Facioscapulohumeral muscular dystrophy (FSHD) is a common hereditary disorder which typically results in scapular winging due to wasting of the periscapular muscles affected by this condition. Scapulothoracic arthrodesis (STA) is the current surgical treatment for FSHD patients with severe winging and preserved deltoid muscle. There are several different techniques in the literature such as multifilament cables alone and cable or cerclage wires combined with single or multiple plates. We prefer cables without plates as it provides independent strong fixation points and strongly recommend utilization of autograft. The functional results of studies report that regardless of the technique used, shoulder elevation and thus quality of life is improved, as shown with outcome scores. There are several complications associated with STA. Pulmonary complications are common and usually resolve spontaneously. Meticulous surgical technique and effective postoperative analgesia may reduce the incidence. Scapular complications which are associated with the fixation may be encountered in the early or late period, which are related to the learning curve of the surgeon. In conclusion, STA is a reliable solution to a major problem in FSHD patients that helps them maintain their activities of daily living until a cure for the disease is found. A successful result is strongly dependent on patient selection, and a multidisciplinary team of neurologists, geneticists and orthopaedic surgeons is required to achieve good results.

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Facioscapulohumeral dystrophy (FSHD) typically affects the periscapular muscles, resulting in scapular winging. Scapulothoracic arthrodesis (STA) stabilizes the scapula to provide better movement for these patients. Analgesia regimen for FSHD patients who received a single-shot erector spinae plane block (ESPB) and a catheter at the area were retrospectively analyzed in this study. Patients were asked to rate their pain postoperatively and only 5 of 10 patients needed rescue analgesic. No complications occurred. Our experience suggests that continuous ESPB may be helpful for providing analgesia in FSHD patients undergoing STA.

Objective: Autosomal Dominant Polycystic Kidney Disease (ADPKD) is the most common hereditary kidney disease and nephrolithiasis is frequent among ADPKD

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BACKGROUND: This study aims to describe the functional outcome of true and equivalent Monteggia fracture-dislocations in the pediatric population. We also provided a review of the literature about the treatment options. METHODS: Five surgically and three conservatively treated patients were identified who were treated in 2009-2021. The study population consisted of six female and two male patients. The mean age at the time of treatment was 7. The mean follow-up time was 55 months (range, 12–128). The Mayo Elbow Performance Score and the Oxford Elbow Score were used for outcome evaluation. Range of motion and grip strengths were also evaluated. RESULTS: There were two Bado type 1 and six Monteggia equivalent injuries. Closed reduction and casting were utilized for the two Bado type 1 injuries as the initial treatment. However, one had a radial head re-dislocation and had to be treated operatively. This patient had a radial head re-dislocation after the surgery and was followed up conservatively. Three Monteggia equivalent injuries were treated with closed reduction and casting, with no complications. One patient had a radial head anterior dislocation with plastic deformation of the ulna, and this patient was managed with CORA-based corrective ulnar osteotomy. For Monteggia injuries, the main treatment objective is to restore the ulnar length. Bilateral computed tomography imaging with 3D reconstruction can be utilized in preoperative planning of Monteggia fracture-dislocations to customize the treatment. Close observation is essential to detect radial head subluxation, which needs early intervention before irreversible changes occur. CONCLUSION: The true/equivalent Monteggia fractures’ main treatment goal is to restore the ulnar length. Conservative treatment, with a close follow-up, is the first option if closed reduction can be achieved. If closed reduction is not possible, careful preoperative planning and early rehabilitation are key to success for management of Monteggia fractures.

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