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CT : 19Y Male with epistaxis and peripheral cynosis |
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SCROLL FOR ANSWER Diagnosis: Pulmonary AVM. Discussion: Osler – Weber – Rendu syndrome - Prevalence 1-2/100000, higher in the Danish island of Fyn, the Dutch Antilles, and parts of France.
- Autosomal dominant
There are four diagnostic criteria.If three or four are met, a patient has definite HHT, while two gives a possible diagnosis: - Spontaneous recurrent epistaxis
- Multiple teleangiectasias on typical locations
- Proven visceral AVM (lung, liver, brain, spine)
- First-degree family member with HHT
- MRI is the best one stop shop investigation modality.
- If a pulmonary AVM is present, chest radiography may show a peripheral noncalcified coin lesion attached by vascular strands to the hilus
- CT scanning may be used to better delineate AVMs of the lung or head. It may also show larger brain abscesses
- Angiography is used to map the exact extent of the vascular lesions, usually when surgery is contemplated
Treatment is supportive and consists of controlling and treating the bleeding. The prognosis of the disease is good as long as bleeding is promptly recognized and adequately controlled. Oral contraceptives Norethindrone acetate and ethinyl estradiol Antifibrinolytics Aminocaproic acid Septal dermoplasty Pulsed dye laser treatment Segmental bowel resection. Embolization, ligation, or surgical excision for enlarging or symptomatic pulmonary AV fistulas or for intrahepatic fistulas causing high-output cardiac failure. Dr. Paresh Desai - Goa |
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USG-Doppler: Young patient with syncopal attacks |
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Diagnosis: Carotid Body tumor. Findings: A vascular mass is seen at the “crotch” of the carotid artery bifurcation which is splaying the ICA and ECA. Discussion: Carotid body tumors are paragangliomas or chemodectomas. In common with the family of other paragangliomas like glomus jugulare, glomus tympanicum, glomus vagale and pheochromocytomas, it is an extremely vascular lesion. It enhances avidly with contrast on CT and MR scans. If a carotid body tumors are detected, careful search of other paragangliomas should be done, especially adrenal paragangliomas. The two are commonly associated. The findings of vascular mass at the carotid bifurcation are characteristic of carotid body tumor. Rarely it can be hypovascular/ avascular. Dr Paresh Desai, MD, Goa |
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