Spinal Dysraphism Presenting as Acro‐Osteolysis: Report of Four Cases

Abstract

The acro-osteolyses are a heterogeneous group of disorders characterized by bone resorption. The disorder may occur as familial, idiopathic, or secondary to vascular, inflammatory, or neurologic conditions. Acro-osteolysis is rare in association with spinal dysraphism. It is even rarer for it to be the presenting symptom in spinal dysraphism. We report here four patients in whom the diagnosis of spinal dysraphism was established while investigating for the various causes of acro-osteolysis. All four patients presented with trophic changes and acro-osteolysis. Hyperhidrosis in the affected limb was seen in three patients. One patient had leg pain, the others had no sensory or motor deficits. Magnetic resonance imaging showed spinal dysraphism in all four patients.

Occult dysraphism presenting with acro‐osteolysis

“…Sethuraman and colleagues described four patients, aged 6, 14, 25, and 32, in whom evaluation for unilateral or asymmetric acro‐osteolysis and trophic changes on the foot led to the diagnosis of occult spinal dysraphism. Midline cutaneous changes were absent in three cases, and one patient had a hairy patch over the lumbosacral area . Gupta and colleagues reported acro‐osteolysis with faun tail nevus in a 5‐year‐old boy with spinal dysraphism .…”

“…Gupta and colleagues reported acro‐osteolysis with faun tail nevus in a 5‐year‐old boy with spinal dysraphism . The clinical sequelae of spinal dysraphism are summarized in Table …”

Lost bones: differential diagnosis of acro-osteolysis seen by the pediatric rheumatologist

“…Lastly, unilateral lower extremity acro-osteolysis has been reported as the presenting feature of occult spinal dysraphism in several patients including cases in children [ 51 , 52 ]. Whereas most causes of acro-osteolysis outlined above would manifest with bilateral involvement, unilateral acro-osteolysis should prompt the clinician to consider local trauma and spinal dysraphism in the differential diagnosis.…”