CHAPTER
102
Dysplastic Scoliosis:
Neurofibromatosis Spinal Pathology
INTRODUCTION
The neurofibromatoses are a spectrum of multifaceted genetic
disorders. An afflicted patient may present with a wide range of
clinical manifestations that include abnormalities of the skin,
nervous tissue, bones, and soft tissues. Most of the original
descriptions of these disorders were by pathologists,29 and the
common characteristic was the presence of fibrous nerve sheath
tumorsneurofibromatathroughout the body. Advances in
genetics and molecular biology have proven that the different
forms are genetically distinct. Clinically, there are several forms
of neurofibromatosis, with peripheral (von Recklinghausens
disease; neurofibromatosis type 1; NF-1) and central (neurofi-
bromatosis type 2; NF-2) being the most widely recognized.20
Other subtypes are recognized, especially segmental neurofi-
bromatosis14 and schwannomatosis.17
GENETICS
NF-1 is one of the most common single gene disorders in
humans, with a prevalence of 1 in 3000 to 4000 individuals.24
There is no gender or ethnic predilection. It is an autosomal
dominant disorder with nearly 100% penetrance; however, the
severity of the clinical manifestations is quite variable.
The responsible gene was localized to chromosome 17
(17q11.2) in 1987. By 1990, the gene was identified and its pro-
tein product characterized. The entire sequence of the
expressed NF-1 gene has been reported.8 Cloning of the gene
has allowed the creation of animal models. The NF-1 locus con-
tains about 350,000 base pairs and at least 59 exons. The prod-
uct of interest to NF-1 is a 2818 amino acid protein called neu-
rofibromin. Although the complete function of neurofibromin
is unknown, there is evidence that one function is that of a
tumor suppressor.28 One domain of neurofibromin downregu-
lates p21-Ras, a key signal transduction protein that itself func-
tions as a cell growth promoter. The active form of Ras is the
guanosine triphosphate (GTP)-bound state. Neurofibromin is
a Ras-specific GTPase activator that enhances the conversion of
active GTP-Ras to the inactive form. Clinical NF-1 develops
when neurofibromin is not present.
Central neurofibromatosis (NF-2) is also an autosomal dom-
inant disorder. NF-2 is not as common as NF-1, affecting about
1062
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Alvin H. Crawford
Keith R. Gabriel
1 in 100,000 individuals. Characteristically, in NF-2 there are
bilateral schwannomas of the vestibular portion of the eighth
cranial nerve. These acoustic neuromas do not occur in NF-1.
Schwannomas of other peripheral nerves, meningiomas, and
ependymomas are also common in NF-2. The gene for NF-2 has
been localized to the long arm of chromosome 22 and has also
been cloned. The gene product is a protein interchangeably
called merlin or schwannomin. Schwannomin also has a role in
tumor suppression.16
Segmental neurofibromatosis outwardly resembles NF-1;
however, as the name would imply, in this form the clinical
manifestations are limited to one segment, or few segments, of
the body. This is believed to be due to somatic mosaicism for
the NF-1 gene mutation.14
A recently defined form of neurofibromatosis, schwannoma-
tosis, involves multiple deep and painful schwannomas.
Although there is overlap with NF-2 in presentation and pheno-
type, schwannomatosis is clinically and molecularly distinct.17
The gene locus is on chromosome 22 but is proximal to the
NF-2 gene.
The orthopedic manifestations of NF-1 (von Recklinghausens
disease; peripheral neurofibromatosis; NF-1), especially the
complications after treatment, are frequent, and enjoy a promi-
nent place in orthopedic literature. Spinal deformity is the most
common of these orthopedic manifestations. Other characteris-
tic findings include congenital tibial dysplasia with anterolateral
bowing or pseudarthrosis of the tibia, similar changes in other
long bones such as in the forearm, overgrowth phenomenon of
the extremities, and soft tissue tumors. In contrast, the other
clinical forms of neurofibromatosis do not commonly have
direct bone involvement or other primary orthopedic manifes-
tations and will not be further discussed in this chapter.
DIAGNOSIS
The diagnosis of NF-1 continues to be based on clinical find-
ings in the majority of cases. Specific criteria for confirming the
diagnosis were approved by the Consensus Development Con-
ference on Neurofibromatosis at NIH in 1987.22 To establish
the diagnosis of NF-1, two or more of the seven clinical criteria
must be present. These criteria have shown to be useful for
diagnosis even in young children (Table 102.1).
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 Chapter 102 Dysplastic Scoliosis: Neurofibromatosis Spinal Pathology
Clinical Diagnostic Criteria
TABLE 102.1
for Neurofibromatosis Type 1
1. Six or more caf au lait macules of greater than 5 mm diameter
in prepubertal individuals, or 15 mm diameter in postpubertal
individuals.
2. Two or more neurofibromas of any type, or one plexiform
neurofibroma.
3. Axillary or inguinal freckling.
4. Optic nerve glioma.
5. Two or more iris Lisch nodules (iris hamartomas).
6. A distinctive bone lesion: Sphenoid wing hypoplasia, thinning
of a long-bone cortex with or without pseudarthrosis.
7. A first-degree relative who has NF-1 by the above criteria.
The development of genetic testing for NF-1 was slowed by
the large complex structure of the NF-1 gene and the wide
diversity of mutations. Approximately 50% of all NF-1 cases are
new mutations, which is 100-fold higher than the usual muta-
tion rate for a single locus and likely reflects the large size of
the NF-1 locus. A vast number of different NF-1 mutations have
been identified, nearly all of which have been unique to a par-
ticular family. Protein truncation assay, as formerly used, was
able to identify only about 70% of pathologic mutations.
