Biomedical subjects

D G Piepgras

Publications and source records attributed to D G Piepgras.

At least 73 records · Page 4Linked to original sources

PubMed1994

Alpha 1-antitrypsin deficiency in intracranial aneurysms and cervical artery dissection.

The pathogenesis of ruptured intracranial aneurysms and cervical artery dissections is poorly understood but may be similar in these two disorders. We report four patients with alpha 1-antitrypsin deficiency who developed a ruptured intracranial aneurysm or spontaneous dissection of the cervical internal carotid artery. Three patients were heterozygous for the deficient allele (PiMZ or PiGZ) and one was homozygous (PiZZ). A deficiency of alpha 1-antitrypsin or one of the other protease inhibitors could result in degradation of the arterial wall through an imbalance between proteolytic enzymes and their inhibitors, thereby predisposing the arterial wall to dissection or aneurysm formation.

Adult↗

PubMed1994

Familial paragangliomas of the head and neck.

OBJECTIVE: It has been known for some time that paragangliomas of the head and neck may be familial. Recent evidence suggests genomic imprinting is an important factor in the clinical development of these tumors. In genomic imprinting the imprintable gene is transmitted in a mendelian manner, but expression of the gene is determined by the sex of the transmitting parent. In the case of paragangliomas, the gene does not result in the development of tumors when maternally inherited. The purpose of this study was to determine if this hypothesis correctly predicted the inheritance pattern of familial paraganglioma in a large series of patients. SETTING: A retrospective review of case records of the Mayo Clinic, Rochester, Minn, a tertiary referral center, was performed for the years 1950 to 1992 to identify patients with familial paragangliomas of the head and neck. PATIENTS: A total of 38 patients with familial paragangliomas of the head and neck were identified in nine kindreds. RESULTS: Carotid body tumors were the most common paragangliomas of the head and neck (34 cases). Glomus jugulare or glomus vagale occurred in eight cases. In 16 (42%) of the patients there were multiple tumors. Surgical complications, primarily cranial nerve palsies, were more frequent for tumors larger than 5 cm in diameter. CONCLUSIONS: An autosomal dominant inheritance pattern could be shown for paragangliomas of the head and neck that was consistent with genomic imprinting. It is recommended that all individuals in kindreds with paragangliomas be screened after the age of 16 years to detect early asymptomatic tumors.

Adolescent↗

PubMed1994

Endovascular treatment of intracranial aneurysms.

OBJECTIVE: To examine the techniques, reported experiences, and advantages and disadvantages associated with the endovascular treatment of intracranial aneurysms. DESIGN: We review the endovascular techniques used for the treatment of intracranial aneurysms and the sequelae of subarachnoid hemorrhage, which have evolved during the past 10 years. MATERIAL AND METHODS: Two broad categories of endovascular therapy for intracranial aneurysms are described: occlusion of the parent artery and preservation of the parent artery by selective occlusion of the aneurysm with balloons or metallic coils. The Mayo protocol for testing tolerance of patients before permanent balloon occlusion of the parent artery is described, as are the types of aneurysms most amenable to this treatment. In addition, use of balloon angioplasty for cerebral vasospasm after subarachnoid hemorrhage is reviewed. RESULTS: Recent improvements in microcatheter technology have facilitated the safe navigation of percutaneously introduced catheters in the intracranial circulation and selective catheterization of intracranial aneurysms. Surgically difficult aneurysms are now being treated with endovascular techniques more frequently than in the past. Early results from animal experiments and human trials have shown that selective occlusion of aneurysms with metallic coils may have a role in the treatment of intracranial aneurysms. Balloon angioplasty of symptomatic cerebral vasospasm has demonstrated improvement in neurologic function in approximately 70% of patients. CONCLUSION: As technology continues to improve and as greater experience is obtained, interventional neuroradiologists will continue to have an increasingly important role in the treatment of intracranial aneurysms.

Angiography, Digital Subtraction↗

PubMed1994

On the inheritance of intracranial aneurysms.

