Aspergillosis in the central nervous system (CNS) is a very rare disease in immune-competent patients. There was a case of a healthy man without a history of immune-compromised disease who had invasive aspergillosis with unusual radiologic findings. A 48-year-old healthy man with diabetes mellitus, presented with complaints of blurred vision that persisted for one month. Brain magnetic resonance imaging (MRI) showed multiple nodular enhancing lesions on the right cerebral hemisphere. The diffusion image appeared in a high-signal intensity in these areas. Cerebrospinal fluid examination did not show any infection signs. An open biopsy was done and intraoperative findings showed grayish inflammatory and necrotic tissue without a definitive mass lesion. The pathologic result was a brain abscess caused by fungal infection, morphologically aspergillus. Antifungal agents (Amphotericin B, Ambisome and Voriconazole) were used for treatment for 3 months. The visual symptoms improved. There was no recurrence or abscess pocket, but the remaining focal enhanced lesions were visible in the right temporal and occipital area at a one year follow-up MRI. This immune-competent patient showed multiple enhancing CNS aspergillosis in the cerebral hemisphere, which had a good outcome with antifungal agents.
Aspergillosis; Cerebral; Enhancement; Immune-competent; Multiple
We describe a 20-year-old man diagnosed with a myelodysplastic syndrome (MDS), admitted to our hospital due to pancytopenia and fever of undetermined origin after myelosuppression with chemotherapy. Disseminated aspergillosis (DIA) was suspected when he developed skin and lung involvement. A rapidly growing mass was detected on the left neck area, during hospitalization. A thyroid ultrasound reported a 3.7 × 2.5 × 2.9 cm oval heterogeneous structure, suggestive of an abscess versus a hematoma. Fine needle aspiration of the thyroid revealed invasion of aspergillosis. Fungal thyroiditis is a rare occurrence. Thyroid fungal infection is difficult to diagnose; for this reason it is rarely diagnosed antemortem. To our knowledge, this is the 10th case reported in the literature in an adult where the diagnosis of fungal invasion to the thyroid was able to be corroborated antemortem by fine needle aspiration biopsy.
Cerebral abscesses are rare but often lethal complications of invasive Aspergillus sinusitis. Treatment is difficult and usually depends on a combination of neurosurgical drainage and intravenous antifungal therapy. We report a case of intracerebral frontal abscess in continuity with the anterior skull base in a 53-year-old immunocompromised female with invasive rhino-orbital aspergillosis. An aperture was created by drilling the anterior skull base during endonasal sinus surgery, and the abscess was drained through its lower pole. The aperture was left open after surgery to ensure complete abscess drainage. Follow-up at 8 months revealed no cerebrospinal fluid leak or meningitis, and no abscess recurrence. This case validates the feasibility of endonasal trans-ethmoidal drainage of intracranial abscesses.
Rhino-orbital sinusitis; invasive aspergillosis; brain abscess; endonasal sinus surgery; trans-ethmoidal access
Invasive fungal infections usually affect patients with immunodeficiencies and very rarely patients with no known or identifiable risk factors. Diagnosis could be delayed in patients without previously known immunodeficiencies due to a low index of suspicion, leading to a delay in treatment and a potential poor outcome. We report a case of a postpartum woman with no history of immuno-compromised disease who developed left hemiparesis with evidence of invasive aspergollosis affecting the nervous system, and leading to fatal outcome. The patient had a mass-like lesion in the neuroimaging with soft tissue shadowing in the chest x-ray leading to initial diagnosis of tuberculosis. The brain biopsy showed changes consistent with a diagnosis of aspergillosis. The source of the aspergillus infection was not clear. Aspergillus infection should be considered in patients with no identifiable immunodeficiencies who have abnormal brain imaging and chest x-ray, as early treatment may alter the outcome.
Cerebral angioinvasion is a fatal complication of disseminated aspergillosis and has been rarely described in diabetic population in the absence of ketoacidosis. We present a case of invasive fungal sinusitis in a 79 year old diabetic man who presented with chronic frontal headaches. Despite appropriate medical and surgical treatment, the disease progressed and the patient eventually succumbed to a fatal ruptured mycotic aneurysm. We emphasize that clinicians should consider this in the differential diagnoses of all diabetics who present with chronic sinusitis, as early diagnosis could be the key in the successful treatment.
