1. Analysis of eighty-one patients with neurofibromatosis showed that sixty-two (76 per cent) had café-au-lait markings; 12 per cent had significant spinal deformity. 2. Thirty-three examples of spinal deformity in neurofibromatosis showed a wide variety of patterns and severity of the adult curve. There was no evidence that there was any recognisable pattern of scoliosis in neurofibromatosis. No evidence was discovered to suggest that any acquired local abnormality of bone contributed to the deformity. 3. Some of the severe deformities showed a pattern similar to that seen in the congenital sco1ioses, and this might be the link between the neurofibromatosis and the spinal deformity.
Four cases are reported in which infantile idiopathic structural scoliosis gradually decreased during the period of active growth.
The etiological factors concerned in paralytic scoliosis are complex. Four main types of paralytic scoliosis can be recognised. 1. The general C-curve due to the body's anatomical attempt to shift its centre of gravity towards the weaker side. Vertebral rotation is not usually marked. This type usually occurs when patients with relatively slight paralysis have been allowed up too early ; it does not usually progress to severe deformity but may occasionally do so, gradually changing into Type 2. This type usually responds well to a period of rest and muscle redevelopment in recumbency. It also responds favourably to correction and fusion because correction is easy and there is little tendency to deterioration. Many of the "successes" of correction and fusion are in this class—almost equal success would often have been gained without "correction." The spine is slightly, but not very, unstable and a relatively localised fusion will give the little extra support that is needed. 2. The "general collapse" type of curve due to extensive spinal weakness. This is the type in which simple head suspension produces marked correction. Rotation is moderate. Provided the patient's general condition is satisfactory extensive spinal fusion is usually the best treatment and produces gratifying improvement. 3. The primary lumbar curve due to a combination of pelvic obliquity, extraspinal imbalance and imbalance of the deep rotator muscles. Rotation is usually marked. Treatment must include the correction of all these factors. In mild cases correction of the pelvic obliquity is enough, but in marked cases the spine must also be corrected. The disability from a lumbar paralytic scoliosis is much greater than that from a lumbar idiopathic scoliosis of the same degree; so correction is necessary in this type. Correction in a Risser-type jacket is often inadequate and recourse to operative correction is usually required. 4. The primary thoracic curve—often associated with weakness of the scapular muscles. The indications for and methods of treatment are practically the same as in primary idiopathic thoracic curves. These curves tend to be progressive and uncompensated. Although the most popular treatment is correction and fusion, wedge osteotomy of the spine gives better correction in intractable cases. The main need is for further investigation into the etiology of paralytic scoliosis so that adequate preventive measures may be undertaken at an early stage. It is essential that every child who contracts poliomyelitis should have his back muscles examined before he gets up. If there is any suggestion of scoliosis further investigations including radiography and electromyography are essential.
1. The prognosis of paralytic scoliosis has been studied by defining curve patterns and establishing the natural development as seen in fully grown patients who have not had surgical correction. 2. The prognosis, unlike that in idiopathic scoliosis, is related to the age of onset of the curvature and the degree of muscle imbalance rather than the site of the primary curve. 3. Paralysis of limb muscles is shown to be unrelated to the development of scoliosis. The intercostal muscles and the lateral abdominal flexors produce scoliosis when weaker on the convex side of the curve. Gravity and the other trunk muscles certainly play a part in the development of lumbar curves but their importance is difficult to assess.
The evolution of an idiopathic scoliosis is determined by the site of the primary curve and by the age of onset. It is significant that thoracic primary curves are commonly severe and the early onset of this curve accentuates this feature. Early operation based on prognosis is practised but sufficient time has not yet elapsed to justify any conclusions.
