James C. Carr, MD, FFR RCSI

• Associate Professor of Radiology and Medicine
• Northwestern University Feinberg School of Medicine
• Director of Cardiovascular Imaging
• Northwestern Memorial Hospital
• Chicago, Illinois

In twothirds of patients women's health center murfreesboro tn purchase discount serophene on-line, the disease remits pregnancy xmas ornaments purchase 100 mg serophene mastercard, usually within 2 years menstruation tent purchase serophene 50mg online, the majority requiring no treatment pregnancy quad screen discount serophene online american express. Joints can develop tenosynovial hypertrophy with little pain or restriction in the early stages menopause belly fat generic serophene 25mg with mastercard. Renal involvement is not uncommon with interstitial nephritis and nephrocalcinosis women's health during pregnancy purchase serophene online now. Meningoencephalitis, which may present with seizures, is more common in childhood sarcoidosis and in those with posterior segment inflammation. Ocular features There is a painful, bilateral panuveitis with granulomatous anterior uveitis. Serous retinal detachments occur, Ocular features Anterior uveitis may start with pain and redness and become chronic and painless. Optic disc swelling is frequent, caused by a granulomatous optic neuropathy, raised intracranial pressure, or secondary to uveitis. As young children with sarcoid may present with uveitis, disease may be very advanced at presentation. Uveitis frequently requires more prolonged and intensive immunosuppression than extraocular sarcoid to obtain clinical remission. Inflammatory bowel disease Crohn disease and ulcerative colitis are associated with ocular inflammation. Renal disease consists of an eosinophilic and mononuclear infiltrate, but granulomas can also be found in the lymph nodes and marrow. Severity ranges from transient rashes and fevers to persistent inflammation with renal amyloidosis, deafness, chronic uveitis, and arthropathy. Familial granulomatous disease Blau syndrome (familial) and early-onset sarcoidosis (sporadic) are sometimes grouped as "pediatric granulomatous arthritis. The type of mutation may influence the severity of ocular involvement in Blau syndrome. Uveitis and non-vasculitic neurological disease Multiple sclerosis this is rare in childhood. Rasmussen syndrome this is a chronic, unilateral encephalitis associated with an ipsilateral chronic uveitis that may present in childhood. The arthritis is a polyarticular granulomatous tenosynovitis, with mean onset at 2 years and commonly involves wrists and fingers. There are also dysplastic joint changes including giant synovial cysts and camptodactyly. Systemic autoinflammatory disorders and familial granulomatous diseases Several familial disorders present with periodic fevers, urticarial rashes, and joint and ocular inflammation. Single-gene disorders of inflammation, like some vasculitides, may be associated with very localized disease. Cryopyrin-associated periodic fever syndromes these immune disorders have a prevalence of 3 per million. Subepithelial corneal opacities and peripapillary atrophy have been reported to be diagnostic but also occur in sarcoidosis. The disease can be severe: half require immunosuppression and 14% develop severe visual loss. Granulomatous infection such as tuberculosis must always be considered in the differential diagnosis. They can be life-threatening diseases that present with ophthalmic signs and sight-threatening disease. Their treatment may require prolonged immunosuppression, which may result in drug-associated ocular complications. Some infections may predispose to vasculitis such as streptoccocal and hepatitis B infections. Some conditions are associated with highly specific autoantibodies and their testing methods continually develop (Table 40. Secondary vasculitides may occur with connective tissue disorders, autoinflammatory diseases, and immunodeficiencies. As fluorescein angiography and reliable static perimetry is often not possible in small children, previous vascular occlusions may only be detected by signs of focal retinal thinning on optical coherence tomography. Ocular involvement in vasculitis There are few diagnostic ocular signs as the pathologic processes may be common to different vasculitis syndromes. In adults, prolonged uveitis accompanying vasculitis is most uncommon: ocular involvement secondary to vasculitis is usually an inflammatory or ischemic optic neuropathy, sclerokeratitis, or orbital inflammation. Episcleritis is more likely to be accompanied by systemic disease in children than in adults; in contrast, most childhood posterior scleritis is idiopathic. Bilateral inflammatory signs of severe systemic disturbance: conjunctivitis, episcleritis, scleritis, anterior uveitis, retinopathy, and optic disc edema and papilloedema. Localized ocular vasculitis: varying with vessel size: peripheral ulcerative keratitis, scleritis, episcleritis, retinal vasculitis, choroidopathy (and central serous retinopathy), optic neuropathy, and orbital inflammation. Focal, necrotizing scleritis with adjacent keratitis is the most common presentation. It is 100 times more common in East Asia, nine times more common in females and, in one-third, starts before the age of 20. Phases of systemic inflammation may precede chronic occlusive vasculopathy by several years. It has been associated with uveitis, scleritis, and idiopathic intracranial hypertension. Diagnosis requires tissue biopsy or abdominal angiography when the clinical picture is unclear. It can be secondary to infections such as hepatitis and streptococci as well as neoplasia. Ocular Conjunctivitis, episcleritis and necrotizing scleritis, and peripheral ulcerative keratitis are found in up to 20% of patients. Choroidal vasculitis is a common histologic change but is usually asymptomatic; retinal involvement is usually an arteriolitis but veins may also be involved. Lupus retinopathy is a sign of severe systemic vasculopathy and may be complicated by hypertensive changes. Later, 20% develop coronary artery aneurysms, some of which may resolve with treatment, but there may be longterm cardiac sequelae. Localized scleroderma is more common in young females and may involve isolated patches of skin (morphea) or a linear patch on the face (en coup de sabre). Choroidopathy is relatively common, arising from choroidal capillary closure and perivascular mucopolysaccharide deposition. Extramuscular