British Journal of Anaesthesia Anaesthesia 83 83 (1): 3–15 (1999)
Medical assessment of the paediatric patient A. E. Black Department of Anaesthesia, Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London WC1N 3JH, UK Br J Anaesth 1999; 83 : 3–15
Careful preoperative assessment is the cornerstone of safe anaesthetic practice as it allows for optimum planning of the child’s anaesthetic and perioperative care. Assessment encompasses evaluation of the child’s present health, past medica med icall and ana anaest esthet hetic ic his histor tory y, and rev review iew of rel releva evant nt investigatio inves tigations. ns. These facto factors rs are then integr integrated ated with the anticip ant icipate ated d eff effect ectss of sur surger gery y to all allow ow pla planni nning ng of an appropria appr opriate te anae anaesthet sthetic. ic. The impor importance tance of preop preoperativ erativee assessment has been highlighted in the recent publication The Anaesthesia Team which states that all patients should passs thr pas throug ough h a pre pre-ad -admis missio sion n rou routin tinee and tha thatt alt althou hough gh many aspects aspects of the assessment assessment can be delegated delegated to members members of the team, ultimately the anaesthetist is responsible for deciding that the patient is optimally prepared. 2 The medical assessment of the patient is only part of this process. Anaesthetists are constantly under time pressure to evaluatee pa at patie tient ntss ef efﬁc ﬁcie ient ntly ly.. Th Ther eree is a tr tren end d to ex expe pect ct th thee anaesthet anae sthetist ist to beco become me more of a ‘per ‘perioper ioperative ative medical specialist’ so ensuring that all medical issues which relate to the safe provision of anaesthesia are addressed. 20 64 The rolee of the pre rol preope operat rative ive ass assess essmen mentt cli clinic nic is bec becomi oming ng increasing incre asingly ly impor important, tant, parti particular cularly ly in the prep preparati aration on of the patient patient adm admitte itted d on the day of sur surger gery y. Whi While le mos mostt work wor k has bee been n rep report orted ed fro from m adu adult lt pra practi ctice, ce, pae paedia diatri tricc
thetic problems should be collected. A systematic approach is important. Factors such as birth and neonatal histories are very important important in the young child. child. Eva Evalua luatio tion n of the cardiac and respiratory systems and the airway is initially required, requ ired, with furth further er examin examination ation indicated acco according rding to clinical requirements. The majority of children admitted for surg su rger ery y ar aree he heal alth thy y, AS ASA A I or II II,, an and d ar aree un unde derg rgoi oing ng relatively relat ively minor sur surgery gery.. Comple Complex x paed paediatric iatric syndr syndromes omes and cong congenital enital malformations, malformations, although often rare rare,, may have important anaesthetic implications; these are reviewed in the specialist reference books and are outside the remit of this article.37 50 Liaison with specialist paediatric medical teams is frequently essential. It is necessary to evaluate the risks and beneﬁts associated with the procedure requiring general anaesthesia and any potential risk factors associated with anaesthesia so that these are discussed with the parents. This article discusses selected conditions, some of which occur occ ur com common monly ly,, suc such h as ast asthma hma,, dia diabet betes es and upp upper er respir res pirato atory ry tra tract ct inf infect ection ion.. Oth Others ers we are mee meeting ting wit with h increasing frequency because of the successes in paediatric care. This includes children with cardiac disease, those who have had transplants and those with neonatal problems.
pat patien ients ts tive are inc increa reasin singly gly being bei ngs. off offere ered d the preo facilit fac ilities iestive of preopera preo perative assessmen asse ssment t clinic clinics. Inadequate Inade quate preopera perative assessment may result in an increase in late cancellation of patien pat ients ts fro from m the the theatr atree lis listt and car carefu efull pre preope operat rative ive assessment encourages considered preoperative testing that is worthwhile and effective.2 21 This decreases unnecessary investigatio inves tigations ns which can be unple unpleasan asantt for the child and result in unnecessary cost and inconvenience to the hospital servic ser vice. e. Rou Routin tinee pre preope operat rative ive inv invest estiga igation tions, s, suc such h as measureme meas urement nt of haemo haemoglobi globin n conc concentra entration tion or urina urinalysis, lysis, 54 58 are not necessary in healthy children. Focusing investigations at speciﬁc patient groups who have particular medical requirements or surgical needs is much more worthwhile. Review of the child’s present and past medical history, and previous anaesthetic anaesthetic records is esse essential ntial for a usef useful ul
There are two gro There groups ups to con consid sider: er: those known to hav havee cardiac disease and those with symptoms or signs suggesting undiag und iagnos nosed ed car cardia diacc dis diseas ease. e. Con Congen genita itall hea heart rt dis diseas easee (CHD) occurs in approximately 0.8% of live births. Fifteen percent of these children also have an extracardiac abnormality ali ty and man many y nee need d inv invest estiga igatio tions ns or sur surger gery y req requir uiring ing anaest ana esthes hesia. ia. The pos possib sibilit ility y of a car cardia diacc les lesion ion sho should uld always be considered in any condition which is known to have such an association, for example tracheo-oesophageal ﬁstula, oesophageal atresia, Down’s syndrome and VATER association.37 50 Signiﬁcant cardiac disease is usually symptoma to mati ticc in ea earl rly y lif lifee wit with h mo most st CH CHD D id iden entiﬁ tiﬁed ed be befo fore re 3 months of age. Successful corrective or palliative surgery is now more frequently undertaken early, and this results in increasing
medical assessment to be completed. Information on current medication, history of allergies and family history of anaes-
numbers of children presenting for subsequent non-cardiac surgery. When cardiac surgery results in a structurally or
functionally normal heart, routine anaesthetic management is app approp ropria riate. te. Aft After er mor moree com comple plex x rep repair airs, s, ana anaest esthet hetic ic considera cons iderations tions need to be delic delicately ately balanced, balanced, and some children, such as those with a single ventricle physiology, should be cared for in a centre with cardiological support services.38 57 Children who have had corrective or palliative cardiac surgery and are well compensated do not have an increa inc reased sed mor mortali tality ty dur during ing sub subseq sequen uentt gen genera erall sur surger gery y. Theree is an increased Ther increased risk of mortal mortality ity,, parti particular cularly ly after
impairment and disse impairment disseminate minated d intra intravascu vascular lar coag coagulopa ulopathy thy being common. Clinical features of CHD outside infancy may be more appare app arent. nt. The These se inc includ ludee the dev develo elopme pment nt of cya cyanos nosis, is, clubbi clu bbing ng and oed oedema ema.. Res Respir pirato atory ry eff effect ectss may inc includ ludee wheeze, wheez e, decr decreased eased exer exercise cise tolera tolerance nce or brea breathles thlessness sness.. Childr Chi ldren en wit with h CHD are at inc increa reased sed risk of res respir pirato atory ry infect inf ection ions. s. Gas Gastro trointe intesti stinal nal sym sympto ptoms, ms, inc includ luding ing poo poorr appetite, appe tite, failu failure re to gain weigh weightt and hepat hepatosple osplenomeg nomegaly aly,,
emergency surgery, in babies less than months who remain rema in phys physiologi iologically cally compromise compr omised d30 6Morta Mortality lityold related relat ed speciﬁcally to anaesthesia in children undergoing corrective cardiac surgery is rare and the risks can be related to the ASA status of the child, as with other surgery.31 CHD has been classiﬁed in many ways and may be very varied var ied and com comple plex. x. The pat pathop hophys hysiol iology ogy may fal falll int into o several categories: shunts, mixing, obstruction and valvular regurgit regu rgitation, ation, with compl complex ex anoma anomalies lies havin having g a combin combination ation of these components. The likely effect of a speciﬁc cardiac lesion needs to be assessed. Lesions such as atrial and ventricular septal defects defects (ASD, (AS D, VSD VSD), ), and end endoca ocardi rdial al cus cushio hion n def defect ectss res result ult in normal nor mal or ove overpe rperfu rfusio sion n of the lun lungs gs and ar aree the theref refore ore usually acyanotic. Excessive blood ﬂow to the lungs, with
may Somelow children congestive cardiac failure have occur. a persistent grade with pyrexia without any identiﬁable infect inf ective ive sou source rce.. Inc Increa reasin sing g sym sympto ptomat matolo ology gy ind indica icates tes poorly controlled disease. Antibiotic prophylaxis, for the prevention of endocarditis, is required for all invasive procedures, but local procedures vary. Prophylaxis is not needed routinely for children who have ha ve ha had d li liga gatio tion n of a PD PDA, A, AS ASD D or ro rout utin inee ca card rdia iacc catheterization.
