A combination of hemivertebrae and diastematomyelia is rare. We have identified 12 such patients seen during a period of 11 years in the orthopaedic, spinal and neurosurgical units in Nottingham and analysed their treatment and outcome.

Study Design. An observational study in patients with congenital spinal deformity. Objective. To review the relationship between hemivertebrae and the posterior spinal elements in congenital spinal deformity. Summary of background data. The vertebral body anomalies of congenital scoliosis have been classified and their natural history is well known. More recently a variety of posterior element anomalies have been classified. The relationship between posterior and anterior structures is important when posterior excision of a hemivertebrae is undertaken. Methods. Patients recorded as having congenital spinal deformity were identified from a database. All available imaging was obtained and reviewed with particular reference to posterior element anatomy as visualised on CT or MRI scans. Results. In total 36 patients with congenital spinal deformity were identified and their imaging was reviewed. 29 hemivertebrae were identified in 24 patients. Clear images were obtained of the posterior elements in 18 cases. 9 isolated hemilaminae (as visualised on coronal CT or MRI) were seen in 8 patients with associated hemivertebrae. In 7 of 9 cases the hemilamina was found to be one level cranial to the hemivertebrae and in the remaining 2 cases it was at the same level. Conclusion. When planning excision of a hemivertebra, whether this is performed by a posterior approach or by combined anterior and posterior surgery, care must be taken to avoid wrong level surgery. Pre-operative assessment with CT and MRI scans is essential. The surgeon must be aware of the high possibility that the posterior element anomaly may be proximal to the vertebral body anomaly both when considering how to excise the hemivertebra and for planning the fixation levels. Statement of interests: The authors declare that they have no competing interests

The purpose of this study was to ascertain whether early diagnosis through antenatal ultrasound screening and intervention lead to a reduction in morbidity associated with congenital scoliosis. Prospective cohort study. All fetuses with vertebral body anomalies detected over a thirteen years period were included. Maternal risk factors, accuracy of antenatal diagnosis, associated fetal anomalies and ultimate outcome are described. Twenty-four fetuses with congenital hemivertebrae were identified from 39,000 antenatal scans (incidence 0.061%). The mothers' median age at conception was 26 years (range 18-40 years). The median fetal gestational age at diagnosis was 20 weeks (range 18-38 weeks). The median gestational age at delivery was 38 weeks (range 27-40). Antenatal ultrasound identified eighteen fetuses with a single hemivertebra, six fetuses with multiple hemivertebrae. Eleven patients (50%) have undergone early surgical intervention with median post-natal follow up was 4 years (range 0-9 years). The median age at surgery was 12 months (range 1.2 - 47 months). The median pre-operative Cobb angle was 33 degrees and the median Cobb angle at final follow up was 25 degrees. In this series 50% of patients required surgical intervention. We felt that pre-natal diagnosis of congenital vertebra abnormally has resulted in early surgical intervention using less complex surgical technique to halt progressive deformity. However, further studies are required to compare the results with the abnormally not detected antenatally

