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0 1998 The American Laryngological, Rhinological and Otological Society, Inc.

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How I Do It

Laryngology and Bronchoesophagology

A Targeted Problem and Its Solution

A Staging System for Assessing Severity of Disease and Response to Therapy in Recurrent Respiratory Papillomatosis
Craig S. Derkay, MD; David J. Malis, MD; George Zalzal, MD; Brian J. Wiatrak, MD; Haskins K. Kashima, MD; Marc D. Coltrera, MD

INTRODUCTION
Recurrent respiratory papillomatosis (RRP) is a perplexing and frustrating disease for both the families it afflicts and the physicians who care for them. Although RRP is a benign disease of viral etiology (most commonly HPV it has potentially morbid consequences owtypes 6 and ll), ing to its involvement of the airway and the risk of malignant conversion. Treatment of RRP has been mainly surgical over the past half century, relying on operative debulking, although adjuvant medical therapies have been utilized for recalcitrant cases. Among the most frustrating aspects of this disease is the observation that whereas some patients demonstrate limited disease with an infrequent need for intervention, others are confronted with recurrent airway compromise and a repeated need for laser surgery. Although it is considered the most common benign neoplasm of the larynx,l RRP is an orphan disease with an incidence in the United States estimated at between 1500 and 2500 new cases per year.2 Owing to the relative paucity of cases and the complicated nature of their treatment, the

Presented a t the Meeting of the Southern Section of the American Laryngological, Rhinological and Otological Society Inc., Orlando, Florida, January 16,1998. From the Department of Otolaryngology Head-Neck Surgery, Eastern Virginia Medical School (c.s.D.), Norfolk, Virginia, the Department Otolaryngology-Head Neck Surgery, Brooke Army Medical Center (D.J.M.), San Antonio, Texas, the Department of Pediatric Otolaryngology, Childrens National Medical Center (c.z.), Washington, DC, the Department of Surgery/Pediatric Otolaryngology, University of Alabama at Birmingham (B.J.w.), Birmingham, Alabama, the Department of Otolaryngology-Head Neck Surgery, Johns Hopkins School of Medicine (H.K.K.), Baltimore, Maryland, and the Department of Otolaryngology-Head and Neck Surgery, University of Washington-Seattle (M.D.c.), Seattle, Washington. Send Correspondence to Craig S. Derkay, MD, Department of Otolaryngology-Head Neck Surgery, Eastern Virginia Medical School, 825 Fairfax Avenue, Suite 510, Norfolk, VA 23507, U.S.A.

majority of children with RRP are cared for in universities, major medical centers, and childrens hospitals. Although several scoring and staging systems have been proposed, clinicians and researchers have not yet adopted a uniformly acceptable nomenclature for describing RRP lesions that is simple yet comprehensive. This has created confusion in the RRP literature and in physician-to-physician communications regarding patients response to therapies. In addition, the absence of a universally accepted staging system has hampered our abilities to accurately report the results of adjuvant therapies or document the natural course of the disease. In conjunction with the Centers for Disease Control and Prevention-sponsored Multi-Institutional Task Force on RRP, the Collaborative Anti-Viral Study Group HPV Subcommittee, and the authors of the most widely used current severity scales, we propose a new severityktaging system for RRP. This format incorporates the best qualities of the existing systems by numerically grading the extent of papillomatosis a t defined aerodigestive subsites, assesses functional parameters, diagrammatically catalogs subsite involvement, and assigns a final numeric score to the patients current extent of disease. Utilizing software designed at the University of Washington (Seattle, WA) and licensed to the American Society of Pediatric Otolaryngology, this staging system is now computerized and available to pediatric otolaryngologists and bronchoesophagologists to allow them to objectively and subjectively measure an individual patients clinical course and response to therapy over time.

TECHNIQUE
This stagingheverity scale (Fig. 1)can be used either manually (with a form stored in the operating suite and attached to the patients chart), via the computerized softDerkay et al.: Recurrent Respiratory Papillomatosis

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STAGING ASSESSMENT FOR RECURRENT LARYNGEAL PAPILLOMATOSIS

PATIENT INITIALS:____ DATE OF SURGERY_________ SURGEON____________ PATIENT ID #____________ INSTITUTION__________ 1. How long since the last papilloma surgery? ____days, ---weeks, --months, ---years ,___dont know, ----this is the childs first surgery 2. Counting todays surgery, how many papilloma surgeries in the past 12 months? 3. Describe the patients voice today: normal--(O), abnormal--( 1 ), aphonic--(2) 4. Describe the patients stridor today: absentJO), present with activity--( 1), present at restL-(2) 5. Describe the urgency of todays intervention: scheduled-_(O),eIective--( 1),urgent__(Z),emergent(3) 6. Describe todays level of respiratory distress: none_-(O), mild_-(l), Mod--(2), severe--(3), extreme--(4) Total score for questions 3-6=--_--

