Henry Halperin, M.D.

• Co-Director, Johns Hopkins Imaging Institute of Excellence
• Professor of Medicine

https://www.hopkinsmedicine.org/profiles/results/directory/profile/0000674/henry-halperin

In one series asthma symptoms in 7 year old order discount montelukast, 30% of patients had rheumatoid nodules in the first year of the disease asthma with bronchitis 4 mg montelukast buy with mastercard. Rheumatoid nodules often occur distal to the olecranon asthma treatment doctors 5 mg montelukast free shipping, on flexor tendon sheaths asthma treatment for children under 3 cheap montelukast 4 mg buy on-line, Achilles tendon and the soles of the feet asthma treatment step up cheap montelukast express. Asymptomatic and lowgrade impaired lung function can be associated with active disease, but progressive fibrosis and methotrexate related lung fibrosis have not been reported in children. These may include: · · · · growth disturbances subluxation joint space narrowing and osseous erosion bone ankylosis. In the paediatric population, the first peak of onset, mainly in girls, occurs during preschool years, and the second is seen during middle to late childhood. Symmetrical arthritis and hand involvement appear to predict future disability and poorer overall wellbeing. The incidence rate also varies with ethnicity and it is more common in nonCaucasian populations. Clinical features Rheumatoid factorpositive polyarthritis is characterized by symmetrical polyarthritis affecting large and small joints and often associated with rheumatoid nodules. Arthritis is typically aggressive, Pathogenesis the pathogenesis of psoriatic arthritis is unclear. Genetic factors or an environmental trigger such as infection or trauma appears to unleash an inflammatory process involving infiltration of lymphocytes, neutrophils and other effectors of innate immunity into entheses and synovium. However, clinical differences include the tendency to develop dactylitis, to involve the wrists and small joints of the hands and feet, and to progress to polyarticular disease in the absence of effective treatment. By contrast, older children exhibit a gender ratio of 1:1, with a tendency to enthesitis and axial disease, more closely related to adult psoriatic arthritis. Enthesitis is defined as the inflammation localized to the insertion of a tendon, ligament, fascia or joint capsule into the bone. It is diagnosed clinically with specific tenderness and occasionally swelling at the specific sites, in the absence of other. It most commonly presents as the classic vulgaris form, although guttate psoriasis is also observed. Psoriasis in children tends to be subtle, with thin, soft plaques that may come and go. Lesions may be isolated to the hairline, umbilicus, behind the ears or in the intergluteal crease, and thereby escape ready notice. Nail changes (pits, onycholysis, horizontal ridging and discolouration) accompany childhood psoriasis in up to 30% of cases. More recent studies documented remission in approximately 60%, although only a minority achieve remission off medications. Clinical, genetic and epidemiological similarities suggest that infections may play a triggering role. Evidence of local inflammatory response to antigens is supported by some antibodies and cellular immune studies, but there is a lack of confirmation of these findings. Symptoms suggestive of sacroiliac or lumbosacral inflammation are sometimes present at onset, but most commonly develop during the disease course. Enthesitis the most important clinical characteristic is the presence of enthesitis. Enthesitis is diagnosed clinically from the presence of marked localized tenderness or swelling at the entheseal insertion into the bone. Observation of stance and gait (including walking on the toes and heels) may reveal altered weight bearing as the child avoids pressure on inflamed entheses. Arthritis Arthritis most commonly affects the joints of the lower extremities, including the hips. Joints of the upper extremities may be affected, but in the absence of lower extremity involvement, this is uncommon. Tenderness can sometimes be elicited by direct pressure over the sacroiliac joint (Patrick test). Sacroiliac joint involvement may be unilateral initially and progress later to involve both joints. The presence of gastrointestinal symptoms should raise the suspicion of arthritis related to inflammatory bowel disease. Poor weight gain and slow growth may be the first clues of gastrointestinal involvement. Undifferentiated arthritis Undifferentiated arthritis accounts for 2­56% of patients in different studies, reflecting the insufficient criteria or overlapping criteria leading to an accurate diagnosis. Treatment of juvenile idiopathic arthritis Juvenile idiopathic arthritis is a complex chronic disease and therefore should be managed by an experienced multidisciplinary team, including a rheumatology consultant, specialist nurse, paediatric physiotherapist, occupational therapist, ophthalmologist, pain specialist, orthopaedic surgeon, immunologist, endocrinologist, radiologist, pharmacist, psychology service and social worker to achieve the best possible outcome. Each member of the team contributes to the holistic management of the patient and family. The treatment approach is to achieve early diagnosis and rapid control of inflammation, minimize the side effects of treatment, and decrease the physical and emotional sequelae. Lack of knowledge about aetiology and pathogenesis, and the variability of disease are limitations. There is general agreement that there are fewer children with severely damaged joints and deformities than even a decade ago. Furthermore, it is important that the child is involved as much as possible in the therapeutic decision making, especially adolescents, leading to better engagement with the disease and treatment. Issues like alcohol, drug use and pregnancy risk are more likely to be managed effectively if the adolescent is fully involved. Patients are advised to avoid highimpact physical activity for 24­48 hours after a joint injection. Therefore, careful injection technique by an experienced clinician must be performed. However, it may be indicated as firstline therapy for severe polyarticular disease. Methotrexate is an antimetabolite with antiinflammatory and immunomodulatory properties. It is a folic acid analogue and inhibitor of dihydrofolate reductase that interferes with purine biosynthesis. Methotrexate also inhibits adenosine deaminase, leading to accumulation of adenosine. Transient elevation of liver enzymes is common so liver function monitoring is routinely performed. Some of the similarities and differences in the specific management of the different subtypes are illustrated in Table 15. Therefore, continuing therapy with oral prednisolone 1­2 mg/kg/day to a maximum of 60 mg/day is frequently necessary. A study of classification criteria for diagnosis of juvenile idiopathic arthritis. The early pattern of joint involvement predicts disease progression in children with oligoarticular (pauciarticular) juvenile rheumatoid arthritis. Association between duration of symptoms and severity of disease at first presentation to paediatric rheumatology: results from the Childhood Arthritis Prospective Study. Dense genotyping of immunerelated disease regions identifies 14 new susceptibility loci fot Juvenile idiopathic arthritis. In: Petty R, Laxer R, Lindsley C, Wedderburn L (eds), Textbook of Pediatric Rheumatology, 7th edn. Polyarticular juvenile idiopathic arthritis­ epidemiology and management approaches. International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton, 2001. Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment. Course, complications and outcome of juvenile idiopathic arthritisrelated uveitis. Journal of the American Association for Pediatric Ophthalmology and Strabismus 2008; 12: 539­545. Clinical assessment usually distinguishes between mechanical and inflammatory problems and an approach to assessment (Table 16. The presence of multisystem features broadens the differential to include connective tissue diseases. The concept of referred pain from the hip or thigh, for example, must be sought in situations where the child has knee pain but there is no evidence of localized disease at the knee. Certain sporting activities such as cricket, bowling or gymnastics pose increased risk of back pain, with possible consequences such as spondylolysis and spondylolisthesis. Isolated back pain in a child under the age of 5 years is very unusual, and should warrant a referral for investigation. Mechanical pain Osteochondritis of the knee (Osgood­Schlatter disease) is common, especially in adolescent boys who are physically active (particularly those who play football or basketball). Many children and adolescents with non specific aches and pains, including growing pains, are found to have joint hypermobility, which is suggested by symmetrical hyperextension at the fingers, elbows and knees (genu recurvatum), and flat pronated feet. Nonspecific aches and pains are also a feature of idiopathic pain syndromes, which are mostly seen in older female children/adolescents; such patients are often markedly debilitated by their pain and fatigue ­ the pain can be incapacitating ­ but the child/adolescent is otherwise well, and physical examination is usually normal. Localized idiopathic pain syndromes most commonly affect the foot or hand, may be triggered by trauma (often mild) and are likened to reflex sympathetic dystrophy. It is important to note that these malignancies may also present with frank arthritis. Osteoid osteomas (the most common benign bone tumour) are usually located in the femoral neck or posterior elements of the spine, and typically cause night pain that can be relieved by salicylates. Arthritis and infection Children with septic arthritis are usually febrile, appear unwell and have severe pain with joint movement. Septic arthritis usually occurs in large joints, generally involving a single joint. Reactive arthritis is usually monoarticular or oligoarticular and follows bacterial infection in the gut (Salmonella, Shigella, Campylobacter, Yersinia), although in the older child and adolescent it is important to consider sexually acquired infection (Chlamydia, Gonorrhoea). Lyme disease following ticktransmitted infection with Borrelia burgdorferi is suggested by the presence of an oligoarthritis, history of a tick bite, erythema chronicum migrans, and a travel history to an endemic area. Severe complications at the time of active disease may include risk of aspiration pneumonia and interstitial lung disease. Diagnosis usually rests on clinical assessment and elevated serum muscle enzymes; most children do not require electromyography or a muscle biopsy except in the absence of typical disease features. Magnetic resonance imaging of the muscles is very useful to demonstrate muscle involvement and monitor disease activity. Sclerodermas Scleroderma in childhood is rare and heterogeneous, and subtypes are determined by the type and number of lesions, the area of involvement and serological abnormalities. The functional and cosmetic impact can be profound, as the lesions may interfere with growth of a limb and subcutaneous tissues (of the face or a limb). Current practice advocates aggressive treatment regimes (corticosteroid and methotrexate) to control disease and limit severe disfigurement and disability. Systemic scleroderma is very rare in children and includes progressive diffuse fibrous changes of the skin and fibrous changes involving internal organs ­ most commonly lungs, gastrointestinal tract, heart and kidneys ­ with a significant mortality. Systemic scleroderma is slowly progressive, has a guarded prognosis and requires potent immunosuppression, although clinical trials are lacking to guide practice. A diagnosis may be suggested by multisystem clinical involvement and laboratory features (Box 16. There is often a broad range of severity among patients with the same genetic disorder, making diagnosis on clinical grounds often challenging. New treatment options have become available for some patients with periodic fever syndromes, and patients require specialist supervision. Children or adolescents present with bone pain, sometimes accompanied by swelling; the most common affected areas are long bones (tibia), but ribs, clavicle, vertebrae or mandible can be involved. Radiographs show osteolytic changes similar to osteomyelitis, and a bone scan may show lesions that are asymptomatic. Many children with rheumatic diseases have continuing disease activity or relapses in adulthood, or sequelae from previous disease activity, which require ongoing medical treatment. Musculoskeletal Disorders in Children and Adolescents 111 Healthcare transition for youths with childhoodonset rheumatic diseases describes the movement of patients from child and familycentred paediatric care to adultoriented healthcare systems. Education and support are paramount, particularly with complex treatment regimes and the impact on adolescent behaviours, such as avoidance of pregnancy and excess alcohol in those taking methotrexate. Both conditions are rare below the age of 50 and peak in patients aged over 70 years. Women are affected three times more frequently than men, and whilst it can affect any ethnic group, it is more common in northern Europeans. This highly disabling condition is the most common inflammatory rheumatological disorder of older people. Whilst it may affect any large or medium sized artery, it has a propensity to affect the branches of the external carotid artery, particularly the posterior ciliary arteries that supply the optic nerve and the superficial temporal artery (hence the use of the alternative name temporal arteritis). Prompt recognition and early treatment are essential to prevent longterm complications, including permanent loss of vision. There are clinical and pathogenetic links between temporal arteritis, giant cell arteritis and polymyalgia rheumatica, which has led to the concept that they are manifestations of a disease spectrum that affects the same disease population. The two entities may occur in the same patient simultaneously, at different time points or independently. Symptom onset is often rapid and dramatic, with bilateral pain and stiffness in the shoulders and hips causing marked functional impairment. Patients often report problems lifting heavy objects, getting off the toilet or rolling over in bed. Atypical presentation may lead to diagnostic difficulty, and as such early referral for specialist assessment can be helpful in nonclassic cases.

