Cenani-Lenz type of syndactyly: a complex type of syndactyly with multiple synostoses.
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Child, Preschool↗
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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 type IV/hexadactyly of feet associated with unilateral absence of the tibia.
We report on a newborn girl with syndactyly type IV, hexadactyly of feet, and right tibial hemimelia. She has 5 other relatives with identical anomalies of the hands and feet transmitted as an autosomal dominant trait. Syndactyly type IV is rare as is absence of the tibia. We suggest the possibility that syndactyly type IV may be a more complex entity, including lower limb malformations, and transmitted as autosomal dominant with variable expressivity.
Abnormalities, Multiple↗
Cleft hand, syndactyly and hypoplastic thumb.
The clinical features of 58 patients with typical cleft hand were examined and compared with 86 patients with syndactyly between the long and ring fingers, 27 patients with syndactyly between the ring, little and other fingers, 53 patients with hypoplastic thumb and 100 patients with symbrachydactyly. The clinical findings of the typical cleft hand resembled those of syndactyly. There were two unusual cases of typical cleft hand associated with hypoplastic thumb, congenital heart anomalies and absence of the axial triradius. One of these also had cleft lip and palate. The critical embryonic period of the heart anomaly is early, while that of the cleft lip and palate is late. These findings suggest that an embryo with typical cleft hand and hypoplastic thumb results from impairments at two different times in the early embryonic period.
Abnormalities, Multiple↗
The pulp plasty: a composite graft for complete syndactyly fingertip separations.
Composite grafts of skin and subcutaneous fat harvested from the glabrous non-weight-bearing areas of the foot were used to graft 34 fingertips after separation of 23 complete syndactyly webs in 13 patients. Simple complete syndactylies accounted for 17% and complex complete syndactylies accounted for 83%; synonychia was encountered in 70%. Follow-up averaged 13.9 months (minimum, 2 months, maximum 33 months). All patients had 100% take of the composite graft. Subjective gradings were 94% good, 6% fair, and there were no poor results. This technique provides a relatively normal contour and satisfactory pad to the fingertip.
Adipose Tissue↗
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 correction of the hand in Apert syndrome.
Surgical correction of syndactyly of the Apert hand should begin by 6 months and be completed by 3 years of age. As much surgery as possible is carried out at each sitting. Digit separation should be in order of functional importance. The first web space is deepened with a four-flap Z-plasty or a dorsal skin flap from the web and index finger. Syndactyly release using a dorsal flap and zig-zag technique is used to create the second and fourth web spaces. The complex long-ring syndactyly often requires a pedicled groin flap for reconstruction and preservation of growth potential. A five-digit hand can be achieved with adequate grasp and stable, sensate, well-aligned digits. These children can attain some degree of independent finger motion and aesthetically acceptable hands with this approach.
Acrocephalosyndactylia↗
Partial unilateral cryptophthalmos with syndactyly, brachycephaly, and renal anomalies.
The cryptophthalmos syndrome consists of cryptophthalmos, dyscephaly, syndactyly, and genitourinary abnormalities. A 2-year-old girl had unilateral partial cryptophthalmos associated with brachycephaly, unilateral syndactyly, and unilateral poor renal visualization.
Abnormalities, Drug-Induced↗
Syndactyly: can web creep be avoided?
Syndactyly is one of the most common congenital hand deformities but there still remains a high incidence of contractures and web creep after attempts at surgical correction using many assorted techniques. Little attention has been paid to the potential junctional scar in the aetiology of web creep. To remedy this, a technique is described which involves a dorsal flap and two palmar laterally-based flaps. This method not only breaks up the palmar junctional scar but also completely reconstructs the web, not just the floor. The procedure can be used in all varieties of syndactyly and has reduced the incidence of creep in a series of 49 webs.
Adolescent↗
Teratogenic relationship between polydactyly, syndactyly and cleft hand.
Several investigators have suggested that polydactyly, syndactyly and cleft hand might have arisen from a common teratogenic mechanism. To confirm this hypothesis, 75 hands with these anomalies were analysed. Advanced cases with central polydactyly or osseous syndactyly in which the fusion area extends as far as the proximal phalanx and metacarpus are identical to typical cleft hand. The author has induced the same deformities using myleran in rat foetuses. The clinical features of these anomalies in rats were the same as those in clinical cases and the critical periods of these anomalies were also the same. The findings suggest that these hand anomalies may appear in human beings when the same teratogenic factor acts on the embryo at the same developmental period and that they should belong to the same teratogenic entity.
Animals↗
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↗
Oculodentodigital dysplasia and type III syndactyly: separate genetic entities or disease spectrum?
A family is described with type III syndactyly and facies resembling the oculodentodigital dysplasia facial phenotype in the absence of any of the other characteristic findings of the latter condition. The relationship between type III syndactyly and oculodentodigital dysplasia is discussed.
Adult↗
Type II syndactyly or synpolydactyly.
A family with syndactyly type II or synpolydactyly is described. The autosomal dominant inheritance is confirmed by this pedigree. In combination of this anomaly a brachymesophalangia of the fifth finger was inherited by most family members. The duplicated phalanx was resected and the syndactyly separated in the proband with excellent functional and cosmetic results.
Abnormalities, Multiple↗
[Dermatoglyphic changes in some human hereditary disorders: syndactyly].
The dermatoglyphic hand prints from 19 patients with different types of syndactyly were analysed. It was shown that some digital triradii and palmar lines were missing and replaced by only one triradius with common radiants and one main palmar line in patients with syndactyly. With fingers fused incompletely so called zygodactylous triradius and the main Z line may appear, instead of or alongside with them. It is proposed that the position of local cell death in the interdigital spaces is determined by positional information which is expressed in the system of polar coordinates.
Dermatoglyphics↗
[Rehabilitation measures and possibilities after surgical treatment of syndactyly].
Physical therapy plays an important role in the postoperative treatment of syndactylies. However, the ultimate outcome depends on the degree of deformity. During childhood a physiotherapeutic program with equipment adapted to the young patients should be offered. 46 patients with syndactyly will be described.
Child↗