left to right shunting of blood, results in ventricular volume overload. This produces early signs of congestive cardiac failure as volume load is less well tolerated than pressure load. Unexplained cyanosis always requires further investigation and in a neonate this is urgent. Lesions that result in underperfusion of the lungs because of right to left shunting such as tetralogy of Fallot, result in cyanosis. Conditions with wit h com comple plex x shu shunts nts,, for exa exampl mplee tra transp nsposi osition tion of the greatt arter grea arteries, ies, are also usua usually lly asso associated ciated with cyan cyanosis. osis. Obstructive lesions, including coarctation of the aorta, aortic or pulmonary valvular stenosis, or interrupted aortic arch, result in ventricular pressure overload which the paediatric myocardium is more able to accommodate for longer, with fewer symptoms, than an increased volume load.
orders, such as muscu orders, muscular lar dystr dystrophy ophy.. Eisen Eisenmenge menger’s r’s syndrome dro me can res result ult whe when n lon long-s g-stan tandin ding g lef leftt to rig right ht intr intraca acardi rdiac ac shunt, such as is present in uncorrected VSD, ASD, PDA and AVSD, AVSD, results in increasing PHT until the shunt reverses produc pro ducing ing a rig right ht to lef leftt shu shunt nt and cya cyanos nosis. is. Sym Sympto ptoms ms of PHT includ includee brea breathless thlessness ness,, parti particular cularly ly on exer exercise, cise, syncope and later, cyanosis, cough and haemoptysis. Sudden death may occur occur.. Signs of right ventricular ventricular hypertrophy hypertrophy includee char includ character acteristic istic features features on chest x-ra x-ray y and on the ECG. Serial ECG and echocardiography provide a measurement of the progressive nature of the illness. Polycythaemia is a late feature. Children with primary PHT may have few symptoms. Anaesthetic Anaes thetic manag management ement requ requires ires balan balancing cing the anae anaessthetic the tic tec techni hnique quess and the therap rapeut eutic ic age agents nts whi which ch hav havee an effect on pulmonary vascular resistance (PVR).9 The degree of rev revers ersibi ibility lity of PHT is ass assess essed ed at ang angiog iograp raphy; hy; thi thiss information may be important when planning which agents may be requ required ired during anaes anaesthesia thesia..
Pulmonary hypertension (PHT) PHT is ra PHT rare re an and d th thee co cond nditi ition on is us usua ually lly se seco cond ndar ary y to congenital conge nital heart disease, chronic airwa airways ys disea disease, se, upper airway air way obs obstru tructio ction, n, ade adenot notons onsilla illarr hyp hypert ertrop rophy hy,, cys cystic tic ﬁbrosis, bronchopulmonary dysplasia or neuromuscular dis-
Clinical features Neonates and young infants with CHD usually present with features of congestive cardiac failure, including tachypnoea, sweati swe ating ng or hep hepato atomeg megaly aly.. Oth Other er fea featur tures es may inc includ ludee cyanosis, cyan osis, incre increasing asing polyc polycythae ythaemia, mia, histo history ry of recu recurren rrentt respiratory infections, wheeze or a generalized pattern of the child failing to thrive. Identiﬁcation of a murmur on routine neonatal review may be the ﬁrst sign of CHD. Neonates with coarctation of the aorta, aortic stenosis, hypoplastic left heart syndrome, or interruption of the aortic arch ar ch or pu pulm lmon onar ary y at atre resi siaa ha have ve a ci circ rcul ulat atio ion n wh whic ich h is dependent on maintenance of the patent ductus arteriosus (PDA) to provide pulmonary blood ﬂow. Prostaglandin E1 is used to prevent closure of the duct. These babies may
The innocent murmur Detection of a murmur on routine preoperative assessment is co comm mmon on.. In Inno noce cent nt mu murm rmur urss ha have ve be been en re repo port rted ed in 8–80% of children. It is important to distinguish between innocent and pathological murmurs, but this can be difﬁcult. The respective features are summarized in Table 1. In th thee as asym ympt ptom omat atic ic ch child ild wit with h a mu murm rmur ur,, th thee two condit con dition ionss whi which ch nee need d to be exc exclud luded ed are hyp hypert ertrop rophic hic obstructiv obstr uctivee card cardiomyop iomyopathy athy and critic critical al aortic stenosis.
be very sick, sic k, of with wit h featur fea tures es multis tisyst ystem failur fai e and need nee dver ayper period iod stabili sta bilizat zation ionofin mul intensi inte nsive veem care. car e. lure Medica Med icall assess ass essmen mentt foc focuse usess on end end-or -organ gan fun functi ction, on, with ren renal al
An ECG leftax ventricular ventr icular hypertr ophy (RV 6e SV1 5 mV mV)) shows and an d le left ft axis is de devi viat atio ion nhypertrophy in bo both th of (RV6 thes th ese conco n14 52 ditions.
Medical assessment of the paediatric patient
Features of cardiac murmurs Table 1 1 Features Innocent
Asymptomatic Soft Ea rl rly syst ol oli c No thrill Disappears with positioning May be a venous hum
Symptomatic Loud Pan or la te te systolic di as ast ol oli c conti nu nuous Thrill
Children who have murmurs with features of an innocent nature, who have no signs or symptoms of cardiac disease and in whom an ECG and possibly echocardiography have been reviewed to ensure there are no signs of ventricular hypertrop hype rtrophy hy,, can be anaes anaesthetiz thetized ed safe safely ly and refe referred rred for cardiological review later (Fig. 1). Some centres use antibiotic prophylaxis routinely in this group. All infants, and those children whose murmurs show pathological features, should sho uld be rev review iewed ed by a pae paedia diatri tricc car cardio diolog logist ist bef before ore 52 anaesthesia.
Anaesthetic assessment Elective non-cardiac surgery must take place when both the child’s general health and their cardiac status are optimal. Assessment initially involves understanding the particular pathophysiology involved. The basic information required includes details of the type of cardiac repair performed and the lik likely ely seq sequel uelae ae of the rep repair air.. Con Consid sidera eration tion mus mustt be given to the present physical condition of the child and the current medical treatment, particularly anti-failure therapy, β-bloc -blockers kers,, antico anticoagula agulants, nts, antih antihypert ypertensiv ensivee medic medication, ation, digoxin digox in or aspir aspirin. in. Blood concentratio concentrations ns of digox digoxin in may need to be checked. If a pacemaker is present, its routine preoperative evaluation is required. Childr Chi ldren en with a sig signiﬁ niﬁcan cantt car cardia diacc his histor tory y sho should uld be reviewed before operation by a paediatric cardiologist and those with signs of cardiac failure or who have arrhythmias need to be reviewed by the paediatric team and their medical condition optimized before surgery. If the child is having regular, infrequent reviews by the paediatric team, the most recent rec ent rep report ort of a sta stable ble sit situat uation ion will pro provid videe suf sufﬁci ﬁcient ent information. Children Childr en with cardi cardiac ac disea disease, se, unde undergo rgoing ing nonnon-card cardiac iac surgery, routinely have an ECG, precordial Doppler echocardiography,, chest x-ray cardiography x-ray,, and laboratory tests, including i ncluding full blood count, urea and electrolytes, creatinine or coagulation studie stu dies, s, as ind indica icated ted.. In add additio ition, n, som somee chi childr ldren en req requir uiree review revie w of their angio angiograp graphy hy resu results. lts. The resu results lts of these invest inv estiga igation tionss tak taken en tog togeth ether er all allow ow ass assess essmen mentt of the their ir current cardiac status. The che chest st x-r x-ray ay pro provid vides es an ind indica icatio tion n of hea heart rt siz size, e, degree of pulmonary vascularity and the presence of intrapulm pu lmon onar ary y pa path thol olog ogy y, su such ch as in infe fect ctio ion. n. An EC ECG G is reviewed, revie wed, noting particularly particularly the pres presence ence of arrh arrhythmia ythmia,,
Fig 1 Preoperative assessment of the child with a murmur.52
incidence of arrhythmias. Echocardiography is one of the mostt use mos useful ful inv invest estiga igation tionss in the child wit with h CHD CHD.. Ech Echoocardiography deﬁnes cardiac structure and function. Trans Tr ansoes oesoph ophage ageal al ech echoca ocardi rdiogr ograph aphy y is bei being ng use used d increasingly in the assessment of paediatric patients, particularly in adolescents in whom a better quality study can be achieved with this method. However, it is invasive, has an incidence of complications and requires general anaesthesia or heavy sedation.35 Angiography is used more selectively in the assessment of the cardiac patient as improvements in echocardiography havee all hav allowe owed d suf sufﬁci ﬁcient ent inf inform ormati ation on to be gat gather hered ed les lesss invasively. It remains particularly useful in determining the coronary anatomy, anatomy, for assessing multifocal pulmonary blood ﬂow and for measuring speciﬁc haemodynamic information, such as pulmonary artery pressures. A baseline S aO2 is useful before anaesthesia as signiﬁcant cyanosis indicates inadequate pulmonary blood ﬂow, right to le left ft sh shun untin ting g or mix mixin ing. g. In a ch child ild wi with th te tetr tral alog ogy y of
and eviden evi ce of rep ventri ven tricul cular hypert hyp ertrop rophy . Som Some cardia car diac c repair rep airs, s, dence such suc h as repair air of artet tetral ralogy ogy ofhy.Fal Fallot lot,, eMus Mustar tard, d, Senn Se nnin ing g or Fo Font ntan an re repa pair irs, s, ar aree as asso soci ciat ated ed wi with th a hig high h
Fallot, presence and frequency of management cyanotic spells are checkedthe and their severity and routine noted. Long-term cyanosis can be complicated by polycythaemia,
hyperviscosity syndrome or coagulopathy. This may result in decr decreased eased card cardiac iac funct function, ion, cere cerebrov brovascu ascular lar occlu occlusive sive episodes, episo des, cere cerebral bral absce abscesses sses or throm thromboemb boembolism. olism. Occa Occa-sionally, if surgery cannot improve pulmonary blood ﬂow, the chi child ld may req requir uiree reg regula ularr ven venese esecti ction on to dec decrea rease se morbidity morb idity from polyc polycythae ythaemia. mia. Venese enesection ction just befo before re anaesthesia should be avoided as it may be accompanied by cardiovascular instability. Polycythaemic children must be kep keptt wel welll hyd hydrat rated ed and ﬂui ﬂuid d res restri tricti ction on min minimiz imized. ed.
