External carotid occlusion as a cause of recurrent ischemia after carotid endarterectomy.

Our experiences with a patient who developed recurrent hemispheric transient ischemic attacks 3 1/2 years after ipsilateral carotid endarterectomy are described. Although repeat arteriography demonstrated smooth and unobstructed common and internal carotid arteries and a normal intracranial circulation, there was complete occlusion of the external carotid artery at its origin, which appeared as a stump or diverticulum at the origin of the internal carotid artery. This stump was filled with atherothrombotic debris and served as a source of emboli to the adjacent and patent internal carotid artery. Excision of the stump effected complete relief of the recurrent ischemic symptoms. The pernicious potential of an external carotid obstruction when adjacent to a patent internal carotid artery should be recognized. To prevent this avoidable complication of carotid endarterectomy, we recommend that the origin of the external carotid artery be treated with the same standards of technical perfection as those customarily applied to the internal and common carotid arteries during this procedure.

Recurrent retinal ischemia beyond cervical carotid occlusions: clinical-angiographic correlations and therapeutic implications.

Seventeen patients with persistent amaurosis fugax ipsilateral to angiographically documented internal carotid artery (ICA) occlusions in the neck have been treated by the authors over the past 5 years. Complete cerebral arteriography in each case demonstrated that the symptomatic ophthalmic artery was perfused exclusively by the ipsilateral external carotid artery (ECA), which invariably had an obstructive and/or ulcerative lesion and its origin, and/or an adjacent residual "stump" of the occluded ICA. In nine patients, retinal artery branch emboli were visible on funduscopy. One patient had angiographic evidence of intracranial embolization via the ophthalmic artery from the ECA. Although ipsilateral superficial temporal-muscle cerebral artery anastomosis in one patient, and endarterectomy of a contralateral carotid stenosis in another patient, failed to relieve symptoms, endarterectomy of the ECA with resection of the "stump" of the occluded ICA effectively terminated symptoms in 10 of 11 patients. Anticoagulant drug therapy promptly abolished symptoms in four nonsurgical patients as well as in two patients with failed operations. It is concluded that recurrent retinal ischemia beyond cervical carotid occlusions frequently results from microembolism via the ipsilateral ECA. Patients with this mechanism of postocclusion recurrent ischemia can be identified on the basis of clinical history, ophthalmological examinations, and complete cerebral arteriography. Termination of embolic phenomena should be the major treatment goal in these individuals, and ECA endarterectomy is recommended. Anticoagulant drugs are an effective alternative treatment in patients who are poor surgical risks.

Von Hippel-Lindau Disease with multiple manifestations: diagnosis and management.

The association of a recurrent cerebellar hemangioblastoma, retinal angioma, pheochromocytoma, renal carcinoma, and multiple renal and pancreatic cysts in one patient is reported, and the pertinent diagnostic studies are discussed briefly. Awareness of the possibility of late manifestations of various components of this syndrome in patients treated successfully for cerebellar hemangioblastoma will lead to their early identification and successful surgical treatment. Genetic counseling for patients affected by this syndrome and their families is recommended.

Carotid ligation for recurrent ischemia due to inaccessible carotid obstruction. Examination of the rationale of this treatment.

Experiences with a patient with symptomatic obstruction to the carotid artery in its petrous segment are described. In spite of the severe stenosis of this vessel, complete arteriography demonstrated excellent perfusion of the symptomatic eye and hemisphere and an ample collateral reserve. Funduscopy confirmed the clinical impression that recurrent retinal and hemispheric ischemia in this patient was the result of microembolism rather than intracranial hemodynamic insufficiency. Consequently, extracranial-intracranial (EC-IC) bypass was believed to offer little benefit to this patient. Abrupt ligation of the internal carotid artery in the neck proved to be an effective method for arresting the embolic discharge from this vessel's inaccessible obstruction, and resulted in prompt and complete relief of ischemic symptoms. It is concluded that identifying the mechanism(s) responsible for recurrent ischemia past uncorrectable carotid obstructions is of paramount importance in order to establish the most appropriate treatment(s). Carotid occlusion is an effective surgical remedy for terminating microembolism from this vessel when it is diseased and incompletely obstructed, and should be considered in selected patients. The importance of angiographic evaluation of naturally occurring EC-IC anastomotic connections in addition to the assessment of intracranial collateral reserves in cases of carotid occlusion is also emphasized.