BACKGROUND AND PURPOSE: The familial occurrence of intracranial aneurysms suggests the presence of a genetically determined underlying arteriopathy. The pattern of inheritance in these families usually is not known. METHODS: A family with seven members with intracranial aneurysms is described and, from the literature before 1994, a total of 238 families with 560 affected members (56% female and 44% male) with intracranial aneurysms not associated with a known heritable disease are reviewed. A segregation analysis was performed on 73 of these families. RESULTS: Two members were affected in the great majority of families (79%); five or more members were reported in only eight families (3%). The most common affected kinship was among siblings. Angiographic screening in 12 families detected an intracranial aneurysm in 29% of 51 asymptomatic relatives. Segregation analysis revealed several patterns of inheritance that were consistent with the compiled pedigrees, but no single mendelian model was the overall best fitting, suggesting that genetic heterogeneity may be important. Twenty-two percent of siblings of male probands had an intracranial aneurysm compared with 9% of siblings of female probands (P = .003). CONCLUSIONS: Genetic heterogeneity may be important in the genetics of intracranial aneurysms. In families with intracranial aneurysms, siblings of an affected male proband may be at a higher risk of developing an aneurysm than siblings of an affected female proband. Screening for intracranial aneurysms in asymptomatic relatives should be considered in families with two or more affected members. In most families, the nature of the underlying arteriopathy remains obscure.

Adult↗

PubMed1994

Coexistence of fibromuscular dysplasia and cystic medial necrosis in a patient with Marfan's syndrome and bilateral carotid artery dissections.

BACKGROUND: A primary arteriopathy is suspected in most patients with spontaneous dissections of the carotid artery, although the nature of this arteriopathy usually remains elusive. Angiographic changes of fibromuscular dysplasia (FMD), however, are found in 10% to 20% of patients with carotid dissections. CASE DESCRIPTION: A 26-year-old woman with Marfan's syndrome presented with bilateral amaurosis fugax after surgical repair of an aortic dissection. Angiography revealed a dissection extending from the ascending aorta into the right internal carotid artery and, separate from the aortic dissection, a dissection of the left internal carotid artery. After surgery, microscopic examination of the right carotid artery revealed a medial dissection but no evidence of an underlying arteriopathy, while the left internal carotid artery displayed the typical features of FMD. Eighteen months later the patient died after resection of a large thoracoabdominal aortic dissecting aneurysm. Microscopic examination of the aorta revealed moderately extensive cystic medial necrosis. CONCLUSIONS: Carotid dissections associated with Marfan's syndrome may be the result of an extension of an aortic dissection or occur isolated from the aorta, even in the same patient. The occurrence of FMD in a patient with Marfan's syndrome in conjunction with previous reports of FMD in a variety of connective tissue disorders suggests that FMD, like cystic medial necrosis, may be a nonspecific disease entity.

Adult↗

PubMed1994

Neurovascular manifestations of heritable connective tissue disorders. A review.

BACKGROUND: Heritable disorders of connective tissue are recognized in a small minority of patients with neurovascular diseases. In this report, we review the neurovascular manifestations of four heritable connective tissue disorders: Ehlers-Danlos syndrome, Marfan's syndrome, osteogenesis imperfecta, and pseudoxanthoma elasticum, as well as two other systemic disorders with potential vascular manifestations: neurofibromatosis and polycystic kidney disease. SUMMARY OF REVIEW: Typical neurovascular complications of Ehlers-Danlos syndrome are carotid-cavernous fistulae, intracranial aneurysms, and cervical artery dissections. Arterial dissections and intracranial aneurysms cause the majority of neurovascular symptoms in Marfan's syndrome. Neurovascular disease is uncommon in osteogenesis imperfecta, although carotid-cavernous fistulae and vertebral artery dissections have been reported. Neurovascular disease in pseudoxanthoma elasticum is characterized by intracranial aneurysms and cerebral ischemia caused by premature arterial occlusive disease. Intracranial occlusive arterial disease is the most common neurovascular manifestation of neurofibromatosis, followed by cervical arteriovenous fistulae and aneurysms and intracranial aneurysms. Intracranial aneurysms are the hallmark of polycystic kidney disease. CONCLUSIONS: Recognition of an underlying generalized connective tissue disorder may be of considerable importance, although marked phenotypic heterogeneity often complicates the diagnosis of these disorders. Conversely, the association of certain neurovascular anomalies with generalized connective tissue disorders and recognition of their basic molecular defect may offer clues to the etiology and pathogenesis of these neurovascular diseases in general.

