Title: Surgical management of the cutaneous manifestations of linear nevus
sebaceus syndrome.
Authors: Alexander Margulis, MD, Bruce S. Bauer, MD, and Julia F. Corcoran, MD.
Linear nevus sebaceus syndrome (LNSS) is characterized by the association of
linear nevus sebaceus with abnormalities of the central nervous system, eyes and
bones, other cutaneous alterations and malignancies (1-3,16,17). The plaques of
nevus sebaceus are hairless and verrucous, and the color ranges from yellow-orange
in white individuals to hyperpigmented in black patients.
Typically, the scalp
and the face are severely involved. Patients with nevus sebaceous, whether
associated with LNSS or solitary, have a 10 to 20 percent risk of the development
of cutaneous neoplasia, especially basal cell carcinoma and syringocystadenoma
pappiliferum (4,5). These tumors usually develop after puberty.
While extensive literature describes the dermatologic, neurologic as well as
ophthalmologic manifestations of LNSS, the surgical approach to these complex head
and neck lesions has not been previously described. We present a cohort of 5
consecutive patients with diagnosis of linear sebaceous nevus syndrome with
lesions covering extensive areas of the face and scalp, all were treated in our
institution within the last 10 years. All patients were treated with expansion of
the adjacent texture, color, and adnexal matched skin as the primary modality of
treatment. Full thickness skin grafts, local skin flaps and prefabricated flaps
were applied for reconstruction of specific areas. These techniques were based on
experience gained in treating our previously published large series of congenital
pigmented nevi (6).
Case reports: Five consecutive patients presented in this review were diagnosed
with linear nevus sebaceus syndrome based on the extensive head and neck sebaceous
nevi associated with abnormalities of the central nervous system (primarily
seizures and developmental delay), eyes and skeletal system. These were selected
from a subset of over hundred patients treated for small, intermediate and large
size sebaceous nevi of the head and neck. All of the patients were girls. The age
ranged from 2 months to 3.5 years. Three patients who have been followed for 2 to
8 years are presented in detail.
Discussion: Linear nevus sebaceus syndrome (LNSS) is characterized by the
association of nevus sebaceus, covering extensive areas on the head and scalp,
with abnormalities of the central nervous system, ophthalmologic and skeletal
changes and malignancies (1,7). The incidence is approximately 1:10,000 live
births, and there is no sexual predilection reported, although our series was
exclusively females. Feuerstein and Mims’ original paper (8) was followed by
extensive literature describing the dermatologic, neurologic and ophthalmologic
manifestations of this disease. The objective of this report is to describe the
surgical approach for the excision and reconstruction of giant sebaceous nevi of
the face and scalp in children with LNSS. To our knowledge, this report represents
the largest surgical series and suggests a reliable approach to the treatment of
the cutaneous manifestations of this syndrome.
Although the exact incidence and timing of malignant degeneration in giant
sebaceous nevi is not known, most authors believe that patients with this
condition, whether associated with LNSS or solitary, have a 10 to 20 per cent risk
of development of cutaneous neoplasia, especially basal cell carcinoma and
syringocystadenoma papilliferum (4,5). The malignant degeneration rarely occurs
before the third decade of life, although earlier cases were described in the
literature. There is little debate regarding the aesthetic deformity presented by
large yellow-orange verrucous covering extensive areas of the face which become
progressively verrucous over time, especially couple of years before and during
the puberty. For these reasons, we strongly feel that multistage excision of as
much nevus as possible and carefully planed reconstruction are indicated in
children with LNSS and are not precluded by other, usually serious medical
conditions associated with the syndrome.
Although extensive review of the central nervous abnormalities is beyond the
objectives of this review, there are few points of greater importance when caring
for these patients. Seizures, either generalized or focal, occur in 76 percent of
patients with LNSS. Patients with widespread involvement of the head, neck and the
upper trunk are affected most frequently. Seizures begin during the neonatal
period when the patient has extensive CNS involvement, but may not begin until 3
years of age with less significant CNS alterations (1). Developmental delay is
found in up to 60 percent of patients and correlates with abnormal cranial CT and
MRI scans. Macroencephaly, paresis, cortical atrophy and variety of tumors
involving the nervous system have been noted (8-11). Thorough neurologic
evaluation and control of the seizures are mandatory prior to surgery. Our
practice has been to delay surgery until patients are seizure free or until
seizure disorder is relatively stable for at least six months to avoid secondary
neurologic damage in the perioperative period.
