Aims

We wished to examine the effectiveness of tibial lengthening using a two ring Ilizarov frame in skeletally immature patients. This is a potentially biomechanically unstable construct which risks the loss of axial control.

Introduction Spinal cord monitoring in posterior scoliosis surgery has become a standard of care. It has been our practice since 1999, to monitor the somato-sensory potential (SEP) and motor evoked potential (MEP) in all posterior cases. We report on and discuss the meaning of alteration in the spinal cord monitor signal that occurred in 15 cases from a total of 165 procedures.

Methods This is a retrospective review of patients from a hospital database. Over a six year period, 167 posterior scoliosis instrumented fusion procedures were performed by paired combinations of the four authors. In 13 cases we have been alerted to a change in one or both signals during the procedure. Associated with these, were two cases of intra-operative cardiac arrest, and six cases of post-operative neurological deficit. All patients remain under continued regular review.

Results In the two cases of intra-operative cardiac arrest, the SEP and MEP signals were lost approximately three minutes prior to the arrest. Both patients had neurological deficits post-operatively, one has totally recovered, and one has a residual complex regional pain syndrome of the right leg. This last patient is the only one of six who has not had complete resolution of the post-operative neurological deficit. In five of the six cases who sustained post-operative neurological deficits, the SEP and usually the MEP was lost and did not return. In the sixth case, the SEP did return. In the remaining seven cases, there were changes of decreased amplitude or increased latency in the SEP or MEP that did not appear to result in a postoperative clinical consequence, however; in two patients, signal changes were directly related to changes in blood pressure, and in two other patients, signal changes were directly related to concave hook placement.

Discussion On review of the management and outcome of these cases, we conclude that profound hypotension will alter the SEP and may herald a catastrophic cardiovascular or neurological event. Furthermore, the modality of continuous spinal cord monitoring can provide specificity in the diagnosis of an actual or impending neurological insult and allow for appropriate and timely intervention. We believe spinal cord monitoring in the posterior approach for spinal deformity is an invaluable tool, and is in fact, mandatory for all idiopathic and ambulant non-idiopathic spinal deformities.

Introduction Chicken studies implicate pinealectomy within a week of hatching as a cause of scoliosis. The nature of the scoliosis has been demonstrated to be similar to that of human idiopathic scoliosis. Scoliosis was not induced following pinealectomy in Rhesus monkeys (primate model). No human studies have been reported. The aim of this study is to determine if idiopathic scoliosis is associated with treatment for pineal lesions (presumably resulting in pinealectomy) in a human paediatric population.

Methods A medical records search was performed in five Australian States for pineal lesions. Identified patients underwent clinical or radiological evaluation for scoliosis. Pathology varied from germ cell tumour, germinoma, pineoblastoma, teratoma to a pineal cyst and an epidermoid cyst. Treatment ranged from biopsy/ extirpation to radiotherapy/chemotherapy.

Results Of 48 identified patients, thirteen are deceased. No scoliosis was present in the last imaging of the deceased. The mean age at presentation was 9.7 years (range 1–18 years). Ten are female. Two males have idiopathic scoliosis (4.2%). One has a 12° right upper thoracic curve (with 32° kyphosis) and the other has a 60° right thoracolumbar curve, requiring a two-stage arthrodesis

Discussion Although the incidence of idiopathic scoliosis in this cohort is greater than expected from Caucasian population studies (2–3%), it is not typical idiopathic-type, which has a female preponderance of larger deformities. New-born chicken studies demonstrate an incidence of between 50 and 100% scoliosis in the three months following pinealectomy. Chickens of both sexes are involved. Fundamental differences exist between chicken and human/primate models including the age at pinealectomy and the anatomical site of the pineal gland. Chickens have a naturally lordotic thoracic spinal curvature whilst humans/primates have a naturally kyphotic thoracic spine. Adolescents with idiopathic scoliosis have either thoracic hypokyphosis or a thoracic lordosis. Contrary to current beliefs, no causal link can be established between pineal lesions and the development of idiopathic scoliosis in a paediatric population.

