Keywords
facial reconstruction - melanocytic nevus - pediatric surgery
Introduction
A congenital melanocytic nevus (CMN) is a dermatological phenomenon characterized
by melanocytic, hairy, noncancerous patches of skin, present at birth or developing
shortly thereafter.[1] CMNs are classified according to the size that they are predicted to reach in adulthood
(projected adult size [PAS]). Giant CMNs (GCMNs; >40 cm PAS) occur in approximately
1/500,000 newborns worldwide.[2] GCMNs sometimes occur along with multiple smaller melanocytic nevi, called “satellite
nevi.”[1]
[2]
While typically benign in nature, GCMNs harbor a risk of malignant transformation
into melanoma.[3] The risk of melanoma appears proportional to the lesion size, reaching up to 10
to 15% risk of a lesion greater than 40 cm in diameter. These patients are also recommended
to undergo MRI screening for deposits of melanocytic cells along the leptomeninges,
called neural melanosis, which can be asymptomatic or present with seizures or developmental
delay.[2]
[3]
[4]
We present the case of a 6-month-old infant with a GCMN of more than 40 cm PAS, which
covered over 50% of the face. The purpose of this case report is to describe and assess
our treatment outcomes as well as highlight the advantages of a multistep surgical
approach compared to other management strategies.
Case Report
A 6-month-old infant was presented to a plastic surgery clinic with a dark and hirsute
GCMN covering approximately 50% of the face, including the entire left cheek, left
eyelid, supra- and infraorbital region, nasal bridge, and forehead. “Satellite nevi”
were recognized on the patient's trunk and both upper and lower limbs ([Fig. 1]). No other anomalies were detected. The patient did not undergo genetic testing
for possible mutations. During the initial visit, a multistep surgical approach was
planned ([Fig. 2]).
Fig. 1 Pictures taken during an initial visit when the patient was 8 months old. (A) Facial giant congenital melanocytic nevus (GCMN). (B) Satellite nevi on lower limbs.
Fig. 2 Timeline presenting the surgeries performed.
The first surgery took place when the patient was 11 months old, beginning with a
partial excision of the nevus from the part of the left supra- and infraorbital area,
nasal bridge, and a fragment of the left cheek. The tissue of the nevus was sent out
for a histopathological evaluation. A full-thickness skin graft (FTSG) was taken from
the left arm and transplanted onto that area to cover the tissue deficiency. The left
arm was closed primarily.
In all the surgeries, the inner part of the arm was consistently chosen as the donor
site for harvesting FTSGs ([Fig. 3]). FTSGs were harvested twice from the left inner arm and once from the right inner
arm. The largest graft measured 10 × 5.5 cm. To achieve optimal aesthetic outcomes
at the donor sites, a wide dissection of the adipocutaneous flaps was performed to
facilitate primary closure. Postoperative scar management included the use of wound
closure strips, which effectively approximated the wound edges, contributing to cosmetically
favorable results.
Fig. 3 (A) A portion of the nevus was surgically removed from the nose, supraorbital and infraorbital
areas, and a fragment of the left cheek. A full-thickness skin graft (FTSG) was taken
from the left arm and transplanted onto the face. The left arm was then closed primarily.
(B) Healed area from which FTSGs were being harvested.
The second surgery occurred 4 weeks later, encompassing further excision of the nevus
from the left cheek. The technique for this surgery was a cervicofascial flap advanced
from the mandibular region, where a cutaneous flap was elevated from the platysma
and moved in the superomedial direction to cover the skin deficiency. In order to
avoid tension, the medial part of the cheek was reconstructed with an FTSG harvested
from the right arm, which was closed primarily ([Fig. 4]).
Fig. 4 (A) Further resection of the nevus from the left cheek. Cervicofascial flap was advanced
from the mandibular region, where a cutaneous flap was elevated from the platysma
and moved in the superomedial direction to cover the skin deficiency. The medial part
of the cheek was reconstructed with a full-thickness skin graft (FTSG) harvested from
the right arm. (B) Healed operated area.
