Managers & Medicine

Children's services are high on the government's healthcare agenda and on many health authority business plans. While the need to distinguish children from adults is recognised for medical, psychiatric and community care, surgical services for children are often regarded as a part of the adult service.

Yet without considering children's specific surgical and anaesthetic needs, services and outcomes may fall short of optimal standards and best practice. A lack of understanding of childhood diseases and abnormalities and a failure to recognise that children, both physically and emotionally, are not simply small adults, is not uncommon and can have tragic results. There are many cases of children being referred to tertiary paediatric surgical centres only after treatment has been unsuccessfully attempted in a local hospital. Some have escaped without lasting effects, but others have sustained permanent damage and several have died.

It is crucial that managers understand the complex issues surrounding general surgery in children and are aware of the standards agreed by professional medical bodies for this service, including the appropriate use of specialist paediatric centres.

What is paediatric surgery?

In the UK, the specialty of paediatric surgery covers only general surgery for children and does not include surgical sub-specialties such as neurosurgery, cardiac surgery, ENT or orthopaedics. Paediatric surgeons in the UK are also trained in paediatric urology, but there is an increasing and appropriate trend for paediatric urology to be provided by specialist paediatric urologists, as has been the case in the US for many years.

Paediatric surgery is perhaps the last bastion of the truly general surgeon. Paediatric surgeons deal with surgical conditions affecting many systems, including the lungs, gastrointestinal tract, liver and biliary system and genital tract. They also help in the management of multiple trauma, tumours and, until recently, most of the problems associated with spina bifida.

In the UK, patients are considered to be children until they reach 16. The term 'neonatal surgery' describes surgical treatment in newborn infants up to four weeks old (or 44 weeks gestational age for babies born prematurely), but this is only one aspect of paediatric surgery.

Paediatric surgeons also deal with general surgical conditions that present before birth, in infancy, in early childhood, in school-age children and in adolescents.

Much general surgery in children can be, and is, adequately performed in district general hospitals by appropriately trained general surgeons. In Scotland, two-thirds of children undergoing general surgical procedures are treated by paediatric surgeons, but in England and Wales the reverse is true.

The key issue is to identify those children who need the specialist expertise of a paediatric surgical team (including surgeons, anaesthetists and nursing staff ) and the specialised facilities offered by tertiary centres.

But in all cases, health service staff who undertake the surgical or anaesthetic care of children should have adequate training and ongoing experience of the conditions they manage, and be able to offer up-to-date treatment in facilities designed with children in mind.

Children are not small adults That children change in size between birth and 16 years of age is selfevident. Less recognised and acknowledged by the lay observer (and some medical practitioners) are the simultaneous changes in physiology and emotional response and the nature of surgical problems that can occur. All have a significant bearing on the need for appropriate services.

Different sizes of children require different sizes of equipment. This includes beds (incubators and cots for infants, for example), but more important, a wide range of anaesthetic equipment, including masks, endotracheal tubes, intravenous access equipment and ventilators.

Operating tables, diathermy equipment and even surgical instruments often need to be smaller, particularly for new endoscopic or laparoscopic procedures.

Diagnostic techniques must also be size-related; this includes blood sampling and radiological investigation. The circulating blood volume in a full-term newborn child is only 280ml (80ml/kg), and in a two-year-old child only approximately 800ml, compared with 5 litres in an adult. Normal adult blood samples of 10-20ml thus represent a significant blood loss in children, and repeated sampling at this level would cause circulatory collapse very quickly.

Micro-sampling and testing facilities are therefore essential for all paediatric patients. Radiological investigations (particularly ultrasound and contrast studies) often require moving equipment, all of which has to be 'downsized' for the paediatric patient.

Less commonly recognised are the huge differences in physiology between adult and child - particularly their cardiovascular and respiratory systems - and the way in which they respond to injury or insult (see box, above). This understanding can mean the difference between life and death. All hospitals that accept children at accident and emergency or for any surgery must be able to provide immediate attention at all times by trained paediatric nurses and medical staff familiar with the normal physiology of childhood and the effects of surgery or trauma.

