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A case report and review of the English literature on the simultaneous dorsal dislocation of the interphalangeal joints of the finger are presented. This injury usually occurs on the ulnar side of the hand of young male athletes. It is easily reduced by a closed technique, although slight limitation of motion and joint swelling may persist.
Unstable, displaced, fractures of the hand present a challenge to surgeons all over the world. Retention of the reduction using a cast, splint or even K wire fixation is difficult during the period of bone healing: also a relatively prolonged period of immobilisation after such treatment leads to stiffness of the metacarpal and finger joints. The picture gets more complicated if the hand suffers more than one fracture. The unique ability of the hand to grasp and grip objects of different size and shape depends on the intact functional anatomy of the hand articulations. Thus accurate and stable reduction of metacarpal and phalangeal fractures is essential for early active mobility with a view to early return to work even before the recovery is complete, especially in the case of an industrial worker. We recommend osteosynthesis as a method of choice for treating such displaced, unstable fractures of the hand as shown by the present retrospective study of fifty patients. We suggest a simple practical method of assessing the results of treatment for severe fractures of the hand.
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To investigate the application of a mini-coil surface system for high-resolution MRI, 60 volunteers were examined in a 1.5-T whole-body scanner. Two replaceable probe heads were available: a circular 2.5-cm coil and a quadratic 5-cm coil, both of which were placed directly on the skin. The skin layers, Achilles tendon and finger joints were examined with the 2.5-cm coil and a FOV of 25 X 25 mm2. A matrix of 256 pixels resulted in a pixel size of 0.098 X 0.098 mm2. For imaging of the carpal tunnel, the 5-cm coil was used in transverse orientation. The FOV was 50 X 50 mm2 so that a matrix of 256 X 256 pixels led to a pixel size of 0.195 X 0.195 mm2. The resulting spatial resolution permitted visualization of the epidermis, dermis and subcutis, resulting in clear definition of anatomical detail of the musculoskeletal system. MRI measurement of skin-layer thickness did not correlate with histometric data (p < 0.05). This discrepancy was due in part to shrinkage of the tumor specimen on histologic preparation. Other causes include the motion artifacts and the limited accuracy of determining thickness on the MRI display unit.
A prospective study of postoperative mobilization of flexor tendon repairs in zone 5 was conducted over a 2-year period between 1994 and 1996 using a controlled active motion (active extension-active flexion) regimen of mobilization. Fifty-two patients, who had a total of 151 flexor digitorum superficialis (FDS) and 103 flexor digitorum profundus (FDP) divisions, were available for review at a mean follow-up of 10 months. Of the 161 fingers with division of one or both flexor tendons, 66% exhibited independent FDS function and 90% achieved good or excellent results of digital range of motion. No rupture of an FDP tendon repair occurred during the study period. The data allowed us to define a new method of classifying the results of treatment of these injuries in terms of the injured wrists as a whole and not simply as a series of isolated observations for each individual finger with divided flexor tendons. The results of recovery of independent FDS action and range of finger movement achieved for injuries in which the flexors of all four fingers had been divided indicate a statistically significant interdependence of injuries of finger flexors of adjacent fingers at the wrist. Multivariate analysis showed the presence of a "spaghetti wrist" injury to have a significant adverse effect on the recovery of the independent FDS action but not on the recovery of the digital range of motion.
PURPOSE OF THE STUDY: Total arthrodesis of the carpal joint is a complex operative procedure that results in the restoration of joint stability in frontal and sagittal planes and improvement in function of the tendons of digits' extensors and flexors. We developed our own method based on the use of a special implant; in this study we report our long-term results. MATERIAL: Since 1992 we have carried out 54 total carpal arthrodeses, using our method, on 51 patients; three patients were treated bilaterally. Both wrists were treated in. This group comprised 34 women and 19 men, with the average age of 47.6 years. We used the modified method mainly in patients with stage IV rheumatic carpal destruction (Larsen classification) or in those with wrist destruction due to arthritis or psoriasis. In two patients, this method was indicated because of a non-reparable lesion of the nervus radialis. It was also used in two patients who had their wrist replacements removed due to failure. METHODS: The procedure was carried out, with a tourniquet applied to the upper arm, from the dorsal approach to the carpal joint. After opening the capsule, using an oscillating saw, we resected the facies articularis radii, the carpal bones, which were freed from cartilage and turned into a cancellous in situ filling, and the distal ulna. This procedure prepared the operation field for the application of our plate. This L-shaped plate, only 2 mm thick, with its concave curve fitting the palm, allows for three-point fixation of the metacarpal region and also maintains slight compression. RESULTS: In all the patients, we achieved osseous fusion detectable by radiography and clinical examination on average at 12 weeks postoperatively. The grasping function of the hand improved in all patients because the carpal axis was adjusted to a functionally convenient position. The resection of the distal ulna, which is a part of our method, removed pain caused by supination or pronation. The patients reported the absence of pain, instability and edema of the wrist. DISCUSSION: The aim of any method for total wrist arthrodesis is the firm fixation of resection-treated articular surfaces of the radius, carpal bones and metacarpal bases for a period long enough to allow for their complete fusion. These techniques involve intraosseous procedures, the use of plates, osteorrhaphy and external fixation. Most of these methods use massive corticocancellous grafts collected from the hipbone crest. The critical point of all methods is fixation of the metacarpal region. CONCLUSIONS: The method described here is based on an original implant in the form of an L-shaped plate that permits sufficient fixation without using grafts taken from the pelvis. In patients with rheumatic arthritis, if needed, it facilitates peritenosynovectomy or reconstruction of spontaneous tendon ruptures in one operation. It does not require long-term immobilization in plaster cast and permits early rehabilitation of finger joints.
