Summary
Juvenile onset recurrent respiratory papillomatosis (JORRP) is caused by human papillomavirus (HPV) types 6 and 11. Transmission is believed to occur vertically from mother to newborn at birth with slow and progressive growth of the papilloma leading to hoarseness and airway obstruction. At present there is no proven medical therapy to “cure” this infectious disease, and the mainstay of treatment is surgical debulking and use of a variety of adjuvant treatments. The goals of treatment stress conservative resection avoiding injury to critical laryngeal structures, achieving a safe and patent airway, preserving voice quality and avoiding the need for a tracheotomy, while prolonging the intersurgical interval. Multiple modalities are available for surgical resection of JORRP with no single option proving to be superior. Indeed, multiple modalities are often combined at any single procedure to optimize the debulking while ensuring no injury to the larynx. It is critical to be fully prepared for an airway emergency and to have all equipment available prior to starting the case. Since surgical resection is not curative, a variety of “adjuvant” treatments have been used in an effort to achieve durable remission or reduce disease burden. Cidofovir and Bevacizumab (Avastin) are the two most widely used intralesional injection “adjuvant” therapies. In the past it was felt appropriate to wait for “severe” disease to be present, requiring repetitive intervention for airway management prior to introducing “adjuvant” therapy. However, the senior author has advocated for early introduction of adjuvant medical therapy to help reduce the number of procedures, and reduce the likelihood of iatrogenic injury to laryngeal structures and potentially achieve durable remission. Communication and coordination with anesthesia for these procedures is critical to successfully managing the airway, as the “shared airway” concept is overtly at play in these patients. Medical personnel should be familiar with HPV as a sexually transmitted disease and understand that the future management of this disease is prevention through vaccination prior to exposure to the virus.
48 Surgical Approach to Juvenile Onset Recurrent Respiratory Papillomatosis
48.1 Introduction
48.1.1 General
Juvenile onset recurrent respiratory papillomatosis (JORRP) is the most common benign neoplasm of the aerodigestive tract. These lesions are caused by human papillomavirus (HPV) in a process marked by slow, progressive growth leading to voice changes with persistent hoarseness and, if not managed, airway obstruction. Though infectious in etiology, no proven predictive curative medical therapies have arisen to date, with surgical debulking for airway and voice preservation being the mainstay of treatment. Disease presentation varies greatly in both extent and location; lesions may be sessile or pedunculated, located in multiple locations within the airway, and focal, multifocal, or diffuse. A key characteristic of JORRP is its persistence in the basal epithelium, resulting in recurrence of the lesions or expression in previously normal-appearing tissue. The natural history is that of persistent and recurrent disease that usually requires multiple surgeries, which often can lead to both physical impairment and psychological trauma.
Although there are many surgical modalities available to treat JORPP, given the variability of involvement of laryngeal structures, no single option is best to debulk the diseased areas. Being knowledgeable about and having available a combination of tools and techniques ensure the optimal management of each unique presentation of disease. Unfortunately, surgical resection of papilloma does not lead to “cure.” The average number of surgeries to remission lies near 20, and it is not uncommon for patients to have 70 to 100 or more lifetime surgeries for this condition. 1 To achieve optimal outcome, the discerning surgeon must account for the nature of the disease, assess the needs and goals of the individual patient, and guide the approach appropriately. This translates into removing disease while avoiding iatrogenic damage to the airway. True remission is likely brought about by as yet unidentified immunologic factors (▶ Fig. 48.1, ▶ Fig. 48.2, and ▶ Fig. 48.3).
48.1.2 Pathophysiology
JORRP is caused by HPV types 6 and 11. The infection is widely considered to be transmitted vertically from mother to newborn during childbirth through an infected cervix/vagina. However, delivery by C-section is not fully protective and, although far less common, C-section births can develop JORRP. Respiratory papilloma initially presents as exophytic cauliflower-like growths primarily at and around the vocal folds, but can involve both the supraglottic and subglottic/tracheal airway. Common sites of disease include: the upper and lower ventricle margins, undersurface of the vocal cord, laryngeal surface of the epiglottis, carina, bronchial spurs, nasal limen vestibule, and the nasopharyngeal surface of the soft palate. 2
48.1.3 Epidemiology
Incidence of RPP in the United States has traditionally been estimated at 4.3 per 100,000 in children and 1.8 per 100,000 in adults. However, with the introduction and wide implementation of HPV vaccination, these numbers are believed to be decreasing. 3
48.1.4 Presentation
Initial presentation in the child occurs on average at 3 years of age, with 75% of patients diagnosed by age 5. 4 The most common presenting symptom is varying severity of progressive dysphonia, or in severe cases, progressive inspiratory or biphasic stridor. Misdiagnosis is common, as presentation may be mistaken for asthma, recurrent croup, or bronchitis. Definitive diagnosis is often delayed for up to one year from initial symptomatology.
