11-18-2008, 11:23 AM
45 yr old male with anemia nd facial telangectasias...recent CCS!i think your telangectasia case was a tough tough one...and you handled it pretty well..srikanthuk.
i would have oredered the following labs...
cbc
bmp
pt/ptt
bleeding time
iron studies
serum vit B12
Serum folate level
peripheral smear
serum haptoglobin
LFTs
UA
CXR
12 lead EKG
FOBT
probably would have admitted him to transfuse...
ordered colonoscopy, even with negative FOBT
and i would not have known what to do after that?? i think...
unless colonoscopy showed AVMs....
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well the case does sound like Osler-Weber-Rendu...or hereditary hemorragic telangectasia.
basically if that is the case...there is no real medical mangement here...
symptomatic management....anemia=transfuse!!! which you did...good job!
the main thing for this HHT case would be to assess your diagnostic/investigative skills...
if we are aware that AV malfomations in the Brain, Liver, GIT, Lungs....are the main problem then we would ...know which studies to order to look for them....
Also HHT gives CCF...so must do an Echocardiogram...secondary to Pulmonary Htn...i think!
In any event this a very tough addition to the CCS case pool....makes me very nervous!
Anyone wants to comment which studies to order for searching the AVMs i the various oragns????
thanks again for sharing!
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#1563618
einstein - 11/18/08 15:11
Pindi you did a good job. After doing all those tests I will just add some tests depending on the clinical scenario:
Arterial blood gas--If a right-to-left shunt is present, the pO2 is low.Performing a hyperoxic test with the arterial blood gas confirms the diagnosis of a right-to-left shunt. Screening with a hyperoxic test is shown to have 100% sensitivity and 40% specificity for the detection of pulmonary AVMs in patients with Osler-Weber-Rendu syndrome who are suspected of having an AVM.
CT scanning --may be used to better delineate AVMs of the lung or head. It may also show larger brain abscesses.
MRI-- scanning is the best noninvasive test to help delineate the extent of pulmonary and CNS AVMs. It also detects telangiectases in the CNS.
Angiography ---is used to map the exact extent of the vascular lesions, usually when surgery is contemplated.
Colonoscopy-- shows GI telangiectases as small well-defined lesions surrounded by an anemic halo.
Barium enema--- is useful only if an ulcer or neoplasm is suspected. GI telangiectases cannot be visualized by this examination.
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#1563621
einstein - 11/18/08 15:14
Diagnostic criteria are based on 4 components.
The diagnosis is considered definite if 3 criteria are present and possible if 2 criteria are present. The diagnosis is unlikely if fewer than 2 criteria are present. The criteria are as follows:
Nosebleeds --- Spontaneous and recurrent
Telangiectasias - --Multiple, at characteristic sites, including the lips, oral cavity, fingers, and nose
Presence of internal lesions --- GI telangiectasia, pulmonary AVMs, hepatic AVMs, cerebral AVMs, spinal AVMs
Family history ---- A first-degree relative with HHT according to these criteria
i would have oredered the following labs...
cbc
bmp
pt/ptt
bleeding time
iron studies
serum vit B12
Serum folate level
peripheral smear
serum haptoglobin
LFTs
UA
CXR
12 lead EKG
FOBT
probably would have admitted him to transfuse...
ordered colonoscopy, even with negative FOBT
and i would not have known what to do after that?? i think...
unless colonoscopy showed AVMs....
~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~
well the case does sound like Osler-Weber-Rendu...or hereditary hemorragic telangectasia.
basically if that is the case...there is no real medical mangement here...
symptomatic management....anemia=transfuse!!! which you did...good job!
the main thing for this HHT case would be to assess your diagnostic/investigative skills...
if we are aware that AV malfomations in the Brain, Liver, GIT, Lungs....are the main problem then we would ...know which studies to order to look for them....
Also HHT gives CCF...so must do an Echocardiogram...secondary to Pulmonary Htn...i think!
In any event this a very tough addition to the CCS case pool....makes me very nervous!
Anyone wants to comment which studies to order for searching the AVMs i the various oragns????
thanks again for sharing!
~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~
#1563618
einstein - 11/18/08 15:11
Pindi you did a good job. After doing all those tests I will just add some tests depending on the clinical scenario:
Arterial blood gas--If a right-to-left shunt is present, the pO2 is low.Performing a hyperoxic test with the arterial blood gas confirms the diagnosis of a right-to-left shunt. Screening with a hyperoxic test is shown to have 100% sensitivity and 40% specificity for the detection of pulmonary AVMs in patients with Osler-Weber-Rendu syndrome who are suspected of having an AVM.
CT scanning --may be used to better delineate AVMs of the lung or head. It may also show larger brain abscesses.
MRI-- scanning is the best noninvasive test to help delineate the extent of pulmonary and CNS AVMs. It also detects telangiectases in the CNS.
Angiography ---is used to map the exact extent of the vascular lesions, usually when surgery is contemplated.
Colonoscopy-- shows GI telangiectases as small well-defined lesions surrounded by an anemic halo.
Barium enema--- is useful only if an ulcer or neoplasm is suspected. GI telangiectases cannot be visualized by this examination.
~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~
#1563621
einstein - 11/18/08 15:14
Diagnostic criteria are based on 4 components.
The diagnosis is considered definite if 3 criteria are present and possible if 2 criteria are present. The diagnosis is unlikely if fewer than 2 criteria are present. The criteria are as follows:
Nosebleeds --- Spontaneous and recurrent
Telangiectasias - --Multiple, at characteristic sites, including the lips, oral cavity, fingers, and nose
Presence of internal lesions --- GI telangiectasia, pulmonary AVMs, hepatic AVMs, cerebral AVMs, spinal AVMs
Family history ---- A first-degree relative with HHT according to these criteria
