Functional Outcomes of Congenital Scoliosis at a Mean 35-Year Follow-up Post In Situ Fusion. Revisiting Patients From the 2002 Goldberg et al Study.

BACKGROUND: The management of congenital scoliosis poses a significant challenge for treating surgeons. The aim of our study was to provide insight into the long-term clinical results of spinal fusion in congenital scoliosis. METHODS: We performed a retrospective review of the scoliosis database in our institution for the period 1976 until 2002 identifying 43 patients with congenital scoliosis who underwent spinal fusion. Patient demographics, diagnosis, levels fused, and radiographs were evaluated. Patients were evaluated for unplanned return to the operating room (UPROR) via SRS 22, EQ5D-5L, and Oswestry Disability Index (ODI). RESULTS: Of the 43 patients who fulfilled the inclusion criteria, 22 patients agreed to participate, 3 patients were known to be deceased and 18 patients were lost to follow-up or declined to participate and were excluded. The mean age of the respondents was 40.7 years (range, 30 to 47 y) with a mean follow-up from index surgery of 35 years (range, 20 to 44 y). At most recent follow-up, 12 patients (54%) underwent UPROR. The mean age at diagnosis was 3.4 years (range, birth to 11.5 y), and the mean age for first surgery was 5.8 years (range, 1 to 13 y). As regards radiologic follow-up; the mean number of levels fused was 5.2 (range, 2 to 12). Thoracic fusion was performed in 17 patients (77%). The mean T1 to T12 height at index surgery and maturity was 166 mm (range, 130 to 240 mm) and 202 mm (range, 125 to 270 mm), respectively. The mean functional scores at follow-up were SRS 22: 4.5 (range, 2.4 to 5), cumulative EQ5D-5L score 7.2 (range, 5 to 15), and ODI: 8% (range, 2 to 30%). All respondents completed high school, 10 patients (45%) completed university, and 2 patients were awarded doctorates. Currently, 17 patients (77%) are in paid employment. CONCLUSIONS: This report constitutes the largest series of patients treated by spinal arthrodesis for congenital scoliosis followed into maturity. We demonstrate the thorax continues to grow after index fusion, patient-reported outcomes were satisfactory with superior educational and employment rates and unplanned return to theatre is rare in adult life. LEVEL OF EVIDENCE: Therapeutic Level IV.

A Long-term Retrospective Evaluation of Functional and Radiographic Outcomes of Pediatric Hip Surgery in Hurler Syndrome.

BACKGROUND: After successful hematopoietic stem cell transplantation, maintaining function and mobility have become key goals in the management of patients with Hurler syndrome, (mucopolysaccharoidosis type 1H). The aim of this study was to establish the functional and radiologic outcomes after hip surgery in patients with this condition who had reached skeletal maturity. METHODS: We prospectively followed 13 mucopolysaccharoidosis type 1H patients with closed triradiate cartilages who had undergone hip surgery in a single institution (Our Lady's Children's Hospital, Crumlin) in early childhood, after successful hematopoietic stem cell transplantation. Functional assessment was performed using the Harris Hip Score. Acetabular and femoral head morphology were defined using a pelvic radiograph. RESULTS: The average age at follow-up was 18.6 years (range, 13.2 to 23.8 y). The average length of follow-up from surgical intervention was 14.6 years (range, 10.3 to 21.6 y). The average Harris Hip Score at follow-up was 61.0 (range, 19 to 91). At follow-up, 4 patients were either wheelchair bound or required a walking frame to mobilize in the community. At follow-up, all hips were in-joint with an average center edge angle of 37.7 degrees (range, 0 to 63 degrees). All hips displayed characteristic medial flattening of the femoral head. Ten hips (of 26 hips) showed radiologic degenerative changes with loss of joint space <2 mm. CONCLUSIONS: Despite the surgical provision of stable well-covered hips, active intervention did not prevent the development of radiologic deterioration and clinically significant hip arthritis. We recommend that pediatric hip surgery in Hurler syndrome be designed with the possibility of early hip replacement in mind. LEVEL OF EVIDENCE: Level III.