Comprehensive genetic testing by direct sequencing of the
NF-1 gene is now available and offers 95% sensitivity.19 This
multistep direct testing method is performed on a fresh blood
sample. The protocol includes protein truncation testing by
fluorescence in situ hybridization analysis for total gene dele-
tions, direct sequencing of the entire coding region for mis-
sense mutations or smaller in-frame deletions or insertions,
long-range reverse transcriptase polymerase chain reaction and
Southern blot analysis for smaller mutations, and cytogenic
analysis for large-scale rearrangements.19
Direct testing is helpful in establishing the diagnosis in uncer-
tain cases, in prenatal diagnosis, and for research. Unfortunately,
none of these tests predict clinical manifestations, severity, or
complications of NF-1. Each person who carries the gene will
eventually show some features of the condition. There seem to
be two chronologic peaks of clinical manifestations for NF-1
patients: one peak from 5 to 10 years of age, and a second from
36 to 50 years of age. At the second peak, 75% of the clinical
problems are related to malignant neoplasms.25
SPINAL DEFORMITIES, GENERAL
Spinal deformity is the most common osseous defect associated
with NF-1, and scoliosis is the most widely discussed of the spi-
nal deformities. However, it is important to emphasize that all
types of spinal deformities, in multiple planes and in any part
of the spine, occur with NF-1.
The true prevalence of spinal deformity in NF-1 is unknown.
Literature reports range from 10% to 77%, with most authors
focusing on screening for scoliosis. Cervical and sacral deformi-
ties are probably underreported. Some studies may be biased
by the referral nature of the institution, or by an investigators
strong interest in spinal deformity. A sampling of reports with
presumed low selection bias suggests that about one out of four
patients with NF-1 will have associated scoliosis. Chaglassian
and colleagues4 reviewed the records of two hospitals over
15 years to identify 141 patients with NF-1 and found that
LWBK836_Ch102_p1062-1077.indd 1063
1063
scoliosis was present in 26% of those patients. A 5-year survey by
Holt10 reported scoliosis in 36% of children having NF-1.
Akbarnia and colleagues1 studied an otherwise unselected popu-
lation of patients from a multispecialty neurofibromatosis clinic
and found a 10% prevalence of scoliosis. Crawford et al5 report a
23% prevalence of scoliosis in a large neurofibromatosis clinic.
It has become conventional to discuss spinal deformity, and
most especially scoliosis, in NF-1 as either idiopathic or dysplas-
tic. There are no specific, universal diagnostic criteria to define
dystrophic, but the characteristic radiographic bone changes
of NF-1 are best described in association with thoracic scoliosis.
The most typical dystrophic curve is short-segmented (usually
involving four, five, or six vertebrae), sharply angulated, and
located in the upper part of the thoracic spine (Fig. 102.1).
Other easily recognizable dystrophic features include scallop-
ing of the vertebral borders, severe rotation of the apical verte-
bra, widening of the spinal canal, enlargement of the neural
foramina, hypoplastic or absent pedicles, paraspinal masses,
spindling of the transverse processes, and rotation of the ribs.6
A spinal deformity that initially appears idiopathic may subse-
quently develop dystrophic features. A report by Funasaki et al
described the development of certain dystrophic features over
time.7 Durrani et al have further documented this modulation
of scoliosis toward the dystrophic form, especially in those patients
who initially present at young ages.6 Modulation in the character
of a deformity over time is unique to NF-1. A nondystrophic curve
can become dystrophic, and a dystrophic curve can acquire fur-
ther dystrophic changes. Modulation may evolve slowly or aggres-
sively. At a certain point, modulation can alter the behavior of the
deformity and herald a course of rapid progression.
The etiology of the dystrophic bone changes remains uncer-
tain. Suggested causes of intrinsic bone pathology have included
endocrine disturbances, primary mesodermal defects, and
direct infiltration of neurofibromatous tissue into bone. Recent
investigations have focused attention on a relative osteoporosis
in the deformed segments.13 The erosive and destructive effects
of adjacent soft tissues, either intraspinal or extraspinal, must
be understood. These soft tissue abnormalities will be empha-
sized later in this chapter.
IMAGING
Radiographic study of the deformed spine should include fron-
tal and lateral views of the cervical, thoracolumbar, and sacral
regions. It is important to see the entire spine because of the
potential for occult deformity in each region. Specific areas of
progressive deformity or severe dysplasia can be investigated
using computed tomography (CT) or magnetic resonance imag-
ing (MRI) (Fig. 102.2). MRI of the entire spine is advised prior
to any spinal surgery for these patients. Conventional high-
volume myelography with CT may be the optimal study for cases
involving especially severe deformity. Because significant lesions
and deformity can be asymptomatic, cervical radiographs are
also recommended for those patients who require endotracheal
anesthesia, who undergo halo-femoral traction, or who present
with neck tumors. Patients with NF-1 may have erosive defects in
the skull. It is important to obtain skull radiographs prior to
applying halo or GardnerWells tong traction pins. It is also
advisable to obtain plain films of the femurs prior to insertion
of femoral traction pins, as there are occasionally dystrophic
areas with multiple fibrous cortical defects around the knee.
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 1064 Section IX Dysplastic and Congenital Deformities