Cerebrovascular Disorders↗

PubMed1994

Perimesencephalic subarachnoid hemorrhage. Additional perspectives from four cases.

BACKGROUND: Nonaneurysmal perimesencephalic hemorrhage, a distinct form of subarachnoid hemorrhage, is a recently described variant of intracranial hemorrhage. We describe two patients who presented with unusual features of this type of subarachnoid hemorrhage and also two patients who had a perimesencephalic pattern of hemorrhage due to a ruptured posterior circulation aneurysm. CASE DESCRIPTIONS: The first patient, a 41-year-old woman with perimesencephalic hemorrhage, underwent an exploratory craniotomy because angiography had suggested an anomaly of the basilar tip. No source of hemorrhage could be identified at the time of surgery. The second patient was a 3-year-old boy who presented with opisthotonos and who was found to have a perimesencephalic hemorrhage. Angiography revealed no source for the hemorrhage. The third patient, a 54-year-old man, had a perimesencephalic pattern of subarachnoid hemorrhage from a vertebrobasilar junction aneurysm associated with a fenestration that was missed on the initial angiographic study. The fourth patient, a 43-year-old man, suffered a perimesencephalic pattern of subarachnoid hemorrhage from a small posterior cerebral artery aneurysm, which had not been recognized on two angiograms. CONCLUSIONS: These patients elaborate on the clinical spectrum of subarachnoid hemorrhage with a perimesencephalic pattern. First, a negative exploratory craniotomy suggests that the source of nonaneurysmal perimesencephalic hemorrhage may not be arterial. Second, nonaneurysmal perimesencephalic hemorrhage may also occur in children. Finally, the index of suspicion for a posterior circulation aneurysm should remain high in patients who present with a perimesencephalic pattern of subarachnoid hemorrhage, and these aneurysms may rise from unusual locations.

Adult↗

PubMed1994

Spontaneous dissections of cervicocephalic arteries in childhood and adolescence.

Among 263 consecutive patients with spontaneous cervicocephalic arterial dissections evaluated at the Mayo Clinic, 18 (6.8%) were 18 years of age or younger (mean age, 12 years). The dissection involved the cervical arteries in 11 patients and the intracranial arteries in seven. Extracranially, the internal carotid artery was involved in eight patients, the vertebral artery in two, and both arteries in one. Intracranially, only the anterior circulation was affected. All the patients had cerebral or retinal ischemic symptoms, usually preceded by headache. Death occurred in a 13-year-old boy with intracranial arterial dissection and coarctation of the aorta. For the 17 remaining patients, the follow-up period ranged from 1 to 21 years. A complete or good clinical recovery occurred in 10 of the 11 patients with cervical arterial dissection but in only four of the seven with dissection of intracranial arteries. Recurrent arterial dissection occurred in two patients with cervical arterial dissections and in one patients with intracranial arterial dissection.

Adolescent↗

PubMed1994

Chronic meningitis: the role of meningeal or cortical biopsy.

Meningeal and cortical biopsies were evaluated in 37 patients (25 men and 12 women; mean age, 54 yr) who had chronic meningitis of an unknown cause between 1985 and 1993 (the era of magnetic resonance imaging). Magnetic resonance imaging with gadolinium contrast was the most useful diagnostic imaging technique, demonstrating meningeal enhancement in 15 of 32 patients (47%). Only 2 of 32 (6%) computed tomographic scans revealed enhancement. A definitive diagnosis was made in 16 of 41 biopsies (39%), but in cases where enhancement was present on either magnetic resonance imaging or computed tomography, a diagnosis was obtained in 80% (12 of 15 cases). Only 2 of 22 biopsies (9%) from nonenhancing regions were diagnostic. Although the locations of enhancement were distributed evenly, biopsies through suboccipital and pterional craniotomies gave the highest diagnostic yields (50%). Furthermore, if the biopsies were obtained from enhancing regions, the yield of these two approaches increased to 84 and 100%, respectively. Of 18 cases in which biopsy samples were taken from both the meninges and cortex, only 1 had cortical involvement alone. The meninges were therefore diagnostic in 15 of the 16 definitive diagnostic cases (94%). Second biopsies were necessary in four cases, of which the three biopsies from enhancing regions were diagnostic. The most frequent causes of chronic meningitis were sarcoid (31%) and metastatic adenocarcinoma (25%).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed1994

Surgical treatment of extracranial internal carotid artery dissecting aneurysms.

