Classification for Thumb Opposition in Children with Thumb Hypoplasia.

Background: The Kapandji scale has 10 levels and includes abduction, rotation and flexion movements. Assessing children with thumb hypoplasia, this scale is poorly understood and confusing. The objective of the study is to evaluate an opposition scale simpler and understandable for children. Methods: It is a validation study of a simplified scale to qualify the opposition of the thumb, in order to asses only the abduction - rotation but not the flexion of the metacarpo-phalangeal (MP) or interphalangeal (IF) joint. The proposed classification goes from 0 to 3 degrees, where 0: No opposition, 1: The thumb touches the middle phalanx of the 2nd finger, 2: the thumb touches the pulp of the 2nd finger and 3: the thumb touches the pulp of the 5th finger. 10 patients with thumb hypoplasia were analyzed by 9 hand surgeons and 9 orthopedic surgeons. Results: The intraclass correlation coefficient shows a very good inter-observer reliability with a kappa of 0.991 (p = 0.000). When correlated by groups, the finding were very good between the hand surgeons group k = 0.980 (p = 0.000) and the orthopedic surgeons group 0.974 (p = 0.000). At 6 weeks a new evaluation was made, the intra-observer reliability was excellent k = 0.995 (p = 0.000). Conclusions: The proposed scale for the evaluation of the abductionrotation of the thumb is validated and useful in the evaluation of the results of a thumb opposition transfer with a good inter-observer and intra-observer reliability between orthopedic and hand surgeons.

Surgical techniques for reconstruction of the hypoplastic thumb.

The reconstruction of a congenital hypoplastic thumb usually involves release of a tight first web space, metacarpophalangeal joint stabilization, reconstruction of intrinsic muscle function, and extrinsic tendon reconstruction, as appropriate. Numerous surgical options and combinations are available, but the approaches vary among surgeons who work in the field of congenital hand surgery and the empirical evidence that allows for evaluation of the results of techniques is scarce. Both the pre-operative assessment and intra-operative findings of all thumb elements - bone, joints, and soft tissues - should be considered in the surgical decision-making and eventually define the methods of reconstruction. This article summarizes the different reconstructive options.

The Neurological Hand Deformity Classification for children with cerebral palsy.

BACKGROUND/AIM: The purpose of this study was to evaluate the reliability of the Neurological Hand Deformity Classification and use it to describe changes in hand deformity over time in children with cerebral palsy. METHODS: We identified 114 video clips of 26 children with cerebral palsy, aged 1-18 years (mean = 8.4, SD = 4.2), performing upper-limb tasks at multiple time points (n = 3-8) at least 6 months apart. Using the Neurological Hand Deformity Classification, three observers classified hand deformity in the video clips. Inter- and intra-observer reliabilities were estimated using Fleiss and Cohen's kappa (κ) and the temporal changes in classification of hand deformity were investigated. RESULTS: Inter- and intra-observer reliability respectively were κ = 0.87 and κ = 0.91. Hand deformity was identified in all children at all time points, even before the age of 2 years. Ten children did not change hand classification, wrist flexion increased in eight, and eight showed changes from wrist flexion to extension or vice versa. CONCLUSIONS: The Neurological Hand Deformity Classification is a reliable tool to classify hand deformity in children with cerebral palsy. For more than one-third of children hand deformity classification did not change. For the remaining children, two patterns of change in hand deformity over time were identified. It is recommended that children with cerebral palsy involving their upper limbs be monitored regularly. SIGNIFICANCE OF THE STUDY: This is the first study to document longitudinal changes in hand deformity in children with cerebral palsy.

Concurrent dorsal dimelia in 160 consecutive patients with congenital anomalies of the hands and feet.

This study was designed to investigate the prevalence of dorsal dimelia in a series of 160 consecutive patients with congenital anomalies of the hands and feet, and to investigate the distribution of dorsal dimelia and the concurrent anomalies. Five cases (3.1%) showed evidence of dorsal dimelia and the distribution of dorsal dimelia was similar to the distribution of concurrent anomalies in all five cases. Another 11 cases of concurrent dorsal dimelia with other congenital anomalies have been reported previously with a positive match in the distributions in all cases. This similarity in the distribution in all 16 reported cases (including the five cases in the current study) is statistically significant. It is concluded that dorsal dimelia in humans is not as rare as it is generally thought to be, and that it may be viewed as an error of dorso-ventral patterning, which occurs in the same distribution as other concurrent anomalies.

On the classification of congenital thumb hypoplasia.