Children's emotional needs are also important. Like adults, children are usually frightened by hospitals, especially in emergencies, and that fear is magnified by the prospect of surgery. The presence of a parent or other familiar adult is often the only reassuring factor.

Thanks to the efforts of Action for Sick Children and the specialist paediatric medical and nursing associations, this is now better recognised when providing surgical services, but many hospitals fail to understand that it has implications throughout the inpatient episode.

Children should be accompanied to the theatre by a parent or carer, be anaesthetised in their presence and awake from anaesthesia to see a familiar face.

On the other hand, while parents or carers should be encouraged to enter the anaesthetic and recovery rooms, it is not reasonable to expose these usually very anxious parents, or their children, to adult patients emerging from major surgery. The only logical conclusion is to separate paediatric and adult surgical patients, ideally in discrete facilities or at the very least by screening in the recovery room.

Post-operatively, parents/carers must be permitted to stay with their child on the ward and encouraged to participate in their care as much as possible. This applies to children of all ages, not just neonates and infants, and in most cases it will assist rapid recovery and discharge. Such arrangements are the norm for most units caring for children, but may be unfamiliar, and even disturbing, for those used to dealing with adults.

These differences also place significant demands on the staff, including nurses, anaesthetists, radiologists and other supporting disciplines such as pharmacy and dietetics. A degree of specialist expertise is essential and must be provided round the clock if quality is to be maintained. This has significant manpower and staff implications which must be accepted by units intending to offer surgical services of any kind to children.

General surgeon or paediatric surgeon?

One of the most difficult and contentious issues is deciding when it is appropriate for general surgical services to be provided by a specialist paediatric surgical unit, rather than the local district general surgeons.

While it is mostly accepted that neonatal surgery should be undertaken by specialists, people tend to see little need to send older infants or children to a regional paediatric surgery unit, especially if this involves travelling a significant distance. However, the general surgical problems and diseases of paediatric practice are different enough from adult general surgical practice to justify a clear distinction between the two.

Essentially, there are three main categories of 'surgical disease' in children:

disorders specific to children;

disorders not specific to children, but which present differently and require different management from the adult version;

disorders occurring in both adults and children that require the same treatment.

Issues concerning the appropriate treatment of these different categories are complex (see box, page 11). In general, the younger the patient, the more likely the child is to require specialist treatment within a tertiary paediatric surgery centre. The major professional bodies (the royal colleges of surgeons, anaesthetists, and paediatrics and child health, the British Association for Paediatric Surgeons) have published a number of guidelines and recommendations.

All agree with the 1989 report of the National Confidential Enquiry into Perioperative Deaths, which states, 'there is no place for the occasional paediatric surgeon or anaesthetist', and confirm that, despite the relative inconvenience, children under five needing general surgery should be treated by a specialist centre, as should those with complex surgical or medical problems.

There are also clear recommendations for training and 'ongoing experience' for district general surgeons and anaesthetists treating older children. The Calman training programmes for general surgeons are likely to reduce the level of exposure to general paediatric surgery, and consequently fewer district general surgeons will have had experience of childhood conditions during their training.

The relative incidences of many childhood surgical conditions are sufficiently low that adequate ongoing experience is only likely by concentrating the paediatric caseload on one surgeon and anaesthetist within a single DGH. This will inevitably lead to difficu lt ies in providing emergency and holiday cover, unless consortia of district general hospitals are formed. In all cases, the regional paediatric surgery centre should provide the 'secondary' paediatric surgical service for patients within its local conurbation.

2The best solution is likely to be the provision of hub-and-spoke outreach clinics and educational meetings by the specialist regional paediatric surgeons in conjunction with their surgical and/or paediatric colleagues at surrounding DGHs. Where this occurs - and there are several successful models, such as those within South Thames, provided by University Hospital Lewisham and St George's Hospital, Tooting - elective patients can be referred for secondary or tertiary care and good collaborative links established within which to manage emergencies.