Invasive aspergillosis is a serious complication of solid organ transplantation. An early diagnosis is hampered by the lack of reliable serum markers and, even if appropriately diagnosed and treated with current antifungal agents, has a high mortality rate. We report a case of invasive pulmonary and cerebral aspergillosis in a renal transplant patient treated with IFN-γ in conjunction with combination anti-fungal therapy for six weeks in whom complete resolution of the fungal infection was achieved. Renal function remained intact throughout the treatment period. Surveillance CT scans of the chest and head showed resolution of prior disease but revealed a new left upper lobe mass four months after completion of treatment with IFN-γ. Biopsy of the lesion was positive for primary lung adenocarcinoma, for which she underwent left upper lobe resection. The pathology report confirmed clear surgical margins and lymph nodes and no evidence of fungal hyphae. IFN-γ should be considered early in the management of invasive aspergillosis in renal transplant patients. To date, allograft rejection has not been encountered.
Astroblastomas are uncommon neuroepithelial tumors of uncertain origin. These occur predominantly in the cerebral hemisphere of young adults and children. They form only 0.45–2.8% of all neuroglial tumors. They can be easily misdiagnosed as they are rarely encountered in clinical practice and share common radiological and histopathologic appearance with other glial neoplasms. A 12-year-old female presented to us with progressive headache and diplopia. Her neurological examination showed right 6th nerve paresis with papilledema. Brain magnetic resonance imaging (MRI) revealed well-demarcated, peripherally enhancing solid cystic mass of 6 cm in right parietal lobe with mass effect. She underwent gross total resection of the lesion through right parietal craniotomy. The histopathologic diagnosis was suggestive of low-grade astroblastoma. The patient had no evidence of recurrence of tumor without adjuvant radiotherapy during the last 14 months of follow-up. Supratentorial astroblastomas are very rare tumors. Complete excision without radiotherapy is sufficient in low-grade variants.
Astroblastoma; cerebral; report
We report the first case of extensive involvement of isolated intracranial Rosai-Dorfman's disease (RDD) in a child. Our case is unique because it presents with involvement of the middle cranial fossa, cavernous sinus, pituitary gland, orbit, ethmoid and sphenoid sinuses. Previous cases of intracranial RDD in children have reported separate involvement of cavernous sinus, suprasellar region, and frontal and petroclival regions. Involvement of the pituitary gland has so far not been reported. A 14-year-old male presented with a medical history of loss of vision, raised erythrocyte sedimentation rate (ESR), and abnormal prolactin and cortisol levels. Radiologically the diagnosis was meningioma. The histopathological diagnosis was RDD with emperipolesis and S-100 positivity. RDD is a histiocytic proliferation of unknown aetiology, which commonly affects lymph nodes. Uncommonly it involves the extranodal sites and rarely the central nervous system (CNS). 80 cases of RDD involving CNS have been reported in the literature, and only 5 were in children. Although the definitive diagnosis of RDD disease is based on the histopathology report, it should be included in the differentials of a lesion mimicking meningioma especially in children.
To demonstrate a variety of MR imaging findings of orbital inflammatory pseudotumors with extraorbital extension.
Materials and Methods
We retrospectively reviewed the MR features of five patients, who were diagnosed clinically and radiologically as having an orbital inflammatory pseudotumor with extraorbital extension.
The types of orbital pseudotumors were a mass in the orbital apex (n = 3), diffuse form (n = 2), and myositis (n = 1). The extraorbital extension of the orbital pseudotumor passed through the superior orbital fissure in all cases, through the inferior orbital fissure in two cases, and through the optic canal in one case. The orbital lesions extended into the following areas: the cavernous sinus (n = 4), the middle cranial fossa (n = 4), Meckel's cave (n = 2), the petrous apex (n = 2), the clivus (n = 2), the pterygopalatine fossa and infratemporal fossa (n = 2), the foramen rotundum (n = 1), the paranasal sinus (n = 1), and the infraorbital foramen (n = 1). On MR imaging, the lesions appeared as an isosignal intensity with gray matter on the T1-weighted images, as a low signal intensity on the T2-weighted images and showed a marked enhancement on the post-gadolinium-diethylene triamine pentaacetic acid (post-Gd-DTPA) T1-sequences. The symptoms of all of the patients improved when they were given high doses of steroids. Three of the five patients experienced a recurrence.
