629 Autonomic learning \200 Dilation (mydriasis) Sympathetic Parasympathetic Constriction (meiosis) Eye III Tear gland Superior cervical ganglion VII Increased secretions Decreased secretions IX Airways Vagus (X) T1 T2 T3 T4 T5 T6 T7 T8 T9 T10 T11 T12 L1 L2 L3 L4 L5 S1 S2 S3 S4 T1 T2 T3 T4 T5 T6 T7 T8 T9 T10 T11 T12 L1 L2 L3 L4 L5 S1 S2 S3 S4 Increased rate and contrachility a i s e h t s e a n A Heart Decreased rate and contractility Vasomotor sudomotor piloerection Skin Liver Pro-expenditure Spleen Stomach Reduced mobility Pancreas Renin release Kidney Duodenum Adrenal Catechol- amine release Pro-storage and increased motility Ilium Colon Relaxes Contracts Sphincter relaxation Rectum Ureter Sphincter and detrusor contraction Ejaculation Uterine relaxation Erection Female Male Bladder Fig 1. The autonomic nervous system may be primitive, but it is anything but simple. Many o f the drugs used in anesthesia (eg atropine, \200 -blockers) have an eff ect on this syste m, and at more than one place at once. This means that it is useful to remind your- self of exactly where each arm of the autonomic nervous system spreads its tendrils, and how it me diates eff ects: Sympathetic Site Pre-ganglionic Neurotransmitter Receptor type All Adrenal gland1 Sweat glands All other ACh NAChR ACh NAChR Post-ganglionic ACh2 MAChR2 NA NAR Parasympathetic Site Pre-ganglionic Post-ganglionic Key: ACh = acetylcholine, NAChR = nicot inic acetylcholine receptor, MAChR = muscarinic acetylcholine receptor; NA = noradrenaline; NAR = noradrenaline receptor 1 The sympathetic \201 bres that innervate the adrenal glan d are pre-ganglionic 2 This is the main oddity: sweat glands have MAChRS in the sympathetic system NB: there are a number of other (co-)transmitters in the autonomic system (such as ATP to adeno- sine receptors). The gut also has a large independent neuronal network. If on ly we could let our minds learn with the same autonomy that it has to control the function of individual organs and homeostasis. Perhaps it is best that we are able to switch off our higher functions from time to time, leav- ing the autonomic nervous system to its usual bu siness! Neurotransmitter Receptor type All All ACh NAChR ACh MAChR Sedation De \201 nition Sedation is a range of depressed conscious levels from relief of a nxiety (minimal sedation) to general anesthesia (see BOX). 630 \200 Doctors in many specialties may be required to administer sedation. \200\207The doctor giv ing moderate or deeper sedation must not also be responsible for performing any procedure ( such as manipulation of a dislocated joint). Her sole responsibility is to ensure that the sedation is adequate, and to monitor the patient \200\231s airway, breathing, and circulati on. Sedation is not a short cut to avoid formal anesthesia, and it does not excuse the pati ent from an appro- priate work-up or reasonable fasting ( \200\207risking aspiration of gas tric contents, p624). Monitoring is mandatory, and must include at least pulse oximetry, HR and BP. 45 It is easy for sedation to become general anesthesia, with its at- tendant risk s (see p628). The loss of the \200\230eyelash re \202 ex \200\231 (gentle stroking of the upper eyelashes to produce blinking) is a good guide to the onset of general anesthesia. In the elderly, \200\201cognition after GA may persist for months; 46 regional anaes- thes ia may decrease it, 47 it is unclear if sedation can obviate it \200\224although it seems r eduction in duration of anesthesia may limit risk. 48 Agents \200 Midazolam: Initial adul t dose 2mg IV over 1min (1mg if elderly). Further 0.5 \200\2231mg IV as needed after 2min. Usual range 3.5 \200\2237.5mg (elderly max 3.5mg). SE: psychomotor function \200\201. 49 \200 Propofol: Widely used for sedation, see p620. Rapid acting anesthetic \200\224but may lead to hypotension and apnoea. In many circumstances (eg manipula- tion of large joint; p ainful dressing changes) a narcotic analgesic may be used too (eg morphine in 1 \200\2232mg aliquots IV, or shorter-acting opioids such as fentanyl) as propofol doesn t have analgesi c properties. a i s e h t s e a n A Ketamine may be administered. This is a dissociative anesthetic agent which can be used as an induction agent (1.5 \200\2232mg/kg), or for procedural se- dation ( 1mg/kg). It produce s deep analgesia with super \201 cial sleep without loss of airway re \202 exes or hypote nsion. Side-eff ects include nausea, vomiting, emergency delirium and hallucinations. Ketam ine and propofol can be com- bined in a single syringe to produce a sedative agent with les s hypotensive eff ects than propofol alone, and less nausea/emergence problems than keta- m ine. 50,51 This is sometimes referred to as a ketofol sedation. \200\207Remember the need for oxygen and equipment to support ventilation as