Introduction
Recurrent respiratory papillomatosis (RRP) is a rare viral-induced disease of the upper aerodigestive tract characterized by epithelial lesions. Caused by human papillomavirus (HPV), this disease presents in both children and adults. RRP is considered a benign neoplastic process; however, the disease can lead to significant morbidity and fatal complications, and there is a risk for RRP to transform into an epithelial malignancy ( Fig. 3.1 ).
The prevalence of RRP is approximately 4 in 100,000, with nearly 2000 pediatric cases diagnosed yearly. Juvenile-onset RRP is typically diagnosed prior to age 5 and is considered more “aggressive” than adult-onset RRP, due to the significant impact the RRP lesions can have on the small pediatric airway. In the United States, affected children may undergo more than four procedures yearly and on average nearly 20 in their lifetime. Adult-onset RRP presents most commonly between 20 and 40 years of age (although it can present at any age), with a higher propensity in men. It also typically has a more indolent course with high remission rates.
There are more than 100 HPV subtypes, though HPV 6 and 11 are most commonly associated with RRP. HPV 11 is typically more aggressive in disease progression. Less frequently, HPV 16 and 18 have been linked to RRP, though there is a higher rate of malignant transformation noted with these subtypes. Vertical transmission is seen in juvenile-onset RRP. The route of transmission is not clearly known. Arguments have been made for hematogenous, intrauterine, and direct contact in the birth canal, as patients with juvenile-onset RRP are typically first born and vaginally delivered. Adult onset is associated with reactivation of pediatric disease or secondary to a sexually transmitted disease.
It is theorized that microtears in the epithelium or co-infection with other microbes allows entry of HPV into the body. For example, increased cervical HPV rates are associated with concurrent Trichomonas vaginalis infections. These infections are believed to weaken the tight junctions of epithelial cells. Phonotrauma has been speculated as the etiology for entry into the vocal folds. After this occurs, an immune response is formed, and most individuals are able to clear the infection over the span of 2 years. Five percent of the population has HPV DNA identified in the larynx, though significantly fewer actually display findings consistent with RRP. The mechanism behind this remains unclear. Various factors including epidermal growth factor receptor, cyclooxygenase-2, and prostaglandins are overexpressed in papillomas, and this is currently an area of research for further treatment.
Spread beyond the larynx is associated with increased morbidity and mortality and found in 30% of children and 16% of adults with RRP. The most common sites of extralaryngeal spread include the oral cavity, trachea, and bronchi. Development of pulmonary RRP is linked to high mortality, despite aggressive medical and surgical management.
Key Operative Learning Points
- 1.
Potassium titanyl-phosphate (KTP) laser offers lesion excision, which is especially advantageous for awake, in-office treatment approaches but is also highly effective when used in the operating room (OR).
- 2.
CO 2 laser and cold knife excision can be employed.
- 3.
Office-based KTP laser is an effective method of removing the lesions and restoration of the voice.
- 4.
Microdébriders can be used for bulky disease in the operative setting but should be used less preferentially in the supraglottis and/or subglottis.
Preoperative Period
History
- 1.
Hoarseness without periods of clarity
- 2.
Progressive stridor, cough, dyspnea
- 3.
Recurrent pneumonia may be an indicator of pulmonary involvement.
- 4.
Maternal or paternal history of genital warts
- 5.
Immunization history
Physical Examination
- 1.
Voice evaluation
- a.
Voice evaluation by a speech language pathologist (SLP) can establish a baseline from which to improve. The patient may require voice therapy after or between surgical procedures, and input from an SLP on the initial visit is helpful. However, voice therapy during active treatment is not usually helpful.
- a.
- 2.
Vocal tract endoscopy
- a.
