Vein of Galen aneurysm: anatomical study of an adult autopsy case

We present a case of vein of Galen aneurysmal malformation in an adult, the rupture of which caused a fatal hemorrhage. Macroscopic examination, together with plastination of specimen with S10, and microscopic examination showed connection of the aneurysm with the right thalamoperforating artery and, bilaterally, with the posterior choroidal and mesencephalic arteries. Histology also showed thinning of the aneurysmal wall, due to marked reduction of the muscular media at the point of rupture, and its connection with an arteriovenous malformation of the left thalamus. A review of the anatomy of the Galenic system shows that the medullary veins curve at an acute angle to form the paired internal cerebral veins, with a sudden change in flow and that, from late fetal life, the vein of Galen curves around the splenium to enter the straight sinus at an angle opposing its flow. Based on anatomical study and a review of the literature, we suggest that aneurysmatic dilatation of the vein of Galen may result from a combination of pathological and predisposing anatomical factors. The anatomical characteristics of the origin of the internal cerebral veins and of the entry of the vein of Galen into the straight sinus make particularly susceptible to the hemodynamic effects of arteriovenous shunts the vein of Galen itself, which is situated in the cisterna ambiens, an anatomical space that does not impede its enlargement.     

Multiple vascular malformation in the 'blue rubber bleb naevus' syndrome: a case with aneurysm of vein of Galen and vascular lesions suggesting a link to the Weber–Osler–Rendu syndrome

A case of Bean's syndrome is reported in which a wide variety of vascular malformations are illustrated. Among these were multiple arterio-venous malformations including a so-called vein of Galen aneurysm. A comparison of this case with those in the literature suggests that the pathology in patients with Bean's syndrome may be more varied than heretofore recognized, and that an overlap between this syndrome and the Osler–Weber–Rendu syndrome may exist.     

Aneurysm of the right renal vein arising from an idiopathic arteriovenous fistula

A renal vein aneurysm is rarely secondary to an arteriovenous fistula. A symptomatic 4.7-cm aneurysm of the right renal vein arising from an idiopathic arteriovenous fistula in a 41-year-old woman is described. Imaging techniques have a critical role in planning the treatment. The 4.7-cm aneurysm showed a venous wall with uneven thickening of the intima, irregular atrophy and architectural disarray of the media, and partial loss of elastic fibers. Potential for rupture supports surgical intervention in a selected patient with a macroaneurysm.     

Life without the vein of Galen: Clinical and radiographic sequelae

A thorough understanding of the anatomy of the pineal region, particularly venous drainage, is critical for gaining open surgical access to the pineal gland. The adverse sequelae after intraoperative venous occlusion are assumed to be catastrophic but have been scarcely reported. We report a case of pineocytoma in which the vein of Galen was ligated without postoperative adverse sequelae. Pineal region anatomy with emphasis on deep veins was reviewed in large anatomical studies. There are tremendous anatomical variations in the vein of Galen and its tributaries. Several confounding factors can be encountered during surgery and may lead to accidental sacrifice of the vein of Galen. Survival after focal occlusion of a major deep vein depends on the development of collateral circulation as shown in our case report. Venous drainage remains the cornerstone in the surgical planning of the pineal region. Anatomical variations and venous collaterals undoubtedly contributed to the mixed reports of adverse sequelae after venous sacrifice. Vein of Galen ligation may be survivable but consequences cannot be predicted without a thorough pre-ligation assessment of regional venous collateral drainage. Thorough understanding of the venous anatomy, meticulous planning of the surgical approach and avoidance of the occlusion of the vein of Galen and its major tributaries are key factors to successful pineal region surgery.     

