A 22-year-old female with a long history of infection with the human immunodeficiency virus (HIV)/AIDS presented to the emergency department at Olive View-UCLA Medical Center with ear pain, weight loss, malaise, and night sweats. The patient was first diagnosed with HIV at age 9, and it was uncertain if she contracted HIV congenitally from her mother, who was HIV positive, or from a subsequent sexual assault by her stepfather, who was also HIV positive. The patient started highly active antiretroviral therapy (HAART) thereafter, but she had been homeless at times and had found it difficult to access care. This difficulty in access resulted in periods of compliance punctuated by periods of medication nonadherence. Prior to this presentation, she had been treated for pulmonary MAI infection as well as Pneumocystis jiroveci pneumonia at an outside institution in 2008.
In August of 2009 the patient was diagnosed with pulmonary MAI and later with disseminated cutaneous MAI when a rash on her upper arms and thighs was biopsied and the culture returned positive for MAI. In October of 2009 the patient reported a history of a MAI bone infection in her left thumb; subsequently, the infection was surgically removed and replaced with filler, with a plan for a future bone graft. The patient was placed on the following HAART regimen, with which she was noncompliant: emtricitabine-tenofovir, raltegravir, and etravirine. The patient's MAI regimen, with which she was only sporadically compliant, was azithromycin, dapsone, and levofloxacin.
After coming to the emergency department, the patient was started on empiric broad-spectrum antibiotics (vancomycin and piperacillin-tazobactam) and was noted to be hypothermic to 35.5 C but hemodynamically stable. The patient's left ear was noted to be tender on manipulation of the pinna, and her left external ear canal was filled with purulent discharge, obscuring her left tympanic membrane from view. The patient's lung and cardiovascular examinations were unrevealing, but hepatomegaly and splenomegaly were noted (liver span, about 20 cm; spleen size, 16 cm). Her upper arms and thighs were notable for an annular erythematous lesion that was reportedly biopsied at an outside hospital and showed cutaneous MAI infection. Her neurological examination was most notable for bilateral brisk (3+/5+) reflexes.
The patient's laboratory testing on admission showed that her electrolytes and liver function tests were normal, except for a mildly elevated alkaline phosphatase level and a low serum albumin level, likely secondary to malnutrition. The patient was noted to be leukopenic and her complete blood count showed a microcytic hypochromic anemia, but she was not noted to be thrombocytopenic.
HIV serologies and the HIV polymerase chain reaction test were positive, with HIV RNA at 236,119 copies/mL. The patient suffered a seizure 2 days after admission and thus cerebrospinal fluid (CSF) analysis was obtained, the results of which were negative. The Veneral Disease Research Laboratory test, M. tuberculosis serological testing, and serology for Coccidioidomycosis immits, Histoplasma Capsulatum, and Cryptococcus Neoformans were all negative.
The patient received a computed tomography (CT) scan of her brain on the day of admission, given the ear and mastoid process pain and the abnormal findings on neurological examination. The CT scan showed opacification of the mastoid air cells and probable left otomastoiditis, so magnetic resonance imaging (MRI) was subsequently performed. A CT of the temporal bone showed left otomastoiditis, generalized cerebral atrophy atypical for age and consistent with HIV, and a rim of aeration around debris in the right mastoid air cells, which also suggested some infection. A sagittal cut of the left mastoid from the preoperative CT, the right mastoid from the preoperative CT, and the post operative CT scan showing the appearance of the left mastoid process after surgery are shown for comparison in Figure 1.
Based on the patient's weight loss and low CD4 count, a disseminated OI was suspected. Imaging of the ear canal/temporal bone and ear was obtained, given the patient's ear pain. The imaging clearly showed otomastoiditis, and, given the clinical picture of ear pain, a mastoidectomy was scheduled and bone biopsies were obtained to identify the causative organism.
Given the patient's night sweats and weight loss, there was a concern for M. tuberculosis infection - particularly, disseminated tuberculosis. Tuberculosis was not isolated from the bone biopsy tissue culture or from any other body fluid culture. However, MAI was identified on several smears and cultures of induced sputum, an aspirate from a left supraclavicular lymph node biopsy, and several stool specimens. A skin biopsy of the patient's skin lesions again showed MAI on a smear. As mentioned, the patient suffered a seizure 2 days after admission, and CSF was obtained via lumbar puncture as part of a workup to investigate whether her seizure was due to an infectious etiology. The CSF analysis initially showed no leukocytes, and an acid-fast bacillus smear was also negative, but the culture of the CSF grew MAI several weeks later.
The patient received a brain MRI after the seizure, and another MRI was performed some weeks after that episode. The first brain MRI (Figure 2A) showed left-sided cerebral edema of the occipital and temporal lobes and a radiographic appearance consistent with PRES. The follow-up MRI performed after the patient's discharge from hospital showed resolution of the cerebral edema and the cerebritis/meningitis (Figure 2B).
A chest X-ray of the patient revealed a wide mediastinum and was suggestive of pulmonary hilar lymphadenopathy. A CT scan of the chest was thus performed and this confirmed diffuse hilar lymphadenopathy of the chest. The largest lymph node measured 4.5 x 3.0 cm. The most demonstrative chest CT images are shown in Figure 3.
After the diagnosis and the medical and surgical treatment of MAI otomastoiditis, the patient was continued on moxifloxacin, ethambutol, rifabutin, azithromycin, and dapsone. The patient was discharged and readmitted twice with continuing sequelae of disseminated MAI infection. She continues to suffer from overwhelming MAI infection, and she has a low CD4 count that has not yet improved as IRIS has delayed the restart of HAART. The patient has also suffered side effects from the five-medication MAI regimen outlined earlier - namely, pancytopenia due to the disseminated MAI infection and the dapsone. The patient remains on prophylactic trimethoprim-sulfamethoxazole, given her history of Pneumocystis Jirovecii pneumonia; on fluconazole as fungal infection prophylaxis; and on acyclovir as varicella zoster prophylaxis, given her low CD4 count. The patient remains critically ill and her MAI remains refractory to the multidrug treatment regimen.