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Difficulties of Cementless Total Hip Arthroplasty in Osteopetrosis: A Case Report

Rahimi H MD, Shahpari O MD, Assadian M MD, Ariamanesh AS MD*

Department of Orthopedic Surgery, Mashhad University of Medical Sciences, Mashhad, Iran

* Corresponding Author

Vol 2, Num 1, January 2015




Total joint arthroplasty may be considered as a last option to treat osteopetrosis associated with osteoarthritis or some other complications. Many intraoperative challenges need to be overcome when performing arthroplasty so we present a 36 years old woman with autosomal dominant (AD) osteopetrosis who was suffering a painful nonunion of subthrocanteric fracture, reffered to our center in Jun 2010.

This patient had previously been operated on for three times and at the last time she underwent open reduction and internal fixation(ORIF) with dynamic hip screw (DHS) which resulted in nonunion associated with destruction of femoral head ,therefore we preferred to do uncemented total hip arthroplasty for the patient. we encountered some difficulties especially during preparation of the femoral canal. The short term post operative clinical and radiological results are good and the patient is walking independently and is pain free at the moment.

Keyword:Osteopetrosis, Autosomal Dominant, Cementless Total Hip Arthroplasty




Osteopetrosis is a group of sclerosing bone dysplasia characterised by diminished osteoclast mediated skeletal resorption (1) leading to universally hard but brittle bone.(2) As first described by Albert Schonberg, a German radiologist in 1904 (3), this inherited disorder of bone metabolism is divided into three clinical forms; (a) severe infantile or malignant autosomal recessive with severe bone marrow failure, pancytopenia, bleeding, infection, failure to thrive, blindness, deafness, hydrocephalus and early childhood death, (b) intermediate autosomal recessive or Marble bone disease with no bone marrow failure, renal tubular acidosis, intracranial calcification, sensorineural hearing loss and psychomotor retardation and (c) AD form.(4-7)

In all three forms of osteopetrosis there is a lack of osteoclast function resulting in decreased bone resorption with increased cortical bone and calcified cartilage.(2)

The AD form can be subdivided into type 1 and type 2 as described by Bollerslev and Anderson (8), in type 1 there is increased thickness of the skull, diffuse osteosclerosis of the lumbar spine and pelvis and symmetrical long bone involvement. Type2 shows more basal skull involvement, a 'rugger jersey' spine and 'endobones' within the pelvis. However, the AD form is compatible with a normal life span (9,10) but causes frequent orthopedic problems, including fractures, coxa vara, long bone bowing, osteomyelitis, osteoarthritis and fracture nonunion.(4,9,11,12,14)

In order to treat the fractures and bone deformities, plating and intramedullary rods are used, but joint replacement is reserved for degenerative joints diseases, but these operations are sometimes extremely difficult because of the bone fragility and obliteration of the medullary canal.(4,15,16) There have been several early reports of cemented or hybrid hip arthroplasty, in these patients. But to the best of our knowledge, there exist a few reports regarding cementless total hip arthroplasties in such patients.

Case Report

On June 2010 a 36 year old woman with osteopetrosis , was referred to our center for treatment of painful nonunion of left subthrocanteric fracture. The radiographs showed destruction of the femoral head and acetabulum due to penetration of DHS nail into the acetabulum ( Figure 1).

Although the patient had undergone three successive operations on, the fracture did not go to union, besides, the lag screw had cut out of the proximal fragment and greater trochanter was separated. The patient was unable to walk without help and the Harris Hip Score (HHS) at the time of referral was 27. Thus, we decided to perform total hip arthroplasty for her.

In preoperative assessment, the blood cell count was normal and general examination appeared good.

As an infection prophylaxis measure, 2 grams of Cephazolin was injected intravenously thirty minutes before the operation and continued for two days postoperatively.

During the surgery, first, the DHS was removed through the posterior approach, although with much difficulties. Then, through a posterior capsulotomy, the femoral head and neck were cut and removed and acetabulum was reamed, again with much difficulties. When the acetabulum was prepared, the metal back was pressfit and augmented with two screws, insertion of which was very hard.

