The Osteogenesis Imperfecta Health And Social Care Essay

Savas Guner MD1*, Necip Guven MD1, Mehmet Ata Gokalp MD1Abdurrahim Gozen MD1 Seyyid Serif Unsal MD1.

1 Department of Trauma and Orthopedic Surgery, Yuzuncu Yil University Medical School Hospital, Van, Turkey,

Savas Guner MD*: gunersavas@gmail.com

Necip Guven MD: ortodr.nguven@gmail.com

Mehmet Ata Gokalp MD: doktorata@hotmail.com

Abdurrahim Gozen MD: abdurrahimgozen@hotmail.com

Seyyid Şerif Ünsal: seyyid.unsal@hotmail.com

Corresponding author *

Savas Guner MD

E-mail: gunersavas@gmail.com

Address: Yuzuncu Yil Universitesi Tip Fakultesi Ortopedi ve Trv. AD, Universite Kampusu, Van/TURKEY

Tel: +90 533 2433873

Fracture in long bones stabilized by Ender nails in patients with "Osteogenesis Imperfecta"

Abstract

Introduction

Osteogenesis imperfecta is a genetic disorder caused by defective type I collagen synthesis. The purpose of this study was to evaluate the results of ender nailing of the bones of the lower extremities in children and adolescents patients with osteogenesis imperfecta.

Methods

This retrospective study was carried out with the approval of the Local Ethics Committee. We have been treated 11 femoral fracture cases with the Ender nail. Data regarding complications and additional procedures were achieved from the clinical records and radiological review.

Results

Nailing was performed in all of 11 patients. The indications for nailing of the 11 femurs included fracture and deformity. All of the femurs were nailed because of fracture and deformity. Union was achieved in all cases within 6.9 weeks (range: 5–10 weeks). No re-fracture was observed in any case.

Conclusions

This study was primarily designed to evaluate the effectiveness of the treatment of patients with osteogenesis imperfecta using Ender nails. Our findings support the intramuscular application of Ender nail as effective in the treatment of the patient with fracture or deformity caused to osteogenesis imperfecta.

Key words: Osteogenesis imperfect, treatment, Ender nail

Introduction

Osteogenesis imperfecta (OI) is a genetic disorder caused by defective type I collagen synthesis. Multiple fractures and deformities of long bones are frequently seen in patients with OI and can influence their ability to walk (1). Correction of bone deformity integrated with bone stabilization has been used for many years in the management of OI, especially since Sofield and Millar reported their study of multiple osteotomies and intramedullary rodding for children (2). Because of the bones are growth, fixed-length rods require frequent revision (-3) and this encouraged the introduction of the telescopic intramedullary rod (TIMR), by Bailey and Dubow (4). The major problem of Bailey-Dubow expanding intramedullar rod was rod relocation frequently combined with perforation of joint, bone and soft tissue. Other complications of Bailey-Dubow expanding intramedullar rod like pseudarthrosis, infections, lack of elongation or over prolongation of the intramedullar rods, and loosening of the T-piece were infrequently observed (5). Revision rates of TIMR have been high since weakness in material or design of the implants and continuing bone growth.

Multiple flexible nails for the fixation of femoral fractures were first reported by Ender (6) in 1970. Ender nail used for a large spectrum of fractures of the femoral shaft, including simple, comminuted or segmental injuries. The use of Ender nail is comparatively easy and quick and requires little specialised instrumentation (6).

The purpose of this study was to evaluate the results of ender nailing of the bones of the lower extremities in children and adolescents patients with OI.

Materials and Methods

Study design

This retrospective study was carried out with the approval of the Local Ethics Committee. We have been treated 11 femoral fracture cases with the Ender nail. The basic characteristics of both groups, including height, weight, and body mass index (BMI), were recorded. Inclusion criteria were those aged 18-70 years, patients who were diagnosed with OI that had been treated ender nailing between January 2010 and November 2012. A total of 11 femoral fractures of patients with OI were treated, using ender nails. Data regarding complications and additional procedures were achieved from the clinical records and radiological review.