There is a wide range of reports on the prevalence of neurological injuries during scoliosis surgery, however this should depend on the subtypes and severity of the deformity. Furthermore, anterior versus posterior corrections pose different stresses to the spine, further quantifications of neurological risks are presented. Neuromonitoring data was prospectively entered, and the database between 2006 and 2012 was interrogated. All deformity cases under the age of 21 were included. Tumour, fracture, infection and revision cases were excluded. All “red alerts” were identified and detailed examinations of the neuromonitoring records, clinical notes and radiographs were made. Diagnosis, deformity severity and operative details were recorded. 2290 deformity operations were performed: 2068 scoliosis (1636 idiopathic, 204 neuromuscular, 216 syndromic, and 12 others), 89 kyphosis, 54 growing rod procedures, and 80 operations for hemivertebra. 696 anterior and 1363 posterior operations were performed for scoliosis (8 not recorded), and 38 anterior and 51 posterior kyphosis correction. 67 “red alerts” were identified, there were 14 transient and 6 permanent neurological injuries. 62 were during posterior stage (24 idiopathic, 21 neuromuscular, 15 syndromic (2 kyphosis), 1 growing rod procedure, 1 haemivertebra), and 5 were during anterior stage (4 idiopathic scoliosis and 1 syndromic kyphosis). Average Cobb angle was 88°. 1 permanent injuries were during correction for kyphosis, and 5 were for scoliosis (4 syndromic, 1 neuromuscular, and 1 anterior idiopathic). Common reactions after “red alerts” were surgical pause with anaesthetic interventions (n=39) and the Stagnara wake-up test (n=22). Metalwork was partially removed in 20, revised in 12 and completely removed in 9. 13 procedures were abandoned. The overall risk of permanent neurological injuries was 0.2%, the highest risk groups were posterior corrections for kyphosis and scoliosis associated with a syndrome. 4% of all posterior deformity corrections had “red alerts”, and 0.3% resulted in permanent injuries; compared to 0.6% “red alerts” and 0.3% permanent injuries for anterior surgery. The overall risk for idiopathic scoliosis was 0.06%

Purpose. To report the results of full vertebral column resection (VCR) for paediatric spinal deformity. Methods and Results. All VCR (n=47) for paediatric spinal deformity were retrospectively evaluated from four university hospitals performing these procedures in Finland between 2005 and 2010. After excluding single hemivertebra (n=25) and resections performed for patients with MMC (n=6), 16 patients with full VCR (mean age at surgery 12.9 yrs [6.5-17.9] AIS 1; NMS 3; Congenital scoliosis 3 primary, revision 4; Kyphosis congenital 2, global 2; NF1 scoliosis 1) were identified. Seven procedures were performed anteroposteriorly and nine posterior-only. Mean follow-up time 1.9 (0.6–5.5) years. Major Curve (MC) averaged preoperatively 85 (58–120) degrees, 31 (14-53) degrees at 6 months, and 37 (17-80) degrees at 2-year follow-up. MC correction averaged 61 (46-86)% in the AP and 64 (57-83)% in the PL group at 6 months and 54 (18-86)% and 60 (41-70)% at 2-yr FU, respectively (NS). Blood loss averaged 3400 (500-8200) mL (NS between groups). The mean SRS-24 total scores were 100 (92-108) for AP and 102 (95-105) for PL group. There was one paraparesis in the AP group necessitating urgent re-decompression with full recovery. One peripheral L5 motor deficit resolved fully within few days (PL). Two junctional kyphosis were observed (one in both group). One one-sided partial lower instrumentation pull-out was observed without need for revision. One pseudoarthrosis occurred in AP group needing revision. Conclusions. Full VCR is rarely needed for paediatric spinal deformity with an estimated incidence of 2.9/million/year. Posterior VCR allows better control of neural elements during deformity correction

