The Madelung deformity of the wrist is due to partial closure of the palmar and Tratamento cirurgico da deformidade de Madelung pela osteotomia de radio e. Sprengel’s deformity is a rare congenital skeletal abnormality where a person has one shoulder Sprengel’s deformity; Wallis–Zieff–Goldblatt syndrome · hand deformity: Madelung’s deformity · Clinodactyly · Oligodactyly · Polydactyly. A deformidade de Madelung apresenta amplo espectro de apresentação clínica, que pode variar dentro de uma mesma família com penetrância de 50%, sendo.

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Congenital deformities of the upper limbs. Overgrowth; Undergrowth; Streeter and others. This article, presented in three sections, review the most commons upper limb malformations and theirs treatments. In this section three there’s a discussion about overgrowth; undergrowth; Streeter Syndrome and other malformations.

The bibliography is continuous since section one. This third and last article of this series, which had the objective of discussing the principles of treatments for congenital abnormalities of the upper limbs, covers the syndromes of overgrowth, undergrowth, Streeter and others that were not classified in the previous parts of this article.

This is exemplified by macrodactyly, which although rare still presents treatment challenges.

There is an accumulation of fatty tissue in the limb or finger affected. It is divided into a static type, when the overgrowth is proportional to the child’s growth, and a progressive type, when this overgrowth is disproportional to the child’s growth. The abnormalities affect tendons, intrinsic tissues, joints and phalanges. There are abnormalities in the peripheral nerves. There is no excessive fatty infiltration.

It generally affects one hemithorax or half of the body, and only rarely one finger in isolation. Maffucci syndrome and Ollier disease: Because of the phenotypic differences between the various types madelkng macrodactyly, there is no defined treatment algorithm. The treatment principles are that an esthetically acceptable hand with pincer capacity for gripping objects should be provided.

Several techniques that mdelung osteotomy, epiphysiodesis, soft-tissue reduction especially of fatty tissuetendon shortening and neurectomy have been used with the aims of impeding overgrowth and reconstructing the appearance of the finger.

Amputation of the radius Fig. Resection of 3rd radius. Preoperative, intraoperative and final result. Microsurgical transposition of a toe to the hand should be considered in cases of severe macrodactyly of the thumb or at the level of the metacarpus.

The term brachydactyly means “short fingers”. In cases in which brachydactyly is associated with syndactyly union of two or more fingersthe term used is brachysyndactyly. D that involve a phalange or metacarpal rarely need treatment. However, from an esthetic point of view, stretching can be performed with or without interposition of a bone graft, especially for the metacarpal. Blauth and Gekeler classified cases of brachysyndactyly as follows: In many cases, there is no indication for surgical treatment because of good functioning.

Stretching of the metacarpals: Transfer of phalange from the non-vascularized foot: Greatly used, with several reports in the literature, but the results have been variable and inconsistent. Microsurgical transfer of toe: This is defined madelng intrauterine strangulation of parts of the fetus by the amniotic membrane, which causes deformities or even intrauterine amputation. The latter is the more accepted theory.


Clinically, it is manifested by distal constrictions in the fingers, syndactyly, acrosyndactyly with fenestrationshypoplastic fingers and amputations. The central fingers are the ones most affected. The thumb is affected in only Some of the classifications that have been described are of little help in choosing the treatment.

The classification most used is the one by Patterson: Presence of simple constriction bands. Initially, Streeter syndrome may require emergency treatment, in cases of distal vascular distress at the constriction band, even if only a few hours or days after birth.

Deformidade de madelung pdf

Resection of the fibrous band Fig. Resection of the constriction bands can be done as a single surgical procedure Fig.

This avoids distal congestion, which in more severe cases may lead to tissue necrosis. In cases of complete syndactyly, the commissure needs to be reconstructed. Toe transfers, pollicization, finger stretching and “on-top plasty” need to be analyzed for each case, in amputations in which there may be a functional deficit.

Madelung deformity is defined as a growth deficit of the anteromedial portion of maelung growth plate of the distal third of the radius. In ddformidade, there is shortening and curvature of the radius, widening of the distal radioulnar joint, dorsal subluxation of the ulna and triangular deformity of the carpus.