An aspiration catheter can also be utilized to reduce the embolic burden that may be suspended between the stented segment and distal filter asthma help purchase generic montelukast. When a balloon occlusion catheter is used for cerebral protection asthma journal montelukast 10 mg low cost, the embolic debris is aspirated before deflation and retrieval of the balloon asthma symptoms tiredness discount 5 mg montelukast with visa. The femoral access site is then secured either through manual compression or with a local clamping device; alternatively asthma histology montelukast 5 mg generic, for faster patient mobilization and greater comfort asthma definition dictionary order montelukast online now, a device may be used for closure. The stents are constructed principally of annular rings interconnected by bridges. Tines refer to the size of each individual hole in the wall of the metallic-alloy stent, which is determined by the number of bridges connecting the annular rings. The smaller the stent tines and the more connected the struts are, the more the stent is a closed-cell design and the more rigid its overall structure is. Therefore, closed-cell stents tend to straighten the vessel more often after deployment. In addition, by virtue of their decreased pore size and increased density of vessel wall and stent construct apposition, they may prevent the extrusion of plaque material through the tines. This makes them more appropriate for symptomatic lesions, which probably have more unstable plaque and exposed debris. However, the rigidity of these stents reduces their conformability around the bends and angles that are frequently encountered in the cervical carotid artery. As a consequence, sharp bends may be exaggerated, potentially worsening poststent kinking of the native vessel. Conversely, open-cell stent designs are less rigid and conform to native vessels more effectively. This feature occurs at the expense of tine density and pore size, which results in the potential for a worse "cheese-grating" effect with resultant release of embolic material, particularly in symptomatic unstable plaques. It has been further supported by the demonstration of a reduction in the frequency of lesions visualized on diffusionweighted imaging with distal embolic protection (49% versus 67%). Subsequently, several devices were developed that act as filter devices to allow continued blood flow and yet obstruct the passage of embolic debris. In any particular case, these devices should be considered for use on the basis of the following attributes: crossing profiles. An inflated balloon in the common carotid artery arrests anterograde flow, and an inflated balloon in the external carotid artery suspends retrograde flow. In this way, flow in the internal carotid artery is stopped, which allows the lesion to be crossed and the stent to be deployed without risk of distal. All these devices consist of polyurethane membranes or nitinol mesh on a nitinol frame. We select the device on the basis of ease of use, crossing profile, and pore size. The benefit of these devices is that they alleviate the need to cross a lesion without protection before it is "secured" by placement of a stent. The concept of proximal protection began with occlusion of the common carotid artery with balloon guide catheters. However, this technique may maintain a potential for distal emboli via reversal of flow from the external carotid artery to the internal carotid artery. This concept first was introduced in 2000 via the Parodi AntiEmbolization Catheter (ArteriA, Inc, San Francisco). This device consisted of a balloon catheter that occluded the common carotid artery and was connected to a sheath that was inserted into the femoral vein. Carotid access is gained directly via a supraclavicular incision and insertion of the working sheath, which thereby avoids catheter manipulation (and potential release of embolic debris) in both the aortic arch and the proximal common carotid artery. Clamping of the external carotid artery is not necessary because of the amount of reversed flow generated by direct insertion of the working sheath (Video 367-1). The device was successfully utilized in 99% of 141 patients enrolled in the study. Although a randomized controlled study demonstrated no significant difference in long-term stent patency when intravascular ultrasonography was utilized,86 this technology may provide insightful, real-time information that is helpful in dictating therapeutic strategies. Its primary use thus far has been to define the extent of plaque formation and evaluate plaque structure as a means to identify so-called vulnerable plaque. We have found it useful during poststenting deployment and poststenting angioplasty to evaluate for correct stent expansion and intrastent or intraluminal thrombus (Video 367-2). When thrombus is identified, we typically use mechanical aspiration to remove friable intraluminal fragments, thereby eliminating the chance of distal embolization. In addition, we have found intravascular ultrasonography particularly useful when there is discrepancy between findings in other modalities. In one case, Doppler evaluation revealed elevated in-stent velocity, but angiography failed to demonstrate significant stenosis. Intravascular ultrasonography revealed significant stenosis, which was subsequently treated by angioplasty. In patients who have concurrent renal dysfunction, hydration includes alkalinization of the urine with 3 ampules of sodium bicarbonate added to each liter of 5% dextrose with normal saline for prophylaxis against contrast-induced nephropathy. Intravenous hydration should be maintained for up to 24 hours after the procedure. Postprocedure hypotension should be aggressively managed with vasoconstrictors such as phenylephrine (Neo-Synephrine) or intravenous infusion of dopamine. Carotid artery disease is frequently associated with coronary artery disease, and sustained hypotension is a harbinger of myocardial ischemia. If a closure device has not been used, the arterial sheath should be removed when the activated coagulation time exceeds 150 seconds. In this system, direct access to the carotid artery is obtained through a small incision made between the clavicular heads of the sternocleidomastoid muscle (A). The carotid sheath is connected to a flow regulator and filter, which is continuous with a sheath inserted in the femoral vein. In this way, blood flow is temporarily diverted away from the brain during treatment. Debris created during the procedure is directed away from the brain (B) and trapped in the filter, and debris-free blood is returned into the femoral vein, which thereby decreases blood loss. All patients routinely undergo cardiac enzyme and electrocardiographic review before discharge. Duplex sonographic evaluation should be obtained before discharge, at 6 weeks, at 3 months, at 6 months, at 1 year, and then annually thereafter. The dual antiplatelet regimen of aspirin and clopidogrel (or ticlopidine) is maintained for 12 weeks after the procedure, after which patients remain on aspirin therapy. In a retrospective study of patients undergoing stenting for carotid stenosis, both de novo (119 arteries) and postendarterectomy (76 arteries), restenosis of 80% or more was detected with Doppler imaging in 5. Thereafter, the risk falls significantly, remaining low for the duration of these studies. During the 4-year study period, the composite primary end point-any periprocedural stroke, myocardial infarction, or death or postprocedural ipsilateral stroke-occurred in 7. In addition, a sudden decline in blood pressure or the onset of severe bradycardia, as may occur during stent placement or angioplasty, may present a major risk for myocardial infarction in patients with severe disease of the left main coronary artery or