OTOLARYNGOLOGY CONSULTATION
Educational Objectives
| The goal of this program is to improve the management of otolaryngologic problems in children. After hearing and assimilating
this program, the clinician will be better able to:
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 | 1. Diagnose salivary gland problems in children.
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| 2. Recommend the appropriate treatment option (nonsurgical vs surgical) for drooling.
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| 3. Describe the risk factors for recurrent respiratory papillomatosis.
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| 4. Discuss the efficacy of the human papillomavirus (HPV) vaccine.
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| 5. Identify and treat late-onset laryngomalacia.
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Faculty Disclosure
In adherence to ACCME Standards for Commercial Support, Audio-Digest requires all faculty and members of the planning
committee to disclose relevant financial relationships within the past 12 months that might create any personal conflicts
of interest. Any identified conflicts were resolved to ensure that this educational activity promotes quality in health
care and not a proprietary business or commercial interest. For this program, the following has been disclosed: Dr. Pransky
is on the Speakers’ Bureau of Merck. Drs. Messner and Richter and the planning committee reported nothing to disclose.
Acknowledgements
Drs. Messner and Pransky were recorded at the Ultimate Colorado Mid-Winter Meeting, held January 29 to February 1,
2007, in Vail, CO, and sponsored by the Department of Otolaryngology, University of Colorado School of Medicine.
Dr. Richter was recorded at the 22nd Annual Meeting of the American Society of Pediatric Otolaryngology, held April 27-
29, 2007, in San Diego, CA, and sponsored by the Combined Otolaryngology Spring Meetings. The Audio-Digest
Foundation thanks the speakers and the sponsors for their cooperation in the production of this program.
| SALIVARY GLAND PROBLEMS— Anna Messner, MD, Associate Professor of Otolaryngology–Head and Neck Surgery
and Pediatrics, Stanford University School of Medicine, Palo Alto, CA
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| Case 1: infant girl, 6 mo of age; face swollen; otherwise thriving; swelling noted 1 to 2 mo earlier; palpate swelling; if
“squishy”—probably hemangioma (or lymphangioma); consider basing diagnosis just on physical examination and
following child; can obtain computed tomography (CT), magnetic resonance imaging (MRI), or fine needle aspiration
(FNA), but not necessary; observe patient over time; if not squishy—possibly parotid tumor (rare in children); compared
to adults, children have higher percentage of malignancies; types of tumors same as in adults; benign tumors include
pleomorphic adenomas and benign mixed tumors; malignant tumors include same group as in adults, with
higher percentage of lymphomas; diagnosis—pleomorphic adenoma; general rule—with firm parotid mass, the
younger the patient, more likely tumor malignant; solid mass needs further work-up
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| Case 2: child, 7 yr of age, has 3-wk history of swelling; solid on CT; diagnosis Burkitt’s lymphoma
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| Case 3: girl, 14 yr of age, has swollen face; alert, cooperative, and intelligent; diffuse bilateral parotid swelling, nontender,
with clear saliva from Stensen’s duct; ongoing for 2 mo; mild enlargement of submandibular glands; needs further
work-up; findings compatible with bulimia; elevated serum amylase and sialadenosis characteristic
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| Mumps: previously most common inflammatory salivary gland disease of childhood; no longer common, because of
immunization; immunocompromised children unable to receive all immunizations and can get mumps; treatment
same as for adults
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| Case 4: boy, 10 yr of age, has swelling by ear; 4 episodes per year; no complaints of dry eyes or dry mouth; swelling alternates
between sides; treated with ibuprofen (eg, Motrin) and ice packs and resolves in 2 to 3 days; normal complete
blood cell count and slightly elevated erythrocyte sedimentation rate (ESR); expanded parotid gland with dilated areas;
juvenile recurrent parotitis—diagnosis; does not occur in adults; most commonly occurs between 5 and 7 yr of
age; usually every 3 to 4 mo; varies between sides; cause unknown; not associated with upper respiratory infections;
dehydration possibly associated; sialography reveals dilated ducts; histologically, inflammatory process around ducts
and lymphoid infiltrate; treatment is observation (removal of parotid glands puts facial nerve at risk); resolves in few
years
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| Case 5: child, 13 yr of age, has 9-mo history of cyst in floor of mouth; findings of incisional biopsy 4 mo earlier consistent
with ranula; mass recurred, with several episodes of spontaneous rupture; swelling of parotid gland present;
