The etiology of short stature affects the clinical outcome of lower limb lengthening using external fixation A systematic review of 18 trials involving 547 patients

Acta Orthopaedica (Impact Factor: 2.77). 04/2014; 85(2):181-6. DOI: 10.3109/17453674.2014.899856
Source: PubMed


Background and purpose
Distraction osteogenesis (DO) has been used to gain height in short statured individuals. However, there have been no studies comparing the clinical outcome of limb lengthening based on the etiology of the short stature. We assessed whether different underlying diagnoses are associated with varied clinical outcomes in these patients.

We performed a systematic review of the literature pertaining to lower limb lengthening using external fixation for short stature. Clinical outcomes including amount of lengthening, healing index (HI), and complications based on the underlying diagnosis for the short stature were documented.

18 clinical studies were included, with 547 patients who underwent 1,581 lower limb segment lengthening procedures. Mean follow-up was 4.3 years. The average age at lengthening was less for individuals with achondroplasia/hypochondroplasia (A/H) (14.5 years) than for those with Turner’s syndrome (TS) (18.2 years) or with constitutional short stature (CSS) (21.7 years). Mean height gained was greater in patients with A/H (9.5 cm) than in those with TS (7.7 cm) or CSS (6.1 cm) group. The HI was better in A/H (30.8 days/cm) and CSS (32 days/cm) than in TS (45.1 days/cm). The reported complication rate per segment was lower for A/H (0.68) and TS (0.71) than for CSS (1.06).

Patients with A/H tolerated larger amounts of lengthening with fewer complications than those with other diagnoses.

16 Reads
  • [Show abstract] [Hide abstract]
    ABSTRACT: We review recent understanding of the pathophysiology, molecular biology, and management of Turner syndrome. Sophisticated genetic techniques are able to detect mosaicism in one-third of individuals previously thought to have monosomy X. Prenatal detection using maternal blood should permit noninvasive detection of most fetuses with an X chromosome abnormality. Disproportionate growth with short limbs has been documented in this condition, and a target gene of short stature homeobox, connective tissue growth factor (Ctgf), has been described. Liver disease is more common in Turner syndrome than previously recognized. Most girls have gonadal failure. Spontaneous puberty and menarche is more commonly seen in girls with XX mosaicism. Low-dose estrogen replacement therapy may be given early to induce a more normal onset and tempo of puberty. Oocyte donation for assisted reproduction carries a substantial risk, particularly if the woman has known cardiac or aortic disease. Neurodevelopmental differences in Turner syndrome are beginning to be correlated with differences in brain anatomy. An increased understanding of the molecular basis for aspects of this disorder is now developing. In addition, a renewed focus on health maintenance through the life span should provide better general and targeted healthcare for these girls and women.
    Current opinion in endocrinology, diabetes, and obesity 12/2014; 22(1). DOI:10.1097/MED.0000000000000128 · 3.37 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Stature lengthening for short stature, often referred to as cosmetic stature lengthening, is controversial. Previous methods and devices have been fraught with high complications rates. The PRECICE intramedullary lengthening nail offers a superior alternative for these patients due to its minimal incision technique, remote controlled gradual distraction, the rate of which can be accurately modulated and the direction reversed, if necessary. The purpose of this study is to report the results of the PRECICE for short stature lengthening and to compare them to previously reported results with other methods. Another objective of this paper is to discuss the indications for treatment by exploring the motivation, selection, and outcome of such treatment. Fifty-one patients were lengthened for short stature with the PRECICE; 25 with the PRECICE 1 (P1) and 26 with the PRECICE 2 (P2). There were 7 bilateral tibial and 22 bilateral femoral lengthenings in the P1 and 4 bilateral tibial and 25 bilateral femoral lengthenings in the P2 groups. In total there were 58 P1 and 58 P2 bone segments lengthened. Lengthening was up to 6.5 cm for P1 and 8 cm for P2 with an average increase of 5.2 cm for P1 and 6.0 cm for P2. There were 7/58 (12.1%) implant failures for P1 and 1/58 (1.7%) for P2. The P1 failures were due to breakage of the nail through a weld in the nail in 4 cases and the breakage of the mechanism in 3 cases. The P2 failure may be related to too little overlap of the wider nail tube into the distal bone segment combined with potential stress fracture propagation due to small slots at the end of the larger tube of the nail. All nail breakages occurred in patients who did not comply with the weight-bearing restrictions. The new clutched mechanism and 1-piece outer tube construction in the P2, prevented fracture through the nail and mechanism failure in the P2 compared with the P1. Stress propagation by the small slots at the end of the P2 led to redesign with elimination of slots and release of the P2.1. There were 2 cases of suspected fat embolism despite venting and 1 deep vein thrombosis upon stopping anticoagulation, from which the patient recovered without further complication. There was 1 femur fracture through an anteroposterior femoral locking screw and 1 bilateral peroneal nerve stretch injury that fully recovered. All patients consolidated the distraction gap of the femurs and/or tibias without additional surgery. All returned to previous activities including sports. In comparison to previously published methods, the P2 had the lowest complication rate with the best overall reported results.
    Techniques in Orthopaedics 09/2015; 30(3):167-182. DOI:10.1097/BTO.0000000000000140