severe triple-vessel disease. Specifically, the presence of large-territory infarction poses a significant risk for hemorrhage. High-risk anatomy includes iliofemoral disease, calcified or tortuous aortic arches, and tortuosity or kinking of the common or internal carotid artery (Table 367-5). Therefore, the surgeon should evaluate each case on an individual basis, weighing the risks and benefits for each patient. When treatment is deemed necessary, creative endovascular solutions can be found even for patients at high risk for complications. For patients with intraluminal thrombus and symptomatic carotid artery disease, the traditional treatment has been heparin and warfarin therapy with reevaluation in 6 weeks to 3 months. Flow-reversal systems also may prove useful in this setting and in the context of kinks that preclude the landing of a distal embolic protection device. DirectCarotidAccess Direct carotid access has its own set of potential complications. The internal jugular vein is positioned adjacent to the carotid artery; inadvertent injury to the vein can usually be managed with manual pressure. For percutaneous carotid access, the use of ultrasonography can help distinguish the artery from the adjacent vein. Complications associated with access via carotid cutdown are similar to those seen in other anterior cervical approaches in neurosurgery. However, because the cutdown is directed toward the common carotid artery, rather than the bifurcation, the risk of injury to the cranial nerves is decreased. Manipulation of the cervical strap muscles may cause swelling and postprocedure pain. However, the development of a postprocedure hematoma must be recognized and managed aggressively because it has the capacity to cause airway compromise. Because approximately 33% of patients have both significant carotid artery disease and severe symptomatic peripheral vascular disease,108,109 the operator should also be familiar with radial and brachial approaches. The true incidence of access site complications is unknown because these are typically not recorded in major carotid stent trials. Micropuncture kits, single wall puncture, and femoral angiography to evaluate access site anatomy and disease mitigate many of these complications, including dissection, occlusion related to the closure device, and groin or retroperitoneal hematomas. GuideCatheterPlacement the external carotid artery and its branches are often used to support a guidewire during the exchange of a diagnostic catheter for a guide sheath or catheter. This maneuver is thought to be safer than exchanging devices within the common carotid artery because it prevents premature crossing of the lesion and showering of emboli. In addition, it provides distal stability that is essential in the setting of a difficult aortic arch that might not be otherwise accessible. The four wire perforations were secondary to advancement of the stiff exchange wire through the facial artery (two cases), the lingual branch (one case), and the artery to the sternocleidomastoid artery (one case). These resulted in hematomas that responded to manual pressure in one case; necessitated embolization with n-butyl cyanoacrylate (glue) or coils in two cases; and necessitated emergency tracheostomy for airway control secondary to massive tongue swelling in one case. First, large branches of the external carotid artery, preferably the internal maxillary artery or occipital artery, should be used for exchange maneuvers. Second, rupture of a branch of the carotid artery can lead to life-threatening airway emergencies. A physician who can intubate the patient or perform an emergency tracheostomy to establish an airway should be readily accessible. Third, carotid interventionists need to be comfortable with microcatheters and embolic agents such as n-butyl cyanoacrylate, coils, silk, Gelfoam (Pfizer, New York), and even autologous clot as a means to stop bleeding from vessel perforations. Although a rare event, perforation of a branch of the external carotid artery can quickly lead to airway compromise if not recognized and treated promptly. An equally rare event is external carotid artery embolism to carotid ophthalmic collateral vessels, which may lead to retinal embolism and blindness. Anatomically, this can occur through several avenues: (1) middle meningeal and superficial temporal collateral vessels to the ophthalmic artery, (2) after internal carotid artery reconstitution, and (3) retrograde embolization through the external carotid artery to ophthalmic and retinal AorticorBrachiocephalicAccess In the process of obtaining access to the common carotid artery with a guide catheter, injury to any of the major aortic branches and brachiocephalic vessels can occur and result in dissection, thrombotic occlusion, embolic infarction, or a combination of these. Many patients with carotid stenosis have severe atherosclerotic debris throughout their arterial trees. Therefore, care should be taken in obtaining access to the proximal carotid artery. Minor, non­flow-limiting dissections of the proximal brachiocephalic vessels can be observed, regardless of whether the patient is receiving a heparin infusion. If this is a concern, but the decision is made not to place a stent in a dissection flap, repeated angiography within 24 hours may be indicated. If wire access is lost, a microcatheter and a soft wire may be used to locate the distal true lumen, with angiography performed via the microcatheter to ensure that the distal wire is indeed in the true lumen. If a sudden neurological change develops, the diagnosis should include hemorrhage and ischemia (most often caused by embolism). The surgeon should obtain rapid access to the vessel suspected of harboring the problem, according to the findings of the neurological examination. If loss of vision occurs, angiography should be conducted immediately to determine the location of the occlusion. Central retinal artery occlusions can sometimes be treated by direct infusion of antiplatelet agents into the ophthalmic artery. Chen and associates115 demonstrated a high rate of microemboli detection, particularly during distal filter deployment (>8000 microemboli) and stent deployment (>7000 microemboli). However, the use of a distal embolic protection device significantly lowered the frequency of Doppler study­detected microembolic signal. The indication or indications for proximal versus distal protection use have yet to be clearly defined. This suggests that proximal protection may be safer in patients with hypoechoic plaques. Dissection through the plaque with or without embolization is an inherent risk that is encountered when a carotid lesion is crossed. If the true lumen can be entered with the distal embolic protection device, it should be crossed and stented along with the stenosis. If the lesion cannot be crossed with the device, a microwire and a catheter should be used to cross the lesion. Then the true lumen can be identified with a microcatheter angiographic run, and the protection device may be brought into position. Contrast material remaining within the arterial wall should cause concern for dissection. For cases of small, asymptomatic, and non­flow-limiting dissections, clinical observation is recommended. Rapid access to the distal intracranial vasculature enables embolectomy or direct delivery of pharmacologic therapy, or both. In addition, as stated previously, if crossing a lesion continues to be difficult or impossible, the procedure should be reassessed and endarterectomy considered. Ideally, the filter should be deployed in a straight segment of the internal carotid artery distal to the stenosis and well opposed to the carotid wall. Most often, this occurs when the operator advances the wire but not the housing sheath of the distal embolic protection device.