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OBJECTIVE: To describe the demographic, clinical, and laboratory features and natural history of patients with systemic sclerosis sine scleroderma (ssSSc), and to compare them with those of patients with SSc and limited cutaneous involvement (IcSSc). METHODS: The University of Pittsburgh Scleroderma Databank served as the data source. Patients were divided into those who had no skin thickening (ssSSc) and those who had skin thickening only distal to elbows or knees and/or of the face (IcSSc) during their disease course. These two groups were compared with regard to demographic characteristics, clinical, laboratory, and serologic features, and survival rates. Chi-square and Student's t-test analyses were performed, and Fisher's exact test was used as appropriate. RESULTS: Of 555 consecutive patients without diffuse cutaneous SSc, 48 (9%) had ssSSc and 507 (91%) had IcSSc. The ssSSc patients had a mean total disease duration of 18.6 years (15.1 years before study entry and 3.5 years of followup after study entry), and had not developed IcSSc or another connective tissue disease. Other than the absence of skin thickening, the ssSSc group had no significant differences in individual internal organ involvements, laboratory features, serum autoantibody type, or survival rate compared with patients with IcSSc. Within the category of lung involvement, patients with ssSSc had a significantly greater frequency of dyspnea with mild exertion or at rest, and a tendency toward reduced carbon monoxide diffusing capacity (<70% of predicted normal) and primary pulmonary arterial hypertension. Patients with IcSSc had significantly more frequent individual manifestations of digital pitting scars, digital-tip ulcers, telangiectasia, and calcinosis than those with ssSSc, in part related to increased time of observation. Puffy fingers and finger joint contractures were detected significantly more often in IcSSc patients. CONCLUSION: Systemic sclerosis sine scleroderma should be included in the spectrum of SSc with limited cutaneous involvement and should not be considered a distinct or separate disorder.
We report on a family of 19 individuals over four generations in which 12 members are affected with a variant of multiple epiphyseal dysplasia. Beginning in childhood, the disease leads to pain and stiffness of knees, ankles, elbows and finger joints. Some adult patients repeatedly suffer from free articular bodies resulting in locking of the joint. Finally, affected individuals are prone to the development of early degenerative joint disease. Mutation screening of candidate regions revealed a novel point mutation at position -1 in the COL9A2 exon 3/intron 3 splicing region. This G --> C substitution most probably induces an alteration of the splicing process. Family screening was carried out by both automated sequencing and by digestion of amplicons with BsaWI. We confirmed the nucleotide substitution in eight clinically affected family members as well as in three presymptomatic young children. Electron microscopy showed that the diameter of collagen fibrils from arthroscopically removed free articular bodies of two patients was not obviously different from that of normal articular cartilage. Together with previous reports our results indicate that mutations leading to skipping of exon 3 within the COL3 domain of the alpha2-chain of collagen type IX may be relatively common in patients with a special subtype of multiple epiphyseal dysplasia (MED) in which the hips are not markedly affected at early age (EDM2). In these patients and their families, mutation screening of the candidate regions may help to confirm the diagnosis, lead to appropriate advice for lifestyle and well based genetic counseling.
We report a 4-year-old boy, a 6-month-old girl, and a 17-week-old fetus all with a chromosomal imbalance derived from a balanced translocation t(13;15)(q34;q26.1) of their father. The boy had a partial trisomy for 15q26.1-qter (46,XY,der(13)t(13;15)(q34;q26.1)) and postnatal overgrowth, as well as craniosynostosis, facial anomalies, and finger joint contractures, while the girl with the same chromosomal aberration did not show overgrowth, although she had similar craniofacial and skeletal abnormalities. The fetus had a partial monosomy for 15q26.1-qter and intrauterine growth retardation (IUGR). Fluorescence in situ hybridization (FISH) analysis with a BAC clone covering the insulin-like growth factor 1 receptor gene (IGF1R) that is located to 15q25-q26 revealed three copies in the boy, one copy in the fetus, and two copies in their phenotypically normal father. Since deletion of IGF1Rhas repeatedly been reported to be associated with IUGR, it is tempting to speculate that the dosage of IGF1R may have determined growth in these children.
