Updated: 10/10/2016

Ochronosis

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Questions
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Evidence
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https://upload.orthobullets.com/topic/9046/images/ochronosis[1]_moved.jpg
Introduction
  • Degenerative arthritis that results from alkaptonuria
  • Pathophysiology
    • excess homogentistic acid is deposited in the joints
    • acid polymerizes in joint and leads to early joint arthritis
  • Genetics
    • rare inborn defect in homogentisic acid oxidase enzyme system 
  • Associated conditions
    • orthopaedic manifestations
      • ochronotic spondylitis
        • commonly occurs in fourth decade
        • progressive degenerative changes in spine
          • calcification
          • narrowing of disc spaces
Presentation
  • Symptoms
    • may complain of black urine
      • caused by polymerization of homogentistic acid 
Imaging
  • Radiographs
    • spine 
      • findings
        • irregular calcification 
        • narrowing of intervertebral discs  
 Treatment
  • Nonoperative
    • no current medical treatment available
 

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Questions (1)

(OBQ11.153) A 55-year-old male presents with a 6 month history of low back pain. Radiographs of the spine are shown in Figures A and B and a lateral knee radiograph is shown in Figure C. A urine specimen is exhibited in Figure D. What is the most likely diagnosis in this patient? Review Topic | Tested Concept

QID: 3576
FIGURES:
1

Disseminated idiopathic skeletal hyperostosis (DISH)

5%

(71/1309)

2

Ochronosis (alkaptonuria)

68%

(889/1309)

3

Chondrodysplasia punctata (Conradiā€“Hünerman)

1%

(16/1309)

4

Homocystinuria

20%

(256/1309)

5

Ankylosing spondylitis

6%

(73/1309)

L 3 C

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Evidences (2)
VIDEOS (1)
Topic COMMENTS (0)
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