Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature. Sinusitis in Immunodeficiency Disorders in Children Sinusitis is a common illness in children. Primary immunodeficiency disease can predispose children to recurrent and chronic sinusitis. A child is designated as "sinusitis-prone" if he or she suffers 3 or more episodes of sinusitis within a year or if he or she requires antibiotic therapy for control of sinusitis for 3 or more months during a year. The majority of sinusitis-prone children are atopic not immunodeficient. The incidence of all immunodeficiency diseases in children is only 0.5%, which is 16 to 40 times less than the incidence of respiratory allergy. The symptoms of sinusitis in the immunodeficient child are the same as in the allergic or immunologically normal child. Nasal obstruction, nasal discharge, and cough are the common symptoms. Allergy patients are more likely to have a family history of allergy, a seasonal pattern of respiratory problems and other allergy-related symptoms and signs. The undiagnosed immunodeficiency children frequently develop bacterial infection in addition to respiratory infections. They always take an antibiotic and become ill shortly after discontinuing antibiotics. Recurrent pneumonia, meningitis, cellulitis, candidiasis, chronic diarrhea, and failure to thrive are often present in more severe childhood immunodeficiency disorders. More commonly, recurrent/chronic sinusitis may be the only indication that a patient is immunodeficient. The presence of a positive family history for immunodeficiency is also common since many of the disorders are hereditary. Immunodeficient children whose primary problem is recurrent/chronic sinusitis have humoral immunodeficiency disease. Patients with defects in T-cell function or neutrophil dysfunction will usually present with a more severe infection, and sinusitis is usually a minor problem. Common Variable Immunodeficiency (CVID) is a disorder in which patients have significantly reduced levels of two or more immunoglobulin classes in serum and are unable to synthesize antibodies to antigens. Usually IgG and IgA are low. Response to primary immunization is poor. B-lymphocyte count is usually normal or near normal. Onset of infection varies from 2 years of age to adulthood. These patients usually suffer recurrent/chronic sinusitis, recurrent otitis media, bronchitis, and pneumonia. IgG comprise 4 subclasses: IgG1, IgG2, IgG3, and IgG4. The most common selective IgG-subclass deficiency in children is IgG2 deficiency. IgG2 or IgG2-IgG4 deficiency frequently occurs with IgA deficiency and ataxia-telangiectasia. These patients are unable to synthesize antibodies against the vaccines of polysaccharide of Hemophilus influenzae or Streptococcus pneumoniae. IgG3 is the most common subclass deficiency in adults. Many antiviral antibodies are related to IgG3 subclass. Selective IgG1 and IgG4 deficiency is rare. In some children subclass deficiency is transient. Some patients may suffer recurrent infections because of their inability to produce specific antibodies to certain antigens. These patients may have normal immunoglobulin levels and IgG subclass levels. In a study by Shapiro, approximately one-third of the patients failed to respond to pneumococcal polysaccharide types. Selective IgA deficiency is the most common immunodeficiency disorder. Because IgA levels rise slowly the diagnosis of IgA deficiency cannot be made before the age of 2. Patients with IgA deficiency associated with selective IgG subclass deficiency appear to be more susceptible to respiratory tract infections than those who do not have IgG deficiency. Patients with X-linked agammaglobulinemia (XLA) are unable to synthesize antibodies to any antigens and are severely deficient in all immunoglobulin classes. They lack B-cells in the blood and plasma cells are absent from bone marrow and lymph nodes. They are predisposed to bacterial infection at 6 to 12 months of age when the maternally-transmitted antibody level diminishes. Pneumonia, meningitis, septic arthritis, cellulitis, and septicemia are common among these patients. Immunoglobulin replacement is definitely indicated for these patients, with good response. Patients with transient hypogammaglobulinemia of infancy (THI) have recurrent upper respiratory