What’s Your Diagnosis?
ANSWER: VOLKMANN ISCHEMIC CONTRACTURES, BILATERAL
The hands and wrists are affected symmetrically apart from sparing of the fifth finger on the left side only (Figures 1, 2, and 3). The upper arms and forearms show no diagnostic changes. Sarcopenia and muscular atrophy are present throughout all limbs as well as the trunk.
The wrists show severe but not unprecedented flexion contractures. Far more striking and aberrant are the metacarpophalangeal deformities; these look extremely painful in that the fingers are bent back so sharply. We could not find any tendon defect in the palm, so we could not infer disruption of tendon pulley as a cause of the disfigurement. Dorsal tendons appear intact on the atrophic back of each hand (see Figure 2).
The fingers are flexed at both interphalangeal joints but show more mobility than the other joints. The fingers do not dig into the palm (see Figure 3). Each thumb is laterally deviated and, in contrast to the fingers, shows hyperextension rather than flexion at its interphalangeal joint. The skin is warm and not pallid, though the surface is somewhat shiny.
We didn’t recognize the pattern, nor did we find a label in the medical records available for review. Information was studied about deformities that complicate Parkinson disease. Nothing remotely related was discovered.
Then we sought further clarification from the patient, from her older medical records, and from the literature. She first asserted that rheumatoid arthritis had been blamed for the changes; we found no such lesions in reviewing the many upper-limb deformities described at one or another stage of rheumatoid arthritis. Our reexamination of the patient was equally unconvincing: no clinical synovitis or any other feature of active rheumatoid disease, nor any pattern we recognize as burnt-out rheumatoid arthritis.
Eventually a best (imperfect) diagnosis emerged: Volkmann ischemic contracture.1-4 Though this was an “Aha!” moment, the patient denied ever hearing the term applied to her case.
FINDINGS IN VOLKMANN CONTRACTURE
This patient's hands show a unique pattern of deformity best explained as Volkmann ischemic contracture, a dreaded sequela of compartment syndrome. Unrelieved compartment syndrome leads to infarction of the flexor digitorum profundus muscle, and then to contracture of the scar remnant. The result is functional and cosmetic catastrophe for the entire wrist-hand unit. This sequence can occur in children following supracondylar fracture of the humerus, eventuating in a severely contracted and functionless forearm. It can also arise from other causes.
Clinically, in a Volkmann contracture the wrist is flexed as our patient’s was; a clawhand deformity is formed because the metacarpophalangeal joints are hyperextended and the proximal and distal interphalangeal joints are flexed. In the staging of the condition, our case best fits stage IV, the second worst.4 The fifth finger on one side was minimally affected—which likely means that the ulnar nerve was relatively spared,4 a known subtype.
WHAT FEATURES DEVIATE FROM EXPECTED?
Extreme deformity of joints from the metacarpophalangeal on distally fits the diagnosis perfectly. However, the expected absence or decrease in sensation to the hand was not present, suggesting—if the patient did not mislead us—that the degree of nerve injury was startlingly little for a Volkmann contracture of this degree.
The forearm is often visibly atrophic compared to its opposite,2 and to musculature elsewhere in the body. Our patient’s was not, but bilaterality hindered the one assessment, and generalized sarcopenia the other. The forearm should be fixed in pronation, and was not.
Bilaterality is not expected unless the same injury, by some horrendous fate, befalls both arms. Yet there was no known antecedent compartment syndrome.