well as appropriate monitoring when unde rtaking any form of sedation. Recovery from anesthesia \200 Oxygen supplementation via facemask: as much and for as lon g as necessary to counteract hypoxia due to diff usion hypoxia, respiratory depression, or ventilation/perfusion mismatch.1 Monitor pulse and BP; keep the patient warm. \200 Look for hypoventilation (?inadequate r eversal \200\224check with nerve stimula- tor; narcosis \200\224reverse opiates with naloxo ne \200\224cautiously to minimize pain; check for airway obstruction). Ensure adequate anal gesia. Return the patient to the ward when you are satis \201 ed with his cardiovascu- lar and respiratory status and pain relief. Give clear instructions on post-operative \202 uid regimens, blood transfusions, oxygen therapy, pain relief, and physiotherapy. Post-operative nausea and vomiting (PONV, p617) is partly preventable by a 5-HT3 receptor a ntagonist, eg granisetron or dexamethasone. 52 Metoclopra- mide is less eff ective. 53,54 1 For a short duration (<5min) while very soluble nitrous oxide is diff using out of the ci rculation into the alveoli, the concentration of O2 in alveolar gas will be falling (diff u sion hypoxia).55 631 Level of sedation Minimal sedation (anxiolysis): is a drug-induced state where the patient is still able to r espond to speech. Cognitive function and coordination are impaired but airway, breathing an d cardiovascular systems are unaff ected. Moderate sedation (conscious sedation): is drug-i nduced reduction of consciousness during which the patient is able to make a purposeful re- sponse to voice or light touch. Response to pain only indicates deeper seda- tion. At this level of sedation no airway adjuncts are required, breathing and cardiovascular function s hould be adequate. Deep sedation: drug-induced reduction in consciousness to a point where the patient cannot be easily roused but does respond purposefully to pain- ful stimuli (wit hdrawal is not purposeful). At this level airway intervention may be required (jaw thrust/ chin lift). Spontaneous ventilation may become inadequate. General anesthesia: drug-induced loss of consciousness during which patients are not able to be roused, even with repeated painful stimulation. Airway typically requires intervention, spontaneous ventilation is fre quently inadequate and cardiovascular function may be impaired. a i s e h t s e a n A (Based on American Society of Anesthesiologists Guidelines, 2009) 56 \200\207Typically pro cedural sedation of patients outside of the operating theater or ICU for is con \201 ned t o ASA 1 or 2 category patients (see p615). Gauging depth of general anesthesia Lack of inappropriate levels of general anesthesia in a patient should be suspected in the presence of: \200 \200\200HRs \200 \200\200BPs \200 Lacrimations \200 Dilated pupilss \200 Movement \200 Laryngospasm \200 Note t hat many of these responses are mediated by autonomic sympathetic drive (=s, see p629), and all should be picked up with appropriate and con- tinual monitoring (p628). Other measures: Bispectral index (BIS) EEG monitoring can help assess the depth of GA \200 \224and can also reduce PONV and anesthetic consumption, but not cost or time to discharge. 57 Evoked potentials (auditory and somatosensory) have also been used. 58 \200\207No sing le method is reliably accurate, and these cannot replace the vigi- lance and clinical suspi cion of the anesthetist. Local anesthetic agents Local anesthesia is used either alone or to supplement general anes thesia, by reducing nerve conduction of painful impulses to higher centers (via the thalamu s), where the perception of pain occurs. Local anesthetics act by im- pairing membrane perm eability to sodium, resulting in a membrane-stabiliz- ing eff ect, thereby blocking impulse propagation. \200\207Regional techniques (i.e. to whole limb/lower half of body) may lead to loss of consciousness or loss of airway, and so require the same facilities, expertise, and precautions as for general anesthesia (eg full resuscitation facilities) Types of loca l anesthetic agents (LA): 632 \200 1 Lidocaine (lignocaine) (t =2h) Max dose in typical healthy adult=3mg/kg IBW up to 200mg. Max dose of lidocaine + adrenaline = 7mg/kg IBW up to 500mg. 2 Prilocaine (t =2h) Moderate onset. The dose is 3 \200\2235mg/kg IBW. 400mg is the maximu m dose in adults. Very low toxicity, so it is the drug of choice for Bier \200\231s block ( IV regional anesthesia). If <6 months old, then particularly susceptible to methaemoglobina emia. 3 Bupivacaine (t =3h) Slow onset and prolonged duration. More cardiotoxic than other local anesthetics. Contraindicated in IV regional anesthesia (Bier \200\231s block). Dose for lo cal in \201 ltration is 2mg/kg IBW to a max of 150mg. 4 Levobupivacaine (isomer of bupivac aine1) is less cardiotoxic. Dose for local in \201 ltration or peripheral nerve block: 2mg /kg (max 150mg). Use <150mg (use 5 \200\2237.5mg/ml solution) for epidural; <15mg for intra thecal. a i s e h t s e a n A 5 Ropivacaine (t =1.8h) Dose: 3mg/kg IBW. Less cardiotoxic than bupivacaine. Less motor bl ock when used epidurally. Contraindicated for IV regional anesthesia and paracervical block in obstetrics. 