48.1.5 Clinical Assessment
In the child with hoarseness, stridor, or airway symptoms, it is critical to distinguish JORRP from other causes of these symptoms. A comprehensive history will address the nature and timing of symptoms, history of prior airway trauma or intubation, congenital anomalies or prematurity, significant comorbidities such as gastroesophageal reflux or history of “recurrent croup”, and associated symptoms. Maternal history of condyloma acuminatum or abnormal Pap testing should be elicited.
Unless acute respiratory distress is present, flexible nasolaryngoscopy with video recording should be done to identify areas of involvement and extent of disease. These images can be reviewed with the parents and shared with anesthesia prior to surgery.
Patients presenting with acute respiratory distress should be stabilized in an emergency department and taken directly to the operating room for management.
48.1.6 Patient Discussion
Once the diagnosis is confirmed histologically, the surgeon should meet with the family to discuss the natural history of JORRP along with expectations and goals of treatment, including the anticipated need for repetitive surgical debulking. Adjuvant therapies can be discussed and a schedule of planned interventions created. The impact of this chronic disease, including the potential affect on voice and the emotional and psychological burden to the patient and family should be addressed. Therefore, expectations ought to be properly framed for the patient: treatment may not be curative, repeat operations are expected, and papilloma may be left behind initially and serially removed in an effort to minimize scarring and long-term mucosal changes.
48.2 OR Preparation
The removal of laryngeal papilloma can be both challenging and stressful for all operating room personnel. Even experienced endoscopists will encounter cases that require the ability to manage an unexpectedly compromised airway.
Prior to the patient entering the OR, it is the responsibility of the surgeon to select and inspect the appropriate instrumentation, brief all involved operative personnel as to the status of the airway, and establish a well-developed plan with anesthesia with contingencies for “the unexpected.”
Specifically, the surgeon and anesthesiologist must partner and discuss how best to share the airway. The goals of anesthesia include providing alveolar ventilation and anesthesia through a potentially compromised airway, while the goals of the otolaryngologist are to obtain an unobstructed view of the airway to permit removal of disease and avoid injury to non-involved tissue. To achieve these goals, the anesthesiologist should be shown a picture or drawing of the involved portion of the airway and review the degree of airway compromise. This permits a thorough discussion regarding an agreed-upon approach to the anesthetic technique (spontaneous ventilation vs. other techniques). The use of paralytic agents, such as succinylcholine, should be discussed as loss of muscle tone in a markedly obstructed larynx could result in the inability to ventilate the patient and precipitate an airway crisis. Fire risks for laser and management of FiO2 during the procedure should also be reviewed. Ideally both the anesthesiologist and otolaryngologist should be comfortable with difficult airway management and spontaneous ventilation technique to best share and manage the airway.
48.3 OR Setup
The OR setup may vary from OR to OR but requires having all possibly needed instruments set up and available. The basic tools needed for RRP endoscopy are listed in ▶ Table 48.1. The video monitors should be placed such that the surgeon, the scrub nurse, and anesthesiologist can see the airway during the procedure. The setup should have the O2 saturation monitor near the surgeon and loud enough for the surgeon to hear.
Instruments |
Telescopes: 0° and 30° |
Bronchoscopes of appropriate size |
Laryngeal spreader |
Ventilating laryngoscopes: Parsons; Benjamin-Lindholm |
Cup forceps: straight, angled and up biting; small and large sizes |
Various sized laryngeal suctions |
Cotton pledget (radiographic markers) |
Topical adrenaline or oxymetazoline for hemostasis |
Monitor and picture/video capability |
Additional supplies for laser therapy |
Water basin filled with saline |
Cloth wrapping for patient facial protection |
Eye protection for OR personnel |
48.3.1 Tools for Removal of Papilloma
Due to the variability of disease severity and location, there is no single universal modality for papilloma removal. Rather than taking a one-size-fits-all approach, individualized therapy means choosing the best combination of tools and techniques to meet the stated goals of conservative treatment. Bulky and sessile disease will be best managed with different tools. See ▶ Table 48.2 for summary of advantages and disadvantages of each tool.