Great care must be exercised in dissecting and preserving the lower division of the facial nerve asthmatic bronchitis bronchial asthma order montelukast 10 mg amex, which is generally the superior-most limit of the exposure asthma symptoms red cheeks cheap montelukast 4 mg fast delivery. To mobilize the hypoglossal nerve in the more superior dissection asthma treatment by fish montelukast 5 mg order line, the descendens branch should be cut asthma symptoms fever order 4 mg montelukast mastercard. Then the main nerve can be retracted anterior and superior to the primary tumor dissection asthma 68 4 mg montelukast purchase otc. Following the tumor resection, sequential closure of the deep muscle and fascia layers gives the best postoperative functional result. Patetsios and colleagues142 reported that in their 30 years of experience with carotid body paragangliomas (29 patients with 34 tumors), complications included two arterial thromboses (7%), five permanent cranial nerve deficits (17%), and one death (3%). Labile blood pressure, presumed to be secondary to baroceptor failure from loss of carotid sinus function, has been reported after resection of a carotid body tumor. Interestingly, first-bite pain occurred in 10 of 25 patients assessed postoperatively. Radiotherapy is not used as the sole primary treatment of carotid body tumors, but it is the preferred therapy in recurrent or residual tumor with intracranial extension. Radiosurgery may be useful for a metastasis or intracranial extension; however, no significant series of this relatively rare pathology has been reported to date. It has been shown that radiation therapy does not eradicate the tumor cells and probably exerts its major effects by inducing a vasculitis within tumor vessels. Carotid body tumors, inheritance, and a high incidence of associated cervical paragangliomas. Endovascular stent treatment of cervical internal carotid artery aneurysms with parent vessel preservation. Cervical artery dissection: clinical features, risk factors, therapy and outcome in 126 patients. Internal carotid artery dissection in a community: Rochester, Minnesota, 1987­1992. Mild hyperhomocysteinemia and low folate concentrations as risk factors for cervical artery dissection. Do extracellular-matrix regulating enzymes play a role in cervical artery dissection Spontaneous and endothelial-independent vasodilation are impaired in patients with spontaneous carotid dissection. Account of the first successful operation performed on the common carotid artery for aneurysm. Extracranial aneurysm of the internal carotid artery: history and analysis of the cases registered up to August 1, 1925. Endovascular stenting of extracranial carotid artery aneurysm: a systematic review. Results of surgical management of internal carotid artery aneurysm by the cervical approach. Fibromuscular dysplasia and other uncommon diseases of the cervical carotid artery. Non-atheromatous stenosis and occlusion of the internal carotid artery and its main branches. Fibromuscular dysplasia: neurological disorders associated with disease involving the great vessels in the neck. Fibromuscular dysplasia of the posterior cerebral artery: report of a case and review of the literature. Cerebral embolism from septal fibromuscular dysplasia of the common carotid artery. Spontaneous dissection of the internal carotid artery associated with fibromuscular dysplasia. Fibromuscular dysplasia: multiple "spontaneous" dissecting aneurysms of the major cervical arteries. Bilateral fibromuscular hyperplasia of the internal carotid arteries with aneurysm formation. Transluminal dilatation of internal carotid artery in fibromuscular dysplasia: a preliminary report. Fibromuscular dysplasia of the internal carotid artery: percutaneous transluminal angioplasty. Percutaneous transluminal carotid angioplasty in fibromuscular dysplasia: case report. Fibromuscular disease of carotid arteries: long term results of graduated internal dilatation. Percutaneous transluminal angioplasty in fibromuscular dysplasia of the internal carotid artery: one year clinical and morphological follow-up. Intimal dissection following percutaneous transluminal carotid angioplasty for fibromuscular dysplasia. Delayed cerebrovascular consequences of radiation to the neck: a clinicopathologic study of case. Carotid artery stenosis in patients who have undergone radiation therapy for head and neck malignancy. Carotid artery repair for radiation-associated atherosclerosis is a safe and durable procedure. Some morphological, histochemical, and chemical observations on chemodectomas and the normal carotid body, including a study of the chromaffin reaction and possible ganglion cell elements. Carotid chemodectoma: a clinical case report and review of the literature [Italian]. Catecholamine-secreting paraganglioma of glomus-jugulare region resembling pheochromocytoma. The influence of oxygen tension and pH on the expression of platelet-derived endothelial cell growth factor/thymidine phosphorylase in human breast tumor cells grown in vitro and in vivo. The presence of platelet-derived endothelial cell growth factor in human endometrium and its characteristic expression during the menstrual cycle and early gestational period. Clinical and pathological considerations of twenty tumors affecting nineteen patients (one bilateral). Chemodectoma (non-chromaffinic paraganglioma) of the carotid body with distant metastases. Soft tissue metastasis of a chemodectoma: a case report and review of the literature. Subsequently, in 1853, Maisonneuve successfully ligated the vertebral artery at the transverse foramen of the sixth cervical vertebra for a stab wound to the neck. In 1888, Matas was the first surgeon who fully excised an aneurysm between the occiput and the atlas through a posterior approach. The role of pathology of the extracranial vessels in relation to cerebral ischemia was emerging,5 and with this revolution in the diagnosis of diseased arteries, revasculariza tion was being performed. In 1958, Crawford and coworkers6 presented their results of surgical treatment of brainstem ische mia by reconstruction of the vertebral artery after removal of atherosclerotic plaque. The next year, Cate and Scott7 first described the technique of transsubclavian endarterectomy of the subclavianvertebral artery. Angiography allowed visualization of other causes of extracra nial vertebral artery disease, including extrinsic compression of the vertebral artery by osteophytes,8 constricting bands,9 and rotational obstruction,10 all of which were diagnosed and treated by surgical decompression. Angiography also provided the first extensive cooperative study of the incidence of extracranial arte rial stenosis caused by atherosclerotic lesions in patients with cerebrovascular insufficiency. In 