A B

Figure 102.1. This 12-year-old girl (A) has multiple physical stigmata of NF-1, and her radiograph
(B) demonstrates typical features of dystrophic thoracic scoliosis.

A C

Figure 102.2. A 9-year-old girl presented with a dystrophic scoliosis. The radiograph (A) shows the dystro-
phic wedging, angulation, and rotation of the apical vertebrae. MRI scans in the frontal (B) and axial (C) pro-
jections show a neurofibromatous mass in the concavity of this curve, extending into the spinal canal.

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 Chapter 102 Dysplastic Scoliosis: Neurofibromatosis Spinal Pathology 1065

CERVICAL DEFORMITY MANAGEMENT neurologic deficits, as well as cervical spondyloptosis or disloca-

Cervical paralysis in neurofibromatosis was reported as early as
1892 by Meslet.18 Most subsequent studies have provided only
incidental references to the association of cervical deformity
and NF-1, either as isolated case reports or as very small series.
However, the significance of cervical involvement should not
be minimized. Reported problems include paralysis and lesser
tion at the atlantoaxial articulation and the subaxial cervical
spine.
Obvious indications for cervical evaluation include neck
pain, torticollis, or dysphagia. Some patients have come to
orthopedic attention during the evaluation of a head or neck
mass by another surgical service, when routine radiographs
reveal the abnormal cervical alignment (Fig. 102.3). Various

A B

C D

Figure 102.3. An 11 year old male was unable to raise his chin off his chest. He had no neurological deficits. (A) The lateral cervical spine
radiograph in neutral position shows kyphosis with anterior subluxation. Lateral radiographs in flexion (B) and in extension (C) show essentially
no change in vertebral alignment. (D) MRI of the spinal cord demonstrated a mass at the mid apical region. Following initial cervical-femoral
traction to reduce the kyphosis (continued)

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 1066 Section IX Dysplastic and Congenital Deformities

E F G

H I J

Figure 102.3. (Continued) (E) biopsy confirmed grade 2 astrocytoma. (F)

Operative photo at time of excisional biopsy of astrocytoma through wide laminec-
tomy. (G) Microscopic view of spinal cord dissection. (H) Two weeks later, sublam-
inar cables were placed under the remaining laminae because there were no
lateral masses and the pedicles had been eroded. (I) Both fibula were attached
from the occiput to the upper thoracic spine. An occipitocervical plate/rod system
was then inserted to T 4. (J) Radiograph prior to staged anterior strut grafting. (K)
One year post-operative lateral cervical spine x-ray. Immobilization was continued
in a non-invasive Minerva because of the need for chemotherapy and concern for
K
bony union.

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 Chapter 102 Dysplastic Scoliosis: Neurofibromatosis Spinal Pathology
types of associated soft tissue abnormalities have been reported,
including anterior meningocele, plexiform neurofibroma, and
dumbbell tumors of the nerve roots.
Cervical scoliosis occurs in NF-1. A single-stage posterior
instrumented fusion, utilizing autologous bone graft and halo
immobilization, is usually sufficient. If comprehensive imaging
reveals significant dystrophic bone changes or adjacent soft tis-
sue abnormalities, then consideration of anterior and posterior
fusion should be entertained to optimize chances of solid
fusion. Imaging should include both MRI for soft tissue assess-
ment and CT for optimal bone definition, as the morphology
of the pedicles may influence the choice of implants. There are
not yet any reported series to show whether posterior segmen-
tal fixation might eliminate the need for halo immobilization
in these patients.
The most common cervical abnormality is a severe kyphosis,
which in itself is highly suggestive of NF-1 (Fig. 102.4). Kyphosis
is also frequently seen following excision of cervical tumors, if
excision includes release of the ligamentum nuchae or resec-
tion of the posterior elements. A cervical decompressive lamine-
ctomy without fusion will virtually always develop secondary
instability and progressive kyphosis.
For mild but progressive cervical kyphosis without dystro-
phic changes, a posterior fusion is the treatment of choice.
Halo traction is a useful preoperative step if the kyphosis is
greater than 45 but still retains flexibility. If the deformity is
rigid, then soft tissue release followed by traction is believed to
be safer by permitting repetitive neurologic assessment during
traction in an awake patient. If sufficient bone stock is present,
internal fixation with rods, wires, screws, or hooks may be used.
Sublaminar wire fixation may be difficult due to dystrophic
changes in the bone and soft tissues.
There is no universally accepted magnitude at which a
kyphotic deformity is judged to be so severe that combined
anterior and posterior fusion is needed, but most reports
seem to indicate about 50 to 70. If greater kyphosis is pres-
ent, and in all cases in which there has been previous lamine-
ctomy, anterior and posterior fusion will be necessary.
Dystrophic changes anteriorly in the vertebral bodies may
lead to instability, sharp angular kyphotic deformity, and
translational malalignments, which also require anterior and
posterior surgery.
When neurologic changes of cord compression secondary
to a flexible kyphosis are present, a gentle trial of closely mon-
itored halo traction is reasonable. Often the myelopathy will
improve, at which time anterior and posterior fusion with
halo immobilization may be performed. If the myelopathy
does not improve, then anterior decompression followed by
anterior and posterior fusion is indicated. Laminectomy alone
in this setting is contraindicated as this will only worsen the
kyphosis.
Kokubun and colleagues12 have reported a case of single-
stage correction with anterior and posterior fusion that obvi-
ated the need for preoperative traction. Through simultaneous
anterior and posterior approaches they were able to distract
anteriorly and compress posteriorly, with the axis of correction
being the posterior longitudinal ligament. Yonezawa and col-
leagues32 have used a similar technique, incorporating poste-
rior instrumentation with pedicle screws. As microvascular sur-
gical techniques continue to be developed and refined, some
centers utilize free vascularized strut grafting to deal with severe
kyphosis.2,23
LWBK836_Ch102_p1062-1077.indd 1067
1067
Some patients may need fusion in the cervical spine and
upper thoracic region. This group of patients may benefit from
a trap door sternal split approach if anterior fusion is
needed.21 The approach allows simultaneous anterior exposure
of the lower cervical and upper thoracic spine (Fig. 102.5).
THORACIC AND LUMBAR DEFORMITY
MANAGEMENT
SCOLIOSIS
Natural History
Few clinical studies have discussed the natural history of unop-
erated spinal deformities in neurofibromatosis. Virtually all of
those studies were done prior to recognition of the possibility
of deformity modulation across a spectrum of nondystrophic to
dystrophic, especially in younger patients.6,7 There is general
agreement that nondystrophic curves usually progress in a
manner similar to idiopathic scoliosis. Dystrophic curves tend
to be aggressively progressive. Winter and colleagues31 reviewed
the natural history, associated anomalies, and responses to
operative and nonoperative treatment in 102 patients with NF-1
and spinal deformity. Nondystrophic curves in 22 patients
behaved according to the primary diagnosis of the curve. Dys-
trophic curves did not respond to brace treatment, and early
fusion was advised for these cases. Calvert and colleagues3
reviewed 47 cases of dystrophic NF-1 scoliosis. Thirty-two of
their patients were untreated for more than 1 year, the average
being 3.6 years, providing the opportunity for retrospective
observation of their natural history. In this subgroup, the
average age at presentation was 9.75 years. The mean initial
scoliosis of 59 increased at an average of 8.1 per year. The
mean initial thoracic kyphosis of 49 increased at a rate of 11.2
per year. The more severe dystrophic changes in the vertebral
bodies were associated with more rapid deterioration.
It is also difficult to compare historical results of the surgical
treatment of neurofibromatosis scoliosis, because the literature
is not consistent in classification and treatment methods.
However, there is universal agreement that it is more difficult
to achieve a stable fusion of dystrophic curves, with a higher
pseudarthrosis rate and curve progression even after appar-
ently solid arthrodesis when operated only posteriorly. Holt
and Johnson reported on treatment of dystrophic curves with
Cotrel-Dubousset instrumentation.11 Three of their patients
showed progression despite instrumentation and fusion. They
advocated bracing if the spine is potentially unstable following
surgery, despite extensive experience with brace-free patients
with idiopathic scoliosis. Sirois and Drennan reviewed 32
patients with neurofibromatosis and spinal deformity, 23 of
whom had dystrophic curve patterns.26 They found a 38% inci-
dence of pseudarthrosis for their subgroup of 15 patients who
underwent isolated posterior fusion, 9 with Harrington instru-
mentation, 3 without instruments, and 1 with Luque instru-
mentation. They recommended treatment of kyphoscoliotic
curves with anterior and posterior fusion and suggested MRI
assessment for possible pseudarthrosis. The report from Wilde
et al presents 25 cases of dystrophic scoliosis at a mean of
9.7 years after surgery.30 Spinal deformity in those patients
tended to progress despite solid anterior and posterior arthro-
desis, especially in cases with hyperkyphosis and short curves.
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 1068 Section IX Dysplastic and Congenital Deformities

