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Brachydactyly

Brachydactyly is a medical term which literally means "shortness of the fingers and toes" (digits). The shortness is relative to the length of other long bones and other parts of the body. Brachydactyly is an inherited, usually dominant trait. It most often occurs as an isolated physical difference, but can also occur with other anomalies as part of many congenital syndromes. more...

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Nomograms for normal values of finger length as a ratio to other body measurements have been published. In clinical genetics the most commonly used index of digit length is the ratio of the 3rd (middle) finger in cm to the hand length in cm. Both are measured in an open hand from the fingertip to the principal creases where the finger joins the palm and where the palm joins the wrist. A nomogram can be found in the Appendix of Jones, ed. Smith's Recognizable Patterns of Human Malformation, 5th edition, Philadelphia: Saunders (1997).

Brachydactyly Type A1

Brachydactyly type A1 is an autosomal dominant inherited disease. Features include: -

  • Brachydactyly
  • Short or absent phalanges
  • Extra carpal bones
  • Hypoplastic or absent ulna
  • Short metacarpal bones

Brachydactyly Type A2

Type A2 is a very rare form of brachydactyly. The phalanges of the index fingers and second toes are shortened.

Other syndromes

In the above brachydactyly syndromes, short digits are the most prominent of the anomalies, but in many other syndromes (Down's syndrome, Rubinstein-Taybi syndrome, etc), brachydactyly is a minor feature compared to the other anomalies or problems comprising the syndrome.

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GENERAL HAND DISORDERS 1
From Journal of Bone and Joint Surgery, 1/1/04 by Ghandour, A

O1301 CONDYLAR FRACTURES OFTHE PROXIMAL AND MIDDLE PHALANGES

A. Ghandour, A. Rogers, DJ. Shewring.

University Hospital of Wales, Cardiff, United Kingdom

Aims: Thirty-three patients with condylar fractures of the middle and proximal phalanges were treated with internal fixation using self-tapping titanium lag screws. The results were studied prospectively and prognostic factors identilied. Methods: The age range was 14-45 (mean 26 years). Five patients presented at more than five weeks post injury. Four patients had bicondylar fractures. The surgical technique, which utilises a lateral approach, is outlined. All surgery was performed by the senior author (DS), semi-electively. within five days of presentation. Patients were seen in the 4th post-operative day for mobilisation and protective splintage. Results: The results were satisfactory in the majority of cases. 21 patients had achieved a full range of movement when reviewed at six weeks and further eight at 12 weeks. The remainder were left with a flexion contracture of 10-35 degrees (mean 26). All patients achieved full flexion. All fractures healed and there was no loss of fixation. Conclusions: Internal fixation using a single lag screw through a lateral approach restores joint congruity, facilitates union and provides fixation stable enough to allow early mobilisation. Bicondylar and proximal phalangeal fractures had a poorer prognosis. Fractures presenting even at eight weeks can be taken down and reduced. Delay in fixation does not appear to influence the long-term outcome.

OO02 FUNCTIONAL OUTCOME AFTER SILASTIC INTERPOSITION ARTHROPLASTY OF METACARPOPHALANGEAL JOINTS IN RHEUMATOID ARTHRITIS

Timo Parkkila, Eero A. Belt. Markku Hakala, Hannu Kautiainen, Juhana Leppilahti

Aims: The aim of the present study was to compare the functional outcome between silastic Swanson and Sutler implants in metacarpophalangeal joints in a prospective and randomised of study of patients with rheumatoid arthritis. Methods: The study was prospective and randomised. Thematerial comprised 53 patients (6 men, 47 women) and 58 hands; a total of 89 Swanson and 126 Sutler implants were installed. The mean follow-up time was 57 (40-80) and 55 (36-79) months, respeclively. Results: Aclive exlension was correcled similarly in bolh groups. In lhc Swanson group from median [interquartile rate (IQR)] 22° (8, 44) to 12° (0.20). and in the Sutler group from 19° (7. 37) to 16 (S. 25). The median aclive flexion decreased less in lhe Suiter group from 75° (69. 84) to 64° (53, 72) vs. Swanson from 83° (77, 90) to 58° (48, 64) during lhe follow-up, and wilh that respecl the difference belween lhe groups was statistically significant (p=0.01). Statislically significanl difference (p-0.03) was delected in correction of ulnar deviation only in ring finger: in lhe Swanson group from median 25°(6°, 34°) ulnar devialion to 1°(2°radial, 9°) radial deviation and in the Sutler group from 14°(7°, 28°) to 5°(0°. 13∞) respectively. Conclusion: It appears that the Sutler implant yields at least as good functional results in the MCP replacement as the Swanson prosthesis.

O1303 LENGTHENING OF DIGITS-A USEFUL ALTERNATIVE IN HAND RECONSTRUCTION

Gopalkrishna Verma, A. Mehta, R. Prabhoo, B.C. Kanaji.B.B. Joshi.

