Selected Arterial and Venous Diseases
Alan G. Rose
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This 2.5-year-old female presented with convulsions and was investigated for systemic hypertension
(160/100 mm Hg). Physical examination showed retarded milestones, normal height and weight plus a
cardiac systolic murmur and bruit over both carotid arteries. Femoral pulses were equal and there
was no radiofemoral delay. Chest radiography showed clear lung fields with left ventricular
enlargement. Renal function was normal with no elevation of urinary vanillyl mandelic acid. Normal
intravenous pyelogram. An aortogram showed bilateral renal arterial stenosis. The patient collapsed
and died less than 10 minutes after the aortogram was completed.
Case 1 - Figure 4 - Histology of aorta seen in Figure 3
Case 1 - Figure 5 - Disordered pattern of lamellar units in the outer media as well as an overall excessive number of lamellar units
Case 1 - Figure 6 - Disordered pattern of lamellar units in the outer media as well as an overall excessive number of lamellar units
This 28-month-old female child was treated with Busulphan and Cytoxan prior to undergoing an unrelated
allogeneic bone marrow transplant (BMT) for Hurler syndrome (dwarfism, mental retardation, skeletal
malformations, hepatosplenomegaly and corneal opacities). A week post BMT she developed severe
respiratory problems and lung biopsy showed idiopathic interstitial pneumonia and fibrosis.
Refractory graft-versus-host disease of the skin and hypertension were additional problems. She also
had gram negative septicemia and peritonitis. Terminally the patient was hypotensive with multiorgan
Case 2 - Figure 1 - Severe intimal thickening due to the accumulation of Hurler cells and fibrosis has led to sub-total luminal occlusion of this major coronary artery
Case 2 - Figure 2 - Relatively normal LAD and diagonal branches of LCA in a 17 year old girl in whom bone marrow transplantation prevented significant coronary arterial disease
Case 2 - Figure 3 - Higher power view of the diagonal branch
The patient was a 3-year-old male, 5 months post unrelated cord blood transplant for Wiskott-Aldrich
syndrome. He was also status post-splenectomy and IV IG therapy. He was re-admitted for a
neutropenic fever and given antibiotics, but he developed hematemesis due to nasogastric tube
irritation plus thrombocytopenia. Severe systemic hypertension required the administration of 3-4
antihypertensive medications. Later he became short of breath and a chest x-ray and CT scan revealed
extensive lung lesions consistent with an opportunistic infection. Grade 3 graft versus host disease
(GVHD) was diagnosed on skin biopsy. Despite antibiotic therapy and intubation, his clinical course
deteriorated and he died.
The patient was a 48-year-old male with α-1-antitrypsin deficiency (AATD) who underwent a right
single lung transplant for emphysema in 1991. Cholecystectomy was performed in 1997, but RUQ pain
continued postoperatively. Radiology showed a 4 cm diameter right intercostal artery aneurysm and a
2.5 cm diameter left intercostal aneurysm. Embolization of the right-sided aneurysm was performed in
1997. Family history: his brother had undergone a lung transplant for AATD and his son had AATD. In
November 1999, he developed a pleural effusion and a drop in hemoglobin due to bleeding from an
intercostal aneurysm. The main aneurysm was found on the 9th intercostal artery. Microcoils were
placed in the T6-10 intercostal arteries with good hemostasis for 2 days, followed by hemoptysis due
to rupture into the left lung by one of multiple intercostal arterial aneurysms. A drop in blood
pressure accompanied the enlarging pleural effusion, which was consistent with hemorrhage. A
cardiovascular radiologist embolized the T8-10 intercostal arteries and achieved good hemostasis.
Patient underwent emergency thoracotomy for evacuation of the hematoma, left pneumonectomy and
resection of T7-10 intercostal aneurysms.
A 25-year-old man presented with symptoms of intermittent claudication of his left lower leg.
Arteriography revealed a smooth-walled narrowing of the popliteal artery. The affected segment of the
artery was resected and replaced by a graft.
Case 5 - Figure 1 - Cystic adventitial disease has produced the space seen in the upper portion of the picture. Portion of the original arterial lumen (with some mural thrombus) is seen below this
The patient was a 67-year-old man who presented with acute abdominal pain, fever and leucocytosis.
Clinical signs of an acute abdomen were present for several hours. Past history elicited a one-month
history of slight abdominal pain and weight loss. An emergency laparotomy was performed and 2
segments of ileum measuring 70 cm and 30 cm in length respectively were resected. While the resection
lines were viable, the central portions of both bowel segments showed hemorrhagic ischemic necrosis.
Subsequent postoperative follow-up for 9 years revealed no evidence of systemic disease.
Case 6 - Figure 1 - Lymphocytic venulitis within bowel wall including thrombosis and evidence of recanalization of organized venular thrombi. Note the normal adjacent small arteries
Case 6 - Figure 2 - Lymphocytic venulitis within bowel wall including thrombosis and evidence of recanalization of organized venular thrombi. Note the normal adjacent small arteries
The patient was a 3 ½ month old female with Noonan syndrome who was extremely cyanotic from birth and
underwent neonatal repair of the following cardiovascular defects: pulmonary valvar stenosis,
aortico-pulmonary window defect, atrial septal defect, aortic valvar stenosis, patent ductus
arteriosus and coarctation of the aorta. The patient also had a perimembranous ventricular septal
defect. The thoracic duct was ligated due to a chylothorax. Her postoperative recovery was
complicated by delayed chest closure, total body edema and she required significant vasopressor
support. She was placed on a respirator and developed renal failure of uncertain etiology for which
peritoneal dialysis was administered. Prior to death she had increasing need for larger amounts of
vasopressors and mechanical ventilation.
The patient was a 9-year-old girl who lived on a farm. She presented to a clinic with a history of
several weeks upper abdominal pain, nausea, vomiting and abdominal swelling. Mild jaundice was
evident. Autopsy revealed a marked "nutmeg" appearance of passive congestion of the liver (Figure 1)
plus ascites. The histology (Figures 5B-5C) shows veno-occlusive disease affecting the centrilobular
and sublobular veins of the liver with intense centrilobular and mid-zonal congestion with loss of
hepatocytes. A reticulin stain is most useful in demonstrating the venous occlusion and / or
The patient was a 27-year-old male with dilated cardiomyopathy who underwent cardiac transplantation.
The section of coronary artery comes from the explanted recipient heart, which showed a
hypersensitivity myocarditis (HSM). No significant coronary atherosclerosis was noted. There was
evidence of myocyte hypertrophy consistent with a dilated cardiomyopathy.
The patient, a 38-year-old male with AIDS, presented with pulsatile masses in both groins. Clinical
examination and subsequent investigations, including a spiral CT scan (Figure 1), revealed the patient
to have multiple aneurysms affecting both femoral arteries, and the right and left popliteal arteries.