Introduction. The potential structural effect of the sacral tilt on the development of disc wedging adjacent to lowest instrumented vertebra (LEV), and consequent postoperative coronal trunk decompensation in the surgical treatment of idiopathic scoliosis are well known. The aim of this study is firstly to establish the possible role of sacral tilt, limb length inequality, and associated pelvic girdle anomalies in the cause of idiopathic scolisosis, and secondly to determine the effect of sacral tilt on adjacent disc wedging below LEV in some idiopathic curves such as thoracolumbar/lumbar curves, and double major curves postoperatively. Methods. Between 2006 and 2008, 159 patients with idiopathic scoliosis from outpatient clinics were included in analyses. In all patients, standing posteroanterior and lateral spinal column and Ferguson radiographs were routinely taken. If direct radiographs suggested iliac bone asymmetry, hemi-pelvis volume measurements were done with CT scan. Curve type, sacral tilt, L5 tilt, iliac asymmetry, and limb-length differences were measured. As a second part of study, 87 surgically treated patients with double major curves were examined retrospectively in terms of adjacent subjacent disc wedging below LEV and sacral tilt. Results. In the first part of the study, the mean age of patients was 14·21 years (range 2–25) and all but 14 were female. The curve types were lumbar in 31 patients, thoracolumbar in 42, double major in 55, thoracic in 27, and triple in four. Sacral tilt towards the convex side of TL/L curve was present in 117 (74%) patients with an average of 8° measured from Ferguson graphs. L5 tilt towards the convex side of TL/L curve, and limb length inequality of less than 5 mm on the convex side of TL/L curve were more prevalent in patients with sacral tilt (57%) than in those without sacral tilt (p=0·021). On hemi-pelvis volume measurement, patients with no sacral tilt (n=42) had no difference in both sides whereas patients with sacral tilt (n=117) showed significant difference, with the convex side being smaller. We noted a correlation between L5 tilt and sacral tilt (p=0·048) and between sacral tilt and hemi-pelvis volume (p=0·024). There was no correlation between sacral tilt and pelvic asymmetry, age, sex, curve type, or amount of limb length inequality. In the second part of the study, disc wedging adjacent to LEV was present in 75 of 87 patients and sacral tilt was present in 72 of 75 (96%) (p=0·008). Six patients had limb length inequality of more than 1 cm, showing the postoperative trunk decompensation, which was corrected by elevating the shorter leg by shoe-lift. Conclusions. This study revealed significant association between sacral tilt and hemi-pelvis hypoplasia independent from age. This association may have a primary role in the cause of this disease. Although LEV is paralel to pelvis, intervertebral disc adjacent to LEV is often wedged and there is lumbosacral hemicurve distally below the instrumentation in patients treated by either anterior or posterior surgery. We believe that lumbosacral hemicurve due to sacral tilt behaves like hemivertebra, which is impossible to detect with preoperative standing anteroposterior radiographs. Ferguson view is necessary to detect the sacral tilt. The postoperative trunk decompensation is greater with presence of sacral tilt and limb length inequality

We report the results of vertebral column resection (VCR) for paediatric patients with spinal deformity. A total of 49 VCRs in paediatric patients from four university hospitals between 2005 and 2009 with a minimum two-year follow-up were retrospectively identified. After excluding single hemivertebral resections (n = 25) and VCRs performed for patients with myelomeningocele (n = 6), as well as spondylectomies performed for tumour (n = 4), there were 14 patients who had undergone full VCR at a mean age of 12.3 years (6.5 to 17.9). The aetiology was congenital scoliosis in five, neuromuscular scoliosis in three, congenital kyphosis in two, global kyphosis in two, adolescent idiopathic scoliosis in one and secondary scoliosis in one. A total of seven anteroposterior and seven posterolateral approaches were used.

The mean major curve deformity was 86° (67° to 120°) pre-operatively and 37° (17° to 80°) at the two-year follow-up; correction was a mean of 54% (18% to 86%) in the anteroposterior and 60% (41% to 70%) in the posterolateral group at the two-year follow-up (p = 0.53). The mean Scoliosis Research Society-24 total scores were 100 (92 to 108) for the anteroposterior and 102 (95 to 105) for the posterolateral group. There was one paraparesis in the anteroposterior group necessitating urgent re-decompression, with a full recovery.

Patients undergoing VCR are highly satisfied after a successful procedure.

Aims

There is a paucity of information on the pre-operative coronal imbalance in patients with degenerative lumbar scoliosis (DLS) and its influence on surgical outcomes.