6 Tetracaine (t =1h) Slow onset. High toxicity. Drops for topical anesthesia to eye (the e ye must be covered with a patch following use), and now topi- cally (AMETOP ) as an altern ative to EMLA . Also available as a gel (com- bined with adrenaline and lidocaine) which c an be used on open wounds 7 Cocaine: very high toxicity. Short duration of action. Used in ENT surgery NB: 0.5% solution=5mg/mL. 1% solution=10mg/mL. So for a 70kg man, the maximum d ose of lidocaine is 20mL of 1% or 10mL of 2% solution. NB: systemic absorption is increased from areas of increased vascularity (eg intercostal blocks, scalp wounds); certain commerc ially available preparations contain adrenaline which slows systemic absorption of LA. Howe ver, systemic eff ects from the adrenaline may arise and prove hazardous, especially in CVS disease or \200\200BP. \200\207Adrenaline is contraindicated in digital or penile blocks , and around the nose or ears (due to concerns of causing local ischaemia/necrosis). Toxici ty From excess dose, too rapid absorption, or direct IV injection. Fea- tures: Peri-oral ti ngling; numb tongue; anxiety; lightheadedness; tinnitus; seizures; apnoea; collapse; direct myocardial depression; coma. Treatment: 59 \200 Stop injection of LA; \200\207Call for h elp; Ensure ABC; Give 100% O2. cvs collapse: Try standard therapies \201 rst; then consid er lipid emulsion (Intralipid ). This may work by binding to the LA and thus reducing the amount of free LA in the circulation. 60 Initial dose of 1.5ml/kg of 20% Intralipid over 1 min (can repeat twice more after 5min) plus infusion (15mL/kg/hr;max combined dose of 12m l/kg). If in circulatory arrest manage with standard ALS protocols plus Intralipid . Recov ery from LA-induced cardiac arrest may take >1h. Seizures: Benzodiazepines/propofol/thiopen tal in small incremental doses. Anaphylaxis Occurs more commonly with the esters (eg tetrac aine/cocaine), but can occur with the amides (1 \200\2235 in above list) ( \200 p237 and OH CM p806). 1 Racemic mixtures contain D & L isomers; the D isomer may cause cardiac problems.61 NB: st ereo-isomerism describes molecules having the same formula but there are 2 possible mirror- image structures (enantiomers). R (rectus)/D (dexter) enantiomers rotate light to the right ; S (sinister)/L (levo) enantiomers to the left. 633 Local anesthesia techniques (see p634 for neuraxial techniques) \200\207Explain the proced ure, as co-operation helps. Get informed consent (OHCM p554). In \201 ltration Use a small gauge needle. Lidocaine 1% is the most common agent. Frequent aspiration is important. Rem ember that after initial injection, subsequent injections through in \201 ltrated areas hu rt less. Likewise, in \201 ltra- tion through the cut edges of a laceration is less painfu l than through the skin. Levobupivacaine 0.25% can be in \201 ltrated around and into the operative wound prior to waking from GA. Topical Tetracaine to eye. Lidocaine 4% spray to c ords prior to intubation (nil by mouth for 4h afterwards avoids aspiration). Nerve blocks L idocaine 1%, prilocaine 1% (3% or 4% is available for dental use), or levobupivacaine 0.25% . Nerve stimulation increases success rate. Ac- curate placement (eg aided by ultrasound-gu ided nerve location) of an appro- priate concentration of agent is the most eff ective meth od of inducing anaes- thesia. A practical example Femoral nerve block: Direct injection or catheter placement into the femoral sheath. a i s e h t s e a n A 1 Mark out the inguinal ligament (pubic tubercle to the anterior superior iliac spine). Palpate, and mark, the femoral artery. 2 Insert short-bevel needle (insulated if nerve stimulator is used) 1cm lat- eral to the ar tery, just below the ligament. A \200\230click \200\231 is felt on traversing the skin, an d again on traversing the fascia lata. Find the position either by eliciting paraesthesia o r with a nerve stimulator. 