Impaired sexual and reproductive outcomes in women with classical forms of congenital adrenal hyperplasia.

OBJECTIVES: The objectives of the study were 2-fold: 1) a detailed description of sexual and reproductive outcomes in adult women with congenital adrenal hyperplasia (CAH) of different phenotypic severity at birth; and 2) comparisons of these outcomes among CAH subtypes and between CAH women and non-CAH control women. DESIGN: This was a cross-sectional study using a face-to-face interview, a written questionnaire, the Female Sexual Function Index, and a gynecological examination. PATIENTS: Patients included 35 women with CAH, representing Prader stages I-V at birth, aged 18-43 yr, who had been treated from birth to adolescence in the same pediatric endocrine clinics. Sixty-nine non-CAH healthy control women were selected from hospital-staff families. RESULTS: None of the CAH women expressed doubts about their gender assignment. Twenty percent (seven of 35) had homosexual inclinations; 23% (eight of 35) were married; three reported a complete lack of sexual activity; and 37% (13 of 35) said they never had heterosexual intercourse with vaginal penetration. Sexual functioning as assessed by the Female Sexual Function Index was much lower in CAH women than controls and lowest in CAH women with high Prader stages. Eighty-one percent (18 of 22) experienced pain during vaginal penetration. Only eight women became pregnant, and 17% (six of 35) had children. CONCLUSIONS: Despite expert medical and surgical care by physicians dedicated to this rare disease, women with CAH still suffer major limitations in their sexual function and reproductive life.

Treatment of Madelung's deformity by lengthening and reaxation of the distal extremity of the radius by Ilizarov's technique.

Madelung's deformity was first described in 1878. It is characterised by a typical deformity of the carpus and not only causes pain but also impedes mobility and aesthetic appearance. Surgical correction can be effected during adolescence, the most frequently employed technique being conical osteotomy. We present a novel technique of lengthening and aligning the distal radial extremity using Ilizarov's technique. Five carpal joints were operated on in three 13-year old girls. An aesthetic effect was obvious in all the cases. Mobility improved by 30 degrees in the direction of the extension and pain always subsided directly after surgery.

[Arthroscopic treatment of chronic anterior instability of the shoulder by staple capsulorrhaphy. Apropos of a series of 55 patients with a minimum of 18 months follow-up]. / Traitement arthroscopique de l'instabilité antérieure chronique de l'épaule par capsulorrhaphie avec agrafage. A propos d'une série continue de 55 cas ayant un recul supérieur à 18 mois.

PURPOSE OF THE STUDY: The authors studied the results of the arthroscopic staple capsulorrhaphy of 55 patients who had recurrent anterior shoulder instability. MATERIALS AND METHODS: There were 38 men and 17 women. The average age at operation was 30.3 years (17 to 68) and the dominant side was injured in 33 patients. 28 (51 per cent) patients had recurrent dislocations, 19 (38 per cent) patients had recurrent subluxations and 8 (14 per cent) complained of a painful shoulder with instability. Multidirectionnal hyperlaxity and glenoid rim fracture cases were excluded from this study. The average duration of symptoms was 43 months (i to 180). At operation, 46 patients had a Bankart lesion (Adolfsson A or C) and 9 had "non Bankart" lesion (Adolfsson B and intra ligamentous disruption). There were 6 (11 per cent) SLAP II lesions and 23 (42 per cent) other glenoid labral tears associated with main instability lesions. 58 staples were inserted. 53 inferior glenoid humeral ligament were fixed to the glenoid rim and in 2 cases we performed a subscapularis tendon tenodesis. RESULTS: The follow-up was continued for at least 18 months after treatment by an examiner different from the operating surgeon. (Average follow-up was 29.8 months). The results were assessed according to "Duplay" rating scale. Overall we have obtained 64 per cent excellent and good results. 71 per cent of shoulders were considered stable at revision whereas 7 per cent showed recurrent dislocation. 60 per cent of patients were able to return to their previous sport level. A limited range of motion was noted in only 11 per cent of cases. On the other hand 54 per cent of patients presented persistent pain. With regard to the shoulder stability, the factors possibly having a negative influence were the occurrence of an initial acute dislocation, the destruction of the inferior glenohumeral ligament (disruption or absence) and the sub-equatorial position of the staple on the anterior glenoid rim. Pain was more frequent in cases where there was associated subacromial impingement and where the staples had been badly positioned. DISCUSSION: We have compared our results with those of other authors who also performed stapling procedures, including different arthroscopic techniques and results of open stabilization surgery. Our results regarding shoulder instability were better than those obtained by arthroscopic sutures, equivalent to those obtained by the "Open Bankart" procedure, but less impressing than those obtained by the "Bone Block" procedure (Patte). However, pain was observed much more frequently than with all the other stabilization techniques, arthroscopic or not. CONCLUSION: Arthroscopic stapling therefore seemed to be less reliable than the "Patte Bone Block" procedure. At present, we reserve arthroscopic stabilization for patients with a good inferior glenohumeral ligament. Until an adapted biodegradable staple is perfected, we still use an anchorsuture technique to avoid pain due to metallic implant.

[Comparative study of fixation mode in total knee arthroplasty with preservation of the posterior cruciate ligament]. / Etude comparative des modes de fixation d'une arthroplastie du genou conservant le ligament croisé postérieur.

PURPOSE OF THE STUDY: We performed a prospective randomized study to compare two fixation modes, with and without cement, for total knee arthroplasty. MATERIAL AND METHODS: The study series was composed of 96 cemented or noncemented total knee arthroplasties performed between May 1993 and October 1995. The only difference was the diamond interface used for cemented prostheses and the mesh interface used for uncemented prostheses. The operator was unaware of the type of fixation until the bone slices had been obtained. We assessed outcome in 73 cases with a mean follow-up of 27 months. The two populations were comparable for preoperative clinical status, bone tophicity and surgical procedure. RESULTS: The mean duration of the operation was sgnificantly longer (> 10 min) for the cemented protheses. The complication rates were comparable but we did have two mobilizations of the tibial implant in the noncemented group. The total scores (127 +/- 29 in the cemented group versus 135 +/- 20 in the uncemented group) were significantly different. There were however more cases with degradation of the controlateral knee in the cemented group although the difference was not significant. When these cases were excluded from the analysis, the total scores for two groups were similar (143 and 140 respectively). Radiographic outcome was quite different with mobilization of the tibial implant in 2 cases and the rate of lucent borders was significantly higher in the noncemented group. DISCUSSION AND CONCLUSION: While the clinical outcome was comparable, the quality of the fixation was significantly better with cemented arthroplasty, which remains the gold standard.