1. Five cases of scoliosis with paraplegia are reported, and thirty-six comparable cases from the literature are reviewed. These forty-one cases have been studied with the object of determining the etiology of scoliosis, the reason why cord compression sometimes develops, and the results of conservative and operative treatment of such compression of the cord. 2. The cause of paraplegia is nearly always compression of the spinal cord by the dura, which, in severe scoliosis, is under longitudinal tension because of its firm attachment to the foramen magnum above and the sacrum below. Such tension, resisting displacement of the spinal cord from the straight line, may be shown to cause incomplete spinal block even when there is no paralysis. 3. When paralysis occurs it usually develops during the years of most rapid growth, the tight dura being unable to accommodate itself to the rate of growth of the spinal column; cord compression is probably increased by narrowing of the dural sac by rotational displacement. 4. The most striking results have been secured by laminectomy with section of the dura and sometimes division of dentate ligaments and tight nerve roots. After such division there is evidence of release of compression: the cord herniates through the dural slit; and spinal pulsation returns. 5. It is important to control bleeding in order to avoid post-operative compression by blood clot; and to prevent leakage of cerebro-spinal fluid through the arachnoid. 6. It is unwise to perform spinal fusion at the same time as decompression because it increases the danger of haematoma formation. Moreover the improvement gained by decompression is maintained even if no fusion of the spine is performed. 7. Conservative treatment of scoliosis with paraplegia should not be continued for long periods unless there is evidence of early and progressive improvement because prolonged compression causes irreversible changes in the cord. 8. In three cases, paraplegia was not due to dural compression: one turned out later to be a case of syringomyelia; one, reported by Heyman, was due to the pressure of a bone spur; and one, reported in this series, was due to a congenital tight band of developmental origin which might have caused the scoliosis as well as the paralysis, and in which, after resection of the band, recovery from the paralysis was complete.
Conventional growing rods are the most commonly
used distraction-based devices in the treatment of progressive early-onset
scoliosis. This technique requires repeated lengthenings with the
patient anaesthetised in the operating theatre. We describe the
outcomes and complications of using a non-invasive magnetically
controlled growing rod (MCGR) in children with early-onset scoliosis.
Lengthening is performed on an outpatient basis using an external remote
control with the patient awake. Between November 2009 and March 2011, 34 children with a mean
age of eight years (5 to 12) underwent treatment. The mean length
of follow-up was 15 months (12 to 18). In total, 22 children were
treated with dual rod constructs and 12 with a single rod. The mean
number of distractions per patient was 4.8 (3 to 6). The mean pre-operative
Cobb angle was 69° (46° to 108°); this was corrected to a mean 47°
(28° to 91°) post-operatively. The mean Cobb angle at final review
was 41° (27° to 86°). The mean pre-operative distance from T1 to
S1 was 304 mm (243 to 380) and increased to 335 mm (253 to 400)
in the immediate post-operative period. At final review the mean distance
from T1 to S1 had increased to 348 mm (260 to 420). Two patients developed a superficial wound infection and a further
two patients in the single rod group developed a loss of distraction.
In the dual rod group, one patient had pull-out of a hook and one
developed prominent metalwork. Two patients had a rod breakage;
one patient in the single rod group and one patient in the dual
rod group. Our early results show that the MCGR is safe and effective
in the treatment of progressive early-onset scoliosis with the avoidance
of repeated surgical lengthenings. Cite this article:
This is a study of children who first attended as infants with either progressive infantile idiopathic scoliosis or congenital scoliosis. All had a pattern of scoliosis in which early and damaging deterioration is inevitable. The infants were treated from before the age of three, initially by plaster casts and then a Milwaukee brace, followed at about the age of ten by correction and fusion. The cases were then observed to the end of growth or near that point. In the main study there were twelve cases, six of progressive infantile idiopathic scoliosis and six of congenital scoliosis, which were followed through this long period. Only one of the twelve had a curve worse at the end of growth compared with the initial radiograph as an infant; this one curve had increased only 16 degrees in almost as many years. Although small, the series does show that it is nearly always possible to control even the most serious scoliosis in an infant, if it is tackled early and unremittingly. There are supportive studies of children who have partially completed this regime, and interim results in a newer group of children with spina bifida and scoliosis.
An attempt to produce scoliosis in young baboons by excision of the heads of ribs failed in thirteen growing animals observed for up to a year after operation. Other investigators have failed to produce scoliosis in primates by similar and other techniques that had successfully produced scoliosis in quadruped animals. The possible reasons for this are discussed, especially in the light of clinical trials that are being carried out with techniques transposed from the quadruped experimental animal to the scoliosis clinic.