Aneurysms of the extracranial internal carotid artery (ICA) are uncommon. A significant proportion of such aneurysms are now recognized to be caused by arterial dissection. In some patients, surgical treatment may become necessary. The surgical treatment of 22 patients with spontaneous or traumatic dissecting aneurysms arising from the extracranial ICAs is reviewed. The mean age of the 7 women and 15 men was 39 years. The aneurysm arose from the proximal third of the extracranial ICA in 1 patient, from the middle third in 1 patient, and from the distal third in 20 patients. Five patients underwent cervical carotid ligation; in 13 patients, the aneurysms were resected, and the ICAs were reconstructed, and 4 patients underwent cervical-to-intracranial ICA bypasses. There were 2 postoperative strokes (9%). Facial and lower cranial nerve palsies were commonly seen after high cervical exposure, but these cranial nerve palsies were transient. There were no long-term neurological sequelae during a mean follow-up of 6.2 years. In our relatively limited experience, extracranial ICA dissecting aneurysms can be treated with acceptable morbidity using a variety of techniques. However, the indications for surgical intervention in these aneurysms remain limited.

Adult↗

PubMed1994

Factors affecting survival of patients with tumors of the anterior skull base.

Fifty-four patients with primary neoplasms of the anterior skull base were treated by craniofacial resection with curative intent. The most common tumor was esthesioneuroblastoma (24), followed by squamous cell carcinoma (10). The overall 2-year and 5-year survivals were 75% and 49%, respectively. High-grade tumors, grades 3 and 4, had a poorer prognosis. Tumor size, dural involvement, sphenoid sinus involvement, age, and sex had no significant influence on survival when examined by multivariate survival analysis. Sixteen complications were noted in the postoperative period. Cerebrospinal fluid leakage occurred in 2 patients and loss of frontal bone occurred in 4. There were no operative or perioperative deaths. Craniofacial resection permits surgical resection of the majority of anterior skull base tumors with acceptable morbidity.

Adolescent↗

PubMed1994

Surgical treatment of recurrent carotid artery stenosis.

Ninety-two surgical procedures were performed in 82 patients for recurrent carotid artery stenosis. The etiology was recurrent atherosclerosis in 45 cases, myointimal hyperplasia in 20, organized thrombus without a significant underlying plaque in 20, and scarring along the proximal arteriotomy site in seven. The operations included a repeat endarterectomy in 66 cases and reconstruction with an interposition graft in 22. All five major neurological complications occurred in symptomatic patients, and included three instances of intraoperative embolization during exposure of the carotid artery. The majority of neurological complications occurred in symptomatic patients who had intraluminal thrombus confirmed at surgery. There were four perioperative deaths, due to cerebral hemorrhage in two patients and myocardial infarction in two. In the patients whose original surgery was performed at the Mayo Clinic, the risk of recurrent carotid artery stenosis was 3.1% with a primary closure compared to 1.6% when a patch graft was used. These results indicate that surgery for recurrent carotid artery stenosis is technically more difficult and carries a significantly higher risk than surgery for primary disease. The difficulty is due to the friable recurrent plaque associated with intraluminal thrombus, which increases the risk of embolization during carotid artery exposure. In the majority of patients with recurrent atherosclerosis, a repeat endarterectomy can be achieved. However, in some patients, there is scarring without a definite plane of cleavage between the recurrent disease and the underlying media, making an endarterectomy difficult. In these cases, excision of the diseased segment and reconstruction with an interposition graft is the best treatment. The findings presented here also suggest that closure of the original arteriotomy with a patch graft decreases the risk of recurrent carotid artery stenosis.

Carotid Stenosis↗

PubMed1994

Intracranial dissection of the distal middle cerebral artery as an uncommon cause of distal cerebral artery aneurysm. Case report.