tract infections including sinusitis. IgG and IgA level is reduced while IgM level is normal. Their hypogammaglobulinemia usually resolves spontaneously by the age of 2. If a patient is considered to be a sinusitis-prone, serum immunoglobulin level as well as IgG subclass level should be measured. If there is a history of respiratory allergy, allergy skin tests and radioallergorbent tests (RAST) should be performed. These tests should detect approximately 75% of the immunodeficiency disorders. If immunologic abnormalities are found in these tests, diphtheria and/or tetanus antibody titers, total hemolytic complement and C4 level should be ordered. Pre- and post-immunization pneumococcal antibody levels should also be measured. If patients are found to have low levels of all immunoglobulin, B- and T-cell populations should be measured to discriminate between XLA (no B-cells) and CVID (normal B-cells). These studies will also detect T-cell related disorders. The management of recurrent/chronic sinusitis in immunodeficiency patients must include simultaneous therapy for their underlying immunodeficiency. For patients with XLA and CVID, immunoglobulin replacement therapy is indicated. Efficacy of immunoglobulin therapy in selective IgG-subclass deficiency is not clearly defined at this time. Immunoglobulin replacement is not recommended for patients with selective IgA deficiency and THI. Intravenous immunoglobulin (IVIG) infusion is the treatment of choice. The medical management of episodes of sinusitis in these patients is the same as for non-immunodeficient patients. Topical nasal saline spray and steroid spray may help to cleanse the mucus membrane and decrease inflammation and swelling. Little information is available regarding the bacteriology of sinusitis in immunodeficient patients. A preliminary study by Lusk et al revealed that the cultures from the sinuses of immunodeficiency patients were similar to those found in non-immunodeficiency patients. They recommended a 4-week treatment regimen using either Augmentin, Pedizole, or Ceftin. If there is an inadequate response to therapy, the patient should be treated with a different antibiotic for another 4 weeks. At this time, a coronal CT scan of the paranasal sinuses should be obtained to evaluate the sinus disease. There is little information on the surgical management of sinus disease in immunodeficient children. In a series of 11 patients, most of the patients who underwent endoscopic ethmoidectomy and maxillary antrostomy, were symptomatically improved, but they continued to require daily antibiotic and monthly IVIG therapy. Endoscopic ethmoidectomy appears to be helpful for reestablishing the patent osteomeatal complexes. Further study is required to determine the role of endoscopic sinus surgery in the management of sinus disease with immunodeficiency disorders. Case Presentation A 2-1/2-year-old white female with recurrent otitis media since 8 months of age, and recurrent upper respiratory tract infections presented with persistent purulent rhinorrhea and chronic cough consistent with chronic sinusitis. She was referred to the TCH Otolaryngology Service for refractory otitis media and sinusitis unresponsive to medical therapy in May, 1993. Even after bilateral ear tube placements, she continued to have recurrent otitis media. She also has a history of mild asthma, but no recurrent pneumonia. Her perinatal history with unremarkable. The family history was only significant for the maternal grandmother and aunt with asthma. On her initial visit to the clinic, her weight was 11.7 kg which was at the 5th percentile for age. Bilateral otorrhea was present with both ear tubes in place. The nose was congested with bilateral purulent rhinorrhea. The tonsillar tissue was normal without cervical lymphadenopathy. Because of continued otitis media and sinusitis, a screening survey for immune problems was undertaken. Quantitative immunoglobulins showed marked depression of IgG and IgA levels with markedly elevated IgM. She also had an extremely low level of all IgG subclasses. Because of these abnormal laboratory results, she was referred to the Allergy/Immunology Department. An extensive immunologic evaluation was performed. Isohematoglutinins showed no antibody formation. Delayed hypersensitivity skin