Potential Correlation between Kienböck's Disease and Hereditary Hemochromatosis: A Case Report

Abstract

Introduction: Avascular necrosis of the lunate bone has been extensively researched, although the etiology of the condition remains controversial. Even though many treatments for the disease exist, a better understanding of the pathophysiology can improve our decision-making between preventive and therapeutic measures. Various hematological disorders have been found to predispose for Kienböck's disease. On the other hand, there has not yet been any reference in literature to a relationship between this condition and hereditary hemochromatosis (HH).

Case report: We present two cases of Kienböck's disease in two patients who are third-degree relatives and diagnosed with HH. A 61-year-old Caucasian female patient with type 1 HH presented with symptomatic Kienböck's disease on the left side. The patient is a third-degree relative of a 51-year-old male Caucasian patient with Kienbock's disease on the right side, known as having the same hereditary hematological condition.

Conclusion: Our findings suggest a potential correlation between the aforementioned conditions. The prevalence of these coexisting pathologies should be studied further.

Keywords: Kienböck; hemochromatosis; lunate; osteonecrosis.

Figure 1

Anteriorposterior and lateral left wrist radiographs of case 1 demonstrating Kienböck’s disease. Radiographs of the left wrist of case 1 at the first presentation showing Lichtman stage IIIb Kienböck’s disease with notable scaphoid flexion and ring sign. Limited degeneration of the neighboring intercarpal joints.

Figure 2

Magnetic resonance imaging-images. T2- and T1-weighted anteriorposterior left wrist images of case 1. T2- and T1-weighted anteriorposterior magnetic resonance images of the left wrist of case 1 at first presentation showing Lichtman stage IIIb Kienböck’s disease. These images depict no significant effusion or degeneration of neighboring joints.

Figure 3

Anteriorposterior and lateral right wrist radiographs of case 2 demonstrating Kienböck’s disease. Radiographs of the right wrist of case 2 at first presentation showing Lichtman stage IIIb Kienböck’s disease with a near total collapse of the lunate bone. Scaphoid flexion and ring signs are seen as well. There’s little degeneration of the neighboring intercarpal joints.