Surgical technique

Under general anesthesia, the patient is placed on traction table. Lateral approach is performed for multiple osteotomies of femur. A subperiosteal resection of the entire shaft of a long bone is performed, divided the bone at selected sites. Two 3.5-mm ender nails are placed through medial and lateral insertion sites at the distal femoral metaphysis in a retrograde, divergent "C" configuration (Figure 1). Placement of Ender nails are confirmed for each stage using fluoroscopy. The bone fragments are threaded the pieces on to Ender nails and then replaced the beaded but straightened shaft (Fig. 1).

Post-operative management

Post-operative immobilization with cast was applied for at least six weeks in all of the patients (If there was a delay in union at any osteotomy site, in that case two to four weeks immobilization time was added). Ambulation with the aid of crutches or walker was encouraged as soon as the osteotomy site had united according to each patient’s special situation. External splint or appliance was not used once the osteotomy site had totally united.

Results

Study subjects included 6 female (54.5%) and 5 male (45.5%) with the mean age of 9.7±1.85 years (range 7–13 years) at last follow-up. Of the total femur studied, we looked at 7 (63.6%) left femur and 4 (36.4%) right femur. The average time from initial nailing to final follow-up period was 20.7 months (6-38 months). Nailing was performed in all of 11 patients. The indications for nailing of the 11 femurs included fracture and deformity. All of the femurs were nailed because of fracture and deformity. Union was achieved in all cases within 6.9 weeks (range: 5–10 weeks). No re-fracture was observed in any case.

The complication rate in these patients was 18.2% (2 patients). The most common complication following nailing was pull-out of Ender nail in femur. Pull-out of Ender nail was seen following nailing in two cases. These cases were revised for stabilization. So, the Ender nails were screwed for prevent this complication in seven cases. Bending of one of the Ender nails or non-union was not observed.

Discussion

OI is a hereditary disease and characterized by frequent long bone fractures. Patients with OI have no cure. Bowing of femur in patients with OI with or without fracture is the main interest of orthopedist. The aim of treatment management should be directed to maximize the affected patient’s function and to prevent deformity and disability resulting from fractures. Severe long bone deformity (prohibiting bracing and ambulation) is indication for surgery of OI. Over many years, surgical correction of bone deformities, physiotherapy, and the use of orthotic support and devices to assist mobility were used treatment (7).

Numerous studies have shown that the frequency of fractures of long bones of the lower extremities, in patients with OI, can be markedly decreased by implanting intramedullary rods into the femur and tibia (8-12). Correction of any deformities of lower limbs and implantation of intramedullary rods is a choice currently available for obtaining this purpose (12, 14). Intramedullary rods that have been used in OI include TIMR, single non-elongating rods and dual non-elongating rods (11, 12). In literature, an unexplained increase in growth of bone was found in some cases treated with TIMR on one side and a non-TIMR on opposite side, based on the premise that extrmity length was equal at the beginning of surgical treatment although the timing of surgery was not similar with a range from 6 weeks to 6 months (15).

We have been using Ender nail in the treatment of OI with long bone fractures in our hospital since 2009. Intramedullary nailing with Ender nails is an easy and safe procedure for correct angulation and fracture treatment to avoid plaster immobilization in diaphyseal fractures of long bones in childhood. In additional, Ender nail is cost effectiveness (6). The current cost of a TIMR at our center is the equivalent of US$400. It is this too much cost that made us look for cheaper alternative methods of fixation. We adopted the technique in 2009 since two Ender pins cost us only an equivalent of US$60. Dual Rush pinning of the femur was advised by Luhmann (12). Dual Rush pinning provides as much support to the bone against a fracture as a TIMR placed centrally within the medullary canal. The results of this study, intramedullary Ender nailing of femur in patients with OI significantly improves the quality of life. In our setting, we currently prefer to use a dual Ender pins in the femur on the basis of the results of this study (Figure 2).

Conclusion

This study was primarily designed to evaluate the effectiveness of the treatment of patients with OI using Ender nails. Our findings support the intramuscular application of Ender nail as effective in the treatment of the patient with fracture or deformity caused to OI.

Conflicts of Interest

The authors declare that no conflict to interest.

Figure legends

Figure 1: After multipl osteotomies were made, two Ender nails were placed.

Figure 2: Two Ender nails were used for fracture in patient with OI (after 2 years to fracture)