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

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

Purpose of the study: Diastematomyelia is a rare spinal cord malformation defined as the presence of two separate spinal cords separated or not by an osseous, cartilaginous, or fibrous septum. Spinal malformations are almost always associated, raising difficult therapeutic challenges. Material and method: We report three cases of congenital kyphoscoliosis associated with diastematomyelia in three girls aged 12, 14 and 15 years. The diastematomyelia was dorsal in one case, thoracolumbar in one and lumbar in the third. For all three patients, the indication for surgery was progression of the scoliosis with development of neurological signs of recent progressive aggravation. Preoperative distraction with a plaster cast was pursued for several months prior to posterior instrumentation. No attempt was made to correct the cord malformation nor achieve major correction of the spinal malformation. The instrumentation bridged the thoracolumbar scoliosis in one case and stopped above the malformation in the two others. Results: The postoperative period was uneventful. There were no neurological complications. Preoperative neurological signs improved after surgery. Control radiographs showed an improvement in the deformity. At mean 6 years follow-up, these patients were not bothered in their everyday life. They had stable deformities which a globally balanced trunk. There were no signs of neurological evolution. Discussion: The therapeutic strategy for diastematomyelia remains a subject of debate. For some authors, the spinal cord should be released systematically which for the majority, this is not necessary except if spinal distraction is planned or if there is a neurological problem. If there is an indication for spinal cord release, any spurs must be removed followed by the necessary dura mater plasty. In our three patients, and in agreement with the neurosurgery team, there was no need for neurosurgical release. The recent development of neurological deficits was explained by the important kyphosis rather by the intramedullary anomaly. Our therapeutic strategy thus focused on treatment of the scoliosis. This enabled us to stabilise the spine, protecting these patients from worsening neurological involvement and enabling good functional outcome. The zone of the malformation was not instrumented in all cases because the posterior arcs were deformed, but also to avoid compromising any future neurosurgical intervention

Objective: To study the long term operative and non-operative outcome in patients with diastematomyelia (DM). Design: A prospectively acquired database of all spinal patients seen jointly by the senior authors (JKW, JLF), was searched for patients with DM. Their notes and the database were then reviewed. Subjects: Thirty-six patients were identified; twenty-one (58%) had associated scoliosis. There were 60 associated abnormalities in the 36 patients, most common being ten (27%) with leg length inequality. Twelve patients (33%) had no radiological bony abnormality. Twenty-four (66%) had neurosurgery, eleven (31%) untethering of filum alone and eleven (31%) with removal of a spur and closure of the DM as well. Nineteen (53%) underwent some sort of neuraxial shortening scoliosis correction/surgery. Twenty-eight (78%) were deemed to have a normal/independent neurological outcome, seventeen (61%) having neurosurgery and twelve (43%) scoliosis surgery. Conclusions: Patients with DM have been followed up for many years. Good neurological outcomes can be anticipated in cases with untethering and with scoliosis correction alone. This series raises the question as to whether any unthethering procedure is necessary in these cases when neuraxial shortening is carried out for scoliosis cases

Of forty-six patients with congenital elevation of the scapula, diastematomyelia was confirmed in four and was probably present in five others. Possible reasons for the previously unsuspected association are presented

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

Statement of purpose: To profile the neurological lesions associated with scoliosis, evaluate the role of preoperative MRI and determine the proportion of patients requiring surgery for an intradural lesion. Methods: The records of patients undergoing surgery to treat scoliosis over a 5 year period were reviewed as part of this retrospective single centre study. Results: 1926 patients underwent 2714 procedures to treat scoliosis. 45 patients from this cohort were referred for an opinion regarding at least one of the following neural axis abnormalities: Syrinx (47%); Chiari malformation and cerebellar ectopia (40%); tethered cord (13%); persistent central canal (9%); diastematomyelia (7%); neurofibromata (7%); syndromes other than neurofibromatosis (7%); tumours (4%) and vascular lesions (2%). 18 patients underwent surgery to treat a neural axis lesion: Foramen magnum decompression (12); cord untethering (4) and the surgical treatment of diastematomyelia (2). Conclusions: The authors believe their series to be the largest to date. Preoperative MRI scans should extend from the cranio-cervical junction to the sacrum, reflecting the potential locations of neural axis lesions. Radiologists present at units treating scoliosis should be able to identify both commonly occurring lesions such syrinx and intradural pathology. A significant proportion of patients required surgery to treat their neural axis lesions. Centres treating patients with scoliosis should therefore have the necessary facilities to treat not only scoliosis but also its associated intradural spinal lesions. Ethics approval: None Audit. Interest Statement: None

Sixty patients with congenital deformities of the spine were operated upon in the past fifteen years using a two-stage procedure. In the fifty patients with scoliosis half of the deformities were due to hemivertebrae and half to unilateral bars. The average correction of the deformity was 47 per cent. Early neurological signs observed in two patients with a diastematomyelia resolved. Of the ten patients with kyphosis nine had neurological signs of impending paraplegia and one was completely paraplegic before operation; all improved markedly. Posterior spinal fusion alone in the rapidly progressing congenital deformity may not prevent further progression, particularly in those cases iwth unilateral bars. Anterior resection of the vertebral body with later posterior fusion with Harrington instrumentation is safe and effective

The August 2023 Children’s orthopaedics Roundup³⁶⁰ looks at: DDH: What can patients expect after open reduction?; Femoral head deformity associated with hip displacement in non-ambulatory cerebral palsy; Bony hip reconstruction for displaced hips in patients with cerebral palsy: is postoperative immobilization indicated?; Opioid re-prescriptions after ACL reconstruction in adolescents are associated with subsequent opioid use disorder; Normative femoral and tibial lengths in a modern population of USA children; Retrospective analysis of associated anomalies in 636 patients with operatively treated congenital scoliosis; Radiological hip shape and patient-reported outcome measures in healed Perthes’ disease; Significantly displaced adolescent posterior sternoclavicular joint injuries.

Aims

To report the outcome of spinal deformity correction through anterior spinal fusion in wheelchair-bound patients with myelomeningocele.