CALVARIAL TUBERCULOSIS IN AN ADOLESCENT GIRL WITH NEUROFIBROMATOSIS TYPE 1

Objectives: We report a 10 year-old girl known to have Neurofibromatosis type 1 (NF-1) who presented with headache of 9 days duration, accompanied by fever, vomiting, pallor, and left ear discharge. A year before her admission, she developed slowly enlarging mass lesions over the lateral aspect of the left supraorbital, left posterior parietal and the right parieto-occipital areas. Methods: Work-up included cranial MRI, cranial and chest CT scans, chest x-rays, blood culture, ear discharge Gram stain, CBC's, Mantoux test, naproxen test, biopsies with AFB and PAS stains of the masses and underlying bone at the right parieto-occipital and left supraorbital areas as well as cervical and mediastinal lymph nodes, TB culture and electron microscopy.

Results: The patient had anemia (<10g/dL). Chest x-rays and CT scan showed several mediastinal masses and a lobulated density on the right side. The cranial MRI and CT scan demonstrated the aforementioned extra-axial mass lesions with noted orbital extension and osteolytic destruction. All biopsies showed chronic granulomatous inflammation with normal mature bone. Gram stain, blood culture, AFB and PAS stains and naproxen test were negative. Mantoux test was positive. No Birbeck's granules were seen on electron microscopy. Conclusion: Our dilemma was whether the mass lesions were part of NF-1 and if these were neoplastic or infectious. The biopsies showed no evidence of a neoplastic process. We considered Langerhans' cell histiocytosis but Birbeck's granules were absent. With a finding of chronic granulomatous inflammation, a positive Mantoux test, and the endemicity of tuberculosis in our country, a therapeutic trial of quadruple anti-Koch's was initiated. Follow-up showed marked improvement.