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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_31_библиотеки_им_акад_М_И_Перельмана
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Fig. 22.3 Fulcrum
A. Şenköylü
22.5 Differential Diagnosis
22 Scheuermann’s Disease
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22.6 Treatment Options
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22.7 Expected Outcomes
22.8 Potential Complications
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Fig. 22.4 Postoperative
A. Şenköylü
22.9 What Should Patient andFamily Know?
Further Readings
Cervical Kyphosis inNeurofibromatosis
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Type I
FedericoCanavese
23.1 Definition
Children with neurobromatosis type I (NF-1) can develop cervical kyphosis (CK).
Severe CK in the setting of NF-1 is a rare manifestation of the disease in the pediatric population, and it is often associated with vertebral dysplasia.
23.2 Natural History
The natural history of patients with CK secondary to NF-1 is not well known due to
the rarity of the condition within this patient’s population.
23
23.3 Physical Examination
Clinical examination reveals a short and hypo-mobile neck. Symptomatic patients
with CK can experience pain or neurological decits although some of them may
have a surprisingly high tolerance for deformity with few symptoms even if spondyloptosis (dislocation of one vertebral body over another) is present.
Supplementary Information The online version contains supplementary material available at
(https://doi.org/10.1007/978- 3- 030- 80356- 8_23).
F. Canavese (*)
Department of Pediatric Orthopedic Surgery, Lille University Center, Jeanne de Flandre
Hospital, Lille, France
Faculty of Medicine Henri Warembourg, Nord-de-France University, Lille, France
© The Author(s), under exclusive license to Springer Nature
Switzerland AG 2022
A. Şenköylü, F. Canavese (eds.), Essentials of Spine Surgery,
https://doi.org/10.1007/978-3-030-80356-8_23
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F. Canavese
A neurological evaluation includes an assessment of pain, numbness, paresthesia, extremity sensation and motor function, muscle spasm, weakness, gait disturbance, and bowel/bladder changes (Video 23.4).
23.4 Imaging
The radiographic diagnosis of CK is straightforward. Lateral radiographs of the
cervical spine show severe CK with dystrophic changes of the vertebrae (Fig.23.1).
The kyphosis can be at the upper or at the sub-axial cervical spine, and it is always
rigid in exion-extension (Video 23.6). Characteristic dystrophic changes are scalloping of the posterior vertebral margins, spinal canal widening, enlarged neural
foramina, defective pedicles, and spindling of the transverse processes.
Computed tomography (CT) scan provides useful information about the osseous
and three-dimensional anatomy of the cervical spine, and it allows estimating the
severity of dystrophic changes in the cervical spine.
Magnetic resonance imaging (MRI) helps to obtain information on soft tissues
and the spinal cord. In particular, MRI can detect coexisting paraspinal and spinal
cord tumors and can evaluate the anatomy of the spinal canal and its relationship
with the cord, the status of the spinal cord, and the presence of dural ectasia
(Fig.23.1). It is important to stress out that MRI must include the brain and the
whole spine, including the sacrum.
It is possible that the patients with NF-1 may have an aneurysm at the vertebral
arteries. Thus, before starting traction, patients should be evaluated by MR
a
Fig. 23.1 A patient with NF-1 and cervical kyphosis (a); MRI shows kinking of the cord and
dural ectasia (b); anterior strut graft (tibia; c)
b
c
23 Cervical Kyphosis inNeurobromatosis Type I
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angiography or contrast-enhanced CT to rule out an aneurysm at the vertebral arteries due to the risk of rupture during traction.
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23.5 Differential Diagnosis
None.
23.6 Treatment Options
Patients with progressive deformity or symptoms should be offered surgery; combined anterior-posterior fusion is indicated in most skeletally immature NF-1
patients with severe CK. Preoperative halo-gravity traction or halo-vest (Video
23.1) is useful to achieve a gradual, although partial, and safe correction of the
CK.In patients with NF-1 smaller traction forces should be used because of the
fragility of the cervical spine due to dystrophic changes.