apnoea apno ea ar aree st stil illl at ri risk sk of ap apno noea eass af afte terr su surg rger ery y an and d anaesthesia. Quantifying the risk is difﬁcult and monitoring this group with an apnoea monitor and oxygen saturation monitor is essential after operation. The age at which the ex-preter ex-p reterm m baby is no longer at risk has been deba debated. ted. This questi que stion on has imp implic licati ations ons for the man manage agemen mentt of you young ng babies bab ies for minor sur surger gery y on a day day-ca -case se bas basis. is. Cot Cotee´ and colleagues attempted to quantify the actual risk of apnoea and rep report orted ed a com combin bined ed ana analys lysis is of eig eight ht pre previo viousl usly y
Chi Childr ldren en with can hsaf safely ely con contin tinue ue rec receiv clear cle ar 56eiving ﬂuids orally up CHD until 2–3 before operation. Ifing a longer preope pre operat rative ive fas fastt is ant anticip icipate ated, d, pre preope operat rative ive i.v i.v.. ﬂui ﬂuids ds should be given.
published studies. concluded there was a 5% risk of apnoea in aThey neonate at 48 weeks PCAatifleast the child was born at 35 weeks and that this same group had a 1% risk of apnoea at a PCA of 54 weeks. 11 The risk increased as PCA decreased. decreased. Iden Identiﬁed tiﬁed risk facto factors rs included gestational age, PCA, history of apnoeas, occurrence of apnoeas in the rec recove overy ry roo room m and ana anaemi emia. a.11 Adminis Administratio tration n of caffeine may be successful in preventing apnoeas in this group of patients.79 Spinal anaesthesia may be associated with a low lower er inc incide idence nce of pos postop topera erativ tivee com compli plicat cation ions, s, including apnoea, hypoxia and bradycardia. 28 45 However, it is useful for only a limited number of surgical procedures. Complication Compli cationss occu occurr with spina spinall anae anaesthes sthesia, ia, parti particular cularly ly inadequate anaesthesia and high spinal block. 24 If sedation or even ‘light’ anaesthesia is used in addition to the spinal
Respiratory disorders Neonates and infants The suc succes cesss of neo neonat natal al car caree has mea meant nt tha thatt inc increa reasin sing g numbers numbe rs of babie babies, s, who may have complicated complicated neon neonatal atal histories, require surgery for a wide variety of conditions. Some present addit additional ional problems durin during g anae anaesthes sthesia ia and many have an increased risk of postoperative complications. The group most frequently studied are babies undergoing repair of inguinal hernia, a condition which is much more common in the preterm neonate. All babies require a careful assessment of their medical history with speciﬁc attention to their birth history, gestational age at delivery and age at surgery sur gery,, which is usual usually ly expr expressed essed as postpost-conc conception eptional al age (PC (PCA). A). Pre Preter term m del delive ivery ry,, deﬁ deﬁned ned as del delive ivery ry at les lesss than tha n 37 wee weeks’ ks’ pos post-c t-conc oncept eption ional al age age,, is kno known wn to be associated with an additional risk of postoperative complications, particularly apnoea and bradycardia. The baby’s present physical condition is determined, with particu par ticular lar emp emphas hasis is on a his histor tory y of pro prolon longed ged int intens ensive ive neonatal care, degree of respiratory reserve, possibility of subglottic subg lottic steno stenosis, sis, pres presence ence of conge congenital nital abno abnormalit rmalities, ies, likeli lik elihoo hood d of ana anaemi emiaa and inf inform ormatio ation n on pro provis vision ion of vitami vit amin n K pro prophy phylax laxis is for pre preven ventio tion n of hae haemor morrha rhagic gic
anaesthet anaest hetic, ic, the inc incide idence nce of apn apnoea oea is not dec decrea reased sed..81 Postoperative apnoea can occur up to 48 h after surgery 47 and there there is no con consen sensus sus as to whe when n the risk becomes becomes 22 45 78 negligible. Apnoeas have been reported in term babies after anaesthesia but are rare and may be related to other medical factors.1 68 A full blood count is routinely carried out on all neonates and preterm preterm inf infant antss as it is dif difﬁcu ﬁcult lt to ide identi ntify fy ana anaemi emiaa clinic cli nicall ally y in thi thiss age group group and it is ass associ ociate ated d with an increased risk of postoperative apnoeas in former preterm babies.80 Haemoglobin concentration is related to the degree of maternal transfusion at birth. The normal haemoglobin concentration in the premature neonate is 18 g dl –1 decreasing to 17 g dl–1 in the term baby and to 10 g dl –1 by 6 months of age. Babies who have prolonged hospital stays are fre freque quentl ntly y ana anaemi emicc bec becaus ausee of rep repeat eated ed blo blood od tes testt requireme requ irements. nts. Card Cardiores iorespirat piratory ory rese reserve rve is very limited in these babies and preoperative correction of anaemia when present may be required.
disease of the newborn. A history of apnoea and bradycardic episod epi sodes es is imp import ortant ant as is a his histor tory y of a con continu tinuing ing or recent additional oxygen requirements, which indicates that the baby may requ require ire addit additional, ional, short-term, short-term, resp respirator iratory y support after operation.
Assessment of respiratory function is reliant on the history and clinical features. Normal newborn babies, born prematurely, are known to have apnoeas of central, obstructive or mixed type. The commonest type of apnoea is that of a centra cen trall aet aetiol iology ogy,, but an obs obstru tructiv ctivee or mix mixed ed pat patter tern n of ap apno noea ea is as asso soci ciat ated ed wi with th mo more re se seve vere re ep epis isod odes es of hypoxaemia.46 Apn Apnoea oeass are fre freque quently ntly acc accomp ompani anied ed by
BPD occ occurs urs as a res result ult of res respir pirato atory ry dis distre tress ss syn syndro drome me which is associated with preterm delivery and the effects of mechanical ventilation. Features of this condition include signs of respiratory compromise related to hyperinﬂation, develo dev elopme pment nt of emp emphys hysema ema or bul bullae lae,, ris risk k of pne pneumo umotho thorax rax,, increased incre ased reactivity reactivity of the airwa airways ys and incre increased ased risk of respir res pirato atory ry inf infect ection ion (Fi (Fig. g. 2). The These se bab babies ies hav havee poo poorr
bradycardia. A sleep study canvery identify the of apnoea but it is not a sensitive test nor useful astype a preoperative 45 47 assessment tool. Premature babies with no history of
pulmonary compliance a resultresistance of development of reteninterstitial ﬁbrosis, increasedasairway and ﬂuid tion. They often remain oxygen dependent, dependent, and diuretics
Medical assessment of the paediatric patient
type, fre type, freque quency ncy and sev severi erity ty of ast asthma hma in an ind individ ividual ual child. chi ld. It is unw unwise ise to und undert ertake ake ele electi ctive ve sur surger gery y with within in 4 weeks of a major exacerbation of asthma. Anaesthesia with tracheal intubation, following recent exacerbation of asthma, is potentially associated with respiratory complications, particularly cough, bronchospasm, increased risk of pneumothorax and postoperative ventilation.48 When seen for anaesthetic review before operation, the child should be well and the asthma should be satisfactorily 29
controlled withthe thechild individual’s If this is not the case should bemedical referredregimen. to the paediatric team tea m and med medica icall the therap rapy y max maximiz imized. ed. Thi Thiss may req requir uiree several days preoperative admission. If the child has symptoms of an upper respiratory tract infection, elective surgery should be postponed as an increase in respiratory complicationss and exa tion exacer cerbat bation ion of ast asthma hma cau caused sed by inc increa reased sed responsiv resp onsivenes enesss of the airwa airways ys have been repor reported ted unde underr these circumstances.34 60 Assess Ass essmen mentt of the chi child’ ld’ss res respir pirato atory ry fun functi ction on is bes bestt achieved by looking for signs of respiratory distress, including tachy tachypnoea pnoea,, use of acce accessor ssory y muscl muscles es of resp respiratio iration, n, and the presence of wheeze or focal signs on examination of the chest chest.. More formal formal testin testing g of pulmonary pulmonary func function tion can be used successfully in children more than 7 yr of age
Fig 2 X-ray showing showing chro chronic nic lung disease, hype hyperinﬂ rinﬂatio ation n and patch patchy y inﬁltration.