The fact that a connective tissue dysplasia is a component of the Martin-Bell syndrome was a fortuitous discovery. A 26-month-old developmentally delayed boy had many signs of a connective tissue dysplasia for which he was referred to a University center where he was found to be fragile (X)-positive without confirmation of a connective tissue problem. Sensitized by these events and observations, we were able to predict at a glance in an unrelated family the fragile (X)-positive status of 2 subsequently referred brothers with mental retardation and prominent manifestations of connective tissue dysplasia. Thus, the Martin-Bell syndrome is an incompletely recessive, pleiotropic trait involving CNS, testes and connective tissues. The characteristic facial appearance of affected males largely represents interaction of mental retardation, congenital CNS based muscle hypotonia and connective tissue dysplasia. At the 1983 NIH workshop on XLMR there was a general consensus that a connective tissue dysplasia is a component of the Martin-Bell syndrome, a fact since confirmed by others on the basis of objective measurements of finger joint hypermobility and frequent presence of mitral valve prolapse.
Thirty anthropometric measurements were analyzed in 147 adults with Martin-Bell syndrome (MBS) (56 men and 91 women) and in a random sample of 108 normal women and 111 men. Results of the univariate comparison of the age, height, or weight-adjusted variables between MBS and normal individuals of either sex indicated that a decrease in stature, in upper limb length, and in upper face height, and an increase in jaw length, chest circumference, and waist width occurred in both affected men and in heterozygous women. While the increase in ear height and breadth and in hypermobility of finger joints and decrease in palm width and bigonial diameter occurred only in affected men, increased bispinal and bitrochanteric diameters, upper arm circumference, and palm and wrist widths were characteristic deviations in heterozygous women. Multivariate analysis in the form of principal components showed some differences in the pattern of interrelationships in individual measures between MBS and normal individuals. In particular, and in contrast with both normal groups, height and weight tended to load on separate components (as did head and midfacial measures) in MBS individuals. A discriminant function based on all body measurements included in this study resulted in almost complete separation of discriminant scores of normal from those of MBS men and in good separation of the scores from normal and heterozygous women. Classification rates based on these functions were from 95% in men to 85% in women. These already high classification rates may be further improved mainly by enlarging the samples and including some other category of traits such as dermatoglyphic measurements.
We report on 2 unrelated patients with Costello syndrome. The first is a 5-year-old girl with "coarse" face, nasal papillomata, redundant skin of feet and hands, hyperextensible hand and finger joints, curly hair, feeding problems due to oral motor apraxia, growth and psychomotor retardation. The second is a 3-year-old boy with "coarse" face, loose skin on hands and feet, curly hair, oral motor apraxia, severe growth and psychomotor retardation. In both patients urine sialic acid levels were found to be repeatedly high. The meaning of this biochemical abnormality is discussed.
A familial case of brachydactyly type C is presented in which hand radiographs of the proposita's parents was considered normal. The metacarpophalangeal profile pattern of the mother's hands demonstrated minimal manifestations typical of brachydactyly type C.
Ehlers-Danlos syndrome (EDS) dermatosparaxis type (type VIIC) and the related disease of cattle dermatosparaxis, are recessively inherited connective tissue disorders, caused by a deficient activity of procollagen I N-proteinase, the enzyme that excises the N-terminal propeptide in procollagen type I, type II, and type III. Although well documented in cattle, to date only seven human cases have been recorded, most of them aged under 2 years. We document the natural history of three patients with EDS dermatosparaxis type, two of whom have been reported before the age of 2 years, and one new patient. The phenotype of the patients, and especially the facial resemblance, is striking, making this a clinically recognizable condition. The most consistent anomalies during the first years of life are premature rupture of the membranes, extreme skin fragility and easy bruising, large fontanels, blue sclerae, puffy eyelids, micrognathia, umbilical hernia, and short fingers. Joint hypermobility becomes more important with age. The children are at risk for rupture of internal organs due to soft tissue fragility, as is illustrated by different internal events in two of the three patients described here. Orofacial features include micrognathia, a frontal open bite, and gingival hyperplasia with varying degrees of hyperkeratosis. The deciduous dentition shows abnormal morphology of the molars, obliteration of the tooth pulp, and severe enamel attrition. The permanent dentition shows agenesis and microdontia of several teeth. Tooth discoloration, dysplastic roots, and tooth pulp obliteration are present in a restricted number of permanent teeth.
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