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.

The purpose of this cohort study is to determine the incidence of all congenital vertebral anomalies detected antenatally through ultrasound. We also reported on the early mortality rate for this patient cohort, as well as the frequency and type of associated congenital anomalies. The East Midlands and South Yorkshire Anomalies Register consists of data on all voluntary reports of congenital anomalies, from an annual baseline birth rate of 67000 births. We analysed all registered congenital anomalies reported over a 10 year period. Between January 1997 and January 2007, 108 vertebral anomalies were reported, excluding spinal dysraphism (incidence 0.01%). 61 of these were detected antenatally (56%), 17 were detected postnatally (16%) and in 30 patients, the precise time of diagnosis was unclear (28%). At the time of analysis January 2007, 45 of 108 patients had died, either in utero or soon after delivery (42%). 12 fetuses remained in utero and 51 infants were alive. The mortality rate for antenatally diagnosed patients was 41% and the majority were electively terminated (72%). 2 fetuses electively terminated had vertebral anomalies in isolation. There is a relatively high incidence of elective termination of pregnancy as a result of antenatal anomaly ultrasound screening. We have evidence to suggest that foetuses with potentially minor congenital anomalies are being electively terminated at approximately 18 weeks gestation. A structured and timely spinal counselling should be offered once antenatal vertebral anomalies have been identified

Aims

Severe spinal deformity in growing patients often requires surgical management. We describe the incidence of spinal deformity surgery in a National Health Service.

Segmental vessel ligation during anterior spinal surgery has been associated with paraplegia. However, the incidence and risk factors for this devastating complication are debated.

We reviewed 346 consecutive paediatric and adolescent patients ranging in age from three to 18 years who underwent surgery for anterior spinal deformity through a thoracic or thoracoabdominal approach, during which 2651 segmental vessels were ligated. There were 173 patients with idiopathic scoliosis, 80 with congenital scoliosis or kyphosis, 43 with neuromuscular and 31 with syndromic scoliosis, 12 with a scoliosis associated with intraspinal abnormalities, and seven with a kyphosis.

There was only one neurological complication, which occurred in a patient with a 127° congenital thoracic scoliosis due to a unilateral unsegmented bar with contralateral hemivertebrae at the same level associated with a thoracic diastematomyelia and tethered cord. This patient was operated upon early in the series, when intra-operative spinal cord monitoring was not available.

Intra-operative spinal cord monitoring with the use of somatosensory evoked potentials alone or with motor evoked potentials was performed in 331 patients. This showed no evidence of signal change after ligation of the segmental vessels.

In our experience, unilateral segmental vessel ligation carries no risk of neurological damage to the spinal cord unless performed in patients with complex congenital spinal deformities occurring primarily in the thoracic spine and associated with intraspinal anomalies at the same level, where the vascular supply to the cord may be abnormal.