27 The French Experience
27.1 Classifying Early Onset Scoliosis
The concept of early onset scoliosis, popularized by Dickson et al, 1 is a good one because on the one hand, it is a paradigm of spinal deformity occurring at a young age (yet to be correctly defined) in a child with a large potential for growth. On the other hand, it is not entirely adequate because conditions with many different etiologies are included within the one term (attractive and popular), even though they have very little in common except for the young age of the patients. We cannot compare localized hemivertebra pathology, in which only one or two vertebrae are involved; paralytic scoliosis in spinal muscular atrophy (SMA), for example, in which the entire spine is involved; and idiopathic infantile scoliosis, in which the entire thoracic spine may be involved. So, if the concept of early onset scoliosis is to be retained, we must add the etiologic subgroups when we describe this condition. There are clearly two large groups of patients, and it is essential not to mix them. It is also clear, unfortunately, when one listens to presentations or reads articles for the evaluation of methods of treatment or the evaluation of instrumentation, that no clear distinction is being made, and I, for one, find this hard to accept.
The danger of relying on this “all-encompassing” denomination of early onset scoliosis is that many surgeons rush to embrace a “new surgical treatment” under the auspices of a “miracle cure” and gradually lose the expertise to provide nonoperative care with proper casting and bracing (care that is time-consuming, not well paid, often ignored, and often unrewarding). They don’t realize that by rushing into surgery they “put their fingers in gear,” after which the only escape is repeated surgeries with an exponentially increasing risk for infections and complications as time goes by. So in my view, and generally in France, it is clear that we must categorize cases of early onset scoliosis based on the following:
27.1.1 Age of the Patient
In my experience, the real patients with early onset scoliosis are those in whom the pathology is recognized and treated between birth and 6 years of age (at entry into elementary school). However, we can also create a “pre-puberty” group from 6 to 9 years of age and a “late early” group at the beginning of puberty for diagnosis and treatment, and finally a “post-puberty” group. This scheme may be useful because the treatment strategy is different for each group.
27.1.2 Etiology
Here, we can allocate patients to one of five groups: (1) idiopathic, (2) paralytic and neuromuscular, (3) congenitally malformed, (4). dystrophic, and (5) iatrogenic. It is also necessary to recognize that inside each group there are multiple subdivisions (e.g., postpolio syndrome is different from SMA and from congenital muscular dystrophy, post-laminectomy scoliosis is different from post-thoracotomy scoliosis, etc.). So finally, each patient should be considered as an individual and unique case. This chapter will concentrate on the first age group:– patients with “real early onset scoliosis” (developing between birth and 6 years of age). However, a permanently uniform thought process must be maintained across all ages and etiologies in the mind of the orthopedic surgeon.
27.2 Complete Clinical Examination and Imaging Are Mandatory as the First Step
After general information about the pregnancy, birth, and family has been obtained and the classic orthopedic inquiries and measurements have been made, the assessment continues with the most complete neurologic examination possible, which includes an examination of the cranial nerves and abdominal skin reflexes. The quality of the soft tissues, skin, joint laxity, and so forth must be assessed. Imaging, including regular radiography, computed tomography (CT), and magnetic resonance (MR) imaging, is frequently useful, if not mandatory, not only for a proper diagnosis (e.g., association of a syrinx with supposedly infantile scoliosis) but also for treatment (e.g., when asymptomatic instability is discovered in a congenital malformation of the craniocervical junction in a patient with chondrodystrophic scoliosis). A three-dimensional reconstruction, especially with a view from the top, serves as a very reliable prognostic indicator for distinguishing among the three types of idiopathic scoliosis: the spontaneously regressive type; the progressive “benign” type of Min Mehta, which we are able to correct completely with cast and brace only; and the progressive malignant type, which is not correctable with nonoperative techniques (Fig. 27.1). This important information was presented at the Scoliosis Research Society meeting in Chicago, Illinois, in 1980 but was unfortunately overlooked, as was its publication in the French literature 2 a few years later. This clinical and imaging investigational phase leads to a more precise approach for determining the etiology of the deformity rather than applying a generic label of “early onset scoliosis.”
27.3 General Philosophy about Concerns and Priorities to Address in Patients with Early Onset Scoliosis
The first priority is to identify significant problems affecting the lungs and respiratory function, which is linked to cardiac function, and subsequently thoracic cage development. Thoracic insufficiency syndrome has been well described by Campbell. We now know that alveolar multiplication stops at about 7 to 8 years of age. This syndrome therefore requires early detection and early therapeutic decision making. There is also a need for the early detection, as soon as possible after birth, of any instability of the spine, from the craniocervical junction to the sacrum, that could lead to an irreversible paralytic disorder after even minor trauma.
The second priority is the evaluation of normal or abnormal growth of the skeleton, especially the spine, 3 , 4 which affects the shape of the spinal canal at any level and can compromise the spinal cord, as well as the evaluation of progressive deformities of the entire trunk (scoliosis, lordosis, or kyphosis) and their subsequent effects on vital organ and locomotor function, balance, and cosmesis.
The third priority is the evaluation of the development of the nervous and muscular systems, which are linked to the ability to maintain erect posture when standing or sitting and to locomotion.