3 Aspiration test, then inject eg 0.25% levobupivacaine 20 \200\22330mL. Speci \201 c peripheral blocks and their uses Block type Bier \200\231s block (p744) Cervi cal plexus Interscalene (brachial plexus) Examples of use Hand/forearm manipulation or surgery Carotid endarterectomy 62 Shoulder sur gery (good prolonged post-op pain relief) Hand/forearm surgery Hip surgery (combined with a sciatic nerve block) Axillary block Lumbosacral plexus (psoas com- partment block) Ilioinguinal \200\223iliohypo gastric nerve Inguinal hernia repair Femoral nerve (see above) Femoral fracture, knee surgery (when com- bined with a sciatic block) Surgery below the kne e Ankle or foot surgery Sciatic nerve Popliteal & saphenous nerves \200\207Ensure that the anesthetized area is po sitioned and protected suffi ciently both intra- and post-operatively to avoid injury. \200\207Complications from nerve blocks include: Nerve damage \200 Overdose \200 Inadvertent IV injection \200 Damage to adjacent structu res \200 Hemorrhage \200 Infection \200 Incorrect nerve block (eg accidental phrenic ner ve block with brachial plexus block) \200 Skin injury (from accidental pressure/heat). Neuraxial anesthesia: techniques \200\207Explain the procedure: co-operation helps. Get in formed consent (OHCM p570). \200\207If unfamiliar or learning, these techniques are only t o be practiced under the direct supervision of an anesthetist. Spinal anesthesia Anesthetic into the sub- arachnoid space. The aim is to anesthetize the spi- nal roots passing throug h here. \200 Insert IV cannula and start IV crystalloid \200\224eg 634 \200 500mL. Check BP; position patient \200\224sitting or left lateral \200 Surgical scrub; mask, gow n & gloves. Prepare Fig 1. Approximation of the subarachnoid space in red on this T2-weighted MRI. Note spinal nerve roots. Courtesy of Norwich Radiology Department back. In \201 ltrate skin with 1 \200\2232mL 1% lidocaine. \200 Insert a 25G spinal needle at L3/4 space (ie below spinal cord). Free \202 ow of CSF con \201 rms correct placement. R otate the needle through 180 to ensure that all the needle aperture is in (avoids patchy blocks). a i s e h t s e a n A Inject 1 \200\2233mL 0.5% Marcain Heavy (bupivacaine + dextrose, a hyperbaric so- lution that falls by gravity). NB: much less LA is needed in pregnancy. Consider adding a small am ount of opioid to prolong the analgesic eff ect. Achieving a low, high, or unilateral block is only possible if the patient is left in position for a protracted period prior to surgery (usually unrealistic). \200 Monitor BP \200\224may \200\201; if so, give crystalloid vasopressors (eg ephedrine 3 \200\2236 mg IV repeated as needed (p632), or phenylephrine 50 \200\223100 g). A small total drug co ncentration is required \200\224producing sympathetic block- ade (vasodilation, \200\201BP ), sensory blockade (numbness) and \201 nally motor block- ade ( \200\201 or absence of l ower limb power). Complications of spinal anesthesia: \200 Total spinal block ( \200\201B P, \200\201HR, anxiety, apnoea, LOC) \200\224see below \200 Headache \200\224see below \200 Urinary retention \200 Perma- nent neurological damage (very rare). Extradural (epi dural) anesthesia 63 This is anesthetic into the extradural space. In- sertion of indwellin g catheter allows prolonged instillation of LA and/or opiates. Larger volumes of LA are req uired than with spinal anesthesia. Opi- ods enhance sensory and not motor block. Lumbar mos t common site, but cervical/thoracic possible (needs great skill). \200 Use aseptic techn ique, with patient sitting, or in Fig 2. Approximation of the extradural space in yellow on this T2-weighted MRI. Courtesy of Norwich Radiology Department the left lateral position. Check BP. Start IV crystalloid \200\224eg 500mL. \200 L3/4 commonest site. In \201 ltrat e 1 \200\2232mL 0.5% lido- caine. \200 Insert 16G Touhy needle until held \201 rm in ligamentum \202 avum ( 2 \200\2233cm) \200 \200\230Loss of resistance \200\231 technique \201 nds epidural spac e: 10mL 0.9% saline via Touhy needle is diffi cult to inject while in ligaments, but once i n the epidural space, sudden loss of resistance enables easy injection. Fine-bore epidural catheter threaded, needle withdrawn, and catheter placed to needle depth plus 3 \200\2235cm. Check you can t aspirate \202 uid from catheter. Administer 2mL test dose of anesthetic and wait 3min. If there has been inadvertent intrathecal placement this will result in spinal anesthesia. \200 Inject requ ired dose, eg 10mL 0.25% plain bupivacaine in 5mL aliquots. \200 Secure catheter in place . \200 Monitor BP every 5min for 15min (slower onset than spinal; therefore hypoten- sion takes longer to be revealed). Complications of epidural anesthesia Dural puncture: <1%. CSF leak may not be obvious; henc e the importance of test dose. Push oral \202 uids, with caf- feine. Nurse \202 at. Giv e analgesics for headache, laxatives to prevent constipa- tion/straining. Blood patch is us ually necessary if headache lasts >24 \200\22348h. Inform a consultant. Vessel puncture and inadvertent injection: Treat with ABC remembering: O2, IVI, pressor drugs, atropine if bra dycardia (due to block of sympathetic out \202 ow to heart T2 \200\2234). Hypoventilation: Motor block of inter- costals; may need control of ventilation. \200\230Total