Presentation
In case of syndromic presentation, prognosis depends on the nature of the associated anomalies. The documents contained in this web site are presented for information purposes only. [orpha.net]
Pediatric Hand and Upper Limb Surgery guides you to the present indications for intervention and care in upper limb pediatric disorders. [books.google.com]
[…] hemimelia have motion and functional limitations as well. absent ulna severely shortened ulna oligodactyly involving the ulnar side of carpals, metacarpals and phalanges fixed flexion deformity of the elbow joint radiohumeral synostosis may sometimes be present [radiopaedia.org]
Entire Body System
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Gaucher Disease
disease EFFECTS OF ENZYME REPLACEMENT THERAPY Mechanical axis following staple epiphysiodesis for limblength inequality Minimal clinically important differences in ASES and simple shoulder test scores after nonoperative treatment of rotator cuff disease [books.google.com]
Musculoskeletal
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Shoulder Pain
pain study findings recently were published by L Murena and coresearchers Recovery of shoulder strength and proprioception after open surgery for recurrent anterior instability a comparison of two surgical techniques Total elbow arthroplasty for distal [books.google.com]
Workup
[…] in isolation, but frequently associated with other congenital malformations No correlation between severity of deformity and presence of associated syndrome or anomaly All children presenting with radial longitudinal deficiency require additional workup [slideplayer.com]
Laboratory studies Laboratory workup may identify genetic or other abnormalities in patients with dysmorphic features, but are not indicated in the absence of syndromic features, including most CLDs. [now.aapmr.org]
Treatment
Management and treatment Treatment depends on the severity of the condition and the associated skeletal anomalies or syndromes. Passive stretching exercises may allow extension of the wrist and elbow to a normal position. [orpha.net]
Make informed clinical choices for each patient, from diagnosis and treatment selection through post-treatment strategies and management of complications, with new evidence-based criteria throughout. [books.google.com]
Prognosis
Prognosis Patients with ulnar hemimelia may have limited motion, function and strength, but in general, the limb and hand are functional. In case of syndromic presentation, prognosis depends on the nature of the associated anomalies. [orpha.net]
[…] lengthening of shortened digits Breast Reconstruction / implant Chest wall deformity Latissimus dorsi transfer Outcomes: Prognosis generally good, with few functional deficits Potential Complications as related to surgical procedures as noted Recommended [posna.org]
If the patient survives these surgeries, the prognosis is usually good. The orthopaedic abnormalities can be treated individually. The non-surgical management of radial club hand involves corrective casting, bracing and physical therapy. [jcdr.net]
Etiology
Etiology The etiology of ulnar hemimelia remains unknown. The deformity is believed to develop between the 4th and 7th week of gestation. Diagnostic methods Diagnosis is based on clinical examination and X-rays. [orpha.net]
Each chapter has a case presentation, series of clinical questions, and fundamentals on etiology and epidemiology, clinical evaluation, and surgical indications. [books.google.com]
Conclusion: Consistent phenotypic pattern of longitudinal limb anomalies evident in two independent subjects suggest a common underlying genetic etiology. [content.sciendo.com]
Etiology Embryologic insult during upper limb development and antero-posterior limb patterning Theory: insult to the zone of polarizing activity (ZPA) Genetics Unknown, but if associated with radial deficiency there can be a deletion of chromosome 22q11 [posna.org]
Epidemiology
Each chapter has a case presentation, series of clinical questions, and fundamentals on etiology and epidemiology, clinical evaluation, and surgical indications. [books.google.com]
Summary Epidemiology Incidence is estimated at 1/100,000-150,000 live births, with a male to female ratio of 3:2. [orpha.net]
Pathophysiology
Pathophysiology Elliott et al studied 28 patients with posterior congenital dislocation of the radial head and proximal radioulnar fusion to help determine whether they are different clinical manifestations of the same primary developmental abnormality [emedicine.medscape.com]
The exact pathophysiology of amniotic band syndrome remains controversial. [now.aapmr.org]
Anatomy and pathophysiology Absolute stenosis is defined by a canal measuring 10 mm in anterior/posterior dimension, while relative stenosis denotes a 13 mm canal. 1 Both radicular and myelopathic complaints arise when the canal is narrowed to these critical [nature.com]
Prevention
These involve excision of synostosis and interposition of inert tissue to prevent reformation of synostosis, However, synostosis typically recurs inspite of the interposition of silicone, fat, or muscle. [boneandspine.com]
Epidemiology including risk factors and primary prevention Congenital limb deficiencies impact 5 to 9.7 births for every 10,000 live births. [now.aapmr.org]
Treatment is aimed at preventing or correcting PIP hyperextension, restoring DIP extension or both. [flinders.edu.au]
However, some authors have recommended resection of the ulnar-distal radial cartilaginous anlage in an attempt to prevent shortening, bowing, and possible malrotation of the radius. [oandplibrary.org]
Classification 9 10 Initial Non-Surgical Management Serial splinting & stretching Initiated as early as is feasible Lengthen the shortened radial soft tissues Obtain passive correction of wrist deformity Reduce the hand/carpus on the distal ulna & prevent [slideplayer.com]