and steroids are frequently required. A preoperative chest x-ray x-r ay is use useful ful and blo bloodod-gas gas ana analys lysis is may ind indica icate te the severity of disease if carbon dioxide retention is present, with or without mild hypoxia. Spinal anaesthesia has been 28
advocated for hernia repair in this group of patients. Babies Bab ies who hav havee bee been n intu intubat bated ed may hav havee sub subglo glottic ttic stenos ste nosis is and this may bec become ome app appare arent nt dur during ing or aft after er anaesthesia, either when a relatively small tracheal tube is used in relation to the size and age of the child or if stridor develops in the postoperative period. The use of atropine as a premedicant is valuable in these babies as it decreases both airway reﬂexes at laryngoscopy and the likelihood of bradycardia. This group of patients may beneﬁt from i.m. atropine premedication as its effect is more reliable than when administered orally orally..25 They may be more likely to have gaseous induction as it is not always easy to achieve vascular access.
Mild asthma featuring episodes of wheezing is extremely comm co mmon on in yo youn ung g ch child ildre ren n an and d ea easi sily ly ma mana nage ged d wi with th bronchodilators. Asthma tends to occur in children with a history of atopy. There is some evidence that the incidence of this condition is increasing, possibly because of environmental factors.77 The majority of children outgrow this type of mild asthma. Severe symptoms occur in less than 10% of children with asthma and require much more aggressive treatment.7 12 Guidelines for the management of childhood asthmaa have recently been published. asthm published.7 Repeated hospital admi ad miss ssio ions ns ma may y be re requ quir ired ed to ma maxi ximi mize ze th ther erap apy y an and d intensive care management is occasionally necessary. This
if the they y are able to coco-ope operat ratee wit with h the procedur procedure. e. Thi Thiss allows the degree of reversibility of the airway resistance to be de dete term rmine ined d an and d th thee am amou ount nt of im impr prov ovem emen entt in pulmonary function tests, with treatment, to be measured. Some children keep regular charts of daily peak ﬂow which allows interpretation of preoperative values in the context of their expected respiratory function. The measured small decrease decr ease in respi respirator ratory y func function tion (FEV1 and FEF FEFR) R) aft after er anaesthes anae sthesia ia and surgery in childr children en with mild, well-c well-conontrolle tro lled d ast asthma hma is the same as tha thatt for children children without without asthma.51 Respiratory mechanics are not different between asthmatic asthm atic and non-a non-asthma sthmatic tic childr children en when anaes anaesthetiz thetized ed 27 with either propofol or halothane. Routine chest x-ray is not required in mild asthma but may ma y be us usef eful ul in mo more re se seve vere re ca case sess to ex excl clud udee ac acut utee infection, the presence of bullae, hyperinﬂation or pneumothorax. Blood-gas analysis is rarely useful in the assessment of asthmatic children but recording of oxygen saturation in air can provide helpful baseline data. Before operation, all children should continue receiving their the ir reg regula ularr med medica ication tion and use the their ir inh inhale alers rs bef before ore anaesthesia. Additional steroid cover may be required for those receiving receiving regu regular lar steroid medic medication ation and for those who have been receiving steroids in the preceding 2 months who may potentially have a degree of adrenal suppression. Inhaled steroids alone do not cause suppression of steroid production prod uction and addit additional ional steroid cover is not required16 (Table 2). High-dose methylprednisolone is used in some centres in severe asthmatics.48
pattern although much less frequent, can be very difﬁcultoftoasthma, manage. A car carefu efull his histor tory y is of mos mostt val value ue in det determ ermini ining ng the
Childr Chi ldren en rec receiv eiving ing theoph ophylli ylline ne may need nee to hav havee µcon concen cen–1trations checked. Thethe therapeutic range isd10–20 g ml . Occasionall Occas ionally y, amino aminophylli phylline ne infus infusions ions are requ required ired in the
Steroid cover for surgery 16 Table 2 2 Steroid On steroids at present
Hydrocortisone 1 mg kg–1 i.v.
1. At induction 2. Every 6 h i.v. after operation until able to take steroids orally 3. Reduce to maintenance level over next 4 days, as tolerated
Offf st Of ster eroi oids ds in pr prec eced edin ing g 2 mo mont nths hs
Hydr Hy droc ocor orti tiso sone ne 1 mg kg–1 i.v.
1. With premedication 2. Every 6 h after operation for 24–48 h 3. Review need for steroids
Off steroids for longer than 2 months
1. No cover but hydrocortisone should be available
peroperative peropera tive perio period d in poorl poorly y contr controlled olled asthmatics and lavage for diagnostic purposes, nasal polypectomy, gastrocareful care ful monito monitoring ring of theop theophyllin hyllinee conc concentra entrations tions is then stomy to aid feeding, insertion of indwelling i.v. lines and essential. vascular access ports for supportive therapy, administration Premed Pre medica ication tion is use useful ful as the str stress ess of sur surger gery y may of nutritional support and long-term medication. prec pr ecip ipita itate te an at atta tack ck of br bron onch chos ospa pasm sm.. Th Thee ch choi oice ce of This group is incre increasing asingly ly being offered offered hear heart–lun t–lung g or anaesthetic is affected by the selection of drugs used and lun lung g tra transp nsplan lantat tation ion as the their ir con condit dition ion det deteri eriora orates tes and thos th osee wh whic ich h ma may y pr pred edis ispo pose se to hi hist stam amin inee re rele leas asee an and d success is reported with this intervention for the treatment bronchoconstriction are avoided. Use of non-steroidal anti- of end stage respiratory disease.66 82 inﬂammatory drugs (NSAID) is debated. They should be avoided in severe asthma, although in milder forms they Assessment and investigations. Assessment of the child with may be safe. Techniques which avoid tracheal intubation, CF aims to identify the extent of the disease. This is initially when appropriate for surgery, may be less likely to provoke from the clinical history, noting particularly the severity of cough and productivity of sputum, frequency of respiratory bronchospasm.60 infections, amount of physiotherapy support required and
Cystic ﬁbrosis (CF)
degree of limitation of exercise. Review by the paediatric team tea m and maximizat maximization ion of med medica icall the therap rapy y are the pre pre-operative oper ative goals. Signs of resp respirator iratory y compr compromise omise include tachyp tac hypnoe noea, a, hyp hyperi erinﬂa nﬂatio tion n of the che chest, st, cra crackl ckles es and wheeze, wheez e, and a prolo prolonged nged expiratory expiratory phase of resp respiratio iration. n. The child should not have any signs of acute respiratory infection. Preoperative S aO2 in air provides a useful baseline. Blood-gas analysis is helpful late in the disease when an increase in carbon dioxide partial pressure is indicative of decompens deco mpensated ated resp respirato iratory ry disea disease se and may incre increase ase the likelihood of postoperative ventilation. Pulmonary function tests allow documentation of the degree of impairment and extent ext ent to whi which ch res respir pirato atory ry fun functi ction on can be imp improv roved ed with bronchodilator bronchodilators. s. Some children with CF are receiving oxygen at home, which indicates that postoperative ventila-
This mul This multis tisyst ystem em dis diseas easee of exo exocri crine ne gla glands nds res results ults in damage to the lung, pancreas and hepatobiliary system. It is in inhe heri rite ted d in an au auto toso soma mall re rece cess ssiv ivee ma mann nner er an and d is common, occurring in 1:2000 of the population. CF presents as me meco coni nium um ile ileus us in th thee ne neon onat atee or la late terr wi with th co coug ugh, h, wheeze, recurrent chest infections, clubbing and a generalized failure to thrive. Improvements in paediatric care have enhanc enh anced ed bot both h the quality quality and length of lif lifee for patients patients with wit h CF and mos mostt chi childr ldren en now rea reach ch adu adulth lthood ood.. Man Many y childr chi ldren en with CF rem remain ain rel relativ atively ely wel welll on reg regime imens ns of regular physiotherapy, prophylactic antibiotics, aggressive managemen mana gementt of acute infection, infection, use of panc pancreatic reatic supplements and nutritional support. The resp respirator iratory y disea disease se usua usually lly pred predomina ominates, tes, resu resulting lting in a progressive deterioration of lung function caused by excessive, viscous tracheobronchial secretions and impaired mucociliary clearance mechanisms. The pattern of respiratory deﬁcit is of a mixed obstructive and restrictive nature. Long-t Lon g-term erm bro bronch nchiec iectas tasis, is, ﬁbr ﬁbrosi osiss and chr chroni onicc air airway way obstruction obstr uction may devel develop, op, resu resulting lting in resp respirato iratory ry failu failure re and cyanosis. Hypercapnia occurs late in the disease with increasing incre asing venti ventilation lation–per –perfusio fusion n misma mismatch. tch. Chro Chronic nic hypo hypoxia xia may lead to pulmo pulmonary nary hypertension hypertension,, cor pulmonale and eventu eve ntuall ally y car cardia diacc fai failur lure. e. Mos Mostt of the mor morbid bidity ity and mortal mor tality ity of CF is rel relate ated d to res respir pirato atory ry inv involv olveme ement. nt.18 Liver disease may result in poor function, coagulopathy or varice var ices. s. Dia Diabet betes es occ occurs urs in app approx roxima imatel tely y 12% of CF patients.