Deep femoral artery aneurysm presenting as lower limb swelling: a case report

We report a rare case of lower limb swelling due to compression of the superficial femoral vein by a solitary deep femoral artery aneurysm. The patient was a 58-year-old man presenting with acute swelling of the right lower limb caused by deep venous thrombosis. A multi-detector computed tomographic scan (CT) confirmed the diagnosis of a deep femoral artery aneurysm and revealed no evidence of aneurysms or occlusive lesions in the other arteries. Aneurysmectomy and ligation of the deep femoral artery were successfully performed. Preoperative multi-detector CT scanning is a valuable, non-invasive diagnostic tool to delineate not only the state of the aneurysm, but also that of the distal arterial tree.     

Mesenteric-portal confluence aneurysm

The authors take interest in the portal aneurysm about the observation of a 52 year-old woman with an echographic lesion in the head of the pancreas. Radiological examination was done with abdominal CT and MRI. Because of no accurate diagnosis, an explorative laparotomy was done and showed an aneurysm at the junction of the portal and superior mesenteric veins. These lesions are rare: they can be acquired particularly with underlying hepatocellular diseases and portal hypertension; they can be congenital due to an incomplete obliteration of the right vitelline vein. If asymptomatic, only a close surveillance must be proposed.     

Symptomatic solitary right renal vein aneurysm: a case report.

Renal vein aneurysms are very uncommon. Those located in the right side are exceptional. A symptomatic, solitary, voluminous aneurysm of the right renal vein in a 33-year-old man is reported. The lesion was diagnosed preoperatively as a renal tumor. The pathologic study of the nephrectomy specimen disclosed a thrombosed saccular aneurysm measuring 5.5 cm with marked medial atrophy of its wall. Recognition that a mass like this reported is of vascular origin is very important to avoid percutaneous biopsy which could be potentially hazardous; and for undertaking conservative prophylactic surgery. Renal venography is indicated for the diagnosis of these lesions.     

RASA1 analysis: clinical and molecular findings in a series of consecutive cases

RASA1 mutations have been reported to be associated with hereditary capillary malformations (CM) with or without arteriovenous malformations (AVM), arteriovenous fistulas (AVF), or Parkes Weber syndrome. But the number of cases with RASA1 mutations reported to date is relatively small and the spectrum of phenotypes caused by mutations in this gene is not well defined. Mutation results and clinical findings in thirty-five unrelated consecutive cases sent for RASA1 molecular sequencing testing at ARUP Laboratories within the last two years were evaluated. Eight individuals had a pathogenic RASA1 mutation of which six were novel. These eight individuals all had CMs (seven had multifocal CMs; one had multiple CMs), and six also had a brain or facial AVM. Two individuals with multifocal CMs including one with a fast flow lesion had a variant of uncertain significance. All other individuals, including sixteen with CMs and one with a vein of Galen aneurysm, tested negative for a RASA1 mutation. Our data suggest that multifocal CM is the key clinical finding to suggest a RASA1 mutation. The clinical diagnostic mutation detection rate among all samples sent for RASA1 testing was 29% (10/35) which increases to approximately 39% (10/26) if patients without CMs are excluded.     

Migration of a fish bone into the right renal vein

We describe a patient in whom a fish bone penetrated the duodenum and migrated into the right renal vein. The bone was successfully removed with surgery. The 75-year-old man was admitted to Nippon Medical School Tama Nagayama Hospital because of right upper abdominal pain persisting for 7 days. The patient's medical history was not relevant to the current disorder. Plain radiography showed no abnormalities. Computed tomography revealed a linear object of high intensity that had penetrated the duodenum and migrated into the right renal vein with thrombus. The object was surrounded by a low-density area, suggesting severe inflammation. The patient had eaten fish 1 day before the onset of abdominal pain. We diagnosed duodenal penetration caused by an ingested fish bone. Endoscopic examination showed erosion, but no fish bone or ulceration was detected in the duodenum. The patient was treated conservatively with fasting, peripheral parental nutrition, and intravenous antibiotics. Three days after admission, non-contrast-enhanced computed tomography showed no movement of the foreign body. The patient continued to have pain, and the decision was made to surgically explore the abdomen. Intraoperative ultrasonography showed that the foreign body had migrated completely into the right renal vein with thrombus. Severe inflammation of the right renal vein was observed. Because we could not remove the foreign body without seriously injuring the right renal vein, right nephrectomy was performed. Macroscopic examination of the surgical specimen confirmed the presence of a fish bone with thrombus in the right renal vein. The patient was discharged 9 days after operation, with no complications.     

