grafts were utilised in the lower eyelids. Additional small nmvi were excised and the wounds closed primarily. Electrolysis had reduced the residual hair on the face. Pilney, Broadbent and Woolf (1967) described their treatment of three patients with giant pigmented nmvi of the face. Early complete excision or replacement with a single sheet of medium thickness skin graft was performed. Skin was obtained from the chest and abdomen. Hynes (1956) mentioned the disadvantages of both techniques : excision and skin grafting and direct closure or serial excision with advancement flaps. According to Hynes, the disadvantages of skin grafting are the length of the operative procedure and also the danger of hmmatoma and consequent residual scarring. He also cited the disadvantages of direct closure or serial excision with advancement flaps: the large number of repeated major procedures and the resulting secondary scarring. As an alternative, Hynes advocated shaving the nmvus deep into the dermis and overgrafting the area with split thickness skin grafts. This technique, however, cannot be employed in hairy nmvi because of the recurrence of the hair growth (McDowell, 1958), which is somewhat diminished but nevertheless troublesome. Despite this complication, Hynes considered the procedure worth doing in patients with extensive lesions in whom complete excision is difficult or unjustified. Cronin (1953) was of the opinion that multiple excisions are indicated in hairy nmvi of the face. For the removal of pigment without destroying hair follicles, for instance, in eyebrows, scalp, he advocated electro-coagulation. He also used the method of shaving to remove pigment while preserving the hair follicles. Cronin discussed the question as to whether the trauma of partial excision could cause the malignant changes to a nmvus and concluded that surgery could be undertaken" without fear of malignant

A portion of the facial area has been replaced by an advancement-rotation flap from the neck. D, Appearance after further advancement rotation of the cervical flap.

4 GIANT HAIRY PIGMENTED N2~VI OF THE FACE 305 C D E FIG. 2 C, Appearance of the patient after excision of the nmvus over the forehead and skin replacement by a split-thickness graft. A portion of the facial area has been replaced by an advancement-rotation flap from the neck. D, Appearance after further advancement rotation of the cervical flap. The skin of the anterior portion of the face. has been advanced over the cheek and nose. E, Final appearance of the patient at the age of 5 years. The skin of the eyelids has been replaced by a split-thickness skin graft. The infra-orbital area has been covered by a thick, split-thickness graft. F, Lateral view of patient at the completion of treatment.

306 BRITISH JOURNAL OF PLASTIC SURGERY change on the intradermal na~vi at any age, but in the case of iunctional or compound na~vi, its use should be restricted to children below the age of puberty"

5 306 BRITISH JOURNAL OF PLASTIC SURGERY change on the intradermal na~vi at any age, but in the case of iunctional or compound na~vi, its use should be restricted to children below the age of puberty" Timing of the Repair.--There are several reasons to start reconstruction of a pigmented nvevus of the face at an early age. (I) Facial disfigurement in a child can have serious psychological consequences. One of our patients was known in the neighbourhood as the " dog-face baby" and her mother became reluctant to leave her home with the baby. (2) Surgical intervention within a pigmented nmvus carries less danger of malignant change prior to puberty (Shaw, 1962). (3) In the very young, the skin is highly stretchable and progressive advancement of surrounding skin is feasible. After each stage the stretched skin appears progressively to loose its tenseness. Pilney, Broadbent and Woolf (1967) advocate treatment before the age of 18 months when a full dermatome drum of skin graft wiu cover most of the defects resulting from excision of the lesion. Choice of the Technique of Repair.--Good skin colour matching is very important. For this reason, skin as close to the defect as possible should be utilised. Advancement flaps from the neck, full-thickness retroauricular or supraclavicular skin grafts, or flaps from the forehead are preferable to abdominal or extremity skin. The final solution to the problem of the giant n~evus of the face appears to be a choice between two methods of treatment: massive excision and skin grafting or repeated partial excision and advancement or rotation of adjacent cervical skin. In certain areas of the face skin grafting is the method of choice the forehead, the eyelids, the nose, the upper lip and to a certain extent the lower lip. This method involves staged segmental excision and skin grafting of the forehead and upper eyelid by means of split thickness skin grafts ; of the lower eyelid, upper lip and lower lip preferably by full thickness skin grafts from the retroauricular or supraclavicular areas if available. The nasal region may also be covered successfully with a full-thickness skin graft ; a median forehead flap was employed in one of our patients (Fig. 3). In the remainder of the cheek area, a soft and mobile portion of the face, skin grafting has not, in our hands, been uniformly successful in growing children. For this reason we prefer staged excision and repeated advancement-rotation of cervical skin, an eminently feasible procedure because of the elasticity and extensibility of infant skin. This extensibility may be explained by the fact that the collagen and elastic fibre networks (Gibson and Kenedi, I967) in the infant and young child are capable of being stretched accordionfashion to a far greater degree than in the adolescent and young adult. After a period of six months to one year the stretched skin shows a marked degree of relaxation which permits a further advancement in subsequent repeated stages. The extensibility of infantile skin is an important factor in deciding upon early surgical intervention in a giant n~evus and performing the first cervical advancement or rotation flap during the first postnatal year. Two additional advantages are offered in the technique of advancement-rotation of the cervical skin : (I) subcutaneous tissue underlying the flap appears to be necessary to resurface the cheek and provide adequate "padding" ; (2) in male patients the hairbearing portion of the cervical skin can be transferred to the cheek, in favourable cases. Advancement-rotation of cervical skin may be extended progressively to reach the medial canthal area (Fig. I, B). Such a degree of advancement causes some distortion at the angle of the mouth which requires secondary adjustment. It may be preferable to limit the advancement of the flap to the cheek area ; the infra-orbital area may be covered by a full thickness or a thick split thickness skin graft (Fig. 2). Small pigmented na:vi distributed throughout the skin require excision and primary