To prevent the femoral shaft fracture, before reaming, we tied multiple stainless steel wires around the proximal femoral shaft. Then, under fluoroscopic control, we tried to create a central medullary canal in the femoral shaft with long 3.2 and 4.5 drill bits. When we became sure that the canal was in the center of the shaft, both in AP and Lateral dimensions, we gradually increased the diameter of canal. When the diameter of this canal became so wide that a Cone Wagner reamer could be inserted, we reamed the canal with the specific reamer. At Then we inserted the trial stem under the flouroscopic control (Figure 2).

Finally, we inserted a Cone Wagner stem size 3 and long enough to bypass the screw holes in the femur. Upon trial reduction, we chose a standard neck length. The greater trochantr was reattached with wiring and augmented with a small allograft. At the end of procedure, a stable reduction was obtained (Figure 3). The wound was sutured in the regular manner on suction drainage.

Postoperative radiographs showed good positioning of the stem and cup. Total amount of intraoperative blood loss was 1700 ml and operating time was 4 hours.

The day after operation the patient began to walk with toe touch wight-bearing with the aid of two crutches.

At the time of the last visit, 18 month postoperatively, the patient is pain free and is walking well without walker and her Harris Hip Score is 70.

    Figure 1: Preoperative pelvic X-ray of the patient with osteopetrosis and painful nonunion of left subtrochanteric fracture.
    Figure 2: Intraoperative flouroscopic view ensuring that the femoral reamer is within the canal.
    Figure 3: Final postoperative X-ray of the patient.


    Osteopetrosis, a congenital bone disease, is the result of the failure of osteoclastic function. This malfunction with decreased resorption of bone leads to universally hard bone with thickened trabeculae and decreased medullary cavity diameter which is brittle and susceptible to fractures. Fractures are often transverse and usually result from minor trauma.(21)

    Although the literature shows that in tarda form (AD) of osteopetrosis, near 40% of patients remain asymptomatic (8,9), multiple orthopaedic peresentations including recurrent fractures especially in lower limbs and degenerative arthritis are common. Fractures in osteopetrosis can occur at any age. As the healing response is variable, management of this patients is challenging and operative intervention is associated with a high rate of intraoperative and postoperative complications.(17) These patients are at risk for periarticular nonunion recalcitrant to treatment (18) and sometimes joint replacement remains as the only solution.

    There are few case reports on hip arthroplasty and technical difficulties in such patients with six month to ten years follow-up.(4,11,14,15,16,19) Most of the reported procedures are cemented or hybrid total hip arthroplasty. In 2004 Gwynne et al. reported first primary uncemented total hip arthroplasty in osteopetrosis.(20)

    During the operation on our patient, we experienced the same problems that Gwynne reported. First, removal of previous failed device embedded in hard bone was very difficult and required caution in order mot to fracture the screws. Surely, any screw breakage may obviate the insertion of the femoral stem. Second, preparation of the medullary canal is extremely difficult and frustrating, although not impossible. We used prophylactic wiring on proximal femur and created medullary canal by high speed burr and power drill, step by step under the fluoroscopic control. On the acetabular side, there was no cancellous bone to provide an environment for interdigitation of the cement with bone. Therefore, a cementless cup was used. Third, due to impaired bone marrow and white cell function (4), these patients are prone to infection. This obliged us to use prophylactic antibiotic, both intra and postoperatively for a longer period than usual.

    In 2004, Gwynee reported the first all cementless total hip arthroplasty for osteopetrosis and now we report a similar case of a revision surgery for previous device failure with acceptable short-term results.

    Rahimi H MD
    Department of Orthopedic Surgery, Mashhad University of Medical Sciences, Mashhad, Iran


    Shahpari O MD
    Department of Orthopedic Surgery, Mashhad University of Medical Sciences, Mashhad, Iran


    Assadian M MD
    Department of Orthopedic Surgery, Mashhad University of Medical Sciences, Mashhad, Iran


    Ariamanesh AS MD
    Department of Orthopedic Surgery, Mashhad University of Medical Sciences, Mashhad, Iran
    Corresponding Author


    None declared.


    Financial disclosure:
    None declared.



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