The deformities are not noted until adolescence. They are generally bilateral and women are more affected than men. Its etiology remains uncertain, but vascular deficits and anomalous ligaments Vickers have been described as the cause of the deformity. No treatment is necessary for asymptomatic patients. For patients who complain of pain, orthoses and rehabilitation are often enough. In cases of untreatable pain and limitations of range of motion that cause difficulties in activities of daily living, surgery is indicated.

In the great majority madelubg cases, the surgical indication comes from a static complaint. Several techniques have been described: Arthrogryposis is a group of deformidadw syndromes that affect madelunv and are characterized by congenital joint contractures in two or more joints. Between and syndromes have been described. The term amyoplasia describes classical arthrogryposis through correlating the joint contractures with the absence of intrauterine muscle function.

The causes are multifactorial and include myopathy, neuropathy, connective tissue abnormalities, diminished intrauterine space, etc. A distal form of arthrogryposis has been described, in which only the hands and feet are affected, thus sparing the major joints.

Distal arthrogryposis responds well to conservative treatment consisting of deformudade and serial orthoses, started soon after birth. Amyoplasia, which is the commonest form and is taken to be the classical form of arthrogryposis, has sporadic incidence and is characterized by symmetrical involvement of the joints, limbs of tubular appearance, absence of flexor skinfolds and normal madeoung.

The fingers are flexed and rigid and the thumb is adducted. The upper limb should be dealt with as a functional unit, in the sense of promoting independence. The treatment priorities are to promote the capacity to communicate, perform activities of daily living, have mobility and be able to walk.


There are some authors who have recommended surgical interventions starting at the age maelung three months. Rotational osteotomy of the humerus may be indicated in some cases. The treatment for the wrist can be done using the following:. Proximal carpectomy, when performed before ossification of the carpal bones, preserves the range of motion of the wrist through bone remodeling.

In older children, recurrence is common. It is done on the mediocarpal joint, in association with stretching of the flexor madeljng.

Regarding the fingers, cases of an adducted thumb should be treated with reconstruction of the first commissure, stretching of the long flexor of the thumb and tenotomy of the adductor and first dorsal interosseous muscle, possibly followed by opponensplasty Fig. Metacarpal-phalangeal arthrodesis of the thumb may be necessary in maturity.

Regarding the elbow, absence of active flexion is a major problem. The first step is to differentiate the cases with presence of passive flexion from those with a rigid elbow. Active flexion is a major challenge.

Deformidade de madelung pdf

The literature presents conflicting results relating to biceps-triceps transfer, pectoralis major-biceps transfer or latissimus dorsi-biceps transfer. Regarding transfer of the triceps to the biceps, the discussion centers on the fact that in the great majority of cases, loss of the triceps promotes deformity in flexion, i. Elbow rigidity and the need to deformmidade the triceps for walking crutches and wheelchair are contraindications for triceps-biceps transfer.

Authors who have advocated these techniques have believed that positioning one limb in flexion and the other in extension would improve performance in activities of daily living. The risk is that wrist and finger flexion is worsened.

Madelung deformity | Radiology Case |

Limbs relaxed A ; attempt to raise the non-operated limb B and elevation of operated limb C. Clearly, there is still no consensus regarding treatments for elbows presenting arthrogryposis, and each case should be assessed and discussed exhaustively with the parents. Deformities of the hand and the fingers. Green’s operative hand surgery. Samson P, Mevio G. Kawamura K, Chung KC. Part II arthrogryposis, camptodactyly, clinodactyly, madelung deformity, trigger finger, and trigger thumb.

Sprengel’s deformity

J Hand Surg Am. James MA, Bednar M. Deformities of the wrist and forearm. Elsevier Churchill Livingstone; Arthrogryposis multiplex congenital amyoplasia: Lippincott Williams Williams; Posterior elbow capsulotomy with triceps lengthening for treatment of elbow extension contracture in children with arthrogryposis.

J Bone Joint Surg Am. Lahoti O, Bell MJ. Transfer of pectoralis major in arthrogryposis to restore elbow flexion: J Bone Joint Surg Br.

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