A B C

D E F

Figure 102.4. This patient presented at age 11 years with a neurofibroma in the upper right
chest, which was resected through an anterior approach. The initial preoperative anteroposterior
(A) and lateral (B) radiographs show vertebral dystrophic changes throughout the cervical spine,
with early kyphosis at the C6-C-7 interval. Four years later, following wide decompressive laminec-
tomy of the mid-cervical spine for neurofibromata, new anteroposterior (C) and lateral (D) radio-
graphs show progressive dystrophic changes and a spectacular kyphosis. The kyphosis was
progressive, even over a 6-month interval (E). The patient remained neurologically intact, probably
owing to the massive enlargement of the spinal canal secondary to dural ectasia as seen on the MRI
scan (F). A photograph of the patient (G) shows the cosmetic effect of the kyphosis and also shows
G
the lordotic thoracic spine, which may contribute to the development of the cervical deformity.

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 Chapter 102 Dysplastic Scoliosis: Neurofibromatosis Spinal Pathology 1069

A B C

D E F

Figure 102.5. Deformity at the cervicothoracic junction may be addressed with a trap-door sternal split-
ting approach. The incision (A) incorporates a conventional neck exposure along the medial border of the
sternocleidomastoid to the sternal notch, continues distally with longitudinal division of the sternum, and
progresses laterally at the fourth interspace. The trapdoor of lateral portion of sternum with attached ribs
(rake retractor in (B)) is lifted laterally, which requires division of the internal mammary vessels. The spine
(fixed blade retractor in (B) is exposed between lung and mediastinum. The innominate and subclavian ves-
sels can be retracted proximally and distally. If dealing with a dystrophic curve at the cervicothoracic junction
this approach allows visualization across this critical area for anterior fusion, which should always be followed
by posterior instrumented fusion (C through F). (C and D: Reprinted with permission of Crawford AH,
Herrera-Soto J. Scoliosis associated with neurofibromatosis. Orthop Clin North Am 2007;38:553562).

