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Hypothelia, syndactyly, and ear malformation--a variant of the scalp-ear-nipple syndrome?: Case report and review of the literature.

The scalp-ear-nipple syndrome is a rare autosomal dominant condition that involves lesions of the scalp, malformed external ears, and absence of rudimentary nipples and breasts. We report a case of a woman with hypothelia, bilateral mildly malformed ears, and syndactyly of the hands and feet, and review the literature on the hypothelia/athelia phenotype. This case may represent a mild phenotype of the scalp-ear-nipple syndrome or a newly recognized entity.

Abnormalities, Multiple↗

Local epidermoplasty for syndactyly.

A new method of treating syndactyly is described. The fingers are separated by graduated steps in a special apparatus using skeletal fixation of the phalanges with wires. Skin grafting is then carried out. Scarring and deformity are avoided. The operation can be used for very young children and our patients were aged from 5 months to 30 years. The results of 60 operations are reviewed: 56 were good, 3 satisfactory and 1 unsatisfactory. The advantages of the procedure are discussed.

Adolescent↗

A new form of spondyloperipheral dysplasia with facial dysmorphism, flattened vertebrae, hypoplastic pelvis, brachydactyly and soft tissue syndactyly.

We report the case of a 9-year-old Japanese boy with spondyloperipheral skeletal dysplasia associated with facial dysmorphism, pelvic abnormalities, and distinctive hands and feet. Radiographic manifestations included mild platyspondyly with posterior scalloping, small flared ilia with shallow acetabulae, mesomelic shortening of long bones, marked delay of carpal bone maturation, and brachydactyly with hypoplastic middle and terminal phalanges bilaterally in both hands and feet. There was bilateral soft tissue syndactyly of the 2nd and 3rd interdigital spaces of the hands, the 2nd interdigital space of the feet, with hypoplastic nails. The clinical and radiographic manifestations in this case appear to represent a unique type of skeletal dysplasia.

Abnormalities, Multiple↗

Median cleft lip, polydactyly, syndactyly and toe anomalies in a non-Indian infant.

A case is reported of a child who presented with a median cleft of the lip and alveolus with polydactyly, a complex form of syndactyly and multiple toe anomalies. This is the fifth case to be reported with these features and the second case to be reported in a non-Indian patient. Our case is characterised by unique features, consisting of a complexity of hand anomalies in association with other facial anomalies such as median alveolar cleft, epicanthus, fusion of the tongue to the floor of the mouth and rare big toe anomalies.

Abnormalities, Multiple↗

Surgical treatment of contracture and syndactyly of children with epidermolysis bullosa.

A new approach to the operative treatment of syndactyly and contracture of the hands of children with recessive dystrophic epidermolysis bullosa is described. It is based upon the principle of surgical release of fingers allowing spontaneous epithelialisation of skin wounds without using skin grafts. Nineteen children had operations using this method, with an incidence of recurrence of 53%. This method has the advantage of a short operating time, simple technique and limited trauma.

Adolescent↗

Reevaluation of a kindred with congenital absence of dermal ridges, syndactyly, and facial milia.

We observed a newborn infant of a previously reported kindred with absent dermal ridge pattern, syndactyly, and facial milia. The infant's features were consistent with three other kindreds, suggesting that this entity is a single disorder with variable expression. Furthermore, this entity should be considered in the differential diagnosis of excessive congenital facial milia and erosions.

Adult↗

A double pulp flap technique for creating nail-folds in syndactyly release.

A method is described of creating nail-folds in the release of cases of complete syndactyly. A double pulp flap was used as a one-stage technique in 13 patients in whom webs were separated. All patients were reviewed after a minimum of one year. Fullness of pulp was achieved in all fingers. The nail-fold was considered normal in 14 of 18 fingers covered with a broad flap and in 8 of 18 fingers covered with a narrow flap. In the remaining cases the nail-fold was small but never absent. No flap loss was encountered and there was no late nail deformity from scarring.

