Aims. Children with spinal dysraphism can develop various musculoskeletal deformities, necessitating a range of orthopaedic interventions, causing significant morbidity, and making considerable demands on resources. This systematic review aimed to identify what outcome measures have been reported in the literature for children with spinal dysraphism who undergo orthopaedic interventions involving the lower limbs. Methods. A PROSPERO-registered systematic literature review was performed following PRISMA guidelines. All relevant studies published until January 2023 were identified. Individual outcomes and outcome measurement tools were extracted verbatim. The measurement tools were assessed for reliability and validity, and all outcomes were grouped according to the Outcome Measures Recommended for use in Randomized Clinical Trials (OMERACT) filters. Results. From 91 eligible studies, 27 individual outcomes were identified, including those related to clinical assessment (n = 12), mobility (n = 4), adverse events (n = 6), investigations (n = 4), and miscellaneous (n = 1). Ten outcome measurement tools were identified, of which Hoffer’s Functional Ambulation Scale was the most commonly used. Several studies used unvalidated measurement tools originally developed for other conditions, and 26 studies developed new measurement tools. On the OMERACT filter, most outcomes reported pathophysiology and/or the impact on life. There were only six patient- or parent-reported outcomes, and none assessed the quality of life. Conclusion. The outcomes that were reported were heterogenous, lack validation and failed to incorporate patient or family perceptions. Until outcomes can be reported unequivocally, research in this area will remain limited. Our findings should guide the development of a core outcome set, which will allow consistency in the reporting of outcomes for this condition. Cite this article: Bone Joint J 2024;106-B(3):277–285

Aims. The aim of this study is to define a core outcome set (COS) to allow consistency in outcome reporting amongst studies investigating the management of orthopaedic treatment in children with spinal dysraphism (SD). Methods. Relevant outcomes will be identified in a four-stage process from both the literature and key stakeholders (patients, their families, and clinical professionals). Previous outcomes used in clinical studies will be identified through a systematic review of the literature, and each outcome will be assigned to one of the five core areas, defined by the Outcome Measures in Rheumatoid Arthritis Clinical Trials (OMERACT). Additional possible outcomes will be identified through consultation with patients affected by SD and their families. Results. Outcomes identified in these stages will be included in a two-round Delphi process that will involve key stakeholders in the management of SD. A final list including the identified outcomes will then be summarized in a consensus meeting attended by representatives of the key stakeholders groups. Conclusion. The best approach to provision of orthopaedic care in patients with SD is yet to be decided. The reporting of different outcomes to define success among studies, often based on personal preferences and local culture, has made it difficult to compare the effect of treatments for this condition. The development of a COS for orthopaedic management in SD will enable meaningful reporting and facilitate comparisons in future clinical trials, thereby assisting complex decision-making in the clinical management of these children. Cite this article: Bone Jt Open 2022;3(1):54–60

Of a consecutive series of 117 one-year-old infants with 130 established dislocations of the hip, 11% failed to respond to primary surgical treatment. Genetic and iatrogenic factors accounted for half the failures. There were no obvious causes in the remainder, though a few had the superficial stigmata of spinal dysraphism, and by two years of age, most of the group had developed a lateral rotation posture of the affected leg associated with a relatively smaller foot on that side. Radiologically, the femoral head had drifted and rotated laterally out of the surgically deepened acetabulum, causing persistent subluxation. Although there was no clinical evidence of sensory or motor denervation, sensory spinal evoked potential tests revealed the presence of neurological deficits in the majority of patients in this group

The August 2024 Children’s orthopaedics Roundup. 360. looks at: Antibiotic prophylaxis and infection rates in paediatric supracondylar humerus fractures; Clinical consensus recommendations for the non-surgical treatment of children with Perthes’ disease in the UK; Health-related quality of life in idiopathic toe walkers: a multicentre prospective cross-sectional study; Children with spinal dysraphism: a systematic review of reported outcomes; No delay in age of crawling, standing, or walking with Pavlik harness treatment: a prospective cohort study; No value found with routine early postoperative radiographs after implant removal in paediatric patients; What do we know about the natural history of spastic hip dysplasia and pain in total-involvement cerebral palsy?; Evaluating the efficacy and safety of preoperative gallows traction for hip open reduction in infants

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.

