Anterior Chest Wall Fistula in a Fourteen-Year-Old

Lucy Jean M. Prince, M.D., Joanne Baerg, M.D., Donald Janner, M.D. Edited by Parvin H. Azimi, M.D. and Moses Grossman, M.D.


Pediatr Infect Dis J. 2002;21(10) 


A 14-year-old Tongan girl presented with a 6-month history of anterior chest wall cellulitis and fistula formation. She had been evaluated for bilateral cervical lymphadenopathy 1 year before this visit by an otolaryngologist, who performed a tonsillectomy. Histologic examination on the moved tonsils was not performed. Cervical adenopathy was reported as multiple, bilateral, firm, nontender, anterior cervical nodes, measuring ~2 cm in diameter, without overlying erythema.

Six months before this evaluation, the patient noticed an opening on her anterior chest wall, which reportedly produced a thick exudative discharge. She was seen by several physicians and was treated with cephalexin without any improvement. There was no additional diagnostic workup performed.

The patient lived in Riverside, CA, and had not traveled. She had no exposures to animals. She denied having fever, night sweats, rashes or cough.

On physical examination the patient was alert and nontoxic. Temperature was 37°C, pulse 91 beats/min, respiratory rate 19 breaths/min, blood pressure 114/63 mm Hg, height 67 inches and weight 104 kg. The skin had good turgor without rash. The pupils were equal and reactive to light and accommodation. The retinal examination was normal. Oral mucosa revealed no thrush or intraoral lesions. Examination of the neck revealed an enlarged right cervical lymph node 4 cm in diameter and an enlarged left cervical lymph node 3 cm in diameter. The enlarged lymph nodes were nontender and firm. In addition there were multiple small cervical lymph nodes palpable bilaterally. There was also a left supraclavicular lymph node 2 cm in diameter. Examination of the heart and lungs was normal. The abdomen was soft and nontender without enlargement of the liver or spleen. Neurologic examination was also normal. The upper anterior chest wall, at the midclavicular line, showed an area of ulceration 5 cm long and 2 cm wide, with a 5-mm opening, which at the time produced no discharge.

Laboratory tests included a white blood cell count of 11 900/mm3 with 54% segmented neutrophils, 37% lymphocytes, 5% monocytes and 4% eosinophils. Hemoglobin was 10.6 g/dl with a platelet count of 366 000/mm3. The erythrocyte sedimentation rate was 37/mm/h; urinalysis showed no red or white blood cells and no casts. Purified protein derivative intradermal skin test showed no induration or erythema in 48 h. Quantitative serum immunoglobulin concentrations revealed IgG 1890 mg/dl, IgM 260 mg/dl and IgA 340 mg/dl. A nitroblue tetrazolium test was normal at 99%. Computed tomographic scan of the neck and chest revealed diffuse cervical and supraclavicular lymphadenopathy with sinus tracts extending from the supraclavicular nodes to the anterior chest wall. There were no areas of focal consolidation or infiltrates in the lungs. A diagnostic procedure was performed.

Histologic examination of the supraclavicular lymph node biopsy showed the presence of spherules consistent with Coccidioides immitis infection. A culture of the lymph node grew C. immitis. Complement fixation antibody titer for C. immitis was 1/562. Cerebrospinal fluid examination revealed 0 white blood cells, 0 red blood cells, glucose 67 mg/dl and protein 20 mg/dl. Complement fixation antibodies for C. immitis in the cerebrospinal fluid were negative. The patient was treated with oral itraconazole 200 mg twice a day. At 4-month follow-up, there was complete resolution of the sinus tract, lymphadenopathy and the chest wall fistula.

C. immitis is a dimorphic fungus indigenous to the soils of southwestern United States and certain areas of South America. Infection occurs with inhalation of airborne organisms.[1,2] Events that result in increased airborne spores are associated with increased incidence of disease. Large dust clouds and landslides in surrounding areas followed a magnitude 6.7 earthquake in Northridge, in Los Angeles County, in 1994. Case-control studies showed that residence in these areas during this time increased the risk of acquiring coccidioidomycosis.[3] Primary infection is asymptomatic in 60% of patients. Symptomatic disease, when it occurs, usually involves a self-limited lower respiratory infection. A few patients develop significant pulmonary or disseminated infection. The most common sites of dissemination are skin, joints, bone and meninges.[1,2] Patients with advanced human immunodeficiency virus infection and those receiving chronic high dose steroids are also at greater risk for progressive disease.[2] Increased rates of dissemination are also found in persons of African-American and Filipino ancestry, although the exact reason for this is not clear.

Chest wall fistula formation is an uncommon clinical presentation of coccidioidomycosis and is not mentioned in standard textbooks and literature.[1,2,4] The differential diagnosis of this patient on admission included tuberculosis, chronic pulmonary infections and actinomycosis. The exact mechanism for the development of thoracic fistula formation in this patient is not known. Possible mechanisms include direct extension from the infected cervical or supraclavicular lymph nodes and/or primary dissemination to the subcutaneous tissue during initial infection.

A variety of means are used to diagnose Coccidioides infection. The standard remains isolation of the organism from a clinical specimen such as spinal fluid, bronchoalveolar lavage or tissue biopsy.[2] Handling of the organism can be hazardous, and laboratory personnel should be notified if coccidioidomycosis is suspected when the specimen is submitted. Serology is very useful in diagnosis and follow-up of patients with suspected or proven coccidioidomycosis. IgM antibodies can often be detected in the first weeks of disease. IgG antibodies, which fix complement, usually appear within the first month. The complement fixation antibody is often nonreactive in primary pulmonary infection but is nearly always reactive in disseminated disease.[5] The high complement fixation titer in the reported patient suggests the presence of extrapulmonary dissemination.

Treatment is usually reserved for patients with significant pulmonary or extrapulmonary disease.[4] Amphotericin is often used for patients with rapidly progressive disease. Triazoles are often used in patients with more chronic processes. A recent study suggested that itraconazole has modest superiority over fluconazole as treatment.[6]

The role of surgical intervention in pediatric coccidioidomycosis has recently been reviewed. Often the primary surgical role will be obtaining specimens (such as lymph node or pulmonary nodule) for biopsy and culture. Often patients who are immunocompromised or have disseminated disease require further surgery such as pulmonary resection or bone debridement.[7] In this particular case excision of the fistula was not required.