MR imaging is useful for demonstrating the presence of a variety of extraorbital extensions of orbital inflammatory pseudotumors.
Orbit, Inflammation; Orbit, Pseudotumor; Orbit, MR
Rhinocerebral mucormycosis is an acute fulminant opportunistic fungal infection usually seen in diabetic or immunocompromised patients. The fungi that cause mucormycosis inoculate the nasal mucosa and may spread to the paranasal sinuses, orbit, and brain. Our patient initially presented with mild ethmoid sinusitis. At that time, brain MRI and contrast-enhanced MR angiography were grossly normal. However, aggravation of sinusitis with extension to the right orbit and anterior cranial fossa rapidly developed within two months. Moreover, an occlusion of the right internal carotid artery was combined. We report a case of a pathologically-proven rhino-orbital-cerebral mucormycosis with serial follow-up imaging for over one year.
Mucormycosis; Carotid artery, Internal; Fungal sinusitis
Allergic bronchopulmonary aspergillosis (ABPA) is a common but frequently misdiagnosed clinical condition. It is usually diagnosed in patients with a long standing history of asthma. Patients with ABPA can have diverse radiological manifestations. Very rarely, lung masses have been reported. We hereby report a case of ABPA in whom a large symptomatic lung mass was the presenting manifestation leading to consideration of lung cancer as a differential diagnosis. The establishment of ABPA as the underlying diagnosis led to conservative medical treatment which was followed by complete resolution of the mass like opacity. The present case highlights that ABPA should be considered as a differential diagnosis whenever encountering a patient with lung mass and history of asthma.
Aspergillosis, allergic bronchopulmonary; asthma; mass
Cylindrocarpon lichenicola is a saprophytic soil fungus which has rarely been associated with human disease. We report the first case of localized invasive cutaneous infection caused by this fungus in a 53-year-old male from the rural midwestern United States with relapsed acute myelogenous leukemia. On admission for induction chemotherapy, the patient was noted to have an abrasive laceration between the fourth and fifth metacarpophalangeal joints and on the dorsum of the right hand, which progressed to frank ulceration following chemotherapy. A biopsy provided an initial diagnosis of an invasive fungal infection consistent with aspergillosis based on the histopathological appearance of the mold in tissue. Multiple positive fungal cultures which were obtained from the biopsied tissue were subsequently identified by microscopic and macroscopic characteristics to be C. lichenicola. The infection resolved following marrow regeneration, aggressive debridement of the affected tissue, and treatment with amphotericin B. This case extends the conditions associated with invasive disease caused by C. lichenicola.
Hematomas caused by ruptured traumatic pseudoaneurysms of the middle meningeal artery (MMA) usually present with extradural hematomas, whereas intradural intraparenchymal hematomas are extremely rare. We report a case of traumatic pseudoaneurysm of the MMA giving rise to an intracerebral hematoma after head trauma. A 70-year-old man suffered a massive intracerebral temporoparietal hemorrhage after a head injury. CT angiogram of the brain revealed a large hematoma in the right middle cranial fossa extending to the right sylvian fissure. Cerebral angiogram also revealed a pseudoaneurysm of the MMA, which was successfully treated surgically. Although traumatic MMA pseudoaneurysm producing intracerebral hematoma (ICH) is rare, it should be considered as a possible cause of intracerebral hematoma.