Sjögren’s syndrome—diagnosis; not necessary to have dry eyes and dry mouth to make diagnosis in children; ESR not
necessarily elevated; Sjögren’s syndrome antigen A (SSA) and antigen B (SSB) elevated; research suggests parotid biopsy
(behind ear) more diagnostic for Sjögren’s syndrome than biopsy from lip, and does not require facial nerve dissection;
ranula—mucocele in floor of mouth; originates from sublingual gland; treatment to tie down ranula with 2.0
or 3.0 silk suture; leave and observe in 1 wk; 50% of ranulas resolve with no other treatment; tying down as effective
as marsupialization; speaker favors tying down ranula with silk suture first and if ineffective, performing definitive
treatment (removing ranula including sublingual gland via mouth); plunging ranula—traditionally excised via neck or
mouth; does not have true capsule; as long as sublingual gland removed, ranula resolves
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| Salivary flow: in unstimulated state, submandibular glands form most of saliva; in stimulated state, parotid gland; normal
adult produces 1 to 1.5 L daily
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| Drooling: swallowing issue; in children, make sure no severe nasal obstruction present; if huge adenoids present and
mouth always open, drooling likely; more commonly, problem of low motor tone; recommend speech therapy, occupational
therapy for oral motor control; reassure parent that condition will improve; in general, drooling after 4 yr of
age abnormal; patients who need treatment neurologically impaired older children; usually results in skin problems
and becomes social issue and issue with communication devices; another option tongue acupuncture; neural innervation
parasympathetic and sympathetic, but mostly parasympathetic; acetylcholine-mediated
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| Nonsurgical treatment options: glycopyrrolate (Robinul)—anticholinergic agent; does not pass through blood-brain
barrier; effects felt throughout body (increased and thickened secretions); effective, but has several side effects; scopolamine
patch—systemic effects; several side effects; botulinum toxin type A (Botox)—local effect (blocks acetylcholine-
mediated release); effective; injected in parotid and submandibular glands on both sides; not permanent
solution; determine number of times patient must change shirt and whether nasal obstruction present; on physical
examination, determine whether child can hold up head and swallow (more likely to do better with treatment);
check for dental problems and tongue control; determine severity and frequency; goal of treatment to retain moist
mouth without drooling; too dry mouth bigger problem than too wet mouth
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| Surgical options: several, but none works perfectly; traditional procedure—remove submandibular glands and reposition
parotid ducts; not done often; bilateral submandibular duct relocation—warn parents that condition does not improve
in ≈20% and may improve initially, then worsen over time; bilateral submandibular duct relocation with
sublingual gland excision—preferred; risk for ranula if ligation of submandibular ducts only treatment; consider saliva
in patient with chronic aspiration pneumonia; dose of Botox—no set amount; speaker injects 25 U into each
gland
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| RECURRENT RESPIRATORY PAPILLOMATOSIS: THE IMPACT OF HPV VACCINE —Seth Pransky, MD, Director
of Pediatric Otolaryngology, Rady Children’s Hospital, San Diego, CA
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| Recurrent respiratory papillomatosis (RRP): challenges include frequent recurrences, requirement for multiple
surgeries, concern for spread of disease, and impact on airway, voice, and quality of life; surgical extirpation rarely
curative
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| Human papillomavirus (HPV): >100 closely related types; DNA virus; epitheliotropic virus, going to squamocolumnar
junctions; types with high risk for malignant change include 16, 18, 31, 33, and 45; types 16 and 18 most concerning
and responsible for two-thirds of all cancer from HPV; low-risk types 6 and 11 responsible for benign low-
grade cervical changes, genital warts, and RRP; HPV responsible for 100% of cervical cancers, 85% of anal cancers,
50% of cancers of vulvovagina and penis, 20% of oropharyngeal cancers, and 10% of laryngeal and esophageal cancers;
mechanism—L1 and L2 regions of virus basis of vaccine (late phase of viral cycle; generate outer envelope); E1
and E2 regions responsible for latching onto host chromosome; E6 and E7 regions responsible for modulating immune
reaction and disrupting cell growth; infection occurs at basal cell of epidermis and can be latent; virus goes through
cell cycle; impact of E6 and E7 on replication and growth of papilloma seen in mid cycle; suppression of P53 and
other tumor-suppressor proteins permits proliferation of papilloma; variation in E6 and E7 region (transforming capabilities)
affects severity of disease; humoral immune response
