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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.

Read more at Wikipedia.org


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Digital ray reconstruction through distraction osteogenesis lengthening
From Journal of Bone and Joint Surgery, 1/1/03 by D, Vekris Marios

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, 14 patients (15 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 (7-48) and the indications were traumatic amputation in 8 and congenital amputation (transverse deficiency, brachydactyly. constriction hand syndrome) in 6. 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 due to hardware failure and the fixator had to be revised and in another bony prominence was noted necessitating trimming. No infection, fracture or half pin loosening were observed.

Conclusions: Callotasis using contemporary monolateral external fixators is a reliable technique for digital ray lengthening. Meticulous preoperative planning and 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.

Vekris Marios D., Darlis N.A., Beris A.E., Mitsionis G.I., Soucacos P.N.

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

Copyright British Editorial Society of Bone & Joint Surgery 2003
Provided by ProQuest Information and Learning Company. All rights Reserved

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