Endoscopy, either through a flexible laryngoscope or 70-degree Hopkins rod, can delineate the extent of disease. Flexible endoscopy affords a view of the nasopharynx, which can often demonstrate concurrent RRP lesions on the pharyngeal surface of the soft palate. Laryngovideostroboscopy can be used as an adjunct to identify more sessile lesions or early recurrences. Chip tip flexible endoscopy can offer better visualization of vascularity and surface contours that often accompany RRP. This technology uses charge-coupled device chips to create digitally based images for endoscopy. Light filters can be employed diagnostically or as an adjunct to office-based KTP laser ablation of RRP.
- a.
- 3.
Tracheoscopy
- a.
In-office evaluation for extralaryngeal disease can be helpful for preoperative planning. Intubation may not be the best choice if subglottic lesions exist, and jet ventilation would need to be pursued.
- a.
- 4.
Auscultation of the lungs
Imaging
Chest radiograph: Not typically indicated but could be used to identify pulmonary disease secondary to obstructive RRP lesions in the lower airways
Computed tomography: Not usually performed unless there is a concern for pulmonary involvement
Additional Testing
- 1.
Pulmonary function tests (PFTs) if there is a concern for pulmonary involvement. Flow loops may be helpful, if there is stridor or dyspnea but no visualized laryngeal disease. It would be used as an adjunct to laryngoscopy and tracheobronchoscopy in cases of more extensive disease.
Indications
- 1.
Dyspnea
- 2.
Dysphonia
- 3.
Globus sensation
Contraindications
None necessary
Preoperative Preparation
- 1.
Discontinue anticoagulant and antiplatelet drugs for 1 week, if possible.
- 2.
Consent for complications: Worsened voice, persistent dyspnea, need for further surgery, infection, injuries associated with suspension laryngoscopy (dental injury, mucosal injury, taste change; see Chapter 2 )
Operative Period
Anesthesia
OR: General, 5.0 microlaryngoscopy endotracheal tube (start with a laser-safe tube if planning on using a laser). This affords good airway control while providing unobstructed visualization of the glottis (see Chapter 2 ).
Office: Topical 4% lidocaine (techniques includes oxymetazoline-lidocaine spray to bilateral nares, insertion of cotton pledgets soaked with 4% lidocaine, nebulizer treatment with 4% lidocaine, topical drip of 4% lidocaine through a channel scope or with an Abraham cannula (see Chapter 1 ).
Positioning
OR: Supine, small round headrest, no shoulder roll, neck flexion may be useful if the larynx is very anterior
Office: Sniffing position, which has the patient rest his or her elbows on thighs, chin out, and nose out as if sniffing a flower
Perioperative Antibiotic Prophylaxis
None necessary
Monitoring
OR: Per anesthesia protocol
Office: Preprocedure vital signs. Brief monitoring for 15 to 20 minutes postprocedure in the waiting room is acceptable; no formal postprocedural monitoring or intraprocedure monitoring is used.
Instruments and Equipment to Have Available
- 1.
KTP laser (or CO 2 laser with pattern generator)
- 2.
Laser precaution equipment
- 3.
Microdébrider (OR)
- 4.
Microlaryngeal instruments
- 5.
Suspension microlaryngoscopy setup
- 6.
Microscope with 400 mm lens
- 7.
Operating chair
- 8.
Laryngotracheal anesthesia
- 9.
Channel flexible laryngoscope (office procedure)
Key Anatomic Landmarks
Anterior commissure: Should be visualized at the time of surgery to evaluate for RRP and also monitor and minimize trauma
Prerequisite Skills
- 1.
Flexible laryngoscopy
- 2.
Direct laryngoscopy
Operative Risks
- 1.
Injury to normal laryngeal structures
- 2.
Intraoperative airway fire
- 3.
Suspension laryngoscopy: Dental injury, mucosal injury, metallic taste. Numbness of the tongue may occur and typically resolves spontaneously over 2 to 6 weeks.
