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Atypical Presentation for Complex Regional Pain Syndrome
Buzniak, D, D.O., Gelzhiser,J, MD
Kent Hospital, Internal Medicine Residency Program; Warwick, Rhode Island
Introduction: Complex Regional Pain Syndrome (CRPS) is a rare disorder, usually of
the extremities, that is often difficult to diagnose and can pose a therapeutic challenge
for physicians. An inciting event such as trauma, surgery or vascular injury can
predispose someone to develop this syndrome. Pain, swelling, skin changes, limited
range of motion, and patchy bone demineralization in the affected extremity are
symptoms characteristic of this disorder.
Case Description: A 22 year old female presented to Kent County Hospital complaining
of a painful blue right foot that started on the day of admission. The patient was involved
in a motor vehicle accident several months prior that resulted in a torn right gluteal
muscle and lumbar disk herniation and developed progressive complications. For the
past month she had swelling of the right foot and ankle with worsening pain. Upon
presentation to the emergency department her right foot was cyanotic with mottling of
the right lower leg, pitting edema and a thready dorsalis pedal pulse. She underwent
emergent angiography by interventional radiology due to concern for acute arterial
occlusion. Angiogram of right lower extremity showed normal arterial anatomy from the
renal arteries to the distal calve arteries with profound distal vasospasm involving all
three calf vessels. Intra-arterial nitroglycerin was infused revealing underlying normal
anatomy. Bony demineralization of the tarsal bones was noted as well which suggested
a diagnosis of complex regional pain syndrome. The patient was admitted following the
procedure and it became a challenge throughout the hospital stay to control her pain. In
addition, the patient had multiple imaging modalities in the hospital to rule out other
diagnoses which were unrevealing. After appropriate workup, she was discharged from
the hospital with a follow up appointment with a specialist in Boston.
Discussion: Complex regional pain syndrome is a challenge to diagnosis and treat. It
typically involves an extremity following trauma with pain out of proportion to the injury.
CRPS is progressive and occurs in three stages with poorer response to therapy in later
stages. The first stage is commonly associated with abnormal vasodilation and a warmer
affected extremity. In contrast, our patient presented with a cold extremity and
decreased perfusion which is only seen in 30% of cases during the acute phase. Prompt
recognition and appropriate intervention including early mobilization can improve the
overall outcome in people with this disorder. While not particularly effective for our
patient, pharmacologic modalities such as anticonvulsants and certain antidepressants
can be helpful in management. Further treatment options for those nonresponsive to
medications include more aggressive measures such as sympathectomy. The overall
goal of treatment in complex regional pain syndrome is to restore movement and
strength in the area involved. It is, therefore, important to keep in mind complex regional
pain syndrome as a potential diagnosis so early intervention and appropriate therapy
can be initiated.