Congenital Talipes Equinovarus (CTEV) occurs in approximately 1 in 1000 live births. Most cases occur as an isolated birth defect and are considered idiopathic. The widespread adoption of the Ponseti technique of serial casting followed by Achilles tenotomy and long term bracing has revolutionised the outcomes in CTEV. In most cases, plantigrade, flexible, pain-free feet may be produced without the need for extensive surgery. It is estimated that about 10% of cases of CTEV are not idiopathic. These feet are stiffer and more challenging to treat. In particular, there is little evidence in the literature concerning the efficacy of the Ponseti method in these cases. In our institution, a dedicated weekly Ponseti clinic has operated since 2005. To date 140 patients have been treated. We prospectively enter all details regarding their management onto an independent international database. The aim of this study was to audit the non-idiopathic cases of CTEV and to assess the effectiveness of the Ponseti technique in these challenging cases. Outcome measures included the Pirani score and eventual need for surgical intervention. We identified 29 cases (46 feet) with non-idiopathic CTEV. This comprises 21% of our workload. Seventeen were bilateral. The commonest diagnoses were neuromuscular conditions such as spina bifida (5 cases) and cerebral palsy (3 cases). There were 4 cases of Trisomy 21. Other causes included Nail Patella syndrome, Moebius syndrome, Larsen syndrome and Ito syndrome. In approximately 12% of cases, the underlying disorder remained undiagnosed despite thorough medical and genetic testing. In cases of non-idiopathic CTEV, the mean starting Pirani score was 5.5 (out of 6). After serial casting and Achilles tenotomy, the average score was 2.0. Twenty-one of 46 feet (46%) ultimately required further surgical intervention (mostly posteromedial release). We found that certain conditions were more likely to be successfully treated with the Ponseti method – these included conditions characterised by ligamentous laxity such as Trisomy 21 and Ehlers Danlos syndrome. All patients showed some improvement in Pirani score after serial casting. We believe that it is essential to attempt the Ponseti method of serial casting in all cases of CTEV. More than half of all non-idiopathic cases will not require further surgical intervention – and those that do are not as stiff thanks to the effects of serial casting. Thus, the surgery required is not as complex as it might otherwise have been. This is the largest series of its kind in the current medical literature.
Hurler syndrome is an autosomal recessive metabolic storage disease, with specific musculoskeletal abnormalities termed dysostosis multiplex. Haematopoietic Stem Cell Transplant (HSCT) increases life expectancy, but its effects on the progression of dysostosis multiplex are less certain. We detail the ongoing follow up of 23 patients (range 2.6 – 20.7 years) at a mean of 8.5 years after successful HSCT, the largest series reported in the literature to date. All patients were clinically examined at an annual multidisciplinary clinic, and serial radiological studies were reviewed to assess development and management of hip dysplasia and genu valgum. All patients demonstrated characteristic acetabular dysplasia and failure of ossification of the superolateral femoral head. Thirteen patients have undergone hip containment, including eight bilateral combined pelvic osteotomy and femoral derotation, at a mean of 4.4 years. Mean preoperative acetabular angle was 34 ± 5°. Long term follow up of older patients (>
8 years, mean 9.9 years after surgery) demonstrated adequate femoral head cover, with mean centre-edge angle of 40 ± 5° (range 32 – 48°). More recently, isolated innominate osteotomy has been used. Genu valgum of variable severity due to failure of ossification of the lateral aspect of the proximal tibial metaphysis was more variable, and seven patients underwent medial epiphyseal stapling at a mean of 7.8 years, decreasing tibiofemoral angle by a mean of 7°. Staple dislodgment, however, was seen in four children. All patients remain independently mobile, but hip stiffness and valgus knees contribute to the early fatigue and hip discomfort seen in older children. Based on our series, we conclude that hip containment surgery has been successful at least into early adolescence, with overall mobility being well preserved. We recommend plating of the proximal tibial epiphysis. Further follow up will monitor the effectiveness of orthopaedic intervention.
