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.
The aim was to demonstrate that day case Anterior Cruciate Ligament (ACL) reconstruction, without the use of a tourniquet, is clinically effective, cost effective, safe and “patient choice” procedure. Fifty patients who underwent day case, arthroscopic, ACL reconstruction without the use of a tourniquet, but using saline and epinephrine, pump regulated, irrigation. The same surgeon performed each case for the period May 2003 to April 2005. Seven patients had their tendons reconstructed with the use of patellar tendon grafts, the remainder, 43 patients, had hamstring tendon grafts. The study included 6 women and 44 men. This prospective study assessed cost effectiveness, clinical efficacy by measuring post-operative pain and postoperative results and finally whether this procedure remained the “patient choice”. The mean age was 30.6 years, (range 16 – 46). In addition to assessing level of immediate post operative pain the patients were also assessed at two weeks and six weeks for pain, range of movement, swelling and for the occurrence of any early post-operative complications. We were able to show that there was a significant cost benefit, approximately one third to a half in comparison to other local surgeons; that the study was clinically effective and that there were no reported early complications; and that all 50 patients would choose to have the surgery again as a day case procedure with this technique. We would like to present day case ACL reconstruction without the use of a tourniquet, as a safe option for the carefully selected patient.
This is a report of 30 patients who underwent arthroscopic, Anterior Cruciate Ligament (ACL) reconstruction without the use of a tourniquet, but using saline and epinephrine, pump regulated, irrigation. Each case was performed as a day case by the same surgeon for the period May 2003 to December 2004. 5 patients had their tendons reconstructed with the use of patellar tendon grafts, the remainder, 25 patients, had hamstring tendon grafts. The study included 4 women and 26 men. This prospective study assessed cost effectiveness, clinical efficacy by measuring post-operative pain and post-operative results and finally whether this procedure remained the “patient choice”. The mean age was 30.6 years, (range 17 – 46). In addition to assessing level of immediate post operative pain the patients were also assessed at two weeks and six weeks for pain, range of movement, swelling and for the occurrence of any early post-operative complications. We were able to show that there was a significant cost benefit, approximately one third to a half in comparison to other local surgeons; that the study was clinically effective and that there were no reported early complications; and that all 30 patients would choose to have the surgery again as a day case procedure with this technique. We would like to present day case ACL reconstruction as a safe option for the carefully selected patient and as a procedure that could perhaps be included in the orthopaedic basket for day case surgery in the UK.
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.
The restoration of pain-free stable function in gleno-humeral arthritic cases in various situations such as rotator cuff deficiency, old trauma and failed total shoulder arthroplasty is a challenging clinical dilemma. The Bayley-Walker shoulder has been designed specifically for very difficult cases where surface replacement devices do not provide sufficient stability. This device is a fixed-fulcrum reversed anatomy prosthesis consisting of a titanium glenoid component with a CoCrMo alloy head that articulates with an UHMWPE liner encased in a titanium alloy humeral component that has a long tapered grooved stem. The centre of rotation of the Bayley-Walker shoulder is placed medially and distally with respect to the normal shoulder in order to improve the efficiency of the abductor muscles. An important problem in devices of this type is obtaining secure and long-lasting fixation of the glenoid component. The glenoid component relies on fixation through the cortical bone by using threads, which protrude through the anterior surface of the scapula at the vault of the glenoid. It is HA coated for subsequent osseointegration. The purpose of this study was to investigate fixation of the glenoid component. A 3D finite element model of the glenoid component implanted in a scapula was analysed using Abaqus. The implant was placed in position in the scapula, with the final 2–3 screw threads cutting through the cortical bone on the anterior side at the vault of the glenoid due to the anatomy in this region. The analysis was performed for two load cases at 60° and 90° abduction. A histological study of a retrieval case, obtained 121 days after implantation, was also conducted. The FEA results showed that most of the forces were transmitted from the component to the cortical bone of the scapula, the remaining load being transmitted through cancellous bone. In particular the area where the threads of the glenoid component penetrated the scapula showed high strain energy densities. Histology from the retrieved case showed evidence of bone remodelling whereby new bone growth resulting in cortical remodelling had occurred around the threads. Both the FEA and histological study show that fixing the component at multiple locations in cortical bone may overcome the problems of glenoid loosening associated with constrained devices. The Bayley-Walker device has been used on a custom basis since 1994; 81 Bayley–Walker shoulders for non-tumour conditions and 43 Bayley-Walker glenoid components have been used in association with a bone tumour implant, with good early results. Radiographically, radiolucencies have not been observed and overall the comparisons with the original Kessel design are positive.
A computer-based image analysis system has been developed as a research tool in total hip replacement. The system has been programmed to take multiple measurements from coronal plane radiographs. Poor quality radiographic images can be enhanced and standardised. The measurements which can be obtained include stem subsidence, cup migration, cup wear, and stem loosening. Reproducibility and accuracy were +/- 0.01 mm and +/- 0.5 mm respectively. The present application is in retrospective research, but prospective monitoring of radiographs is planned.