tion may be nee tion needed ded,, esp especi ecially ally if the pro propos posed ed sur surger gery y causes cau ses spl splint inting ing of the dia diaphr phragm agm.. ECG det detect ectss any evi eviden dence ce of rig right ht hea heart rt str strain ain and ven ventric tricula ularr hyp hypert ertrop rophy hy.. Oth Other er featur fea tures es of car cardia diacc impa impairm irment ent suc such h as the pre presen sence ce of peripheral oedema or hepatomegaly may also be present. Diabetes is managed as routine for diabetic patients. A full blood count reveals anaemia, usually caused by poor nutrition, or increase in leucocyte count with chronic infection. The presence of signiﬁcant liver disease is identiﬁed by increased liver enzyme levels, decreased albumin concentrations and abnormalities of coagulation, which may need to be corrected before operation. A chest x-ray may reveal chronic inﬁltration of the lung, local consolidation, bronchiec bron chiectasis tasis,, cardi cardiac ac enlar enlargemen gementt or peric pericardia ardiall or pul-
Anaesthesia required for all interventions common paediatric surgical conditions andisalso for speciﬁc related to the manage man agemen mentt of CF CF.. The These se inc includ ludee bro bronch nchiol iolar ar alv alveol eolar ar
monary effusions (Fig.of3). Features indicative end-stage disease include an FEV1 less than 30%, hypoxic or hypercapnic respiratory failure,
Medical assessment of the paediatric patient
of laryngospasm. In practice, only 0.5% of patients in this study actually had their surgery postponed.65 Prudent Prude nt judge judgement ment is requ required ired before proceeding proceeding with elective anaesthesia when a child is symptomatic of URTI or is recovering from a URTI. The difﬁculty when assessing the child comes in quantifying the risk. Several retrospective and prospective studies have examined if there is actually an increase in respiratory complications in children with a URTI and the evidence is conﬂicting. Several points should be note no ted. d. Fi Firs rst, t, mo most st stud st udie ies s ha have ve ASA incl in clud uded main ma inly ly or exclusively day-case patients who are I ed or II. Second, the diagnostic criteria for URTI vary. Most studies exclude children who are systematically unwell, those with a fever greate gre aterr tha than n 38° 38°C, C, and those who hav havee whe wheeze eze or oth other er chest signs on auscultation of the chest. If URTI is a risk factor, what other factors increase that risk ri sk?? Ch Child ildre ren n ag aged ed le less ss th than an 1 yr ap appe pear ar to ha have ve an increased incre ased incidence incidence of airwa airway y compl complicatio ications ns as do those anaestheti anae sthetized zed by less expe experienc rienced ed anaes anaesthetis thetists ts and those 10 65 undergoing airway surgery surgery.. Tracheal Tra cheal intuba intubation tion may also increase the likelihood of an intraoperative respiratory event but the reported incidence of this complications varies considerably. In Cohen and Camer Cameron’ on’ss study study,, cough cough,, laryn laryngospa gospasm, sm,
Fig 3 X-ray X-ray show showing ing cyst cystic ic ﬁbro ﬁbrosis, sis, hype hyperinﬂ rinﬂatio ation, n, peri peribron bronchial chial thickening and bronchiectasis.
with or without cor pulmonale, decreasing exercise tolerance, increasingly frequent and prolonged hospital admission si ons, s, an and d fa failu ilure re to ga gain in we weig ight ht de desp spit itee at atte temp mpts ts to 41
supplement the diet. Poor preoperative nutritional status is an important factor and associated with increased mortality after major surgery, such as transplantation.66 Frequently this situation can be improved with early nutritional support. Gastro-oesophegeal reﬂux may also be present. Review of the child’s microbiological results may reveal bacterial bacte rial colon colonizatio ization n freq frequentl uently y with multipl multiply y resi resistant stant organism org anisms. s. Preca Precautions utions to avoid cross infec infection tion are very important.
bronchosp bronch ospasm asm and dec decrea rease se in oxy oxygen gen sat satura uratio tion n wer weree reported to be increased 2–7 times in children with URTI undergoing anaesthesia and by 11 times if intubation of the trachea was required.10 However, Tait and Knight’s study showed sho wed no inc increa reased sed ris risk k of com complic plicati ations ons in chi childr ldren en who wer weree sym sympto ptomat matic ic or non non-sy -sympt mptoma omatic tic of a UR URTI TI undergoing minor surgery surgery..71 They subse subsequent quently ly repor reported ted that the increase in complications occurred mainly in those children who had a recent infection, particularly if they had been bee n int intuba ubated ted,, rat rather her tha than n in tho those se with current current UR URTI, TI, 72 even eve n tho though ugh 24% had pos positiv itivee vir viral al cul cultur tures. es. Children who had rec receiv eived ed an ana anaest esthet hetic ic dur during ing a UR URTI TI had a shorter shor ter dura duration tion of illnes illness. s.71 Some anaesthetic agents are known to inhibit viral growth in vitro and the mechanisms resulting in hypersensitivity of the respiratory system are complex and multiple.34 Children with URTI are also more likely like ly to hav havee mor moree tra transi nsient ent dec decrea reases ses in S aO2 in th thee perioperative period.17 42 49 Perhaps Perha ps the most important factors are pres present ent history and parental opinion. Schreiner and colleagues noted that the pare parents’ nts’ interpretation interpretation of their child’s child’s sympt symptoms oms was more accurately associated with risk of laryngospasm than assessment of URTI features.65 On the day of surgery, these factors need to be taken into account when evaluating the child. A chest x-ray and leucocyte count are occasionally indicated. Those with mild symptoms of runny nose, sneeze, mild fever 38°C and mild cough could be considered for surgery after canvassing pare pa rent ntal al op opin inio ion n as to wh whet ethe herr th thee ch chil ild d is ge gene nera rally lly
Upper respiratory tract infection (URTI) URTI occur commonly in childhood with a reported frequen qu ency cy of 2– 2–9 9 ep epis isod odes es pe perr ye year ar in th thee no norm rmal al ch child ild.. Chronic nasal discharge, with features very similar to URTI, is also common, particularly in a child who suffers from adenoidal hypertrophy. Some studies show an increased incidence of complications during anaesthesia for up to 6 weeks after a URTI.73 Ther Th eree ar aree sp spor orad adic ic re repo port rtss in th thee lit liter erat atur uree of se seve vere re morbid mor bidity ity and indeed indeed mor mortal tality ity whe when n the pre presen sence ce of a URTI is the only preoperative indication of any disorder. Complete Compl ete lung collap collapse, se, pneum pneumothor othorax ax and myoca myocarditis rditis aree so ar some me of th thee ma majo jorr, ra rare re ev even ents ts wh whic ich h ha have ve be been en 36 43 83 reported. It has been recommended that surgery is postponed for 4–6 weeks after each URTI. This may cause considera cons iderable ble upset to the child and inconvenience inconvenience to the parent and hospital service if frequent cancellations occur. Schreiner and colleagues, in their study of more than 15 000
children undergoing procedures, estimated that if all children with mildday-case symptoms had been postponed, 2000 children child ren would have been canc cancelled elled to preve prevent nt 15 episo episodes des
unwell. with moderate tohsevere URTI have featur fea tures es of Those system sys temic ic illn illness ess,, suc such as mya myalgi lgia, a,who pyrexi pyr exia, a, anorexia, malaise or headache, and those with a productive
cough and signs of a lower respiratory tract infection should be postponed for 4–6 weeks.73
progressi progre ssive ve con conditi dition. on. The chi child’ ld’ss his histor tory y ide identi ntiﬁes ﬁes the possib pos sibilit ility y of end end-or -organ gan dam damage age and tho those se tha thatt req requir uiree additional preoperative investigations. Relatively common problems prob lems inclu include de acute ches chestt synd syndrome, rome, cere cerebrova brovascula scularr accidents, hepatosplenomegaly, splenic sequestration crisis and aplastic crisis. Chro Chronic nic lung damage, resulting from multiple pulmonary crises and repeated infection, is manifested feste d as pulmon pulmonary ary ﬁbrosis and rest restrictiv rictivee lung disease. Long term, this may result in pulmonary hypertension and