In 1937, Müller introduced the concept of a teratological sequence of thumb hypoplasia with increasing severity from mild deficiency, through severe deficiency, to thumb absence. Blauth subsequently detailed five specific grades. In 1992, Manske and McCarroll altered Blauth's classification such that Grade 3 was sub-divided into Grades 3A and 3B, according to a presence or absence of the proximal metacarpal. Buck-Gramcko added a Grade 3C in which there was only a remnant metacarpal head. This article investigates their publications and those of others to identify 'who said what' and clarify the definitions of grades of thumb hypoplasia. A modification of Blauth's classification is proposed, which retains the integrity of the concept of Müller and the skeletal and soft tissue grading of Blauth, but which also incorporates the disparate anomalies that may present in Grades 2 and 3 hypoplastic thumbs.

[Description of an original conservative method for the surgical management of the Blauth IIIb thumb hypoplasia: "relative lengthening - thumb stabilization"]. / Apport d'une technique originale de chirurgie conservatrice dans la prise en charge des hypoplasies du pouce de type IIIb : « l'allongement relatif-stabilisation de la colonne du pouce ¼

PROBLEMATIC: Thumb hypoplasia is a rare congenital hand disorder. Despite the recent clarifications concerning many surgical indications, dealing with Blauth IIIb type remains a polemic subject. We hereby suggest "The relative lengthening - thumb stabilization", an original and simple conservative surgery technique for the Blauth IIIb type, in order to satisfy parents and big kids requests of a "five fingered hand". The whole hypoplastic thumb is lowered in a way that lengthens it relatively. An intentional pseudarthrosis is performed between the first metacarpal base and the second metacarpal diaphysis, creating a "new trapeziometacarpal joint". PATIENTS AND METHODS: The suggested technique was performed on four IIIb type hypoplasic thumbs of three kids since 1997. RESULTS: The average postoperative follow-up is 9.6years (3.5 to 12.5years). The average Kapandji score is 5.75 (5 to 6). The key pinch prehension strength is by 21.5% in relation to the normal hand and the global prehension strength (according to Jamar Test) is by 67%. The global pinch is possible and natural in all cases. DISCUSSION AND CONCLUSION: The surgical management of the type IIIb thumb hypoplasia is a real challenge. We regard this technique of relative lengthening with thumb stabilization as an interesting alternative to the other microsurgical conservative surgeries.

Congenital upper limb deficiencies and associated malformations in Finland: a population-based study.

PURPOSE: To calculate the national incidence of upper limb deficiencies and associated infant mortality in children in Finland using the International Federation of Societies for Surgery of the Hand (IFSSH) classification. Radial ray deficiency, ulnar ray deficiency, central ray deficiency, transverse arrest, phocomelia, undergrowth, and constriction band syndrome with skeletal defects were evaluated. METHODS: We reviewed upper limb deficiencies among all 753,342 births in Finland during 1993 to 2005 reported to the Finnish Register of Congenital Malformations. Classification of these upper limb deficiencies was done according to a modified IFSSH system. We calculated incidence, gender and side distributions, frequency of associated anomalies, and infant mortality rates in different subtypes of the deficiencies. Familial occurrence of congenital upper limb defects was recorded. RESULTS: A total of 419 cases (234 male, 185 female) of upper limb deficiencies were identified. The national incidence of upper limb deficiencies was 5.56 per 10,000 births and 5.25 per 10,000 live births. The most common upper limb abnormality was radial ray deficiency (138), followed by subgroups of undergrowth (91), upper limb defects due to constriction band syndrome (51), central ray deficiency (41), and ulnar ray deficiency (33). Perinatal mortality was 14%. Infant mortality among children with upper limb deficiencies was 137 per 1,000 live births, compared with an overall infant mortality of 3.7 per 1,000 live births in Finland. Additional birth defects were found in 60% of these children. Prevalence of upper limb defects in relatives of the census population was 2% (11 of 419). CONCLUSIONS: The national incidence of upper limb deficiencies is 5.25 per 10,000 live births. Congenital upper limb deficiencies are associated with additional birth defects in two thirds of cases. These children, especially children with radial ray deficiency, have a high perinatal mortality rate. When divided into subgroups using IFSSH classification, differences emerge in both associated anomalies and mortality.

[Blauth classification of thumb hypoplasia: thought and proposition of modification]. / La classification de Blauth des hypoplasies du pouce : réflexion et proposition de modification.

Using the conclusions of the previous article concerning the therapeutic indications for handling hypoplastic thumb, we suggest an amendment to the current Blauth classification. This alteration is based on the management of this congenital anomaly, whereas the Blauth classification describes the morphological characteristics of the malformation. It appears for us more usable in practice by its easiness to be memorized.