Since the outcome of the Bristol heart surgery cases considered by the General Medical Council, clinicians and managers involved in offering general surgical services to children will have to account for differences in their outcomes when compared with national standards.

How children's physiology differs from adults 4This is most marked in the cardiovascular and respiratory systems and is exemplified by changes in the normal values expected for pulse rate, blood pressure and respiratory rate.

The normal values in infants change slowly during childhood until normal adult values are achieved at adolescence. Nurses or doctors used to dealing with adults may not immediately recognise that a small infant with apparently 'normal' adult values for heart and respiratory rate is severely ill and may be about to die.

Children also have a particularly responsive peripheral vascular system, allowing them to lose up to a quarter of circulating blood volume without any drop in central blood pressure. Consequently, the signs of 'shock' in children (common in many emergency surgical conditions or after trauma) are much more subtle and easily overlooked by those used to dealing with adults. Failure to recognise these subtle signs and react appropriately may result in irreversible organ failure and even death.

Paediatric surgeons recognise that the most important predictor of outcome is the pre-operative status of the patient: children who are underresuscitated before surgery may develop irreversible renal failure after the operation, regardless of the surgeon's skill. Examples exist in every region of emergency surgical interventions for intussusception or pyloric stenosis in young children at DGHs in which the perceived need for urgent surgery has outweighed the importance of preoperative resuscitation - with disastrous results (see page 10).

1A further challenge is the need for accurately measured fluid replacement and drug administration.

As size increases between birth and adolescence, a child's fluid and energy needs change and may be transiently but significantly increased before and after surgery.

These needs are almost entirely weight-related, and all intravenous fluids, blood and drugs have to be calculated individually for each patient and reviewed frequently, sometimes within the hour. The small amounts required to make a difference in infants and small children are often overlooked by practitioners in adult services until overt signs of organ failure supervene.

Recommendations for non-specialist paediatric surgery Minimum population of 200,000 Designated general surgeon for provision of non-specialist paediatric surgery Must have six months' training in an accredited paediatric surgery centre Must care for enough children each year to maintain a high level of competence Must have at least one operating list a week dedicated to children

Must maintain continuing medical education in paediatric surgery Designated anaesthetist for children must meet the criteria set by the Royal College of Anaesthetists for paediatric anaesthesia Paediatrician to be involved with the care of all children under five years of age Dedicated children's wards/day unit to which all children should be admitted

Separate amenities for children within the A&E department Nurses with special training in children Immediate access to paediatric intensive care beds Arrangements for continuous cover by staff with adequate training and continuing experience in paediatric surgery (ie collaboration with neighbouring DGH or arrangements to transfer to specialist centre)

Recommendations for specialist paediatric surgery centres Minimum population of 2.5 million At least five accredited paediatric surgeons (one specialising in paediatric urology) Fully trained paediatric anaesthetists Full range of specialist services for children Specialist paediatric nurses and paediatric critical care staff

Facilities designed for children (including A&E, outpatient departments, wards, operating theatres, day care unit, radiology suite and laboratory services) Full support services for children Accommodation for parents (with unrestricted access to their children)

Catalogue of errors - a real life case study An eight-month-old baby girl was admitted to her local district general hospital with a short history of screaming, drawing up her legs and the passage of a blood-stained stool. A correct diagnosis of intussusception was made by the admitting paediatric registrar.

The general surgical registrar was called, but because he had never seen such a case before, he called his consultant, who asked for the theatre to be ready on his arrival 30 minutes later. The possibility of radiological treatment instead of surgery was never discussed.

The paediatricians inserted an intravenous line and prescribed maintenance fluids appropriate for the child's weight. The anaesthetic registrar who saw the child preoperatively suggested giving more fluid. But because the IV line had stopped working and the paediatrician was busy in A&E, the nurses decided it could be done in theatre.

