A 4-year-old boy was brought to the Emergency Department (ED) from a rural area with complaints of fever, headache, and weight loss. He was suffering from these symptoms for the past 4 months, which had worsened in the past week. His brother informed that the child had been unwell since infancy with several episodes of respiratory infection and has had several hospital admissions. Recently, he had started complaining of severe persistent diffuse headaches along with nausea, vomiting, and anorexia. He also suffered from low grade fever intermittently over this period of time. Physicians in his locality who had treated him with antibiotic courses for chest infections started him on antituberculosis (ATT) drugs 2 months back after an MRI, which suggested meningeal enhancement and small ring enhancing lesions. He had no history of seizures or loss of consciousness. One of his brother had passed away at the age of 8 years who was considered to suffer from disseminated tuberculosis. On examination, the child was listless, emaciated, and extremely irritable. He had left-sided weakness in both the upper and lower extremities.
An urgent computed tomography (CT) scan of the head showed multiple rings enhancing the left parietooccipital region, with significant surrounding edema and mass effect [Figure 1a]. His white blood cell count was 17900/uL, with neutrophils 77.4% and lymphocytes 15.7%. Hemoglobin was 10.9 and erythrocyte sedimentation rate (ESR) was 88 mm/h. We involved pediatric physicians and infectious disease specialists to perform a neuronavigation-guided craniotomy for the excision of these abscesses along with the walls, which contained thick purulent material. Microscopic examination revealed granulomas with fugal hyphae [Figure 2a and b]. Presence of Aspergillus was further confirmed on culture. Postoperatively, he was successfully extubated and shifted to the intensive care unit for postoperative monitoring. His weakness also improved. Considering his chronic cough and repeated chest infections, we obtained a tracheal aspirate for examination, which was positive for Mycobacterium tuberculosis polymerase chain reaction assay. Considering the history, we screened the child for immune deficiency disorders. Nitroblue-tetrazolium (NBT) was found to be negative, which established the diagnosis of CGD.
Intravenous (IV) voriconazole was commenced at 6 mg/kg/dose every 12 h for 2-3 days, which was later switched to 6 mg/kg/day orally. Five-drug ATT treatment was restarted for the management of pulmonary TB. He was also given broad-spectrum antibiotics. Pneumococcal and meningococcal vaccines were administered as prophylaxis, and co-trimoxazole was included in the regimen for the prevention of pneumocystis carinii pneumonia.
The child showed slow improvement, however, the treatment course was complicated by fever, seizures, and hyponatremia. Repeat CT scans did not show residual or recurrent abscesses [Figure 1b]. After stabilization of the medical condition, he was discharged. About 10 days after discharge, his family brought him to the ED with complaints of drowsiness. CT scan showed hydrocephalus [Figure 1c]. A temporary cerebrospinal fluid (CSF) diversion was performed, followed by a ventriculoperitoneal shunt once the CSF culture was reported negative. The child was again discharged after stabilization. He died 4 months later at home in respiratory distress. The child could not be brought to us, and hence no autopsy was performed to determine the actual cause of his death.