Port wine stains (capillary vascular malformations) of the face are the hallmark of the Sturge-Weber syndrome. WOUNDS IN PORT WINE STAINS
• Port wine stains are comprised of a massive excess of capillaries. Therefore, wounds in port wine stains tend • Compression bandages are important before and after suturing wounds in port wine stains.
S T U R G E - W E B E R S Y N D R O M E
• Local anesthesia is the same as for any wound.
• Extensive electrocoagulation of bleeding dermal blood vessels should not be done in order to avoid E M E R G E N C Y R O O M
• Deeper wounds require dermal suturing to obtain perfect dermal alignment. Resorbable 4.0 sutures should • Cutanous sutures should be non resorbable, 4.0 or 5.0 and removed in 7-10 days.
• Patients should wear a compressive bandage for 24 hours following suturing.
• Patients should be seen in follow up the next day.
The major neurological features of patients with Sturge-Weber syndrome include seizures, headache, focal PYOGENIC GRANULOMAS IN PORT WINE STAINS
neurologic deficits and cognitive and psychological impairment. • Pyogenic granulomas are neovascular growths that are seen in normal skin, but also occur commonly in port SEIZURES
• They have a very friable surface and bleed frequently.
• Seizures develop in 70 to 80% of the patients.
• Local anesthesia is the same as for any skin injury.
• The risk of developing seizures is highest in the first two years.
• The simplest and best single treatment for pyogenic granulomas is cauterization or Laser photocoagulation of • 75% of those with seizures have the initial event during the first few months of life often in conjunction with hemiparesis or other focal deficits.
• Bleeding from the subsequent wound is best controlled by pressure and gentle electrocoagulation.
• The natural course of Sturge-Weber syndrome depends on the presence, persistence and resistance • Reoccurrence of the lesion can occur requiring re-treatment.
to treatment of seizures. Onset of seizures before 1 year of age, hypsarrhythmic EEG pattern, and the • Excisional surgery should not be performed because it is more difficult, will lead to a larger scar and may also occurrence of episodes of status epilepticus predict an unfavorable outcome.
induce the development of satellite pyogenic granulomata.
• Seizures are usually focal motor or secondarily generalized tonic clonic seizures, but infantile spasms, • For lesions less than 5 mm, pulsed dye laser treatment if available, is simple and may be effective.
myoclonic seizures and atonic seizures occur.
• Many patients have frequent and repeated seizures but some children have only occasional seizures.
• EEG studies document decreased amplitude and frequency of electrocerebral activity over the affected hemisphere. Diffuse, multiple and independent spike foci are commonly present.
• Common skin conditions such as eczema, acne, impetigo and the majority of skin diseases may be treated • CT scan demonstrates calcification, atrophy of the brain, and enlargement of choroids plexus on the the same as in patients without port wine stains.
side of pial angioma. MRI of brain is unlikely to be performed in the emergency setting, but should be obtained as part of planned workup. Gadolinium should be used to demonstrate the extent of the Compiled for the SWF by the following Professional Advisory Board Members: angioma, but this may not be well demonstrated in very young infants.
AHM Mahbubul Huq, M.D., PhD and Harry T. Chugani, M.D.
• When a child presents with a new-onset seizure, a neurologic consultation should be sought. Routine Department of Pediatrics and Neurology, Wayne State University, Detroit, MI studies including blood glucose, electrolytes, serum calcium and magnesium and CBC should be Alex V. Levin, M.D., MHSc, FAAP, FAAO, FRCSC, Department of Ophthalmology, obtained to evaluate for precipitating factors. EEG and neuroimaging studies such as CT or MRI scan The Hospital for Sick Children, University of Toronto of brain should also be obtained. Aggressive antiepileptic regimen should be established from the first seizure.
Joseph G. Morelli, M.D., University of Colorado • Arrest of status epilepticus is of utmost importance. Immediate management includes assessment Health Sciences Center, Dept. of Dermatology, Denver, CO of vital functions, establishment of intravenous lines, blood sampling for investigation of possible metabolic or infectious causes and IV benzodiazepines such as lorazepam. IV benzodiazepines may John Bodensteiner, M.D., Pediatric Neurology, St. Joseph’s Children’s Health Center, Phoenix, AZ be repeated if necessary. If seizures are not controlled within minutes of giving benzodiazepines, IV fosphenytoin or phenobarbital should be given in adequate doses. For resistant status epilepticus, Gerald Goldberg, M.D., Pima Dermatology, Tucson, AZ close EEG monitoring and supervision by specialized medical and nursing personnel in intensive care Cameron Parsa, M.D., The John’s Hopkins Hospital, Ophthalmology Department, Baltimore, MD unit are required. General anesthesia with IV barbiturates or other agents, neuromuscular blockade and respiratory support may be needed.
• Approximately half of the patients achieve complete control with standard antiepileptic agents and a The Sturge-Weber Foundation
significant proportion of the remaining patients achieves partial control.
• In uncontrollable cases, focal cortical resection or hemispherectomy can improve seizure control and Phone: 973-895-4445 • Fax: 973-895-4846 • E-mail: [email protected] *The information contained herein is specific to the United States. We recognize that emergency procedures in other countries may differ. HEADACHE
• About half of the patients complain of headaches. Migraine represents the most frequent type of STURGE-WEBER SYNDROME
• Headaches commonly occur after epileptic seizures.
