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Children are not small adults and their developmental status and metabolic needs must be considered carefully by those entrusted with their medical care. In a paediatric consultation, the history (anamnesis) is especially important, as it is essential to have a clear idea of the nature of the problem before examining the child. This is because children are often reluctant to be examined by strangers.

ENT surgery accounts for nearly 40% of all paediatric surgery, as recorded in the report of the Childrens’ Surgical Forum published by the Royal College of Surgeons of England (2000) “Childrens Surgery: a first class service”. The UK National Service Framework for Children, together with this report, require that children should be nursed on dedicated paediatric wards, staffed by trained nurses with both paediatric and ENT training, and that ENT surgeons who operate on children, and anaesthetists who anaesthetise them, perform sufficient numbers of operations to keep their skills honed. Other countries will have similar principles and clinicians should be aware of them.

The paediatric anaesthetist works very closely with his ENT surgical colleague. Surgery may be undertaken on the airway, responsibility for which is, of necessity, shared by both practitioners, who must understand each other’s requirements to ensure safe patient care. Good communication and understanding between surgeon and anaesthetist are essential for the safe management of the more challenging problems of paediatric ENT surgery.

Most people feel rather uneasy when reminded that we share 90% of our DNA with our ape ancestors. The idea that we evolved from monkeys was Darwin’s most controversial claim in the Origin of the Species, much more unsettling than his hypothesis that the mechanism of evolution was by natural selection; “nature red in tooth and claw” actually fitted very easily with Victorian beliefs in unbridled capitalism. However, human descent from apes was embarrassing and seemed to denigrate our spirituality, so it was natural to seek ways of distancing ourselves from biology. The most quintessentially human attribute to emphasise was obviously the human ability to communicate by speech and language. Hence, until very recently it was believed that speech was uniquely human. Even chimpanzees, it was thought, could not be taught to communicate beyond rudimentary emotional calls. The power of speech had been bestowed on humans to make us human, all at once by a lucky mutation in the 1% of DNA we do not share with chimps. Chomsky and followers thought that this endowed us with an “encapsulated linguistic processing module” (Chomsky 1975) and a generative language “instinct” (Pinker and Jackendoff 2005).

One of the most remarkable things about human development is that within the span of approximately 5 years, babies progress from making only physiological noises, such as crying and coughing, to being fluent users of the language (or languages) spoken around them without receiving any direct instruction to do so. Mastery of such complex skills as knowing when to use the pluperfect tense, embedding dependent clauses and using thousands of different words productively occurs before children begin formal, academic schooling. This mastery occurs in children living all over the world, hearing thousands of different languages, under vastly different socioeconomic conditions.

Paediatric laryngology is a discipline that is in its infancy when compared to the practice of adult laryngology. One reason is that a young child is often more difficult to examine than an adult, which can impede our ability to make an accurate diagnosis. Another is the lack of knowledge that has existed about the fine structure and development of the vocal fold as a child grows. Furthermore, outcome-based results have not previously existed for our treatments of common paediatric vocal fold pathologies.

The prevalence of permanent sensorineural hearing loss in the UK has been shown to be approxymately 1:1000 children under 3 years and 2:1000 of children under 16 years (Fortnum et al. 2001). The late identification of congenital hearing impairment in particular may lead to delay in language development, significant educational underachievement and ultimately reduced employment opportunities. A Health Technology Assessment review conducted by Bamford and Davis in 1997 highlighted that large numbers of hearing-impaired children were being diagnosed late, and recommended the introduction of a universal neonatal hearing screening programme (Newborn Hearing Screening or NHS), which has since been established in the UK and which exists in other countries (Davis et al. 1997).

ENT surgeons dealing with children need to know the ENT features of a few common syndromes, for example Down syndrome, Treacher Collins syndrome and Goldenhar syndrome, as they are likely to meet children with these conditions in their paediatric practice. The ENT surgeon may be presented with two distinct clinical scenarios. In the first, a patient with a known syndrome presents with an ENT problem. In this instance, the ENT surgeon needs to be aware of the ENT features of the syndrome so that the appropriate investigations and management can be instigated. The second and less common situation is when the ENT surgeon suspects that a child may have a syndrome that has not yet been diagnosed. This is rightly the province of the geneticist and, while it is intellectually stimulating to try to piece together a syndrome, it is best to avoid mentioning any suspicions at this stage. Much anxiety can otherwise result from ready access to the Internet. Nevertheless, they should know how to find out about these less common syndromes. This chapter provides a brief list of the more common ENT-related syndromes.

Over recent decades, antenatal diagnosis has been greatly enhanced by high-resolution ultrasonography, enabling the identification of potential airway anomalies in utero. The development of rapid-sequence magnetic resonance imaging (MRI) scanning of the foetus further enhances our diagnostic capabilities pre-natally. The role of the paediatric otolaryngologist in the management of these problems has become increasingly important, because the development of minimally invasive endoscopic techniques and advancements in intrauterine surgery and in endoscopic equipment now make the pre-natal surgical correction of airway conditions a possibility. Thus, airway obstruction in the unborn baby can be diagnosed early, and with a multidisciplinary approach, planned management of the possible neonatal airway compromise has become routine. The paediatric ENT surgeon’s principal remit in this team is to secure the airway at the time of an ex-utero intrapartum treatment (EXIT) procedure. EXIT describes the technique of undertaking a Caesarian section approach, but delivering only the head and upper torso through the uterine incision, while maintaining the maternal foeto-placental circulation.

Many adults and children with an underlying immunodeficiency can present to the ENT surgeon. This chapter deals with the presentation, investigation and management of immunocompromised children in ENT practice. The reader should also consider the chapter on cystic fibrosis and primary ciliary dyskinesia. Both primary immunodeficiency (PID) and human immunodeficiency virus (HIV) infection are discussed, as PID is under-diagnosed and retroviral infection is an increasingly common problem in the UK. Immunodeficiency is a complex area of medicine and this chapter is not meant to be exhaustive, but instead we aim to outline basic principles to guide clinical practice