spinal \200 \231 \200\224ie injection of a large epidural dose into the CSF. Marked hypotension. Apnoea . Loss of consciousness. Treatment: ABC resuscitation and 100% 02. Treat \200\201BP. \200 Death will occur from as- phyxia if treatment is not prompt. Epidural haematoma or abscess : Aim for early diagnosis to prevent permanent CNS damage. \200 Get emergency neurolog- ic al review and MRI. 64 Other: Patchy or unilateral block. Nerve root damage. Bene \201 ts o f epidural anesthesia In obstetric practice (p66) there is no excess risk of cesarean secti on, 65 though superior outcome with regional anesthesia compared with GA has not been con \201 rmed. 66 Epidural local anesthetics cause less GI paralysis compared with systemic or epidural opioids, with comparable pain relief. 67 Epidurals may also \200\201post-op risk of respiratory failure. 63 Caudal (sacral epidural) Left lateral; prone or semi-prone posit ions; aseptic technique. Palpate sacral hiatus (4 \200\2235cm above coccyx tip). This is of ten not easy. Another method is to palpate the posterior superior iliac spines: the line jo ining them forms the base of an equilateral triangle with the sacral hiatus at the apex. In sert 21G block needle perpendicular to skin through the sacrococ- cygeal membrane into the sacral canal. A 23G needle may be useful for infants. Aspirate, and inject up to 20mL 0.5% bupivacaine in the adult. If injecting is diffi cult (there should be no resistance), or sw elling occurs, the needle is in the wrong place \200\224so stop! Withdraw the needle and st art again. Indications Provides anesthesia for the sacral region \200\224commonly used in c hildren. Use- ful, eg in scrotal surgery, low cavity forceps (needs experience because of r isk of injecting into baby \200\231s head), hernias or hemorrhoids. GA or LA? Abdominal, pe lvic and lower limb surgery are ideal for regional neur- axial techniques (eg hernia repair , THR). However, in day-case surgery neuraxial techniques may be associated with a prolonge d stay. 68 For some operations, outcome may be better with neuraxial techniques \200\224one study found marginal bene \201 ts of fewer deaths and DVTs in hip fracture surgery. MET69 Remember that they can also be used as an adjunct to GA, reducing the stress response and post- operative pain. 70 Absolute contraindications to all neuraxial anesthesia: 635 a i s e h t s e a n A 1 Anticoagulant states (risk pressure damage to cord from bleed \200\224there is an extensive local vertebral venous plexus). 2 Local sepsis (risk of introducing infection to CSF). 3 Shock or hypovolaemic states (eff ective reduction in circulating volume due to vasodilatation). 4 Raised intracranial pressure ( \200\203coning). 5 Unwilling or uncooperative patient. 6 F ixed output states (eg mitral and aortic stenosis). Relative contraindications to all neuraxial anesthesia: \200 Neurological disease \200 \224procedure may be blamed for change in state. \200 Ischaemic heart disease. \200 Spi nal deformity or previous surgery. \200 Bowel perforation (theoretical risk of \200\200p arasympathetic activity, peristalsis and peritoneal soiling). Pain John Keats, the best known medical stu- dent to die of unrequited love, showed that th e life of the spirit entails the capacity to feel pain, but, as usual, Nature has been over -generous in endowing us with this capacity \200\224so making the treatment of pain paramou nt: analgesia promotes wellbeing, sleep, and the honeyed indolence preceding recovery or th e easeful passage into obliv- ion. We tend to think simplistically about pain, assuming tha t when pain is submitted to us we must respond with some- thing analgesic or anesthetic \200\224but there are other approaches \200 see quote. 636 \200 That some stream of lightning From the old man in the skies Can burn out that suffering No right-taught man denies. But a coarse old man am I, I choose the second best, I forget it all awhile Upon a woman \200\231s breast. WB Yeats Daybreak a nd a candle-end Remember also that pain relief aids physiotherapy (allowing coughing and mobility), prevent ing pneumonia. Pain also exacerbates hypo/hyperventila- tion, hypertension, and tachycardia , and can lead to urinary retention. Methods of analgesia (See OHCM p576 and OHCM p534)1 1 Oral: Try paracetamol: 1g/6h \200\224then: \200 NSAIDs: Diclofenac 50mg/8h (remember dan ger of GI bleed; cover with ran- itidine or misoprostol; caution in asthma). Eff ects on re nal function are minimal if pre-op U&E is normal 71 but be cautious if hypovolaemic. \200 Tramadol 50 \200\223100mg/4 \200\2236h PO/IV. Fewer SEs than morphine, but less potent. \200 Opioids, eg morphine sulfate solution. NB: most are poorly absorbed from the gut. \200 Neuropathic agents: eg gabapentin for neuropathic pain associated with chronic regiona l pain syndrome (CRPS, p739) or diabetic/vascular neuralgia. See BNF section 4.8.1. a i s e h t s e a n A 2 Sublingual: Buprenorphine (an uncommonly used synthetic opiate; \200\230con- trolled \200\231 drug): 0.4mg/6h sublingually, or buccal fentanyl drops/lozenges. 