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Exophthalmos-Enophthalmos

Orbital Position Abnormalities - Overview

Abnormal positioning of the globe within the orbit can indicate various systemic and local pathologies. Early recognition is crucial for appropriate management.

Key Points

  • Exophthalmos: Forward displacement of globe
  • Enophthalmos: Posterior displacement of globe
  • Both can be unilateral or bilateral
  • Requires prompt evaluation in pediatric cases

Exophthalmos (Proptosis)

Definition

Anterior displacement of the globe beyond the orbital rim. In children, any asymmetry >2mm is significant.

Etiologies in Children

  • Orbital Tumors
    • Rhabdomyosarcoma (most common primary orbital malignancy)
    • Neuroblastoma metastasis
    • Optic nerve glioma
    • Lymphangioma
    • Capillary hemangioma
  • Inflammatory Conditions
    • Orbital cellulitis
    • Orbital pseudotumor
    • Thyroid eye disease (rare in children)
  • Developmental Conditions
    • Craniosynostosis syndromes
    • Crouzon syndrome
    • Apert syndrome

Clinical Features

  • Red Flags
    • Rapid onset
    • Associated pain
    • Decreased vision
    • Color vision changes
    • Resistance to retropulsion
  • Associated Signs
    • Exposure keratopathy
    • Conjunctival injection
    • Diplopia
    • Optic nerve compression

Enophthalmos

Definition

Posterior displacement of the globe within the orbit. Significant if >2mm compared to fellow eye.

Pediatric Causes

  • Congenital Conditions
    • Linear scleroderma (Parry-Romberg syndrome)
    • Microphthalmos
    • Congenital facial hemiatrophy
  • Acquired Conditions
    • Orbital trauma with fracture
    • Silent sinus syndrome
    • Orbital fat atrophy
  • Metastatic Neuroblastoma (late stage)

Clinical Features

  • Deepened superior sulcus
  • Apparent ptosis
  • Narrowed palpebral fissure
  • Usually unilateral

Assessment & Imaging

Clinical Measurement

  • Hertel Exophthalmometry
    • Normal values vary by age and race
    • Newborn: 10-12mm
    • Children: 14-20mm
    • Difference >2mm is significant
  • Digital Photography
    • Standardized views
    • Serial monitoring

Imaging Studies

  • CT Orbit
    • Bony details
    • Orbital contents
    • Sinuses evaluation
  • MRI Orbit
    • Soft tissue details
    • Optic nerve evaluation
    • Intracranial extension
  • Ultrasound
    • Dynamic assessment
    • Vascular lesions
    • No radiation exposure

Management Approach

Exophthalmos Management

  • Emergency Management
    • Vision-threatening cases
    • Orbital compartment syndrome
    • Lateral canthotomy if needed
  • Specific Treatment
    • Based on underlying cause
    • Antibiotics for infection
    • Steroids for inflammation
    • Chemotherapy for malignancy

Enophthalmos Management

  • Conservative Care
    • Observation in mild cases
    • Lubricants for exposure
    • Regular monitoring
  • Surgical Options
    • Orbital implants
    • Volume augmentation
    • Fracture repair if needed

Complications Prevention

  • Regular corneal protection
  • Vision monitoring
  • IOP monitoring
  • Color vision assessment
Further Reading


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