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Coccidioides immitis: An unexpected fungal pathogen causing retropharyngeal abscess
Otolaryngology–Head and Neck Surgery (2007) 136, 500-501
CASE REPORT
Coccidioides immitis: An unexpected fungal pathogen causing retropharyngeal abscess J. Andrew Sipp, MD, Sarah K. Wise, MD, Steven E. Sobol, MD, MSc, and Seth A. Yellin, MD, Atlanta, GA
R
etropharyngeal abscess (RA) is a common problem encountered by otolaryngologists. Patients typically present with a history of fever, decreased oral intake, lethargy, and decreased neck mobility, with airway obstruction complicating more advanced cases. RA typically results from bacterial suppuration of retropharyngeal lymph nodes, which may occur alone or following an acute episode of tonsil, upper respiratory, or dental infection. Fungal organisms are very rarely implicated as the pathogenic organism. We present a case of RA caused by the fungal organism Coccidioides immitis. A healthy 18-year-old African-American male, who resided in Georgia, moved to Arizona to attend college. Early in his second semester, he developed fatigue, a low-grade cough, and sore throat. Multiple courses of antibiotics and antihistamines were instituted, but over the next two months he began to experience exercise intolerance, odynophagia, and voice change. In spite of these symptoms, he was highly functional, taking college classes and working part time. His parents, after hearing his change in voice and learning of his symptoms, convinced him to return home for evaluation. On physical examination the patient was afebrile, with stable vital signs, and nontoxic in appearance. Neck flexion was limited by pain. There was severe oropharyngeal swelling and laryngeal edema. He was comfortable talking throughout the patient interview, although his voice was hyponasal and lower in pitch than baseline. His white blood cell count was 12.5. CT scan demonstrated the presence of bilateral retropharyngeal fluid collections (Fig 1). The patient underwent an awake tracheotomy and transcervical drainage of his bilateral fluid collections. Cultures and stains were sent for bacterial, fungal, and mycobacterial
From the Department of Otolaryngology–Head and Neck Surgery, Emory University School of Medicine. Presented at the Annual Meeting of the Society for Ear, Nose, and Throat Advances in Children, Toronto, Ontario, Canada, December 2004.
pathogens. A repeat CT scan on postoperative day three demonstrated resolution of the patient’s RA and a new finding of cervical osteomyelitis, for which he was placed in a c-collar by the orthopedic service. On postoperative day 3 the patient’s fungal stains were positive and he was started on amphotericin by the infectious disease service. Cultures confirmed Coccidioides immitis as the offending pathogen. Immunological workup, including HIV testing, was negative. The patient was decannulated and discharged to home on postoperative day 13 with a six-week course of intravenous liposomal amphotericin. An MRI performed on postoperative day 26 revealed progression of the osteomyelitis and reaccumulation of the patient’s RAs (Fig 2). Repeated transcervical drainage of his abscesses was performed and confirmed persistent Coccidioides immitis infection. Fluconazole was added to his antifungal regimen. He was discharged to home on amphotericin and fluconazole 10 days after admission. Outpatient MRI two weeks after discharge demonstrated complete resolution of his infection. There has been no evidence of local or disseminated disease at his 18-month follow-up.
DISCUSSION Coccidioides immitis is a fungal organism native to arid regions of North and South America. There are over 100,000 cases in the United States annually. Incidence is higher during winter months when there is accumulation of moisture and subsequent periods of dusty conditions. It is estimated that over 60% of infections are asymptomatic. Reprint requests: J. Andrew Sipp, MD, 314 Marlborough St., Apt. 2, Boston, MA 02116. E-mail address: [email protected].
0194-5998/$32.00 © 2007 American Academy of Otolaryngology–Head and Neck Surgery Foundation. All rights reserved. doi:10.1016/j.otohns.2006.08.003
Sipp et al
Coccidioides immitis: An unexpected fungal . . .
Approximately 40% develop a febrile illness commonly known as “San Joaquin Valley Fever.”1 Half of those symptomatic develop abnormal chest x-rays. Severe pneumonia, cavitary lesions, and pulmonary nodules have also been reported. Approximately 1% of infected patients develop disseminated disease involving the meninges, skin, bone, or soft tissues. Symptomatic coccidioidomycosis is more common in non-Caucasian races, at the extremes of age, and in the immunocompromised. Systematic investigation identified African-American race, income less than $15,000/year, and pregnancy as independent risk factors for disseminated disease.2 Lower socioeconomic status as a risk factor is thought to stem from increased exposure to outdoor labor. In the head and neck Coccidioides immitis has been reported to cause deep neck space, laryngeal, lymph node, and temporal bone infections.3,4 In our case, and in a previous report, there was cervical osteomyelitis in association with Coccidioides retropharyngeal abscess.4 It is unclear whether Coccidioides osteomyelitis is a cause or a complication of the RA. Cervical osteomyelitis with RA is most commonly reported in disseminated mycobacterial tuberculosis, although it has been reported with other bacterial infections.5 Osteomyelitis in association with RA should raise suspicion of atypical organisms.