In this study we evaluated the results of midtarsal release and open reduction for the treatment of children with convex congenital foot (CCF) (vertical talus) and compared them with the published results of peritalar release. Between 1977 and 2009, a total of 22 children (31 feet) underwent this procedure. In 15 children (48%) the CCF was isolated and in the remainder it was not (seven with arthrogryposis, two with spinal dysraphism, one with a polymalformative syndrome and six with an undefined neurological disorder). Pre-operatively, the mean tibiotalar angle was 150.2° (106° to 175°) and the mean calcaneal pitch angle was -19.3° (-72° to 4°). The procedure included talonavicular and calcaneocuboid joint capsulotomies, lengthening of tendons of tibialis anterior and the extensors of the toes, allowing reduction of the midtarsal joints. Lengthening of the Achilles tendon was necessary in 23 feet (74%). The mean follow-up was 11 years (2 to 21). The results, as assessed by the Adelaar score, were good in 24 feet (77.4%), fair in six (19.3%) and poor in one foot (3.3%), with no difference between those with isolated CCF and those without. The mean American Orthopaedic Foot and Ankle Society midfoot score was 89.9 (54 to 100) and 77.8 (36 to 93) for those with isolated CCF and those without, respectively. At the final follow-up, the mean tibiotalar (120°; 90 to 152) and calcaneal pitch angles (4°; -13 to 22) had improved significantly (p < 0.0001). Dislocation of the talonavicular and calcaneocuboid joints was completely reduced in 22 (70.9%) and 29 (93.6%) of feet, respectively. Three children (five feet) underwent further surgery at a mean of 8.5 years post-operatively, three with pes planovalgus and two in whom the deformity had been undercorrected. No child developed avascular necrosis of the talus. Midtarsal joint release and open reduction is a satisfactory procedure, which may provide better results than peritalar release. Complications include the development of pes planovalgus and persistent dorsal subluxation of the talonavicular joint. Cite this article: Bone Joint J 2014;96-B:837–44

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

Study Design: Retrospective case series. Objective: To evaluate the clinical outcome, radiographic results and complications associated with single rod anterior instrumentation in neuromuscular thoracolumbar scoliosis. Methods: Retrospective study with mean follow up of 35 months. Subjects: Nine patients (6F, 3M), mean age 15 years, were operated on between 1994–2000. This heterogeneous patient group consisted of five cases of spinal dysraphism, one prune belly syndrome, one arthrogryposis, one myotonic dystrophy and one congenital myopathic dystrophy (muscle-eye-brain-syndrome). All patients were ambulatory and had minimal pelvic obliquity (< 15degrees). Outcome measures: Pre-operative, post-operative and final follow-up measurements of Cobb angles, apical vertebral translation (AVT), thoracic kyphosis, lumbar lordosis, sagittal and coronal balance were recorded along with operative complications, pseudarthrosis, metalwork failure and loss of correction. Results: There was one rod breakage and one case of proximal thoracic curve progression requiring supplementary posterior surgery. For the remaining 7 patients, the average corrections for Cobb angle was 62% (52 to 20 degrees), AVT was 53% (5.7 to 2.7cms), and both thoracic kyphosis and lumbar lordosis remained unchanged. No pseudarthrosis, vascular or neurological complications were encountered. Subjectively results were excellent in six and good in one. Conclusions: Selective anterior instrumentation for neuromuscular scoliosis using a single rod resulted in acceptable clinical and radiographic outcomes in this highly selected series. Advantages include preservation of distal lumbar motion segments whilst maintaining sagittal and coronal alignment. We believe that this method of scoliosis correction has a definite yet select role in patients who are ambulatory, have minimal pelvic obliquity (< 15degrees), non-progressive pathology and near normal mental function