A single-center prospective evaluation of the Ponseti method in nonidiopathic congenital talipes equinovarus.

BACKGROUND: The Ponseti method has revolutionized the management of idiopathic congenital talipes equinovarus (CTEV). However, nonidiopathic CTEV is still often primarily treated by extensive surgical soft tissue release. We believe that nonoperative treatment of these patients using the Ponseti method may give very satisfactory results. METHODS: We examined the demographics of nonidiopathic CTEV and the success of the Ponseti method in this population over a 5-year period. We treated 29 patients with 43 nonidiopathic and 97 patients with 138 idiopathic CTEV feet. Patients with nonidiopathic CTEV made up 23% of all cases. The commonest etiologies were arthrogryposis (5 cases), trisomy 21 (4 cases), and spina bifida (3 cases). Average follow-up was 39 (nonidiopathic group) and 35 months (idiopathic group). RESULTS: The Ponseti method was initially successful in 91% of nonidiopathic and 98% of idiopathic feet. Recurrence of deformity occurred in 44% of nonidiopathic and 8% of idiopathic feet. Thirty-seven percent of nonidiopathic feet required extensive surgical release compared with 2% in the idiopathic group. CONCLUSIONS: Although the success rate of the Ponseti method in nonidiopathic CTEV is inferior to that in idiopathic CTEV, 63% of our nonidiopathic patients did not require extensive surgery. We believe that the Ponseti method should be used in all cases of nonidiopathic CTEV. LEVEL OF EVIDENCE: Level III--prospective cohort study.

Multimodality treatment of a complex series of parallel pathologies in a 16-year-old male that ultimately leads to bilateral hip arthroplasty surgery: A case report.

INTRODUCTION: Femoral head osteonecrosis is associated with significant sequelae for the patient in the long-term. A combination of factors are thought to contribute to the pathophysiology behind this debilitating disease process. PRESENTATION OF CASE: We report on a 16-year-old adolescent male requiring staged bilateral hybrid hip arthroplasty within 3years of diagnosis of acute lymphoblastic leukaemia that ran a complicated course of multimodality treatment. DISCUSSION: The case examines the literature on femoral head osteonecrosis in this population cohort and the challenge for the orthopaedic surgeon in the management of this increasingly prevalent condition. CONCLUSION: In a young population cohort, it is important to have arthroplasty surgery as part of the management armamentarium, especially when coupled with complex pathology.

Mobility in Hurler syndrome.

Hurler syndrome is an autosomal recessive metabolic storage disease. Hematopoietic stem cell transplant increases life expectancy, but the effects on associated musculoskeletal abnormalities remains unclear, and long-term data are limited. We detail the follow-up of 23 patients at a mean of 8.5 years after successful hematopoietic stem cell transplant. All patients underwent clinical examination at an annual multidisciplinary clinic. 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. Eight patients underwent bilateral pelvic and femoral derotation (mean age at surgery, 4.4 years); 4 patients had pelvic osteotomy only. Mean preoperative acetabular angle was 34 degrees. Genu valgum of variable severity due to failure of ossification of the lateral aspect of the proximal tibial metaphysis was observed early, and 6 patients underwent medial epiphyseal stapling, decreasing tibiofemoral angle by a mean of 8 degrees. Clinically, all patients were independently mobile, with restriction of internal hip rotation being the most significant clinical finding. Valgus knees and pronated feet were a typical finding. This cohort represents one of the largest available for study, and ongoing review will clarify the progression of musculoskeletal problems and determine the effectiveness of orthopaedic intervention.

Scoliosis: a review.