501
Figure 2 MRI demonstrating recurrence of retropharyngeal abscess and osteomyelitis. Postoperative photographs 6 months after a single-stage reconstruction with microsurgical repair of a single artery with subsequent leech therapy. Excellent aesthetic results are achieved despite avulsion injury and single-vessel repair.
Management of head and neck Coccidioides immitis may involve surgical intervention to drain fluid collections or to confirm the diagnosis. Medical therapy consists of intravenous amphotericin followed by oral antifungals. Prolonged oral therapy, often years of treatment, is recommended because of the possibility of late relapse.1 Guidelines for exact duration of therapy are not definitive. The patient in this report was surprisingly nontoxic and non–ill appearing in his presentation. This atypical presentation of RA prompted suspicion of a nonbacterial etiology. Although rare, fungal infection needs to be in the differential diagnosis of atypical RA, with appropriate stains and cultures obtained in order to expedite appropriate management and avoid potential morbidity.
REFERENCES
Figure 1 abscesses.
CT neck demonstrating bilateral retropharyngeal
1. Chiller T. Coccidioidomycosis. Infect Dis Clin North Am 2003;17(1): 41–57. 2. Rosenstein NE, Werner B, Kao A, et al. Risk factors for severe pulmonary and disseminated coccidioidomycosis: Kern County, California, 1995-1996. Clin Infect Dis 2001;32:708 –15. 3. Arnold MG, Arnold JC, Bloom DC, et al. Head and neck manifestations of disseminated coccidioidomycosis. Laryngoscope 2004;114(4):747– 52. 4. Copeland B, White D, Buenting J. Imaging case study of the month: coccidiomycosis of the head and neck. Ann Otol Rhinol Laryngol 2003;112(1):98 –101. 5. Feidas A, Ferguson JV, Nelson JE, et al. Cervical vertebral osteomyelitis presenting as a retropharyngeal abscess. Clin Infect Dis 1994; 18(6):992– 4.
CASE REPORT
Coccidioides immitis: An unexpected fungal pathogen causing retropharyngeal abscess J. Andrew Sipp, MD, Sarah K. Wise, MD, Steven E. Sobol, MD, MSc, and Seth A. Yellin, MD, Atlanta, GA
R
etropharyngeal abscess (RA) is a common problem encountered by otolaryngologists. Patients typically present with a history of fever, decreased oral intake, lethargy, and decreased neck mobility, with airway obstruction complicating more advanced cases. RA typically results from bacterial suppuration of retropharyngeal lymph nodes, which may occur alone or following an acute episode of tonsil, upper respiratory, or dental infection. Fungal organisms are very rarely implicated as the pathogenic organism. We present a case of RA caused by the fungal organism Coccidioides immitis. A healthy 18-year-old African-American male, who resided in Georgia, moved to Arizona to attend college. Early in his second semester, he developed fatigue, a low-grade cough, and sore throat. Multiple courses of antibiotics and antihistamines were instituted, but over the next two months he began to experience exercise intolerance, odynophagia, and voice change. In spite of these symptoms, he was highly functional, taking college classes and working part time. His parents, after hearing his change in voice and learning of his symptoms, convinced him to return home for evaluation. On physical examination the patient was afebrile, with stable vital signs, and nontoxic in appearance. Neck flexion was limited by pain. There was severe oropharyngeal swelling and laryngeal edema. He was comfortable talking throughout the patient interview, although his voice was hyponasal and lower in pitch than baseline. His white blood cell count was 12.5. CT scan demonstrated the presence of bilateral retropharyngeal fluid collections (Fig 1). The patient underwent an awake tracheotomy and transcervical drainage of his bilateral fluid collections. Cultures and stains were sent for bacterial, fungal, and mycobacterial
From the Department of Otolaryngology–Head and Neck Surgery, Emory University School of Medicine. Presented at the Annual Meeting of the Society for Ear, Nose, and Throat Advances in Children, Toronto, Ontario, Canada, December 2004.
