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Curettage of Giant Congenital Melanocytic Nevi in Neonates
A Decade Later
Linda E. De Raeve, MD;
Diane I. Roseeuw, MD, PhD
Arch Dermatol. 2002;138:943-947.
ABSTRACT
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Background Currently, there is tremendous uncertainty regarding how giant congenital
melanocytic nevi (GCMN) should be treated. Our approach to patients with GCMN
is based on 2 main considerations: (1) obtain an acceptable cosmetic result
to decrease the psychosocial inconvenience to the patient, and (2) attempt
to minimize the risk of malignancy. For the past 10 years we have treated
GCMN by curettage in the neonatal period. We report our experience and results.
Observations Sixteen neonates with GCMN were treated by curettage between 1990 and
2000. Biopsy specimens were obtained and the patients received close clinical
follow-up. In most patients cosmetic and functional results were good, and,
to date, no melanoma has been observed in this series.
Conclusions Curettage offers an adequate alternative to surgical excision when performed
during the first 2 weeks of life. Patients and parents are pleased with the
cosmetic and functional results and thereby suffer less from the psychosocial
inconvenience caused by these lesions. Careful long-term follow-up of these
children is essential to monitor final cosmetic outcome and reduce the potential
for malignancy.
INTRODUCTION
GIANT CONGENITAL melanocytic nevi (GCMN) are disfiguring potentially
malignant lesions present at birth.1-3
Most authorities agree to remove these nevi whenever possible and as early
as possible.4-5 Unfortunately,
these lesions are mostly too large to be removed by classic surgery. Partial
removal of GCMN by debulking the superficial pigmented nevus cells by curettage,
first described by Moss,6 is an alternative
to excision surgery and produces good cosmetic results. We have used this
technique for the past 10 years in the treatment of neonates with GCMN in
the first weeks of life. We report our experience herein.
PATIENTS AND METHODS
During a 10-year period starting in 1990, we treated 16 neonates (9
girls and 7 boys) with GCMN by curettage. The GCMN were located on the scalp
in 3 patients, on the face in 1, on the trunk in 7, on the upper extremities
in 3, and on the lower extremities in 3. Most of the GCMN were dark brown
in color and hairy, and 9 of our patients also had satellite lesions. Before
starting this procedure, information on curettage, other possible treatments,
and malignancy risk was given to the parents. Curettage was performed in the
first weeks of life under general anesthesia as a 1-stage procedure in all
neonates. Prior to or during curettage, biopsy specimens were obtained for
histopathologic evaluation.
We scraped the GCMN with a sharp curette from the center to the periphery
of the nevus. The curettage procedure was completed in 30 to 120 minutes.
The earlier in life the curettage was performed the easier it was to find
the cleavage plane. Bleeding was controlled by simple compression with sterile
gauzes.
Immediately after curettage, dressings with dextranomer wound paste
or alginate wound dressings were applied. These dressings were changed once
daily during the first 2 days or twice daily if needed. Thereafter, petrolatum
on nonadherent dressings were applied on the treated area, and these dressings
were changed daily for 10 to 14 days until entire reepithelialization was
completed.
Patients were followed up monthly for the first 3 months, then every
3 months for up to 1 year, and every 6 months thereafter . Follow-up visits
included a total body skin examination to assess the cosmetic outcome and
to detect any evidence of malignant change within the nevus or somewhere else.
Complete photographic documentation was obtained for all patients.
RESULTS
All neonates tolerated the intervention well, and no major complications
occurred during hospitalization. In 2 patients infection with Staphylococcus aureus was managed with systemic antibiotics. The wounds
following curettage healed quickly with minimal scarring. The immediate follow-up
results of all patients were impressive (Figure 1). The treated regions mostly remained pale for the first
year (Figure 2A and B). Thereafter,
a slight repigmentation was observed in most cases (Figure 2C), except in 2 in which no repigmentation took place (Figure 3). This occurred in cases in which
there was no hair growth. Repigmentation was most obvious in cases with more
notable hypertrichosis.
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Figure 1. A, Giant congenital melanocytic
nevus on the trunk; curettage performed at day 1. B, Healing takes place rapidly;
result after 2 weeks.