Skeletal alterations are found in 68 percent of patients with LNSS.
Kyphoscoliosis is the most common skeletal anomaly but may not become evident
until late childhood. Hemihypertrophy and other deformities of the extremities,
such as pas equinovarus, genu valgum, bone hypoplasia and syndactily can occur
(1,13). Osteolytic lesions and fractures have developed occasionally, and vitamin
D-resistant rickets has been reported (12).
Tissue expansion has given surgeons the capability to harvest large flaps of
color-, thickness- and texture-matched skin while simultaneously minimizing donor
site defects. Since its introduction, tissue expansion has been used to solve a
wide variety of reconstructive problems, with a significant increase in the
refinement of its application (15). All the patients in this series were treated
with an expansion of the adjacent skin as the primary treatment modality.
Traditional methods such as skin grafting, although providing acceptable coverage
for upper face reconstruction in the adult population, rarely provide optimal
results in children, even when entire aesthetic units are grafted. Tissue
expansion and flap design must be optimized to ensure that the landmarks of the
facial aesthetic units will be disturbed as little as possible. In particular,
emphasis is placed on brow symmetry, hairline position, hair direction and scar
orientation (6,14). The incision for the expander placement may be placed along
border of adjacent normal skin or adjacent area in line with planned scar. We
always use the largest expander possible beneath the uninvolved skin and
occasionally even carry the expander under the lesion. Expanders are typically
overexpanded as well.
The lesions of nevus sebaceus typically appear as one or two wide nevi on the
scalp and forehead with multiple additional thin linear segments vertically
positioned on the face. Midline linear lesions are common. This unique
configuration allows partial or complete excision of these linear segments when
expanders are being placed and again when the expanded flaps are transposed, in
order to minimize the overall number of surgical procedures. Philosophically, the
plan is to excise as much of the large nevus segments as possible along with many
of the thinner linear areas early, leaving final excisions and grafting to ear
until an older age. Simultaneous scar revisions can be completed in those
procedures.
Reconstruction of the entire cheek aesthetic unit is a difficult problem in
pediatric population. There is a little skin laxity to allow local advancement of
“face lift” type flaps. Skin grafts on the cheek often result in very poor
aesthetic and functional outcome in these growing patients. In one patient in our
series, reconstruction of the entire cheek unit was achieved by staged
transposition of a prefabricated expanded flap from the lateral neck and shoulder
area. Although technically demanding, this technique provided a large flap matched
for color and texture for resurfacing of the entire cheek, that can be further
reexpanded once in place.
Skin grafts can be used in specific areas with aesthetically pleasing results
which stand up to growth over time (15). Large expanded full thickness skin grafts
are invaluable in obtaining single unit, color compatible skin coverage of the
periorbital and nasal areas. Skin grafts are also frequently used to resurface the
non hair bearing areas above and posterior to the auricle, after advancing
expanded scalp flaps to reconstruct the hairline. The ear itself can be covered
with full thickness skin graft in a staged fashion starting when the child is 5
years of age. Larger anterior surface coverage has been accomplished using a near
total postauricular full thickness skin graft from the contralateral ear (for
optimal color match). The postauricular donor site is closed with a full
thickness skin graft harvested from the groin.
While we would suspect a higher rate of infection in these patients, to date, none
of the serial intralesional excisions, of either the larger nevi or linear strips,
have been complicated by wound separation or delayed healing. All children are
covered with a broad spectrum antibiotic at the time of expander placement and
continued until drain removal (5-7 days). In these cases, in particular, our
threshold for resuming antibiotic coverage if irritation or excoriation of any of
the lesions occur, is low.
Given the scattered distribution of these complex lesions, long term follow up is
required to assure that minor areas of residual lesion are monitored to minimize
risks of malignant degeneration. These small residual lesions should be of a size
that later treatment under local anesthesia would be possible, but simultaneous
(and medically indicated) excision may be carried out under general anesthesia
along with revision of some of the scars from the prior surgeries.
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