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| Laryngeal RPP: has 100% prevalence of HPV infection; often combination of HPV types 6 and 11; more common in
lower socioeconomic groups, firstborn infants, young mothers, vaginal deliveries, and when papilloma evident at time
of delivery; greater susceptibility to HPV in younger women; lower socioeconomic status associated with higher number
of sexual partners; firstborn infants associated with longer labor, resulting in longer exposure to virus; incidence—
≈1500 new cases of juvenile-onset papilloma annually in United States (≈2500 cases of adult-onset); ≈50,000 surgical
procedures; costs ≈$150 million annually to manage; incidence low in infants from caesarean delivery; 30% of infants
have positive HPV smear from pharynx, but very few infected (reason unknown); American College of Obstetrics and
Gynecology—does not recommend caesarean delivery for HPV-positive women; Danish study (2003)—showed 231
times greater chance of child developing RRP if mother had papilloma at time of delivery; concluded that in woman
with genital warts, delivery time >10 hr associated with 2-fold greater risk for disease, and caesarean delivery not protective
against RPP; did not find young maternal age or being firstborn specific risk factors (related more to number of
sexual partners and degree of activity of disease at time of delivery); HPV 11 not as benign as previously thought; extralaryngeal
disease—10% to 14% of cases spread beyond larynx; pulmonary spread 3% to 5%; malignant transformation
3% to 5% (can occur in absence of eg, irradiation, smoking); classically occurs in lungs after distal spread
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| HPV vaccine: viral coat protein called “virus-like particles (VLPs)”; VLPs—created based on L1 (late-phase portion;
outer structural core) of virus; noninfectious agent; generates humoral immune response; not cross-reactive (ie, type-specific);
approved by Food and Drug Administration (FDA) in June 2006 for women 9 to 26 yr of age for prevention of cervical
cancer and genital warts; Centers for Disease Control and Prevention (CDC) Advisory Committee on Immunization
Practices (ACIP) unanimously recommended routine vaccination for girls 11 to 12 yr of age; can give as early as 9 yr of
age; catch-up vaccinations for women 13 to 26 yr of age; insurance pays for women and girls (out-of-pocket cost for parents
who want their sons vaccinated); efficacy—highly immunogenic, with protection ≤5 yr; in studies, vaccine prevented
HPV-related cervical precancers and noninvasive cancers, 95% of low-grade cervical dysplasia and precancers caused by
4 types of HPV, and 99% of genital warts caused by types 6 and 11; few side effects; 100% efficacy for types 6 and 11 in
protocol population at 5 yr; well tolerated, except for slight injection-site erythema; good antibody titers to types 6, 11,
16, and 18; reduces incidence of persistent HPV infection and HPV-associated diseases, eg, anogenital and cervical disease;
cost—injections $120 each (at initiation, 2 mo, and 6 mo later); second vaccine—bivalent (types 16 and 18); high
levels of protection demonstrated; approval expected soon; does not replace Papanicolaou (Pap) screening; HPV types
31, 33, and 45 (not covered by vaccines) also generate cancer; receiving vaccine while infected with particular type will
not treat that infection, but will prevent infection from other types and may play role in reducing recurrence of type patient
infected with; men may not have visible lesion (critical part of vector pattern for transmission of disease); vaccine
will result in reduced number of abnormal Pap tests and, ultimately, cervical surgery; implications for RRP—takes decades
to obtain herd immunity; ultimately expect to see fewer cases of RRP and head and neck cancer associated with
HPV; no impact on active disease
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| LATE-ONSET LARYNGOMALACIA: A DANGEROUS DISEASE VARIANT —Gresham T. Richter, MD, Clinical
Fellow, Department of Pediatric Otolaryngology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH
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| Definition: generalized hypotony of larynx; clinically seen as medial prolapse of supraglottic structures upon inspiration
into laryngeal inlet; leads to varying degrees of upper airway obstruction
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| Presentation: most common congenital laryngeal anomaly; patients present at 2 wk to 3 mo of age with various respiratory
and feeding difficulties; often self-limited; clinically and experimentally tied to gastroesophageal reflux disease
(GERD); rarely seen in older children; however, case reports of laryngomalacia seen during exercise, various states
(eg, sleep), and those adults who have had neurologic insult; atypical features; diagnosis in older child often obscured
and delayed; endoscopically, airways show laryngeal hypotonia and supraglottic prolapse; in exercise-induced laryngomalacia,
upon inspiration, medial prolapse and rotation of arytenoids into airway seen, leading to obstruction; clinical
characteristics not well defined for patients with later onset; management and etiology undetermined
