Updated: 5/24/2021

Neonatal Forearm Compartment Syndrome

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  • 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 24hours 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
    • 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
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