Patients with Glycogen storage disease type V can develop acute compartment syndrome (ACS) — an urgent limb-threatening complication. The clinical picture is dominated by relentless limb pain that is not relieved by rest, combined with signs including pallor, absence of pulse, paralysis, and paraesthesia.
ACS is a clinical diagnosis. The hallmark is persistent, unrelenting pain that does not ease with rest. The accompanying signs — pallor, absent peripheral pulse, paralysis, and paraesthesia — reflect the progressive vascular and neural compromise within the affected limb and confirm the urgency of the situation.
Management requires a prompt surgical intervention directed at the affected limb. Speed of diagnosis and intervention is a key determinant of outcome. The full structured regimen and decision criteria are available in the protocol below.