3 Inhalational: N itrous oxide/oxygen (Entonox ), useful for labor pains, changing dressings, and physiotherapy. 4 Intramuscular: Rarely used \200\224eg morphine 10mg IM; Pethidine 100mg IM 5 Subcutaneous : Used in palliative care e.g diamorphine. 6 Intravenous: Boluses or continuous infusion. P atient-controlled analgesia (PCA). The patient can give himself boluses, avoiding the risks of continuous infusion. Remember to program maximum dose limit. 7 Regional anesthesia (RA): p634. Epidurals (opiates, or LA, boluses or con- tinuous infusi on). Many techniques used (intercostal nerve, brachial plexus, femoral nerve blocks). 8 Transcutaneous fentanyl patches. 9 Transcutaneous electrical nerve stimulation: (TENS). Chronic pain No single treatment will be eff ective for every patient. Treat- ment may be b roadly categorized into three categories \200\224pharmacological, physical and psychologica l. The British Pain Society recommends individual- ized pain management programs based on t he principles of cognitive be- havioural therapy for those with chronic pain which cannot b e remedied with drug and physical treatments alone. These consist of education on pain phys i- ology, psychology and self management of pain problems. 72 Pharmacological treatments in clude simple analgesia, opioids tricyclic antidepressants, neuro- pathic pain medicines (e. g gabapentin) and epidurals for low back pain. Inter- ventions which may help certain patie nts include acupuncture and hypnosis. Pain in children See p719 for narcotic and other anal gesia in painful condi- tions such as sickle-cell disease. For post-op relief, see p172. 637 Quality in anesthesia Deming \200\231s de \201 nition: Quality is meeting customer require ments at a price they are willing to pay. On this view, anesthesia has various \200\230cus tomers \200\231 rat- ing its quality: surgeons, hospitals, tax-payers, and patients, and th ese groups may have con \202 icting requirements. 73 The most important customer is the patient, and their chief requirement is pain relief \200 \224but don \200\231t focus on this narrowly. Patients are also very keen to have a friendl y anesthetist explaining what to expect. This is the chief area of disparity between what a nesthetists think patients should want and what they actually want. The best approach to an esthesia does not regard these tasks as separate: they are complementary.1 a i s e h t s e a n A EBM in anesthesia: possible limitations In anesthesia research, surrogate or intermediate o utcomes predomi- nate as the end-points, which is a weakness when the results serve as the evidence on which to base clinical decisions. More importantly, in interven- tions requirin g skill, dexterity, and dynamic decision-making, do not assume that equally good outcomes a re achievable by all, by the simple application of the results of EBM. 74 That said, sectio n 1 of Bandolier \200\231s Little Book of Pain 75 2 off ers valuable insight on the approac h to the evidence base for the treat- ment of pain. \201\201 Patient-centered anesthesia Anesthetists must form brief but intimate relationshi p with their patients, often under diffi cult circumstances. As well as doing a good techni cal job, they need to be aware of subjective areas that are of particular importance to pat ients. These are centered around physical comfort and respect. In one study, ratings for in formation provision, involvement, and emotional support were rated signi \201 cantly less important than physical comfort and respect. Ratings did not diff er very much vis vis i npatient vs day surgery, surgical service, type of anesthetic, or anesthetist. 76 Comfort m ay center around needleless induction of anesthesia and good peri-operative care in non- fr ightening surroundings. 77 Also, avoiding nausea/vomiting (p617) are top priorities. 78 NB: information booklets can improve satisfaction. 79 Patient-centered anesthesia cannot \202 ourish in a vacuum: if the whole con- text of car e is patient-centered the need for anesthetic care itself may be less. For example, in obst etrics where there is a one-to-one relationship be- tween the midwife and the mother, the n eed for epidurals is 50% less (and the 2nd stage of labor is shorter) than when less perso nal methods are used. 80 One way to improve patient-centered anesthesia is to control distr actions in the anesthetic work place: time and motion studies show that it is rela- tively easy for anesthetists to be distracted by extraneous interruptions. 