pathogens. A repeat CT scan on postoperative day three demonstrated resolution of the patient’s RA and a new finding of cervical osteomyelitis, for which he was placed in a c-collar by the orthopedic service. On postoperative day 3 the patient’s fungal stains were positive and he was started on amphotericin by the infectious disease service. Cultures confirmed Coccidioides immitis as the offending pathogen. Immunological workup, including HIV testing, was negative. The patient was decannulated and discharged to home on postoperative day 13 with a six-week course of intravenous liposomal amphotericin. An MRI performed on postoperative day 26 revealed progression of the osteomyelitis and reaccumulation of the patient’s RAs (Fig 2). Repeated transcervical drainage of his abscesses was performed and confirmed persistent Coccidioides immitis infection. Fluconazole was added to his antifungal regimen. He was discharged to home on amphotericin and fluconazole 10 days after admission. Outpatient MRI two weeks after discharge demonstrated complete resolution of his infection. There has been no evidence of local or disseminated disease at his 18-month follow-up.
DISCUSSION Coccidioides immitis is a fungal organism native to arid regions of North and South America. There are over 100,000 cases in the United States annually. Incidence is higher during winter months when there is accumulation of moisture and subsequent periods of dusty conditions. It is estimated that over 60% of infections are asymptomatic. Reprint requests: J. Andrew Sipp, MD, 314 Marlborough St., Apt. 2, Boston, MA 02116. E-mail address: [email protected].
0194-5998/$32.00 © 2007 American Academy of Otolaryngology–Head and Neck Surgery Foundation. All rights reserved. doi:10.1016/j.otohns.2006.08.003
Sipp et al
Coccidioides immitis: An unexpected fungal . . .
Approximately 40% develop a febrile illness commonly known as “San Joaquin Valley Fever.”1 Half of those symptomatic develop abnormal chest x-rays. Severe pneumonia, cavitary lesions, and pulmonary nodules have also been reported. Approximately 1% of infected patients develop disseminated disease involving the meninges, skin, bone, or soft tissues. Symptomatic coccidioidomycosis is more common in non-Caucasian races, at the extremes of age, and in the immunocompromised. Systematic investigation identified African-American race, income less than $15,000/year, and pregnancy as independent risk factors for disseminated disease.2 Lower socioeconomic status as a risk factor is thought to stem from increased exposure to outdoor labor. In the head and neck Coccidioides immitis has been reported to cause deep neck space, laryngeal, lymph node, and temporal bone infections.3,4 In our case, and in a previous report, there was cervical osteomyelitis in association with Coccidioides retropharyngeal abscess.4 It is unclear whether Coccidioides osteomyelitis is a cause or a complication of the RA. Cervical osteomyelitis with RA is most commonly reported in disseminated mycobacterial tuberculosis, although it has been reported with other bacterial infections.5 Osteomyelitis in association with RA should raise suspicion of atypical organisms.
501
Figure 2 MRI demonstrating recurrence of retropharyngeal abscess and osteomyelitis. Postoperative photographs 6 months after a single-stage reconstruction with microsurgical repair of a single artery with subsequent leech therapy. Excellent aesthetic results are achieved despite avulsion injury and single-vessel repair.
Management of head and neck Coccidioides immitis may involve surgical intervention to drain fluid collections or to confirm the diagnosis. Medical therapy consists of intravenous amphotericin followed by oral antifungals. Prolonged oral therapy, often years of treatment, is recommended because of the possibility of late relapse.1 Guidelines for exact duration of therapy are not definitive. The patient in this report was surprisingly nontoxic and non–ill appearing in his presentation. This atypical presentation of RA prompted suspicion of a nonbacterial etiology. Although rare, fungal infection needs to be in the differential diagnosis of atypical RA, with appropriate stains and cultures obtained in order to expedite appropriate management and avoid potential morbidity.
REFERENCES
Figure 1 abscesses.
CT neck demonstrating bilateral retropharyngeal
1. Chiller T. Coccidioidomycosis. Infect Dis Clin North Am 2003;17(1): 41–57. 2. Rosenstein NE, Werner B, Kao A, et al. Risk factors for severe pulmonary and disseminated coccidioidomycosis: Kern County, California, 1995-1996. Clin Infect Dis 2001;32:708 –15. 3. Arnold MG, Arnold JC, Bloom DC, et al. Head and neck manifestations of disseminated coccidioidomycosis. Laryngoscope 2004;114(4):747– 52. 4. Copeland B, White D, Buenting J. Imaging case study of the month: coccidiomycosis of the head and neck. Ann Otol Rhinol Laryngol 2003;112(1):98 –101. 5. Feidas A, Ferguson JV, Nelson JE, et al. Cervical vertebral osteomyelitis presenting as a retropharyngeal abscess. Clin Infect Dis 1994; 18(6):992– 4.