A 2002 study by Goldberg et al showed that surgery before age 10 for infantile onset idiopathic scoliosis (diagnosis <
4 years, Cobb angle =>
10°) preserved neither respiratory function nor cosmesis, and has not been contradicted. In 2005, Mehta re-emphasised scoliosis correction by serial cast-bracing, while Thompson et al reported satisfactory results with growing rods. An analysis of the status quo of a cohort of patients with infantile idiopathic scoliosis (other diagnoses and syndromes excluded), managed by cast-bracing, was undertaken, asking whether interim progress was acceptable or demanded a change of protocol. Of 35 patients born between October 1993 and December 2002,15 have completely resolved, age at diagnosis 1.6 ± 0.96 years, Cobb angle 20.3°±11.9, RVAD 11.1°±13.8, latest age 4.1± 2.3. 20 were prescribed cast-bracing, age at diagnosis 1.8±0.9 years, Cobb angle 47.3°±12.6, RVAD 29.6±24.5, age at treatment was 2.1±1.0 years. Cobb angle (p<
0.001) and RVAD (p=0.001) were larger in the treated group, but age at presentation was the same (p=0.473). Surgery was performed on 3 children unresponsive to initial casting, at ages 3.2, 3.6 and 3.7, and in 3 at ages 8.6, 10.1 and 11 years. 3 children, aged 6.0, 8.1 and 11.3 are out of brace with straight spines and 11 are stable in brace. Infantile idiopathic scoliosis seems programmed to resolve or progress according to initial severity and in line with growth rate. Those who respond to casting in infancy generally remain stable until near puberty when surgery is uncontroversial. Those who progress relentlessly and immediately in cast remain the issue, as reports of newer methods include a wide range of ages and diagnoses and give their outcome in terms of Cobb angle only. It has not yet been shown that any treatment will alter their prognosis so constant analysis of all outcome parameters is essential.
It is customary to analyse scoliosis as a mechanical failure: first there is a straight spine (=normal), then an habitual and collapsing posture (=disease) and finally, structural remodelling (Hueter-Volkmann effect = scoliosis). This hypothesis makes two practical predictions:
There is a disease process causing the pathological posture. The purpose of gatherings such as this is to identify this pathology, thus far without success. Early diagnosis will permit early non-operative treatment which will halt or reverse the remodelling and reduce the occurrence of severe deformity and the need for corrective spinal surgery. The failure of school scoliosis screening to achieve this end is well documented, but the consequence for the underlying hypothesis has not been analysed. Screening failed, not because it was unable to detect scoliosis, but because scoliosis did not behave as the hypothesis predicted.
There is little data regarding the epidemiology of childhood injury in Ireland. This is difficult to obtain retrospectively. The aim of this study was to prospectively evaluate paediatric trauma referrals to our department, describe their epidemiology, and identify potentially preventable injuries in children. Our unit at the National Childrens Hospital is located in a growing suburban area in South Dublin. Injury surveillance was conducted on orthopaedic referrals by distributing a form to parents of children attending fracture clinics or admitted acutely for surgery. Parents were asked for demographic information, and a brief description of the injury in terms of location, mechanism and circumstance of injury. Diagnosis was completed by the attending doctor and data was transferred to a computerised database. We analyzed data from the 397 referrals in the first month of this study. The mean age of injury was 9.1 years and the male: female ratio was 1.3:1. The peak hour of injury was 7 – 8 pm. Only 33% of injuries occured during the weekend. 62% of injuries were due to falls, usually form the standing position. The most common location for injury was in or about the home (39%), and other notable locations were school (16%) and sportsfield (14%). 61% of falls greater than 1 metre occurred at home, mostly from walls and childrens slides. 20% of injuries occurred while participating in organized sport, including Gaelic football, soccer and hurling. Injuries occuring during unsupervised sport were more likely to need surgery. Domestic ‘bouncing castles’ and trampolines, increasingly popular in our area, were a notable cause of significant trauma to the upper limb. 7% of injuries occurred by falling from a bicycle, but vehicular road traffic accident was an uncommon cause of injury. 263 children had confirmed fractures, other injuries consisting largely of sprains to the ankle, elbow and wrist. Predictably, the bones most commonly fractured were the radius (41%), phalanges (15%) and humerus (11%). 20% of fractures needed operative management, mostly forearm manipulation under anaesthesia. 63% of operative cases were performed outside of normal working hours. Several countries utilise injury surveillance as a means of development and evaluation of injury prevention strategies. In our initial study, basic surveillance has outlined local characteristics of chilhood trauma, and some trends were noted. In particular, we suggest home injuries need further attention in out catchment area.
