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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↗

Island flap reconstruction of the web space in congenital incomplete syndactyly.

A number of techniques have been described for the correction of incomplete syndactyly, some of which may produce obvious dorsal scarring of the fingers or hand while others rely on the use of skin grafts. Many of the methods require complex planning. We present our experience of a new technique which simplifies the operative planning, allows a natural looking web space to be reconstructed with minimal dorsal scarring and should minimize the need for skin grafts. Seven patients (nine webs) who underwent correction of incomplete syndactyly were reviewed (follow-up range, 6-32 months). Only one patient early on in the series required a small skin graft to cover a residual defect, following which modifications to the flap design were made. All the web spaces healed without complication and at review there were good functional and aesthetic results.

Adolescent↗

Genetic analysis of syndactyly in German Holstein cattle.

Congenital syndactyly with a variable number of affected feet was observed in eight black and white German Holstein calves. Analysis of the pedigree data revealed that all affected individuals could be traced back to a single founder. The pedigree was consistent with monogenic autosomal recessive inheritance and variable expressivity. Bovine syndactyly or "mulefoot" has been previously shown to map on the telomeric end of bovine chromosome 15 and we performed PCR genotyping of microsatellite markers spanning 27 cM of this chromosomal region to test the new cases for genetic linkage with the phenotype. The haplotype segregation confirmed the suggested inheritance pattern of the mulefoot mutation in this family and markers RM004, BM848 and BMS820 showed significant linkage to the phenotype. The results confirmed the chromosomal location of the mulefoot gene in this pedigree. Furthermore the study demonstrated that although marker testing has been available for nearly a decade the use of mulefoot carriers in cattle breeding remains uncontrolled. The presented family provides a resource for positional cloning of the causative mutation.

Animals↗

Repair of complete syndactyly by tissue expansion and composite grafts.

Repair of complete syndactyly by a combination of tissue expansion and composite grafts from the glabrous non-weight bearing areas of the foot has been performed on three syndactylies in two patients. The commissure and the lateral areas of the proximal and middle phalanges were covered with expanded skin and the separated fingertips were covered with composite grafts. Without using an ordinary skin graft, this method can provide aesthetically excellent results with good skin colour and texture.

Female↗

Long-term results of syndactyly correction: full-thickness versus split-thickness skin grafts.

In order to compare the long-term results of full-thickness and split-thickness skin grafts after the correction of congenital syndactyly, 27 patients have been investigated after an average follow-up of 21 years. Post-operative functional and cosmetic results have been assessed by patient records, questionnaires and physical examination. The webs that had received split-thickness grafts showed more flexion and extension lags and the overall spreading of the operated fingers was significantly decreased compared to the control fingers. On the other hand, more re-operations because of web creep had to be performed after full-thickness grafts. Hyperpigmentation and hair growth in the grafts was found in most of the full-thickness grafts, while breakdown of the graft was found in some of the split-thickness grafts. Therefore, based on the results of this study, either full- or split-thickness skin grafts can be used when treating of congenital syndactyly.

Adult↗

Intra-uterine and juxtanatal repair of syndactyly in foetal mice.

39 foetal mice with genetic syndactyly were identified in utero at 17 days of gestation, and the right hindfoot extruded through the uterus. The syndactylous digits were separated by simple incisions. In one group (n = 25) digit separation was maintained during wound healing by the interdigital application of a silver microclip. Digit separation was also assessed in a second group of newborn mice less than 24 hours old (juxtanatal population, n = 24). Two foetuses (5%) and six newborns (25%) developed digital necrosis following microclip application. In the remaining microclipped animals (23 intrauterine and 10 juxtanatal), microclip application maintained digit separation, allowing wound healing to occur with epithelialization of the separated digits. No inflammation or scar formation occurred. In the third group (n = 22) without microclip application, the digital skin reapproximated and webbing recurred during wound healing. These studies demonstrate the need to maintain digit separation during wound healing following intra-uterine or juxtanatal syndactyly repair.

Animals↗

Correction of syndactyly using a dorsal omega flap and two lateral and volar flaps. A long-term review.

The long-term results of a technique for correction of syndactyly are reported. The technique consists of a dorsal omega flap and a palmar anchor forming two palmar and lateral flaps. A long-term review was made of 50 patients with a minimum of 8 years follow-up operated over a period of 10 years. A total of 122 web spaces in simple, complex and syndromic syndactyly were operated on. Most patients achieved satisfactory reconstruction of the web spaces, resulting in a web of good shape. At long-term review, web creep was recorded in eight webs, and skin contractures in three fingers. This study shows the technique to be effective in reconstructing web spaces and in minimizing the prevalence of complications.

Age Factors↗

Syndactyly correction without skin-grafting.

Syndactyly correction results in skin deficiency. Skin grafting is avoided by the use of an extended dorsal interdigital flap for the web and approximation of the side flaps to cover the length of the digits. The technique has been used in 17 cases with all varieties of syndactyly. There were no early complications. The follow-up averaged 2 years. There were two cases of web creep but no contractures. The method combines the advantage of direct suture with an acceptable frequency of web creep.

Child↗

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↗

Symphalangism associated with constriction rings: syndactyly and brachytelophalangy in a black patient.