6 (;IANT IlAIRY PIGMENTED N.'~.Vi OF THI-." FACE 307 closure of the resulting small defect. Scalp lesions, well covered by hair, are left undisturbed. Special mention must be made of the management of n~evi invading the conjunctival A " ~. Pzgmented 'w"-~~ ~"1 hairy.-..,.=. ~nevus t.';~'~ :.. ;.k, i t:,,,',','::~k.:: ":,~,--".":.?~ ", ~.4~..;:..~. : -.,,- ' ' ---,~ '~7' ~- ' ;;,; ~" " ~... %'t;.j,:.:.: : Split graft used to cover forehead area ~ ",.. ~ r ~.:~: ~ ~k~ ) f. #, ~,"-~'-.c Advancement flop to cover cheek area I '...k I g.&-, $! ":v~ ~ ~-,L- Z, '1. Further ) " advancement of cheek and neck skin t L Lid skin. i "~ ':~" replaced,,.. by split "'-... graft }:It;. 3 Diagrammatic representation of various stages in the treatment of the patient shown in Figure t. sac and those of the eyebrow. The irregularity and roughness of the na~vus on the eyelid margin and inner aspect of the eyelid causes corneal irritation and may lead to ulceration. Repeated shaving of the nxvus has been an adequate form of treatment and has obviated

7 308 BRITISH JOURNAL OF PLASTIC SURGERY the need for conjunctival replacement by a graft. The procedure is performed by means of a piece of razor blade under visual magnification. It was found preferable to excise the eyebrow on the affected side rather than attempt to leave a strip of eyebrow containing pigmented tissue (Fig. I, D). In conclusion, it is advocated that an eclectic approach be adopted in the treatment FIG. 3 of giant hairy n~evi of the face. Certain areas such as the forehead, the eyelids, the lips and the nose may be satisfactorily covered after excision of the n~evus by skin grafts. The cheek area appears to be more acceptable in appearance when an advancement-rotation cervical flap is employed. SUMMARY After a review of the incidence of malignant change in nmvi, serial excision of giant nmvi of the face employing an advancement-rotation flap from the neck and skin grafts for the remaining facial areas is advocated. The results obtained by this technique are demonstrated in two cases. REFERENCES BLOCK, L. J. and CONWAY, H. (1963). Plastic reconstr. Surg. 31,472. CONWAY, H. (1939). Surgery, St. Louis, I6, 585. CONWAY, S. and JEROME, A. J. (1954). Plastic reconstr. Surg. 14, 200. CRONIN, T. I). (1953)- Plastic reconstr. Surg. IX, 94. FIGI, F. A. (1943). Surg. Clins N. Am. 23, lo59. GIBSON, T. and KENEDI, R. M. (1967). Surg. Clins N. Am. 47, 279. GILLIES, H. D. and MILLARD, D. R. (1957). " The Principles and Art of Plastic Surgery." Boston : Little, Brown & Co. GREELEY, P. W., MIDDLETON, A. G. and CURTIN, J. W. (1965). Plastic reconstr. Sug. 36, 26. HYI~S, W. (I956). Br. J. plast. Surg., 9, 47. McDOWELL, A. G. (1958). Plastic reconstr. Surg. 21, 487. MCWHORTER, H. E. and WOOLNER, L. B. (1954). Cancer, N.Y., 7, 564. PILNEY, F. T., BROAI)BENT, T. R. and WOOLE, R. M. (1967). Plastic reconstr. Surg. 40, 469.` SI-IAW, M. H. (1962). Br. J. plast. Surg. 15, 426. ': TRUAX, K. F. and PAGE, H. G. (1953). Ann. Surg. I37, 255. WILLIAMS, H. F. (I954). Cancer, N.Y. 7, 163.

cally, a distinct superior crease of the forehead marks this spot. The hairline and

cally, a distinct superior crease of the forehead marks this spot. The hairline and 4 Forehead The anatomical boundaries of the forehead unit are the natural hairline (in patients without alopecia), the zygomatic arch, the lower border of the eyebrows, and the nasal root (Fig. 4.1). The