81 Exactly how to limit this in busy NHS practice is a challenge. Patients can be confused by an uncertain locus of responsibility. In some cultures (eg in J apan) chief responsibility for peri-operative care lies with the surgeon. In other cultures , responsibility is shared \200\224with confusing results unless the surgeon and the anesth etist co-operate closely. 82 1 See the Bandolier website for the 2007 Oxford league table of analgesia effi ciency, whic h outlines the NNT for diff erent analgesics in acute pain.83 2 The authors would like to t hank Mr Christopher Constant for bringing this resource to their attention. 638 10 Unusual eponymous syndromes See also OHCM p708 \200\223731. To have any disease is unfortunate, but to have a rare dise ase is doubly so: the patient must often wait for ages for a diagnosis, and then he must co ntend with his physician \200\231s lifelong morbid interest in him. Having a rare disease i s common! ( 30 million in Europe): 5000 diseases have prevalences of <1 : 2000. 1 See rare. diseases.org & geneticalliance.org for assistance with rare diseases. Alport \200\231s synd rome x-linked or auto- somal recessive sensorineural deafness, pyelonephritis, haematuria, and renal failure (glomerulonephritis+ basket weaving of GBM). X-linked forms are caused by mutations in COL4A5 genes that encode the \200 5- chain of type-IV collagen. \201\227 : No eff ective treatment is available, but they may do well on dialysis or after kidney tran splant. Bone marrow transplant might be an option in future. Typical age at death ( \200 ): 20 \200\22330yrs.2,3 Asperger \200\231s syndrome Autistic features (p394) without autistic aloneness or linguistic diffi culty. It is less severe than autism.4 It is possible to tea ch better recognition of emotions and how to predict emotional responses.5 Bardet \200\223B iedl syndrome Autosomal re c e s s i ve A key cause of chronic renal failure in chil- dren. 6 Signs: Renal calyceal clubbing/blunting, calyceal cysts fetal lobulation; retinal dyst rophy; IQ \200\201; hypogonadism; obesity; anosmia; polydactyly. Lack of paraparesis distin guishes it from Laurence \200\223Moon \200\223Biedl synd., p648. \200 : \200 \211\2101.3: 1.7 Batten \200\231s syndrome (juvenile neuronal ceroid lipofuscinoses) \200\200Apoptosis of photoreceptors & neurons from defects in the CLN3 gene causes vision \200\201, child- ho od dementia, \201 ts, ataxia, spasticity, athetosis, dystonia, and early death (in teens) . Tests: Skin biopsy;8 lipopigments in lymphocytes and urine. 9 Becker \200\231s muscular d ystrophy x - l i n ke d recessive There are mutations in the dystrophin gene (Xp21), but un like Duchenne (p642) (where there is near-total loss of dystro- phin) there is \200\230sem ifunctional \200\231 dystrophin, and milder symptoms and later onset, slower progression bu t more calf enlargement in adolescence. \201\227: Symptomatic. Steroids may help (monitor creatine kinase).10 Genetic counseling11,12 (p154). Beckwith \200\223Wiedemann syndrome is the chief cause of macroglossia;13 other signs: macrosomia, visceromegaly, omphalocoele, he mihypertrophy, micro- cephaly, hypoglycaemia, feeding diffi culty. Overexpression of IGF2 g ene at lo- cus 11p15 causes embryonal tumors (esp. Wilms, p133) in 8%.14 Bourneville \200 \231s disease (tuberous sclerosis; epiloia=EPIlepsy, LOw Intelligence + Adenoma sebaceum, s ee \201 g 1) Autosomal dominant (AD) multi-organ cal- ci \201 ed hamartomatous tubers. L oci on 9q34 (TSC1, making harmatin) & 16p13 (TSC2 makes tuberin; mutations here are worst). Prognosis: often benign. Diagnosis: Try to get 2 major features (*), or 1 major and 2 mino r.15 \200 Eye/CNS: Fits (at presentation in 90%), 16 autism, 17 subependymal astro- cytom a*, hydrocephalus, periventricular calci \201 cation, \200\201IQ, phacomata (white or yel low retinal tumors in 50%), retinal nodules* & achromatic patches. \200 Skin and hair: H ypomelanic macules ( \200\230ashleaf \200\231 macules, which are Wood \200\231s lamp +ve)*, adenoma sebaceum (warty nasolabial angio \201 bromas*; \201 g 1), caf -au-lait spots, butter \202 y rash, periungual \201 bromas* ( \201 g 2), skin tags, sa- cral plaques (s hagreen patches, like shark skin), white lock. \200 Mouth and teeth: Pitted tooth enamel, gingival \201 bromas. \200 Bone: Sclerotic lesions, phalangeal cysts, hypertelorism. \200 Kidney: Angiomyolipomas*, multiple cysts, haematuria. \200 Lungs: Honeycomb lung, p neumothorax, \200\230muscular hyperplasia of lung \200\231. \200 Heart: Rhabdomyomas*, B P \200\200, cardiomyopathy, haemopericardium. \200 Lymph nodes: Lymphangiomyomatosis*, Ca stleman tumor. \200 Bowel: Polyps Peutz \200\223Jeghers, OHCM p700. \200 Liver and p eritoneum: Carney \200\231s complex (p699). Also: Precocious puberty. \201\201 The hidden faces of Jack, some gunpowder \200\224and a spark We are surrounded by