tests showed positive reactivity for Candida and tetanus. She had mild antibody response to diphtheria, tetanus, and H. Flu type b antigens, but not to pneumococcal polysaccharide. Her T-lymphocytes were normal in number but mildly decreased in their response to mitogens. She was diagnosed with hypogammaglobulinemia with hyper-IgM. She was started on monthly intravenous immunoglobulin replacement therapy in May, 1993. Her recurrent otitis media and sinusitis are currently under much better control except for occasional ear and sinus infection near the time for readministration of immunoglobulins. She may need more frequent immunoglobulin replacement in the future. Bibliography Baroody FM, Naclerio RM. An overview of immunology. Otolaryngol Clin North Am 1993;26:57-591. Berdal P, Brandtzeg P, Froland S, et al. Immunodeficiency syndromes with otorhinolaryngological manifestations. Acta Otolaryngol 1976;82:185-192. Bortin MM, Rimm AA. Severe combined immunodeficiency disease. JAMA 1977;238:591-600. Buckley RH. Immunodeficiency diseases. JAMA 1987;258:2841-2850. Burks AW, Sampson HA, Buckley RH. Anaphylactic reactions after gamma globulin administration in patients with hypogammaglobulinemia. NEJM 1986;314:560-564. Claman HN. The biology of the immune response. JAMA 1987;258:2834-2840. Corey JP, Seligman I. Otolaryngology problems in the immune compromised patient. Otolaryngol Head Neck Surg 1991;104:196-203. Harris JP, South MA. Immunodeficiency diseases: head and neck manifestations. Head Neck Surg 1982;5:114-124. Kimmelman CP, Potsic WP. Immunodeficiency in pediatric otolaryngology. Am J Otolaryngol 1979;1:33-38. deShazo RD, Lopez M, Salvaggio JE. Use and interpretation of diagnostic immunologic laboratory tests. JAMA 1987;258:3011-3031. Lusk RP, Muntz HR. Endoscopic sinus surgery in children with chronic sinusitis: a pilot study. Laryngoscope 1990;100:654-658. Lusk RP, Polmar SH, Muntz HR. Endoscopic ethmoidectomy and maxillary antrostomy in immunodeficient patients. Arch Otolaryngol Head Neck Surg 1991;117:60-63. Manning SC. Pediatric sinusitis. Otolaryngol Clin North Am 1993;26:623-638. Muntz HR, Lusk RP. Signs and symptoms of chronic sinusitis. In: Lusk RP, editor. Pediatric Sinusitis. New York: Raven Press, 1992:1-5. Oxelius VA, Berkel I, Hanson LA. IgG2 deficiency in ataxia-telangectasia. NEJM 1982;304:1476-1477. Oxelius, VA. Chronic infection in a family with hereditary deficiency of IgG2 and IgG4. Clin Exp Immunol 1974;17:19-27. Polmar SH. The role of the immunologist in sinus disease. J Allergy Clin Immunol 1999;90:511-514. Polmar SH. Sinusitis and immune deficiency. In: Lusk RP, editor. Pediatric Sinusitis. New York: Raven Press, 1992:53-58. Shackelford PG, Polmar SH, Mayus JL et al. Spectrum of IgG2 subclass deficiency with recurrent infections. J Pediatr 1986;108:647-653. Shackelford PG, Granoff DM, Polmar SH, et al. Subnormal serum concentration of IgG2 in children with frequent infections associated with varied patterns of immunologic dysfunction. J Pediatr 1990;116:529-538. Shapiro GG. Sinusitis in children. J Allergy Clin Immunol 1988:81:1025-1027. Shapiro GG, Virant FS, Furukawa, et al. Immunologic defects in patients with refractory sinusitis. Pediatrics 1991;87:311-316. Stiehm ER. Immunodeficiency disorder: general considerations. In: Stiehm ER, editor. Immunologic Disorders in Infants and Children. Philadelphia: Saunders, 1989:157-195. Tinkelman DG, Silk HJ. Clincial and bacteriologic features of chronic sinusitis in children. Am J Dis Child 1989;115:28-32. Umetu DT, Ambrosino DM, Quinti I, et al. Recurrent sinopulmonary infection and Impaired antibody response to bacterial capsular polysaccharide antigen in children with selective IgG-subclass deficiency. NEJM 1985;313:1247-1251. Wald ER, Byers C, Guerra, et al. Subacute sinusitis in children. J Pediatr 1989;115:28-32. Wald ER. Microbiology of acute and chronic sinusitis. In: Lusk RP, editor. Pediatric Sinusitis. New York: Raven Press, 1992:43-52. Wasserman RL. Antibody deficiency: IgG sublcass deficiency and vaccine nonresponder states. Pediatr Infect Dis J 1990;9:424-433. Grand Rounds Archive | Department Home page BCM Public | BCM Intranet | Privacy Notices | Contact BCM | BCM Site Map | ©2001-2006 Baylor College of Medicine
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