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| Study: 229 patients >2 yr of age diagnosed with laryngomalacia from 1998 to 2005; 17 children with mean age of 6.6 yr
with laryngomalacia (all had absent signs and history of congenital laryngomalacia); patients divided into 3 groups according
to symptoms; feeding difficulties (7 patients)—all toddlers; presented with choking and coughing with feeding,
variable weight loss, and failure to thrive; all diagnosed by endoscopic evaluation of swallowing, which showed posterosuperior
arytenoid redundancy and prolapse that worsened only during feeding (none had stridor); all patients had
GERD, although symptoms not resolved with daily proton pump inhibitor and histamine (H2 )-receptor antagonist; all
underwent supraglottoplasty (primarily reduction of supra-arytenoid tissue but not aryepiglottic folds); none had recurrence
(average follow-up 10.4 mo); sleep disorders (7 patients)—had varying degrees of upper airway obstruction during
sleep; only 2 had stridor during sleep (all school-aged with normal sleep studies); those with fixed component of
sleep apnea removed by adenotonsillectomy had persistent symptoms (43% GERD); flexible laryngoscopy and microlaryngoscopy
showed evidence of supra-arytenoid redundancy and prolapse; all had symptom resolution with reduction
of prolapse; exercise intolerance (3 patients)—all adolescents; presented with stridor, shortness of breath, and retractions
during exercise; unresponsive to inhaled β-agonist therapy; underwent treadmill tests or hyperventilation studies
with flexible laryngoscopy and showed no paradoxic cord movement but arytenoid rotation and prolapse into airway;
underwent arytenoid reduction and had symptom resolution; flow-volume loop another way of evaluating patients; evidence
of supra- or extrathoracic obstruction
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| Mechanism of late-onset laryngomalacia: 4 categories of laryngomalacia; diagnosed at early age in congenital
group, with all areas of larynx involved; other 3 groups have evidence of arytenoid redundancy and require reduction
for improvement of symptoms (variants of laryngomalacia or late-onset laryngomalacia); from standpoint of etiology,
not due to immaturity, anatomy, or neurologic insult; proposed concept—susceptible larynx in which neuromuscular
integrity disrupted and exacerbated by anatomy of supra-arytenoid level and perhaps GERD; laryngeal adductor reflux
(LAR) proposed in past, as larynx requires stimulus to maintain tone; disrupted stimulus leads to reduced tone and supraglottic
prolapse; GERD—decreased neurosensitivity of larynx and disrupted feedback, leading to reduced tone; removal
of insensate tissue leads to recovery; in sleep disorders—possible that larynx susceptible, with supra-arytenoid
redundancy that prolapses during sleep, increasing intrathoracic pressure; exacerbated by GERD (reduces sensation
and tone of larynx); central mechanisms in sleep contribute to reducing tone in larynx (eg, hypoxia, hypercarbia); necessary
to remove prolapsed supra-arytenoid tissue; in exercise intolerance—susceptible larynx, with exaggerated resistance
at supraglottic level; Bernoulli effect contributes; GERD may reduce laryngeal tone in these patients
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Suggested Reading
Baden LR et al: Human papillomavirus vaccine--opportunity and challenge. N Engl J Med 356:1990, 2007; Cox
JT: Epidemiology and natural history of HPV. J Fam Pract Suppl:3, 2006; Denoyelle F et al: Failures and complications
of supraglottoplasty in children. Arch Otolaryngol Head Neck Surg 129:1077, 2003; Ellies M et al: Tumors of
the salivary glands in childhood and adolescence. J Oral Maxillofac Surg 64:1049, 2006; Ellies M et al: Up-to-date
report of botulinum toxin therapy in patients with drooling caused by different etiologies. J Oral Maxillofac Surg
61:454, 2003; Hockstein NG et al: Sialorrhea: a management challenge. Am Fam Physician 69:2628, 2004; Mahoney
MC: Protecting our patients from HPV and HPV-related diseases: the role of vaccines. J Fam Pract Suppl:10,
2006; Manning SC et al: Laryngeal anatomic differences in pediatric patients with severe laryngomalacia. Arch
Otolaryngol Head Neck Surg 131:340, 2005; Nichols JR et al: Human papillomavirus infection: the role of vaccination
in pediatric patients. Clin Pharmacol Ther 81:607, 2007; Ruparelia S et al: Predictors of remission in juvenile-
onset recurrent respiratory papillomatosis. Arch Otolaryngol Head Neck Surg 129:1275, 2003; Schraff S et al: American
Society of Pediatric Otolaryngology members' experience with recurrent respiratory papillomatosis and the use
of adjuvant therapy. Arch Otolaryngol Head Neck Surg 130:1039, 2004; Tasca RA et al: Recurrent respiratory papillomatosis.
Arch Dis Child 91:689, 2006; Valera FC et al: Evaluation of the efficacy of supraglottoplasty in obstructive
sleep apnea syndrome associated with severe laryngomalacia. Arch Otolaryngol Head Neck Surg 132:489, 2006;
Zacharisen MC et al: Recurrent respiratory papillomatosis in children: masquerader of common respiratory diseases.
Pediatrics 118:1925, 2006.
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