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Congenital syndactyly: a reappraisal.

Digital syndactyly is a common congenital anomaly and is associated with other anomalies affecting the digits, the hand, the arm or even the entire body. In the past it has been considered a single entity. However, recent information provided by experimental embryology, studies of morphogenesis and other sources, suggests that there are at least two distinct entities: primary syndactyly due to interference with the sequence of events that normally culminate in the division of digits into discrete parts and secondary syndactyly due to readhesion of adjacent digits as a result of close contact between raw surfaces. Primary syndactylyl may arise de novo, or it may occur as a manifestation of a mutation phenomenon or as a genetically controlled syndrome. Secondary syndactyly is the result of mechanical adhesion of adjacent parts involved in a general reparative or healing process. The initial insult leading to amputation of a portion of the digits may or may not be genetically controlled; adhesion is fortuitous. Management of syndactyly is determined by the complexity of the malformation. For simple cutaneous syndactyly the surgeon should make a zigzag incision and provide a rectangular, proximally based flap for the floor of the web, usually before the child is 2 years of age. A full- or split-thickness skin graft should be applied to the defects. For complex deformities involving several digits and associated with postural or osseous malformation, several operative procedures may be required. The surgeon must be vigilant to note and correct the sequelae resulting from recurrent contractures and imbalances associated with growth.

Humans

Syndactyly of upper limb. Morphogenesis, classification, and management.

The distinction between primary and secondary syndactyly is made on the basis of their relation to the morphogenesis. The latter is associated with antecedent malformation which results in subsequent refusion of parts. Based on these considerations, a practical classification is suggested. The management of the various types of malformations is considered in relation to the complexity of the structures involved. Certain deformities require very early treatment in order to permit prehensile function to the developing infant, or to release the impaired part. It is desirable to complete all surgery before the child enters school. The severe complex syndactyly could rarely be solved by one operation. Normal appearance and function are rarely attained and, with growth, newer problems of imbalance and contracture appear. While rehabilitation of the hand is seldom needed for the children with cutaneous syndactyly, the severe deformities associated with complex syndactyly often require supervision in order to regain the potential function and simple dynamic splints are very helpful.

Contracture

Syndactyly--a review and long term results.

Syndactyly is classified and the principles of its surgical treatment discussed. The notes of eighty-five patients who had 222 webs between them were reviewed. A long term follow-up was carried out on thirty-two of these patients who had seventy-six clefts separated. The sexual and anatomical distribution of the syndactyly was investigated. The results of surgery were assessed including complications, and the relationship of complications to the type of graft used and the age at operation. It is suggested that complicated syndactyly is often separated at too early an age.

Age Factors

Evaluation of the operative treatment of syndactyly.

A review of the records of 61 patients with 176 surgically treated webs was completed to evaluate the results of the different operative techniques used to separate the fingers. The postoperative follow-up period averaged 14 years, with a range from 2 to 38 years. The patients were assessed in two groups: those with major associated anomalies and those in whom syndactyly was the principal anomaly. Recurrence of the webs and flexion and extension contractures occurred more often when split-thickness grafts were used. To obtain a satisfactory result, a second procedure was necessary in 59% of patients with major associated anomalies, and in 30% of the patients who had syndactyly as the principal abnormality. Two types of patients were found to require subsequent operations: children treated prior to 18 months of age and those with complex syndactyly.

Abnormalities, Multiple

Syndactyly with Larsen's syndrome.

The association of syndactyly with Larsen's syndrome is reported. A revised listing of syndromes which may be associated with syndactyly is presented.

Abnormalities, Multiple

A family with syndactyly type II (synpolydactyly).

Syndactyly Type II is reported in eight members of a family in four generations. Affected individuals show two distinctive patterns of variation in the expression of the gene. Distortion of dermatoglyphic patterns is associated with the severe but not the mild manifestation of the malformation. The diagnostic significance of minimal features of the condition is discussed. Linkage data suggest that loci for Syndactyly II and for blood-group antigens ABO, MNSs, P, Rh and Kell are not closely linked.

Adult

Syndactyly and split hand. Supplement.

In a series of studies of clinical and radiological features of hand anomalies, the author has recently encountered cases which may be explained by reference to the relationship between syndactyly and split hand. These cases suggested how the typical split hand is formed prenatally, and it is concluded that the typical split hand should not be classed as "Arrest of Development of Parts" due to embryonal failures.

Fingers

Lateral-volar finger flap for the treatment of burn syndactyly.

To completely open the web space in burn syndactyly and provide tissue that is pliable and elastic, we describe a flap from the lateral and volar surfaces of an adjacent finger which will completely cover the opened defect. The results of this procedure in 24 web spaces are reported.

Adolescent

An operation for syndactyly, and its results.

We describe our method of operation for syndactyly repair and our results in 62 patients (74 hands, 143 commissures), all treated more than two years ago. The operation was performed on patients aged from 17 months to two years (except those with acrosyndactyly). Postoperative web formation was not seen in any of these patients.

Acrocephalosyndactylia

Cryptophthalmos, dyscephaly, syndactyly and renal aplasia. Report of a case.

A danish girl with incomplete, bilateral cryptophalmos together with assoicated craniofacial malformations, laryngeal hypoplasia, syndactyly, unilateral renal aplasia and slight external genital abnormalities is reported. Chromosomal abnormalities or metabolic disorders were not demonstrated. As the condition is extremely rare, some clinical and pathological fingings previosuly described in analogous cases are mentioned, together with a few pathogenetic mechanisms. Finally, a discussion regarding the aetiology of the condition is presented. Most authors are convinced that the malformative pattern is a syndrome with an autosomally recessive genetic basis. Added envronmental influence explains the wide range of manifestations and the varying gene expressivity.

Abnormalities, Multiple

Problem of sex ratio in cases of type I syndactyly.

Fifty pedigrees of type I syndactyly were analysed for sex ratio and segreation pattern. Thirty-four of the pedigrees were from the published reports; 16 were collected in the State of Utah. Pedigrees with affected individuals showing webbing between the second and third toes are characterized by a sex ratio of affected individuals favouring males and a highly significant excess of affected sons of heterozygous fathers. A similar distorted segregation pattern is present in those pedigrees when the webbing involves the second and third toes and/or the third and fourth fingers, but not in those pedigrees when the webbing involves other digits. The reason for the distorted segregation pattern is unknown. Hypothesis include abnormal chromosome segregation and gametic selection.

Female

Surgical treatment of simple syndactyly.

During the period 1963--74 a total of 20 operations for simple syndactyly were performed on 34 fingers. A dorsal rectangular flap and thick split skin grafts were regularly used except in 2 cases where full thickness grafts were used for covering the skin defects. There were no secondary operations. The period of observation after 13 operations was 4 years or more. It is concluded that the dorsal rectangular flap technique combined with thick split skin grafts give satisfactory results.

Child

Oligodactyly and multiple synostoses of the extremities: two cases in sibs. A variant of Cenani-Lenz syndactyly.

Two sibs with a rare phenotype of oligodactyly with metacarpal, carpal, radioulnar, and metatarsal synostoses and shortening of the forearms were born of healthy parents. Genetic counselling with regard to future children of one of these sibs was given on the assumption of an autosomal recessive inheritance, which is highly probable, judging from the other rare familial cases found in the literature.

Adult

Syndactyly and split hand.

Careful analysis of clinical and radiological features of both webbing of normal fingers and typical split hand, suggests that these two types of malformations should not be classified separately. Study of human embryos and experimental malformations of the apical ectodermal ridge, supplies a resonable explanation for the close relation between webbing of normal fingers and typical split hand.

Female