Sickle cell disease (SCD) Children with SCD may present for many types of surgery. The com common monest est pro proced cedure uress lin linked ked to the their ir dis diseas easee are cholec cho lecyst ystect ectomy omy for gal galll sto stones nes and spl splene enecto ctomy my,, but incide inc identa ntall sur surger gery y inc includ ludes es all fre freque quentl ntly y und undert ertake aken n paediatric surgical conditions.44 As always, elective surgery is associated with a lower complication rate than emergency procedures and allows time for careful reasoned management of the child. Preparation of children with SCD needs close liaison with the haematological and surgical teams. Screening
Children who come from an ethnic group which has a high incidence of SCD are screened routinely using the Sickledex test which detects HbS to values of 20–25%. It does not identify ident ify the conce concentrati ntration on or the accom accompany panying ing types of haemog hae moglob lobin. in. Ele Electr ctroph ophore oresis sis,, by hig high h pre pressu ssure re liq liquid uid chromatography,, accurately measures concentrations of HbS chromatography and identiﬁes the presence of HbA2, F, C, D or others. 19 Sickledex screening in children less than 6 months of age is not reliable as screening provides a positive test, when HbS is present, but cannot reliably provide a negative result as the variable concentration of HbF in this age group can, if hig high, h, mas mask k the pre presen sence ce of HbS HbS.. Ele Electr ctroph ophore oresis sis in neonat neo nates es and you young ng inf infant antss cor correc rectly tly ide identiﬁ ntiﬁes es hae haemomoglobinopathies and some centres use this method for neonatal nat al scr screen eening ing on cor cord d blo blood. od. It is imp import ortant ant to kno know w whether a baby has SCD as although the presence of HbF offers some protection against a sickle crisis, it can occur particularly in the sick neonate. Clinical review of a blood ﬁlm can identify the presence of sickle cells in some HbSS patien pat ients ts but detectio detection n of HbA HbAS S or HbS HbSC C var varian iants ts is not likely by this method. Childr Chi ldren en wit with h sic sickle kle cell tra trait it (Hb (HbAS) AS) have les lesss tha than n 50% sickle haemoglobin, are asymptomatic and their cells sick si ckle le on only ly in ex extr trem emee co cond nditi ition onss of ac acid idos osis is,, co cold ld or hypoxia. Sickle cell trait is not associated with increased risk of anaesthesia and no special preparation is required.67 Sickle cell disease HbSS has greater than 75% HbS with the remainder being HbF (SS disease) or HbC (SC disease). SC disease is associated with a higher haemoglobin concentrat tr atio ion n an and d le less ss sy syst stem emic ic up upse set, t, al alth thou ough gh it al also so ha hass a 19 44 signiﬁ sig niﬁcan cantt ris risk k of acu acute te sic sickle kle cri crises ses.. Children Childr en with sickle thalassaemia have 75% HbS and a mixture of HbF and HbA; they are also at risk of sickle crisis.
the development cor pulmonale. Routine chesttests x-ray is useful, and formalof assessment using lung function may be required. Cardio Car diomyo myopat pathy hy may dev develo elop p as a res result ult of chr chroni onicc anaemia or ﬁbrosis. Serial echocardiography estimates the degree of impaired cardiac function. Pericardial and pleural effusions may be part of this scenario. Major complications after general anaesthesia, including death from myocardial necrosis, have been reported in SCD. 63 Renal damage also occurs with VOD, with infarcts in the re renal nal med medull ulla, a, pap papilla illary ry dam damage age and nec necros rosis. is. End End-stage renal failure may result and renal transplantation has been bee n use used d in som somee cen centre tress for the man manage agemen mentt of this complication.26 Occlusive disease of the bone also occurs. Transfusion considerations
The clinical picture of SCD is variable, with some children showing minimal symptoms or signs. Features of chronic anaemia, anae mia, prese presence nce of mild jaund jaundice, ice, hepat hepatosple osplenomeg nomegaly aly
The whole practice of exchange transfusion in sickle disease has been questioned.19 74 In a large collaborative study of patients patien ts with SCD unde undergo rgoing ing surg surgery ery,, the incide incidence nce of complic com plicatio ations ns was onl only y 0.3 0.3%. %. The maj majori ority ty of chi childr ldren en undergoing tonsillectomy or adenoidectomy were transfused before bef ore ope operat ration ion,, as wer weree app approx roxima imatel tely y 50% of tho those se undergoing myringotomy. It was noted that complications were less in those patients who had undergone transfusions, indicating that transfusion would continue to have a role in the pre prepar paratio ation n of the these se pat patien ients ts des despite pite the ris risks ks and concerns.44 There is concern that the risks of transfusion may outweigh outweigh the ris risk k of dev develo elopme pment nt of a sic sickle kle crisis crisis during a carefully managed anaesthetic. Complications of transfusio trans fusion n includ includee infec infection, tion, haemo haemolysis, lysis, produ production ction of antibodies, and future difﬁculties with cross matching blood produc pro ducts ts and hae haemat matolo ologic gical al tre treatm atment ent,, suc such h as bon bonee marrow marro w trans transplant plantation, ation, which is used in young patients patients with seve severe re compl complicatio ications ns of sickle disease. disease.75 For minor surgery, when there is no risk of abdominal splinting, which may cau cause se limi limitat tation ion to lun lung g fun functi ction on aft after er ope operat ration ion,, the pot potent ential ial ris risks ks inv involv olved ed with blo blood od tra transf nsfusi usion on may outweigh the potential advantages. Thee de Th deci cisi sion on as to wh whet ethe herr a ch child ild wi will ll ne need ed a pr preo eope pera rativ tivee blood transfusion has to be discussed with the haematology team responsible for the child’s care. If it is decided that an exchange transfusion will beneﬁt the child, this is usually done to achieve a HbS concentration of less than 40% and a haemoglobin concentration greater than 10 g dl–1. These
or the effects vaso-occlusive disease are often present. VOD of presents as a painful crisis(VOD) associated with any of the sickling sickling states. states. It is a ser seriou iouss and frequen frequently tly
variables are for most However, multiple transfusio trans fusions ns sufﬁcient over a protr protracted actedsurgery. preoperat preo perative ive prep preparati aration on period may be required, particularly in children with HbSC
Medical assessment of the paediatric patient
disease who tend to have higher haemoglobin concentrations concentrations and therefore take longer to exchange transfuse. A more conservative approach is the use of a blood transfusion to achiev ach ievee a nor normal mal hae haemog moglob lobin in con concen centra tratio tion n whi which ch has bee been n reported to result in a similar incidence of complications in the perioperative period in patients with SC or SCD as the use of conventional exchange transfusion.74 If a child has had a pre previo vious us cer cerebr ebrova ovascu scular lar eve event nt or is und under ergoi going ng cardiac card iac surgery, surgery, conce concentrati ntrations ons of HbS less than 8% are
required. Children, in common with adults, demonstrate an increase in blood sugar associated with the stress response of surgery. BM stix are checked regularly after operation. After surgery, when they are able to eat, children take their usual dose of insulin and normal diet. Diabetic children who are undergoing more major surgery or wh who o ha have ve po poor or co cont ntro roll of bl bloo ood d gl gluc ucos osee sh shou ould ld be admitted admitt ed earli earlier er for stabi stabilizatio lization n of insuli insulin n requ requireme irements, nts, and monitoring of blood sugar and HbA1 concentrations.
