Neuro-ophthalmology
Mostafa Khalil, Omar Kouli in The Duke Elder Exam of Ophthalmology, 2019
AD multisystem disorder characterized by the following. Facial angiofibroma.Ash-leaf spots: Hypopigmented macules on the skin.Seizures.Cognitive impairment.Multiple intracranial and/or retinal astrocytic hamartomas Glial tumours of retinal fibre layer that arise from astrocytes.They are commonly referred to as mulberry lesions due to their multinodular appearance. On fundoscopy they appear as well-defined elevated creamy white lesions.Associations - Tuberous sclerosis (most common association)- Neurofibromatosis- Retinitis pigmentosa (less common; lesions are non-calcified)
Sinonasal tumours
Neeraj Sethi, R. James A. England, Neil de Zoysa in Head, Neck and Thyroid Surgery, 2020
The evaluation of patients with juvenile angiofibroma relies on diagnostic imaging. CT hallmarks include widening of the sphenopalatine foramen and pterygopalatine fossa, and anterior bowing of the posterior wall of maxilla. MRI typically reveals signal flow voids. The diagnosis is made radiologically; preoperative biopsy is not recommended due to the bleeding risk. Angiography providing information on the vascular supply of the tumour to assist surgical planning and providing a means by which to embolise the main feeding branches. The Pittsburgh staging system assesses two important tumour attributes: route of intracranial extension and extent of vascular supply from the internal carotid artery [25].
The ear, nose and sinuses
Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie in Bailey & Love's Short Practice of Surgery, 2018
Juvenile angiofibroma is an uncommon condition that affects adolescent boys and may lead to massive life-threatening episodes of bleeding. Diagnosis is made with contrast CT or MRI. Anterior bowing or indentation of the posterior antral wall (Holman–Miller or antral sign) is the classical finding, but may be seen in other expansive lesions in this area. It is a very vascular tumour, which should not be biopsied because of the risk of uncontrollable haemorrhage. Excision is best carried out by an experienced surgeon and is usually performed endoscopically often using image guidance (Figure46.43). Preoperative embolisation of the feeding blood vessels may help to reduce blood loss during surgery.
Nasopharyngeal rhinosporidiosis with intracranial extension masquerading as juvenile angiofibroma: an unusual entity
Published in British Journal of Neurosurgery, 2022
Gautam Dutta, Ghanshyam D. Singhal, Daljit Singh, Hukum Singh, Arvind Kumar Srivastava, Anita Jagetia
For our case, the juvenile suffered from recurrent epistaxis, which is the typical clinical picture of JNA. On physical examination, the mass was located within the nasopharyngeal area which was friable having tendency to bleed. Therefore, the tentative clinical diagnosis was JNA considering the much greater frequency of angiofibroma compared to any other pathology for the age group. The angiographic finding suggested this was a hyper-vascular mass, and we obliterated the feeding vessels with embolization. With this typical clinical pictures and angiographic findings, we continued to favor the clinical diagnosis of JNA. Because surgical removal is the treatment of choice for juvenile angiofibroma, we performed surgical excision of the mass after embolization, without suspecting another diagnosis until the biopsy reports were obtained.
Bilateral juvenile nasopharyngeal angiofibroma: A rare case report
Published in Acta Oto-Laryngologica Case Reports, 2021
Marlinda Adham, Kartika Hajarani, Lisnawati Rachmadi, Indrati Suroyo
Occurring almost exclusively in adolescent males, juvenile nasopharyngeal angiofibroma is a benign fibrovascular tumour. The expression of sex hormone receptors helps explain its sex and age predilection. At present, preoperative embolization is recommended to minimize surgical blood loss. Surgery remains the standard of care. Both endoscopic and open approaches offer effective treatment of JNA with similar rates of recurrence. This case showed complete separate bilateral tumours with separate blood supply. Suspecting bilateral juvenile angiofibroma in patient with two non-contiguous mass in head and neck region is a must. Proper diagnosis of bilateral JNA is helpful in guiding management and lead to good results.
Cellular angiofibroma of the orbit
Published in Orbit, 2021
G.J. Hötte, R.M. Verdijk, M. Gardeniers, D. Paridaens
In conclusion, cellular angiofibroma is a benign mesenchymal tumor that deserves a place in the differential diagnosis of solid tumors in the orbit. Clinical and histopathological characteristics resemble those of cellular angiofibroma found elsewhere in the body.
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