Hemophilia is an inherited (sex-linked recessive) anomaly of blood coagulation that appears clinically only in males. Patients with this disease have a decreased or absent serum concentration of antihemophilic globulin and suffer a lifelong tendency for spontaneous hemorrhage.
The major radiographic changes in hemophilia are complications of recurrent bleeding into the joints.
Submucosal bleeding into the wall of the gastrointestinal tract may develop in hemophilia. This most commonly involves the small bowel and produces a short or long segment with regular thickening of folds (picket-fence or stack-of-coins appearance). In the colon, bleeding may produce the thumbprinting pattern of sharply defined fingerlike marginal indentations along the contours of the colon wall.
Abnormalities of the urinary tract in hemophilia include intrarenal and perinephric hematomas, calyceal defects resembling papillary necrosis, and global renal enlargement. Obstructive uropathy may be caused by ureteral blood clots or be due to incomplete resorption and subsequent fibrosis of a retroperitoneal hematoma.
Thrombocytopenia is the most common abnormality of hemostasis resulting in bleeding in the surgical patient. A wide variety of diseases can lead to reduction in the platelet count and purpuric bleeding. Thrombocytopenia may be primary and idiopathic or secondary to such conditions as replacement of the bone marrow by leukemia, metastatic tumor, or fibrous tissue; drug reaction; systemic lupus erythematosus; or congestive splenomegaly due to portal hypertension.
The major radiographic changes caused by thrombocytopenia primarily involve the gastrointestinal tract.Hemorrhage info the small bowel produces characteristic uniform, regular thickening of small bowel folds in the affected intestinal segment. Splenomegaly is present in patients with thrombocytopenia due to bone marrow failure, portal hypertension, and idiopathic thrombocytopenic purpura. In the last condition, splenectomy is often required to remove this important site of platelet destruction and major source of synthesis of platelet antibodies.
Spontaneous bleeding may be a complication of anticoagulant therapy with either heparin or the coumarin and indandione derivatives. Hemorrhage into the gastrointestinal tract produces a symmetric spikelike configuration simulating a stack of coins or picket fence; in the colon, a pattern of thumbprinting may be produced. Concomitant bleeding into the mesentery often results in an intramural or extrinsic mass, flattening of folds on the mesenteric side of the bowel, and separation and uncoiling of bowel loops. Bleeding secondary to anticoagulant therapy may cause a rectus sheath hematoma (simulating appendicitis) or a retroperitoneal hematoma, both of which can be detected by CT or ultrasound.
Polycythemia vera is a hematologic disorder characterized by hyperplasia of the bone marrow that results in increased production of erythrocytes, granulocytes, and platelets. Increased blood viscosity, increased platelet count, and an increased tendency toward stasis may lead to spontaneous thrombosis in the cerebrovascular and peripheral vascular systems. Paradoxically, patients with polycythemia vera also have a significant tendency to spontaneous hemorrhage. Because of these potential complications in the patient with polycythemia vera, surgery should be avoided if possible and at least be deferred until medical management has returned the blood volume, hematocrit, and hemostatic process to normal.
The spleen is often massively enlarged in polycythemia vera and may present as a left upper quadrant mass. Increased blood volume can lead to prominence of the pulmonary vascular shadows, usually without the cardiomegaly associated with the increased pulmonary vascularity in patients with congenital heart disease. Intravascular thrombosis may cause pulmonary infarctions that appear as focal consolidations or bands of fibrosis. Up to 20 percent of cases of polycythemia vera terminate as myeloid metaplasia with anemia and marked splenomegaly.
rectus atelectasis in colon · body habitus related hypoventilation with minor atelectasis in the left base · difference in hemophilia and thrombocytopenia bleeding ·