The synthesis of all these evaluation techniques must always be in the mind of the orthopedic surgeon. Does the deformity modify the shape of the spinal canal and risk damage to the dural sac or the spinal cord? Particularly in kyphoscoliosis, the inner part of the canal may be regular or may have a step-off. Do the vertebral bodies create a kind of “billot”? Is there any cartilaginous protrusion of the disks? Is there any immediate or potential instability? How is the alignment of the pedicles? of the facets? Is the deformity rigid or flexible? The clinical examination is crucial, particularly the neurologic examination, as well as imaging, including traction, bending, and “billot” films. It is therefore mandatory to perform CT and MR imaging, sometimes with three-dimensional reconstruction, for the initial assessment to determine the etiology. For example, infantile scoliosis can be considered idiopathic until MR imaging demonstrates a syringomyelia, which then changes the diagnosis to neurologic scoliosis.
Another question is, What is the influence of the spinal deformity on the adjacent vital structures? Are they normal or abnormal? Are the malformations cardiac, pulmonary (dysplasia, agenesis), thoracic (rib synostosis or agenesis), muscular (aplasia of the thoracic or abdominal walls, of the diaphragm), or visceral (kidneys or bowels)? What are the effects of all these different surrounding pathologies on the normal life and development of the child? All this must be meticulously assessed before any decision for treatment is made.
27.4 Most Important Principles of Treatment
27.4.1 Nonoperative Procedures
The basic requirements for such treatment in a growing young child are the following 4 : cause no thoracic compression, correct or prevent spinal collapse, attempt to produce a derotation of the deformity without too much constraint on the body structures, and allow the most normal life for the child (playing, walking, running) so as to produce the fewest possible psychological adverse effects.
Casting
Casting (Fig. 27.2) is by far the technique of choice, to be started as soon as the deformity is noticeable, in some cases soon after birth. It is always done while general anesthesia is administered via a nasotracheal tube because traction to the head– pelvis unit (always mild) is required (the patient’s teeth will crush the tube if intubation is done through the mouth). Mild traction on the casting frame makes the procedure easier for the patient, technician, and surgeon. It also reduces the rate of respiratory and digestive complications. The child’s body is meticulously draped with two or three cotton jerseys, and particular care is taken to avoid creating folds in contact with the skin.
When the deformity is relatively flexible, the EDF (elongation, derotation, and flexion) technique, with the use of linen straps and hand moulding, is our preferred method. During the preparation, we place pieces of felt and padding around the thorax, particularly below the clavicle area, to be removed when the cast is dry, giving room for thoracic expansion and preventing excessive vertical orientation of the ribs. The pressure on the ribs is applied to the convex side below the apex in a posterolateral direction. Windows are created in the cast to allow pressure and counterpressure, produce the desired derotation effect, and minimally restrict the vital capacity.
When the deformity is large and stiff, immediate traction and derotation bands alone will not work. It is necessary to reduce the curve progressively by using the elongation cast of Donaldson and Stagnara. The principle of this cast is to apply progressive distraction between the head and the pelvis through two turnbuckles fitted between the two parts of the cast and turned twice a day by half a turn, causing no more than 4 to 5 mm of elongation each day. This technique is similar to that previously described, with four precise points to be located:
A meticulous and precise moulding by hand of the pelvic girdle.
The same careful moulding of the occipitomandibular fitting.
The addition of sufficient layers of felt and padding all around the thoracic cage, which are removed after the cast dries to allow the skin to be free of pressure and the rib cage to have plenty of room for motion.
Minimum of traction to the head–pelvis unit when the patient is on the cast frame.
For patients with very stiff curves, we recommend adding a halo ring to the skull and proceeding to elongation with the child in a lying position and 3 to 4 kg of traction on the halo, which is generally kept on during the night. The halo is not linked to the cast in order to allow rotation of the head so as to prevent degenerative changes of the posterior facets of the cervical spine. This technique addresses the viscoelasticity of all the soft tissues surrounding the spine skeleton (ligaments, joints capsule, tendons, and muscles). It is used in conjunction with respiratory positive pressure ventilation machines and is continued until improvement in the respiratory measurement graphics reaches a plateau. Treatment should be continued with localized surgery, as in the case of a congenital dystrophic anomaly, or if there is no surgical indication, we change the cast to a new one made according to the EDF principles. The cast is changed as many times as necessary (generally every 3 months) until brace treatment is started. Local hygiene of the skin under the cast is essentially maintained by a regular change (every 15 days) of the thin cotton jersey in contact with the skin.
Bracing
Braces for young children must fulfill many requirements:
They must be easy to fit by only one person (generally the mother).
There must be no constriction of the thoracic cage in order to prevent respiratory compromise.
Bracing is better done after primary casting.
If possible, the braces must be adjustable in height, width, and body thickness to follow growth for a minimum of 1 year.
Their action must be passive or active, or both, according to the etiology.
The Garchois brace (Fig. 27.3) with or without a chin piece is my favorite for all types of early onset scoliosis, especially when the patient has poor-quality muscles. Monovalve braces or thoracolumbosacral (TLSO) types are difficult to use and often harmful for the thorax. The Milwaukee brace is good for patients with normal muscles but requires a very good and trained physiotherapist. For an underarm brace when muscles are normal, the three-dimensional carbon brace with its derotational and kyphogenic effects is recommended in cases of significant lordoscoliosis. All braces are custom-made, and the expertise of the orthotist is crucial.
Respiratory assistance with machines and physiotherapy is also an important part of the treatment of early onset scoliosis and is better done under the guidance of a pediatric respiratory physician.
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