An aneurysmal dissection of a right middle cerebral artery (MCA) branch is described in a 56-year-old woman. The abnormality was an incidental finding on computerized tomography and subsequently appeared on magnetic resonance imaging performed to evaluate the patient for subjective pulsatile tinnitus. The intracranial aneurysm was documented to have enlarged on serial angiography over a 6-week interval. Treatment was believed to be necessary because of the unknown etiology of the aneurysm, with the differential diagnosis including mycotic or neoplastic aneurysm with a risk of hemorrhage. The lesion was excised and flow to the distal MCA branch was preserved with an anastomosis of the superficial temporal artery to the MCA. The aneurysm, which developed at the level of the sylvian fissure, proved on pathological study to be related to a focal dissection of the MCA branch. The radiographic appearance and pathological findings are presented. Focal dissection must henceforth be included in the differential diagnosis of peripheral cerebral artery aneurysms.

Aortic Dissection↗

PubMed1993

Carotid endarterectomy in patients with contralateral carotid occlusion.

In this study, we assessed the results of carotid endarterectomy in 357 patients with a carotid stenosis and contralateral carotid occlusion. The overall major neurologic morbidity was 0.6%, and the minor morbidity was 1.1%. The causes of four perioperative deaths (1.1%) were myocardial infarction in two patients, ruptured abdominal aortic aneurysm in one, and respiratory complications in one. Therefore, an excellent result was achieved in 97.2% of patients. With occlusion of the carotid artery for the endarterectomy, 165 patients (46%) had appreciable attenuation in intraoperative electroencephalographic findings and a decrease in cerebral blood flow to approximately 10 ml/100 g of brain tissue per min that necessitated placement of a shunt. This high percentage of profound electroencephalographic and blood flow changes during carotid occlusion suggests that the potential for collateral blood flow in this group of patients is minimal. These results demonstrate that a carotid endarterectomy can be performed at low risk in patients with a contralateral carotid occlusion. We advocate annual noninvasive carotid testing for patients with asymptomatic carotid stenosis and contralateral carotid occlusion. If progression of the stenosis is evident, a prophylactic endarterectomy should be considered because these patients may have a higher risk for cerebral infarction than do patients with a unilateral asymptomatic stenosis.

Aged↗

PubMed1993

Ocular motor nerve palsies in spontaneous dissections of the cervical internal carotid artery.

Cranial nerve palsies affecting ocular motor function in patients with spontaneous cervical internal carotid artery dissections are rare. Among 155 patients with spontaneous dissections of the cervical internal carotid artery, four (2.6%) had transient third, fourth, or sixth cranial nerve palsy. The third nerve was involved in two patients, the fourth nerve in one, and the sixth nerve in one. Three patients had ipsilateral headache or facial pain, one had bilateral headaches, and three had oculosympathetic palsy. None had any associated cerebral or retinal ischemic symptoms. Cervical internal carotid artery dissection should be included in the differential diagnosis of palsies of the third, fourth, or sixth cranial nerve, especially when associated with ipsilateral headache or facial pain. Interruption of the nutrient arteries supplying these cranial nerves could explain their involvement by internal carotid artery dissection.

Adult↗

PubMed1993

Transitional cavernous aneurysms of the internal carotid artery.

Twenty-three cases of transitional cavernous aneurysms are presented. Aneurysms of this subgroup (of a total of 118 cases of cavernous sinus aneurysms) arise entirely from within the cavernous sinus but project into the extracavernous intradural subarachnoid space, thus resembling other intracranial aneurysms in their increased risk of rupture. Six aneurysms were small (less than 15 mm), 6 were large (15 to 25 mm), and 11 were giant (more than 25 mm). Thirteen patients (57%) had a subarachnoid hemorrhage, nine patients (39%) had compressive symptoms, and one patient (4%) was asymptomatic. A direct surgical approach was performed successfully in 18 cases (78%), and indirect bypass methods were performed in 5 cases. The overall surgical outcome was excellent in 87% of the patients, with three complications (13%) including one fatality. It is suggested that this subgroup is a different entity from other cavernous or extracavernous aneurysms and should be managed aggressively with direct clipping whenever possible because of the increased risk of subarachnoid hemorrhage. A simplified numerical classification system of clinoidal-region aneurysms of the internal carotid artery (including transitional aneurysms) is also proposed.

