Congenital Volkmann Syndrome and Aplasia Cutis of the Forearm

Abstract

In our opinion, CVIC should be considered a particular form of ACC in which an external noxa affects the forearm, increasing the intracompartmental pressure and leading to muscle and nerve ischemia. Therefore, we propose that the definition of Volkmann ischemic contracture should be maintained only for the acquired forms with an evident etiology and that Frieden's classification scheme for ACC type VII needs to be reformulated.

Infant with multiple ulcerations on the scalp

“…The scalp is the most affected area, accounting for greater than 85% of the cases with solitary lesions 3 . ACC is classified into nine subsets based on location and associated abnormalities 4,5 . Our patient had the non‐membranous variant, belonging to Group 1, defined as ACC on the scalp without associated comorbidity.…”

Cutaneous ulcer on the forearm of a newborn

“…It is characterized by muscle necrosis and nerve palsy due to the increased intracompartmental pressure . Neonatal VIC, also named neonatal compartment syndrome, is very unusual . Typically, skin lesions in neonatal VIC present as swellings with large bullae that rapidly evolve into deep ulcers and large necrotic areas, which become covered by eschars.…”

“…1 Neonatal VIC, also named neonatal compartment syndrome, is very unusual. 2,3 Typically, skin lesions in neonatal VIC present as swellings with large bullae that rapidly evolve into deep ulcers and large necrotic areas, which become covered by eschars. The most common location is the forearm.…”

Practical Review on the Contemporary Diagnosis and Management of Compartment Syndrome

“…37 Early detection of associated skin changes, such as ecchymosis, bullae, or distal gangrene, should prompt emergent surgical intervention within hours of presentation to prevent contracture and functional limitations. 38,39…”

“…37 Early detection of associated skin changes, such as ecchymosis, bullae, or distal gangrene, should prompt emergent surgical intervention within hours of presentation to prevent contracture and functional limitations. 38,39 It should be noted that current compartment measurement thresholds of greater than 30 mm Hg are still used in this population. 40 Pediatric patients may face long-term consequences from fasciotomies due to scar formation and risk of contracture with growth-related changes.…”