Vascular Tumors in the Neonate

Key Concepts

• Most common soft tissue tumors in infancy
• Range from benign to life-threatening lesions
• Can be isolated or part of syndromes
• Dynamic growth characteristics
• Location determines risk and management

Epidemiology

• Incidence: 4-5% of infants
• Female predominance (3:1)
• Risk factors:
  • Prematurity
  • Low birth weight
  • Multiple gestation
  • Advanced maternal age
  • Placental abnormalities

ISSVA Classification

Benign Vascular Tumors

• Infantile Hemangiomas (IH):
  • Superficial
  • Deep
  • Mixed
  • Reticular/abortive
  • Others
• Congenital Hemangiomas:
  • Rapidly Involuting (RICH)
  • Non-Involuting (NICH)
  • Partially Involuting (PICH)
• Tufted Angiomas
• Spindle Cell Hemangiomas

Locally Aggressive Tumors

• Kaposiform Hemangioendothelioma (KHE)
• Retiform Hemangioendothelioma

Malignant Vascular Tumors

• Angiosarcoma
• Composite Hemangioendothelioma

Growth Characteristics

• Proliferative Phase:
  • Rapid growth (0-12 months)
  • VEGF-dependent
  • Increased endothelial turnover
• Involuting Phase:
  • Gradual regression
  • Apoptosis-driven
  • Variable duration

Clinical Assessment

History Taking

• Time of appearance
• Growth pattern
• Associated symptoms
• Family history
• Pregnancy complications

Physical Examination

• Morphology:
  • Color
  • Texture
  • Consistency
  • Border characteristics
• Distribution:
  • Focal vs segmental
  • Single vs multiple
  • Anatomic location

Imaging Studies

• Ultrasound:
  • First-line imaging
  • Doppler flow patterns
  • Depth assessment
  • Tissue characterization
• MRI:
  • Extent definition
  • Flow dynamics
  • Tissue composition
  • Associated anomalies
• Other Modalities:
  • CT angiography
  • Nuclear medicine studies
  • Angiography

Treatment Approaches

Active Monitoring

• Indications:
  • Small, uncomplicated lesions
  • No functional impairment
  • Expected spontaneous involution
• Monitoring Schedule:
  • Regular photography
  • Growth measurements
  • Complication surveillance

Medical Management

• Beta-blockers:
  • Propranolol (first-line)
  • Dosing: 2-3 mg/kg/day
  • Duration: 6-12 months
  • Monitoring requirements
• Corticosteroids:
  • Systemic
  • Intralesional
  • Topical
• Other Agents:
  • Sirolimus
  • Vincristine
  • Interferon

Interventional Procedures

• Laser Therapy:
  • Pulsed dye laser
  • Nd:YAG laser
  • CO2 laser
• Surgery:
  • Excision
  • Debulking
  • Reconstructive procedures
• Embolization:
  • Arterial embolization
  • Sclerotherapy

Complications & Special Considerations

Life-threatening Complications

• Kasabach-Merritt Phenomenon:
  • Profound thrombocytopenia
  • Coagulopathy
  • Associated with KHE
• Airway Compromise:
  • Subglottic lesions
  • Neck involvement
  • Emergency management
• High-output Cardiac Failure:
  • Large lesions
  • Multiple lesions
  • Hepatic involvement

Functional Complications

• Visual:
  • Amblyopia
  • Astigmatism
  • Strabismus
• Feeding difficulties
• Speech problems
• Musculoskeletal issues

Long-term Follow-up

• Growth monitoring
• Psychosocial support
• Cosmetic outcomes
• Functional assessment
• Quality of life evaluation

Associated Syndromes

• PHACE Syndrome:
  • Posterior fossa anomalies
  • Hemangiomas
  • Arterial anomalies
  • Cardiac defects
  • Eye abnormalities
• LUMBAR Syndrome
• Blue Rubber Bleb Nevus Syndrome