The Laryngoscope
Lippincott-Raven Publishers, Philadelphia
0 1998 The American Laryngological,
Rhinological and Otological Society, Inc.
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 per-
plexing and frustrating disease for both the families it af-
flicts and the physicians who care for them. Although RRP
is a benign disease of viral etiology (most commonly HPV
types 6 and ll),it has potentially morbid consequences ow-
ing to its involvement of the airway and the risk of malig-
nant conversion. Treatment of RRP has been mainly surgi-
cal 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, East-
ern Virginia Medical School (c.s.D.), Norfolk, Virginia, the Department Oto-
laryngology-Head Neck Surgery, Brooke Army Medical Center (D.J.M.),
San Antonio, Texas, the Department of Pediatric Otolaryngology, Chil-
dren’s 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, Mary-
land, 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 Oto-
laryngology-Head Neck Surgery, Eastern Virginia Medical School, 825
Fairfax Avenue, Suite 510, Norfolk, VA 23507, U.S.A.
Laryngoscope 108: June 1998
~-
majority of children with RRP are cared for in universities,
major medical centers, and children’s hospitals.
Although several scoring and staging systems have
been proposed, clinicians and researchers have not yet
adopted a uniformly acceptable nomenclature for describ-
ing RRP lesions that is simple yet comprehensive. This
has created confusion in the RRP literature and in physi-
cian-to-physician communications regarding patient’s re-
sponse to therapies. In addition, the absence of a univer-
sally 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 quali-
ties of the existing systems by numerically grading the ex-
tent of papillomatosis a t defined aerodigestive subsites,
assesses functional parameters, diagrammatically cata-
logs subsite involvement, and assigns a final numeric
score to the patient’s current extent of disease. Utilizing
software designed at the University of Washington (Seat-
tle, WA) and licensed to the American Society of Pediatric
Otolaryngology, this staging system is now computerized
and available to pediatric otolaryngologists and bronchoe-
sophagologists to allow them to objectively and subjec-
tively measure an individual patient’s 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 patient’s chart), via the computerized soft-
Derkay et al.: Recurrent Respiratory Papillomatosis
935
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 ,___don’t know,
----this is the child’s first surgery
2. Counting today’s surgery, how many papilloma surgeries in the past 12 months? - ._
3. Describe the patient’s voice today:
normal--(O), abnormal--( 1 ), aphonic--(2)
4. Describe the patient’s stridor today:
absentJO), present with activity--( 1), present at restL-(2)
5. Describe the urgency of today’s intervention:
scheduled-_(O),eIective--( 1),urgent__(Z),emergent(3)
6. Describe today’s 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____ Laryngeal surface_____
Aryepiglottic folds: Right--- Left----
False vocal cords: Right-- Left----
True vocal cords: Right--- Left_____
Arytenoids: Right____ Left _____
Anterior commissure------
Posterior commissure------
Subglottis -_____--
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 standard-
ized diagram, indicates sites of biopsy and laser treat-
ment, documents sites in which adjuvant drug therapy
has been administered, and answers six questions regard-
ing the patient’s clinical course (interval of surgery, total
number of recent surgeries, urgency of this surgery, qual-
ity of voice, degree of stridor at the time of this surgery,
and degree of respiratory distress). A clinical score is gen-
erated by summing the scores for each of the subjective
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
patient’s clinical status.
DISCUSSION
A standard system of objective scoring of RRP dis-
ease was designed to provide the clinician and RRP re-
searcher 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 sys-
tem was first introduced by Kashima et al.3 as part of the
Papilloma Study Group multi-institutional interferon

Laryngoscope 108: June 1998 Derkay et al.: Recurrent Respiratory Papillomatosis

936
 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 la-
ryngeal subsite information (including a lack of choice of
side of involvement), subjectivity in deciding the percent-
age of airway lumen encroachment, and the absence of
any clinical measure of disease severity. Lusk et al.5 pro-
posed a system for estimating the volume of laryngeal pa-
pilloma 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 sub-
jectivity among observers. Lusk's system, too, has no func-
tional component. Zalzal (Zalzal G, Personal communica-
tion) and others have utilized an intraoperative laryngeal
diagram to serially record disease involvement with their
RRP patients. Although convenient for the individual sur-
geon, this method is not well suited for reporting of re-
sults 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 in-
corporates more anatomical sites and a subjective sever-
ity rating. It differs from the current proposal in its scor-
ing scale, the exact anatomical sites reported, and our
addition of functional measures of assessment.
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
Laryngoscope 108: June 1998
the previously devised methods is proposed. The system
has been computerized to add to its ease of usage and uni-
formity.The software is available through the American So-
ciety 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 na-
tional registry of RRP patients and enhance future RRP re-
search endeavors by simplifying nomenclature and identi-
fying potential research subjects from across the nation.
ACKNOWLEDGMENT
The authors would like to thank the OTOBASE de-
velopment 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 Soci-
ety of Pediatric Otolaryngologyfor their generosity in pro-
viding this software to their membership.
BIBLIOGRAPHY
1. Jones SR, Myers EM, Barnes L. Benign neoplasms of the lar-
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2. Derkay CS. Multi-disciplinary Task Force on Recurrent Res-
piratory Papillomas: a preliminary report. Arch Otolaryn-
go1 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.
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937