Objective: To evaluate the subjective clinical outcomes, radiographic results and complications associated with single solid rod anterior instrumentation in neuromuscular scoliosis. Design: Retrospective clinical case series with a mean follow up of 30 months (range 24 – 42 months). Subjects: 9 consecutive cases (6F, 3M) with a mean age 15 years (range 11 – 24 years), underwent single solid rod anterior instrumentation of their neuromuscular thoracolumbar scoliosis between 1994 and 2000. The heterogeneous patient group consisted of 5 spinal dysraphism, and 1 each of prune belly syndrome, arthrogryposis, myotonic dystrophy and congenital myopathic dystrophy (muscle eye brain syndrome). All patients were ambulatory and had minimal pelvic obliquity (< 15 degrees). Outcome measures: Pre-operative, post-operative and final follow up measurements were collected for 1) Cobb angles, 2) apical vertebral translation (AVT), 3) thoracic kyphosis (T5-12) and 4) lumbar lordosis (L1-5). Operative complications, pseudarthrosis, metalwork failure and loss of correction were also recorded. Results: There was 1 each of rod breakage and upper thoracic curve progression requiring supplementary posterior surgery. For the remaining 7 patients, the average follow-up corrections for Cobb angle was 56% (49 to 22 degrees), AVT was 49% (5.1 to 2.6 cms), and both the thoracic kyphosis and lumbar lordosis remained unchanged. No significant loss in correction occurred during the post-operative period to final follow-up in all the above parameters. No pseudarthrosis, vascular or neurological complications were encountered. Subjectively, there were 6 excellent and 1 good results. Conclusions: In this limited case review, selective anterior instrumentation for neuromuscular scoliosis using a single solid rod system resulted in acceptable clinical and radiographic outcomes. Our results appear to compare favourably with those published for the recommended method of posterior instrumentation. Advantages include preservation of distal lumbar motion segments whilst maintaining segmental saggital and coronal alignment. We believe that this method of scoliosis correction has a definite yet select role in patients who are ambulatory, have minimal pelvic obliquity (< 15 degrees), non-progressive pathology and near normal mental function

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.

Aims

Patients undergoing femoral lengthening by external fixation tolerate treatment less well when compared to tibial lengthening. Lengthening of the femur with an intramedullary device may have advantages.

We report the outcome of 28 patients with spina bifida who between 1989 and 2006 underwent 43 lower extremity deformity corrections using the Ilizarov technique. The indications were a flexion deformity of the knee in 13 limbs, tibial rotational deformity in 11 and foot deformity in 19. The mean age at operation was 12.3 years (5.2 to 20.6). Patients had a mean of 1.6 previous operations (0 to 5) on the affected limb. The mean duration of treatment with a frame was 9.4 weeks (3 to 26) and the mean follow-up was 4.4 years (1 to 9). There were 12 problems (27.9%), five obstacles (11.6%) and 13 complications (30.2%) in the 43 procedures. Further operations were needed in seven patients. Three knees had significant recurrence of deformity. Two tibiae required further surgery for recurrence. All feet were plantigrade and braceable.

We conclude that the Ilizarov technique offers a refreshing approach to the complex lower-limb deformity in spina bifida.

Scapulothoracic fusion (STF) for painful winging of the scapula in neuromuscular disorders can provide effective pain relief and functional improvement, but there is little information comparing outcomes between patients with dystrophic and non-dystrophic conditions. We performed a retrospective review of 42 STFs in 34 patients with dystrophic and non-dystrophic conditions using a multifilament trans-scapular, subcostal cable technique supported by a dorsal one-third semi-tubular plate. There were 16 males and 18 females with a mean age of 30 years (15 to 75) and a mean follow-up of 5.0 years (2.0 to 10.6). The mean Oxford shoulder score improved from 20 (4 to 39) to 31 (4 to 48). Patients with non-dystrophic conditions had lower overall functional scores but achieved greater improvements following STF. The mean active forward elevation increased from 59° (20° to 90°) to 97° (30° to 150°), and abduction from 51° (10° to 90°) to 83° (30° to 130°) with a greater range of movement achieved in the dystrophic group. Revision fusion for nonunion was undertaken in five patients at a mean time of 17 months (7 to 31) and two required revision for fracture. There were three pneumothoraces, two rib fractures, three pleural effusions and six nonunions. The main risk factors for nonunion were smoking, age and previous shoulder girdle surgery.

STF is a salvage procedure that can provide good patient satisfaction in 82% of patients with both dystrophic and non-dystrophic pathologies, but there was a relatively high failure rate (26%) when poor outcomes were analysed. Overall function was better in patients with dystrophic conditions which correlated with better range of movement; however, patients with non-dystrophic conditions achieved greater functional improvement.