features are more common in children, including subcutaneous calcification and vasculitis. One-quarter of patients have other connective tissue disease, particularly rheumatoid arthritis. Episcleritis and scleritis are the most frequent patterns, with keratitis, uveitis, and retinal vasculitis occurring rarely. There may be an autoantigen shared between the cornea, large vessels, and the inner ear. Pediatric inflammatory brain disease and localized vasculitis Pediatric inflammatory brain disease may cause focal neurological events such as stroke or diffuse inflammation 398 presenting with encephalitis: there is a large differential diagnosis which includes many conditions causing ocular inflammation and systemic vasculitis. Visual disturbance is more commonly associated with encephalopathic and vasculitic disorders. It is a granulomatous and necrotizing vasculitis and there may be paravascular demyelination. Systemic treatment: indications At some point, the risks of cataract and glaucoma with continuous topical steroid treatment are sufficiently high to justify systemic immunosuppression if prolonged remission is never achieved. The aim of treatment is to achieve a sufficiently long remission that withdrawal of treatment is not followed by an early recurrence. It may be best to achieve 3 years of remission before tailing off treatment in those who are at most risk. Complex, frequent regimens of drops are to be avoided: compliance is greatly reduced particularly at school. It is preferable to use a single steroid and vary its frequency rather than swap between steroid preparations. If needed more than four times a day, then a different route of steroid use is indicated. The aim of treatment is to minimize the acute risk of damage at times of high activity, achieve remission, and maintain this for a sufficient length of time to reduce the risk of short-term relapse. Azathioprine,73 everolimus,74 ciclosporin,75 and tacrolimus76 have all shown efficacy in childhood and adult uveitis. It remains unclear whether dual therapy with conventional immunosuppressives adds benefit, and treatment switching of single agents ought to be considered before using multiple therapies, which greatly increase drug toxicity and complications. The choice of biologic is likely to be determined largely by cost and regulatory restrictions rather than high-level clinical evidence. Indications of treatment efficacy the ocular signs relevant to titrate treatment remain unclear in all forms of uveitis although cell counts and vitreous flare are both central to drug trials. There may also be an entirely different set of measures relevant to assessing treatment response in those already under treatment, i. As complete remission is associated with no further inflammatory complications, this it is the aim of treatment. Visual loss is associated with surgery for cataract, glaucoma, as well as hypotony, so treatment should be aimed at preventing these conditions. Biologics Biologic agents are monoclonal antibodies that bind specific molecular targets, in contrast to conventional immunosuppressants. Their use in treating inflammatory disease first developed in adult inflammatory arthritis. The use of many biologics has been reported in pediatric uveitis; recent reviews rely on case series only and do not indicate a clearly preferred class specifically more efficacious in the various uveitis types. Since response of all treatments is likely to be related to the level of ocular damage at time of treatment initiation, the timing of treatment escalation is critical. Children with mild bilateral cataracts and persistent inflammation can undergo a normal education with 6/18 vision and surgery might be deferred for long periods, until disease activity subsides. Surgery on a unilateral cataract during the first years of life may often result in an aphakic amblyopic eye that adds little to the visual function, but also allows monitoring of posterior segment disease, which, if untreated, may result in an unsightly painful eye. Disease activity must be rigorously controlled, as postoperative inflammation can be unpredictable and severe. When cataract surgery is planned, patients should be started on systemic immunosuppression in order to see whether disease can be completely controlled without topical medication. Treatment should be increased before, at the time of surgery, and for at References least 2 months after. Some patients with preoperative macular edema require 4 or 5 months of systemic steroids as well as second-line immunosuppression before maximum postoperative acuity is achieved. When planning surgery in cases with a short history, one should assume that inflammation may become more difficult to control for some years afterwards. Periocular or intraocular steroids given intraoperatively reduce the need for systemic steroid in the perioperative period but increase the risk of glaucoma. Posterior synechiae can be more extensive than is apparent on slit-lamp examination and intraoperative iris damage is frequent. Pupillary membranes may be vascularized and bleed intraoperatively but occasionally may be simply peeled off the anterior capsule. Techniques of lens removal depend on the possibility of capsulorrhexis, visibility, and hardness of the lens. Occasionally, calcified subcapsular plaques and pigment clumps related to posterior synechiae complicate capsulorrhexis. Patients and their parents should be counseled preoperatively about the need for vigorous patching after surgery. Treatment of glaucoma Many glaucoma surgical treatments have been tried including goniotomy and cycloablation. The use of tube drainage devices has greatly improved the prognosis of aphakic glaucoma. Steroid-induced glaucoma in a quiet eye may respond well to conventional assisted trabeculectomy. Whatever the success of angle surgery and cyclodestructive procedures in the short-term, they may reduce the effectiveness of later drainage procedures. Proposed outcome measures for prospective clinical trials in juvenile idiopathic arthritis-associated uveitis: a consensus effort from the multinational interdisciplinary working group for uveitis in childhood. Visual loss associated with pediatric uveitis in English primary and referral centers. Characteristics of childhood uveitis leading to visual impairment and blindness in the Netherlands. Intermediate uveitis in children and young adults: differences in clinical course, associations and visual outcome.