Four months later, another resection was done to address the nevus on the forehead,
left temporal region, left lower eyelid, and right cheek. An FTSG taken from the left
arm was engrafted into the mentioned areas.
At the age of 3 years, a surgical procedure targeted the nevus within the left lower
and upper eyelids, coupled with a reconstruction, that used an FTSG from the left
arm. Central temporary tarsorrhaphy was done to ensure proper healing. Resection of
the remaining nevi within the margins of the postoperative scars was executed ([Fig. 5]).
Fig. 5 (A) Resection of the remaining nevi within the margins of the postoperative scars was
executed. Central temporary tarsorrhaphy was done to ensure proper healing of the
eyelids. (B) Results after almost a year after surgery.
Reconstruction of the left lower eyelid with a cartilage from the left auricle was
done 1.5 years later with a lowering of the palpebral margin. The auricular cartilage
was used to reconstruct the tarsal plate of the lower eyelid. Skin defects resulting
from the nevus excision were covered with an FTSG from the area posterior to the right
auricle ([Fig. 6]).
Fig. 6 Reconstruction of the left lower eyelid with a cartilage harvested from the left
auricle with excision of the remaining nevi within the margins of the scars.
This patient remains under the plastic surgeon's observation. In the resected areas,
no signs of reappearance of the nevus were detected; therefore, it was deemed successful.
Genetic screening for NRAS and BRAF mutations was advised ([Fig. 7]).
Fig. 7 Most recent pictures taken of our patient.
The overall quality of life and functional and aesthetic outcomes were evaluated 2
years after the last surgery. We used the Pediatric Quality of Life Inventory (PedsQL)
parent and patient report for young children aged 5 to 7 years, which is a subjective
questionnaire filled in by parents and their child separately. Total scores of 84.4
for the parent report and 83.1 for child report were achieved, which are considered
highly satisfactory and mean a high health-related quality of life (HRQL). [Table 1] depicts the results.
Table 1
Results of the Pediatric Quality of Life Inventory Generic Core for young children
aged 5 to 7 years questionnaire
|
Dimensions
|
Number of items
|
Score for parent report
|
Score for child report
|
|
Physical functioning
|
8
|
87.5
|
87.5
|
|
Emotional functioning
|
5
|
90
|
90
|
|
Social functioning
|
5
|
80
|
80
|
|
School functioning
|
5/6
|
80
|
75
|
|
Total
|
23
|
84.4
|
83.1
|
Note: The questionnaire evaluated four dimensions of the patient's life in the past
month. Scores are presented on the scale of 0 to 100. The higher the score, the better
the health-related quality of life.
Discussion
This study describes a technique for management of a facial GCMN with the use of a
fascio-cervical flap, serial excision, and multiple FTSGs harvested from an inner
part of the arm. Surgical excision of a GCMN requires careful consideration of several
factors, including malignancy risk, cosmetic outcomes, psychological impact, and the
risks associated with repeated general anesthesia.
The inner part of the arm serves as an optimal donor site for FTSGs due to small skin
tensioning, substantial subcutaneous adipose layer that enables primary closure, and
very small density of hair follicles that tend to be lighter in color and similar
shade to the skin of the patients face. Moreover, this body area is usually hidden
and is less vulnerable to sun exposure, which is important regarding the scarring
process. Skin in that region is also thinner, so when harvested properly it does not
require additional thinning, which reduces the operative time.[5]
Our case report details a novel surgical approach for this type of lesion. The uniqueness
of our case lies in the novel approach to this type of reconstruction. In this patient,
we did not use tissue expanders and a large part of the tissue defect was covered
by advancing a fasciocutaneous cervicofacial flap. Moreover, it is unique because
the patient was 1 year old at the time of the surgery. The uniqueness of our case
is the lesion itself as it is very rare for a child to be born with a GCMN covering
over 40 cm of their face. A nonstandard method of treating the donor site scar (with
consistent tight taping with wound closure strips, placed under tension to bring the
ridges of the scar together) allowed for the harvesting of another large FTSG from
the same site just 6 months later.
