Acta Neurochir (Wien) (1996) 138:944-950 Acta Neurochirurgiea 9 Springer-Verlag1996 Printed in Austria Cranial and Intracranial Aspergillosis of Sino-Nasal Origin Report of Nine Cases Naim-Ur-Rahman 1, A. Jamjoom, S. S. A. AI-Hedaithy 2, Z. A. B. Jamjoom t, M. O. AI-Sohaibani 3, and S. A. Aziz 3 1 Division of Neurosurgery, College of Medicine, King Saud University, Riyadh, Saudi Arabia, 2Department of Microbiology, College of Medicine, King Saud University, Riyadh, Saudi Arabia, and 3Department of Pathology, College of Medicine, King Saud University, Riyadh, Sandi Arabia Summary This paper is an attempt at defining the most efficacious surgi- cal and antifungal therapy for invasive cranial and intracranial aspergillosis, and is based on experience with nine non-immuno- compromised patients treated and followed-up by the authors between 1983 and 1994; as well as on the summary of previously reported cases and advances in therapy of this condition. Depend- ing on the degree of aspergillar involvement of the cranial base and intracranial structures, a classification, with implications for treat- ment and prognosis, is also proposed. Two patients had extracranial skull base erosion; whereas relentlessly progressive granulomas, mimicking malignancy, invaded the skull base and intracranial contents in seven cases. Of these seven patients with cranial and intracranial invasion, two died of acute intracranial haemorrhage due to fungal invasion of cere- bral blood vessels. In two patients, complete surgical eradication of the disease proved impossible due to cavernous sinus involvement, while residual aspergillomas are still present in orbit and paranasal sinuses (PNS) in a further two patients in spite of multiple surgical procedures and prolonged antifungal chemotherapy (AFC). What appears to be a cure has been effected in one patient only. Multiple therapeutic strategies were used. Biopsy plus systemic AFC was ineffective, surgical drainage and debridement plus systemic AFC resulted in long-term survivals but no cure. Radical surgery in con- junction with systemic and local (intracavitary) AFC should be considered to improve an otherwise poor prognosis. Keywords: Antifungal chemotherapy; invasive aspergillosis; invasive skull base mycosis; skull base surgery. Introduction Invasive aspergillosis is characterized by tissue invasion with aspergillus hyphae (Fig. 2) and, unlike the non-invasive forms, it has a high morbidity and mortality [1, 4, 7, 16]. Aspergillosis of the central ner- vous system (CNS) is often a fatal disease, regardless of the mode of therapy [4]. Even with surgical and antifungal therapy, very few long-term survivors of cranial and intracranial aspergillus granuloma have been reported in the literature [4, 7]. Most often seen in the immunocompromised host, it is notoriously refractory to systemic antifungal agents and intrathe- cal antimycotics [4]. Classically, aspergillus infection of the CNS is caused by a haematogenous spread from the lung [11, 16] and manifests itself as meningitis, cerebritis, abscess, granuloma or vasculitis [4]. The cases pre- sented here were unusual in many ways; thus direct extension from nose and PNS was responsible for CNS involvement, patients were non-immunocom- promised and the disease ran a more protracted course. Pathological lesions and tissue reaction to fungal infection was also atypical. Thus, chronic granulomatous inflammation and dense fibrosis with embedded septate hyphae, some engulfed by multinu- cleated giant cells (Fig. 2) produced expanding mass lesions. Suppuration and abscess formation was rarely seen. Because of these atypical features, the initial diagnosis was in error in five out of nine cases. The commonest misdiagnoses included primary and sec- ondary cranial and intracranial tumours. Treatment of these invasive forms of cranial and intracranial aspergillosis is most often ineffective and usually requires multiple therapeutic strategies, including systemic and local antifungal agents and surgery, ranging from biopsy to more radical proce-