Acute angulation at the thoracolumbar junction with segmental subluxation of the spine occurring at the level above an anteriorly hypoplastic vertebra in otherwise normal children is a rare condition described as infantile developmental thoracolumbar kyphosis. Three patient series with total of 18 children have been reported in the literature. We report five children who presented with thoracolumbar kyphosis and discuss the treatment algorithm. We reviewed the medical records and spinal imaging at initial clinical presentation and at minimum two-year follow-up. The mean age at presentation was eight months (two to 12). All five children had L2 anterior vertebral body hypoplasia. The kyphosis improved spontaneously in three children kept under monitoring. In contrast, the deformity was progressive in two patients who were treated with bracing. The kyphosis and segmental subluxation corrected at latest follow-up (mean age 52 months; 48 to 60) in all patients with near complete reconstitution of the anomalous vertebra. The deformity and radiological imaging on a young child can cause anxiety to both parents and treating physicians. Diagnostic workup and treatment algorithm in the management of infantile developmental thoracolumbar kyphosis is proposed. Observation is indicated for non-progressive kyphosis and bracing if there is evidence of kyphosis and segmental subluxation deterioration beyond walking age. Surgical stabilisation of the spine can be reserved for severe progressive deformities unresponsive to conservative treatment.

Cite this article: Bone Joint J 2015;97-B:982–7.

Aims

We reviewed 34 consecutive patients (18 female-16 male) with isthmic spondylolysis and grade I to II lumbosacral spondylolisthesis who underwent in situ posterolateral arthodesis between the L5 transverse processes and the sacral ala with the use of iliac crest autograft. Ten patients had an associated scoliosis which required surgical correction at a later stage only in two patients with idiopathic curves unrelated to the spondylolisthesis.

A combined anterior and posterior surgical approach is generally recommended in the treatment of severe congenital kyphosis, despite the fact that the anterior vascular supply of the spine and viscera are at risk during exposure. The aim of this study was to determine whether the surgical treatment of severe congenital thoracolumbar kyphosis through a single posterior approach is feasible, safe and effective.

We reviewed the records of ten patients with a mean age of 11.1 years (5.4 to 14.1) who underwent surgery either by pedicle subtraction osteotomy or by vertebral column resection with instrumented fusion through a single posterior approach.

The mean kyphotic deformity improved from 59.9° (45° to 110°) pre-operatively to 17.5° (3° to 40°) at a mean follow-up of 47.0 months (29 to 85). Spinal cord monitoring was used in all patients and there were no complications during surgery. These promising results indicate the possible advantages of the described technique over the established procedures. We believe that surgery should be performed in case of documented progression and before structural secondary curves develop. Our current strategy after documented progression is to recommend surgery at the age of five years and when 90% of the diameter of the spinal canal has already developed.

Cite this article: Bone Joint J 2013;95-B:1527–32.

An eight-week-old boy developed severe thoracic spondylodiscitis following pneumonia and septicaemia. A delay in diagnosis resulted in complete destruction of the T4 and T5 vertebral bodies and adjacent discs, with a paraspinal abscess extending into the mediastinum and epidural space. Antibiotic treatment controlled the infection and the abscess was aspirated. At the age of six months, he underwent posterior spinal fusion in situ to stabilise the spine and prevent progressive kyphosis. At the age of 13 months, repeat imaging showed lack of anterior vertebral body re-growth and he underwent anterior spinal fusion from T3 to T6 and augmentation of the posterior fusion. At the age of five years, he had no symptoms and radiographs showed bony fusion across the affected levels.

Spondylodiscitis should be included in the differential diagnosis of infants who present with severe illness and atypical symptoms. Delayed diagnosis can result in major spinal complications with a potentially fatal outcome.

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 tubes (type 1 diastematomyelia), and 32 patients had single dural tubes (type 2 diastematomyelia). Radiographs, CT myelography, and MRI showed characteristic kyphoscoliosis, widening of the interpedicle distance, and bony, cartilaginous, and fibrous septum. The incidences of symptoms including characteristic changes of the dorsal skin, neurological disorders, and congenital spinal or foot deformity were significantly higher in type 1 than in type 2. Surgery is more effective for patients with type 1 diastematomyelia; patients without surgery showed no improvement.