A 7-year-old girl presented with symptoms of constriction ring syndrome, syndactyly, and brachytelophalangy in the presence of proximal unilateral symphalangism. Physical findings revealed previous surgical correction of constriction rings of the right index and long fingers, a short right thumb with a hypoplastic distal phalanx, a shortened long finger with a stiff proximal interphalangeal joint and characteristic absence of skin creases, and incomplete syndactyly of the right first, second, and third web spaces. A brief review of symphalangism and a surgical treatment plan are presented. This is believed to be the first case report of symphalangism in a black patient with multiple congenital anomalies.

Abnormalities, Multiple↗

The three-square-flap method for reconstruction of minor syndactyly.

A three-square-flap procedure is described for surgical treatment of minor syndactyly. The average follow-up period in 58 cases (48 patients) was 2 years 4 months. In the three-square-flap method, the interdigital space is regarded as a cube and flaps are designed on each surface of the cube (dorsal side, interdigital surface, and volar side); the flap on the dorsal side forms the basal surface of the new interdigital portion. This technique is most suitable for reconstruction of minor three-dimensional contracture syndactyly. This method does not require skin grafting; the design is uncomplicated and easy to understand, and the operative procedure is simple.

Adolescent↗

Concomitant syndactyly and polydactyly in a pediatric foot.

Syndactyly and polydactyly in a child may warrant surgical treatment to avert resultant emotional and psychologic problems. The authors present a unique case report of a bilateral polydactyly of the hallux and bilateral syndactyly of the second and third toes in a normal 6-year-old Hispanic female. The article will discuss the surgical management of these problems, using the skin harvested from resection of the extra hallux for the adjacent side of the lesser toes after desyndactyly.

Abnormalities, Multiple↗

Genotype-phenotype analysis in Apert syndrome suggests opposite effects of the two recurrent mutations on syndactyly and outcome of craniofacial surgery.

Apert syndrome is an autosomal dominant condition characterized by craniosynostosis and severe syndactyly, caused by two recurrent mutations in the fibroblast growth factor receptor 2 gene (FGFR2). The genotype-phenotype correlations of 21 patients with Apert syndrome were analysed as to the craniofacial appearance following surgery and the degree of syndactlyly. The craniofacial appearance following craniofacial surgery was better in patients with the P253R mutation, whereas these patients showed a more pronounced severity of the syndactyly.

Acrocephalosyndactylia↗

Congenital syndactyly: defatting facilitates closure without skin graft.

We attempted to correct 24 syndactylies without skin grafts in 16 consecutive patients. Digital volume was decreased by defatting the full length of the fingers and the interdigital space. Closure of the zigzag incisions on both fingers could then be achieved with minimal tension and did not seem to be related to the type of flap used for commissure resurfacing. We used 2 different types of flap closure. We noted that defatting was a more important determinant of whether the wound could be closed without graft than flap configuration. The new type of flap may represent an improvement for web appearance but is not a determination for finger closure. Only a complex syndactyly involving 3 adjacent atrophied fingers required a skin graft for separation. The results were evaluated after a mean follow-up period of 22 months. Three patients had a complication: an intraoperative digital nerve laceration, a scar contracture, and a recurrence of the treated commissure due to local infection. Two patients needed another surgical procedure. Supple and good quality scars were observed in the other patients. No conspicuous change in finger contour compared with the nonsyndactylized fingers was observed.

Adipose Tissue↗

Nail fold creation in complete syndactyly using Buck-Gramcko pulp flaps.

We have used the double pulp flap technique described by Buck-Gramcko for nail fold creation in 75 fingertips after separation of 38 complete syndactyly webs in 27 patients. The operative technique is described and the results are discussed. Nail patterns in these complex syndactyly webs are analysed and a classification is proposed.

Fingers↗

Full-thickness skin grafts from the cubital fossa for syndactyly-release.

In syndactyly-release, skin defects along the sides of the fingers are usually covered with full-thickness skin grafts harvested from the groin. Since January 1997, we have been routinely harvesting full-thickness skin grafts from the cubital fossa instead. Skin grafts from the cubital fossa are softer and more pliable than those from the groin. Furthermore, they give a superior color match and cannot give rise to the growth of unsightly and embarrassing pubic hair in the hand. Donor-site morbidity seems to be nearly negligible, particularly if the donor site scar line is situated along one of the transverse skin creases of the cubital fossa. In cases of multiple syndactylies in a single hand, full-thickness skin grafts can be repeatedly harvested from the cubital fossa.

Child↗

Localization of a gene for syndactyly type 1 to chromosome 2q34-q36.

Syndactyly type 1 (SD1) is an autosomal dominant limb malformation characterized in its classical form by complete or partial webbing between the third and fourth fingers and/or the second and third toes. After exclusion of a candidate region previously identified for syndactyly type 2 (synpolydactyly), we performed a genomewide linkage analysis in a large German pedigree. We found evidence for linkage of SD1 to polymorphic markers on chromosome 2q34-q36, with a maximum LOD score of 12.40 for marker D2S301. Key recombination events in affected individuals defined a 9.4-cM region between markers D2S2319 and D2S344. The identification of the responsible gene will give further insights into the molecular basis of limb development.

Chromosome Mapping↗