Athanasios N Chalazonitis: rg. This article has been cited by other articles in PMC. Abstract Background Hughes-Stovin syndrome is a rare entity. Case presentation We report the case of an 18 years old, greek male patient with Hughes-Stovin syndrome, who initially presented with deep vein thrombosis.
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Athanasios N Chalazonitis: rg. This article has been cited by other articles in PMC. Abstract Background Hughes-Stovin syndrome is a rare entity. Case presentation We report the case of an 18 years old, greek male patient with Hughes-Stovin syndrome, who initially presented with deep vein thrombosis. Pathogenesis, imaging investigation and treatment of this syndrome are also briefly discussed. Conclusion In young men presenting with venous thrombosis as revealed on imaging examination, with platelet count and coagulation tests within normal and hemoptysis the eventuality of Hughes-Stovin syndrome is to be considered.
Background Hughes-Stovin syndrome is a rare condition first described in [ 1 ] which is characterized by multiple pulmonary artery aneurysms and peripheral venous thrombosis. Typical symptoms are recurrent fever, chills, haemoptysis and coughs and it usually affects young men.
The natural course of the illness is usually fatal because of fulminant haemoptysis. Case presentation An 18 years old, Greek male patient presented with edema of his left leg.
His vital signs including temperature, arterial pressure and heart rate were normal. Clinical examination was negative. Contrast Enchanced Computed Tomography CT of the abdomen and pelvis was also performed in order to exclude a major intra-abdominal malignancy and thrombosis of left external iliac vein was depicted as well. A history of migraine during the last 3 years and anorexia nervosa was also reported. After 10 days on heparin therapy, dry cough begun, followed by an episode of moderate haemoptysis — ml.
At review his vital signs were normal and his platelet count and coagulation tests were within normal limits. There was no leukocytosis, and the erythrocyte sedimentation rate was normal. Skin examination was normal. Chest postero-anterior plain film showed a nodular opacity in the mid right lung field. Lung window settings of the MSCT revealed a right lower lobe ground glass opacity probably representing a pulmonary hemorrhage, while left lower lobe parenchyma appeared normal.
MR brain venography showed chronic thrombosis of superior sagittal and transverse sinuses Fig.
Aneurysmal rupture leading to massive hemoptysis and death These stages usually evolve successively. The first and second phases have to be present for the diagnosis of HSS while the third phase is the usual ultimate outcome for untreated patients. The typical presenting features of HSS are related to the presence of the pulmonary aneurysms and peripheral venous thrombosis. These signs and symptoms are listed in figure 1 [ 8 , 16 — 18 ]. Patients can have seizures, diplopia and cephalalgia secondary to raised intracranial pressure consequent to cerebral venous sinus thrombosis [ 1 , 19 ]. The raised intracranial pressure also accounts for the papilledema of the optic disc observed in some patients with HSS [ 20 ].
It occurs predominantly between the 2nd to 4th decades. There is a recognized male predilection. Pathology Typically affects pulmonary, and frequently bronchial, arteries and large systemic veins. Progression Typically disease is clinically characterized 3 phases 5: stage I: thrombophlebitis stage II: formation and enlargement of pulmonary aneurysms stage III: aneurysmal rupture Exact features will depend on the stage. May show: systemic thrombi in the vena cava, cerebral sinuses, or limb veins pulmonary arterial occlusions due to emboli or thrombi bronchial arterial aneurysms.