Maria A Stefanou, Dimitrios Pasparakis, Chrisa Tzoumaka-
Bakoula, Nikolaos Papandreou, 2
nd
Orthopaedic Department
‘Panayiotis & Aglaia Kyriakou’ Children’s Hospital, Athens, Greece
Dimitrios Mastrokalos, Panayiotis J Papagelopoulos, 1
st
Ortho-
paedic Department, University General Hospital ‘Attikon’, Athens,
Greece
Correspondence to: Maria A. Stefanou, MD, MS, 2
nd
Orthopae-
dic Department ‘Panayiotis & Aglaia Kyriakou’ Children’s Hospital,
9, Gelonos st, 11521, Athens, Greece
Email: maria_stefanou@hotmail.com
Telephone: +30-6937-888-030
Received: September 30, 2014 Revised: October 25, 2014
Accepted: October 30, 2014
Published online: December 23, 2014
ABSTRACT
AIM: The effectiveness of the Ilizarov ring fixator in correcting
achondroplasia’s lower limb deformities is known. However, whether
the long term postoperative result is comparable to normal population
standards, has not been analyzed.
MATERIALS AND METHODS: Nineteen (19) achondroplastic
patients, 12 males and 7 females, aged 19-38 years, who at the age
of 9-19 years, had undergone both tibia and femur lengthening,
using the Ilizarov method, were included in the study. Patients were
evaluated 5-19 years after their last surgery, using standardized long
lower limb anteroposterior and lateral standing radiographs. Tibial and
femoral lengthening gain was measured. A comparison between the
achondroplastic patients at follow up and comparative radiographic
parameters of the normal population was made, concerning– at the
frontal plane- LPFA (lateral proximal femoral angle), LDFA (lateral
distal femoral angle), MPTA (medial proximal tibial angle), LDTA
(lateral distal tibial angle) and MAD (mechanical axis deviation) and
– at the sagittal plane- PDFA (posterior distal femoral angle), PPTA
(posterior proximal tibial angle) and ADTA (anterior distal tibial angle).
RESULTS: Mean angle values at follow up were: LPFA 118, LDFA
95.5, MPTA 87.8, LDTA 93, PDFA 85.1, PPTA 84, ADTA 88.3
while MAD mean value was 28. LPFA, LDFA, LDTA, PPTA, ADTA
and MAD values were statistically significantly different (p<0.001)
between achondroplastic patients and normal population.
CONCLUSIONS: The use of the Ilizarov method for lower
limb deformity correction, in achondroplastic patients, provides a
functional length gain. It substantially corrects the three-dimensional
deformities of the disease but, it does not restore the radiological
image within normal standards. Level of Evidence Level II,
Retrospective Prognostic study.
© 2014 ACT. All rights reserved.
Key words: Achondroplasia; Lower limb lengthening; Deformity;
Ilizarov
Stefanou MA, Pasparakis D, Mastrokalos D, Tzoumaka-Bakoula
C, Papandreou N, Papagelopoulos PJ. Radiographic Assessment of
Lower Limb Lengthening in Achondroplastic Patients, Using the Il-
izarov Frame: A 5-19 Year Follow up Study. International Journal of
Orthopaedics 2014; 1(4): 140-145 Available from: URL: http://www.
ghrnet.org/index.php/ijo/article/view/975
INTRODUCTION
Achondroplasia, the most common form of dwarfism, is characterized
by defective endochondral ossification, due to defect in the gene-
located at the end of the short arm of chromosome 4- that encodes for
fibroblast growth factor receptor-3
[1]
. The disease’s prevalence in the
US is 0.36-0.6/10.000 live births
[2]
and no satisfactory pharmaceutical
causal form of treatment has been found.
The patients are characterized by rhizomelic short-limbed stature,
lumbar lordosis, forehead prominence and a low nasal bridge
[3]
.
Achondroplastic patients have a normal life expectancy
[4]
.
Radiographic Assessment of Lower Limb Lengthening in
Achondroplastic Patients, Using the Ilizarov Frame: A 5-19
Year Follow up Study
Maria A Stefanou, MD, MS, Dimitrios Pasparakis, MD, PhD, Dimitrios Mastrokalos, MD, PhD, Chrisa Tzoumaka-
Bakoula, MD, PhD, Nikolaos Papandreou, MD, PhD, Panayiotis J Papagelopoulos, MD, PhD
140
Int Journal of Orthopaedics 2014 December 23 1(4): 140-145
ISSN 2311-5106 (Print), ISSN 2313-1462 (Online)
Online Submissions: http://www.ghrnet.org/index./ijo/
doi:10.6051/j.issn.2311-5106.2014.01.33
© 2014 ACT. All rights reserved.
International Journal of Orthopaedics
ORIGINAL ARTICLE