The treatment of acetabular dysplasia in adolescents (age>
12) is difþcult and various complex pelvic osteotomies have been described. The aim of surgery being improvement in pain and to delay the onset of secondary osteoarthrosis.
We performed limb lengthening and correction of deformity of nine long bones of the lower limb in six children (mean age, 14.7 years) with osteogenesis imperfecta (OI). All had femoral lengthening and three also had ipsilateral tibial lengthening. Angular deformities were corrected simultaneously. Five limb segments were treated using a monolateral external fixator and four with the Ilizarov frame. In three children, lengthening was done over previously inserted femoral intramedullary rods. The mean lengthening achieved was 6.26 cm (mean healing index, 33.25 days/cm). Significant complications included one deep infection, one fracture of the femur and one anterior angulation deformity of the tibia. The abnormal bone of OI tolerated the external fixators throughout the period of lengthening without any episodes of migration of wires or pins through the soft bone. The regenerate bone formed within the time which is normally expected in limb-lengthening procedures performed for other conditions. We conclude that despite the abnormal bone characteristics, distraction osteogenesis to correct limb-length discrepancy and angular deformity can be performed safely in children with OI.
Complications other than AVN were re-subluxation (3), redislocation(4), fractures (1), ankylosis, LLD(4), infection (2). There was only 1 (5.9%) complication in primary referral group and 13 (30.2%) in tertiary referral group (p=0.050). Tonnis grade of subluxation, presence of ossific nucleus, tear drop shape, Mose’s grading, CE angle of Wiberg were documented but were not found to significantly affect the outcome.
Upper limbs are commonly involved in Arthrogyposis Multiplex Congenita. They may be involved in isolation or in combination with lower limbs. There are two patterns of involvement in upper limbs. The most common (type I) pattern presents with adduction and internal rotation at the shoulder, extension at the elbow, pro-nation of the forearm and flexion deformity of the wrist, indicating involvement of the C5 and C6 segments. These deformities can be quite disabling and may require surgery to help improve function. We present our long-term results with pectoralis major transfer procedure (as modified by senior author MJB) to restore elbow flexion in seven patients (ten procedures). Results: Early results in all our patients were quite encouraging. Six patients retained useful power in transferred pectoralis major muscle and maintained the arc of flexion, which was attained following tricepsplasty. However, as children were followed up a gradually increasing flexion deformity and decreasing flexion arc were observed in eight elbows. The onset and progression of flexion deformity was gradual and progressive. The flexion deformity reached ninety degrees or more in all cases. Conclusions: Results of pectoralis major transfer to treat extension contracture of the elbow in arthrogryposis deteriorate with time due to development of recalcitrant flexion deformity of the elbow. Presently we recommend this procedure on one side only in cases of bilateral involvement because if one procedure is carried out it would be possible for this hand to get to the mouth for feeding and the other unoperated side would be able to look after the perineal hygiene
Children with osteogenesis imperfecta(OI) have multiple long bone fractures with subsequent deformities. The mainstay of treatment is correction with multiple osteotomies and intramedullary fixation. The Shefffield intramedullary telescoping rod system has been successful in the treament of long bone fractures and deformities (Wilkinson et al ,JBJS-B,1998) Bisphosphonates (Pamidronate -1- 1.5mg/kg/day)have been used as adjuvant therapy in the treatment of OI since the last five years. The perceived benefits include reduction in fracture frequency, improvement in bone density and a general feeling of well being. We present our experience of five cases of OI who developed infections around thier Sheffield telescoping rods while on Pamidronate therapy. There was only one case of sepsis over a ten year period(over eighty patients)in a previously reported series from our centre. The time interval between the start of Pamidronate therapy and the diagnosis of infection varied between 12–36 months ie. between 4–12 cycles of Pamidronate (parenteral administration over a three day period at three month intervals). All patients had their intramedullary rods in situ from anywhere between 2–7 years. The infections were low grade with a 2–3 month period of dull ache prior to actual presentation. Intrestigly though all patients had multiple rods in situ, only one of their femoral rods was affected and they did not have any other infective focus at the time of diagnosis. Three patients presented with thigh abcesses while the other two presented with ipsilateral knee pain and effusion. All had raised inflammatory markers, radiological signs of sepsis with Staph Aureus the commonest infecting organism. Those cases presenting with abcesses were treated by drainage and rod removal, however only antibiotics were sufficient in the rest. The relationship between Pamidronate therapy and these infections is not absolutely clear and has not been reported previously. The possible links are discussed and a high degree of suspicion is recommended for those cases of OI on bisphosphonate