Oral corticosteroid use is effective for cutaneous hemangiomas: an evidence-based evaluation women's health quinoa salad order serophene 100mg on-line. Pharmacologic therapy for periocular infantile hemangiomas: a review of the literature breast cancer grades buy serophene. Treatment of cutaneous hemangiomas by the flashlamp-pumped pulsed dye laser: prospective analysis menstruation 8 weeks postpartum best order serophene. Early surgical intervention as definitive treatment for ocular adnexal capillary haemangioma womens health unc purchase 50 mg serophene with mastercard. Severe hypothyroidism caused by type 3 iodothyronine deiodinase in infantile hemangiomas womens health 8 veggie burgers cheap serophene 50 mg otc. Prospective study of the frequency of hepatic hemangiomas in infants with multiple cutaneous infantile hemangiomas current women's health policy issues cheap 100 mg serophene. Efficacy of propranolol in hepatic infantile hemangiomas with diffuse neonatal hemangiomatosis. Orbital-adnexal lymphangiomas: a spectrum of hemodynamically isolated vascular hamartomas. Complications of intralesional bleomycin in the treatment of orbital lymphatic malformations. Congenital orbital vascular malformation complicated by massive retrobulbar hemorrhage. Update on Sturge Weber Syndrome: diagnosis, treatment, quantitative measures, and controversies. Basal tearing is present in infants from birth, and reflex tearing begins at any time from birth to several months of age. The majority of the gland lies within this fossa, but the lateral horn of the levator palpebrae superioris separates this orbital part from the palpebral lobe, which extends anteriorly into the superotemporal conjunctival cul-de-sac. The ducts of the gland pass through the palpebral lobe and open on to the conjunctiva in the superior fornix. The lacrimal gland is innervated via the facial (afferent) and trigeminal (efferent) nerves. The accessory glands of Kraus and Wolfring sit in the superior conjunctival fornix. Introduction the lacrimal system consists of a secretory portion and a drainage system. With the meibomian glands and the goblet cells, they secrete the components of the tear film. The tear film has three layers: the inner mucin layer secreted by the conjunctival goblet cells, the intermediate aqueous layer secreted by the lacrimal and accessory lacrimal glands, and the outer, oily layer secreted by the meibomian glands. The accessory lacrimal glands produce basal tear secretion; the lacrimal gland is responsible for reflex tearing in response to noxious or emotional stimuli. The drainage system consists of the lacrimal puncta, canaliculi, lacrimal sac, and the nasolacrimal duct. This active system pumps tears from the conjunctival sac into the inferior meatus of the nose. Tears protect the eye by surface lubrication, provision of oxygen and antibacterial substances such as immunoglobulin (Ig)A, IgG, and lysozyme, and mechanical removal of irritating substances and cellular debris. Lacrimal problems in children usually relate to underproduction of tears, causing dry eyes, which is rare but potentially sight threatening, or reduced drainage of tears, which is much more common but less serious (Box 21. Anomalous lacrimal ductules that secrete tears on to the skin rather than the conjunctival sac may be found near the lacrimal gland, around the lateral canthus, or in the preauricular region. A drainage system may not be present in such cases and an enlarging orbital mass may develop. Crocodile tears (see Chapter 95) occur from congenital aberrant innervation between the fifth and seventh cranial nerves causing tearing with chewing or sucking. It may be due to absence of the lacrimal gland or to it being ectopic in the orbit. Lacrimalgland Embryology the lacrimal gland develops from ectoderm that is supported by mesodermal connective tissue. Dacryoadenitis Dacryoadenitis is usually associated with viral infection, mumps, infectious mononucleosis, herpes zoster, tuberculosis, brucellosis, histoplasmosis, or gonococcal infection. A primary clinical feature of dacryoadenitis is the "S sign" in which there is drooping of the lateral aspect of the upper lid. In the long term, dacryoadenitis may damage the lacrimal gland and cause reduced tear secretion. Dacryoadenitis must be differentiated from a lacrimal gland infarct, which occurs in children with a sickle cell crisis. Drainage failure (epiphora) Congenital nasolacrimal duct obstruction Skeletal and sinus abnormalities Lid malposition Punctal malposition Punctal occlusion Anomalous drainage system Acquired causes Acquired tear deficiency may be due to pathology of the lacrimal gland, causing failure of tear production, or to conjunctival damage (see Chapter 31), leading to ductule obliteration. It can be a primary autoimmune event or associated with rheumatoid arthritis or systemic lupus erythematosus. The associated peripheral corneal vascularization and scarring can be sight threatening. A reduced tear meniscus is evident with punctate keratopathy, particularly affecting the interpalpebral zone. Treatment of dry eyes involves copious use of artificial tears and temporary or permanent punctal occlusion in severe cases. Immunomodulation may have a role to play in secondary lacrimal gland failure, including that due to infections. Blepharitis should be treated with lid hygiene, lubricants, and Lacrimal tumors (see Chapter 26) Lacrimal tumors are very rare in children. Prolapse of the lacrimal gland, which is commonly bilateral, may present as a subconjunctival mass in the upper outer fornix. This may occur with craniofacial anomalies (see Chapter 28) due to reduced orbital volume and increased orbital pressure. Thelacrimaldrainagesystem Embryology the lacrimal outflow system develops between the maxilla and the lateral nasal process from surface ectoderm. The nasolacrimal duct opens into the inferior meatus of the nose just before or after term birth. There may be a failure of this canalization process at any part of the system, but this is most frequent at the lower end. The puncta should be in contact with the globe at the medial aspect of the upper and lower lids. The proximal part of the canaliculus, the ampulla, is a slightly dilated vertical