1/31. brachydactyly type B: case report and further evidence for clinical heterogeneity.We present a male child with digital and radiographic findings consistent with brachydactyly type B. His left hand had 2-4 syndactyly, shortened first and fifth digits and single palmar and fifth finger flexion creases. The nail on the second finger was hypoplastic. The right hand had a small thumb and curving of the second finger towards the midline. The left foot had a short left hallux with an absent nail, 2-4 syndactyly and an absent toe. His right foot had a short, dorsiflexed hallux with a hypoplastic nail, 2-3 syndactyly and an overlapping toe. Radiographs showed asymmetrical hypoplasia of the middle and terminal phalanges and there were no extra-articular findings to suggest Sorsby syndrome. brachydactyly type B has classically been described as hypoplasia or absence of the terminal phalanges with absent nails. Although other digital anomalies have previously been described with brachydactyly type B, the absent digit, atypical syndactyly and marked asymmetry in this child make this case a good illustration of the clinical heterogeneity that can be associated with this type of brachdactyly. We provide a brief review of syndromes in which similar digital malformations have been reported.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
2/31. Dominant inheritance of cleft palate with minor abnormalities of hands and feet: a new syndrome?We report a family in which four members of three generations are affected by median cleft palate. The proband and her mother have additional features including bilateral single transverse palmar creases, broad great toes and hypoplastic fifth toenails. Dominant isolated cleft palate has rarely been reported, and there are no previous reports of dominant cleft palate with these associated features. We believe that this represents a previously unreported syndrome, which needs to be considered when assessing recurrence risk for cleft palate.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
3/31. Precision grip with congenital hypoplasty or hypofunction of the thumb.We tested the grip in four patients with congenital defects of the hand and either a hypoplastic thumb or a thumb with impaired inervation. Small objects were taken by a scissors grip between the fingers. In a hand with radial duction in the manus vara congenita, during strengthening of the wrist, the grip from the ulnar side between the fourth and fifth fingers was changed to the radial side between the second and third fingers. Large objects were gripped by all the three-phalanx fingers into the palm in a horizontal position. In case 4 with hypoplasia of the thumb grade IIIC by the classification of Blauth and Buck-Gramcko, we describe a transposition of the index finger to the site of the thumb and the hypoplastic thumb to the site of the index finger. It is obvious that the precision grip is affected by the thumb length and strengthening of the ulnar side of the wrist. We assume that the scissors grip is the earliest precision grip in the evolution of the primate hand.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
4/31. Autosomal recessive multiple pterygium syndrome: a new variant?Multiple pterygium syndromes include at least 15 different entities characterized by multiple pterygia or webs of the skin and multiple congenital anomalies. We describe a female infant who presented with a distinct constellation of multiple anomalies consisting of pterygia of the inguinal, intercrural and popliteal areas, flexion contractures and arthrogryposis of some joints, craniofacial anomalies including ectropion, medial canthal web, blepharophimosis, hypoplasia of nose, oral and nasopharyngeal cavities, vocal cords and tongue, micrognathia, orolabial synechiae secondary to pterygia, low set ears, alopecia, sad and expressionless face, short neck, asymmetric nipples, anal stenosis, rectal polyp, hypoplastic labia majora, complete syndactyly of all fingers and toes, pes equinovarus, bandlike web between feet, and absence of the nails and phalangeal-palmar creases. Radiological examination showed synostosis, absence or hypoplasia of metacarpal, metatarsal and phalangeal bones on feet and hands, and hypoplasia of pelvic bones and scapulae. This pattern of anomalies does not fit entirely any of the known multiple pterygium syndromes. Autosomal recessive inheritance is most likely due to the presence of three similarly affected siblings and normal parents.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
5/31. A congenital hand deformity: Dupuytren's disease.A 10-month-old child presented with a lack of extension at the distal interphalangeal joint. Despite the absence of trauma, a provisional diagnosis of mallet finger led to treatment using a short dorsal splint. Four months later the flexion had increased and included the proximal interphalangeal joint. palpation revealed a palmar cord on the lateral aspect of the finger. Surgery disclosed a typical Dupuytren cord and the histology supported this diagnosis. There was no known family history of the disease. There was no sign of recurrence 27 months after surgery in this case of Dupuytren's disease that was present at birth.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
6/31. An anomalous flexor digitorum sublinis to index finger with absent lumbrical.A case is presented in which one tendon of the flexor digitorum sublimis was partly replaced by muscle in the palm and there was absence of the associated lumbrical.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
7/31. Callotasis lengthening in patients with brachymetacarpia.Callotasis lengthening is an established method, but few cases of metacarpal lengthening have been reported. The authors performed callotasis lengthening to treat brachymetacarpia in six digits in three patients. The patient age at the time of distraction ranged from 10 to 19 years. The period of application of an external fixator averaged 13.9 weeks (range 10-19 weeks). All the metacarpals achieved the target length, and all patients were satisfied with the esthetic improvement. The length of the metacarpal distraction averaged 15.2 mm (range 10-18 mm). Complete consolidation of the transport gap was achieved for five of six digits. One digit that had a history of previous lengthening required bone graft. The average healing index was 62.3 days/cm. There were no serious complications. In four digits, the range of flexion of the metacarpophalangeal joint was increased after lengthening, and this enabled the patient to use the digit easily in a palmar grasp. Brachymetacarpia is an appropriate indication for callotasis lengthening in congenital deformities of the upper extremities.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
8/31. A minimal form of proteus syndrome presenting with macrodactyly and hand hyperplasia.proteus syndrome is a rare congenital disorder characterized by progressive course and great variability of clinical presentation with partial gigantism of extremities, hemihyperplasia with macrocephaly, epidermal nevus, mesodermal hamartomas and the presence of peculiar cerebriform masses on the palms/soles. Many atypical cases have been reported and this is probably due to the mosaicism of the genetic disorder displaying different clinical features. We describe a patient with an extremely mild form of proteus syndrome presenting macrodactyly and hyperplasia of one hand which was misdiagnosed until the age of 33 years.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
9/31. Congenital duplication of the palm in a patient with multiple anomalies.In 1995, Parr and McMahon described a syndrome of congenital duplication of footpads in mice which lacked a protein called 'Wingless-type mouse mammary tumour virus integration site family member 7a" (Wnt-7a). This syndrome has not been described in humans and the following report describes a similar syndrome in a Saudi girl. The role of Wnt-7a in the development of the limb along the dorso-ventral axis is discussed, along with interaction between the Wnt-7a and other axes of limb growth.- - - - - - - - - - ranking = 4keywords = palm (Clic here for more details about this article) |
10/31. Probable new syndrome in a Mexican family with congenital palmar polyonychia and postaxial limb defects.Congenital palmar polyonychia (CPP) is a rare and usually sporadic birth defect. We report on a Mexican girl with CPP of both 5th fingers and her sister with ectrodactyly with ulnar ray deficits and agenesis of the ulna. In previous reports, CPP has been seen in ectrodactyly with involvement of the ulna and ulnar digital rays and with postaxial polydactyly. Such findings observed in our patients can be considered a form of CPP. Autosomal dominant inheritance of CPP is more likely based in previous informative families with vertical transmission and instances of male-to-male transmission. The present and two previous families with affected sibs only may represent parental gonadal mosaicism, whereas de novo mutation or incomplete evaluation of relatives could be an explanation for sporadic cases. Variable expression of the CPP phenotype may be a new autosomal dominant entity, i.e., a CPP-postaxial limb defect syndrome.- - - - - - - - - - ranking = 5keywords = palm (Clic here for more details about this article) |
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