aimed for, but can be difﬁcult achieve. glucos glu cose e and insulin ulin infusion infus ion isadjustments set up bef before ore surger sur gery y. Hypovolaem Hypov olaemia, ia, dehyd dehydration ration to and hyperosmol hyper osmolality ality,, all of A Hourly BM stix ins evaluation allows to be made which increase blood viscosity, must be avoided in patients to the reg regime imen, n, ens ensuri uring ng car carefu efull con contro troll of blo blood od sug sugar ar with wi th SC SCD. D. Th Thes esee ch child ildre ren n ar aree at in incr crea ease sed d ri risk sk fr from om during and after surgery. The particular regimen is tailored infect inf ection ion as a res result ult of dep depres ressed sed imm immuni unity ty,, dec decrea reased sed to the child’s needs and may require addition of potassium splenic function secondary to infarction and poor white cell and higher concentrations of dextrose.29 function. They should receive prophylactic antibiotics. Tourniquets are avoided whenever possible. Mild meta- Latex allergy bolic acidosis and an increase in serum lactate occurs during Per Periop iopera erativ tivee ana anaphy phylac lactic tic rea reacti ctions ons to late latex x hav havee bee been n and soon aft after er rel releas easee of the tou tourni rnique quett bec becaus ausee of the increasingly reported. These are type I, IgE-mediated hyperresulta res ultant nt sta stasis sis and tiss tissue ue aci acidos dosis. is.8 If a to tour urni niqu quet et is sens sensitivity itivity reactions reactions which can be very severe, severe, althou although gh essential then the limb must be carefully exsanguinated and the there re hav havee bee been n no report reported ed dea deaths ths dur during ing ana anaest esthes hesia ia the tourniquet used for the minimum time necessary. When fro from m thi thiss con conditi dition. on. Pat Patien ients ts who hav havee had a sus suspec pected ted used carefully, there is no additional morbidity associated intraoperative anaphylactic reaction should be investigated with tourniquet use in patients with sickle cell trait. to ide identi ntify fy the cau cause. se.3 Fre Freque quentl ntly y the dia diagno gnosis sis of lat latex ex allergy is made retrospectively and latex may now be the Diabetes mellitus commonest cause of intraoperative sensitivity reactions. Thee ma Th majo jori rity ty of ch child ildre ren n wi with th di diab abet etes es ha have ve in insu sulin lin-Several groups of paediatric patients are at speciﬁc risk dependent type 1 disease. It is the commonest endocrine of this conditio condition. n. Som Somee chi childr ldren en are known to be late latexxdiso di sord rder er in ch chil ildr dren en,, af affe fect ctin ing g 1 in 50 500, 0, wi with th a pe peak ak sensitive as they have had previous documented anaphylaxis incidence at 7 yr. Children have rapidly changing energy to late latex. x. Chi Childr ldren en who have spi spina na biﬁ biﬁda da hav havee 500 500–10 –1000 00 needs, and control of blood sugar can be particularly difﬁcult times the risk of latex allergy, although the reasons for this in older children who may have variable food intake and are not cle clear ar..32 40 53 70 It may be tha thatt rep repeat eated ed sur surgic gical al difﬁculties in complying with insulin therapy because of the interventions in these children causes the high incidence in psychological demands of adolescence. adolescence. Long-term effects of this group, rather than any genetic predisposition to latex diabet dia betes es can be dec decrea reased sed if goo good d met metabo abolic lic con contro troll is allergy.59 Anoth Another er identi identiﬁed ﬁed highhigh-risk risk group are children achiev ach ieved. ed. End End-or -organ gan dam damage age,, so imp import ortant ant in the adu adult lt wh who o ha have ve a hi hist stor ory y of a se sens nsiti itivi vity ty re reac actio tion, n, su such ch as patient, is not usually a feature of childhood disease. It is bronchospasm, urticaria or eye irritation to balloons or other essential that children with poorly controlled diabetes are latex-containing toys. admitted early for stabilization of blood sugar management. Emergency surgery surgery in poorly controlled diabetics, especially assessment In order visit, to identify children at riskask atspeciﬁcally the preoperative the anaesthetist must about thos th osee wi with th ke keto toac acid idos osis is,, is as asso soci ciat ated ed wi with th in incr crea ease sed d sensitivity to latex products and also to certain foodstuffs, morbidity. Abdominal symptoms may be part of the diabetic includ including ing avocado, kiwi fruit fruit,, chest chestnuts nuts or bana banana, na, as there ketoacidotic picture rather than a pathology needing surgical is thought to be a cross sensitivity between latex and fruit intervention. If possible, surgery should be postponed until pro protei teins. ns. A cas case-m e-matc atched hed rev review iew of chi childr ldren en with spi spina na the child’s condition is improved. biﬁda indicated the additional risk factors for latex anaphylA review of the child’s current management, including axis during anaesthesia as a history of a contact allergy to personal records of blood sugar control and insulin require- latex, atopy or food allergies, non-white race and a history ments,, is helpf ments helpful ul in asse assessing ssing their requirements, requirements, taking of greater than nine previous surgical procedures. 40 Healthy into account the type of surgery planned. A review by the ch child ildre ren n wit with h a hi hist stor ory y of at atop opy y an and d as asthm thmaa ar aree al also so paediatric team is usually undertaken. For minor surgery, considered at increased risk. diabetic children can be treated as day cases, but surgery should be planned early on the theatre list. Their morning Screening for latex allergy insu in sulin lin is om omitt itted ed an and d th thei eirr bl bloo ood d su suga garr an and d ur urea ea an and d Preoperative screening to identify latex sensitivity is pos59
ele electr ctroly olytes tes checke cked. d. is Excess Exc essive ive sta starva tion n per period s ar aree avoided. Theche BM stix measured inrvatio theatre, butiods usually during short procedures no additional dextrose or insulin is
sible and hastests beenfor suggested for high-risk groups. and RAST and ELISA latex-speciﬁc IgE antibodies skin prick testing identiﬁes children sensitized to latex but these
Table 3 Latex 3 Latex allergy: preoperative preparation. Patients who have not had a reaction but are in a high-risk group (e.g. history of spina biﬁda, genitourinary abnormalities abnor malities or multip multiple le surg surgical ical procedures) are not routinely pretreated nor are there any special precautions precautions used. A high index of suspicion is maintained during and after the case
have had long, complicated complicated hosp hospital ital cour courses ses during their illness and transplantation surgery. They are very aware of the seriousness of their condition and are often fearful or even morbid in their outlook. Older children, particularly teenagers, may be unwilling to comply with their therapeutic regime reg imens ns and up to 20% sto stop p the their ir imm immuno unosup suppre pressi ssive ve therapy unilaterally. Parents are usually very well informed on all aspects of care and can often furnish all the up to datee inv dat invest estiga igation tion res results ults and det detail ailss of pos post-t t-tran ranspl splant ant
Patients with a history of anaphylaxis to latex or allergy to latex or rubber are pretreated with: Methylprednisolone 1 mg kg–1 (maximum 50 mg) 6 hourly i.v. Ranitidine Raniti dine 1mg kg–1
6 hourly i.v. over 20 min
250 µ g kg–1
1–5 yr 6–12 yr
2.5–5 mg 5 –10 mg all 6 hourly i.v. slowly
All given 6 hou hourly rly i.v.– i.v.– 2 dos doses es bef before ore operatio operation n and continue continued d for 24 h after operation
tests lack speciﬁcity for predicting the likely occurrence of an anaphylactic reaction during anaesthesia. The RAST test is no longer considered sufﬁciently speciﬁc, with 20–45% of patients having a negative RAST test, but positive skin tests. A standardized latex skin prick test solution is not wide wi dely ly av avai aila lable ble an and d th thee te test st its itsel elff ca can n pr prec ecip ipita itate te an anaphylactic reaction. It has been suggested that a RAST test is performed ﬁrst and if negative, a skin prick test is checked.76 Ele Elevat vation ion of tot total al ser serum um IgE in com combin binati ation on with wi th a cl clini inica call hi hist stor ory y is a mo more re sp spec eciﬁ iﬁcc pr pred edic ictor tor of anaphylactic reactions occurring during surgery in children with spina biﬁda.40 Management
requirements. Issues common to all groups of transplant patients include the constant threat of rejection of the transplanted organ, risk of infec infection, tion, and effe effects cts of immuno immunosuppr suppressiv essivee and steroid therapy. Signs of potential rejection must be sought and are dep depend endent ent on the ind individ ividual ual or organ gan tra transp nsplan lanted ted.. Infect Inf ection ion rem remain ainss an imp import ortant ant cau cause se of mor morbid bidity ity and mortality. The increased risk of infection includes hepatitis, cytomegalovirus (CMV), Epstein Barr and other bacterial, viral or fungal infections. Opportunistic infection, particularly of the lungs, is a frequent problem while the patient remains on immunosuppressive drugs and steroids. Patients who have had transplants, and are CMV-negative, require CMV negative blood. Any patient considered at risk from graft vs host disease from blood transfusion should receive irradiated products. There is also an increased risk of the child developing tumours, particularly lymphomas. Many children remain on cyclospor cyclo sporin in which is poten potentially tially hepatotoxic hepatotoxic and nephr nephrootoxic, and often results in hypertension. It may also impair marrow marr ow func function tion and caus causee gastr gastrointes ointestinal tinal upset. Cyclosporin spo rin con concen centra tratio tions ns mus mustt be in the the therap rapeut eutic ic ran range ge throughout the perioperative period. Azathioprine and ALG can cau cause se thr thromb ombocy ocytop topeni enia. a. Ste Steroi roids ds may pro produc ducee the typicall cush typica cushingoid ingoid featu features, res, hyper hypertensio tension n and abnor abnormal mal glucos glu cosee tol tolera erance nce.. Sup Supple plemen mental tal ste steroi roids ds are req requir uired ed (Table 2) Somee pai Som pain n man manage agemen mentt str strate ategie gies, s, suc such h as epi epidur durals als,, may be rel relativ atively ely con contra traind indica icated ted in chi childr ldren en rec receiv eiving ing immunosupp immuno suppress ressive ive medic medication ation beca because use of the poten potential tial risk of infection and clotting abnormalities, but this remains debatable.
