C. de Bodman

The arthritic wrist. I - The degenerative wrist: Surgical treatment approaches

UNLABELLED The primary goal in treating a degenerative wrist is to provide pain relief, while maintaining strength and mobility if possible. After failure of the recommended conservative treatment, the choice of approaches can be made from a large collection of techniques, some which are well validated. Partial wrist fusion, particularly the Watson procedure, results in a pain-free wrist in 80% of cases, with 50% of the mobility preserved, good grasping strength and stable results for at least 10 years. Proximal row carpectomy provides similar results if the cartilage on the head of the capitate is preserved and the patient is not involved in heavy manual labour. Complete denervation provides pain relief in almost 80% of cases while preserving motion and strength. This is a safe and effective option, with no age limit, that still allows other procedures to be performed in the future. Total wrist fusion also has its place in revision, and even as first-line treatment, because of the reliable outcome in terms of pain and strength, high satisfaction rates, little to no repercussions linked to the loss of mobility and fewer complications. Other techniques are now available. The partial or complete resection of a carpal bone and placement of an implant is back in vogue because of the availability of pyrocarbon. Such implants are an option in the future for localized osteoarthritis or even diffuse affections, and a useful alternative to more invasive procedures. The use of a rib cartilage graft to partially or completely replace a carpal bone or resurface the radius has promising results in terms of pain reduction and fusion. The role of total joint replacement must be defined relative to the classic, reliable techniques that have long-term outcome data. LEVEL OF PROOF IV.

The progression of lumbar curves in adolescent Lenke 1 scoliosis and the distal adding-on phenomenon.

INTRODUCTION The postoperative deterioration of the curve below spinal fusion instrumentation resulting in a distal adding-on (AO) phenomenon in idiopathic adolescent scoliosis (IAS) frequently requires surgical revision with disappointing secondary clinical results. HYPOTHESIS Analysis of AP (coronal) range of motion (cROM) and lateral (sagittal) range of motion (sROM) on dynamic (side-bending, flexion, extension) X-rays to determine the choice of the lowest instrumented vertebra (LIV) can help reduce distal adding-on. The goal of this study was to study the postoperative progression of the lumbar curve in Lenke 1 scoliosis operated on with a LIV based on dynamic X-ray results. MATERIALS AND METHODS Right-sided Lenke 1 IAS that was treated surgically by posterior arthrodesis alone with a follow-up of at least 2 years was included in the study. The following radiographic parameters were evaluated: the Cobb angles of the curves, reducibility of the curves, the apex of the scoliosis, the central sacral vertical line, the stable vertebra (SV), the neutral vertebra (NV), the distances between the CSVL and the centroids of the LIV and of the first vertebra below instrumentation, as well as the tilt of the superior endplates. sROM and cROM were determined on dynamic X-rays. RESULTS Fifty IAS were evaluated/185 files. Only three cases fulfilled the criteria for AO including two that were secondary to peri- or postoperative complications. The lumbar curve presented with a loss of correction of 0.9° at one year and 1.14° at the final follow-up. None of the parameters studied were correlated to the deterioration of the lumbar curve. DISCUSSION The choice of the LIV has been shown to influence the deterioration of the lumbar curve and the development of AO. The choice of the LIV based on an analysis of AP (coronal) and lateral (sagittal) range of motion seems to prevent the development of AO. LEVEL OF EVIDENCE 4, retrospective study.

Lumbosacral arthrodesis for neuromuscular scoliosis using a simplified Jackson technique

UNLABELLED Treating patients with severe neuromuscular scoliosis by long spinal fusion improves their quality of life and provides significant comfort for the patient and caregivers. But lumbosacral (L5-S1) fusion is challenging in these patients because of the significant deformities that result in poor bone anchoring quality and a risk of impingement between the skin and implants. In 1993, Jackson described a L5-S1 fusion technique using S1 pedicle screws and intrasacral rods (implanted under X-ray guidance) that are linked to the construct above with connectors. The goal of this study was to evaluate the clinical and radiological results and the postoperative complications of a simplified version of this technique, which does not require connectors or X-ray guidance. MATERIALS AND METHODS Thirty-three patients were evaluated with a minimum follow-up of 4years (average 82months). Frontal balance, sagittal balance, Cobb angle, sacral slope, lumbar lordosis and lateral pelvic tilt in the frontal plane were assessed on preoperative, postoperative and follow-up X-rays. Intraoperative and postoperative complications were recorded. RESULTS Complete fusion was obtained in 32 patients. The average Cobb angle was 62° initially and was reduced to 20° after surgery and 24° at the final follow-up. The average lateral pelvic tilt was 10.3° (0 to 26°) initially; it was surgically corrected to an average of 7.5° (0 to 24°); the average secondary loss of correction was 1.2° (0 to 9°). The sacral slope was corrected to an average of 11.2°; an average of 0.2° had been lost at the last follow-up (0 to 18°). Although the average for lumbar lordosis was unchanged, the standard deviation went from 29° to 16° after the corrective surgery and 17° at the last follow-up, with large cluster of measurements around the average value of 40°. The deformity correction was comparable to the results with other techniques (Galveston, sacroiliac screws); the complication rate was similar but the non-union rate was lower. This simplified Jackson technique appears to be an effective, simple method for L5-S1 fusion to correct neuromuscular scoliosis as it provides stable results over time. LEVEL OF EVIDENCE Level IV, retrospective study.

Le priapisme : une complication pédiatrique grave de la maladie de Fabry

Fabry disease is an X-linked recessive lysosomal storage disorder caused by α-galactosidase A deficiency. Although the disease presents in childhood, diagnosis is often delayed to adulthood or missed, presumably due to the lack of specificity of the symptoms and to the absence of major complication during the paediatric years. We report a 9-year-old boy known to have a Fabry disease who presented an episode of priapism. Successful treatment was achieved by repeated corporeal aspiration under general anaesthesia. This case is the fifth report of priapism in children with Fabry disease, suggesting that priapism may be a severe vascular complication of the disease during infancy. This report emphasizes the importance of an early diagnosis and treatment of Fabry disease, including enzyme replacement therapy, to prevent major disease-associated morbidity and to optimize patient outcomes.