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 1070 Section IX Dysplastic and Congenital Deformities
Prognostic features for this progression included vertebral sub-
luxation, disc wedging, and peripheral skeletal dystrophy.
At the time of this writing, there are no published series to
demonstrate whether the results of surgical treatment incorpo-
rating pedicle screw fixation might be different from those
studies in which segmental hook constructs were used. Our cur-
rent experience leads us to suggest preoperative CT with three-
dimensional reconstruction to determine the adequacy of the
pedicles in these patients.
Treatment of Nondystrophic Curvature
The nondystrophic curvature, which mimics idiopathic scolio-
sis, is the most common spinal deformity in neurofibromato-
sis. In general, nondystrophic curvatures should be treated
with the same management algorithms as idiopathic scoliosis.
However, when patients are followed over an extended period
of time, there is a higher incidence of progressive deformity,
including possible pseudarthrosis following instrumentation
and fusion, than is found in ordinary idiopathic scoliosis. In
addition, curves may modulate and develop dystrophic char-
acteristics over time.6,7 The entire spine should be imaged by
CT myelography or MRI prior to surgery. Not infrequently,
dystrophic features may be identified with comprehensive
imaging, so that the scoliosis will be reclassified and treatment
redirected. Titanium instrumentation systems are recom-
mended to minimize interference with possible subsequent
imaging. Postoperative bracing is strongly encouraged for all
of these patients, even when segmental instrumentation is
used.
Treatment of Dystrophic Scoliosis
The dystrophic curve in NF-1 virtually always progresses. Brac-
ing is not effective in the management of these deformities.
Early fusion is the best treatment. The need to evaluate the
spinal canal with MRI or high-volume myelography prior to
treatment cannot be overemphasized. This level of imaging
would demonstrate any paravertebral tumors and assist the sur-
geon in planning the operative approach.
When the dystrophic scoliosis is recognized, it is important
to evaluate the sagittal contours. Significant or unusual sagittal
deformity, especially kyphosis, implies more severe dystrophic
change in both bone and paraspinous soft tissue, and heralds a
more challenging treatment course. The following recommen-
dations should apply to those curves having relatively normal
kyphosis and lordosis:
A. Those patients in all age groups having dystrophic curva-
tures of less than 20 may be monitored for progression at
standard intervals.
B. Infantile, juvenile (early onset), and young adolescent
patients who have dystrophic scoliosis are challenging treat-
ment groups. Posterior spinal fusion alone is relatively con-
traindicated in such young patients who have progressive
dystrophic deformities because of the high prevalence of
pseudarthrosis, and of curve progression even with an
apparently solid posterior fusion. The most predictable and
successful management in these situations is anterior and
posterior fusion.
Fusion in the young individual stunts the growth of the
truncal height only minimally because the curve is usually
short and the involved vertebrae do not have normal growth
LWBK836_Ch102_p1062-1077.indd 1070
potential. In the setting of dystrophic neurofibromatosis
curves, the relative risks of the crankshaft phenomenon, as
contrasted to the inherent difficulties of the underlying dis-
order, are undetermined at this time. Immature dystrophic
curves do not always progress after posterior fusion, perhaps
due to a lack of normal anterior vertebral growth potential
in dystrophic situations. Analysis is further confused by the
tendency for dystrophic curve progression even after suc-
cessful anterior and posterior fusion.
Subcutaneous expandable growing rod instrumentation
may provide correction and stabilization while allowing fur-
ther growth. The technique is attractive, especially with the
recent technological designs of universal instrumentation
and localized fusion of anchor sites. There are no reported
series yet to demonstrate the efficacy of this treatment in
NF-1. Based on our own limited experience, we now recom-
mend that younger patients in the range of 5 to 10 years of
age who have progressive curves of less than 60 may be
treated with a dual expandable growing rod technique.
Lengthening of the instrumentation is performed regularly
at 6-month intervals. For those patients younger than 5
years, but whose curves are larger than 60, we perform
anterior longitudinal ligament and anterior annulus release
without fusion and apply halo-femoral traction on a hori-
zontal turning frame for no less than 10 days, followed by
application of the growing rod technique (Fig. 102.6).
C. For adolescent patients who have progressive curves with
greater than 20 but less than 40 of angulation, spinal
fusion should be performed. Posterior fusion is most often
elected, and combined procedures are not routine for
curves in this group. Preoperative imaging of the entire spi-
nal canal is mandatory. If dural ectasia or tumors adjacent
to the spine are identified, anterior and posterior fusion
may be best even for these moderate curves. Care should be
taken not to inadvertently penetrate into the canal during
posterior exposure, as the bone may be soft and the poste-
rior elements may be very thin. Dissection with electrocau-
tery is recommended, rather than use of periosteal elevators.
The fusion should extend at least from the neutral vertebra
above to the neutral vertebra below. Meticulous fusion tech-
nique is required, with ablation of all facet joints and
removal of all soft tissue from interposition in the area of
the bone graft. Autogenous bone graft is preferred. Seg-
mental instrumentation with titanium implants should be
used when possible. Dystrophic vertebrae are not always
good recipients for hooks because of osteoporosis and
deformity of the posterior elements, and passage of sublam-
inar wires is hazardous if there is associated dural ectasia.
Pedicle screw anchors may provide the best foundation;
however, preoperative imaging is required to validate pedi-
cle morphology. Postoperative cast or orthotic immobiliza-
tion is strongly recommended. The fusion should be
evaluated at 6 months by thin slice CT. If there is any ques-
tion of failure of consolidation, reoperation with additional
bone grafting is advised and off-label use of biologic
adjuncts such as bone morphogenic proteins may be con-
sidered. There is now a case report questioning whether
malignant conversion of a plexiform neurofibroma and sub-
sequent rapid growth of the sarcoma might be related to
use of bone morphogenic protein. We agree with the
authors of the report: This one case is not conclusive, but
caution is advised.27
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 Chapter 102 Dysplastic Scoliosis: Neurofibromatosis Spinal Pathology 1071

A B

C D

Figure 102.6. This 5-year-old patient with a severely dystrophic kyphoscoliosis (A, B) underwent prelimi-
nary anterior release through a double trapdoor approach, posterior release, and halo-femoral traction to
decrease the severity of the curve. Dual growth rods were provided (C, D) and continuing management
includes a CTLSO. (A, B, C, D: Adapted from Crawford AH, Schorry EK. Neurofibromatosis Update. J
Pediatr Orthop 2006;26(3):413--423).