portion 1 mm in length in the young child. The upper and lower canaliculi join to form the common canaliculus that enters the lateral wall of the lacrimal sac. The lacrimal sac extends superiorly under the medial canthal ligament to form its fundus. The nasolacrimal duct exits from the lower end of the sac and passes in a downward, lateral, and slightly posterior direction. This duct is surrounded by bone in its upper part but becomes membranous inferiorly. The nasolacrimal duct opens into the medial wall of the inferior meatus of the nose via the valve of Hasner. This ostium is found under the inferior turbinate of the nose, approximately 1 cm directly behind the entrance of the nose in the baby. During blinking, when the lids close, the canaliculi are shortened and narrowed by contraction of the pretarsal orbicularis muscles. Simultaneously, the same muscles pull the lateral sac wall, creating negative pressure inside the sac, sucking fluid into the expanded sac. Further lid closure causes contraction of the orbicularis oculi muscle, which squeezes the tears from the sac into the nasolacrimal duct. This causes a vacuum within the system into which tears enter via the puncta, and the cycle begins again. A Congenital abnormalities Common abnormalities, include narrowing (stenosis), blockage (atresia), complete absence (agenesis), or duplication (accessory channels) of any part of the system. A membranous obstruction at the distal end of the nasolacrimal duct is the commonest abnormality, causing congenital nasolacrimal duct obstruction. Children with craniofacial abnormalities (see Chapter 28), particularly clefting syndromes, have complex anomalies of the lacrimal outflow system that may involve large areas being either blocked or absent. Congenital dacryocystocele must be differentiated from a meningoencephalocele, a meningocele, a midline nasal dermoid cyst (see Chapter 29), or an infantile hemangioma (see Chapter 20). Treatment of a dacryocystocele involves observation during the first 2 weeks of life, during which time most spontaneously improve. Massage over the lacrimal sac may be undertaken to encourage rupture of the distal cyst into the nasal cavity. If acute dacryocystitis develops, intravenous antibiotics should be given prior to surgery. Congenital nasolacrimal duct obstruction Congenital nasolacrimal duct obstruction results from delayed maturation of the lacrimal system where it enters the nose, resulting in a persistent membranous obstruction at the valve of Hasner. The diagnosis is made on a history of a watery eye that has been present from the first few weeks of birth. Some children develop a the lacrimal drainage system mucopurulent discharge that may be constant or intermittent. The eye remains "white" without evidence of active infection, although conjunctivitis may complicate the condition. Percentage of eyes still unresolved There is an increased tear meniscus and there may be stickiness or crusting on the lashes. A fluorescein disappearance test should be performed on children with epiphora to determine if there is evidence of lacrimal outflow obstruction. The tear meniscus is evaluated at 2 and 5 minutes (and at 10 minutes in equivocal cases). Parental education should provide reassurance and information about the etiology and natural history. Cleansing the lids and lashes with cooled boiled water, gently expressing the contents of the lacrimal sac proximally into the conjunctival sac,18 maintains flow in the system and prevents stagnation, reducing any sticky discharge. Massage of the sac may also increase hydrostatic pressure within the lacrimal system. Vaseline (or liquid paraffin) should be applied to the periocular skin to protect and treat any areas of redness or broken skin. The lacrimal drainage system Antibiotics are not required and should be avoided unless there is evidence of conjunctivitis. Swabs for bacterial growth should only be performed under these conditions, as "pathogenic" bacteria are frequently commensals in the conjunctival sacs of normal infants and do not require antibiotic treatment in white watering eyes. In the past, probing was advocated at presentation or after a short period of conservative treatment. It has been shown that the earlier probing is performed, the greater is the success rate. This reduces the success rate of probing in older children and increases the requirement for more extensive surgery. In the small number of cases that remain symptomatic, the cause of failure can be identified and a management plan formed. This can only be achieved under general anesthesia using a laryngeal mask following full nasal preparation. Probing should be carried out in a step-wise fashion, identifying the patency or obstruction of each area between the puncta and end of the nasolacrimal duct. Probing is a blind procedure and depends on awareness of resistance to the probe as it passes through the system. The use of a nasal endoscope permits direct visualization of the lower end of the nasolacrimal duct, which assists in the diagnosis and management. This is easier if the lid is held taut by pulling the eyelid laterally to straighten out the canaliculus. The lacrimal system should be syringed with fluorescein-stained saline using a disposable cannula. Syringing takes place via each punctum and any areas of resistance or any regurgitation of fluid or mucus are noted. The inside of the nose should be inspected with an endoscope or fluorescein retrieved via a nasal or pharyngeal aspirate. The passage of fluorescein and the amount of resistance are important factors in deciding the site and nature of any abnormality of the lacrimal outflow system. If a nasal endoscope is used, it should be introduced into the inferior meatus after infracture of the inferior turbinate. This maneuver is often therapeutic as it opens up a narrow inferior meatus and may stretch a stenotic ostium. The system should then be probed via the upper canaliculus, as this is anatomically favorable for the onward, downward passage of the probe into the nasolacrimal duct and therefore may be less likely to result in canalicular damage. The probe is passed in the same manner as the dilator until it reaches a hard stop that indicates the medial wall of the sac.

