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Poor results of drilling in early stages of juxta-articular osteonecrosis in 12 joints affected by Gaucher disease.

Lebel E, Phillips M, Elstein D, Zimran A, Itzchaki M - Acta Orthop (2009)

Bottom Line: Gaucher disease is heterogeneous.We found equally poor outcome with and without drilling.Effective intervention can only be achieved by improving our understanding of bone physiology and pathophysiology in Gaucher disease.

View Article: PubMed Central - PubMed

Affiliation: Department of Orthopaedic Surgery, Shaare Zedek Medical Center, Jerusalem, Israel.

ABSTRACT

Background and purpose: Gaucher disease is heterogeneous. One of the most devastating complications is bone involvement, ranging from mild osteopenia to osteonecrosis, but no markers have been discovered to predict onset and/or progression. We describe our experience in a large referral center using drilling for juxta-articular osteonecrosis in young patients with Gaucher disease.

Patients and methods: We retrospectively reviewed medical data from all patients who were recommended to undergo drilling for osteonecrosis of juxta-articular bone of the femoral head, the humeral head, or upper tibia for acute osteonecrosis at a pre-collapse stage.

Results: 11 patients (mean age 34 years) underwent drilling of 12 joints with juxta-articular osteonecrosis; 3 (mean age 51 years) refused intervention. 9 joints that were drilled showed advancing joint degeneration within 0.5 to 4 years. 3 joints have undergone replacement. Of the 3 joints that did not undergo drilling, 2 have undergone replacement and 1 has collapsed with osteoarthritis.

Interpretation: We found equally poor outcome with and without drilling. Effective intervention can only be achieved by improving our understanding of bone physiology and pathophysiology in Gaucher disease.

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Related in: MedlinePlus

Typical view of Gaucher bony milieu (taken during drilling).
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Figure 0001: Typical view of Gaucher bony milieu (taken during drilling).

Mentions: The size of lesions is not stated in this report since radiological findings within the bone marrow are evident in all cases but delineation of borders of osteonecrosis and Gaucher-related lesions are difficult to define. Invariably (and universally), the medullary lesions in Gaucher bone are accompanied by abnormal matrix composition (Figure 1) that often raise conflicting radiological signals and complicate the reading of acute or chronic involvement. The presence of artifacts on MRI further confuses attempts at delineation of the borders of the affected area.


Poor results of drilling in early stages of juxta-articular osteonecrosis in 12 joints affected by Gaucher disease.

Lebel E, Phillips M, Elstein D, Zimran A, Itzchaki M - Acta Orthop (2009)

Typical view of Gaucher bony milieu (taken during drilling).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2823173&req=5

Figure 0001: Typical view of Gaucher bony milieu (taken during drilling).
Mentions: The size of lesions is not stated in this report since radiological findings within the bone marrow are evident in all cases but delineation of borders of osteonecrosis and Gaucher-related lesions are difficult to define. Invariably (and universally), the medullary lesions in Gaucher bone are accompanied by abnormal matrix composition (Figure 1) that often raise conflicting radiological signals and complicate the reading of acute or chronic involvement. The presence of artifacts on MRI further confuses attempts at delineation of the borders of the affected area.

Bottom Line: Gaucher disease is heterogeneous.We found equally poor outcome with and without drilling.Effective intervention can only be achieved by improving our understanding of bone physiology and pathophysiology in Gaucher disease.

View Article: PubMed Central - PubMed

Affiliation: Department of Orthopaedic Surgery, Shaare Zedek Medical Center, Jerusalem, Israel.

ABSTRACT

Background and purpose: Gaucher disease is heterogeneous. One of the most devastating complications is bone involvement, ranging from mild osteopenia to osteonecrosis, but no markers have been discovered to predict onset and/or progression. We describe our experience in a large referral center using drilling for juxta-articular osteonecrosis in young patients with Gaucher disease.

Patients and methods: We retrospectively reviewed medical data from all patients who were recommended to undergo drilling for osteonecrosis of juxta-articular bone of the femoral head, the humeral head, or upper tibia for acute osteonecrosis at a pre-collapse stage.

Results: 11 patients (mean age 34 years) underwent drilling of 12 joints with juxta-articular osteonecrosis; 3 (mean age 51 years) refused intervention. 9 joints that were drilled showed advancing joint degeneration within 0.5 to 4 years. 3 joints have undergone replacement. Of the 3 joints that did not undergo drilling, 2 have undergone replacement and 1 has collapsed with osteoarthritis.

Interpretation: We found equally poor outcome with and without drilling. Effective intervention can only be achieved by improving our understanding of bone physiology and pathophysiology in Gaucher disease.

Show MeSH
Related in: MedlinePlus