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Figure 2. A, Giant congenital melanocytic
nevus on the back. B, Result at age 5 months. C, Result at age 17 months showing
some repigmentation and hypertrichosis.
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Figure 3. A, Giant congenital melanocytic
nevus on the arm. B, Cosmetic result 5 years later.
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In 2 patients hypertrophic scarring occurred (Table 1). One case was on the scalp and resulted in cicatricial
alopecia (patient 2). This might be owing to the fact that on the scalp, it
is more difficult to find the cleavage plane, and curettage may be too deep.
In patient 12 there was some delayed wound healing, and we suspect this might
have been the cause of hypertrophic scarring in this patient. In most of the
patients, some hypertrichosis developed later in life, starting at age 1 year.
This was especially true in 1 patient who had GCMN extending from the scalp
to the face.
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Neonatal Curettage Results in 16 Patients*
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The histologic features of the nevi prior to curettage showed that the
upper dermis was entirely occupied by heavily pigmented nevus cells whereas
the lower dermis contained a less dense diffuse infiltration of nonpigmented
nevus cells. The curetted material consisted of epidermis with underlying
upper dermis.
Positive HMB-45 staining results from the superficially located dermal
pigmented nevus cells and negative staining results from the deeper dermal
nonpigmented nevus cells demonstrate that the biological behavior of these
2 populations of nevus cells seems to be different. In addition, they are
different in their expression of melanocytic antigen.
Follow-up biopsy findings showed that the entire upper dermis was composed
of a dense connective tissue with some degree of sclerosis, but the heavily
pigmented nevus cells were no longer seen. The HMB-45 staining results were
negative. The deeper dermis still contained a diffuse infiltration of nonpigmented
nevus cells as observed prior to curettage but did not have pigmented nevus
cells; there was no immunoreactivity for HMB-45.7-8
As it was more difficult to find the cleavage plane at the border of
the lesion, we also performed a histopathologic examination comparing the
center of the nevus with the border of the nevus; except for a less dense
infiltration of nevus cells at the border, we did not find any significant
difference between these 2 sites.
To date, no malignant melanoma was observed in any of our patients during
this follow-up period, and patients and parents were pleased with the cosmetic
result.
We performed magnetic resonance imaging (MRI) studies in 1 patient (patient
12) as this boy had some developmental delay, but the MRI findings were normal.
In the other patients, we did not perform MRI studies as the role of MRI in
the routine evaluation of the patients remains to be determined.9
We recommend clinical follow-up by the neuropediatrician and that MRI studies
are performed only in those individuals with neurologic signs or symptoms.
COMMENT
Treating a child with GCMN is a challenge. To date, no absolute guidelines
to treat these nevi have been given to our knowledge, and therefore, this
subject remains one of the most controversial issues in pediatric dermatology.1, 10-11 The first problem
these lesions pose is that they have an increased malignancy risk, estimated
to be around 5%2-4,12-13;
50% of malignant degeneration in these nevi occurs before puberty and most
often in the first years of life. Additional reasons to remove these lesions
early is that they are disfiguring lesions that can be an aesthetic tragedy
and contribute to severe psychological sequelae. However, the desired end
point of treating GCMN still is unclear. Do we have to completely remove GCMN,
or should we aim to remove most melanocytes with a more acceptable cosmetic
result? Complete removal of these lesions is usually difficult and sometimes
impossible without functional or cosmetic mutilation. Moreover, even after
complete excision of GCMN down to the fascia, the malignancy risk is not completely
eradicated as malignant melanoma can occur at extracutaneous sites.14
Advances in the treatment of GCMN have been made by using techniques
such as tissue expansion,15 cultured epithelial
autografts or allografts,16 and dermabrasion.17 However, the most promising results of treating GCMN
were originally reported by Moss6 in 1987.
He performed curettage on GCMN during the first weeks of life to remove the
superficially distributed nevus cells based on the fact that at that time,
there seems to be a cleavage plane between the upper and the lower dermis.