• Differentiate from cellulitis by history of long recumbent period, recurrence, absence of fever, absence of • In a few patients headaches may be due to glaucoma.
tenderness, lack of true erythema (see above), lack of warmth to touch, and resolution within hours with • Headache should be managed as in other persons. However due to increased risk of focal neurologic deficits, vasoconstrictor agents such as triptans and ergots should probably be avoided.
• When a patient presents with severe headache for the first time, neurologic consultation should be • May present with ipsilateral enlarged eye, photophobia, tearing, cloudy cornea, decreased red reflex, and obtained to establish a diagnosis. Neuroimaging studies may be necessary to evaluate for intracerebral or subarachnoid hemorrhage. Cerebrospinal fluid analysis may exclude subarachnoid hemorrhage or • All signs and symptoms not required for diagnosis.
• Requires urgent referral to ophthalmologist if any suspicious signs or symptoms.
• Acute presentation of recurring severe headaches may be treated with oral or parenteral analgesics and antiemetics (e.g. IM ketorolac/IV prochlorperazine). Neuroimaging studies are indicated for recurring • Usually presents with no symptoms or signs except enlarged optic nerve cup but may have some of the headaches that do not fulfill the strict definition of migraine or other primary headache disorder. For recurring migraine-type headaches, preventive therapy may be needed.
• Asymmetric refractive errors with less hyperopia, or more myopia, on the affected side. • Intraocular pressure significantly higher than in the contralateral uninvolved eye.
• Be sure to check vision in both eyes, with glasses on and/or with pinhole to get best corrected visual FOCAL NEUROLOGIC DEFICITS
• Hemiplegia occurs in at least one-third of the cases and is localized to the side opposite to the facial • Especially in children with seizures and those who have had hemispherectomy. Most common is one • Hemiplegia commonly first appears after an episode of seizure and may become more severe with the • Decreased peripheral vision common either due to brain involvement or glaucoma.
• Retinal detachment and other complications from choroidal exudates are very rare.
• Hemianopia is present in majority of patients, either alone or in association with hemiparesis.
• Refer to ophthalmologist if new discovery of decreased vision in one eye and absent or white (leukocoria) • Transient hemiplegias not following an epileptic attack and sometimes accompanied by migraine-like headaches are observed in many cases of Sturge-Weber syndrome. These hemiplegic episodes are apparently not of epileptic nature and may be a consequence of vasomotor disturbances within or SPECIAL CONSIDERATIONS
• Intracranial hemorrhage is quite rare but has been documented in few cases.
• All newborn babies with SWS and a port wine mark affecting the eyelids and/or brain involvement should • When a child presents with acute focal neurologic deficit, determination of the cause is essential in see an ophthalmologist in first few weeks of life. Children with port wine birthmark involving lids may be orientating therapy. Neuroimaging studies such as CT or MRI scan of head should be performed and a at life long risk of glaucoma and require periodic eye examination, often under anesthesia or sedation in neurologic consultation should be obtained.
the first years of life until able to be done awake.
• Low dose aspirin has been used in some cases to prevent recurrent venous thromboses that are • Any child who has had prior eye surgery and presents to the Emergency Department with a red eye supposed to cause stroke-like episodes and focal deficits. However, lack of controlled clinical trials and (above baseline), pain, or any other ocular concern (including conjunctivitis) should be urgently referred to the clinical variability of the syndrome makes it impossible to determine whether aspirin is helpful.
Children with glaucoma are often on multiple medications. In general: OPHTHALMIC PRESENTATIONS
Ophthalmic abnormalities are part of the Sturge Weber syndrome (SWS). The information below is designed to Red top drops = pupil dilators (sympathomimetics or parasympatholytics).
offer Emergency Room physicians insight into the common eye findings unique to SWS and the special ocular Green top drops = pupil constrictors (parasympathomimetics or sympatholytics).
problems that patients with SWS may experience. Purple top drops = alpha agonists or epinephrine type compounds.
Orange top drops = carbonic anhydrase inhibitors.
Others: topical prostaglandin analogues, antibiotics, steroids and other anti-inflammatory drugs may be in use. May also use be using oral steroids or carbonic anhydrase inhibitors for eye problems.
• Port wine birthmark involving the eyelids.
• Differentiate from erythema by lack of tenderness, presence of sharply demarcated edges often • Some bottles may contain more than one agent (combination preparation).
respecting the midline, and lack of warmth to touch.
• Children with SWS on certain alpha agonists (e.g. brimonidine) may be more prone to sedative side • Red eye (prominence of conjunctival and episcleral vessels).
• Differentiate from ocular inflammation by lack of discharge, lack of pain, clearly visible tiny vessel • If a child has had a glaucoma drainage tube implant (also called seton or tube), there may be a visible complexes, tortuous and teleangiectatic in appearance, and absence of symptoms.
whitish square at the edge of the cornea in one quadrant. This is tissue used to cover the plastic tube.
• Retina and red reflex appearing redder than usual or than in the uninvolved contralateral eye.
• If a child has had glaucoma surgery, he or she may have a cystic appearing mass (bleb), either vascular • Due to choroidal hemangioma (choroid lies underneath retina).
or avascular, in one or more quadrants beyond the edge of the cornea. This is the expected area of fluid • Differentiate from retinal hemorrhage by absence of distinct blood spots and appearance of diffuse redness. Particularly obvious in unilateral SWS when compared to unaffected eye.


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