Cases reported "Tuberous Sclerosis"

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1/4. tuberous sclerosis presenting for laparotomy.

    A 30-year-old female patient with tuberous sclerosis presented for anaesthesia and surgery for haemorrhagic renal angiomyolipoma. The anaesthetic management of this case was tailored to the prevention of seizures. Diagnostic features and possible complications of the disease are also described.
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2/4. Anaesthesia and tuberous sclerosis.

    The anaesthetic management of a patient with tuberous sclerosis undergoing two-stage scoliosis surgery is described. The patient suffered from severe mental retardation, seizures and facial angiofibromas. General anaesthesia using isoflurane and nitrous oxide in oxygen, supplemented with opioid analgesia and hydralazine, and labetalol to induce hypotension, appeared to be satisfactory. Postoperative recovery was delayed and complicated by pleural effusion, sputum retention and mild seizures. tuberous sclerosis is an autosomal dominant disease well known for its neurocutaneous manifestations. Other organs such as the heart, lungs and kidneys may be involved. The potential problems in the anaesthetic management of a patient with tuberous sclerosis are discussed.
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3/4. aortic aneurysm in a four-year-old child with tuberous sclerosis.

    We present a case of aortic aneurysm in a four-year-old child complicated with tuberous sclerosis. We used the same general principles as for adult patients and successfully managed our patient. Our methods included the use of isoflurane plus epidural anaesthesia, dopamine to maintain blood pressure, and induced mild hypothermia to reduce brain metabolism and to prevent spinal cord damage during aortic cross-clamping. Intensive monitoring including EEG was beneficial to the anaesthetic management.
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4/4. Anaesthesia for dental conservation in a patient with tuberous sclerosis.

    General anaesthesia for a patient with tuberous sclerosis was complicated by epilepsy. The choice of drugs was related to control of his fits and intercurrent therapy. Thiopentone, vecuronium, and nitrous oxide with isoflurane were satisfactory.
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