Esophageal air trapping seen on lateral chest X-ray is highly suggestive of esophageal obstruction breast cancer october order serophene 50 mg free shipping. The patient holds their own tongue in their fingers with a piece of gauze wrapped around the tongue that allows for better grip pregnancy risks over 40 purchase serophene with paypal. Food boluses sometimes need to be broken into a number of pieces to facilitate removal womens health usa 2012-06pdf order serophene with american express. Edema and mucosal tearing should be treated with intravenous corticosteroids to reduce swelling womens health jackson ms buy generic serophene pills. Analgesia should be prescribed and the patient warned that if symptoms worsen or they become unwell menstrual jokes order serophene 25mg with amex, then they should return to the hospital as objects can become completely embedded in the tissue and therefore not visible menopause vs pregnancy symptoms order serophene 50 mg online. These symptoms and signs indicate the need for further investigation and general or cardiothoracic surgery consultation. Frequency of esophageal foreign bodies and their site of impaction in patients presenting with foreign body aerodigestive tract. Factors predictive of risk for complications in patients with oesophageal foreign bodies. Penetration refers to the entry of swallowed material into the endolarynx, but not below the level of the vocal folds. Aspiration is an important symptom to elicit from a patient, because aspiration may result in life-threatening respiratory complications, such as laryngospasm, bronchospasm, tracheobronchitis, and recurrent pneumonia. A multidisciplinary approach for the evaluation and treatment of patients with suspected aspiration is optimal, as there are many possible etiologies and several Chapter 50: Aspiration complementary approaches to treatment. Aspiration after the pharyngeal phase may occur either during the esophageal phase of swallowing or even after this. This is usually the result of retained material in the hypopharynx that enters the lower airway when a breath is taken after the swallow, or occurs with the regurgitation of material from the esophagus or from a pharyngeal pouch. The oral phase is further divided into two parts: the oral preparatory and the oral propulsive stages, both of which are under voluntary control. The pharyngeal and esophageal phases are the involuntary or reflexive stages of swallowing. For a safe and efficient swallow, precise coordination of the activity of all involved structures by the neurological system is crucial. A detailed description of swallowing physiology is outside the scope of this chapter; however, it is useful to review the mechanisms that protect the lower airway thus preventing aspiration. The larynx moves superiorly and anteriorly, and is then tucked under the base of the tongue. Etiology the varied etiologies of aspiration may be classified according to (a) the site of the dysfunction (oral cavity/ pharynx or esophagus) and (b) the type of dysfunction (structural or neuromuscular). Aspiration is more common in the elderly, secondary to age related deterioration of nerve and muscle function (Clave, et al. Clinical Presentation Patients with aspiration may present with a variety of symptoms, the most obvious of which is coughing or choking when swallowing or soon after swallowing. However, it is important to be aware that some patients do not cough after aspirating. This phenomenon is referred to as silent aspiration (Ramsey, Smithard and Kalra, 2005). These patients may present with recurrent lower respiratory infections and they require evaluation to exclude aspiration as a cause for their infections. These assessments are preferable to relying on clinical evaluation, such as a bedside swallowing assessment, which is often unreliable (Ramsey, Smithard and Kalra, 2006). The most common signs looked for during a bedside swallowing assessment are the height of laryngeal excursion, a cough or a wet voice. However, the patient with silent aspiration will not demonstrate these signs, and so a bedside assessment cannot exclude aspiration. Aspiration may occur at three times during swallowing: during the oral phase, during the pharyngeal phase, and after the pharyngeal phase (sometimes after the swallow has been completed). Aspiration during the oral phase of swallowing is usually caused by the premature spillage of food or fluid into the pharynx owing to poor control of the bolus by the tongue. This results in the bolus encountering the larynx before it has prepared for the transit of the bolus through the pharynx. Aspiration during the pharyngeal phase of swallowing is caused by 450 Section 2: Laryngology Table 50. Additional investigations may be necessary depending on the findings of the initial assessment. Neuromuscular disease Myaesthenia Gravis Muscular dystrophy Poliomyelitis Myopathies Presbyphagia History the symptoms of aspiration have been described previously. The characteristics of each symptom (including duration, onset, and progression) should be described. Of particular importance are: (a) the timing of the symptoms during eating or drinking: during the oral phase, during the pharyngeal phase ("the swallow"), or after the pharyngeal phase and (b) what consistency of material-food or fluid-is aspirated. Understanding the timing of the aspiration helps to localize the site of dysfunction, while determining solid versus liquid aspiration may help to suggest the underlying etiology. Sometimes patients with swallowing problems may alter their diet in subtle ways without realizing they are doing so, and therefore a record of what type of food and fluids a patient is currently consuming is also useful. Unintentional weight loss is an obvious feature that suggests that a patient is not able to meet their nutritional demands. Head and neck symptoms such as neck masses, mucosal masses or ulceration, throat or ear pain, trismus, dysphonia, and more general symptoms such as fatigue, weakness, or changes in gait, change should be elicited. The underlying etiology may also produce other symptoms such as dysphonia, sore throat, and otalgia. Alternatively patients may present with longstanding symptoms suggestive of chronic low grade aspiration. A full medical history, a history of any previous surgery to the head, neck, chest, or abdomen, a medication history, and a social history (including tobacco and alcohol consumption) will help to highlight any potential underlying cause for the aspiration. Fluoroscopy is performed to image the oral cavity, pharynx, and esophagus in sequence, with video recording of the examination for later review. While a barium swallow can detect aspiration, it does not assess the oral and pharyngeal phases of swallowing, and cannot give information about the mechanism of aspiration. The pharynx and larynx are best assessed using transnasal flexible endoscopy that is readily available to all Otolaryngologists. Neurological examination of the aspirating patient should include not only a cranial nerve examination, but also a general neurological assessment. If an esophageal problem is suspected, the esophagus may also be examined by otolaryngologists trained in transnasal esophagoscopy. Fiberoptic Endoscopic Evaluation of Swallowing Fiberoptic endoscopic evaluation of swallowing is a procedure for evaluating the oropharyngeal swallow function (Langmore, Schatz and Olsen, 1988; Leder, 1998). A flexible endoscope is passed into the pharynx to visualize the base of tongue, hypopharynx, and larynx. Fiberoptic endoscopic