Trastorno de la alineación de articulaciones metacarpofalángicas en una adolescente con artritis idiopática juvenil / Metacarpophalangeal joint alignment disorder in a teenager diagnosed with juvenile idiopathic arthritis

La artritis idiopática juvenil es una enfermedad inflamatoria, sistémica y crónica que afecta a personas menores de 18 años de edad. Tiene diversas formas clínicas de presentación, pero todas y cada una de ellas pueden causar un elevado número de complicaciones articulares y extraarticulares que pueden desencadenar discapacidad funcional y disminución de la percepción de la calidad de vida relacionada con la salud. Se presenta la imagen poco frecuente de alteraciones de la alineación en las articulaciones metacarpofalángicas de ambas manos en una paciente de 14 años de edad con antecedentes de 3 años de evolución de artritis idiopática juvenil. Se administró tratamiento con esteroides y medicamentos modificadores de la enfermedad. A los 3 meses se encontraba estable, con una escala visual análoga de dolor ocasional de 2 puntos y con esquema terapéutico adecuado para el control de su enfermedad de base(AU)

Juvenile idiopathic arthritis is an inflammatory, systemic and chronic disease that affects people under 18 years of age. It presents various clinical forms of presentation but each and every one of them can generate a high number of joint and extra-articular complications that can trigger functional disability and decrease in the perception of health-related quality of life. There is a rare image of alterations in the alignment at the level of metacarpophalangeal joints of both hands in a 14-year-old patient with a history of three years of evolution of juvenile idiopathic arthritis(AU)

Polydactyly, postaxial, type B.

BACKGROUND: Postaxial polydactyly, type B is the most common type of polydactyly. The vestigial sixth finger is attached by a narrow neurovascular pedicle to the lateral aspect of the hand or foot at the level of the metacarpal-phalangeal joint or the metatarsal-phalangeal joint. The occurrence of this type of polydactyly varies among racial groups, by sex and sidedness. Postaxial polydactyly, type A is a fully developed extra digit on the lateral aspect of the hand or foot with a bifid fifth or sixth metacarpal/metatarsal and is much less common. METHODS: In a malformations surveillance program, the frequency in racial groups, sex ratio and the frequency of other anomalies can be established. RESULTS: Five hundred forty-five affected infants were identified from 1972 to 2012 in the surveillance of 289,365 liveborn and stillborn infants and elective terminations because of fetal anomalies detected prenatally. Postaxial polydactyly, type B was an isolated anomaly in 95% of the affected newborns. There were more affected males than females. Black infants were affected more often than White infants: 0.91/100 vs. 0.035/100 infants. The dangling extra digit was much more common in the hands than in the feet. CONCLUSIONS: Postaxial polydactyly, type B is almost always an isolated, mild malformation with no medical significance. Postaxial polydactyly, types B and A occurred in several infants, suggesting that either the underlying mutation(s) can cause both types of postaxial polydactyly or that some affected infants have more than one mutation. Autosomal dominant inheritance with variable expressivity is postulated.

A retrospective study of radiographic abnormalities in the repositories for Thoroughbreds at yearling sales in Japan.

This study aimed to evaluate whether radiographic abnormalities at yearling sales were associated with the failure to start racing at 2-3 years of age. Radiographic abnormalities in the carpal (n=852), tarsal (n=976), metacarpophalangeal (n=1,055), and metatarsophalangeal joints (n=1,031) from 1,082 horses, recorded at yearling sale, were reviewed. Eighty-two horses (7.6%) failed to start racing. Radiographic abnormalities such as wedged or collapsed tarsal bones, irregular lucency of a sagittal ridge at the distal aspect of the distal third metatarsal bone, and proximal dorsal fragmentation of the first phalanx in metatarsophalangeal joints were associated with failure to start racing in these horses. In the follow-up survey of 12 horses with one or more these radiographic abnormalities, the horses failed to start racing due to reasons unrelated to these radiographic abnormalities such as pelvic fractures (2 horses), fracture of a distal phalanx (1 horse), cervical stenotic myelopathy and proximal sesamoid fracture (1 horse), superficial digital flexor tendonitis (2 horses), laryngeal hemiplegia (1 horse), economic problems (2 horses) and unknown causes (3 horses). Although radiographic abnormalities at yearling sales can be associated with failure to start racing at 2-3 years of age, these radiographically detected abnormalities might not necessarily cause that failure.

Evaluation of the first web-space narrowing in congenital anomalies with Z-deformity.