1968, stenosis was proposed to be a compromised lumen of more than 50% by the Joint Study of Extracranial Arterial Occlusion. For the first time, this study provided a frequency distribution of sites of stenosis in the extracranial vertebral artery,11 identifying at least some degree of stenosis in 22% and 18% of left and right proximal vertebral arteries, respectively, and 5% to 6% of the distal extracranial vertebral artery. Today, posterior circulation stroke still accounts for 30% to 40% of all ischemic strokes, a subset of which is secondary to isolated extracranial vertebral artery disease. Treatment options have evolved to include endovascular options, although surgical intervention remains a valid option for extracranial vertebral artery revascularization in many instances. Most physicians use the presence of any two of the common symptoms to define the syndrome. The designation is helpful because the associated pathology can differ among the segments. First Vertebral Artery Segment (V1) the V1 extends from the superior portion of the subclavian artery to enter the transverse foramen of C6. Instead of arising from the superior portion of the left subclavian artery, the left vertebral artery can arise from the proximal sub clavian trunk. These symptoms are caused not only by embolic or thrombotic sources but also by hemodynamic mechanisms. As the vertebral artery enters the transverse foramen of C6, it ascends on a vertical path through the upper cervical foramen until it approaches C2. From the C2 transverse foramen, it courses slightly anteriorly to pass through the transverse foramen of C1. This segment can be extremely tortuous, which can make the endovascular placement of a stent in the distal extracranial vertebral artery difficult. Third Vertebral Artery Segment (V3) the V3 extends from where it exits the transverse foramen of C1 to its entry through the atlantooccipital membrane. Large or small vessel occlusive disease occurs more commonly in the posterior circulation than thromboembolism. Hemodynamic changes can also result sys temically from cardiac insufficiency or postural hypotension. The pathology that causes hemodynamic changes and the sources of emboli often overlap. Emboli or thrombi may originate from vertebral arteries, subclavian artery, aortic arches, patho logic heart valves, abnormal cardiac wall behavior, or arrhyth mias. Atherosclerotic plaque is usually the source of emboli or thrombi from the aorta or subclavian or vertebral arteries. Embolism arising from within or outside the vertebral system seeks the terminal branches of the basilar artery or the posterior cerebral arteries. Consequently, symptoms can manifest as simple cranial nerve palsies or brainstem vascular syndromes. Acute visual field defects or symptoms of occipital lobe infarction can also be pre senting symptoms. The underlying cause of extracranial vertebral disease can be separated into three primary diseases in which both mechanisms- hypoperfusion and emboli-can play a role in the following symptoms: atherosclerosis, dissection, and extrinsic compression. In addition, subclavian steal syndrome is a distinct entity that leads to hemodynamic impairment of the vertebral artery. Although it is a potential source of thromboembolic plaque, it can cause significant hypoperfusion by obstructing blood flow. One of the most extensive studies on the incidence of extracranial disease in symptomatic patients was presented by Hass and associates11 from the Joint Study of Extracranial Arterial Occlusion. They found that the most common site of plaque formation in the vertebrobasilar arterial system was the origin of the proximal vertebral artery (right vertebral artery, 18. In this region, it is believed that the blood flow is dampened as it passes through the transverse foramina. Atherosclerosis occurs less frequently intracranially, in the mid basilar artery, and at the entry of the vertebral artery through the dura. The natural history of extracranial vertebral artery atheroscle rotic disease is not well known. Moufarrij and associates reviewed 96 patients with vertebral disease, 89 (93%) of whom had proxi mal extracranial disease at the vertebral origin. Recurrent stroke risks in patients with symptomatic extracranial vertebral atherosclerosis have not been well defined, although estimates of stroke risk in patients with symptomatic intracranial vertebrobasilar disease are 10% to 15% per year. The extracranial vertebral artery is divided into the V1 segment, from its origin at the subclavian artery to its entry into the transverse foramen of C6; the V2 segment, from the transverse foramen of C6 to the transverse foramen of C1; and the V3 segment, from the exit from the transverse foramen of C1 to its entry through the dura. Vertebro basilar symptoms arise from interruption of the blood supply to the brain and brainstem. The interruption can be the result of hypoperfusion caused by hemodynamic changes or by thrombo embolic sources. Spontaneous dissec tions are associated with systemic diseases affecting the arterial walls. In both the carotid and vertebral arteries, fibromuscular dysplasia is the most common associated condition in spontane ous dissection. It tends to affect areas where there is significant movement of the cervical spine and therefore occurs in the middle and distal segments of the vertebral artery. The formation of pseudoaneurysms is also quite common, although these lesions are often asymptomatic. Angiogram showing retrograde vertebral blood flow in a patient with subclavian steal syndrome. As indicated, the right vertebral artery contrast injection flows retrograde into the left vertebral artery and into the subclavian artery, owing to the proximal stenosis in the subclavian artery. This type of injury can also be created iatro genically from chiropractic manipulation. The most frequent site of thrombosis is at the level of C2 in the distal vertebral artery. This tendency may reflect the posterior placement of the verte bral foramina with respect to the vertebral body. The vertebral artery has an increased vulnerability to compression by sublux ation of the cervical apophyseal joints. Subclavian Steal Syndrome Subclavian steal syndrome was first described by Reivich and colleagues36 in 1961 when they discovered reversed flow in the vertebral artery. It is caused by stenosis or occlusion in the subclavian or innominate artery proximal to the vertebral artery. Most of these symptoms are caused by use of the extremities when the demand for blood flow is increased and the pressure sink becomes more pronounced. Cardiac disease such as dys rhythmias, cardiac insufficiency, and infarction can result in poor cardiac output. The anterior scalene muscle has been found to compress the vertebral artery at the level of C6.