evaluation of swallowing is a very useful examination because it is a quick examination that can be performed either at the bedside or in the office, and does not expose the patient (or the examining clinician) to radiation. However, the view of the pharynx and larynx is lost during the pharyngeal phase of swallowing close (the "whiteout" period) and so the bolus is not seen as it passes through the pharynx. Investigations Radiology Radiology plays an important role in the evaluation of aspirating patients. A chest X-ray is helpful to assess the lungs for signs of infection, but will not give any information about the site or cause of the disordered swallowing. The patient was aspirating secondary to the effects of the large cervical osteophyte (double arrow) secondary to diffuse idiopathic skeletal hyperostosis. Treatment There are four main aims in the treatment of the aspirating patient: the avoidance of respiratory complications, the provision of adequate nutrition and hydration, the maintenance of psychological wellbeing, and the preservation of the airway and of phonation. Medical Medical treatment is used initially if a patient presents with respiratory complications. Until a full evaluation to determine the site and severity of swallowing dysfunction is completed, the patient should be made nil by mouth, and nutrition and hydration provided by alternative routes, such as nasogastric tube feeding. Note the blue colored fluid pooling in the right pyriform fossa and postcricoid region. Additional Investigations Depending on the findings of the preliminary evaluation of the patient, further investigations may be undertaken. Specific neurological investigations will be required to confirm certain diagnoses, such as a muscle biopsy for myopathies, or a Tensilon (edrophonium) test for myasthenia gravis. Nutritional Support the assessment of nutritional deficits by a clinical dietician is useful, so that the optimal nutritional supplements and therapeutic feeding regimens can be prescribed. Clinical dietitians also play an important role in the use of texture modified food and fluids (see below). Swallowing Therapy Speech language therapists use a variety of postural adaptations, swallowing maneuvers, and manipulation of food and fluid consistencies in order to minimize or prevent aspiration (Hegland and Murry, 2013). The clinician must first identify the site of swallowing dysfunction and the underlying cause of the aspiration in order to determine which techniques to use. The compensatory techniques described below commonly used strategies to rehabilitate the aspirating patient. The chin tuck is a posture that widens the valecullae by tilting the epiglottis posteriorly thus narrowing the airway. The head turn redirects the bolus to the stronger side if there is a unilateral weakness or structural defect of the pharynx. The patient is instructed to turn their head toward the side that is weak or has a structural defect, just before the oral propulsive phase. The efficacy of the head turn technique is often enhanced when paired with the chin tuck technique. A good example of the use of these techniques is in cases of vocal fold paralysis: a patient with a left vocal fold paralysis is instructed to look down toward the left axilla when drinking or eating, ensuring that their torso remains forward during swallowing. Patients with poor lingual control may aspirate thin fluids because of premature spillage of the thin fluids from the oral cavity into the valleculae or more distally prior to the laryngeal protection. However, most, if not all, patients do not like to take thickened fluids because they do not quench the thirst. H&N cancer patients are typically able to use an alternative technique, such as head tilt to the better side to avoid the need for drinking thickened fluids. Unlike H&N cancer patients, patients with neurological disorders may not possess adequate cognitive function, coordination, strength or energy levels to use postural techniques, and so they are usually given modified fluids. Texture modified food (such as soft, minced, or pureed food) is easier to chew and to manipulate, and requires less effort to swallow for those individuals who are easily fatigued. It may be recommended for patients with aspiration, as well as patients with obstructive dysphagia. Structural abnormalities are usually easier to correct surgically than neuromuscular disorders, although some isolated neurological disorders, such as a recurrent laryngeal palsy, respond well to surgical treatment. Glottic insufficiency secondary to vocal fold paresis or paralysis may be associated with aspiration as well as dysphonia. The restoration of glottic competence using techniques such as injection laryngoplasty and laryngeal framework surgery is effective in improving swallowing, including patients presenting with acute vocal fold paralysis (Carrau, et al. Hypopharyngeal pharyngoplasty in conjunction with laryngeal framework surgery may optimize the functional outcome of patients with aspiration secondary to a high vagal palsy (Mok, Woo and Schaefer-Mojica, 2003). The paralyzed pyriform sinus is resected to remove insensate and redundant mucosa, and the inferior constrictor muscle repositioned to reduce hypopharyngeal pooling and to improve pharyngeal tone. Tracheostomy is commonly described as a treatment for aspiration, but it is more accurately classified as an adjunctive measure to improve pulmonary toilet and pulmonary ventilation. A cuffed tracheostomy tube can offer some protection of the lower airway from aspiration, but may predispose patients to aspiration (Schonhofer, et al. Chapter 50: Aspiration Aspiration Associated with Tracheostomy the mechanisms for this can be classified as either: Mechanical (impaired laryngeal elevation, esophageal compression, and reduced cough effectiveness) or Neurophysiological (loss of subglottic pressure, reduced glottic closure reflex, reduced cough, and disruption of glottic coordination) (Buckwalter and Sasaki, 1984; Song, Schwartz and Blitzer, 2009). Cricopharyngeal myotomy is the gold standard treatment for swallowing dysfunction secondary to cricopharyngeal achalasia or a pharyngeal pouch (Pitman and Weissbrod, 2009). For patients with intractable aspiration, surgery to minimize or prevent lower airway contamination should be considered. Laryngeal closure procedures may be performed as either open techniques or endoscopic techniques (Habal and Murray, 1972; Takano, et al. Other disadvantages include the need for a tracheostomy to provide a safe airway, the loss of phonation, and technical difficulty. While laryngectomy is the definitive procedure for aspiration, it is irreversible and the patient must live with a stoma. Diverting the paralyzed larynx: a reversible procedure for intractable aspiration. If recovery is not expected, an irreversible procedure may be used, and laryngectomy remains the most reliable procedure to achieve separation. Pathophysiology, relevance and natural history Of oropharyngeal dysphagia among older people. Subperichondrial cricoidectomy: an alternative to laryngectomy for intractable aspiration. The tracheoesophageal diversion and laryngotracheal separation procedures for treatment of intractable aspiration. Efficacy and safety of acute injection laryngoplasty for vocal cord paralysis following thoracic surgery. A multidisciplinary approach to the evaluation and treatment of patients with aspiration is crucial. Accurate identification of the site of swallowing impairment, the severity of aspiration, and its underlying cause directs treatment.