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 1072 Section IX Dysplastic and Congenital Deformities
When severe dystrophic bone changes are evident even in
curves of moderate magnitude (20 to 40), anterior fusion
should be performed in addition to posterior fusion. This
increases the fusion rate and reduces the risk for postopera-
tive curve progression, which can occur despite solid poste-
rior fusion.
D. Primary combined anterior and posterior fusion is recom-
mended for those dystrophic curves with coronal plane
deformity exceeding 40, even if the sagittal contour is rela-
tively normal. Autogenous bone graft is used in all areas,
and contoured segmental instrumentation is used. A post-
operative Risser cast or thoracolumbosacral orthosis (TLSO)
is used for all of these patients.
As the coronal plane deformity increases, there is ever
more propensity for concomitant sagittal plane deformity.
Recommendations for dealing with very large curves inevitably
overlap or duplicate the discussion of kyphoscoliosis. Staged
anterior release, anterior and posterior release, and traction
are all considered when dealing with these severe deformities.
Winter et al reported that, in patients with a severe curve and a
flexible kyphosis, preoperative traction improved pulmonary
function, improved minor neurologic deficits, and diminished
the curve size before fusion.31 Halmai et al reported their pro-
tocol for treating dystrophic curves of greater than 60 by plac-
ing the patient in halo-vest traction for an average of 3 weeks
before surgery.9
We recommend initial anterior release and fusion with
morselized allograft, nasojejunal tube alimentation, and inter-
val halo-femoral traction on the horizontal turning frame for
rigid curves approaching 90. This is followed by instrumented
posterior fusion. For curves of greater than 100 in any plane,
anterior release and fusion with concomitant posterior release
is followed by nasojejunal tube alimentation and craniofemoral
traction for no less than 10 days prior to definitive fusion.
Careful neurologic monitoring should be documented during
periods of traction.
KYPHOSCOLIOSIS
The term kyphoscoliosis describes cases in which the defor-
mity includes a kyphosis of greater than 50. Severe kyphosis
associated with only mild scoliosis is relatively uncommon. The
curves typically include severely dysplastic vertebrae. The apex
is sharply angulated, with vertebral bodies so deformed and
attenuated that it may be impossible to identify them on rou-
tine radiographs. The kyphosis can create a hairpin deformity,
where the upper and lower limbs of the kyphosis subluxate
sideways and come to lie in bayonet apposition (Fig. 102.7).
The pathologically increased spinal flexion of a kyphotic defor-
mity may lead to neurologic impairment.
Surgical management of kyphoscoliosis and neurofibroma-
tosis begins with appropriate imaging. A hyperextension cross-
table lateral radiograph, made with the patient lying supine
over a bolster, should be performed to determine the flexibility
of the kyphosis. Evaluation of the entire spine by MRI or a high-
volume CT myelogram is required. For neurologically intact
patients with a kyphotic angle of greater than 50, anterior and
posterior fusion should always be performed. For the anterior
fusion, thorough intervertebral disc space exposure is extremely
important. Dystrophic vertebral bodies tend to bleed freely
from any exposed cancellous area. The end plate is the stron-
LWBK836_Ch102_p1062-1077.indd 1072
gest portion and should be protected by dissection through the
annulus fibrosis instead of sharp dissection of the endplate apo-
physis. The anterior fusion should be extended through the
entire structural area, or even one to two levels past both end
vertebrae. Tibial or fibular strut bone graft should be used
when possible, and one should attempt to get the struts into the
vertical weight-bearing axis of the torso. A vascularized rib strut
graft may be considered. No soft tissue should be allowed to
interpose between the grafts, and all grafts should have contact
with each other and with the spine. The difficulty of obtaining
a solid fusion is such that we consider the use of biologic
adjuncts such as bone morphogenic proteins, even though this
is an off-label application.
Neurofibromas, and especially plexiform neurofibromas,
seem to be frequently located directly in the path of the opti-
mal anterior approach. Those located in subcutaneous and
muscular tissue planes may be resected or debulked if they
restrict access, but it is neither necessary nor practical to com-
pletely remove these benign masses. Many may be simply
retracted aside during the dissection. Enlarged fibromatous
intercostal nerves running along the inferior surfaces of the
lateral and anterior portions of the ribs may also be retracted
aside and seldom interfere with the initial steps of an anterior
thoracic approach. Thoracoscopic technique may be used if
elected, with access portals advanced bluntly over the superior
margin of the ribs.
Neurofibromas directly adjacent to the spine present a more
challenging problem. Bone graft embedded in or even directly
touching neurofibromatous tissue will regularly be resorbed.
Sufficient tissue must be resected so that no neurofibroma
remains touching the bone graft at the completion of the
fusion. Unfortunately, this may require sacrifice of segmental
nerves.
We recommend an anterior approach from the convex side
in almost all cases. Approaching from the concavity is techni-
cally more demanding, and often the apical vertebra is sublux-
ated or so severely rotated that it is not in alignment with the
rest of the spine. If neurofibromatous tissue in the concavity
must be resected, finding and controlling segmental vessels can
be quite problematic.
Paraplegia is not uncommon in NF-1 patients with severe
kyphosis and is second only to paraplegia in patients being
treated for congenital kyphosis. Causes include the kyphosis
itself or the impingement of lesions such as pseudomeningoce-
les, dural ectasia, or intraspinal neurofibroma against the spi-
nal cord. If there is spinal cord impingement, the problem
should be approached directly: anteriorly with partial corpec-
tomy for anterior lesions, and by hemilaminectomy for poste-
rior lesions. Either approach should be followed by anterior
and posterior fusions. Laminectomy for anterior kyphoscoliosis
cord compression is absolutely contraindicated. Rib protrusion
into the canal may cause either an insidious onset of parapare-
sis or acute paralysis after trauma or following deformity correc-
tion if unrecognized (Fig. 102.8). Osseous dysplasia, interverte-
bral foraminal enlargement, and rotation of vertebral bodies
all may contribute mechanically to allow the heads of the rib to
displace into the canal. Rib osteotomy should be carried out
before instrumented correction in these patients, lest the cor-
rection cause the rib to impale the spinal cord.
If a flexible kyphosis is causing paraplegia, the treatment
following MRI assessment should be craniofemoral traction
(with extreme caution), with close neurologic or evoked
8/26/11 3:02:01 PM
 Chapter 102 Dysplastic Scoliosis: Neurofibromatosis Spinal Pathology
A B
C
potential monitoring during the course of the traction. With
a flexible kyphosis, traction may correct some of the defor-
mity and also reduce spinal cord compression to perhaps
improve the neurologic deficit. Anterior release, decompres-
sion if needed, and fusion should be performed, followed by
posterior fusion.
Traction should not be used15 if the kyphosis is rigid. Traction
in these patients stretches the mobile spinal segments that are
located cephalad and caudad to the kyphosis, increasing the
tension and point compression on the spinal cord at the
LWBK836_Ch102_p1062-1077.indd 1073
1073
Figure 102.7. This patient (A) was neu-
rologically intact at the age of 46 years. Her
only reported symptom was shortness of
breath with exertion. Her dystrophic
kyphoscoliosis had progressed despite an
attempted posterior spinal fusion at the age
of 8 years, and an anterior and posterior
fusion at the age of 18 years. The lateral
radiograph (B) shows a kyphosis so severe
that the upper and lower portions of the
spine lie horizontally beside each other in a
hairpin configuration. This creates an axial
projection of the mid thoracic spine (C)
seen in the right chest on the routine poster-
oanterior radiograph.
midportion of the apex, which may cause further damage.
Therefore, direct anterior release, disc excision, and interverte-
bral fusion followed by 7 to 10 days of traction, followed by
instrumented posterior spine fusion are recommended.
Not every patient obtains solid fusion even following these
procedures. Augmentation of the fusion mass should be per-
formed at 6 months postoperatively if pseudarthrosis is sus-
pected because of loss of correction. The off-label use of
adjuncts such as bone morphogenic proteins should be consid-
ered in these difficult situations. Possible reasons for failure of
8/26/11 3:02:01 PM
 1074 Section IX Dysplastic and Congenital Deformities