No one noticed that the baby had not passed any urine for several hours, and her rapid heart rate was attributed to pain. In the operating theatre, the anaesthetist reinserted the IV line and began to give more fluid, but the surgeon was impatient to start and began to operate before the child was properly rehydrated.

On opening the abdomen, a typical intussusception was found, which was easily reduced. The bowel looked a bit bruised, but the surgeon thought it would survive, and left it in place.

Postoperatively the baby was given maintenance fluids only, and failed to pass any urine. Her heart rate remained rapid and again was attributed to pain, so more sedation was given. Because the child was severely dehydrated, the sedation had a profound effect and soon the baby was barely breathing.

The nurses called the crash team, who correctly diagnosed dehydration and administered lots of fluid.

But by this time the baby's kidneys were not working and she had still not passed any urine. She became visibly swollen, especially in the abdomen, and the surgeon, worried that the bruised bowel had perforated, recommended another operation immediately.

The anaesthetist registered concern at this, but had little experience of intussusception and was convinced by the surgeon of the urgency of the operation. The baby had still not passed any urine when her abdomen was reopened. Apart from severe swelling and oedema of the bowel, no abnormality was found, so after 20 minutes, the surgeon attempted to close the abdomen. Unfortunately, the tissues were so swollen that he was unable to close the wound and had to cover the exposed abdominal contents with wet swabs.

At this point the tertiary paediatric surgery centre was contacted and sent a paediatric intensive care retrieval team to transfer the child to the centre, fully intubated and ventilated. Abdominal closure was out of the question, so the paediatric surgeons sutured an artificial patch in place over the bowel as temporary protection.

The baby spent the next four weeks in intensive care, on kidney dialysis, artificial ventilation and intravenous nutrition. Two weeks after the operation, it was finally possible to close her abdomen, but the baby's kidneys took much longer to recover.

Against all odds the baby survived, with no long-term sequelae.

Not surprisingly, the parents had serious questions about the initial management of their child. An internal inquiry by the DGH found many errors, all due to the staff 's inexperience of managing intussusception in small infants. A successful legal action against the trust followed.

Despite this, and the relative proximity, just 20 miles away, of the tertiary centre to the DGH, the trust was unwilling to transfer all future cases to the tertiary centre. However, it agreed to try to ensure better protocols for future management.

Fur ther reading Report of the British Association of Paediatric Surgeons. British Medical Journal 1992; 304(6836): 1194.

Surgical Services for the Newborn.

Royal College of Surgeons of England/British Association of Paediatric Surgeons, 1992.

Audit Commission. Children First: a study of hospital services. HMSO, 1993.

The Provision of General Surgical Services for Children. Senate of Surgery of Great Britain and Ireland, 1998.

Categories of 'surgical disease' in children and their appropriate treatment Disorders specific to children Rare but complex congenital abnormalities such as abdominal wall defects, where the baby is born with some or all of the abdominal organs outside the body, and congenital diaphragmatic hernias, where the baby's intestines develop in the chest and the lungs are compromised before birth. These can now be diagnosed before birth, and the surgeons who deal with the baby should be involved with the obstetrician in counselling and advising the parents before its birth.

Acquired disorders specific to children, such as necrotising enterocolitis - a severe inflammation of the bowel most often seen in premature babies. All are specific to newborn infants and usually present as emergencies in the first few days or weeks of life. Mostly their incidence is sufficiently low that experience and expertise can only be gained if the patients are treated in centralised specialist facilities, a need exacerbated by the complexity of the supporting treatment and anaesthesia. Consequently, these conditions should be referred to a specialist neonatal and paediatric surgery centre.

Disorders not specific to children, but which present differently and require different management from the adult version All have some peculiar feature in childhood that requires specialist knowledge in the surgeon, as well as the special paediatric skills of the anaesthetists and nursing staff. While most cases are uneventful, those which are not require immediate support by specialist teams.