Objective: To review the hip subluxations or dislocations occurring during femoral lengthening in patients with congenital longitudinal lower limb deficiencies. Methods: Sixty-three patients with congenital longitudinal lower limb deficiencies underwent femoral lengthening using either De Bastiani, Villarubias or Ilizarov technique. Acetabular index, medial joint space, CE angle of Wiberg, acetabular angle of Sharp and neck-shaft angle were measured on anteroposterior radiographs of hip before, during and after lengthening. The Acetabulum was considered dysplastic when the Sharp angle was more than 45 degrees. Hip was considered to be subluxed when the medial joint space increased during lengthening. Results: During femoral lengthening, eleven hips sub-luxed as measured by the increase in medial joint space and one hip dislocated. All these hips had a preoperative acetabular index more than 25 degrees, CE angle less than 20 degrees and Sharp angle more than 45 degrees. The average neck-shaft angle was 75 degrees. Following subluxation, lengthening was stopped and the hips were reduced in hip spica after adductor and sartorius tenotomies. In one patient femoral shortening and acetabulo-plasty had to be done to reduce the subluxation. No case of avascular necrosis was noted. Conclusion: Hip subluxation during femoral lengthening of congenital longitudinal lower limb deficiencies tends to occur when the acetabular index is more than 25 degrees, Sharp angle is more than 45 degrees, CE angle is less than 20 degrees and when there is associated femoral coxa vara. Careful preoperative assessment is required, and if need be hip reconstruction prior to lengthening. Close monitoring during lengthening is recommended.
This study demonstrates that the NP documentation follows the guidelines identified by the RCGP, conversely it was not possible to assess from the GP documentation if all the steps had been followed. The mean average LBOS in the NP patients was slightly higher than those in the GP group, was this because these patients were having guideline applied care as opposed to “usual care”? Evaluation of the patient recall of information shows the NP sent five patients for X-ray even though this did not occur and is not recommended in guidelines. Conversely twenty-three patients can remember being given the “Back Book” by the GP but this was only documented in three cases. We believe that patient recall demonstrates an ineffective way to measure outcome and funding allocation for back pain management and needs to more accurately reflect the evidence.
Increased incidence of complications has been reported when lengthening limbs with underlying bone disorders such as dysplasias and metabolic bone diseases. There is a paucity of literature on limb lengthening in Osteogenesis Imperfecta (OI), probably due to the concern that the bone containing abnormal collagen may not tolerate the external fixators for a long term and there may not be adequate regenerate formation from this abnormal bone. We performed limb lengthening and deformity correction of nine lower limb long bones in six children with OI. Four children were type I and two were type IV OI as per Sillence classification. The mean age was 14.7 years. All six children had lengthening for femoral shortening and three of them also had lengthening for tibial shortening on the same side. Angular deformities were corrected during lengthening. Five limb segments were treated using a monolateral external fixator and four limb segments were treated using an Ilizarov external fixator. In three children, previously inserted femoral intramedullary nails were left in situ during the course of femoral lengthening. The average lengthening achieved was 6.26 cm. Limb length discrepancies were corrected to within 1.5 cm of the length of the contralateral limb in five children. In one child with fixed pelvic obliquity and spinal scoliosis, functional leg length was achieved. The mean healing index was 33.25 days/cm of lengthening. Among the complications significant ones included, one deep infection, one fracture through the midshaft of the femur, and development of anterior angulation deformity after the removal of the fixator in one tibia. Abnormal bone of OI tolerated the external fixator throughout the period of lengthening without any incidence of migration of wires and screws through the soft bone when distraction forces were applied. The regenerate bone formed within the time that is normally expected in limb lengthening procedures performed for other conditions. We conclude that despite abnormal bone characteristics, limb reconstruction to correct limb length discrepancy and angular deformity can be done safely in children with OI.
