Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2013 Jun 15;6(2):205-13.
Epub 2013 Jun 25.

Surgical treatment in Osteogenesis Imperfecta - 10 years experience

Affiliations

Surgical treatment in Osteogenesis Imperfecta - 10 years experience

I Georgescu et al. J Med Life. .

Abstract

Introduction: Osteogenesis imperfecta (OI) is a very rare disease compared to other afflictions, running the risk of social isolation for children and their parents, due to the problems specific to the disease. All the social, psychological and physical disadvantages must be removed or at least mitigated, all within the society's limited resources. In Romania, this situation has led in the last couple of years to the selection of a number of extremely severe cases, which could not be solved by orthopedic and classic surgical treatment methods. These patients exhibit gracile long bones, which are distorted, often with cystic degeneration at the level of the extremities, pseudarthroses, limb length discrepancies, most of them being unable to walk, being condemned to sitting in a wheelchair.

Aim: This paper deals with the experience of the Orthopedics Department of "Maria Sklodowska Curie" Clinical Emergency Hospital for Children, in Bucharest, in the field of surgical treatment for moderate and severe forms of OI, within the time frame of May 2002-May 2012. For the first time in Romania, on May 20, 2002, the team led by Professor Gh. Burnei, MD, has implanted telescopic rods in the femur and tibia of a patient with OI. One of the most important themes, of great interest in the orthopedic surgery, is the osteoarticular regularization and reconstruction in severe forms of OI, which should allow the patients to stand and walk. These cases are a challenge for the surgeon, who is in the position of applying new, complex procedures, or perfecting, modifying and adapting techniques that have already been established. The aim of the surgical treatment is the increase of the quality of life of these children and adolescents and of their social integration.

Methods and results: In the above-mentioned period, from the OI patients who are in the evidence of our clinic, 32 were operated on, totaling 81 surgeries. Out of these, 28 patients, aged 2-27 years, have benefited from reconstructive surgery of the pelvic limbs. Sofield-Millar osteotomies were practiced and 69 Sheffield telescopic rods were implanted in 25 patients and 43 surgeries. The coxa vara / valga correction using the Sheffield rod was applied in 6 patients and 8 hips, respectively. Circular or monoplane external fixators were used in 7 patients for the correction of deformities, lengthening and arthrodiastasis. 9 patients have benefited from various forms of bone transplant: pedicled grafts, auto- and/or allografts. An original bone reconstruction procedure is currently being studied and will be useful in the treatment of large bone defects and the thickening of the gracile diaphyses, which consists in practice of a massive contribution of free bone grafts, auto- and/or allogenic, bone substitutes and, in selected cases, periosteal substitutes, in a composite stratified construction. Postoperatively, 15 patients are able to walk while being supported by crutches or walking frames, 5 patients walk independently and 8 are still wheelchair-bound. It is important to mention that 8 children who were preoperatively dependant on the wheelchair are now walking!

Discussion: The surgical treatment in severe forms of OI must be adapted to each case. No matter the surgical technique used, well known or innovative, it is convenient if it restores the ability to walk of a youngster who has been forced to use a wheelchair for almost 20 years and who has suffered dozens of unsuccessful surgeries. The current paper mainly describes the difficulties the surgeon has to deal with while treating the severe, neglected cases of OI, sometimes incorrectly cared for and labeled as inoperable.

Keywords: bone grafts; coxa vara; fractures; osteogenesis imperfecta; pseudarthrosis.

PubMed Disclaimer

Figures

Fig. 1
Fig. 1
Congenita A type of osteogenesis imperfecta in a 2-month-old child
Fig. 2
Fig. 2
A 13-year-old patient, displaying an extremely severe form of OI, with monstrous deformities of the pelvic limbs, who has never walked. For each pelvic limb, monoplane external fixators have been used in order to elongate the soft tissues and the bone, thicken the diaphysis by lateral traction on olive wires and realign the segments. All 4 segments have been splinted with Sheffield rods and rib allografts were needed around the extremely thin diaphysis. The reconstructive surgery has restored orthostatic position with the help of bilateral knee-ankle-foot orthotics.
Fig. 3
Fig. 3
Coxa vara correction on Sheffield rod
Fig. 4
Fig. 4
Tibial double focus lengthening - 7 cm were gained (radiological images from the process of progressive lengthening)
Fig. 5
Fig. 5
Loose tibial hypotrophic pseudarthrosis in a 21-year-old patient with OI, who has not walked since she was 2 years old. B. Fibular pedicled autogenous graft, by open lateral transport, after the surgical treatment of the pseudarthrosis using a Sheffield rod. C. Radiological aspect at 1-year postoperatively. At present, the patient walks with crutches.
Fig. 6
Fig. 6
Intraoperative and postoperative radiological aspect of the rib allografts around a femoral diaphysis, connected with slowly absorbable thick sutures
Fig. 7
Fig. 7
A. Double pseudarthrosis of the right femur in a 27-year-old patient, operated when she was 19 years and 10 months old, by using the Sofield-Millar technique. The Sheffield rod broke 6 years after surgery. B. Massive bone grafting of the right femur using a composite construction
Fig. 8
Fig. 8
Burnei´s procedure
Fig. 9
Fig. 9
Possible complications after Sheffield telescopic rodding: bending, disengagement, fracture of the rod, migration

Similar articles

Cited by

References

    1. Zeitlin L, Fassier F. Modern approach to children with osteogenesis imperfecta. J Pediatr Orthop B. 2003;26:148–149. - PubMed
    1. Plotkin H. Two Questions about Osteogenesis Imperfecta. J Pediatr Orthop. 2006;26:148–149. - PubMed
    1. Kocher MS, Shapiro F. Osteogenesis imperfecta. J Am Acad Orthop Surg. 1998;6:225–236. - PubMed
    1. Burnei G, Vlad C. Osoegenesis Imperfecta: Diagnosis and Treatment. J Am Acad Orthop Surgery. 2008;16:356–366. - PubMed
    1. Shapiro F. Consequences of an osteogenesis imperfecta diagnosis for survival and ambulation. J Pediatr Orthop. 1985;5:456–462. - PubMed

Publication types

LinkOut - more resources