Some examples include:

Inguinal hernias Common in adults and usually due to acquired weakness of the muscles in the groin area. In children the defect is congenital, the muscles are not weak, and the surgical procedure for correction is completely different. Moreover, the major risk in a hernia operation in males, the most commonly affected sex, is damage to the vas deferens or to the artery supplying the testis. In infants these vessels are extremely tiny - less than 1mm in diameter - and even minor trauma during surgery may be catastrophic.

Quality can only be assured if the operator has been trained appropriately and is performing sufficient procedures to maintain expertise. It is not a routine operation, nor is it appropriate for surgeons used to handling more robust adult tissues. These factors are particularly important when dealing with emergency hernia operations.

Pyloric stenosis An obstruction to the outlet of the stomach which causes repeated vomiting and weight loss. Usually caused by ulcers or gastric cancer, but in children it is simply a thickened muscle at the outlet to the stomach, which can be easily divided.

The paediatric variety usually presents at about four weeks of age, and thus while surgical treatment is relatively simple, it requires anaesthetic and nursing staff with special expertise in treating small infants. Babies present an anaesthetic challenge because of their size, and require detailed attention to the fluid and electrolyte imbalances caused by prolonged vomiting. There is considerable evidence that the complication rate after this procedure is higher in non-specialist centres.

Intussusception Occurring in both adults and children, the bowel becomes 'telescoped' into itself, causing intestinal obstruction and ultimately becoming gangrenous. In adults it is usually caused by an abnormal bowell growth which has to be surgically removed. In children it is common between six months and two years of age and is usually precipitated by enlargement of glandular tissue in the intestine, often caused by viral infection. It can usually be reduced (reversed) radiologically without surgery, only rarely requiring bowel resection.

The most common error in managing paediatric intussusception is failure to recognise symptoms of dehydration and shock. The speed with which inadequate fluid replacement can progress to multi-organ failure and even death is frightening, and too many cases end up in intensive care after inappropriate initial management (see opposite).

These disorders accounted for most non-cardiac adverse outcomes in reports by the National Confidential Enquiry into Perioperative Deaths, and the appropriate location for treatment is being discussed at the Royal College of Surgeons of England.

Disorders occurring in both adults and children and requiring the same treatment These include conditions such as appendicitis and undescended or torted testes. They are common enough for enough experience to be gained in most district general hospitals, and the surgical procedures are identical. Unfortunately, even these conditions can display some differences requiring decisions over when to retain the patient locally and when to transfer to a specialist centre.

Appendicit is In under-fives this is often difficult to diagnose because children of this age cannot articulate their symptoms accurately. In addition, the disease progresses rapidly to peritonitis because the normal ratelimiting defence mechanisms are not fully developed. Therefore the patient may be much more unwell than a 'normal case', making complications more frequent.

Undescended testes Surgical treatment is similar in infancy, childhood or adolescence.

But there is significant evidence that the outcome is less satisfactory if the operation is delayed beyond two years of age. All screening programmes now aim to identify patients in infancy, so the operation can be performed early. Consequently, the anaesthetic and nursing needs are more specialised and are often not available in a DGH.

Evelyn Dykes is senior lecturer in paediatric surgery, United Medical and Dental Schools of Guy's and St Thomas' and University Hospital Lewisham, London.

REFERENCES

1 Atwell J, Spargo P. The provision of safe surgery for children. Archives of Diseases of Childhood 1992; 67(3): 345-49.

2 British Association of Paediatric Surgeons. A Guide for Purchasers and Providers of Paediatric Surgical Services. Edinburgh, 1994 (revised edition 1995).

3 British Paediatric Association. The transfer of Infants and Young Children for surgery. London, 1993.

4 Campling E, Devlin H, Lunn J. The Report of the National Confidential Enquiry into Perioperative Deaths. London, 1990.

5 Department of Health. The Welfare of Children and Young People in Hospital. London: HMSO, 1991.

6 Atwell J. Infantile hypertrophic pyloric stenosis: where should it be treated? (Invited comment). Annals of the Royal College of Surgeons of England 1993; 75: 34-37.