A 47-year-old female presented to our outpatient department with complaints of drooping of the right eyelid and painless swelling of the right cheek for 1 year. She also complained of difficulty in mouth opening for the past 6 months. The swelling had been progressively increasing and showed no postural variation or pulsatility. The mouth opening was restricted to less than 1 finger, and the patient could drink only liquids using a straw. At the time of presentation, she did not give any history of fever or past medical history of diabetes, high-risk behavior, heart problems, or any other conditions that would predispose her to an intracranial infection.
On examination, the swelling was nontender, hard diffuse without any clear margins or defined edges. It was arising from the maxilla and was not independently mobile off the same. The right eye proptosis was axial and nonpulsatile with no extraocular movement restriction. The vision was 6/24 in both eyes on Snellen's chart. Rinne's test showed that the right ear had no air or bone conduction while on the left side air conduction was more than bone conduction. Weber test was lateralized to the left side. Rest of the examination was unremarkable. Laboratory investigations were normal.
After clinical evaluation, a large lesion involving the maxilla, orbit, and temporal base with extension to the middle ear was suspected of the possibility of slow-growing neoplasm being a possibility. She underwent a computed tomography (CT) scan, which showed diffuse hyperostosis of the right frontal, squamous temporal, sphenoid and zygomatic bones, and an en-plaque ground glass lesion, involving the basitemporal bone [Figure 1a and b]. On magnetic resonance imaging (MRI), lesion showed a T1 hypointense and T2 hyperintense contrast-enhancing extra-axial lesion located in the right frontotemporal bone with dural thickening and possible brain parenchymal involvement [Figure 1c and d]. Extension was also noted into the right temporomandibular joint and maxillary sinus and orbit, explaining her clinical presentation.
Diagnosis of an en-plaque meningioma was suspected and she underwent a right frontotemporal craniotomy and complete excision of the intracranial lesion and partial decompression of involved areas of bone [Figure 1e], with areas of dural involvement [Figure 1f] being reconstructed with a Fascia lata graft taken from the right thigh. Intraoperative frozen section revealed a granulomatous lesion, and hence, aggressive resection was avoided. Postoperatively, the patient was conscious and without any fresh neurological deficits.
Due to the endemic nature of tuberculosis in India and squash report of pathology, diagnosis of tubercular granulomatous inflammation was made and the patient was started on antitubercular drug therapy and discharged on antitubercular therapy with a hospital stay of 5 days. She was also put on antiepileptic drugs (Phenytoin) for prevention of postoperative seizures and steroids to counteract tubercular arteritis. Human immunodeficiency virus (HIV) was negative on repeated testing from two different centers. However, she reported back to the emergency 3 days after discharge in an unconscious state. According to the relatives, patient was complaining of mild headache since the time of discharge, and on the 3rd day after discharge, in the morning patient was found unconscious in her bed. At the time of presentation, patient vitals were not recordable, her GCS was 3/15, with bilateral pupils nonreactive and fixed. She was immediately given cardiopulmonary resuscitation, but could not be revived despite aggressive resuscitative measures. The exact cause of death could not be ascertained, but could be flare up of the primary disease as later seen in brain autopsy.
Subsequent histopathological analysis revealed a suppurative granulomatous inflammatory pathology with dense fibrosis of the stroma. The thickened fibrotic dura showed multiple discrete and confluent suppurative microabscess rimmed by histiocytes and granulomatous inflammatory cells along with multinucleate giant cells (foreign body type). Many of the microabscesses showed colonies and collections of slender filamentous bacteria with evidence of neutrophilic reaction. Rest of the stroma showed dense fibrosis with scattered lymphoplasmacytic infiltrate and reactive blood vessels. The adjacent brain parenchyma included showed cerebritis. The colonies were highlighted on Grams stain and Gomori methenamine silver stain. Staining for acid-fast bacilli was negative for tubercular and Nocardia species. These findings on histopathological examination resulted in the final diagnosis of a chronic necrotizing granulomatous inflammation with Actinomyces-like organisms [Figure 2].
Partial autopsy limited to the removal of brain revealed presence of bacterial colonies with a suppurative granulomatous inflammation, as seen in the biopsied tissue. There were no other lesions on the external examination at autopsy.