Thirty four patients underwent 34 single entry percutaneous physiodesis (SEPP) of both distal femur and proximal tibia between July 1996 and June 2004. Twenty six patients had attained maturity and the rest continue to be followed up. There were 10 females and 16 males. The mean ages were12.8yrs (range11–14yrs) and 13.8yrs (range11–15) respectively. All patients underwent at least three assessments of limb length discrepancy(lld) using CT Scannograms. The Mosely’s straight line graph was then used to predict lld and timing of correction. The procedure was performed under image intensifier control using a 6.5mm drill passed through a small incision. The drill was passed in three directions through a single entry . The physis was curetted. The mean lld at SEPP was 3.36cm(range1.5–5.9cm). The prediction of lld at maturity after SEPP was a mean of 1.4cm(range0.2–2.5cm) and final lld was a mean of 1.38cm (range0.3–2.5cm). The accuracy of prediction was found to have a mean of 0.44cm (range0–0.7cm). One patient (6%) complained of knee pain for about 2 weeks that settled. The rest had no complications. We feel that this technique is minimally invasive with a cosmetic scar, has a shorter hospital stay, low complications and is reliable for phuseal ablation. This technique aided by the CT scannogram and a Mosely’s straight line graph provides a reliable and effective method in the management of small amounts of lld.
The aim of this study is to assess the success of posterior lumbar interbody fusion in the treatment of degenerative spinal instability. Clinical outcome was assessed by the postoperative symptomatic relief, complications rate and the fusion rate. The fusion rate was assessed using plain radiographs and the Brantigan and Steffee scoring system. Functional outcome was measured by the improvement in the Oswestry disability index, PROLO score, return to work and satisfaction with the surgical outcome. The determinants of functional relief were analysed against the improvement in disability using multiple regression analysis.
Back pain screening clinics are established to clinically screen patients with back pain for organic lumbar pathology. The aim of this study is to assess the relationship between clinical signs of organic pathology and the level of disability as measured by functional outcome scores. The correlation between the level of psychological morbidity, length of symptoms and presence of past history of symptoms against the level of disability was statistically assessed.
Langerhans-cell histiocytosis (LCH) is a reactive proliferative disease characterized by the accumulation of abnormal histiocytes. The disease is broadly divided into two groups, unisystem and multisystem disease. The aetiology of LCH is unknown; the disease is currently accepted to be a reactive process rather than a malignancy. Localized LCH of bone is a benign tumour-like condition, which is characterized by a clonal proliferation of Langerhan’s-type histocytes, which infiltrate bone and cause osteolytic lesions. The common bones involved include – skull, pelvis, and diaphysis of long bones. We wanted to determine whether patient demographics at the time of presentation could help determine the clinical course and eventual outcome of the disease. We prospectively reviewed 68 patients with a primary diagnosis of LCH. Forty-six patients had unisystem disease, 22 had multisystem disease. There was a statistically significant difference in the age of presentation between the two groups. There were 6 deaths, all had multisystem disease. Of the 46 patients with unisystem disease, 31 (67.3%) underwent orthopaedic surgical intervention, 26 open biopsies and 5 curettage and bone grafting of lesions of the humeras (2), skin, clavicle and skull (1 each). There was a statistically significant difference in the average length of follow-up, between the 2 groups. We recommend closed and prolonged multidisciplinary follow-up of patient initially presenting with multisystem disease. Patients with unisystem disease can safely be discharged after a short follow-up period.
Comparing Groups 1 &
2, girls in Group 1 were younger and smaller at diagnosis with lower Cobb angles. They were older at menarche, but this was inevitable from the selection criteria, and more likely to progress (p<
0.001), to receive a brace (p=0.047) and to undergo surgery (p=0.043). Age, final Cobb angle and height at discharge were not significantly different.