Our case report presents a novel surgical approach to addressing this type of lesion.
The uniqueness of this case lies in the employed reconstruction method. Notably, tissue
expanders were not used; instead, a significant portion of the tissue defect was covered
by advancing a fasciocutaneous cervicofacial flap. Additionally, the surgery was performed
on a 1-year-old child. The rarity of the lesion itself further underscores the distinctiveness
of this report. Furthermore, a nonstandard method of managing the donor site scar—utilizing
consistent, tight taping with wound closure strips applied under tension to approximate
the scar edges—enabled the successful harvesting of another large FTSG from the same
site just 6 months later.
Cheng et al[1] discovered that surgical excision led to shorter postoperative hospitalization,
quicker recovery, and lower number of complications in comparison with tissue expanding
or skin grafting. Tissue expanders were associated with higher psychological burden,
lasting up to 6 months, and increased pain, frequent office visits, and functional
disability in children.[1] Rotation or transposition of expanded flaps creates longer and more visible scars
when compared to linear scars from serial excision.[6]
[7] In our case, a single-step tissue expansion or serial excision without skin grafting
cannot address the entire defect.[8] Kim et al,[6] in a review of 88 medical records, compared single and serial expansion techniques
for such lesions and concluded that infection and revisional operation rates were
significantly higher in the serial expansion groups.
While the literature underscores the advantages of an early surgical intervention,
consensus regarding the optimal age for excision remains a subject of ongoing debate.[9] We began treatment at a very young age, because of the psychological burden of a
GCMN that was distorting the child's appearance and also affected the parent's well-being.
As proper psychosocial functioning is at high stake, several different studies point
toward the importance of cosmetic outcomes. Koot et al[10] measured the psychosocial functioning of 29 children affected by GCMNs. Thirty percent
of them scored under the 5th percentile, particularly in social competence. The children
used to avoid situations when they would have to undress or uncover the affected body
parts and 70% of mothers reported that they found it grueling and hard to accept having
a child with a GCMN.[4] In this case report, scores of 84.4 for the parent report and 83.1 for the child
report were achieved using the PedsQL. Our results are satisfactory in comparison
to the literature, especially considering that our patient struggled with a facial
GCMN.
Another reason behind early intervention is the risk of developing melanoma. Scard
et al,[11] in their systematic review on the risk of melanoma in melanocytic nevi of all sizes,
report that the median age of melanoma onset is 22 years for patients with small-
and medium-sized CMNs, compared to 9.5 years for those with large CMNs or GCMNs. Thus,
treatment aims at lowering the risk of metastasis in the future. The incidence of
melanoma after 15 years was 0.132% in patients who received partial treatment (partial
surgery, laser, dermabrasion, curettage, or peelings), while no patients who underwent
complete excision developed melanoma within the 15-year follow-up period.[11]
The limitations of our treatment lie in the unknown etiology of the nevus, due to
the absence of the genetic testing provided by the patient. Regrowth of the nevus
around the palpebral margin was observed, which was why the patient was qualified
for another surgery. Fortunately, no signs of regrowth were observed in other excised
areas even after almost 7 years since surgery.
Conclusion
In conclusion, while FTSGs rank lower than tissue expansion on the reconstructive
ladder, this case highlights their successful use, when combined with local tissue
transfer for the complete excision of a GCMN in challenging areas such as the face.
To our knowledge, this is the first report of this technique with a follow-up and
quality of life assessment. Wide suprafascial dissection of the arm tissues allows
for the harvesting of exceptionally large skin grafts with primary wound closure.
Selecting the inner arm as the donor site seems to be a promising solution for achieving
good aesthetic results.