Acute lateral dislocation of the patella has been associated with disruption of the medial restraints of the patella. Following non-operative management there is a redislocation rate of up to 44%. This is an observational study testing whether sonography is a reliable method of assessing the medial retinaculum after acute dislocation of the patella. Ten patients following acute patellar dislocation had an ultrasound scan (USS) performed by an experienced musculoskeletal radiologist. Each patient subsequently had an examination under anaesthetic, arthroscopy, and repair of the ruptured structures. The ultrasound reports were compared to the surgical findings to determine the accuracy of this investigation. USS located deficiencies in the ligamentous attachments to the medial border of the patella and the presence of avulsed bony fragments, all of which were confirmed at operation. The sonographic diagnosis of haematoma or torn fibres in the vastus medialis obliquus corresponded with our operative findings. The most significant findings were the correlation of free fluid around the medial collateral ligament (MCL) with avulsion of the femoral attachment of the medial patellofemoral ligament (MPFL) and the presence of avulsed fragments of bone from the medial border of the patella. Sonography, in cadaveric studies consistently identifies the retinacula and like MRI offers a distinctive constellation of findings that can be used in diagnosis and therefore play a significant role in directing surgical management of these patients. We have found Sonography to be readily available and accurate. This report does not include surgical outcome since the follow up is short and incomplete. We do, however, feel that ultrasound shows the state of the soft tissue restraints of the patella following lateral dislocation.
113 consecutive patients with soft tissue sarcoma treated by excision and reconstructive flaps were studied to assess the risk of complications and to compare local tumour control with those in whom primary wound closure was possible. Minimum follow-up was 24 months and mean age was 55 years (16–95). The sarcoma was located in the lower extremity in 83 and upper extremity 30 patients. Significant wound complications developed in 37 patients (33%). The most common complications were wound infections or partial necrosis occurring in 16% (18/113) and 13% (15/113) respectively. Complete flap necrosis requiring flap removal occurred in 6 patients (5%). Three patients (2.3%) required amputation as a result of complications. Significant risk factors for development of wound complications include location of tumour in the lower limb compared to upper limb (relative risk 2.3, p=0.02) and use of pre-operative radiotherapy compared to no or post-operative radiotherapy (relative risk 2.05, p=0.02). There was no difference in rates of complications in patients with free or pedicled flaps, tumours <
or >
5cm, distal or proximal location of tumour. The rates of negative excision margins (80%) and wound complications in patients who required reconstructive flaps were not different from that for the other patients treated at our centre who did not require reconstructive flaps. The use of soft tissue reconstructive flaps did not reduce the risk of positive excision margins or the rates of wound complications. The risk of amputation secondary to flap complication or failure is low.
812 consecutive patients with soft tissue sarcoma of the extremity were studied to compare the characteristics and outcome of patients who had primary amputations and limb preserving surgery. Patients with primary amputations were more likely to have metastases at presentation, high-grade tumours, larger tumours and were older. The most frequent indications for primary amputation were tumour excision which would result in inadequate function and large extracompartmental tumours with composite tissue involvement including major vessels, nerves and bone. The requirement for primary amputation was a poor prognostic factor independent of tumour grade, tumour size and patients’ age.
To determine if rates of local recurrence and metastasis differ in upper versus lower extremity sarcomas. Prospectively collected data relating to patients undergoing limb-sparing surgery for extremity soft tissue sarcoma between January 1986 and April 1997 were analysed. Local recurrence-free and metastasis-free rates were calculated using the method of Kaplan and Meier. Univariate and multivariate analyses of potential predictive factors were evaluated with the log-rank test and the Cox proportional hazards model. Of 480 eligible patients, 48 (10. 0%) had a local recurrence and 131 (27. 3%) developed metastases. Median follow-up of survivors was 4. 8 years (0. 1 to 12. 9). There were 139 upper and 341 lower extremity tumours. Upper extremity tumours were more often treated by unplanned excision before referral (89 vs 160, p<
0. 001) and were smaller (6. 0cm vs 9. 3cm, p<
0. 000). Lower extremity tumours were more often deep to or involving the investing fascia (280 vs. 97, p<
0. 003). The distribution of histological types differed in each extremity. Fewer upper extremity tumours were treated with adjuvant radiotherapy (98 vs. 289, p<
0. 000). The 5-year local recurrence-free rate was 82% in the upper and 93% in the lower extremity (p<
0. 002). Local recurrence was predicted by surgical margin status (hazard ratio 3. 16, p<
0. 000) but not extremity (p=0. 127) or unplanned excision before referral (p=0. 868). The 5-year metastasis-free rate was 82% in the upper and 69% in the lower extremity (p<
0. 013). Metastasis was predicted by high histological grade (hazard ratio 17. 28, p<
0. 000), tumour size in cm (hazard ratio 1. 05, p<
0. 001) and deep location (hazard ratio 1. 93, p<
0. 028) but not by extremity (p=0. 211). Local recurrence is more frequent after treatment for upper compared with lower extremity sarcomas. Variation in the use of radiotherapy and differences in histological type may be contributory. Metastasis is more frequent after treatment for lower extremity sarcomas because tumours tend to be large and deep.