• Phaeochromocytoma
    • Associated with 50% mortality in unexpected emergency situations
    • Catecholamine secreting tumour of chromatin cells
    • Features
      • Rule of 10's
        • 10% familial
        • 10% bilateral
        • 10% extra-adrenal
        • 10% malignant
      • 90% occur in the renal medulla
      • Present in 4th decade
      • Most produce mainly NA
        • Can secrete adrenaline, VIP, ACTH, somatostatin and neuropeptide Y
    • Pathophysiology
      • Severe hypertension predominates
      • Paroxysmal palpitations, sweating, pallor, anxiety, tremor, weakness, chest pain
      • Triggers of paroxysms due to sneezing, alcohol, exercise, anxiety
      • Complications
        • IHD, MI, CCF, arrhythmias, pulmonary oedema
        • Cerebrovascular events
        • Haemorrhage into tumour
      • Iatrogenic factors
        • Suxamethonium
        • Metoclopramide
        • Nicotine
        • Naloxone
        • LP, labour, intubation
    • Investigations
      • Urinary catecholamines
      • Urinary VMA
      • Urinary metanephrine
      • Free plasma:urinary metabolites best assessment
      • Radiological
        • Abdominal CT/MRI
        • MIBG scan if not tumour seen on CT
    • Associated syndromes
      • MEN 2
        • Phaeo, medullary thyroid carcinoma, hyperparathyroidism (2 thyroidy things)
      • MEN 3
        • Phaeo, medullary thyroid carcinoma, neuromas of the GIT, marfanoid body habitus
      • Neurofibromatosis
        • 1% develop phaeo
      • Von Hippel Lindau
        • Assd with cerebellar haemangioma
    • Anaesthesia
      • Large IV access
      • CVC and IABP
      • Haemodynamic compromise
        • Induction
        • Intubation
        • Pneumoperitoneum if done by laparoscopy
        • Tumour manipulation
        • Ligation of venous drainage
          • Sudden drop in BP
          • Treat initially with fluids
          • Vasopressin may be useful due to alpha receptor downregulation
        • Have vasodilators present
          • Magnesium
          • Labetalol (10-20mg)
          • SNP, GTN, Nicardipine
          • Isoflurane
        • Tachyarrhythmias
          • Can be managed with esmolol
      • Post op
        • HDU or ITU care
        • May develop hypoglycaemia
    • Pre-op
      • Assess for signs of end organ damage
      • Catecholamine cardiomyopathy seen in 50%
      • FBC may show raised Hct
      • ECG - ventricular hypertrophy
      • Targets of pre-op
        • Good BP control (<160/90)
        • Postural hypotension not (<80/45)
        • Absence of ST changes
        • No more than one premature ventricular complex every 5 mins
        • Nasal congestion
        • Treatment
          • Phenyoxybenzamine + β-blocker
          • Always α-block before β-blocking
          • Phenoxybenzamine can cause post-op hypotension
          • Started >14 days prior to surgery and stopped 1 day prior
          • Started at 10mg bd and titrated up to 60-200mg daily
          • Prazosin and doxazosin are alternatives and because they are selective α1 blockers, no β-blocker is required
    • CEACCP
      Link:ceaccp.oxfordjournals.org/content/3/1/20.full.pdf?sid=c8b1d800-e3ba-41f8-8576-0d95489f76c4