Traumatic pseudoaneurysm; Middle meningeal artery (MMA); Intracerebral hematoma
A 69-year old man presented to us with decreased vision in his right eye and a relative afferent pupillary defect. Under the presumption that he was suffering from retrobulbar optic neuritis or ischemic optic neuropathy, visual field tests were performed, revealing the presence of a junctional scotoma. Imaging studies revealed tumorous lesions extending from the sphenoid sinus at the right superior orbital fissure, with erosion of the right medial orbital wall and optic canal. Right optic nerve decompression was performed via an endoscopic sphenoidectomy, and histopathologic examination confirmed the presence of aspergillosis. The patient did not receive any postoperative antifungal treatment; however, his vision improved to 20 / 40, and his visual field developed a left congruous superior quadrantanopsia 18 months postoperatively. A junctional scotoma can be caused by aspergillosis, demonstrating the importance of examining the asymptomatic eye when a patient is experiencing a loss of vision in one eye. Furthermore, damage to the distal optic nerve adjacent to the proximal optic chiasm can induce unusual congruous superior quadrantanopsia.
Aspergillus; Hemianopsia; Optic nerve; Scotoma
Aspergillus fumigatus is one of the most ubiquitous of the airborne saprophytic fungi. Humans and animals constantly inhale numerous conidia of this fungus. The conidia are normally eliminated in the immunocompetent host by innate immune mechanisms, and aspergilloma and allergic bronchopulmonary aspergillosis, uncommon clinical syndromes, are the only infections observed in such hosts. Thus, A. fumigatus was considered for years to be a weak pathogen. With increases in the number of immunosuppressed patients, however, there has been a dramatic increase in severe and usually fatal invasive aspergillosis, now the most common mold infection worldwide. In this review, the focus is on the biology of A. fumigatus and the diseases it causes. Included are discussions of (i) genomic and molecular characterization of the organism, (ii) clinical and laboratory methods available for the diagnosis of aspergillosis in immunocompetent and immunocompromised hosts, (iii) identification of host and fungal factors that play a role in the establishment of the fungus in vivo, and (iv) problems associated with antifungal therapy.
Chronic necrotizing pulmonary aspergillosis usually occurs in mildly immune-compromised hosts or those with underlying pulmonary disease. The radiographic pattern of chronic necrotizing pulmonary aspergillosis is typically a progressive upper lobe cavitary infiltrate with pleural thickening. We report here an atypical case of chronic necrotizing pulmonary aspergillosis mimicking lung cancer, which developed into a disseminated fatal disease in an older woman with no comorbidity.
An 80-year-old Japanese woman was referred to our hospital for a chest roentgenogram abnormality. Repeated fiber-optic bronchoscopy could not confirm any definite diagnosis, and she refused further examinations. Considering the roentgenogram findings and her age, she was followed-up as a suspected case of lung cancer without any treatment. Then, 10 months later, she complained of visual disturbance and was admitted to our department of ophthalmology. She was diagnosed as having endophthalmitis. After treatment with corticosteroids for 20 days, she developed acute encephalitis and died four weeks later. Autopsy revealed dissemination of Aspergillus hyphae throughout her body, including her brain.
In older patients, even if they do not have any comorbidity, chronic necrotizing pulmonary aspergillosis should be added to the differential diagnosis of solitary pulmonary lesions in a chest roentgenogram.
Spontaneous spinal epidural haemorrhage is a rare condition. The initial clinical manifestations are variable. Nonetheless, most spinal cord lesions result in paraparesis or quadriparesis, but not hemi-paresis, if motor function is involved. We report on a 69-year-old man who presented initially with right-side limb weakness. He was initially misdiagnosed at emergency room with a cerebral stroke and treated inappropriately with heparin. One day after admission, correct diagnosis of acute spinal epidural haematoma was based on the repeated neurological examination and cervical magnetic resonance imaging study. The patient underwent emergency surgical decompression and hematoma removal. The pathogenesis of the haematoma could have been due to hypertension, increased abdominal pressure and anticoagulant therapy. We emphasize that patients with hemi-paresis on initial presentation could have an acute spinal epidural haemorrhage. We also draw the misdiagnosis to the attention of the reader because early recognition of spontaneous spinal epidural haematoma is very important for prompt and appropriate treatment to improve the overall prognosis.
