summary Neonatal Forearm Compartment Syndrome is a devastating upper extremity condition in neonates where the osseofascial compartment pressure rises to a level that decreases perfusion to the forearm and may lead to irreversible muscle and neurovascular damage. Diagnosis is different from compartment syndrome in adults with the primary finding in neonates being skin lesions such as bullae, eschars, ulcers, with the presence of digital and hand ischemia/edema. Treatment is usually emergent fasciotomies. Epidemiology Incidence rare limited to case reports, largest series is 24 cases over 20 years Demographics age bracket neonates during first 24 hours of life Anatomic location forearm, wrist, hand (equal R:L distribution) unilateral dorsum more common than volar Risk factors hypercoagulable states polycythemia prematurity oligohydramnios maternal diabetes multiple gestation abnormal lie neonatal respiratory distress Etiology Pathophysiology mechanism of injury possible birth trauma (see below) idiopathic is most common pathophysiology exact mechanism unknown, although both extrinsic and intrinsic factors are believed to be involved extrinsic (mechanical compression with forearm being trapped between structures) fetal posture oligohydramnios umbilical cord loops amniotic band constriction direct birth trauma intrinsic (clotting) hypercoagulable state producing arterial/venous compression Presentation History idiopathic (no obvious cause) is most common cause Symptoms common symptoms all patients present with skin lesion (wide spectrum) bullae erythema ulcerative distal digital/hand edema eschar fingertip gangrene duration presents at birth Physical exam inspection skin lesion bullous swelling, erythema nerve involvement (radial nerve and PIN > ulnar = median) may have lack of spontaneous limb movement Imaging Radiographs recommended views AP and lateral forearm radiographs findings skeletal changes happen late (more evident when the child grows) physeal distortion (widening, flaring, premature closure, angular deformity, shortening) limb length discrepancy MRI indications late-presenting cases without edema, but with extensive full-thickness necrosis and extreme contractures (where fasciotomy is likely to be futile) may help delineate full extent of underlying necrosis and guide muscle debridement Studies Labs indications to rule out infection, cellulitis findings CBC, ESR, CRP within normal values Compartment pressure indications is NOT done in neonates because no standards for acceptable pressure gradients (delta value) neonate’s DBP at birth is <40mmHg and a small increase in compartment pressure rapidly impairs muscle perfusion Differential Cellulitis distinguishing features mother does not show signs of infection, has negative cultures Necrotizing fasciitis most easily mistaken for compartment syndrome, and only diagnosed/confirmed at operation distinguishing features only involves skin, subcutaneous tissue treated with excision, not fasciotomy Vascular injuries associated with brachial plexus lesions distinguishing features absent pulses and Doppler studies Treatment Nonoperative anticoagulants and thrombolytics indications hypercoagulable states Operative emergency immediate fasciotomy indications emergent surgery is usually indicated diagnosis of compartment syndrome technique release volar, dorsal and mobile wad compartments release carpal tunnel may need split thickness skin graft outcomes best outcomes if diagnosed and treated within first 24 hours of life salvage surgery indications late sequelae techniques neurolysis debridement of dead muscle contracture release soft tissue resurfacing angular correction limb lengthening staged flexor/extensor tendon reconstruction outcomes outcomes are inferior to early fasciotomy Complications Most common complications: Ischemic muscle contracture muscle debridement and contracture release Fingertip gangrene Physeal distortion limb lengthening angular correction Nerve dysfunction neurolysis Prognosis Natural history of disease usually missed initially, detected only after complications ensue prognostic variable negative missed diagnosis has worst prognosis Outcomes with treatment prognostic variable favorable early fasciotomy has best prognosis