A B

Figure 102.8. (A) Several axial CT images show the erosive effects of dural ectasia. There is significant
widening of the spinal canal, and the erosion of the lamina, transverse process, and pedicle on the right has
permitted displacement of a rib into the canal. (B) The three-dimensional reconstruction of the rib protru-
sion best illustrates this problem. (B: Reprinted with permission from Crawford AH, Parikh S, Schorry EK,
Von Stein D. The immature spine in type-1 neurofibromatosis. J Bone Joint Surg Am 2007;89-A(Suppl 1):
123142).

fusion would include technically inadequate procedures or SACRUM AND SPONDYLOLISTHESIS

pressure erosion from enlarging neurofibromas, dural ectasia,
and meningoceles.
Rarely, the deformity itself or the surrounding soft tissue
abnormalities may be so severe that direct instrumentation is
simply not possible (Fig. 102.9). Following anterior and poste-
rior fusion with bridging vertebral body screws or innovative
spinopelvic fixation, prolonged halo casting/bracing may be
needed. Careful evaluation of the fusion mass by bone scan or
high-volume contrast CT should be done at 6 months, with pos-
terior reexploration and augmentation of the fusion mass if
there is any question of weakness. Prolonged immobilization of
a year or more may be required.
LORDOSCOLIOSIS
Lordoscoliosis refers to a small subset of dysplastic cases in
which the sagittal plane deformity includes actual thoracic lor-
dosis, with the angular measurement being of a negative mag-
nitude. Dystrophic thinning of the posterior elements and the
possible presence of intracanal dural ectasia make planning
instrumentation to correct the deformity a challenge. Kyphosis
will develop above the thoracic lordosis in most of these patients
(Fig. 102.4). The cervical spine and cervicothoracic junction
must be evaluated and monitored.
We recommend posterior fusion utilizing contoured titanium
segmental instrumentation for the treatment of progressive
lordoscoliosis. The need for a concomitant anterior procedure
must be individualized, as no firm guidelines can be extracted
from the scant literature.
In neurofibromatosis, dural ectasia, tumors, and the attendant
bony deformity may occur in the sacrum. It is important to
evaluate the entire spine, including the sacral segments, in
every patient.
Spondylolisthesis in patients with NF-1 is rare. Early on, the
listhesis is usually the result of pathologic elongation and thin-
ning of the pedicles, and not just the pars interarticularis.
Increased diameter of the spinal canal is the cause of the prob-
lem, secondary to dural ectasia with meningoceles, or plexi-
form neurofibromata of the lumbosacral roots. The vertebral
bodies are secondarily affected. This is an insidiously progres-
sive disorder that will gradually intensify the deformity.
The treatment in severe spondylolisthesis is anterior and
posterior spinal fusion. We recommend at least an L4-to-sacrum
anterior and posterior fusion with lumbosacral instrumenta-
tion. Postoperative immobilization is strongly recommended.
The fusion mass must be assessed at 6 months, with repeat bone
grafting if there is any question about consolidation.
SOFT TISSUE PROBLEMS
DURAL ECTASIA
Dural ectasia refers to a circumferential dilatation of the dural
sac from an unknown etiology. This expanding dura is extremely
thin and friable, and the enlarged area contains increased cere-
brospinal fluid with a brownish proteinaceous material. Expand-
ing dura erodes the surrounding bony structures, destabilizing