Intracranial embolization via external carotid artery: report of a case with angiographic documentation.

This report describes our experiences with a patient who developed delayed recurrent retinal and hemispheric ischemia distal to an old internal carotid artery occlusion in the neck. Fundoscopy and sequential cerebral arteriography documented that recurrent ischemic symptoms in this individual were the result of embolic fragments arising from the "stump" of the occluded internal carotid artery and from a diseased external carotid artery. These emboli traversed the external carotid artery and its orbital and intracranial anastomotic connections to reach the symptomatic eye and hemisphere. Ischemic symptoms in this patient were effectively terminated with anticoagulant therapy. We believe that this patient graphically documents that post-occlusion microembolism via the external carotid artery does indeed occur, and probably accounts for post-occlusion recurrent ischemic attacks more frequently than is currently appreciated. Recognition of this phenomenon is of importance because of its significant therapeutic implications. In these situations treatment modalities which terminate embolic phenomena would appear to have a more rational basis than do surgical procedures designed primarily to augment collateral blood flow to the symptomatic organ(s).

Acute abdomen in children with infected ventriculoperitoneal shunts.

During the past 48 months, seven children with infected ventriculoperitoneal shunts with acute abdominal emergencies in the absence of neurological signs or symptoms were encountered. Initial confusion in the correct diagnosis led to unnecessary laparatomy in three children and a dangerous delay in the initiation of appropriate treatment in all seven patients. The correct diagnosis was made by analysis of ventricular fluid obtained from the shunt reservoir. Diversion of infected CSF from the inflamed peritoneal cavity combined with intravenous and intraventricular antibiotics resulted in prompt resolution of abdominal signs and successful sterilization of the CSF. Total shunt replacement in uncontaminated CSF followed by postoperative antibiotic therapy administered intravenously effected complete cures in all patients. Awareness of this syndrome and its proper management is of paramount importance.

Gram-positive peritonitis in patients with infected ventriculoperitoneal shunts.

Patients with infected ventriculoperitoneal shunts may, in some situations, present with prominent abdominal signs and symptoms and no neurologic complaints. As a consequence, the correct diagnosis may be confused initially, and the appropriate therapy may be dangerously delayed. Definitive diagnosis is established by percutaneous tap of the shunt reservoir and analysis of the ventricular fluid. If the offending organism is gram-positive, externalization of the shunt combined with systemic and intraventricularly administered antibiotics, without laparotomy, is the treatment of choice. If gram-negative organisms are identified, exploratory laparotomy is indicated to rule out a ruptured viscus. In establishing the correct diagnosis and determining the appropriate therapeutic priorities, the co-operative efforts of the general, as well as the neurologic, surgeon are required.

External carotid artery in internal carotid artery occlusion. Angiographic, therapeutic, and prognostic considerations.

Twenty-three instances of internal carotid artery occlusion occurring with minimal neurological deficit in 22 patients are described. Although each of these patients was referred to the neurosurgical service for evaluation for an extracranial-intracranial microvascular bypass procedure, complete arteriographic evaluations of their cerebrovasculature suggested that alternative methods should be the treatment of choice. For each patient reported the ipsilateral external carotid artery was demonstrated by angiography to be an important source of collateral blood supply to the cerebral hemispheres or retinae distal to the occluded internal carotid arteries. Ten patients with no significant atherosclerotic narrowing or ulceration of the external carotid artery have remained free of symptoms of cerebral ischemia for 6 to 40 months. In twelve patients who developed delayed recurrent cerebral or retinal ischemia ipsilateral to their internal carotid artery occlusion, there were found obstructive and/or ulcerative plaques involving the common and/or external carotid arteries. Thromboendarterectomy in 11 of these patients gave complete relief of ischemic symptoms during the 4 to 36 months of postoperative follow up. One of these 12 patients refused operation and went on to develop a major cerebral infarction. Angiographic identification of a functionally important external carotid artery ipsilateral to an internal carotid artery occlusion carries considerable prognostic and therapeutic significance.

Reconstitution of "totally" occluded internal carotid arteries: angiographic and technical considerations.