Spontaneous spinal epidural haemorrhage; Hemi-paresis; Cerebrovascular disease
Cavernous haemangioma(CH) is mostly intraconal, single and unilateral in location. A 32 year old female presented with painless progressive growth near the inner canthus of right eye along with swelling of right upper eyelid and superomedial quadrant of the right orbit for five years. MRI showed lesions involving the preseptal space and extraconal compartment of the orbit at the superomedial aspect continous with the conjunctival swelling. Excision biopsy of the growths via anterior orbitotomy (vertical eyelid split technique) was done. Histopathological findings confirmed orbital cavernous haemangioma along with conjunctival CH.
We report a rare case of multiple cavernous haemangiomas arising from the conjunctiva as well as the superomedial orbit. Complete removal of the tumour is possible even in such difficult cases as the CH is totally encapsulated and meticulous surgical dissection can give good cosmetic result to the patient.
Carcinoma ex pleomorphic adenoma (CXPA) is an aggressive salivary gland malignancy and rare in minor salivary gland. A soft palate CXPA initially presenting as direct cavernous sinus (CS) invasion is very rare.
A 60-year-old male had a 3-month history of a small soft palatal mass with progressing left cheek numbness, proptosis, and disturbed vision. Biopsy of soft palatal tumor showed pleomorphic adenoma. Magnetic resonance imaging showed a tumor involving left maxilla, and extended from pterygopalatine fossa, inferior orbital fissure to CS. Excision of tumor revealed CXPA. Adjuvant concomitant chemo-radiation therapy (CCRT) was given. The tumor recurred 5 months later in left CS which was re-treated with CCRT. The disease status was stable at 2 years after the diagnosis of CXPA.
We present this case to emphasize that patients with symptoms such as facial numbness, proptosis and disturbed vision should be carefully investigated for lesions invading CS by perineural spread.
Background/aim: To describe the characteristic constellation of historical, clinical, radiographic, and histopathological findings of localised invasive sino-orbital aspergillosis based on the authors’ recent experience of four consecutive cases presenting over a 6 month period. Treatment and outcome are reviewed.
Methods: A case series of four patients with review of the English language literature.
Results: There have been 17 reported cases of invasive sino-orbital aspergillosis in healthy individuals over the past 33 years. The authors report four patients who presented during a 6 month period with persistent and significant pain followed by progressive ophthalmic signs—clinical histories reflecting the literature. Similar imaging findings were also noted: focal hypodense areas within apical infiltrates on contrasted computed tomography correspond to abscesses seen at surgery, and sinus obliteration or involvement of the adjacent sinus lining was noted on magnetic resonance imaging. Bone erosion (often focal) was also seen. There is frequently a delay in making the correct diagnosis, and often disease progression occurs despite treatment.
Conclusions: The authors encountered four cases of invasive sino-orbital aspergillosis, three of which occurred in otherwise healthy individuals. The clinician must be aware of the characteristic presentation so that earlier diagnosis, management, and improved outcomes can be achieved.
orbital aspergillosis; orbital infection
Primary renal aspergillosis is rare in diabetic patients. Diagnosis of localized primary renal Aspergillus infection in diabetic patients requires careful investigations due to its benign presentation and lack of associated systemic clinical features. There is also paucity of information on the role of conservative treatment of such localized infection with antifungal agents only. Here, we describe a case of localized renal aspergillosis in a type 2 diabetic patient with a brief review of literature.
We describe a case of unilateral renal aspergillosis following intracorporeal pneumatic lithotripsy (ICPL) in a type 2 diabetic man. The patient presented with mild pain in the left lumbar region and periodic expulsion of whitish soft masses per urethra, which yielded growth of Aspergillus fumigatus. He was treated initially with amphotericin B; however, it was stopped after 2 weeks, as he could not tolerate the drug. Subsequently, he was successfully treated with oral itraconazole.
Localized renal aspergillosis may be suspected in diabetic patients having history of urinary tract instrumentation, mild lumbar pain, passage of suspicious masses in urine and persistent pyuria. Examination of the suspicious substances expelled per urethra is essential for diagnosis as routine multiple urine analysis may yield negative results. Conservative treatment with oral itraconazole alone is effective in cases with incomplete obstruction.