Introduction: The arguments for and against school screening for scoliosis are long since over, and centres have continued or ceased as they thought best and as funding allowed. However, the programmes did amass considerable volumes of observations that, being part of the over-all epidemiological picture, could advance our understanding of adolescent idiopathic scoliosis and of minor asymmetries of back shape. Methods and Results: A retrospective examination of the records from the school screening programme at this centre concentrated on subjects with minor asymmetry, those who at first review did not qualify as ‘scoliosis’ yet were noted to have failed the forward bend test. There were 91,811 examinations on 55,484 girls: 2170 were classified as ‘non-scoliosis asymmetry’. Of these, 1574 were noted but not referred; 360 were reviewed in clinic without radiograph,; 107 had straight spines on radiograph and 221 had Cobb angles <
10°. Eleven are known to have progressed to 10° or more, three passed 25°, two passed 40° and one underwent surgery. This gives an incidence in this subgroup of 0.51% for defined scoliosis. For scoliosis =>
25°, it was 0.14%; for scoliosis =>
40°, 0.092%; and 0.046% for surgery, none of which shows a significant difference from the equivalent rates for the population as a whole. (0.6% Cobb angle =>
10°, 0.2% Cobb angle =>
25°, 0.08% Cobb angle =>
40°, 0.045% surgery. (Goldberg CJ et al. (1995). Spine. 20(12):1368–1374). Conclusion: These findings are in accordance with previous reports on school screening, and it is not proposed to re-open the discussion. Their relevance is their relationship to significant scoliosis: since these children are not at increased risk of developing deformity, they cannot be, as has been proposed (Nissinen et al (2000) Spine. 25:570–574) instances of mild or early scoliosis, and they do not need intensive investigation, follow-up or treatment. Non-scoliosis asymmetry is closer to the increased fluctuating asymmetry displayed by this age group (Wilson and Manning. (1996) Journal of Human Evolution. 30:529–537) and begs a more biological approach to spinal deformity, asymmetry and back shape.
Introduction: Historically, the spinal curvature of adolescent idiopathic scoliosis was considered a life-threatening occurrence, which would result in early death from cardio-respiratory compromise. Consequently, corrective surgery had the primary intention of preventing this unacceptable outcome: cosmetic improvement was considered to be certainly important, but not the prime objective of the treatment. More recent work (e.g. Branthwaite MA. (1986) Br.J.Dis.Chest. 80:360–369) has shown that, while significant deformity presenting in early childhood does carry this outlook, those with an adolescent onset should not be significantly affected in this way. Consequently, any surgery recommended is primarily cosmetic, to improve the deformity when it is unacceptable to the patient and her parents. This, of necessity, changes the criteria by which treatment outcome should be assessed. Scoliosis surgery has generally been judged by the correction in Cobb angle and, more recently, the derotation of vertebrae. However, it is well known that neither factor accurately expresses cosmesis, the criterion by which the patient will judge the operation. Surface topography attempts to quantify the external appearance of a patient and so the cosmetic effect of surgery. Since 1995, when a surface topographic system (Quantec) was acquired by this department, 61 patients were operated for adolescent idiopathic scoliosis, of whom 35 underwent anterior release and posterior fusion for rigid thoracic curves. Methods and Results: Pre- and post-operative radiographs were compared with topographic results from the same periods and with the latest scan at last review. The mean pre-operative Cobb angle was 74.5° and, postoperatively was 40.7°, a mean correction of 45.4% and was statistically significant (p<
.001). This was accompanied by statistically significant reductions in upper and middle topographic spinal angles (p=0.001), an increase in thoracic kyphosis (p<
0.05), a decrease in lumbar lordosis (p=0.001), lower rib hump (p<
0.05), Suzuki hump sum (a measure of back asymmetry, p=0.001) and posterior trunk asymmetry score (POTSI, a measure of trunk balance, p=0.003). At final follow-up a mean of 2.2 years later, topographic spinal angles and POTSI maintained their improvement, still being statistically significantly less than their pre-operative values. Thoracic kyphosis, lumbar lordosis, rib hump and Suzuki hump sum had returned towards pre-operative levels and no longer showed statistically significant differences. Conclusions:This confirms previous reports of the recurrence of the rib-hump. In conclusion, after two-stage spinal fusion for adolescent idiopathic scoliosis, significant improvement in cosmetic appearance can be achieved. However, over time certain aspects of the original deformity, particularly distortion of the back surface (rib hump or asymmetry) recurs.
Posterior lumbar interbody fusion is a well described procedure for the treatment of back pain associated with degenerative disc disease and segmental instability. It allows decompression of the spinal canal and circumferential fusion through a single posterior incision. Sixty-five consecutive patients who underwent posterior lumbar interbody fusion (PLIF) using carbon cages and pedicle fixation between 1993 and 2000 were recruited and contacted with a postal survey. Clinical outcome was assessed by the assessment of postoperative clinical findings and complications and the fusion rate, which was assessed using standard X-rays with the scoring system described by Brantigan and Steffee. Functional outcome was measured by using improvement in the Oswestry Disability Index, PROLO score, return to work and satisfaction with the surgical outcome. The determinants of functional relief were analysed against the improvement in disability using multiple regression analysis. The mean postoperative duration at the time of the study was 4.4 years. The response rate to the survey was 84%. Overall radiological fusion rate was ninety eight percent. There was a significant improvement in Oswestry Disability Index P<
0.001. There was 85% satisfaction with the surgical procedure and 58% return to pre-disease activity level and full employment. In the presence of near total union rate we found preoperative level of disability to be best the determinant of functional recovery irrespective of age or the degree of psychological morbidity (p<
0.0001). The combination of posterior lumbar interbody fusion (PLIF) and posterior instrumented fusion is a safe and effective method of achieving circumferential segmental fusion. This procedure gives sustained long-term improvement in functional outcome and high satisfaction rate. Direct relationship between preoperative level of disability and functional recovery suggests that spinal fusion should be performed to alleviate disability caused by degenerative spine.