Aneurysm of the great cardiac vein

Anatomical variations in the cardiac veins have the potential to cause iatrogenic injuries during cardiac surgical procedures or cardiac resynchronization therapy. We present a case of an 86-year-old man, which presented with a great cardiac vein aneurysm. The great cardiac vein arose near the apex of the interventricular sulcus to the right of the anterior interventricular branch (AIB) of the left coronary artery and crossed the AIB anteriorly to the left. The great cardiac vein aneurysm appeared to be due to a possible distal constriction of the great cardiac vein by a small muscular branch of the circumflex branch and a possible proximal constriction by the left marginal artery. Cardiologists who interpret imaging of the cardiac veins and cardiac surgeons who operate close to the great cardiac vein should be aware of such a variation.     

Vein of Galen aneurysm malformation (VGAM): closing the management loop

We report a case where an antenatal scan showed a cerebral vessel dilatation consistent with a vein of Galen malformation (VGAM). After delivery the diagnosis was confirmed on MRI scan. The infant's condition was stable in relation to the two important clinical indicators, mainly not in congestive cardiac failure and no hydrocephalus. The vein of Galen assessment score as used in Hopital Bicetre was 20/21. The scoring system is based on a multiorgan assessment including the five parameters cardiac, cerebral, respiratory, hepatic and renal (Table 1). A score of < 8 indicates no treatment, 8-12 means immediate intervention while a score > 12 delays intervention to circa six months of age. At four months the head circumference accelerated with hydroceaphalus on scans and the infant became clinically symptomatic. Urgent embolisation was performed. The procedure uses N-butylcyanoacrylate (NBCA) glue as the embolisation agent, which in this case was inserted through the left posteriomedial choroidal artery. Complete obliteration was achieved. The infant subsequently required insertion of a ventriculoperitoneal shunt for the hydrocephalus component. The infant has had no recurrence. He remains well on objective examination he has mild developmental delay. In summary this case highlights the value of antenatal diagnosis,the VGAM assessment score to determine the optimal time of intervention and the effectiveness of embolisation.     

An autopsy case of Kawasaki disease with reference to occurrence of acute coronary thrombosis in the convalescent stage.

A one-year, four month-old boy who had suffered from Kawasaki disease died suddenly during convalescence despite intensive gamma-globulin treatment. Autopsy revealed a) sausage-like aneurysms of the left and right coronary arteries and fresh thrombosis in the right coronary aneurysm, b) fresh transmural myocardial necrosis in the whole wall of the left ventricle and the anterior part of the wall of the right ventricle, and c) swelling of the cervical lymph nodes and thymus (60 g). Histologically, fibrocellular thickening of the intima and destruction of the media and internal elastic lamina were conspicuous in the area of the aneurysm, but those of the intima and media in the areas adjacent to the aneurysm were mild. Abrupt narrowing of the lumen at the border between the aneurysm and periphery of the right coronary artery was detected, and this may have been responsible for formation of the thrombus in the right coronary aneurysm. In the systemic arteries, perivascular fibrosis was very noticeable despite less severe injury to the intima and media. These findings suggest that severe inflammation of the periarterial regions was present in the acute phase. The lymph system still showed inflammation, supporting the infectious or toxic nature of Kawasaki disease.     