Fusion can be associated with vertebral osteotomy to restore the sagittal alignment
of the cervical spine (20% to 25% risk of new neurologic decits), or it can be achieved
“in-situ” with an anterior (strut graft) and posterior bone graft (Fig.23.1). Halo should
be maintained postoperatively up to 10 to 12weeks as it is possible that the anterior
strut graft can partially dislodge at halo-removal (Videos 23.1, 23.2, 23.3 and 24.4).
23.7 Expected Outcomes
Signicant radiographic correction can be achieved with corrective surgery although
it carries a relatively high risk of new neurologic injury. On the other hand, fusion
“in-situ” does not attempt to correct the deformity above the correction obtained
with traction, and it has almost no risk of new neurological injury. Whatever the
technique, erosion of bone usually continues after surgery and bone graft can get
resorbed leading to revision surgery with new bone graft application (posteriorly in
most cases, though anterior surgery may be necessary too).
23.8 Potential Complications
The onset of pain and neurologic symptoms are possible. The presence of coexisting paraspinal and spinal cord tumors increases the risk of hemorrhaging and spinal
cord injury during corrective surgery. Post-operative hematoma, causing breathing
difculties by compressing the airways, is possible and therefore patients should be
monitored carefully.
Malignant transformation of neurobroma into neurobrosarcoma (nerve sheath
tumors) is possible throughout the whole life of the patient (Chap. 59).
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F. Canavese
23.9 What Should Patient andFamily Know?
Close follow-up is extremely important because complications and deformity progression are frequent.
Further Readings
Helenius IJ, Sponseller PD, Mackenzie W, etal. Outcomes of spinal fusion for cervical kyphosis in
children with neurobromatosis. J Bone Joint Surg Am. 2016;98(21):e95.
Crawford AH, Schumaier AP, Mangano FT.Management of cervical instability as a complication
of neurobromatosis type 1 in children: a historical perspective with a 40-year experience.
Spine Deform. 2018;6(6):719–29.
Congenital Kyphosis
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24
MichaelRuf
24.1 Definition
Congenital kyphosis is a sagittal plane deformity caused by malformations taking
place during the early embryological period (Chap. 12). Posterior hemivertebra
(failure of formation) leads to short angulated kyphotic deformity while anterior bar
(failure of segmentation) leads to anterior vertebral body coalition. As a consequence, the anterior and posterior growth of the spine is not synchronous (unbalanced; posterior>anterior) and induces progressive kyphosis. Mixed deformities
are frequent. A further cause of congenital kyphosis is the hereditary absence or
weakness of the posterior structures of the spine (failure of the posterior tension band).
24.2 Natural History
Congenital kyphosis and kyphoscoliosis usually tend to progress rapidly, resulting in severe deformity (2.5°-5°/year in deformity secondary to failure of formation, more rapid in mixed type deformity). Progression of the curve accelerates
during the adolescent growth spurt while it slows down at skeletal maturity [1].
The thoracolumbar (T-L) junction is the most affected spinal segment; in particular, increased kyphosis at the T-L junction leads to thoracic hypo-kyphosis and
lumbar hyper- lordosis needed to maintain trunk balance.
Supplementary Information The online version contains supplementary material available at
(https://doi.org/10.1007/978- 3- 030- 80356- 8_24) .
M. Ruf (*)
Center for Spine Surgery, Orthopedics, and Traumatology, SRH Klinikum KarlsbadLangensteinbach, Karlsbad, Germany
e-mail: Michael.Ruf@srh.de
© The Author(s), under exclusive license to Springer Nature
Switzerland AG 2022
A. Şenköylü, F. Canavese (eds.), Essentials of Spine Surgery,
https://doi.org/10.1007/978-3-030-80356-8_24
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Congenital kyphosis has a much higher risk to develop neurological decits
compared to congenital scoliosis: the short angulation acts as a hypomochlion
impairing the spinal cord over time resulting in myelopathy. It is important to stress
out that patients with congenitally dislocated spine (CDS) can develop severe neurologic symptoms following minor trauma; a proportion of patients with CDS may
injure the spinal cord in utero.