BACKGROUND: In patients with congenital anomalies of the thumb, the metacarpophalangeal joint often undergoes radial deviation to compensate for a narrow first web space. In this study, we evaluated the relationship between the thumb and index finger in patients with arthrogryposis multiplex congenita (AMC), by measuring the thumb-index angle on radiographs taken while the patient held a styrene foam cone. METHODS: Investigation was carried out on nine patients (13 cases: four bilateral and five unilateral) with AMC. The average patient age was 4.4 years (range, 2.6-7.2 years). Overhead radiographs were obtained while the patient held a styrene foam cone. The radiographs were used to evaluate the apparent thumb-index web angle, the thumb to index finger metacarpal angle (1-2MCA), and the first metacarpophalangeal angle (1MPA). RESULTS: In the five unilateral cases, significant differences were seen on the affected versus unaffected sides in 1-2MCA and 1MPA. All 13 cases were treated surgically, and significant differences were observed between the groups before surgery and 2 years after surgery in the 1-2MCA and 1MPA. In addition, the mean postoperative 1MPA in the bilateral cases was significantly smaller compared with the unilateral cases. CONCLUSIONS: This radiographic technique enabled us to evaluate the severity of the thumb-index web-space narrowing and the radial instability of the thumb metacarpophalangeal joint in patients with AMC. The bilateral cases tended to show better rehabilitative improvement compared with the unilateral ones, probably because the latter patients could use their unaffected hand.

Superimposed electrical stimulation improves mobility of pre-stiff thumbs after ulnar collateral ligament injury of the metacarpophalangeal joint: a randomized study.

OBJECTIVE: This study aimed at testing the ability of the superimposed electrical stimulation technique to restore the mobility of pre-stiff thumbs after operative repair for rupture of the ulnar collateral ligament. MATERIAL AND METHODS: Eight patients demonstrating a pre-stiff metacarpophalangeal joint were involved in two rehabilitation sessions of a counterbalanced design. In the voluntary contraction session, they performed 20min of repeated active flexions of the impaired metacarpophalangeal joint. In the superimposed electrical stimulation session, they performed 20min of percutaneous neuromuscular electrical stimulations which were superimposed to voluntary flexion. RESULTS: Mean active range of motion improvement from pre- to post-session was significantly greater in the superimposed electrical stimulation condition compared to the voluntary contraction condition (11±5 deg versus 3±4 deg; P<0.01). CONCLUSION: Superimposing electrical stimulation to voluntary contractions is an efficient technique to improve active range of motion of the pre-stiff metacarpophalangeal joint of the thumb.

Metacarpophalangeal joint locking in 3 family members with brachymesophalangy: case report.

We report painful locking of the metacarpophalangeal joint in a man, his mother, and his sister, all of whom have brachymesophalangy. Surgical excision of loose osteocartilaginous fragments relieved their symptoms. The fragments appeared to be unfused ossicles of the metacarpal head, unique to patients with brachymesophalangy.

The prevalence of the sesamoid bones of the hand: a systematic review and meta-analysis.

The literature contains various estimates of the prevalence and distribution of the sesamoid bones in the hands. The aims of this systematic review are to provide a better estimate of the frequency of hand sesamoids and its association with variables such as ancestry, gender, and side. Nineteen studies met the inclusion criteria. The pooled rates of the sensitive meta-analyses from large-sample studies in adults showed: (a) true overall rates of 99.9% for the metacarpophalangeal (MCP) joint of the thumb (MCP-I), 53% for the interphalangeal joint (IP-I), 43.4% for the MCP of the index (MCP-II), 1.47% for the MCP of the medius finger (MCP-III), 0.6% for the MCP of the ring finger (MCP-IV), and 67.7% for the MCP of the auricular finger (MCP-V); (b) true radiological rates of 99.9% for the radial thumb sesamoid, 99.6% for the ulnar thumb sesamoid, 47.8% for IP-I, 40% for MCP-II, 1.3% for MCP-III, 0.8% for MCP-VI, and 62.8% for MCP-V. Black, Middle Eastern, and European ancestries conferred significantly higher sesamoid frequencies at IP-I, MCP-II, and MCP-V, respectively. There was a significant association with female gender at MCP-II, MCP-IV, and MCP-V, with ORs of 1.53, 4, and 1.3, respectively, and a nonsignificant "female" trend for the other locations. There was no significant association with hand side. The pooled rates of hand sesamoids in children aged 10-17 years were 92.7, 42.2, 33.8, 0.5, 0.3, and 36.5% for MCP-I, IP-I, MCP-II, MCP-III, MCP-IV, and MCP-V, respectively. The findings of this evidence-based anatomical review provide quantitative evidence that the incidence of sesamoid bones in human hands depends on genetic rather than functional factors.