Scoliosis, lateral curvature of the spine, has been studied since Hippocrates' time, but remains a disputed subject in orthopaedic surgery, because of its several varieties, unknown cause and unpredictable course. A review of 30 years' experience in a paediatric orthopaedic unit was undertaken to clarify the problem. Patient records, collected prospectively, were examined to demonstrate the incidence, prevalence, extent, course and outcome of the commonest variety, adolescent idiopathic scoliosis. Reference was made to the results of previously published studies. Records from a school screening programme showed that, while slight degrees of spinal curvature were widely prevalent in the community, these were of no clinical significance and major cosmetic deformity was rare: only 8 in 10,000 adolescent girls had Cobb angles of 40 degrees or more and only half of these underwent surgical correction. Evidence to support non-operative treatment could not be demonstrated and it did not reduce the incidence of surgical intervention. Because the course of scoliosis did not seem to correspond with much published work, or with current hypotheses of aetiology, a rethinking of the whole subject is advocated. An alternative model of pathogenesis deriving from developmental biology was proposed. While advances in surgical methods have been significant, the core problems of aetiology and natural history remain. Until they are resolved, all conclusions on management must be provisional. This is where innovative thinking needs to be directed.

A randomized trial of 'soft cast' for distal radius buckle fractures in children.

A buckle fracture is a stable fracture where there is a dorsal cortex compression of the distal radius. A total of 117 children with buckle fracture treated at our institution were randomised prospectively into two treatment groups: soft cast or rigid cast. The rigid cast group attended clinic after three weeks for removal of the cast. In the soft cast group, the cast was removed by parents at home after three weeks. Telephone follow-up was carried out after 4 to 5 weeks post fracture. Both groups had full recovery as compared to the uninjured side and parents were satisfied with the treatment. Only one patient in the soft cast group had a problem as compared to 5 in the rigid cast group (p = 0.035, using chi square test). When given a choice, parents of children in both groups opted for future treatment with a soft cast (p < 0.01 using chi square test). Our study showed that buckle fractures of the distal radius can safely be treated with a soft cast without the need for more than one fracture clinic appointment.

Pediatric trampoline injuries.

INTRODUCTION: The recreational use of trampolines has increased dramatically during the last 10 years. There has been a striking increase in the number of children presenting to fracture clinics with injuries associated with trampoline use. This increase in trampoline injuries has been reported in North America, but there has been a paucity of research in this area in Europe. METHODS: We prospectively recorded details of patients presenting to our institution, Our Lady's Children's Hospital, Crumlin (Dublin, Ireland), during the busy summer months of June, July, and August 2005. Details recorded included type and mechanism of injury, the mode of referral, treatment, inpatient days, outpatient visits, specific details relating to trampoline safety, and an analysis of the cost of medical care. RESULTS: There were 101 patients treated for trampoline-related injuries in 3 months from June to August 2005. This represented 1.5% of the total attendances to the emergency department. The average age was 8.5 years (range, 1.4-17.4 years). There were 55 fractures, 38 soft tissue injuries, 5 head injuries, and 5 neck injuries, with an average Pediatric Trauma Score of 11.4. Fifty seven percent (58/101) of patients were on the trampoline with at least 1 other person. Twenty patients (19.8%) were admitted to hospital requiring 71 inpatient days. Twelve patients were treated in theatre. There were 163 fracture clinic visits, 212 x-rays, and 2 magnetic resonance imaging scans. CONCLUSIONS: Trampolines are a high-risk activity with the potential for significant orthopaedic injury. In Ireland, we have recently seen a dramatic increase in pediatric trampoline-related injuries mirroring the trend in the United States during the last 10 to 15 years. We found that more than 1 individual on a trampoline is a major risk factor for injury, where the lightest person is 14 times more likely to be injured than the heavier. The lighter person also has a greater chance of being injured with smaller numbers on the trampoline. We reiterate the American Academy of Pediatrics policy statement advice that trampolines be used only in supervised training programs--never at home, in outdoor playgrounds, or in schools. The public should be made aware of the potential dangers of trampolines through public health campaigns, radio, and television.

Treatment of femoral shaft fractures in children using the "Tobruk" method.

The "Tobruk" technique of plaster augmentation of a Thomas' splint can be used for the treatment of femoral shaft fractures in children. The radiological and clinical data of 118 patients treated using this method were reviewed over a consecutive 3-year period. The mean age of the patients was 4.25 years. Mean hospital stay was 14.11 days. Mean time spent in the "Tobruk" splint was 44.77 days. Loss of reduction during splintage occurred in 9.32% of patients. Skin complications occurred in 5 patients (4.2%), and cast repairs were necessary in 3 patients. "Tobruk" splinting is a relatively safe and effective way of treating femoral shaft fractures in children. Although its use is not widespread, especially in the United States, until better results are achieved with spica casting and elastic stable intramedullary nails, it does provide a valid option in the treatment of these fractures. A controlled randomized prospective trial with long-term follow-up is needed to fully elucidate its value.