._

FOR EACH SITE, SCORE AS: O= NONE, 1= SURFACE LESION, 2=RAISED LESION, 3=BULKY LESION LARYNX: Epiglottis Lingual surface____ Aryepiglottic folds: Right--False vocal cords: Right-True vocal cords: Right--Arytenoids: Right____ Anterior commissure-----Posterior commissure------_____-Subglottis

Laryngeal surface_____ Left---Left---Left_____ Left _____

TRACHEA Upper one-third ___________ Middle one-third___________ Lower one-third___________ Bronchi: Right--Left ____ Tracheotomy stoma__________ OTHER: Nose---Palate----_ Pharynx---Esophagus_--Lungs-----Other_______

..................................................................
TOTAL SCORE ALL SITES:

______

TOTAL CLINICAL SCORE:-----

Fig. 1. Stagingkeverity scale.

ware (both IBM- and Macintosh-compatible for laptop o r desktop computers), or with both methods. In a fashion similar to Kashima et al.3 and Wiatrak,4 the operating surgeon assigns a score of 0 to 3 (0 = absent, 1 = surface lesion, 2 = raised lesion, and 3 = bulky lesion) to each site in the aerodigestive tract. A composite score is generated by summing the scores a t each involved site. In addition, the surgeon denotes the laryngeal lesions on a standardized diagram, indicates sites of biopsy and laser treatment, documents sites in which adjuvant drug therapy has been administered, and answers six questions regarding the patients clinical course (interval of surgery, total number of recent surgeries, urgency of this surgery, quality of voice, degree of stridor at the time of this surgery, and degree of respiratory distress). A clinical score is generated by summing the scores for each of the subjective
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assessments. The severity rating (score) is automatically tabulated for the surgeon in the computerized version. The process requires less than 5 minutes, creates a record that accurately reflects disease status, assures complete data collection that is suitable for data analysis, and is sensitive enough to detect subtle changes over time in the patients clinical status.

DISCUSSION
A standard system of objective scoring of RRP disease was designed to provide the clinician and RRP researcher with an accurate evaluation of disease severity a t any single observation and to assess disease course over time. The concept of a uniform staging/grading system was first introduced by Kashima et al.3 as part of the Papilloma Study Group multi-institutional interferon
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study and continues to be used by several of the original participants. However, Kashima's system has not gained universal acceptance because it suffers from limited laryngeal subsite information (including a lack of choice of side of involvement), subjectivity in deciding the percentage of airway lumen encroachment, and the absence of any clinical measure of disease severity. Lusk et al.5 proposed a system for estimating the volume of laryngeal papilloma occluding the glottic airway by dividing the right and left halves of the glottis into three equal parts. Their system fa& to take into consideration disease outside of the larynx and suffers from a high degree of potential subjectivity among observers. Lusk's system, too, has no functional component. Zalzal (Zalzal G, Personal communication) and others have utilized an intraoperative laryngeal diagram to serially record disease involvement with their RRP patients. Although convenient for the individual surgeon, this method is not well suited for reporting of results among a cohort of patients. The diagram also does not accommodate disease outside of the larynx and tells little about the clinical status of the patient. Wiatrak4 has adopted a modification of the Kashima method that incorporates more anatomical sites and a subjective severity rating. It differs from the current proposal in its scoring scale, the exact anatomical sites reported, and our addition of functional measures of assessment.

the previously devised methods is proposed. The system has been computerized to add to its ease of usage and uniformity.The software is available through the American Society of Pediatric Otolaryngology for use by its members and their colleagues in bronchoesophagology. It is hoped that this tool will strengthen the efforts to develop a national registry of RRP patients and enhance future RRP research endeavors by simplifying nomenclature and identifying potential research subjects from across the nation.

ACKNOWLEDGMENT
The authors would like to thank the OTOBASE development team a t the University of Washington (Seattle, WA) for their selfless efforts on behalf of RRP patients everywhere and the executive board of the American Society of Pediatric Otolaryngologyfor their generosity in providing this software to their membership.

BIBLIOGRAPHY
1. Jones SR, Myers EM, Barnes L. Benign neoplasms of the larynx. Otolaryngol Clin North Am 1984;17:151-62. 2. Derkay CS. Multi-disciplinary Task Force on Recurrent Respiratory Papillomas: a preliminary report. Arch Otolaryngo1 Head Neck S u r g 1995;12:1386-91. 3. Kashima H, Leventhal B, Mounts P, Papilloma Study Group. Scoring system to assess severity and course in recurrent respiratory papillomatosis. In: Howley PM, Broker TR, eds. Papillomauiruses; Molecular and Clinical Aspects. New York: Alan R Liss; 1985:125-35. 4. Wiatrak BJ. Recurrent respiratory papilloma scoring scale. In press. 5. Lusk RP, McCabe BF, Mixon JH. Three-year experience of treating recurrent respiratory papilloma with interferon. Ann Otol Rhino1 Laryngol 1987;96:158-62.

CONCLUSION A comprehensive, simple, and widely available system

for assessing severity of disease and response to therapy in patients with RRP that incorporates the best attributes of

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