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.

Arthrography in thumb polydactyly with bifurcation at the interphalangeal or metacarpophalangeal joints provides practical information at surgery.

We carried out arthrography in 19 thumbs of 18 patients in whom duplication was observed at the interphalangeal (Wassel type II) or metacarpophalangeal (Wassel type IV) joints on radiographs. The average age at surgery was 12.3 months and average duration of post-surgical follow-up was 21.3 months. Based on the arthrographic findings, the types of cartilaginous connections were subdivided into five groups. In group 1, there was a cartilaginous connection at the base of duplicated phalanges. In group 2, there was a cartilaginous connection of the radial digit between the distal and proximal phalanges, or between the proximal phalanx and metacarpal. In group 3, the phalanges separated at a common joint without any cartilaginous connection. In group 4, the radial digit demonstrated fibrous attachment to the capsule without any joint formation. In group 5, each joint was completely separated without any cartilaginous connection. These arthrographic findings could not be detected on radiographs. Different surgical procedures were carried out according to the form of cartilaginous connection.

Correction of Wassel type IV thumb duplication with zigzag deformity: results of a new method of flexor pollicis longus tendon relocation.

The purpose of this study is to present a treatment algorithm and a method of flexor pollicis longus tendon relocation for Wassel type IV thumb duplication with zigzag deformity. Forty-two thumbs in 42 patients were included in this study and the mean follow-up was 4 years. In addition to excision of the extra digit, tendon relocation and metacarpal and/or proximal phalangeal osteotomy were carried out, based on the degree of angulation at the metacarpophalangeal and interphalangeal joints. Tendon relocation was achieved using our pull-out suture technique. The results were assessed using an evaluation form for thumb polydactyly provided by the Japanese Society for Surgery of the Hand. At the time of latest clinical contact, eight cases were rated good, 31 cases fair, and three cases poor. It is useful to decide surgical procedures according to the degree of angulation of the metacarpophalangeal and interphalangeal joints and flexor pollicis longus tendon relocation is important to prevent malalignment at the interphalangeal joint.

Hypothesis: terminal transverse limb defects with "nubbins" represent a regenerative process during limb development in human fetuses.

BACKGROUND: Terminal transverse limb defects with nubbins occur in one arm at one of several levels (distal humerus, proximal forearm, wrist, and at the metacarpal-phalangeal joint in the hand). The associated nubbins contain osteocartilaginous tissue and small nails and are not associated with evidence of amnion disruption. METHODS: We present affected newborn infants whose terminal transverse limb defects are at one of these three levels: proximal forearm, elbow, or metacarpal-phalangeal joint. RESULTS: We hypothesize that the presence of residual digit-like structures reflects a regenerative process that has occurred during limb development in these infants. DISCUSSION: Only limited regeneration of digit-like structures can occur in the human fetus.

Type IV-D thumb duplication: A new reconstruction method.

INTRODUCTION: In the Wassel type IV classification category, the thumb is duplicated from the metacarpophalangeal joint; this abnormality accounts for approximately 50% of the cases of thumb duplication. Type IV is divided into four subtypes in which the IV-D type, or convergent (9% of cases), is the most complex form because both thumbs are hypoplastic with a divergent metacarpophalangeal joint and a convergent interphalangeal joint. Reconstruction is prone to axis deformity and ligament laxity, whereas the Bilhaut-Cloquet technique's main pitfall is nail dystrophy. We propose a modified Bilhaut-Cloquet procedure to avoid these complications. MATERIAL AND METHOD: Four males (mean age: 11 months; range: 10-12 months) with IV-D thumb duplication were operated on using a modified Bilhaut-Cloquet procedure. SURGICAL TECHNIQUE: The skin is preliminarily marked, taking into account the excision of the central skin and the more hypoplastic nail of the two (most often the radial nail). Both proximal phalanges are split longitudinally and the central halves discarded. An oblique osteotomy is performed at the base of the distal phalanx of the ulnar thumb duplicate (the less hypoplastic) and the radial wedge is excised. The same osteotomy is applied to the distal phalanx of the radial thumb duplicate, but the radial wedge is preserved. The proximal phalanx and the bases of the distal phalanx are joined by bone suture. Axis correction and ligament stability are thus achieved without nail surgery. RESULTS: The patients were examined with a mean 24 months of follow-up (range: 12-36 months). The result was good in all four cases according to the Horii score. DISCUSSION AND CONCLUSION: This procedure combines an excision of the central part of the proximal phalanx and partial excision of the base of the distal phalanx. It provides axis correction and stabilization of the interphalangeal joint while avoiding subsequent nail dystrophy because a single nail is preserved. Preliminary results are encouraging: no axis deformity, instability or nail dystrophy has been noted. Nonetheless, the long-term results need to be evaluated. LEVEL OF EVIDENCE: IV retrospective study.

