Volar ligament release and distal radius dome osteotomy for correction of Madelung's deformity

PURPOSE: Madelung's deformity is usually recognized near the completion of skeletal growth and corrective osteotomy of the radius is frequently performed to treat the deformity and reduce pain. This study reviewed the clinical and radiographic results of a volar approach, ligament release, and dome osteotomy technique for treatment of this deformity. METHODS: Between 1990 and 2000, 26 wrists in 18 patients were treated with a volar ligament release and dome osteotomy of the distal radius. The average age of the patients at the time of surgery was 13 years. All patients were available for review at an average of 23 months after surgery. Radiographs before surgery and at final follow-up evaluation were analyzed for the extent of correction. RESULTS: All patients treated with this corrective osteotomy reported a reduction in pain and improved appearance. Patients showed improvements in forearm supination and wrist extension, with no loss of pronation or flexion. Improvements in the radiographic parameters of radial inclination and lunate subsidence also were observed. Four wrists required concurrent ulnar-sided surgery, and 3 additional wrists required staged ulnar shortening. One patient required a Darrach resection 3 years after the index procedure. CONCLUSIONS: The results of volar dome osteotomy provide improved range of motion, improved appearance, radiographic correction, and pain relief while preserving soft-tissue support for radial rotation around the distal ulna. Long-term follow-up evaluation is ongoing.     

Achilles tendon lengthening for ankle equinus deformity in hemophiliacs: 23 patients followed for 1-24 years

BACKGROUND: Bleeding in the calf or ankle joint may lead to ankle equinus deformity, particularly in childhood and during adolescence. We assessed the long-term functional and radiographic results after Achilles tendon lengthening for ankle equinus deformity in hemophiliacs. PATIENTS AND METHODS: Between 1975 and 1986, 30 hemophilic patients with pes equinus were surgically managed by Achilles tendon lengthening. Of these, 23 were followed up prospectively twice a year for an average of 13 (1-24) years. The mean age at operation was 29 (12-46) years. The clinical results were documented according to the score of the Advisory Committee of the World Federation of Hemophilia (WFH), while radio-graphs were evaluated using the Pettersson score. On average, preoperative ankle equinus deformity was 21 (5-55) degrees. Mean range of motion was 21 (5-42) degrees prior to surgery. RESULTS: At the first postoperative examination 1 year after surgery, 21/23 cases were improved, and 9/21 reached dorsiflexion to at least neutral position. At the last follow-up, ankle equinus deformity was 10 (4-20) degrees on average. 20/23 patients still showed significant improvement compared to their condition before surgery. 7 patients still had complete correction of the equinus deformity, while mean range of motion decreased constantly over the observation period. The clinical score was significantly improved 1 year after surgery and diminished only slightly afterwards. Radio-graphic outcome deteriorated, with scores rising from 4.3 (1-10) points preoperatively to 7.3 (3-12) points at last follow-up. INTERPRETATION: Most patients treated for hemophilic pes equinus by Achilles tendon lengthening experienced long-term benefit concerning the equinus deformity, but gradually lost overall movement of the ankle joint. Progression of the ankle arthropathy cannot be hindered.     

The effect of pre-existing medical conditions and age on mortality after injury

BACKGROUND: Pre-existing medical conditions (PMCs) have been shown to increase mortality after trauma even after adjustment for the effect of chronological aging. It has been suggested that there is an interaction between injury severity and physiologic reserve, such that diminished physiologic reserve will have an adverse effect on survival at lower injury severity, but that at higher levels of injury severity, physiologic reserve will have much less of an impact. METHODS: Records of 65,743 patients, admitted after trauma, were extracted from the database of the United Kingdom Trauma Network to explore the impacts of age, gender and PMCs on mortality, and modification of these effects by severity of injury. RESULTS: PMCs were categorized as absent (23%), present (23%), or unrecorded (54%). There was an increase in mortality with increasing age at all levels of injury severity. Presence of a PMC was associated with a marked increase in mortality of patients with minor injuries (odds ratio [OR] = 5.9, 95% confidence interval [CI] 4.4, 8.0) or moderate injuries (OR = 2.0, 95% CI 1.4, 2.9), but not in those with more severe injuries (OR = 1.1, 95% CI 0.9, 1.4). The impact of age and male gender were also somewhat more pronounced for patients with less severe injuries. CONCLUSION: These findings support the hypothesis of an interaction between physiologic reserve and injury severity, where PMCs are associated with increased mortality when combined with low to moderate severity injuries, but not when combined with more severe injuries.     

No evidence for association of OAS1 with type 1 diabetes in unaffected siblings or type 1 diabetic cases

Type 1 diabetes is a common autoimmune disorder that is strongly clustered in families. As the sharing of alleles of the HLA class II genes cannot explain all of this aggregation, alleles of multiple other loci are involved. Recently, it was reported that an A/G splice-site single nucleotide polymorphism (SNP; rs10774671) in the OAS1 gene, encoding 2'5'-oligoadenylate synthetase, was associated with a protective effect against type 1 diabetes in unaffected siblings, and yet affected siblings showed random transmission. Since this finding is difficult to explain biologically, we genotyped the OAS1 SNP in 1,552 type 1 diabetic families from the U.K., U.S., Romania, and Norway and in 4,287 type 1 diabetic cases and 4,735 control subjects from the U.K. We found no evidence of association in either unaffected (relative risk 1.00; P = 0.94) or affected (1.00; P = 0.96) siblings or in the case-control study (odds ratio 0.99; P = 0.83). These results suggest that additional evidence of association of a low penetrance effect in common disease should be sought when the primary result comes from unaffected siblings in the absence of any effect in cases.