An attempt has been made to determine the aetiological factors in infantile idiopathic scoliosis from a clinical, genetic and epidemiological survey of 134 infants, ninety-seven of whom developed a curve in the first six months of life. Plagiocephaly was present in all cases; mental retardation occurred in 13 per cent of males with progressive scoliosis; congenital dislocation of the hip occurred in 3.5 per cent of cases and congenital heart disease in 2.5 per cent; and inguinal hernia was found in 7.4 per cent of males. Approximately 3 per cent of parents and 3 per cent of sibs had the same deformity, thirty times the general population frequency for the Edinburgh area. Other positive findings included an excess of breech presentations and of premature, low birthweight males, and a preponderance of curves developing in the winter months. Infants with progressive scoliosis tended to have older mothers and to come from poorer families. Only three children, all with resolving scoliosis, habitually lay prone in early infancy, in marked contrast to North American infants where this posture is usual. The almost complete absence of infantile idiopathic scoliosis in North America is noted and it is thought that the two facts may be related. The aetiology is likely to be multifactorial, with a genetic tendency to the deformity which is either "triggered off" or prevented by external factors.
1. Eleven patients with juvenile rheumatoid arthritis, most of them young adults at a terminal stage, were found to have structural scoliosis with curves measuring between 20 and 80 degrees. 2. The common feature was severe and protracted rheumatoid disease. 3. The characteristics of the spinal curves are analysed; the longer curves may have been caused by muscle imbalance and the shorter curves possibly by asymmetrical involvement of the inter-apophyseal joints. 4. It is suggested that juvenile rheumatoid arthritis is an unusual etiological factor of scoliosis.
1. The results of 243 thoracoplasty operations are discussed. It was found that scoliosis developed in over 99 per cent of cases and that the curve was convex towards the side of operation. The angle of curvature correlated closely with the number of ribs removed. 2. If the head, neck and tubercle of the rib and the transverse process of the corresponding vertebra were all removed the degree of scoliosis was increased, whereas if a thoracoplasty was performed with apicolysis and embedding of the scapula the spinal deformity was less. Possible explanations for these phenomena are discussed. 3. No evidence was found to support the theory of causation by imbalance between the paired "pneumatic cylinders" (lungs and thoracic cage) supporting the thoracic spine. 4. The scoliosis was found to progress for many years; some factors influencing this course are discussed. 5. No correlation was found between the age of the patient at the time of thoracoplasty and the degree of subsequent scoliosis, but severe spinal deformity can arise even in older adults.
1. Idiopathic scoliosis is a familial condition. 2. The findings suggest either dominant or multiple gene inheritance, but a larger series is needed before a firm conclusion can be drawn. 3. The infantile and adolescent types of scoliosis seem to share the same basic etiology, because their families contain instances of each. 4. Infants with resolving scoliosis have affected relatives in the same proportions as in the main group, suggesting this is a mild form of the same disorder. 5. In this series all infants seen with scoliosis under one year of age had plagiocephaly, which was usually transient. 6. Mental defect and epilepsy are the commonest findings associated with scoliosis. 7. In adolescent scoliosis the age of the mother is significantly raised by comparison with the expected figure for the normal population.
1. Evidence is presented that the basic lesion in scoliosis is relative lengthening of the anterior components of the spine compared with the posterior elements. 2. The logical treatment is to reduce this relative lengthening either by lengthening the posterior elements or shortening the anterior elements. This may be achieved by anterior lumbar wedge osteotomy or by epiphysiodesis; and correction of lumbar lordosis can improve a thoracic scoliosis.
"Wedge excision" of the apex of the curve is the rational way of correcting a scoliosis. It is a straightforward procedure which is successful in practice.
Diastematomyelia is a rare congenital abnormality
of the spinal cord. This paper summarises more than 30 years’ experience
of treating this condition. Data were collected retrospectively
on 138 patients with diastematomyelia (34 males, 104 females) who
were treated at our hospital from May 1978 to April 2010. A total
of 106 patients had double dural