Child, Preschool↗

Foot-like congenital macrodactyly with syndactyly of the hand.

A case of monstrous deformity of the right hand in a six-year-old girl is reported. The index, middle and ring fingers showed extreme macrodactyly including the metacarpals belonging to these fingers. This deformity, which was associated with syndactyly of the middle and ring fingers, was caused by bone overgrowth in gigantic fashion accompanied by enlarged volar digital nerves and studded with irregular nodules of fibro-adipose tissue in these fingers. No genetic abnormalities were found.

Child↗

Full thickness grafts taken from the plantar instep for syndactyly release.

Thirteen digital clefts (eight patients) were reviewed in which full-thickness plantar instep skin was used in syndactyly release. The results are presented, emphasising the good colour match of plantar instep skin with digital skin. There was a surprising lack of morbidity in the thirteen donor sites.

Adolescent↗

Three cases of syndactyly, polydactyly, and hypoplastic triphalangeal thumb: (Haas's malformation).

Three cases of a complex malformation of the hand involving syndactyly, polydactyly, and hypoplastic triphalangeal thumb are presented. In all three patients, six metacarpal bones and hypoplasticity of the thenar muscles were present. In one patient, both hands, except the most radial digits of the left hand, were completely fused by soft webs as reported by Haas and Ofodile. In two other patients, the extent of the webs differed from Haas's case, but one of them was similar to Ofodile's first case. In all instances the supernumerary digit had a triphalangeal configuration and the thenar muscles were hypoplastic, often associated with some degree of webbing.

Adult↗

A three-flap web-plasty for release of short congenital syndactyly and dorsal adduction contracture.

This article describes a technique for interdigital web space deepening, which is applicable to incomplete syndactyly proximal to the proximal interphalangeal joint and to dorsal web space adduction contracture resulting from thermal injury. It consists of a dorsal rectangular flap and two palmar triangular flaps. The flaps are reversed to release dorsal adduction contracture. This procedure provides a broad commissure with a natural slope. A skin graft is avoided. A mathematical model shows that when the length-width ratio of the flaps is 1:1, the surface area of each flap is greater than or equal to the surface area of the defect it covers and the length of every flap edge is equal to or greater than its corresponding defect edge. If a web has a low apex angle and the skin is elastic, the length-width ratio may be as great as 1.5:1. The flaps will still cover the defects and small inequalities between certain flap and defect edges will be accommodated.

Contracture↗

Keloid formation in a simple syndactyly release: a case report.

Keloid formation on the palms of the hands and soles of the feet is rare. A keloid developed 2 months after release of simple syndactyly in a young black child. The process of keloid formation is not completely understood, and surgical correction is not effective.

Fingers↗

Long QT syndrome associated with syndactyly identified in females.

The identification of female children with this syndrome is evidence that this disorder is not X-linked in inheritance. Possible inheritance modes still include autosomal recessive or, more likely, a de novo mutation, given the absence of family history in any of the patients. Children of both sexes with syndactyly should be screened with an electrocardiogram for this syndrome. Female children with this syndrome may have an increased risk of sudden death similar to male children.

Electrocardiography↗

The "seagull" flap for syndactyly.

A technique is described for the treatment of post-burn syndactyly using a seagull shaped flap to produce a realistic commissure that does not subsequently advance distally. It can release volar digital contractures at the metacarpo-phalangeal level and import skin with tactile "adherence" into the distal palmar area.

Burns↗

Syndactyly correction.

The operation to be described has been used by the author, with minor modifications, for over 10 years. A series of 57 cases is presented, all of which were simple, uncomplicated examples of syndactyly. The correction is based on separation of the cleft into rectangular flaps combined with removal of interdigital fat. Performed at one year, this allows complete closure of the ulnar side of the cleft and, in mild degrees, complete closure of the radial side also. A dorsal island flap is used to reconstruct the web. The results have been assessed with a minimum follow-up of 2 years and are discussed.

Female↗

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↗