Introduction Delayed puberty and delayed skeletal maturation have been implicated as risk factors for the progression of idiopathic scoliosis. Genetic defects (Turner syndrome) and hypothalamic- pituitary disorders are known causes of delayed puberty. Although it is recognized that the incidence of idiopathic scoliosis is elevated in Turner syndrome, human studies regarding the incidence/severity of scoliosis in children with suprasellar, hypothalamic region and pituitary tumours/ disorders is deficient.

Methods A medical records search in five Australian states for suprasellar, hypothalamic region and pituitary tumours/disorders was performed. Identified patients underwent clinical or radiological evaluation for scoliosis. Pathology varied from suprasellar-hypothalamic region tumours, pan-hypopituitarism, pituitary tumours and growth hormone deficiency as well as a craniopharyngioma, arachnoid cyst, retinoblastoma and encephalocele.

Results Of 23 identified patients, ten are female. Mean age at presentation was 8.4 years. Three have right thoracic scoliosis with a Cobb angle less than 20 degrees. Two are males; one with pituitary hormone deficiency and the other with Cushing’s disease treated with radiotherapy. The only female is on a growth hormone treatment program for idiopathic growth hormone deficiency.

Discussion The only female with scoliosis was 12 years old. Delayed puberty could not be linked to either male with scoliosis. Although the incidence of idiopathic scoliosis in this cohort is greater than expected from Caucasian population studies (2–3%), the male preponderance is unusual. No relationship between delayed skeletal maturation and idiopathic scoliosis could be established.

Over a nine-year period, 20 feet with persistently symptomatic talocalcaneal coalition were treated by resection of the bar. The 17 patients were all under 16 years of age. Excellent or good long-term results were achieved in the ten feet in which preoperative coronal CT had shown that the area of coalition measured 50% or less of the area of the posterior facet of the calcaneum. In these feet heel valgus was less than 16 degrees and there were no radiographic signs of arthritis of the posterior talocalcaneal joint. Talar beaking was present in 70% of these feet but it did not impair the clinical result. Fair or poor results were observed in the ten feet in which preoperative CT had shown the area of relative coalition to be greater than 50%. In these feet, heel valgus was greater than 16 degrees and most had narrowing of the posterior talocalcaneal joint and impingement of the lateral process of the talus on the calcaneum.

We have evaluated two methods of surgical treatment of adolescent idiopathic double major scoliosis in 59 patients. In group 1, 31 patients were treated by fusion of the upper curve only. In group 2, 28 patients had lumbar fusions also including most of the lower curve. The magnitude of the lower curve and the correction obtained in traction were good indicators of the correction achieved postoperatively. Forty-four patients were reviewed at a minimum of 10 years after operation. Those in group 1 showed sustained improvement of the lower curve with minimal stiffness and pain. Group 2 patients had lumbar curves of similar severity at review, but had significantly more low back pain and stiffness. The number of lumbar segments which remained mobile appeared to be a critical factor in determining the outcome. Selective fusion of the upper curve in double major scoliosis produces satisfactory results if the lumbar curve is less than 50 degrees. It may also be appropriate for flexible lumbar curves of larger angle.

Sixty-nine patients with congenital abnormality of the femur were reviewed. Their manifestation of femoral dysplasia ranged from an intact femur approximately 60 per cent of the length of the normal leg to a subtotal absence of the femur in which only the femoral condyles remained, often with a congenital fusion of the knee joint. Two groups were defined: Group I consisted of those with congenital hypoplastic femur in which the hip and knee could be made functional and where, in some patients at least, leg equalisation was possible; Group II consisted of those with true proximal focal femoral deficiency where the hip joint was never normal and the knee joint was always useless. The patients in each group were examined and evaluated with respect to clinical signs, surgical procedures performed, and prosthetic requirements and function. A protocol of treatment for both groups is suggested.

The operative technique for combined fusion of the knee and rotationplasty of the limb in the management of congenital deficiency of the femur is presented. The technique described allows earlier definitive prosthetic fitting of a child with proximal femoral deficiency; it has reduced the number of operative procedures needed to obtain the optimal function from that deficient limb; and it has enabled these procedures to be performed at an earlier age. The technique differs from those previously described and represents a significant improvement in management of the patient with femoral deficiency.