Adult↗

PubMed1993

Seizure outcome in patients with surgically treated cerebral arteriovenous malformations.

A series of 280 cases of cerebral arteriovenous malformations (AVM's) treated surgically between June, 1970, and June, 1989, is reviewed with particular focus on the preoperative seizure history and follow-up seizure status. Follow-up evaluation (mean duration 7.5 years) was achieved in 98% of cases and was accomplished through re-examinations, telephone interviews, and written questionnaires. Overall, 89% of the surviving patients with a follow-up period of greater than 2 years were free of seizures at last examination. Of the 280 patients in this series, 163 had experienced no seizures preoperatively. A recent follow-up study (with a minimum duration of 2 years or to death) was available in 157 of these 163 cases; 21 patients had died. Of the 136 surviving patients, only eight (6%) were having new ongoing seizures. In the 128 (94%) who had remained seizure-free, 73% were receiving no anticonvulsant agents while 27% were taking anticonvulsant prophylaxis. The 2-year minimum follow-up study in 110 of the 117 patients with preoperative seizures revealed that eight (7%) had died. Of the 102 surviving patients, 85 (83%) were seizure-free (with 48% no longer receiving anticonvulsant therapy), while 17 (17%) still suffered intermittent seizures. However, of these 17 patients, 13 reported their seizures to be improved compared to preoperatively; the seizures were the same in two patients and were worse in two patients. An actuarial analysis was conducted comparing the life expectancy of patients following surgery for AVM's with the expected survival of a general white population of the same age and sex in the West Northcentral region of the United States. No statistically significant difference was found. There were seven perioperative deaths (three from cerebral hemorrhage, two from pulmonary emboli, and two from obstruction of venous drainage) and 22 deaths during the follow-up period. Of these 22 deaths, the cause was unknown in four patients, apparently unrelated to the AVM in 13, and directly or indirectly related to the patient's neurological condition prior to surgery or due to surgery performed for resection of the AVM in five. There was a statistically significant relationship between the size and location of the AVM and the clinical presentation. Patients with small AVM's (< 3 cm) were more likely to present with hemorrhage whereas those with large AVM's were more likely to present with seizures.(ABSTRACT TRUNCATED AT 400 WORDS)

Actuarial Analysis↗

PubMed1993

Occlusive hyperemia: a theory for the hemodynamic complications following resection of intracerebral arteriovenous malformations.

An alternative theory is proposed to explain the brain edema and hemorrhage that may occur after resection of high-flow intracerebral arteriovenous malformations (AVM's). This theory, termed "occlusive hyperemia," is based on a retrospective analysis of operative dictations along with postoperative imaging studies (191 angiograms and 273 computerized tomography scans) in 295 cases of intracerebral AVM's operated on at the Mayo Clinic between 1970 and 1990. In this series, 34 cases (12%) of postoperative deterioration were documented, of which 15 were due to incomplete resection of the AVM. Of the remaining 19 cases, six had brain edema alone and 13 had hemorrhage with edema, despite complete excision of the AVM. In these 19 cases, the AVM's were greater than 6 cm in diameter in 10 patients, between 3 and 6 cm in six, and less than 3 cm in three. Obstruction of the venous drainage system was observed in 14 (74%) of the 19 cases. Ten of these 14 were due to obstruction of the primary venous drainage of the brain parenchyma immediately surrounding the lesions, while four were due to obstruction of other venous structures. In no case was a rapid circulation identified on postoperative angiograms. The flow pattern was slow or stagnant in former AVM feeders and their parenchymal branches. It is proposed that postoperative intracranial hemorrhage and/or brain edema in AVM patients may be due to: 1) obstruction of the venous outflow system of brain adjacent to the AVM, with subsequent passive hyperemia and engorgement; and 2) stagnant arterial flow in former AVM feeders and their parenchymal branches, with subsequent worsening of the existing hypoperfusion, ischemia, and hemorrhage or edema into these areas. Supportive hemodynamic evidence for this theory was derived from the literature.

Adult↗