A retrospective study was performed in 18 patients
with achondroplasia, who underwent bilateral humeral lengthening
between 2001 and 2013, using monorail external fixators. The mean
age was ten years (six to 15) and the mean follow-up was 40 months
(12 to 104). The mean disabilities of the arm, shoulder and hand (DASH) score
fell from 32.3 (20 to 40)
pre-operatively to 9.4 (6 to 14) post-operatively (p = 0.037). A
mean lengthening of 60% (40% to 95%) was required to reach the goal
of independent perineal hygiene. One patient developed early consolidation,
and fractures occurred in the regenerate bone of four humeri in
three patients. There were three transient radial nerve palsies. Humeral lengthening increases the independence of people with
achondroplasia and is not just a cosmetic procedure. Cite this article:
The use of recombinant human bone morphogenetic protein-2 (rhBMP-2) for the treatment of congenital pseudarthrosis of the tibia has been investigated in only one previous study, with promising results. The aim of this study was to determine whether rhBMP-2 might improve the outcome of this disorder. We reviewed the medical records of five patients with a mean age of 7.4 years (2.3 to 21) with congenital pseudarthrosis of the tibia who had been treated with rhBMP-2 and intramedullary rodding. Ilizarov external fixation was also used in four of these patients. Radiological union of the pseudarthrosis was evident in all of them at a mean of 3.5 months (3.2 to 4) post-operatively. The Ilizarov device was removed after a mean of 4.2 months (3.0 to 5.3). These results indicate that treatment of congenital pseudarthrosis of the tibia using rhBMP-2 in combination with intramedullary stabilisation and Ilizarov external fixation may improve the initial rate of union and reduce the time to union. Further studies with more patients and longer follow-up are necessary to determine whether this surgial procedure may significantly enhance the outcome of congenital pseudarthrosis of the tibia, considering the refracture rate (two of five patients) in this small case series.
We present a retrospective study of 27 patients treated by callus distraction using a
Deformity of the forearm due to growth disturbance of the ulna occurs in a number of conditions such as ulnar deficiency, multiple exostoses, and neurofibromatosis. We report a previously unrecognised form, caused by focal cortical indentation. We have treated five children with this condition, three girls and two boys; the mean age at presentation was 5 years (2 to 8). The deformity was first recognised about the age of two years, and progressed gradually. The radiological findings were the same in all cases. The focal cortical indentation was seen at the distal end of the ulna with anteromedial bowing and dysplasia. The radial head was dislocated posterolaterally. In one patient the histological findings at the site of indentation were of a fold of tissue resembling periosteum, which interfered with enchondral ossification. Treatment by ulnar lengthening using an external fixator and osteotomy which corrected both the ulnar deformity and reduced the dislocated radial head in two cases gave the best results.