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Introduction
  • Definition
    • forearm compartment syndrome in a neonate (presents differently from adults) 
      • does not present with classic “p”s
  • Epidemiology
    • incidence
      • rare
      • limited to case reports, largest series is 24 cases over 20 years)
    • demographics
      • gender ratio
        • male : female ratio (L1 common cond / L3 rare cond.)
      • age bracket
        • neonates during first 24hours of life
    • body 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
  • 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
  • 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
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
Differentials
  • 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
 

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