Evaluation of function and appearance of adults with untreated triphalangeal thumbs.

PURPOSE: Triphalangeal thumb is a congenital malformation characterized by an additional phalanx of the thumb. Although surgical treatment of this condition is common practice, in the past this was not generally advised. Therefore, a population with an untreated triphalangeal thumb is still present. The purpose of this study is to compare function and appearance of adults with an untreated triphalangeal thumb to a normal population. METHODS: Twelve adults with 23 hands with an untreated triphalangeal thumb, unilateral or bilateral, were examined using objective measurements (thumb movement, joint instability, pain, and strength) and subjective measurements (visual analog scale; Disabilities of the Arm, Shoulder, and Hand questionnaire; and Short Form 36 health survey). RESULTS: Objective measurements showed no limitations in range of motion or in grip and pinch strength. No joint instability was found in the interphalangeal joints. Five thumbs had instability in the metacarpophalangeal joint. Strength of the thumb in anteposition was diminished to 64% compared to a normal population. Opposition was diminished to 62%, and metacarpophalangeal joint flexion strength was diminished to 61%. The patients scored lower compared to a normal population for the domain of social functioning in the Medical Outcome Study 36-item short form health survey; the Disabilities of the Arm, Shoulder, and Hand questionnaire showed no differences. Visual analog scale scores for appearance of the thumb were scored low (2.2 of 10) by the adults, in contrast to visual analog scale scores for function (7.7). CONCLUSIONS: The examined group of adults with an untreated triphalangeal thumb had adequate thumb movement. Thumb strength was diminished for all specific thumb functions (anteposition, opposition, and thumb flexion), as low as 55%, compared to normal controls. Self-rated scores indicate that patients perceived their functionality as good. The appearance, however, was rated much lower, implying a dislike of the thumb by the patients. This indicates that the main impact of an untreated triphalangeal thumb in daily functioning might not be the diminished function but rather the dissimilar appearance. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Locking of the metacarpophalangeal joint as a result of the shape of the metacarpal head in achondroplasia.

A 21 year-old woman with achondroplasia exhibited locking of the metacarpophalangeal joint of the right index finger which required surgery to release the joint. Locking was confirmed to be due to entrapment of the fan-like part of the radial collateral ligament on a metacarpal head prominence arising from epiphysial thickening.

Surgical correction of metacarpophalangeal valgus deformity in an alpaca.

A 1-month-old alpaca cria presented with a 13 degree valgus deformity of the left metacarpophalangeal joint. The angular limb deformity was centered on the distal metacarpal physes. Transphyseal bridging of the physes was recommended. Two 2.7 mm cortical bone screws were placed either side of the distal metacarpal physes and a figure of eight wire was placed medially around the screw heads. The screws extended through the medial metacarpus into the axial cortex of the lateral metacarpus. Seven weeks after surgery the limb was straight and the screws and wire were removed. Transphyseal bridging of the distal metacarpal physes can be effectively used for the treatment of metacarpophalangeal valgus in crias with open physes.

Soft tissue reconstruction for type IV-D duplicated thumb: a new surgical technique.

Type IV-D duplicated thumb has the most complex anomalies and difficulties for treatment among polydatyly. Double osteotomy is usually recommended to gain the best cosmetic and functional outcome. However, 4 cases of type IV-D duplicated thumb were treated only by soft tissue procedure in this study. At operation, a conjoined A2 pulley was routinely identified, and the flexor pollicis longus (FPL) was found bifurcated distal to the conjoined pulley in every of these cases. Instead of double osteotomy, a soft tissue procedure that included centralization of FPL and A2 pulley reconstruction was pursued to correct these special anomalies. The overall clinical results were evaluated by a modification of the Tada scoring system based on the range of motion, joint stability, alignment of the remaining thumb, and subjective opinion regarding the reconstructed thumb after an average follow-up of 3.3 years (range, 2.5-4.7 years). According to the scoring system, the results were rated as good in 3 cases and fair in 1 case. From the results, the A2 pulley reconstruction and FPL centralization could prove to be an effective method for the treatment of type IV-D duplicated thumb and could efficiently avoid the residual angular deformities. Therefore, we recommend this soft tissue procedure as an alternative surgical technique to the double-osteotomy procedure for treating a type IV-D duplicated thumb in a very young child, whose bone is still not mature enough for holding the fixing pins.