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x-ray revealed a dense tortuous cylindrical nodule at leukemia and aplastic anemia were ruled out because
the posterior RUL and a pleurodiaphragmatic adhe- of a normal blood morphology and bone marrow
sion on the left. Possibility of an A-V malformation findings.
and TB granuloma was entertained. Pulmonary and
carotid arteriography were contemplated but was not Hereditary Hemorrhagic Telangiectasia is the more
done due to financial constraint. ECG was normal, probable consideration for this case. It is autosomal
ABG showed hypoxemic hypocardia. Two units of dominant disease characterized as a sytemic fibrovas-
FWB were tranfused. Post BT Hgb and Hct were. cular dysplasia in which telangiectasia, A-V malforlow,
patient continued to have on and off epistaxis mation and aneurysm maybe widely distributed through
necessitating transfusion of 4 units of FWB in a week out the body vasculature. This is recognized as a
time. classic triad of telangiectasia, recurrent bleeding and
family history. The lesions maybe pinpoint in size
On the 10th hospital day bone mar'row aspiration or larger and form nodular vascular tumor, sometimes
biopsy was done to rule out blood dyscrasia, result it appear as a spider-like lession. Significant syruprevealed
normal bone marrow findings, tomatology include recurrent bleeding from mucosal
telangiectasia, hypoxemia, cerebral embOlism and brain
On the 13th hospital day the patient bled pro- abscess due to pulmonary A-V malformation.
fusely, she underwent emergency bilateral external
carotid artery ligation in an attempt to control epis- Epistaxis is the most common form of bleeding
taxis. Post operative Hgb and Hct were still below and it is the first hemorrhagic event in I_Iereditary
normal value prompting another series of blood trans- Hemmorhagic Telangiectasia. In our patient several
fusion. Patient continued to have episodes of epis- attempts have been done to control epistaxis i.e., local
taxis which spontaneously stopped. Rigid nasopharyn- pressure, anterior and posterior nasalpacking, ligation
goscopy was done revealing an erythematous maculo- of the blood supply of nasal cavity . Yet inspite of
papular lesion on the left side ol_ the nasal septum these interventions, there were still episodes of bleedlocated
posteriorly, ing. Bilateral external carotid artery ligation completely
failed to control the hemorrhagic event in this
Upon discharge patient continued to experience patient because of the rich vascular channols supplyepistaxis
relieved by local pressure. Two months later ing the nasal cavity.
profuse nose bleeding recurred and she lost consciousness
for which she was rushed to Ospital ng Maynila Hereditary Hemorrhagic Telangiectasia is the most
and was re-admitted. Contrast C.T. scan was done common disease associated wth pulmonary A-V fiswhich
revealed normal findings, tula. Pulmonary fistula maybe solitary of multiple,
majority of which • receive blood supply from the
In summmary, we are presented with a 31 y.o pulmonary artery and are right to left shunts. Right
female patient with the following salient features; to left shunts allows desaturated blood to enter the
recurrent epistaxis, erythematous maculo-papular systematic circulation to cause cyanosis by hypoxemia,
lesion on the nasal septum clubbing and cyanosis of polycythemia and clubbing.
nail beds, a blowing murmur at the right interscapular
area which appear to be extracardiac in origin, normal On chest x-ray the typical pulmonary A-V
hematologic studies except for anemia, possibility of malformation is peripheral, circumscribed, aon calcific
an A-V formation on chest radiography and a family nodule connected to the hilus by vascultr strands.
history which revealed that her grandmother had similar
experience. Withthese, the possible underlying cause Management of this case is variable depending
of recurrent epistaxis should be explored, on the severity of the disease. Oral iron and folic
acid supplements maybe necessary to avoid iron
Infection would not produce such prolonged and deficiency. Transfusion requirements were variable.
massive epistaxis. There was no history nor evidence
of recent nasal truma. Patient was not hypertensive. Treatment modalities to control bleeding such as
Hematologic dysfunctions such as acute leukemia, local pressure, topical vasoconstrictors, claemical or
aplastic anemia, and Heriditary Hemorrhagic Telang- electrical cat, tery and arterial ligaton offer only
iectasia should be considered. However, acute
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