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Although it may seem natural to rest the back of a surgical instrument on the edge of the craniotomy or other surface asthmatic bronchitis home remedies order montelukast 10 mg amex, this practice should be avoided asthma definition bts purchase montelukast 10 mg on-line. It is preferable to rest a hand on the drapes and maintain the instrument free of contact except at the surgical interface asthmatic bronchitis risk factors order cheap montelukast on line. This provides tactile feedback that is helpful in minimizing force applied to the surgical bed as well as adjacent tissue asthma fef25 75 montelukast 5 mg buy mastercard. Gentle maneuvers limit direct tissue trauma and prevent the transmission of forces from a heavy-handed dissection within the cavity asthma treatment 4 hives montelukast 5 mg purchase on line. Meticulous hemostasis prevents postoperative hemorrhage, but also allows for better postoperative imaging of the lesion. Postsurgical blood products in the resection cavity can mimic residual cavernous malformation. Careful side-by-side comparison of preoperative and postoperative imaging should be executed before considering reoperation for residual lesion. Most supratentorial craniotomies can simply be replaced after primary dural repair. Surgical Approaches As skull base surgery has evolved, there has been an appropriate abandonment of procedures with the highest rates of morbidity, such as transpetrosal approaches. The large majority of posterior fossa cavernomas can be accessed via one of the following standard approaches or their variations: retrosigmoid, far lateral, midline suboccipital, telovelar, orbitozygomatic, and supracerebellar-infratentorial. Supratentorial deep-seated lesions are accessible using an orbitozygomatic, interhemispheric transcallosal, or supracerebellar transtentorial approach. Patients with thin habitus and flexible necks can be maintained supine with a small shoulder roll and have their head turned and flexed. We prefer a park bench position to avoid neurovascular compromise from turning the head laterally and for surgical access to the field. A linear skin incision is placed two fingerbreadths, or approximately 4 cm, behind the tip of the mastoid and is extended approximately 2 cm above a line connecting the root of the zygoma to the inion. This line, the superior nuchal line, estimates the location of the transverse sinus. It is our practice to carry sharp dissection down to the fascial layer and elevate a few millimeters of scalp anteriorly and posteriorly in the suprafascial plane. Once the muscle is divided, a subperiosteal elevation of the musculocutaneous flaps is performed and self-retaining retraction is placed. The posterior portion of the mastoid and the beginning of the digastric groove should be exposed as well as the asterion. The craniectomy is then extended with rongeurs or a diamond bur until the transverse sigmoid junction and the posterior edge of the sigmoid sinus are visualized. The dura can then be opened in a curvilinear fashion based on the posterior margin of the sigmoid sinus. The dural flap is then deflected with sutures to keep it out of view and to help prevent it from drying and shrinking during the procedure. We typically dissect inferiorly toward the foramen magnum using a neurosurgical cotton patty to protect the cerebellar hemisphere and sharply open the arachnoid of cisterna magna. Once the cerebellar hemisphere has relaxed, the eleventh cranial nerve is usually visible as it passes through the foramen magnum and can be followed cranially to the tenth and ninth nerves. Gentle retraction on the flocculus and choroid plexus exiting the foramen of Luschka will allow the arachnoid over the seventh and eighth cranial nerves to be identified. Looking in a more cranial direction, the petrotentorial junction can be identified. Gentle dissection along the junction will allow visualization of the arachnoid over the superior petrosal vein. Dissection more medially allows the surgeon to visualize the fourth cranial nerve along the edge of the tentorium. Pathology located in the pons, lateral cerebellar hemisphere, cerebellopontine angle, and pontomedullary junction can be accessed via this approach. In order to expose the lateral surface of the middle cerebellar peduncle, the superior limb of the cerebellopontine fissure must be identified and the arachnoid of the fissure opened sharply. Care should be taken to preserve the vein of the superior cerebellopontine fissure while opening the arachnoid widely to expose the surface of the middle cerebellar peduncle. The anterior inferior cerebellar artery typically passes between the facial and vestibulocochlear nerves and then runs along the surface of the middle cerebellar peduncle, providing another anatomic landmark. Once the middle cerebellar peduncle is exposed, intraoperative navigation can be used to assist in identifying an optimal location for a corticotomy. Closure begins with meticulous hemostasis and ensuring removal of all neurosurgical cotton patties. Dural suture is then passed through the dural edge, through the suturable DuraGen, and then through the edge of the dural opening. This creates a sandwich effect and holds the dural graft firmly up against the intradural edges of the durotomy. A thin layer of fibrin glue is then applied to the suture line and a piece of dry noncompressed Gelfoam is placed in the epidural space. A skull base plate or titanium mesh is cut to fit and secured to the edges of the craniectomy with self-tapping screws. If the leak resumes after clamping the drain, then a wound revision and/or lumboperitoneal or ventriculoperitoneal shunt should be considered depending on the clinical situation. Far-LateralApproach the far-lateral approach provides the surgeon with an anterolateral trajectory to the brainstem and eliminates the need to traverse contaminated mucosal structures through the transoral or transfacial route. First described by Heros, the far-lateral approach provides access to the vertebrobasilar junction, inferolateral pons, anterolateral medulla, and upper cervical spinal cord. Several different positions can be used for this approach including park bench, sitting, lateral decubitus, and supine with the head rotated. It is our practice to use the park bench position to optimize exposure, minimize the need to rotate the head, and maximize surgeon comfort. Once the patient is in park bench position, the head is flexed and turned slightly to the contralateral side, the vertex is tilted toward the floor, and gentle traction is used to increase the interval between the foramen magnum and the ring of C1. The ipsilateral shoulder should be rolled forward and can be held down with tape to prevent movement when the table is adjusted. Intraoperative monitoring should be used to evaluate before and after positioning to help avoid nerve injury caused by positioning. Many different skin incisions have been described for the farlateral approach, including