LWBK836_Ch102_p1062-1077.indd 1074 8/26/11 3:02:02 PM

 Chapter 102 Dysplastic Scoliosis: Neurofibromatosis Spinal Pathology 1075

C D

Figure 102.9. This adolescent presented with spondyloptosis and associated olisthetic scoliosis (A and B).
Dural ectasia had caused complete erosion of the left lumbosacral articulation (C), with mechanical instabil-
ity. Treatment proceeded in three stages: the vertical expandable prosthetic titanium rib (VEPTR) (provided
initial coronal plane realignment and basic stabilization, after which limited anterior fusion with instrumenta-
tion was done. (continued )

the vertebrae. Dural ectasia may in some cases expand the spi-
nal canal so much that the spinal cord is not injured even if
spinal dislocation occurs.
MENINGOCELE
The meningocele consists of a protrusion or outpouching of the
dural sleeve through an intervertebral foramen or an erosion of
the vertebral body. It contains an expansion of the subarachnoid
space filled with cerebrospinal fluid and represents a localized
variation of the same process as dural ectasia. Meningocele can
occur at any level but is most often recognized in the thoracic
spine. Meningocele is one of the possible causes of erosive dys-
plastic bone changes.
Meningocele is usually asymptomatic and presents as an inci-
dental finding on chest radiographs. Confirmation of the diag-
nosis is accomplished through use of imaging studies such as
ultrasound, contrast myelography, or MRI (Fig. 102.10). Once

LWBK836_Ch102_p1062-1077.indd 1075 8/26/11 3:02:03 PM

 1076 Section IX Dysplastic and Congenital Deformities

Figure 102.9. (Continued) The final stage (E and F) of treat-

ment included posterior instrumented fusion with iliac screw pel-
vic fixation. (A and B: Reprinted with permission from Crawford
AH, Herrera-Soto J. Scoliosis associated with neurofibromatosis.
E F
Orthop Clin North Am 2007;38:553562).

A B

Figure 102.10. This 18-year-old patient (A) has many of the skin changes seen in
NF-1. His scoliosis is sharply angular (B), and the dystrophic changes distort the upper
thoracic vertebrae so severely as to make them almost indistinguishable. The coronal
C
section of the MRI (C) shows an intrathoracic meningocele at the apex of the curve.

LWBK836_Ch102_p1062-1077.indd 1076 8/26/11 3:02:06 PM

 Chapter 102 Dysplastic Scoliosis: Neurofibromatosis Spinal Pathology
recognized, asymptomatic lesions should be observed. Surgical
intervention is indicated for progression in size, progressive
bony erosion, pain, neurologic injury, or respiratory distress.
Ligation or excision may be performed via laminectomy or
posterolateral extradural approach. If the meningocele is mas-
sive and producing respiratory symptoms, it should be
approached anteriorly by thoracotomy or video-assisted thora-
coscopy. Consultation with a neurosurgery colleague is highly
recommended.
DUMBBELL LESIONS
A dumbbell lesion is a solitary neurofibroma that grows in
such a way that segments of the tumor are constricted by a nar-
row intervertebral foramen, through the interlaminar space of
the vertebra, or through the dura mater. The tumors may
occur at any level of the cord. Indications for resection are
rapid increase in size, progressive bony erosion, pain, or neu-
rologic symptoms.
ENLARGED VASCULAR CHANNELS
Significant bleeding may occur during spinal surgery because
of plexiform venous abnormalities and increased vascularity
of neurofibromatous tissue. Preoperative planning should
include provision for blood salvage and replacement. Our
patients receive the antifibrinolytic agent tranexamic acid,
and we use high Bovie settings (about 60) to help limit blood
loss. We use various commercial products for hemostasis, such
as those combining gelatin granules with topical thrombin
(Floseal, Baxter). We also make our own paste of thrombin
with gelatin sponge (Gelfoam, Pfizer), cellulose sponge
(Surgicel, Johnson & Johnson Gateway), and microfibrillar
collagen (Avitene, Davol) for this purpose. Routine wound
drainage should be used. Deep hemovac drainage is necessary
in all patients having spinal canal exploration because of
the significant bleeding that may occur once the patient is
normotensive.
SUMMARY REMARKS
Over the past decade there have been remarkable and almost
revolutionary advances in understanding the genetics, bio-
chemistry, and molecular biology of NF-1. Treatment of spinal
disorders for these patients has evolved to incorporate the
increased use of traction, circumferential fusion, pedicle screws,
titanium universal instrumentation systems, and electrophysio-
logic monitoring. The surgical stabilization of these deformi-
ties occasionally requires creativity on the part of the surgeon.
There should be full array of implants including sublaminar
wires, bone morphogenic protein, and methyl methacrylate at
their disposal. The most dangerous situation for the neurologi-
cally intact NF-1 patient and the surgeon is still the instrumen-
tation or manipulation of the spine in the presence of
unrecognized intraspinal lesions. The requirement for preop-
erative MRI or CT myelography of the entire spine simply can-
not be overstated. The patient with NF-1 most often presents
LWBK836_Ch102_p1062-1077.indd 1077
1077
without any neurologic deficit despite even spectacular spinal
deformity. The surgeons responsibility is to stabilize the spine
in the most expedient, safe, and permanent manner without
causing neurologic injury.
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