Prenatal and preimplantation diagnosis is possible when the mutation(s) causing disease in the family is known womens health jackson ca generic serophene 50 mg without prescription. Although there is no specific treatment available for these disorders womens health keller tx buy serophene 50 mg with amex, research is resulting in clinical trials menopause 46 25mg serophene with amex. We are cautiously optimistic that effective interventions will be available in the near future menstruation 6 days late buy serophene now. The provision of best refractive correction women's health center hilo discount serophene american express, appropriate low vision aids and educational support is very important menstrual underpants discount serophene 100mg otc. Photophobia may be prominent and tinted spectacles or contact lenses and a sunhat may improve comfort and vision. Diseases caused by defects in the visual cycle: retinoids as potential therapeutic agents. Restoration of photoreceptor ultrastructure and function in retinal degeneration slow mice by gene therapy. Developmental macular disorders: phenotypes and underlying molecular genetic basis. Clinical spectrum of chromosome 6-linked autosomal dominant drusen and macular degeneration. Congenital coloboma of the macula, together with an account of the familial occurrence of bilateral macular coloboma in association with apical dystrophy of the hands and feet. A molecular genetic investigation of two families with macular dysplasia in association with digit abnormalities. Clinical features of progressive bifocal chorioretinal atrophy: a retinal dystrophy linked to chromosome 6q. Slightly raised jet-black lesion in the mid-periphery with an associated small visual field defect. Unilateral, multiple brown patches in the retinal pigment epithelium of no functional significance. Mandibular osteomas and dental anomalies such as missing or supernumerary teeth are also common. Multiple adenomatous colorectal polyps (>100) appear between the second and fourth decades. The fovea usually retains normal architecture preserving normal visual acuity but cases have been described with foveal involvement. Sharply defined, nonpigmented lesion in the temporal region of the macula with a characteristic point aimed toward the fovea. Histopathology Astrocytomas contain elongated fibrous astrocytes with interlacing cytoplasmic processes. Aggressive tumors are composed of larger, plump pleomorphic cells with abundant eosinophilic cytoplasm. Aggressive lesions associated with retinal neovascularization, vitreous hemorrhage, or macular edema have been treated with systemic or intravitreal bevacizumab, alone or in combination with vitrectomy or intravitreal triamcinolone. Visual loss occurs through chronic leakage and exudation, leading to cystoid macular edema and exudative and tractional retinal detachment. Between 10% and 15% of lesions are found in the juxtapapillary region with sessile, endophytic (most common), or exophytic growth patterns. Preretinal membrane traction may cause spontaneous vitreous hemorrhage59 that may require vitrectomy. Occasionally, photocoagulation and cryotherapy have been used to treat these lesions and, recently, regression with intravenous infliximab has been reported. In severe cases, folds extend to outer retina with retinal thickening and disorganization and underlying choroid thinning. The equatorial and peripheral retina is usually affected with a predilection for the temporal side. Preschool-age children usually present with strabismus or leukocoria; older children, with subjective visual loss or on routine vision screening. Poor vision is present in over 50% as a result of exudative retinal detachment or macular involvement. The "hallmark" is vascular telangiectasia, shown in this peripheral fluorescein angiogram, and aneurysmal dilation of the retinal vessels. Retinal hemorrhages cause transient visual disturbance or loss when involving the central macular area and can arise following exertion or Valsalva maneuvre. Eyes with Coats disease are occasionally enucleated because of the inability to exclude retinoblastoma. Slow progression to retinal detachment is seen in 64% of patients over a 5-year period. Poor prognostic features include: postequatorial involvement, diffuse and superior retinal location, retinal macrocysts, failed resolution of fluid after treatment, and retinal detachment at presentation. Ablation of peripheral telangiectasia and aneurysms is more effective for stage 2 disease. In cases of retinal detachment, surgical correction by drainage of subretinal fluid and/or vitrectomy combined with laser or cryotherapy has been described,93 although in many cases the visual outcome is limited due to the presence of a macular fibrotic scar, which may represent a retinal angiomatous proliferation associated with a chorioretinal anastamosis and pigment epithelium detachment. During the late phase angiogram, the retinal macrovessel remains preferentially hyperfluorescent compared to other retinal vessels. Congenital hypertrophy of the retinal pigment epithelium: enhanced depth optical coherence tomography in 18 cases. Solitary congenital hypertrophy of the retinal pigment epithelium: clinical features and frequency of enlargement in 330 patients. Usually discovered at routine examination or when the patient has presented with a neurological episode, they require no treatment. Decompensation of a congenital retinal macrovessel with arteriovenous communications induced by repetitive roller coaster rides. Combined hamartoma of the retina and retinal pigment epithelium in 77 consecutive patients: visual outcome based on macular versus extramacular tumor location. Photoreceptor loss overlying congenital hypertrophy of the retinal pigment epithelium by optical coherence