The authors describe their experiences with five patients with delayed transient ischemic symptoms homolateral to internal carotid artery (ICA) occlusions. In each instance, initial arteriograms were interpreted as showing irreversible occlusions of these arteries in the neck and microvascular bypass procedures were contemplated. However, after repeat arteriographic evaluations with a modified injection technique, certain angiographic features were identified that suggested the mechanism of the symptoms in these patients and that their ICA's could be reconstituted in the neck. This was successfully accomplished in each patient with complete relief of ischemic symptoms. The angiographic technique employed and the arterial flow patterns identified in these patients are discussed. The details of the operative technique are described. It is concluded that routine arteriographic techniques may be inadequate to identify the reversible carotid occlusion. This may account for some of the confusion which surrounds the surgery of these vessels as well as the failure of extracranial-intracranial bypass to relieve recurrent ischemic symptoms in these individuals.

Congenital stenosis of the cervical spine: diagnosis and management.

An analysis of 11 cases of congenital stenosis of the cervical spine seen over the past three years is reported. The authors' experiences at an urban community hospital, as well as a large Veterans Administration Hospital, lead them to conclude that this disorder is a neurologically significant anomaly which is probably more common than published reports would imply. It can be clinically and radiographically distinguished from "pure" cervical spondylosis, to which it is related, and its treatment should be appropriately modified. It appears to have a predilection for young adult black males, and cervical myelopathy is the predominant clinical feature. Varying degrees of trauma, a disease endemic to the inner city, plays a major role in precipitating the neurological catastrophes associated with this potentially correctable disorder. Proper management of this entity demands a heightened awareness of its existence as well as a high standard of neurological and roentgenographic diagnosis, and operative performance. A flexible operative strategy which takes into account the specific biomechanical factors involved in this disorder as well as the patient's individual physiological and social status is imperative. Surgery offers a good opportunity for improving neurological function.

Cervical carotid aneurysm presenting as recurrent cerebral ischemia with head turning.

Extracranial carotid artery aneurysms are uncommon lesions with protean manifestations. This report describes a patient in whom the presenting symptom of a right carotid aneurysm was recurrent right hemisphere ischemic attacks when he turned his head to the left. The angiographic and operative findings explained the mechanism(s) of his symptoms. The importance of such symptoms is that they should suggest a mechanical etiology and that the probability of a surgically correctable lesion exists. Arteriography is the only reliable means of making a definitive diagnosis and should be considered early in the evaluation.

Dilated episcleral arteries--a significant physical finding in assessment of patients with cerebrovascular insufficiency.

Dilated episcleral vessels associated with ipsilateral internal carotid artery occlusions have been previously reported though not widely appreciated. These ocular changes have been presumed to be manifestations of ocular ischemia. The authors have recently encountered this sign in seven patients and in none was there evidence of ocular ischemia. In addition to an ipsilateral internal carotid artery occlusion, arteriograms demonstrated that the major source of blood supply to the homolateral cerebral hemisphere was by retrograde flow through markedly enlarged ophthalmic arteries filled in retrograde fashion from dilated external carotid collateral channels in the orbit. This association of dilated episcleral arteries as a sign of increased orbital blood flow and the major source of collateral blood supply to the homolateral cerebral hemisphere has not been previously reported. We reemphasize the importance of a careful examination of the episcleral vessels in patients suspected of having internal carotid artery occlusions.

Extrinsic neural influences on gastrointestinal motility.

The gastrointestinal tract is capable of carrying on all its major functions after all extrinsic nerves have been cut. This automaticity is due to the local nervous mechanisms in the walls of the gastrointestinal tract and the inherent properties of the smooth muscles in its walls, and gastrointestinal hormones. All levels of the central nervous system have been shown, by stimulation and ablation studies, to influence the motility of the entire gastrointestinal tract. Throughout many cerebral areas there are loci which, on stimulation, exert both inhibitory, and less often, excitatory influence on gastrointestinal motility. These influences are mediated by sympathetic and parasympathetic visceral efferent nerves, as well as humoral agents from the neurohypophysis. Thus, they impose an influence of higher control on the automatically efficient intrinsic motility. They are guided by information received from visceral, cranial, and somatic afferents, as well as intracerebral, or psychic inputs. Under normal circumstances they only influence gastrointestinal activity as will best afford the optimal functioning of a performance done automatically with efficiency and finesse.