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:
We present the results of the surgical correction of lower-limb deformities caused by metabolic bone disease. Our series consisted of 17 patients with a diagnosis of hypophosphataemic rickets and two with renal osteodystrophy; their mean age was 25.6 years (14 to 57). In all, 43 lower-limb segments (27 femora and 16 tibiae) were osteotomised and the deformity corrected using a monolateral external fixator. The segment was then stabilised with locked intramedullary nailing. In addition, six femora in three patients were subsequently lengthened by distraction osteogenesis. The mean follow-up was 60 months (18 to 120). The frontal alignment parameters (the mechanical axis deviation, the lateral distal femoral angle and the medial proximal tibial angle) and the sagittal alignment parameters (the posterior distal femoral angle and the posterior proximal tibial angle) improved post-operatively. The external fixator was removed either at the end of surgery or at the end of the lengthening period, allowing for early mobilisation and weight-bearing. We encountered five problems and four obstacles in the programme of treatment. The use of intramedullary nails prevented recurrence of deformity and refracture.
We report the results of using a combination of fixator-assisted nailing with lengthening over an intramedullary nail in patients with tibial deformity and shortening. Between 1997 and 2007, 13 tibiae in nine patients with a mean age of 25.4 years (17 to 34) were treated with a unilateral external fixator for acute correction of deformity, followed by lengthening over an intramedullary nail with a circular external fixator applied at the same operating session. At the end of the distraction period locking screws were inserted through the intramedullary nail and the external fixator was removed. The mean amount of lengthening was 5.9 cm (2 to 8). The mean time of external fixation was 90 days (38 to 265). The mean external fixation index was 15.8 days/cm (8.9 to 33.1) and the mean bone healing index was 38 days/cm (30 to 60). One patient developed an equinus deformity which responded to stretching and bracing. Another developed a drop foot due to a compartment syndrome, which was treated by fasciotomy. It recovered in three months. Two patients required bone grafting for poor callus formation. We conclude that the combination of fixator-assisted nailing with lengthening over an intramedullary nail can reduce the overall external fixation time and prevent fractures and deformity of the regenerated bone.
