Aim

The aim of the study was to characterise gait in patients with achondroplasia post lengthening.

Purpose

To examine the effect of limb lengthening on the quality of life of people who have disproportionate short stature (DSS).

The availability and usage of portable image intensifiers has revolutionised routine orthopaedic practice. Many procedures have become simpler, easier, less invasive and less time-consuming. Extensive use of fluoroscopy can, however, result in significant radiation exposure to operating staff. An accumulated dose of 65 (Sv after multiple exposures has been reported to increase the risk of thyroid cancer many years later. Previous studies have shown that it is possible to exceed this dose during various orthopaedic procedures. Though thyroid shields are extensively available most orthopaedic surgeons do not use them. The present study was aimed at measuring the scattered dose to thyroid during DHS/IMHS for neck of femur fractures and IM nailing for long bone fractures and thereby emphasise the need for operating theatre personnel to wear a thyroid shield.

A prospective study of 32 consecutive procedures was carried out. The EDD Unfors dosimeter was used to measure the tissue specific exposure dose to thyroid. Measurements were also obtained from the mobile C-arm fluoroscope unit, which calculated the total number of images and the total dose and duration of radiation for each procedure. Other factors including the grade of surgeon, the total number of theatre personnel wearing the lead gown and/or the thyroid shield and the duration of surgery were also recorded.

In 32 procedures, the dose of 65 (Sv was exceeded 13 times; 8 times during DHS/IMHS and 5 times during IMN. The average thyroid dose was 142 (Sv during IMN and 55 (Sv during DHS. Only 9 of 223 (4%) theatre personnel were using a thyroid shield in spite of its availability. The results suggest that the thyroid is frequently exposed to potentially harmful radiation during these procedures. Strict inclusion of a thyroid shield as a part of routine radiation protection is recommended.

Opinions about the treatment of Perthes' disease vary widely. However there is no disagreement about the need for containment during fragmentation stage to create an optimum biomechanical environment for remodelling of femoral head. Types of containment may vary. Younger children do well irrespective of the method of containment. Older children usually require surgical containment. The present study was aimed at evaluating the results of different methods of surgical containment in different age group and identifying specific factors that alter the final outcome and prognosis.

107 cases were reviewed retrospectively. 21 cases were excluded due to lack of records. 86 hips were available for clinical and radiological evaluation. 31 patients were under 7 years and required Varus osteotomy (VO). 55 patients were above 7 years. VO was performed in 30 hips and Shelf containment was done in 25. Case notes were reviewed for demographic details, surgical details and clinic letters. Radiographs were reviewed for Herring's grading, Stulberg staging, containment indices, centre edge angle, lateral pillar height, Mose index, neck shaft angle and shelf width.

In all patients, there was an improvement in pre-operative symptoms and summated range of motion, especially abduction. Good functional and radiological outcome was seen in age group < 7 years. In older children, outcome was good to satisfactory with Herring grade B. Stulberg grading worsened with advancing age and Herring grade C, irrespective of the method of containment. Persistence of varus neck shaft angle and trochanteric overgrowth were significant problems with VO. Although all containment indices improved with Shelf group, Stulberg grading remained poor in most patients.

The lateral pillar classification and age strongly correlate with final outcome. Herring group C had the least favourable result. Stulberg staging remained poor in older children irrespective of the method of containment.

Aim: Spinal stenosis is a known entity in achondroplasia and a need for screening for the symptomatic children was identified in a tertiary limb reconstruction service. The aim of this study was to evaluate whether clinical and radiological imaging would identify the at risk group.

Materials and methods: 205 achondroplastic children were treated at our service in the last 20 years. A prospective clinical screening programme for spinal stenosis which also included the MRI imaging of entire spine was however introduced only in 1996. 26 patients were available for this study. Case notes were reviewed for clinical symptoms and neurological signs. MR images were assessed for stenosis especially at Cervico medullary junction and lumbar level. Canal diameters were measured at all spinal levels from CMJ to lumbo-sacral junction. Neurosurgical interventions were recorded. An attempt was made to identify high risk factors that required surgical intervention.

Results: Delayed milestones were observed in 7 patients. Positive history of back pain or radicular pain was obtained in 10 whereas 10 patients had symptoms of neurogenic claudication. Brisk reflexes and clonus were consistent findings. Severe foramen magnum narrowing was observed in 23 patients and 13 showed ‘kinking’ or ‘pinching’, suggesting severe compression. Of the 17 patients seen by neurosurgeons 6 required single or multiple surgical decompressions. Ventriculo-peritoneal shunt was inserted in 4. 5 children required CMJ decompression and one required lumbar canal decompression. There was a 7 mm difference in AP diameter measured on MRI at CMJ between the two non operated and operated groups. Female patients with delayed milestones and CMJ narrowing to less than 25 mm were identified as high risk.

Conclusion: The cause of increased morbidity and mortality in young achondroplasts is attributable to severe CM compression. Sudden death can occur by acute or gradual progressive encroachment of respiratory centre at medulla oblongata. We were able to identify symptomatic patients early before developing myelomalacia and cord changes by screening all symptomatic children with achondroplasia. We propose that female gender, especially with delayed milestones as another high risk factor. We stress upon the importance of developing a nationalised selective screening programme with guidelines in specialist orthopaedic and joint multi-disciplinary Skeletal Dysplasia clinics.

Abstract: Arthrograms are commonly carried out in conjunction with an examination under anaesthesia before surgical intervention in Perthes disease. It is routinely undertaken as an independent day case procedure in the radiology department. Our protocol dictates that a containment procedure is considered in patients who lose their ability to abduct the affected hip during the fragmentation stage of the disease. The main role of the arthrogram and EUA is to confirm that the affected hip has adequate abduction under anaesthesia to meet the prerequisites of a varus osteotomy and to confirm that the head is containable with no hinge abduction. This study was carried out to investigate whether arthrogram findings ever altered the pre operative clinical decision. Our findings question the role of arthrography as a routine before surgical intervention and the cost benefit ratio is evaluated.

Material and methods: The study was carried out at Alder Hey Hospital, UK. 107 cases were reviewed retrospectively. This included the treatment decision before arthrogram on the last clinic letter, any major findings on arthrogram and change in treatment decision after the procedure. The acetabular head index, central edge angle and the lateral pillar height were measured on routine antero-posterior radiographs and arthrogram on affected side.

Results: 22 patients were excluded from the study due to inadequate records. Out of the remaining 85 there was no change in decision in 69 patients after the procedure. Only 5 patients required different management after arthrogram than what was planned earlier based on clinical findings. We found a good correlation between AHI measured on pre-operative x-ray and the arthrogram. The study revealed that the surgical decision based on clinical findings, was not altered by arthrography in most cases. The EUA findings were more valuable. It helps the surgeon to decide the angle of varus osteotomy, required to contain the femoral head in acetabulum and confirms an adequate abduction to undertake the procedure. This quick assessment can be carried out at the time of the proposed osteotomy under the same anaesthesia without the need for arthrography. In conclusion we recommend that arthrography should be reserved for questionable cases where clinical findings are inconclusive and therefore the appropriateness of treatment is uncertain