M. Ruf
24.3 Physical Examination
Physical examination includes documentation of
– Location and severity of the kyphosis.
– Tenderness on palpation/percussion.
– Compensatory lordotic spinal segments.
– Trunk balance (plumbline).
– Flexibility of the kyphotic segment (active and passive correction).
– Neurological examination (Video 24.4); if necessary, the realization of motor-
evoked (MEP) and somatosensory-evoked potentials (SSEP) is recommended.
– Pulmonary function test.
– Clinical pictures of the patient.
24.4 Imaging
Full-length standing anterior-posterior and lateral radiographs of the spine (including the hips) demonstrate the kyphotic deformity as well as the compensatory
changes (thoracic hypo-kyphosis and lumbar hyper-lordosis) (Video 24.6). Lateral
radiographs allow measurement of the local kyphosis due to the malformation as
well as the total thoracic kyphosis, the lumbar and cervical lordosis, the pelvic incidence and pelvic tilt, the sacral slope, and the plumbline. Lateral radiographs with a
fulcrum at the apex of the kyphosis are useful to evaluate the exibility of the
deformity.
Magnetic resonance imaging is mandatory to rule out compression of the spinal
cord as well as signs of myelopathy.
Computed tomography scan with three-dimensional reconstruction is important
to evaluate the anatomy and the location of the malformation.
24.5 Treatment Options
Early treatment is required, as congenital kyphosis is usually progressive and the
risk of neurologic impairment is high. Conservative treatment is not effective: neither a brace, or cast, nor physiotherapy can stop the progression of the deformity;
24 Congenital Kyphosis
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delay in treatment will allow progression of the deformity and of the compensatory curves.
Surgery is the treatment of choice. It should be performed early in order to avoid
rigid deformity as well as neurologic impairment.
Surgical options include posterior-anterior and single-posterior procedures.
Anterior-only correction without posterior instrumentation will result in progressive
kyphosis during growth.
In the case of the posterior hemivertebra causing kyphosis, the abnormal vertebra
is resected through a posterior approach. The adjacent discs are removed, and an
anterior spacer/cage is inserted. Stabilization and correction are achieved by posterior compression instrumentation (Chap. 12); the anterior bar can be resected from
posterior or an additional anterior approach is performed (Fig.24.1).
The apex of the congenital deformity tends to fuse over time. Thus, delayed
treatment is more challenging, requiring an osteotomy to achieve correction. A posterior wedge osteotomy or vertebral column resection can usually be performed via
a single-posterior approach, since the apex of the deformity is shifted backward [2,
3]. An anterior approach may be necessary in revision cases or in case of abnormal
course of the great vessels. The spinal cord has to be handled with extreme care; the
use of intraoperative monitoring is strongly recommended. Tension or translation
must be avoided. Posterior instrumentation in combination with stable anterior support provides safe and reliable stability (Fig.24.2) (Videos 24.1 and 24.3).
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24.6 Expected Outcomes
Without corrective surgery, reduction of pulmonary function and progressive neurologic compromise have to be expected without treatment in most cases. Early treatment can restore a physiological sagittal prole, thus avoiding the development of
neurologic impairment. Delayed treatment requires more extensive surgery, with a
higher risk for neurological decits and poorer outcomes with respect to trunk
balance.
24.7 Potential Complications
Neurological risk is substantial in severe cases requiring extensive osteotomies for correction, especially in combination with pre-existing myelopathy, or in revision cases.
24.8 What Should Patient andFamily Know?
Congenital kyphosis is a serious disorder with a potentially dramatic outcome. In
mild deformity, regular follow-up is needed; in case of progression, early surgery is
recommended.
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