This technique offers a good alternative to classic surgery when the nevi
are too large to perform complete excision, and it is a technique that is
of benefit for these children.7
From our experience we want to stress 2 limitations. First, the curettage
has to be performed during the first 2 weeks of life as this is the time when
it is easy to find the cleavage plane. It becomes progressively more difficult
later on, and cosmetic results are not as good. In 1 newborn in our series
who was born prematurely, curettage was performed at day 1. The cleavage plane
was the easiest to find in this neonate, and the skin came off easily. Therefore,
we advise using this technique as early as possible, preferably during the
first 2 weeks of life. We do not yet know why this cleavage plane is only
found in the first weeks of life. It is present in the center of the nevus
but is less clearly present at the margin of the lesion, and it disappears
after some weeks. This could be explained by the fact that neonatal skin is
fragile and that many nevus cells are present in these lesions. It is not
yet known whether neonatal skin is fragile because of an immaturity of a component
of the basement membrane or because the interdigitation of the rete ridges
is qualitatively reduced. As this cleavage plane is not present in healthy
neonatal skin and is less clear at the border of GCMN, it is possible that
the bulk of nevus cells in the superficial dermis disrupts the normal dermal
components such as collagen and elastin and therefore plays a role in this
cleavage plane. Our histopathologic study findings indeed showed a less dense
infiltration of nevus cells at the border of GCMN compared with the center
of the lesion. The confluence of melanocytes in the dermoepidermal junction
may also weaken the connection of the basement membrane zone. The same biologic
traits that lead to this cleavage plane might be what predisposes these lesions
to ulceration as described by Giam et al.18
The second limitation of the technique is the need of a specialized pediatric
anesthetist and use of the intensive care unit.
The advantages of the technique are numerous. It is simple and does
not necessitate complicated tools; a relatively atraumatic procedure with
minimal blood loss; and a 1-stage procedure that is well tolerated by the
neonate. Healing takes place in 10 to 14 days, and cosmetic results are good.
Moreover, an important reduction in the number of heavily pigmented nevus
cells occurs. Whether the risk of malignancy is thus reduced is still uncertain,
but it could be lessened by a reduction in the total number of these biologically
different superficial nevus cells.
These patients can still develop melanoma in other cutaneous or extracutaneous
sites.14, 19 The GCMN can be associated
with leptomeningeal melanocytosis, and in a series reported by Marghoob et
al,4 a melanoma in one of these patients was
reported in the retroperitoneum. Rhodes et al20
stated that melanomas arising within GCMN may also arise from the deep component,
and there is concern that it might be more difficult to detect this after
curettage.5 We believe that it is easier to
detect eventual malignant growth in GCMN treated with curettage as this treated
area is less pigmented and changes can be observed more easily. We stress
the importance of regular, long-term follow-up for these patients.
After reporting our first results in 1996,7
2 other groups also described satisfactory cosmetic results with this technique.21-22 Based on our 10-year experience and
follow-up of 16 patients, functional results after curettage of GCMN in the
neonate are excellent and cosmetic outcome is favorable. The reduction in
pigmentation is satisfactory; in most patients curettage results in soft pale
scars occasionally with some repigmentation. This reduction in pigmentation
facilitates clinical follow-up. In some patients secondary hair growth may
occur. Cosmetic results therefore seem to decrease slightly with time, and
this should be explained to parents.
In conclusion, this technique has its place in the treatment of GCMN
as cosmetic and functional results are usually better than those obtained
by other surgical techniques. However, in some areas, such as the scalp, we
do not recommend this technique, and tissue expanders might be a better solution
since curettage in this location may cause cicatricial alopecia. On the other
hand, for some localizations, such as GCMN occurring circumferentially around
a limb or other areas where GCMN are difficult to excise, curettage may be
the only possible treatment. We therefore consider treatment for each patient
individually after weighing the risks and benefits of other possible treatments
and considering the parents' wishes. Whether malignancy risk is reduced by
curettage is still a point of discussion and needs further investigation.
AUTHOR INFORMATION
Accepted for publication October 31, 2001.
Corresponding author and reprints: Linda E. De Raeve, MD, Department
of Dermatology, Academisch Ziekenhuis Vrije Universiteit Brussel, Laarbeeklaan
101, B-1090 Brussels, Belgium (e-mail: linda.deraeve{at}az.vub.ac.be).
From the Department of Dermatology, Academic Hospital Vrije Universiteit
Brussel, Brussels, Belgium.
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