eponyms commemorating the Great and the Good, from the Reith Lectures and the Booker Prize , to the 100 proof of Jack Daniels and Johnnie Walker. Medical eponyms are pickled in som ething almost as intoxicating: the hidden recesses of our own minds. We store away the biza rre, the fearsome, and the mundane \200\224and then, years later, as if playing some dreadf ul game of snap, we match these features with the person sitting in front of us, and say: \200\234Dandy \200\223Walker! \200\235 or \200\234Prader \200\223Wil- li! \200\235 Here we deal a pack and a half (84 cards) to play with, plus a few jok- ers, and fascinating and f rightening games they can be. But as the years go by we wonder more and more about the peop le behind the eponyms. We read about these quacks and geniuses \200\224but it is always rat her unsat- isfying: history shows us everything except the one thing we want to see: the sp ark that made these eponymous characters truly original. We resign ourselves to the fact th at we can only ever see one face of the jack. More years pass and inexplicable events teach us that we all have hidden faces we never directly see or know (p325). So the possibility arises that these hidden faces are regarding each other. That is the sensation we have on c onjuring with the names of Dandy and Walker and the rest: a sensation that we are not alone \200\224that we are accompanied. 639 s e m o r d n y s s u o m y n o p e l a u s u n U . n o i t c a r t s i d e l b a d r o f f a n u n a s \200\231 t i : k n u b n a h t e s r o w s i Whether we are connoisseurs of Johnnie Walker or Dandy \200\223Walker and its diagnosis, we are relying on hidden processes going on in the dark over many years, to give us the spiri t that burns with a steady \202 ame when we are mixed with gunpowder \200\224and that spa rk. This \200\230steady \202 ame \200\231 is the old de \201 nition of alcoholic proof above 100 .1 It is also our reward for having transformed raw knowledge into something ill uminating, by the hidden processes of fermentation and distillation. y r o t s i h l a c i d e M ? y r o t s i h t u o b a s e r a c This special learning sometimes goes to our heads, and, drunk on knowl- edge, we career up and down the wards causing havoc until we learn to apply judgment as well as knowledge (as Benjamin Disraeli observed: in order to be successful as a the Queen \200\231s First Minist er, it is unpardonable to have a good memory: it is vital to be able to forget). o h W Fig 1. Adenoma sebaceum. Fig 2. Periungual \201 broma. Both \201 gures courtesy of Dr Samuel da Silva. 1 Spirits were once graded by the gunpowder test: a mixture of water and alcohol \200\230p roved \200\231 itself if one could pour it on gunpowder and a spark could induce it to burn with a steady \202 ame. If it did not, the liquor was too weak. A \200\230proven \200 \231 liquor was de \201 ned as 100 degrees proof (100 ).18 Briquet \200\231s syndrome (fat-folder syndrome) Hypochondria (p334) with soma- tization wi th >13 medically unexplained symptoms in various organs (see BOX). Bruton sex-linked \200 agammaglobulinaemia See p199. Buchanan \200\231s syndrome (truncus arteriosus) A single art ery arises out of the base of the heart, supplying pulmonary and systemic vasculature. The aorta may be divided. There is cyanosis from birth. Surgical correction is possible. Capgra s syndrome (clonal pluralization of the self)19 A \200\230delusion of doubles \200\231 tha t oneself or a friend has been replaced by an exact clone, who is an impos- tor. Cause: Psy chosis; head injury; B12 \200\201; pituitary or right occipitotemporal le- sion; multiple s clerosis. 20 Quetiapine may help. Myself am ever mine own counterfeit Castleman \200\231s d isease Angiofollicular lymph node hyperplasia + benign vascular mediastinal tumor. 2 types: Hyaline vascular and plasma cell type (T \200\200, anae- mia, weight \200\201). Frank ly mphoma is rare. POEMS syndrome may be present (BOX). 22 Chediak \200\223Higashi syndrome Im munode \201 ciency, hypopigmentation, photo- phobia, nystagmus, weakness, tremor, fever, p latelets \200\201, liver \200\200, lymphoma. WBCs contain big peroxidase granules. Cause : 1q43 mutation (CHS1/LYST gene).23 Fatal in 90% by 10yrs of age without marrow transplanta tion. 24 Conradi \200\223H nermann syndrome (chondrodysplasia punctata) Saddle nose, nasal hypoplasia, frontal bossing, short stature, stippled epiphyses, optic atro- phy, cataracts , IQ \200\201, \202 exural contractures. Cause: Genetic or eff ects of warfarin given dur ing the 1st trimester of pregnancy. 25 Cornelia de Lange syndrome Autosomal dominant mutati on in NIPBL gene on 5p13 causes short stature + abnormal head shape (brows meet in midline) hirsutism, low-set ears, wide-spaced teeth, single palmar crease, IQ \200\201, \201 t