Surgical Technique
- •
OR: Turn the bed 90 degrees. Keep the bed level with a headrest under the head. Apply a tooth guard to protect the maxillary dentition. All exposed facial and upper body skin are covered with wet towels if a laser is to be used. Airway evaluation should now be performed with examination of the oral cavity, oropharynx, hypopharynx, endolarynx, subglottis, and trachea to accurately stage the sites and extent of disease. Use the largest laryngoscope possible. Advance the laryngoscope until optimal exposure of the endolarynx is achieved. Suspend the laryngoscope. Use angled telescopes in 0, 30, and 70 degrees to (1) map out all RRP disease locations and nature, and (2) take photographic documentation. This helps in visualizing the locations of the lesions. The ventricle, undersurface of the vocal folds and anterior and posterior commissures are best visualized with 30- and 70-degree telescopes. The operating microscope is then brought in to further visualize the affected regions. A ½-by 3-inch pledget soaked in saline is placed in the subglottis above the endotracheal tube cuff if the CO 2 laser is to be used. A small amount of papilloma is excised with cold instruments and sent for biopsy. The remaining papillomas are now removed using the surgeon’s method of choice:
- •
KTP laser removal of RRP can be done in several methods.
- 1)
KTP removal of RRP (outside-in)
To minimize the risk of thermal injury to the vocal fold while using the KTP laser for removal of RRP, the KTP laser is fired directly at the RRP, with the laser energy and working distance adjusted to result in a “blanching” effect on the RRP with the KTP laser energy. If the surgeon feels that this treatment effect, KTP-1, will be adequate, then the KTP treatment is done. Additional KTP laser energy can be applied to the operative site, which will cause small epithelial “craters” (i.e., disruption of the epithelium of the RRP tissue). Often this KTP-2 treatment effect will be sufficient for successful RRP treatment. If the RRP is bulky, additional KTP laser energy can be applied (often with laser fiber direct contact), resulting in a KTP-3 treatment effect. If the surgeon’s goal is complete removal of the RRP at the time of surgery, after the entire area of RRP has been “blanched” from a conservative and minimal KTP laser energy delivery approach, a suction (5 or 7 French) is used to remove the treated RRP surface. If there is additional RRP present after the treated disease has been removed by suction or cup forceps, the same use of the KTP laser can be employed until there is no gross RRP present at the operative site (KTP-4).
- 2)
KTP removal of RRP (inside-out)
Working within the plane between the papilloma and the normal underlying true vocal fold tissue, the lesions can be removed in their entirety while staying as superficial as possible. The KTP laser is often used for areas of minimal disease and can be time consuming if used in an angiolytic fashion. Alternatively, it can be used to remove bulky lesions via a dissection technique that seeks to achieve KTP-4 laser effects. This focuses on ablation of the interface between the normal vocal fold and RRP: Use settings of approximately 30 to 35 watts, 15 millisecond pulse width, and 2 to 3 pulses per second. Beginning at an area of transition between RRP and normal epithelium, the laser can be used to blanch this area until it can be sloughed off with another tool such as suction. Once this plane is established, dissection of the tissue can be performed along the entirety of the undersurface of the lesion (see
).
- 1)
- •
“Cold steel” may be used to dissect just below the epithelium and remove the RRP lesion, trying to preserve as much lamina propria as possible.
- •
CO 2 laser with pattern generator may also be considered when available and used to ablate the lesions.
- •
The microdébrider can be employed to remove bulky lesions and especially above and below the true vocal folds; however, it is not recommended to be used aggressively on the true vocal fold. Often the microdébrider is employed to debulk, and the KTP laser or cold steel can resect the lesions on the true vocal fold tissue.
- •
Oxymetazoline or 1:10,000 epinephrine-soaked pledgets are used for hemostasis. At the end of the procedure, laryngotracheal anesthesia is sprayed into the endolarynx for topical anesthesia.
- •
Care must be taken at the anterior commissure of the vocal folds. Leaving some residual RRP on one side and staging the surgery into two parts is often employed to avoid a laryngeal web. Alternatively, when using KTP or CO 2 laser, a small amount of RRP can be blanched but left in place (not suctioned off) on one side of the anterior commissure to slough on its own ( Fig. 3.2 ).
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