Laryngeal papillomatosis, also known as recurrent respiratory papillomatosis (RRP), is a rare, benign neoplastic disease characterized by the growth of papillomas (warts) in the larynx and other areas of the respiratory tract.
Epidemiology
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1. Incidence:
RRP is a rare disease, with an estimated incidence of 4.3 per 100,000 children and 1.8 per 100,000 adults.
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2. Age:
RRP can affect individuals of all ages, but it is more common in children under 5 years old (juvenile-onset RRP) and adults between 20-40 years old (adult-onset RRP).
Pathophysiology
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1. Papillomas:
RRP is characterized by the growth of benign papillomas in the larynx, trachea, and other areas of the respiratory tract.
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2. Common sites:
The most common sites affected are the vocal cords, followed by the supraglottis, subglottis, and trachea.
Viral Biology of HUMAN PAPILLOMA VIRUS (HPV):
HPV 6 and HPV 11 are types of human papillomavirus (HPV) that belong to the Alphapapillomavirus genus.
Characteristics
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1. Low-risk HPV types:
HPV 6 and HPV 11 are considered low-risk types, primarily associated with benign lesions.
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2. DNA virus:
HPV is a double-stranded DNA virus.
Genome Structure
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1. Circular genome:
The HPV genome consists of a circular double-stranded DNA molecule.
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2. Early (E) and late (L) genes:
The HPV genome contains early genes (E1-E7) involved in viral replication and transcription, and late genes (L1-L2) encoding capsid proteins.
Viral Replication
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1. Infectious cycle:
HPV infects basal epithelial cells, and the viral genome is replicated in the nucleus.
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2. Viral shedding:
HPV is shed from the surface of infected epithelial cells.
Disease Association
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1. Genital warts:
HPV 6 and HPV 11 are commonly associated with genital warts (condyloma acuminatum).
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2.Recurrent respiratory papillomatosis (RRP):
HPV 6 and HPV 11 can also cause RRP, a rare disease characterized by benign papillomas in the respiratory tract.
Transmission
The transmission of the disease can occur through:
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1. Sexual transmission:
Sexual transmission: HPV 6 and HPV 11 can be transmitted through sexual contact.
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2. Vertical transmission:
Vertical transmission: HPV can be transmitted from mother to child during childbirth, potentially leading to RRP in infants.
Immune Response
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1.Cell-mediated immunity:
Cell-mediated immunity plays a crucial role in controlling HPV infection.
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2. Humoral immunity:
Antibodies against HPV can provide some protection against infection.
Understanding the viral biology of HPV 6 and HPV 11 is essential for developing effective prevention and treatment strategies.
Symptoms and Signs
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1. Hoarseness:
Changes in voice quality or hoarseness are common symptoms.
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2. Respiratory symptoms:
Patients may experience stridor, wheezing, or shortness of breath.
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3. Dysphagia:
Difficulty swallowing can occur if the papillomas obstruct the airway.
Investigations
The investigations typically include the following:
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1.Laryngoscopy:
Flexible or rigid laryngoscopy is used to visualize the larynx and diagnose RRP.
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2. Biopsy:
A biopsy may be performed to confirm the diagnosis and rule out other conditions.
Complications
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1. Airway obstruction:
Large papillomas can obstruct the airway, leading to respiratory distress.
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2. Voice changes:
RRP can cause persistent voice changes or hoarseness.
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3. Malignant transformation:
Rarely, RRP can undergo malignant transformation to squamous cell carcinoma.
Management
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1. Surgical management:
Surgical removal of papillomas is the primary treatment for RRP.
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2. Adjuvant therapies:
Adjuvant therapies, such as antiviral medications (e.g., cidofovir) or interferon therapy, may be used to reduce the frequency of surgical interventions.
Surgical Management
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1. Microdebrider:
A microdebrider is a surgical instrument used to remove papillomas.
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2. CO2 laser:
CO2 laser surgery is another option for removing papillomas.
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3. Other surgical techniques:
Other surgical techniques, such as cold instrumentation or pulsed dye laser, may be used depending on the location and extent of the papillomas.
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