Overgrowth conditions: a diagnostic and therapeutic conundrum.

Over the past five decades, one of the major problems in the treatment of overgrowth syndromes of the hand has been classification. Currently, a number of specific conditions with known natural histories have been delineated and reviewed. Treatment is highly individualized. Amputation is still recommended for grotesque deformities of the upper limb. Early aggressive surgery, when indicated, will improve contour, function, and appearance. Specific therapeutic techniques include radical debulking, microvascular neurolysis, vascular reconstruction, epiphysiodesis, and, in some cases, thumb or digit replacement. Long-term outcomes remain in the good-to-fair range and are dependent upon the condition, reconstructive techniques employed, age of patient, and the severity of deformity.

[Hand deformity in adult rheumatoid arthritis and juvenile chronic arthritis]. / Deformacje reki w przebiegu reumatoidalnego zapalenia stawów u doroslych i mlodzienczego idiopatycznego zapalenia stawów.

Adult rheumatoid arthritis (RA) is the most common of rheumatoid diseases, that may cause hand dysfunction in some patients. Its equivalent in children is juvenile chronic arthritis (JCA). The aim of our study was to evaluate differences in hand deformity between children with JCA and adults with RA. The prospective study was performed on two groups of patients: 15 with JCA (average age 13.1 years, range from 9 to 18 years) and 15 with RA (average age 53.6 years, range from 42 to 60 years). Both groups were similar in terms of Seyfried classification system and duration of the disease--7.9 years for children and 8.6 for adults. Clinical assessment was performed according to Swanson and Seyfrieda classification system. Patients with RA had only radial wrist "deviation" and those with JCA had both radial and ulnar wrist deviation. In MCP joints in adult's group fingers were always in ulnar position and in children's group finder position was opposite to wrist position. "Swan neck" deformity of fingers from II to V was found in both groups. "Buttonhole deformity" was more often seen in older group. Pain of wrist and in IP joints was more often found and was more severe in RA group. Hypertrophy of synovium and subluxation of IP and wrist joust were found with similar frequency in both groups. In other joints subluxation was rare. Concluding, radial wrist deviation is typical for RA patients. Children with JCA had both radial and ulnar wrist deviation. In MCP joints deformity is always opposite to wrist deviation.

A method for shortening instruments using the Rasch model. Validation on a hand functional measure.

BACKGROUND: Many measurement instruments, particularly measures of hand functional ability, frequently comprise a large number of items. Reduced versions of these instruments can facilitate their use. This work proposes a new method for shortening an instrument. METHODS: The method proposed was based on a scale of item difficulty calculated using the Rasch model. It was applied on a hand functional measure comprising 67 tests. The sample included 194 patients with hand lesions. The shortened instrument obtained was compared with those provided by classic methods used in the literature, with item random choice, and with shortened versions proposed by four independent experts, two rehabilitation physicians and two occupational therapists, who are clinicians familiar with the tool. All the statistical analyses were carried out on a random sub-group of two-thirds of the sample. A cross validation was then carried out on the remaining third. RESULTS: The reduction obtained had score non significantly different from that of the original instrument. In addition, the intra-class correlation coefficient and the Cronbach alpha coefficient were high. Among the different degrees of reduction investigated, the 12-item version seemed to be appropriate. Our method appeared to provide better results in terms of discriminant validity and internal validity than the choices of the four experts. The reductions produced were also better than those obtained by classic methods based on principal component analysis and multiple linear regression, as well as those obtained by random choices of items. CONCLUSION: The method presented is pertinent and useful. The reduction obtained appeared to be better than the choices of experts and the reductions provided by classic methods. The method could be used in other fields.

[Psychogenic spastic hand]. / La main spastique psychogène.

Twelve patients seen over a 8-year period with psychogenic spasms of the hand are reported. Six elderly patients presented with extrinsic flexion of the two or three medial fingers with sparing of the thumb and index. Six other patients had various hand attitudes following coincidental but not causal trauma to the upper extremity. Because of the differences in clinical presentation, age of patients and coincident trauma in some cases, the former patients were characterized as type I psychogenic spasms and the latter as type II. A major recurrent depression was the commonest psychiatric diagnosis (DSM-III-R, Axis I) with a concurrent dependent or borderline personality (DSM-III-R, Axis II). The diagnosis of psychogenic spasm of the hand is a diagnosis of exclusion, that requires a multidisciplinary approach including surgeon, neurologist, psychiatrist and rehabilitation therapist. Electromyographic studies are used to rule out a neurological or muscular etiology of the condition. Surgical treatment was uniformly unsuccessful and is probably contraindicated. Only one patient had a nearly complete spontaneous recovery.