the hockey-stick, curvilinear paramedian, and linear paramedian. Currently, it is our practice to use a paramedian linear incision almost universally for our far-lateral approaches. This reduces the bulk of the musculocutaneous flap that limited the anterolateral line of site experienced with the old hockey-stick incision. However, the paramedian incision requires early identification of the vertebral artery within the suboccipital triangle. Layer-by-layer muscular dissection provides the surgeon with anatomic landmarks necessary to safely expose the horizontal segment of the vertebral artery as it passes over the ring of C1 and penetrates the dura and the C1 nerve root. The posterior arch of C1 is then thinned out with a diamond drill and removed with a rongeur. Removal of the bone can be taken laterally to the foramen transversarium, which can be opened, allowing the transposition of the vertebral artery if necessary. The occipitoatlantal membrane is then dissected off the foramen magnum to allow for a suboccipital craniotomy. A trephine is placed superior and medially in the exposure so that it is beneath the ipsilateral transverse sinus and over the cerebellar hemisphere. A lateral suboccipital craniotomy can then be completed through the foramen magnum. A combination of rongeur and diamond drill can be used to extend the craniotomy to the posterior edge of the sigmoid sinus superiorly. The foramen magnum should be drilled laterally to include the posteromedial third of the occipital condyle. A safe technique for drilling the condyle is to start within the condyle and slowly expand the bony removal, leaving a thin "eggshell" of bone at the margins. The condylar emissary vein is the anatomic landmark for the anterior border of bony removal. If injury to the vein occurs, it is easily dealt with using topical hemostatics, bipolar cautery, or bone wax. The hypoglossal canal is anterior and medial to the anterior third of the condyle and should not be visualized during this stage of the exposure. The dura can then be opened sharply in a curvilinear fashion with the flap based laterally. The upper cervical spinal cord, medulla, lower cranial nerves, and cerebellar tonsil can then be visualized. On opening of the arachnoid and mobilization of the cerebellum, the tonsillar segment of the posterior inferior cerebellar artery can be identified and followed back to the vertebral artery. The vertebral artery can be visualized cranially up to the vertebrobasilar junction in some cases. Special attention should be paid to the vasculature when dealing with pathology at the cervicomedullary junction. Injury to arterial contributors to the anterior spinal artery can have dramatic consequences. This approach will give access to the vertebrobasilar junction, inferolateral pons, anterolateral medulla, and upper cervical spinal cord. Closure is performed in a similar manner to that described earlier for the retrosigmoid approach. It is more common with a far-lateral approach to have a craniotomy large enough to plate back to the skull versus using a skull-based plate or mesh. However, the sitting position risks venous air embolism and is uncomfortable for the surgeon. It is our practice to use the park bench position for the majority of our supracerebellar infratentorial approaches. A military chin tuck is useful when positioning patients for this approach because it places the tentorium at a more favorable angle while translating the head posteriorly and clear of the shoulders. Intraoperative navigation is recommended and can be used before preparing the patient to confirm a favorable angle of the tentorium and to landmark the transverse sinuses and torcula. The craniotomy should be planned to cross the sinuses and torcula to allow for superior retraction of the tentorium. A linear incision extending just above the inion down to the spinous process of C2 is typically sufficient for this approach. The surgeon must be wary of aggressive retraction, which can risk occlusion of the sinuses. Once the dura is open, the microscope should be angled cranially as far as possible to visualize the superior surface of the cerebellum without significant retraction. Midline bridging veins from the superior surface of the cerebellum to the dural sinuses should be coagulated and divided at the cerebellar surface. Aggressive early retraction risks avulsion from the dural sinus and severe venous hemorrhage. In the case of dural hemorrhage, a piece of Gelfoam that is oversized compared with the dural violation should be placed over the defect and compressed with a cotton pad until hemostasis is obtained. Direct coagulation is not recommended, and it can result in dural retraction increasing the violation into the sinus. Dissection along the superior surface of the cerebellum will lead to the arachnoid over the tectum and the deep venous system. This arachnoid is typically relatively thick and should be divided sharply, close to the cerebellar surface. Once the arachnoid is opened, the bilateral internal cerebral and basal veins of Rosenthal can be identified. With identification and protection of the deep venous system, the cavernous malformation can then be resected. Variations of this approach include the lateral supracerebellar infratentorial and the supracerebellar transtentorial approaches. The paramedian approach also avoids the majority of the tentorial bridging veins, which are typically clustered along the midline. The transtentorial variation is performed by incising the tentorium parallel to the straight sinus. This can provide access to the inferior and mesial cortical surfaces of the temporal lobe, the posterior thalamus, and the rostral mesencephalon. These approaches can extend rather deeply, and long bipolar cautery, microdissectors, and forceps should be available. Lighted suction tips can also be advantageous for surgical visualization in these deep exposures. The 6 thalamic regions: surgical approaches to thalamic cavernous malformations, operative results, and clinical outcomes. When considering periventricular cavernous malformations, a variation on the two-point method can be applied, substituting the ependymal surface for the pia. More laterally located malformations may benefit from a contralateral interhemispheric approach. This allows for a more lateral trajectory without requiring significant hemispheric retraction. Our preference is to position the patient supine with a shoulder roll to allow the head to be turned so that the superior sagittal sinus is almost parallel with the floor. The vertex is then angled superiorly and, in the case of an anterior interhemispheric approach, the neck is slightly extended. Frameless stereotactic navigation can then be registered and used to assist in the planning of the incision and craniotomy. Veins draining into the sagittal sinus should be identified on preoperative imaging and avoided whenever possible.

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References

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