Jess Research and Development Centre, Mumbai (India)

Aims: In reconstruction of hrachydactyly. either post-traumatic or congenital, we present our method of skeletal lengthening by distraction. Methods: We treated 32 short digits in 15 patients. Posttraumatic: lOpatients and Congenital shortening: 5patients (Average age Syears (3.4 - 7.7 years) in congenital group). For traumatic amputation duration between injury and surgery: average 14weeks ( 10-19.Sweeks). Concerned phalanx exposed through dorsal approach. Transfixing k-wires passed perpendicular to phalangeal shaft above and below proposed osteotomy and connected on either side to a distractor. Latency period: 5days. Distraction rate: 0.8 mm/day. Serial x-rays at 3weekly intervals. In opatients with deficient new bone, bone grafting was done using iliac crest graft. Fixator left in situ till radiological evidence of bony healing than wires removed under local anaesthesia and patients were started on vigorous physiotherapy for gaining range of movements. Spatients required deepening of the web space in addition to above-mentioned procedures. This procedure was required in cases having more than one short digit. Results: Average length gained: 18mm. Average duration of distraction: 42.26days. Fixator in situ: average 92days (80-136days). Initial stiffness of digits was seen in 50% of cases, which eventually responded to physiotherapy. & all patients had an excellent range of motion. Conclusions: This method provides patients with good function and cosmetic hand. Any surgeon not familiar with microvascular surgery can use this technique.

O1304 DIGITAL RAY RECONSTRUCTION THROUGH DISTRACTION OSTEOGENESIS LENGTHENING

Marios D. Vekris.. N.A. Darlis, A.E. Beris, G.I. Mitsionis, N.V. Kordalis, RN. Soucacos.

University of Ioannina, School of Medicine, Department of Orthopaedic Surgery, Ioannina, Greece

Aim: Adequate length is an important prerequisite for a functional digit. Over the last 20 years small external fixators have been developed allowing the principles of distraction osteogenesis to be applied to the small bones of the hand. We present our experience in digital lengthening with the contemporary designs of external fixators. Methods: From 1998 to 2001,20 patients (26 rays) were treated with metacarpal or phalangeal lengthening through distraction osteogenesis using a monolateral frame with two half-pins on each site of the osteotomy. The mean age of the patients was 21 years (6-48) and indications included traumatic amputation in 13 and congenital amputation (transverse deficiency, brachydactyly. constriction band syndrome) in 7. The mean distraction period was 3 weeks and the mean consolidation period 7 weeks. No protective splinting or additional bone grafting was necessary. Results: The distraction callus consolidated in all patients.The mean total length gained was 17.5 mm (68% of the original length). The mean treatment time was 2,8 days for every mm of length gained. One patient suffered angulation at the distraction site and the fixator had to be revised. No infection, fracture or half pin loosening were observed. Conclusions: Callotasis is a reliable technique for digital ray lengthening. Meticulous surgical technique and close observation of the patient during the distraction phase are necessary in order to avoid complications. Over 2 cm of lengthening can be achieved without bone grafting

01305 POLLICISATION BY GRADUAL DISTRACTION

Mehta Amit, G.G. Verma. R. Prabhoo. B.G. Kanaji, B.B. Joshi.

Jess Research and Development Centra. Mnmbai (India)

Aims: To describe a new & safe technique of gradual distraction for pollicisation of index finger, to which the blood supply is doubtful.This procedure was used in cases where conventional methods of pollicisation are not possible or are risky. Methods: This technique has been utilised in 5 cases (2 traumatic loss of thumb at carpo-metacarpal joint, and 3 cases of absence of thumb associated with radial club hand). The age group was 3-18 years. The procedure consisted of first stage, a webplasty between second & third fingers, osteotomy of the base of second metacarpa! and gradual wide abduction of the index linger by distraction. In second stage, the second metacarpal is recessed and rotated, to the position of thumb. At third stage, tendon transfer may be needed for securing the forceful opposition. Results: In all operated 5 cases, desired position of the thumb was obtained along with good function. Conclusion: Gradual distraction for pollicisation may be used in cases where the blood supply of index finger is not based on known specific blood vessel but depends on collateral blood supply. Conventional methods cannot be used or are risky. This technique provides a safe and economical alternative for reconstruction of the thumb, which is a new addition to the armamentarium in thumb reconstruction.

01306 HOMODIGITAL ISLAND FLAPS FOR THE TREATMENT OF FINGERTIP AMPUTATIONS

S.E. Varitimidis. A.H. Zibis, Z. Dailiana, G. Basdekis, K.M. Malizos.

Orthopuedic Dept. University Hospital of Thessaly, Larissa Greece

Introduction: Amputation of the fingertip with loss of the finger pulp, exposed bone and nail bed injury is a common problem, not infrequently neglected. Fingertip reconstruction requires new pulp glabrous skin coverage with sensitivity, buttressed by the nail. The aim of this study is the analysis of our experience from the use of a homodigital, island flap for the reconstruction of amputated fingertips Materials and Methods: tile homodigital island flap was applied in 46 patients (57 fingers), at every level of finger amputation with special indication on fingertip coverage. For the index and the middle fingers, use of the ulnar bundle is preferred. For the thumb, ring and little finger use of the radial neurovascular bundle is preferable. The neurovascular bundle is well dissected from the Hap to the base of the finger. After suturing of the flap the donor area is covered with split thickness skin graft from the hypothenar. The finger is mobilized one week after the procedure. Results: Postoperatively. ROM was normal in 45 lingers, 6 fingers had 100 loss of extension at the DIP and 6 fingers had loss of motion between 100-200 .TPD was 4mm (3-10mm). Three patients had cold intolerance for 8 months. There was no neuroma or sensitive scar formation and no need for a second operation. According to patients cosmesis was acceptable in all fingers. Conclusions: The use of homodigital island flap provides excellent functional reconstruction of the fingertips after a complex tissue loss in Alien III and IV amputations It is a straightforward operation carried out under wrist block with excellent cosmetic results.

Copyright British Editorial Society of Bone & Joint Surgery 2004
Provided by ProQuest Information and Learning Company. All rights Reserved

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