Microdebrider: Ideal for addressing bulky exophytic disease rapidly and efficiently. Sessile lesions and papilloma located within the ventricles or at the anterior commissure may be difficult to remove effectively and safely with the microdebrider. There are two sizes of rotating blades (2.9 and 3.5) and two types of blades available (skimmer and cutting). Most often the 2.9 skimmer blade will be used at a setting of 500 to 800 RPM. There is a learning curve when first utilizing the microdebrider to ensure no damage to normal tissue. Bleeding that occurs is best controlled with topical adrenaline or oxymetazoline on cotton pledgets (▶ Fig. 48.4).
CO2 laser: CO2 laser ablates tissue via thermal destruction. 5 These lasers have been used for >40 years and are familiar to most laryngeal surgeons. Although still available to be coupled to a microscope with a micromanipulator, visualization with this approach is limited to line-of-sight only. Many prefer the use of a flexible fiber placed through an appropriate hand piece or suction. Working with telescopes, the flexible fiber permits access to difficult to visualize areas and is excellent for removal of sessile lesions. It is manually focused 1 to 2 mm above the papilloma and permits precision ablation of the lesions. Using the laser on bulky disease may be time consuming and the heat generated and depth of laser penetration (100–300 µm) may cause thermal injury. However, the laser can be used as a surgical knife to separate the papillomatous lesion from the underlying tissue if a clean tissue plane is available. It is best to reduce the wattage and use in pulsed delivery mode to reduce injury to deeper tissue planes (▶ Fig. 48.5).
532 nm Potassium-titanyl-phosphate (KTP) laser: An angiolytic laser which penetrates epithelium to photocoagulate microvasculature. The 532-nm wavelength targets oxyhemoglobin, disrupting blood supply while achieving hemostasis and sparing surrounding tissue. 6 The 400 to 600 µm depth of penetration is ideal for blood vessel ablation. The KTP laser settings include power, pulse width (ms), and pulse frequency. Classic settings are 30 to 35 W/15 ms/2 Hz. The KTP is available with 0.4- and 0.6-mm-sized fibers. As hemoglobin absorbs this laser wavelength, it is best used when there is no bleeding present. Bulky lesions can be difficult to manage with the KTP, yet the various benefits include the ability to manage sensitive areas such as the anterior commissure and true vocal cord, access of difficult-to-reach regions such as the ventricles or infraglottis, and its precision for small lesions and tissue-sparing properties (▶ Fig. 48.6).
Laser safety: Due to risk of catastrophic fire, the use of laser technology requires additional intraoperative personnel, equipment, and patient precautions. The Association of Surgical Technologists’ Standards of Practice for Laser Safety includes the following measures:
A well-trained laser safety officer, eye protection for both OR personnel and patient, personal protective equipment, and a nonflammable teeth protector.
To prevent accidental injury to the eyes and face, the patient’s head should be wrapped in a moistened towel. Combustible material must not be present in the airway when a laser is used, including an endotracheal tube (ETT) or cotton pledgets. If the patient cannot be managed with spontaneous ventilation, an appropriate laser-resistant wrapped ETT must be used. Prior to the surgeon initiating use of the laser, the anesthesiologist should reduce the inhaled fraction of inspired oxygen (FiO2) to the minimum amount tolerated by the patient to maintain safe hemoglobin oxygen saturation. A syringe filled with saline and a basin filled with sterile water should be immediately available for operative personnel to rapidly extinguish an airway fire. 7 , 8
Laryngeal cold ablation (Coblation): A novel method using localized radio frequency energy transduction to denature viral proteins and destroy papilloma without compromising hemostasis. 9 Best used at a power level of 7 to 9 with a slow drip of saline to ensure an appropriate environment for the coblation plasma field. To ablate papilloma, place the tip of the wand over the lesions at a 90° angle where possible to minimize surrounding thermal damage. Coblation wands come in pediatric and adult sizes with one or three electrodes, respectively. Classically the smaller pediatric wand is used in JORRP patients. Depending on the location of the papilloma, one may see tissue quivering when the energy is applied (▶ Fig. 48.7, ▶ Fig. 48.8, and ▶ Fig. 48.9).
Cup forceps: The primary surgical mode prior to advent of the laser, cup forceps are used to take biopsies for histopathologic assessment and HPV typing, remove larger bulky exophytic lesions, or to remove small irregular tissue that remains after using other modalities. Straight, up-biting and left or right-angled cup forceps are available. The cup grasps the lesion and gently separates the abnormal tissue from the underlying epithelium. If the papilloma does not detach easily with gentle force, release grip and reposition to prevent underlying tissue damage. Bleeding can occur and, as with a microdebrider, is controlled with topical hemostatic agents on cotton pledgets (▶ Fig. 48.10).
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