Langerhans' cell histiocytosis of the clivus: case report and literature review.

This report describes a 5-year-old girl with Langerhans' cell histiocytosis (LCH) of the clivus. To date only five patients, including our patient, have been described with LCH at this site. Our patient differs from those previously reported by her atypical clinical presentation with torticollis, but without a sixth nerve palsy. In addition, she is the first patient to present with concomitant disease elsewhere at the time of diagnosis, i.e. both femoral necks and left proximal humerus. Our patient thus presents unique features and underlines the importance of including LCH in the differential diagnosis of erosive lesions of the clivus.

Growth patterns in children with congenital vertebral anomaly.

STUDY DESIGN: Height and weight data in children with congenital vertebral anomaly, recorded routinely during clinical follow-up, were analyzed. OBJECTIVES: To determine whether these children are significantly deficient in growth or show an abnormality of maturation. SUMMARY OF BACKGROUND DATA: Structural abnormalities in the vertebral column, and surgery to prevent or correct deformity secondary to this, clearly have the potential to affect the individual's final height. It is not clear, however, whether this is the whole problem, or whether there are other factors to be considered. METHODS: The height and weight of patients with all forms of spinal deformity are routinely recorded at outpatient visits. Data from patients with congenital vertebral abnormalities were analyzed and compared with the national standards. To test the possibility that the observed short stature might be familial, family groups (parents and siblings of patients) were invited to be measured. RESULTS: At the end of growth, patients with congenital vertebral anomalies were smaller than average and also smaller than their parents and siblings. This was more marked in girls and in the presence of associated anomalies, especially when multiple, as in VACTERL association (vertebral, anal, cardiac, tracheal, esophageal, renal, and limb anomalies). It was also exacerbated by surgery, which had a demonstrable stunting effect on the spine. DISCUSSION: Although shortening of the spine was demonstrated in patients with prior spinal surgery, the contributions of the fact of surgery and the presumed severity of anatomic abnormality could not be differentiated. The observations that, even without surgery, girls were generally on a lower percentile than boys and that both have a tendency to lose height at growth spurt invite further investigation. CONCLUSIONS: The shortening of the spine by surgery is not a contraindication to treatment, because a straight, balanced spine must always be preferable to a longer, crooked and unbalanced one. However, it is another facet to be remembered when considering the management plan of the whole child.

Long-term results from in situ fusion for congenital vertebral deformity.

STUDY DESIGN: Retrospective review of long-term outcome of fusion in situ for congenital vertebral anomaly with particular emphasis on cosmesis and the incidence of reoperation. OBJECTIVE: Examination of the success rate of this procedure and of risk factors for failure. BACKGROUND: Fusion in situ is the accepted prophylactic treatment to prevent deformity in congenital vertebral anomalies that have a high risk of progression or have been shown to be deteriorating. METHODS: Records of patients who were at least 15 years of age at last examination were reviewed retrospectively. Consideration was given to cosmetic outcome and to the incidence of reoperation. RESULTS: There were 43 patients in this category, 19 boys and 24 girls, who were at least 15 years of age when last seen. Reoperation had been performed in 11 cases (25.6%). The main finding was that, although the Cobb angle of the fused segment of spine remained constant after fusion, a curve sometimes developed in the whole spine, sometimes (but by no means always) centered on that fused segment. Cosmetic deformity continued to progress in a number of cases. CONCLUSIONS: Localized fusion, whether posterior alone or anterior and posterior combined, was effective in preventing progression of the Cobb angle of the congenitally malformed area but did not control the overall deformity that developed or progressed with growth. Current concepts of the pathomechanism of deformity do not adequately explain the observations, and a more biologic approach is suggested.