Porphyrias are a heterogenous group of diseases that may result in disabling or life threatening neurovisceral symptoms and/or cutaneous photosensitivity. In acute intermittent porphyria, the clinical features, particularly neurological symptoms, may be life-threatening and disabling. Conventional treatment with human hemin, though effective in reducing symptoms, does not reverse neuropathy when structural nerve damage has occurred and may cause intense phlebitis. Liver transplantation (LT) may be considered as treatment for those with repeated life-threatening acute attacks resulting in poor quality of life, requirement of ventilatory support, and progressive loss of venous access due to hemin infusion. Patients with variegate porphyria (VP) present after puberty with neurovisceral symptoms and skin manifestations. LT resolved VP in the 1 patient reported in the literature. Aminolaevulinic acid dehydratase deficient porphyria is a rare autosomal recessive disorder and a child who presented with failure to thrive and required transfusions and parenteral nutrition did not improve with LT. In erythropoietic protoporphyria (EPP), there is excessive production of protoporphyrin in the bone marrow. Protoporphyrin is hepatotoxic and pigment loading of hepatocytes and bile canalicular sludging may result in progressive cholestasis and cirrhosis. LT is beneficial for such patients with end-stage liver disease. Perioperative management includes use of filters on operative lights to prevent skin burns and intestinal perforation. Other concerns include development of neuropathy, biliary complications, and recurrent liver disease. This review addresses the rationale, patient selection, evaluation, management issues, and technique of performing LT in various types of porphyria.
Hypothermia is a frequent and sometimes clinically important problem during orthotopic liver transplantation. Numerous methods have been suggested to reduce intraoperative heat loss and promote active warming. In this study we compared an electric under mattress, a warm air under mattress and a forced warm air convective heating blanket. The forced air convective warming system was shown to produce significantly higher patient temperatures than the two other systems.
This is the official guideline endorsed by the specialty associations involved in the care of head and neck cancer patients in the UK. The anaesthetic considerations for head and neck cancer surgery are especially challenging given the high burden of concurrent comorbidity in this patient group and the need to share the airway with the surgical team. This paper provides recommendations on the anaesthetic considerations during surgery for head and neck cancer.Recommendations• All theatre staff should participate in the World Health Organization checklist process. (R)• Post-operative airway management should be guided by local protocols. (R)• Patients admitted to post-operative care units with tracheal tubes in place should be monitored with continuous capnography. Removal for tracheal tubes is the responsibility of the anaesthetist. (R)• Anaesthetists should formally hand over care to an appropriately trained practitioner in the post-operative or intensive care unit. (G)• Intensive care unit staff looking after post-operative tracheostomies must be clear about which patients are not suitable for bag-mask ventilation and/or oral intubation in the event of emergencies. (R)
SummaryIn this study we compared urinary bladder and oesophageal temperatures with the core temperature obtained from a pulmonary artery catheter in patients undergoing orthotopic liver transplantation. The bladder temperature was a closer approximation to pulmonary artery temperature in this group of patients than the oesophageal temperature and has much to recommend it in terms of convenience, safety and postoperative patient comfort.
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