Question

Madelung's deformity - some sort of bone disorder is this treatable at the age of 27 (female. My sister have it, her one of the bone in hands is abnormal xtra growth may be if not treatable in india thn which country which hospital is the best.

Dr.Julie Mercy J David · Accepted Answer

Madelung's deformity is usually characterized by malformed wrists and wrist bones, accompanied by short stature and is often associated with Léri-Weill dyschondrosteosis. It has only been recognized within the past hundred years. Causes of Madelung Deformity
There are four types of Madelung deformity
Post traumatic
Following trauma that disrupts growth of the distal radial ulnar-volar physis.
Dysplastic
Associated with bone dysplasias like multiple hereditary osteochondromatosis, Ollier disease, achondroplasia, multiple epiphysial dysplasias, and the mucopolysaccharidoses. This type can also be seen secondary to sickle-cell disease, infection, tumor, and rickets.
The most important dysplasia associated with Madelung deformity, however, is Leri-Weill dyschondrosteosis.
Genetic
About 30% of cases of Madelung deformity are transmitted in an autosomal dominant fashion though there us a variable expression and 50% penetrance.
Madelung deformity is bilateral in about 50% of the cases.
Most common chromosomal association is with Turner syndrome where mutation has been found in short stature homeobox-containing gene, SHOX, present on X chromosome. But families with this mutation and individuals with Turner syndrome and families with a history of MD have been shown to exhibit a variable expression of MD and dyschondrosteosis. This raises a possibility of a modifier gene on another area of the X chromosome or on an autosomal gene may be involved.
Idiopathic
Where no cause or association can be found.
The exact nature of the pathologic process that causes the disturbance in the growth of the distal radial physis is unknown.
When Madelung deformity is a hereditary disorder, it is transmitted as an autosomal dominant trait with incomplete penetrance.
Sporadic forms do occur.
It is more common in the females and involvement is frequently bilateral. Treatment of Madelung Deformity
Surgery is indicated for relief of pain and cosmetic improvement. Function is usually only minimally improved.
Nonoperative management may be helpful in skeletally mature individuals with mild-to-moderate short-term wrist pain due to distal radioulnar joint or radiocarpal joint.
The treatment involves splintage and decrease in activity levels.
The pain that is caused by the tension within the Vickers ligament does not improve with these measures and may need Release of the ligament alone or in combination with an osteotomy
When patient has attained skeletal maturity, the treatments to be considered are osteotomy and aadioscaphocapitate arthrodesis if joint congruency can be achieved.
Darrach or Sauve-Kapandji procedure if distal radioulnar joint congruency cannot be achieved.
No specific contraindications to surgery exist other than those associated with any elective surgical procedure.
The decision of surgery is guided by patients age and remaining growth potential, severity of deformity and symptoms along with radiographic findings
Surgical procedures either correct the primary deformity of the radius or make compensatory change in the ulna or both. The goal of ulnar procedures is to change the relationship of the relatively long ulna to the radius. Vickers physiolysis
When the deformity is noticed early and significant growth remains, changing the growth pattern of the distal radial physis to correct the deformity is possible. The Vickers ligament originates on the radius in a fossa, is 5-7 mm thick and inserts into the anterior surface of the lunate and the anterior radioulnar ligament portion of the triangular fibrocartilage complex. This ligament could be a cause of pain in Madelung deformity.
It is said that the lesion is both bony and ligamentous and physiolysis [resecting the bony and ligamentous lesion] allows for a normal and compensatory growth to correct the deformity.
Osteotomy of radius
This is considered in older child when remaining growth is insufficient. The osteotomy can correct the position of the distal radiocarpal joint surface and brings the radius and ulna into a more proper position. It could be closing or opening wedge osteotomy. An ulna-shortening procedure can be performed along where required.
Ulnar Procedures
Because the radius is volar, the ulna appears to be subluxated dorsally and the incongruence at the distal radioulnar joint and impingement of the radius on the ulna in supination could lead to pain and stiffness.
Following ulnar procedures have been described to allow rotation
Ulnar shortening
Darrach Procedure
Sauve-Kapandji procedure
Fusion of distal radioulnar joint
Arthrodesis
This is considered in the skeletally mature patient who presents with pain and in whom physical examination demonstrates limitation of motion and severe radiocarpal joint incongruity
Prognosis of Madelung Deformity
Pain relief and correction of the cosmetic deformity are main goals of treatment and most of patients attain both of them. Range of motion usually is only moderately improved at best.

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