Recurrent Respiratory Papillomatosis

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Recurrent Respiratory Papillomatosis

• Michael E. Decherd, MD
• Ronald W. Deskin, MD
• April 7, 1999

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Overview

• Warty growths in aerodigestive tract
• Natural history unknown
• Caused by human papillomavirus
• Can lead to airway obstruction
• Treatment essentially palliative

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History

• 1923 Ullmann injects papilloma into own forearm
• 1956 Association made with maternal condylomata
• 1972 CO2 Laser used for debulking
• 1973 Virus identified in electron micrographs

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Basic Science

• Icosahedral, naked, supercoiled DNA
• Papovavirus family
• PApillomavirus (HPV)
• POlyoma
• JC virus -- progressive multifocal
  leukencephalopathy
• BK virus -- isolated from urine of renal
  transplant patients
• siman VAculolating virus

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Basic Science, cont.

• Over 90 subtypes
• Subtypes arranged in families by DNA homology
• RRP usually HPV 6,11
• HPV 16, 18 implicated in cervical cancer

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Epidemiology

• Bimodal
• juvenile, 75 by age 5
• incidence 4.3/100,000
• adult, usually young adulthood
• incidence 1.8/100,000

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Epidemiology, cont

• Clinical Triad
• firstborn
• teenage mother
• vaginal delivery
• Cost 151 million annually

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Transmission

• Acquired during vaginal birth
• 10-25 childbearing-age women with HPV in
  cervical swabs
• Hematogenous
• Ascending
• Contact with anogenital lesions

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Cesarean?

• Shah 109 children with RRP, only 1 born by
  C-section
• No consensus
• Literature does not generally endorse in absence
  of other indications

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Diagnosis

• Symptoms
• Hoarseness
• Weak cry
• Chronic cough
• Stridor
• Visualize lesions
• Biopsy

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Histology

• Epithelial projection with fibrovascular core
• Viral histological findings
• parakeratosis
• acanthosis
• koilocytosis
• Virus demonstrated in normal epithelium

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Location (Squamocolumnar junction)

• Limen vestibuli
• Nasopharyngeal surface soft palate
• Midzone, laryngeal surface of epiglottis
• Upper and lower margins of ventricle
• Undersurface of ventricles
• Carina and bronchial spurs

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Course

• Variable by patient
• Frequent debulking mainstay of Rx
• Avoid damage to normal tissue
• May require tracheotomy
• ?predisposes to distal spread

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Course, cont.

• Spontaneous remission
• 1/3 by age 40
• Distal disease -- bad
• Malignant degeneration

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Anesthesia

• Apnea
• Laser tube
• Jet ventilation
• Spontaneous ventilation

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Treatment, Surgical

• Cold steel, less common
• CO2 Laser
• protect airway from fire
• protect health care personnel
• laser masks
• adequate suction

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Treatment, Medical

• Interferon
• I3C
• Acyclovir
• Retinoids
• Photodynamic therapy
• Methotrexate
• Cidofovir
• Ribavirin

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Alfa-Interferon

• Derived from leukocytes, now recombinant
• Dose 3MU per m2 BSA
• Bad side effects
• Healy Initial benefit seen at 6 mo. not
  sustained at one year
• Neutralizing antibodies may play a role

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Indole-3-carbinol

• Derived from cruciferous vegetables
• Over-the-counter
• Dose 200-400 mg adults, 100-200 mg kids
• well-tolerated
• Affects estrogen metabolism

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Acyclovir

• Thought that Herpesvirus coinfects many cases
• Limited studies

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Retinoids

• Vitamin A derivative
• Alters epithelial differentiation
• May synergize with interferon

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Photodynamic Therapy

• Patient is given IV photosensitizing agent
• Concentrated in proliferating tissues
• Ablated with argon dye laser
• Photosensitivity limits usefulness, lasts
  weeks/months

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Conclusion

• Morbid disease we still do not understand
• Treatment is debulking, airway protection
• Future therapies are under investigation