An Autopsy Case of Kawasaki Disease with Reference to Occurrence of Acute Coronary Thrombosis in the Convalescent Stage

A one-year, four month-old boy who had suffered from Kawasaki disease died suddenly during convalescence despite intensive gamma-globulin treatment. Autopsy revealed a) sausage-like aneurysms of the left and right coronary arteries and fresh thrombosis in the right coronary aneurysm, b) fresh transmural myocardial necrosis in the whole wall of the left ventricle and the anterior part of the wall of the right ventricle, and c) swelling of the cervical lymph nodes and thymus (60 g). Histologically, fibrocellular thickening of the intima and destruction of the media and internal elastic lamina were conspicuous in the area of the aneurysm, but those of the intima and media in the areas adjacent to the aneurysm were mild. Abrupt narrowing of the lumen at the border between the aneurysm and periphery of the right coronary artery was detected, and this may have been responsible for formation of the thrombus in the right coronary aneurysm. In the systemic arteries, perivascular fibrosis was very noticeable despite less severe injury to the intima and media. These findings suggest that severe inflammation of the periarterial regions was present in the acute phase. The lymph system still showed inflammation, supporting the infectious or toxic nature of Kawasaki disease. Acta Pathol Jpn 42: 604–613, 1992.     

Successful management of duodenal varices by balloon-occluded retrograde transvenous obliteration.

We describe a case of duodenal varices successfully treated with balloon-occluded retrograde transvenous obliteration (BRTO) alone. A 63-year-old man presented with tarry stools. Computed tomography demonstrated dilated varices around the duodenum. Emergency endoscopic examination revealed varices of the second portion of the duodenum without active bleeding. There was no evidence of bleeding in the esophagus or stomach. Colonoscopy revealed no bleeding sites in the colon. On the day after admission, a superior mesenteric arteriogram obtained in the venous phase demonstrated a collateral vein from the inferior pancreaticoduodenal vein to the inferior vena cava. Retrograde venography performed via the right femoral vein confirmed that the right inferior adrenal vein was the draining vein and that the collateral vein was occluded. There were no tarry stools after BRTO. The patient was discharged 7 days after BRTO. Two months after discharge, computed tomography showed no dilated varices around the duodenum. Three months after discharge, endoscopy confirmed the absence of varices. No bleeding has been detected as of 10 months after discharge. We conclude that BRTO is an effective treatment for duodenal varices.     

Neutrophils as mediators of human skeletal muscle ischemia-reperfusion syndrome.

Nine patients with aortic aneurysm undergoing arterial reconstruction with temporary aortic occlusion were studied. Since a typical condition of ischemia-reperfusion of the muscles of the lower limbs was created during this surgery, muscle biopsies from the right femoral quadriceps as well as blood samples from the homolateral saphenous vein were taken: (1) before clamping of the aorta, (2) just before declamping, and (3) 30 minutes after reperfusion. Light microscopy revealed a consistent granulocyte infiltration in the ischemic and reperfused skeletal muscle. Ultrastructural damage to the muscle fibers was seen during ischemia and became more severe upon reperfusion. The recruitment of granulocytes into the muscle tissue paralleled the activation of the blood complement system and an increase in circulating neutrophils. Although a spontaneous superoxide anion (O2-) generation from such granulocytes cannot be proved, upon stimulation with formyl-methionyl-leucyl-phenylalanine neutrophils showed a reduced ability in O2 free radical production at the end of ischemia and enhanced O2- generation at reperfusion as compared with the controls. All these findings indicate an active role of granulocytes in the genesis of reperfusion-induced tissue injuries.     

Intrathoracic aneurysm of the right subclavian artery presenting with hoarseness: a case report

Intrathoracic segment of the subclavian artery is an unusual location for peripheral arterial aneurysms. They are normally caused by atherosclerosis, medial degeneration, trauma, and infection. We report a case of a patient with right subclavian artery aneurysm presenting with hoarseness. Chest radiograph demonstrated a superior mediastinal mass. Laryngoscopy showed a fixed right vocal cord. By chest computed tomography, magnetic resonance imaging, and angiography, preoperative diagnosis was established as a saccular aneurysm with afferent loop and efferent loop. Patient underwent complete resection of the aneurysm followed by endto-end anastomosis via median sternotomy. Postoperative pathology was consistent with an atherosclerotic aneurysm filled with thrombus. After surgical operation, hoarseness is still continued.     