Management requires scrupulous preoperative preparation to decrease the risk of anaphylactic response during anaesthesia. Regimens vary but usually include provision of a latex-free environment and preoperative administration of steroids, H2 antagonists and antihistamines (Table 3). Latexfree equipment is available and guidelines for the management of anaphylaxis during anaesthesia should be in place at all anaesthetic settings.13 Some centres feel that pretreatment is of limited value and that it may mask the early signs of anaphylaxis.76 Instead, they recommend that when providing anaesthesia for a susceptible patient, the environment is kept latex-free. This management may be preferable in that it avoids potentially sensitizing a child who is in one of the high-risk groups. The cost and social implications Renal transplantation of pre pretre treatm atment ent are rel releva evant nt whe when n the add additio itional nal time Assessment focuses on the child’s general physical health required for admission, medications used with their potential and current renal function. It is important to determine if side si de ef effe fect ctss an and d ne need ed to av avoi oid d da day y ca care re ar aree ta take ken n in into to children with successful renal transplants have normal renal consideration. function or whether they continue to have some limitation of function. This requires review of urea, electrolyte and Children who have had major organ transplants creatinine concentrations, full blood count and coagulation The quality and length of life of many children with end- studies. Glomerular ﬁltration rate (GFR) is often approxistage major organ failure have been improved greatly with mately 50% of normal values and creatinine concentration successful transplantation surgery. These children may then may remain slightly increased even with a well functioning present for surgery for procedures either related or unrelated transplanted kidney. If creatinine concentration if increased to thei th eirr tr tran ansp spla lant nt.. medical This Th is is needs. a gr grou oup that atfrequently hass va ha vari ried ed and an d 6 p th sometimes complex They require additional addit ional psychological psychological prep preparati aration on and supp support ort as they
by more than 10% should above their usual value, thefunct possibility of acute rejection reje ction be cons considere idered. d. The function ion of the transplanted kidney tends to decline gradually, as shown
Medical assessment of the paediatric patient
by increasing creatinine concentration and decreasing GFR. Renal excretion of drugs is usually normal but drugs which could potentially damage the kidney, such as NSAID, are avoided. avoid ed. The chro chronic nic anaemia of rena renall failu failure re is usua usually lly resolved after transplantation but some children remain on erythropoeitin. Assessment of the limbs for planned vascular access is important so that vessels which may be useful for vascular shunts later in life are preserved. Prolonged periods of fasting are avoided and an i.v. infusion may be required
pacemaker is present and if so a routine pacemaker check is required. Lung or heart–lung transplant
The co The comm mmon ones estt gr grou oup p of ch child ildre ren n wh who o ha have ve a lu lung ng or hear he art– t–lu lung ng tr tran ansp spla lant nt ar aree th thos osee wi with th cy cyst stic ic ﬁb ﬁbro rosi sis. s. Unfortunately, the long-term survival for lung transplantation is les lesss tha than n tha thatt for heart transpla transplanta ntatio tion n as man many y patients develop obliterative bronchiolitis which is part of the rejection process and appears to be difﬁcult to prevent.55
before oper before operation ation to ensur ensuree mainte maintenance nance of intrav intravascu ascular lar volume. volum e. Hyper Hypertensio tension n is a freq frequent uent feature and child children ren are usual usually ly rece receiving iving many drug drugs, s, inclu including ding hydr hydralazi alazine, ne, nifedipine, labetalol and diuretics. Prophylactic antibiotics are usually required.
Recurrent Recurr ent che chest st inf infect ection ion is com common mon.. Rou Routin tinee clin clinica icall assessment of present general health, exercise tolerance and identi ide ntiﬁca ﬁcatio tion n of any fea featur tures es ind indica icativ tivee of rej reject ection ion or infection is undertaken. Preoperative investigations include full blood count, urea, electrolyte and creatinine concentrations, lung function tests. measurement of arterial pressure and urinalysis. ECG, chest x-ray and pulmonary function test te stss ar aree pe perf rfor orme med. d. If FE FEV V1 and FVC mea measur sureme ements nts decrea dec rease se by mor moree tha than n 15% of the chi child’ ld’ss usu usual al val values ues,, further investigation with bronchoscopy, bronchial alveolar lavage and transbronchial biopsy may be required to determine if rejection or infection is present. 82
Intrathoracic organ transplantation
After succ successf essful ul trans transplanta plantation tion sur surgery gery,, childr children en usual usually ly have multiple gene general ral anae anaesthet sthetics ics for routi routine ne postpost-trans trans-plant investigations. They also require anaesthesia for other childh chi ldhood ood sur surgic gical al pro proced cedure ures. s. Whe When n ass assess essing ing suc such h patients, recent results of all follow-up investigations must be available. Liaison with the transplant team is essential.
Liver transplantation Heart transplantation
Children can be very well after successful liver transplanta-
The long-term outcome after paediatric heart transplantation tion and hep hepati aticc met metabo abolis lism m may be ret return urned ed to nor normal mal.. is bet better ter tha than n tra transp nsplan lantati tation on inc includ luding ing the lun lungs. gs.15 33 Most children return to normal activities.84 The majority of Children with heart transplants tolerate subsequent general patients remain on cyclosporin and steroids after transplant, surgical interventions well.62 althou alt hough gh som somee nee need d ant antihy ihyper perten tensiv sive, e, diu diuret retic ic or ant antiiAs with oth other er tra transp nsplan lants, ts, the com common mon pro proble blems ms are convulsant medications also. Assessment includes identifyrejection and infection.4 5 Other major complications after ing the fea featur tures es of imp impair aired ed live liverr fun functio ction, n, in par particu ticular lar cardiac card iac trans transplanta plantation tion inclu include de pulmo pulmonary nary hyper hypertensi tension, on, evidence of residual portal hypertension. This may include coronary artery disease and lymphoproliferative disease.4 5 ascites, oedema or a history indicative of the presence of As par partt of rou routine tine pos post-t t-tran ranspl splant ant car care, e, chi childr ldren en nee need d oeso oesophage phageal al varic varices, es, impair impairment ment of respi respirator ratory y func function tion investigatio inves tigations, ns, inclu including ding endo endomyoca myocardial rdial biops biopsy y and cardi cardiac ac cau caused sed by ven ventila tilation tion–pe –perfu rfusio sion n mis mismat match ch and alv alveol eolar ar angiography, which require general anaesthesia. hypoventilation. In addition to routine preoperative evaluPreoperat Preo perative ive asse assessmen ssmentt includ includes es revie reviewing wing the child child’s ’s ation of renal function and full blood count, a coagulation history and present condition. Potential features of rejection screen and liver function tests are important, although most includ inc ludee dec decrea reased sed app appeti etite, te, gen genera erall mal malais aise, e, pyr pyrexi exiaa or are in the normal range after successful transplantation. 84 irritability. Other features include ﬂuid retention and signs of Acu Acute te rej reject ection ion is sho shown wn by the pre presen sence ce of cho choles lestati taticc 5 cardiac card iac failu failure. re. Ana Anaest esthes hesia ia dur during ing an epi episod sodee of rej reject ection ion is jaundice reﬂected in abnormal liver function tests, prolonged prot othr hrom ombi bin n tim time, e, ly lymp mpho hocy cyto tosi siss an and d eo eosi sino noph phil ilia ia.. associated with increased morbidity and should be avoided pr Prothrombin time is the earliest indicator of impaired liver whenever possible An ECG, echocardiography and chest x-ray are part of function although it may remain within the normal range the routine preoperative assessment. The ECG is reviewed, until 70% of liver function is lost. An INR greater than 1.4 looking looki ng for arrhythmias, arrhythmias, change in axis or decr decrease ease in total is an indication that signiﬁcant impairment of liver function 61 voltages. Ech Echoca ocardi rdiogr ograph aphy y pro provid vides es an ind indica ication tion of is present. present function, with decrease in shortening fraction being References indicative of failing cardiac function. Onee of th On thee ca caus uses es of de deat ath h in ch chil ildr dren en af afte terr ca card rdia iacc 1 Andropoulos DB, Heard MB, Johnson KL, Clarke JT, Rowe RW. Postanesthe Posta nesthetic tic apnea in full term infan infants ts after pyloromyotomy. pyloromyotomy. transplant is coronary artery disease; this may be asymptom Anesthesiology 1994; 1994; 80 : 216–19 atic as these children do not feel ischaemic pain. Evidence 2 Association Association of Anaes Anaestheti thetists sts of Great Britain and Irela Ireland. nd. The of coronary artery disease was reported in 15% of children Anaesthesia Team. London: Association Association of Anaes Anaesthetis thetists ts of Great 5 61 in the Stamford series and in 3% of the Hareﬁeld series. Britain and Ireland, 1998
3 Ass Associ ociati ation on of Ana Anaest esthet hetist istss of Gre Great at Bri Britai tain n and Ire Irelan land. d. Anaphylactic Reactions Associated with Anaesthesia. Lo Lond ndon on:: Association Associ ation of Anaes Anaestheti thetists sts of Great Britain Britain and Ireland, 1990
Additional complications renal from immuno immunosuppr suppressiv essivee include therapy thera py,decreased , hype hypertens rtension ion function and the 69 complicatio compl ications ns of stero steroid id thera therapy py.. Rar Rarely ely a per perman manent ent
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