Sellar aspergillosis is a rare infection commonly mistaken for a pituitary tumour. We present a rare case of pituitary fossa Aspergillus fumigatus mycetoma in an immunocompetent 90-year-old female, who presented with headaches. Magnetic resonance imaging scans demonstrated an enhancing pituitary fossa mass that appeared to infiltrate the sphenoid sinus, suggestive of an invasive tumour. Stereotactic trans-sphenoidal resection confirmed localized A. fumigatus infection. The abscess was debrided and the dura was left intact. Her headaches resolved post-operatively and she was treated with voriconazole. This indicates that aspergilloma should be considered as a differential for an unexplained pituitary lesion even in elderly immunocompetent patients.
Itraconazole is an anti-fungal agent widely used to treat various forms of mycosis. It is particularly useful in allergic bronchopulmonary aspergillosis and severe asthma with fungal sensitization. Side effects are uncommon and usually mild. Mild neuropathy is noted to occur very rarely. We present an unusual and, to the best of our knowledge, as yet unreported case of severe neuropathy and peripheral edema due to itraconazole in the absence of a concomitant risk factor.
A 72-year-old Caucasian man was started on itraconazole following diagnosis of severe asthma with fungal sensitization. One month later he presented with severe bilateral ankle edema with an elevated serum itraconazole level. The itraconazole dose was reduced but his ankle edema persisted and he developed weakness of all four limbs. Itraconazole was completely stopped leading to improvement in his leg edema but he became bed bound due to weakness. He gradually improved with supportive care and neurorehabilitation. On review at six months, our patient was able to mobilize with the aid of two elbow crutches and power had returned to 5/5 in distal extremities and 4+/5 in proximal extremities. The diagnosis was established based on the classical presentation of drug-induced neuropathy and negative investigatory findings for any alternative diagnoses.
We report the case of a patient presenting with an unusual complication of severe neuropathy and peripheral edema due to itraconazole. Clinicians should be alert to this association when encountered with neuropathy and/or edema in an itraconazole therapy recipient.
While biliary cystadenoma and biliary cystadenocarcinoma involving the liver are not uncommon, biliary cystadenocarcinoma of the gall bladder is an extremely rare lesion and can be very difficult to diagnose.
A 50-year-old Indian woman presented with pain and swelling in the right hypochondrium. An ultrasonography revealed a cystic lesion arising from the gallbladder fossa. This lesion was initially managed with aspiration and antibiotics by the treating physician. The patient was referred for surgical management because the abscess was not resolved through conservative treatment. A diagnosis of an infected nonparasitic cyst was made and deroofing of the cyst was performed. A histopathological examination of the excised cyst wall showed cystadenocarcinoma. The patient subsequently underwent a successful surgical excision of the lesion.
Infective lesions of the liver are common in developing countries and are usually managed through aspiration and antibiotics. Cystadenocarcinoma of the gallbladder needs to be considered in the differential diagnosis of cystic lesions arising from the gallbladder fossa. A high index of suspicion and cytological examination from the wall of such complex lesions will help in the timely management of such lesions.
Ischemic stroke by septic embolism occurs primarily in the context of infective endocarditis or in patients with a right-to-left shunt and formation of a secondary cerebral abscess is a rare event. Erosion of pulmonary veins by a pulmonary abscess can lead to transcardiac septic embolism but to our knowledge no case of septic embolic ischemic stroke from a pulmonary abscess with secondary transformation into a brain abscess has been reported to date.
We report the case of a patient with a pulmonary abscess causing a septic embolic cerebral infarction which then transformed into a cerebral abscess. After antibiotic therapy and drainage of the abscess the patient could be rehabilitated and presented an impressive improvement of symptoms.
Septic embolism should be considered as cause of ischemic stroke in patients with pulmonary abscess and can be followed by formation of a secondary cerebral abscess. Early antibiotic treatment and repeated cranial CT-scans for detection of a secondary abscess should be performed.