Cardiac tamponade due to a rupture of the coronary arteriovenous aneurysm--a case report.

We experienced an unusual case of cardiac tamponde caused by a rupture of the coronary arteriovenous aneurysm in a 54-year-old woman. The patient was suffered from sudden chest pain and syncope, and was initially managed by pericardiocentesis following an echocardiogram which revealed a massive pericardial effusion with signs of cardiac tamponade. She was referred to our hospital under the impression of aortic dissection with cardiac tamponade. She underwent an emergency operation and was found to have a 2 x 2 cm sized bleeding cystic mass protruding from the proximal anterior descending coronary artery. The aneurysm was excised and the openings connected with the coronary artery and right ventricular outflow tract were closed with sutures from the inside of aneurysm. Subsequent coronary arteriography supported the diagnosis.     

An autopsy case of primary aortoduodenal fistula complicated by both gastric carcinoma and acute gastric mucosal lesions

We present an autopsy case of aortoduodenal fistula complicated by both gastric carcinoma and acute gastric mucosal lesions(AGML). A 73-year-old man with a past history of gastric ulcer, myocardial infarction, and arteriosclerosis of the right femoral artery presented to Osaka National Hospital complaining of intermittent claudication of the right lower extremity. After a diagnosis of arteriosclerosis obliterans, percutaneous angioplasty was performed. The postoperative course was initially good. However, two weeks after the angioplasty procedure he suddenly vomited blood. Emergency endoscopy revealed both polypoid gastric carcinoma and AGML, which were considered to be the major cause of his gastrointestinal bleeding. And he died of uncontrollable gastrointestinal bleeding two weeks after this examination. Autopsy showed an atherosclerotic aneurysm of the abdominal aorta that had ruptured into the third part of the duodenum. To our knowledge, there has been no previous autopsy on a primary aortoduodenal fistula from an atherosclerotic abdominal aneurysm complicated by similar hemorrhagic gastric lesions. This case emphasizes the difficulty of making a clinical diagnosis of primary aortoduodenal fistula.     

Incidental finding of right renal venous aneurysm in a patient with symptomatic ipsilateral renal carcinoma: a case report

BACKGROUND: True venous aneurysms of the renal veins are very uncommon lesions. Diagnosis is incidental, and thrombosed aneurysms may simulate solid renal masses. METHODS AND RESULTS: A case of right renal vein aneurysm incidentally found in a patient with a ispilateral renal carcinoma and abdominal aortic aneurysm is reported. While CT examination suggested a high-flow arteriovenous (A-V) malformation, a selective angiographic study identified two separate and independent pathologic conditions (venous aneurysm and intratumoral, acquired A-V fistulae). Successful preoperative embolization of the renal tumor was obtained and surgical treatment (nephrectomy+aneurysmectomy) was uneventful. CONCLUSIONS: Although uncommon, venous renal aneurysms require an accurate preoperative diagnosis; this case is interesting because the coexistence of renal tumor with acquired A-V fistulae raised the prospect of a large, high-flow A-V communication with secondary venous enlargement. The integrated imaging studies were basic to differentiate acquired, tumor-induced A-V fistulae found in the lower renal pole from the true venous aneurysm located in the upper pole. To our knowledge, this is the first report of such a condition.     

Clinico-anatomical analysis of the causes of death in ruptures of intracranial arterial aneurysms

Causes of lethal outcome in patients after subarachnoid hemorrhage due to cerebral aneurysm rupture were analysed. The major death cause in the operated patients was cerebral edema and dislocation, while in non-operated ones it was the penetration of blood into the ventricles of the brain. Cerebral edema and dislocation syndrome were mainly caused after surgery by ischemic changes in cerebral tissue. A relationship between the aneurysm localization and the extent of subarachnoid hemorrhage was revealed as well as between the hematoma localization and incidence of hemorrhage into the ventricles of the brain.