tomography. Congenital Hypertrophy of the retinal pigment epithelium: prevalence and ocular features in the optometric population. Malignant transformation of congenital hypertrophy of the retinal pigment epithelium. Adenocarcinoma arising from congenital hypertrophy of the retinal pigment epithelium. Value of the congenital hypertrophy of the retinal pigment epithelium in the diagnosis of familial adenomatosis polyposis. Screening guidelines and Premorbid diagnosis of familial adenomatosis polyposis using linkage. Bilateral congenital grouped pigmentation of the retina in one girl and bilateral congenital albinotic spots of the retina in her sister. A unique presentation of grouped congenital hypertrophy of the retinal pigment epithelium. Congenital simple hamartoma of the retinal pigment epithelium: a study of five cases. Congenital simple hamartoma of the retinal pigment epithelium: optical coherence tomography and angiography features. Review of spectral domain-enhanced depth imaging optical coherence tomography of tumors of the retina and retinal pigment epithelium in children and adults. Optical coherence tomography and electrophysiological findings in torpedo maculopathy. Spectral-domain optical coherence tomography of astrocytic hamartomas in tuberous sclerosis. Optical coherence tomography of retinal astrocytic hamartomas in a 4-year-old boy affected by tuberous sclerosis. Exudative retinal astrocytic hamartoma diagnosed and treated with pars plana vitrectomy and intravitreal bevacizumab. Combined bevacizumab and triamcinolone acetonide injections for macular edema in a patient with astrocytic hamartomas and tuberous sclerosis. Sirolimus for retinal astrocytic hamartoma associated with tuberous sclerosis complex. Vasoproliferative tumors of the ocular fundus: classification and clinical manifestation in 103 patients. Clinical characteristics of ocular angiomatosis in von Hippel-Lindau disease and correlation with germline mutation. Sessile and exophytic capillary angiomas of the juxtapapillary retina and optic nerve head. The tumor suppressor protein vhl targets hypoxia-inducible factors for oxygen-dependent proteolysis. An analysis of phenotypic variation in the familial cancer syndrome von hippel-lindau disease: evidence for modifier effects. Benefits and complications of photodynamic therapy of papillary capillary hemangiomas. Intravitreal ranibizumab therapy for retinal capillary hemangioblastoma related to von hippel-lindau disease. Intravitreal ranibizumab, photodynamic therapy, and vitreous surgery for the treatment of juxtapapillary retinal capillary hemangioma. Massive exudative retinal detachment following photodynamic therapy and intravitreal bevacizumab injections in retinal capillary hemangioma. A case of Coats disease with visual recovery from no light perception vision after vitrectomy. Retinal angiomatous proliferation with chorioretinal anastomosis in childhood coats disease: a reappraisal of macular fibrosis using multimodal imaging. Hereditary infantile hemiparesis, retinal arteriolar tortuosity, and leukoencephalopathy. Abnormally distributed branches of the retinal vessels, enlarged macular arteries and long cilioretinal arteries. Swept-source optical coherence tomography of retinal cavernous hemangioma: a new imaging modality. Recent insights into cerebral cavernous malformations: a complex jigsaw puzzle under construction. Optical coherance tomography in retinal cavernous hemangioma may explain the mechanism of vitreous hemorrhage. An unusual hamartoma of the pigment epithelium and retina simulating choroidal melanoma and retinoblastoma. Surgical management of epiretinal membrane in combined hamartomas of the retina and retinal pigment epithelium. Spectral domain optical coherence tomography of combined hamartoma of the retina and retinal pigment epithelium. Combined hamartoma of the retina and retinal pigment epithelium: findings on enhanced depth imaging optical coherence tomography in eight eyes. Proliferation of the juxtapapillary retinal pigment epithelium simulating malignant melanoma. Surgical outcomes of epiretinal membranes associated with combined hamartoma of the retina and retinal pigment epithelium. A population-based study of Coats disease in the United Kingdom I: epidemiology and clinical features at diagnosis. Clinical variations and complications of coats disease in 150 cases: the 2000 sanford gifford memorial lecture. Congenital retinal macrovessel: atypical presentation using optical coherence tomography. Development and spontaneous resolution of serous retinal detachment in a patient with congenital retinal macrovessel. These classical flecks are not always present at diagnosis, but can appear later in the course of disease. These lesions may look like flecks, dots/drusen, or crystals and can be associated with a variable degree of retinal dysfunction. Clinical evaluation It is important to inquire about visual symptoms such as blurred vision, night blindness, or light hypersensitivity and to obtain a family history. Examination of relatives can be helpful, as other affected family members may remain asymptomatic. Current or previous systemic drug administration and dietary habits should be inquired after. Careful note should be taken of any other medical disorders particularly those associated with malabsorption. When examining the child, the distribution of retinal deposits, their depth in the retina, and whether they are crystalline in nature should be noted. Fundus photograph of the left and right eyes of a 9-year-old individual with Stargardt disease (top row). Fundus autofluoresence imaging (bottom row) revealed hyperautofluorescent lesions corresponding to the flecks and abnormal autofluorescence at the foveal region.

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