Background The non-motorised microscooter has become the urban transport of choice for children in Ireland. Recently, Josefson highlighted the rising trend of scooter-related injury in the US and predicted possible significant impacts in human and socioeconomic terms. Materials and Methods. A prospective study was undertaken of all referrals with scooter injuries to accident and emergency departments and fracture clinics in the first three months of the year. These cases were then reviewed at 6 months post injury Results: There were 151 microscooter injuries seen in the first 3 months of the year, Forming over 4% of all trauma seen over this period. Eighty nine of the patients (59%) were female, and the mean age at presentation was 8.5 years (range 3–15 years). The peak referral rates for January, February and March measured 48%, 29% and 23% respectively. A survey of attending paediatric outpatients over this period revealed that 75% of households possessed at least 1 scooter, and in those households with children aged between 4 and 14 years, the rate of micoscooter possession increased to 83%. Eighty four children suffered fractures and dislocations, 59 suffered soft tissue injuries, 8 had isolated head injuries. Upper limb fractures and dislocations were the most common injury (75 of 84 bone and joint injuries). Fracture of distal third of radius and ulna, was the most common single injury. upper limb fractures wer seen frequently. A high proportion of these had apex dorsal angulation with or without displacement (Smith deformity). Lower limb fractures were relatively rare. The pattern of soft tissue injuries and lacerations mostly affected the head and neck 25 (17%), the lower limb was involved in 19 (13%) and upper limb in 15 (10%) of patients. No major head injuries occurred. Only 5 patients had any adult supervision at the time of their injury. No children wore any formal protective clothing or apparatus. In the 84 patients who had suffered bony injury, at 6 months, 110 patients (73%) had
Assessment and referral of spinal disease in a primary care setting is a challenge for the general practitioner. This has led to establishment of spinal assessment clinic to insure prompt access to the patient who requires treatment by a spinal surgeon. These clinics are run by a trained physiotherapist who liaises with a member of the spinal team and decides the need for referral to the spinal clinic on the bases of the patient’s history and clinical examination. In our clinic each patient is also assessed with Oswestry disability index, Short form-36, visual analogue score and hospital anxiety score (HADS), although these scores do not contribute to the clinical decision-making. The aim of this study is to assess the screening value of Oswestry disability score, Short form-36 scores in diagnosing acute spinal pathology. Sixty-nine patients who were referred to the spine clinic from the assessment clinic between March and December 2001 were recruited. Sixty-nine age and sex-matched patients were randomly chosen from five hundred and twelve patients who were seen in the spinal assessment clinic and did not need referral to the specialised spine clinic. The Oswestry disability score, Short form-36 scores and pain visual analogue scores between the two groups were statistically compared. The correlation between the level of psychological morbidity, length of symptoms and presence of past history of symptoms against the level of disability was statistically assessed. Although there was a significant increase in the level of disability in the referred group with each score (Oswestry Disability Score P<
0.001, SF-36 physical component score P=0.014, Visual analogue pain score P<
0.001). The variation in the scores makes the scoring system unspecific for use as a screening tool. We also found strong relationship between psychological disability and length of symptoms indicating the need for prompt treatment for back pain.
Neonatal septic arthritis is a true orthopaedic emergency posing significant threat to life and limb.
Posterior lumbar interbody fusion is a well-described procedure for the treatment of back pain associated with degenerative disc disease and segmental instability. It allows decompression of the spinal canal and circumferential fusion through one posterior incision. The aim of this study is to assess fusion rate as well as long term outcome of this procedure.
Fusion rate was assessed using standard X-rays with scoring system described by Brantigan and Steffee.
Surgical correction of spinal deformity in patients with neural axis abnormalities has established risks of causing further neurological injury. It is necessary to identify individuals with a predisposition for such abnormalities before treatment is instituted.