ABC of General Paediatric Surgery

INGUINAL HERNIA, HYDROCELE, AND THE UNDESCENDED TESTIS

Mark Davenport

Clinical embryology and anatomy of the inguinal region

dutomna '~The testis is formed from coelomic epithelium and primordial germ cells in a longitudinal fold high on the posterior abdominal wall at a similar level to the developing kidneys. As gestation proceeds, the testis migrates down the posterior wall towards the deep inguinal ring, probably under the control of the hormone mullerian duct inhibitory factor. The gubernaculum, a condensation of mesenchyme, forms within the future inguinal canal and guides the testis through the layers i_______ of the body wall towards the scrotum. Two factors seem to be important in this second stage-the release of testosterone from the fetal testis itself and an intact genitofemoral nerve, which probably \ l <; releases substances causing gubernacular contraction. A tongue of the peritoneal cavity also precedes the migrating testis through the canal-the processus vaginalis. After birth this peritoneal communication should obliterate and disappear, but failure to do this may lead to two of the commonest problems of this region, hernias and

.....%'..%-.

hydroceles.

Anatomy of the processus vaginalis.

Inguinal hernias
Anatomically these are virtually all indirect and often complete (that is, the sac comes all the way t-o the scrotum). Infantile hernias occur in about 1-2% of births and are much more common in premature babies than in full term infants. They appear as an intermittent, usually reducible, lump in the groin. The correct management is a surgical herniotomy when the child's condition allows. In most uncomplicated cases this should be within two or three weeks of diagnosis. Infant hernias should be referred to regional centres where appropriate anaesthetic and surgical support is available to allow early repair rather than waiting until an infant is old enough, perhaps over 1 year old, for it to be repaired locally.
Inguinoscrotal hernia.

-nguinoscrotal hernia. |

Inguinal hernias in infants need early referral for surgery because of the high incidence of complications
Almost one third of premature infants weighing less than 1000 g will develop a hernia

Most infant hernias tend to become complicated, usually by becoming irreducible and obstructed. The lump becomes tender, and the infant starts to vomit and refuse feeds. This is a surgical emergency
as not

only is te incarcerated bowel at risk but the testis is also as its

Huge, acutely obstructed inguinoscrotal hernia.

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vessels become compressed at the neck of the hernial sac. In fact, testicular atrophy is not uncommon if the treatment is substantially delayed. An attempt should be made to reduce the hernia by taxis, since if this is successful it can buy time for a planned rather than an emergency repair. Although inguinal hernias are less common in girls, they too may incarcerate, and the most commonly incarcerated viscus is the ovary.
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Hydroceles
The formation of a hydrocele in a child implies the presence of an unobliterated or patent processus vaginalis that allows fluid to track down from the peritoneal cavity. Hydroceles are scrotal swellings that are usually asymptomatic and often have a bluish appearance. They are also commonly bilateral. The testis can be easily distinguished. Most infant hydroceles (perhaps 90%) will spontaneously subside owing to ongoing changes in the processus. Although hernias and hydroceles coexist (they have similar anatomy), only a confident diagnosis of a hernia requires early surgical intervention. Some hydroceles persist beyond infancy, and some occur for the first time later in childhood. These hydroceles do require an operation for the processus to be ligated and the distal sac emptied of fluid.
Left sided hydrocele.

Undescended testes

npalpabl a
al
AU*nt

Abnormalities in the complex migration

pattern

of the human testis

may

lead

to

several clinical possibilities which have the common feature of the testis not being in the

Dysplastic

scrotum.

A careful examination should

determine whether the testis is palpable or

impalpable.

Possible

reasons

for testis not being in scrotum.

Palpable testis In most cases the testis is palpable, and there are three common
causes.

Retractility is common and occurs when there is an exaggerated cremasteric reflex pulling a normal testis back into the groin at the least provocation. It is diagnosed by careful examination and perhaps from the patient's history (the testis may have been noted to have been within the scrotum during infancy). It is usually possible to manipulate the organ down into the scrotum, where it should lie without tension or restriction. If this is so, and a second examination a few months later may be needed to confirm it, then no further intervention is needed as increasing age causes the testis to spontaneously reside for longer periods in the scrotal pouch. There is one catch that has recently been documented-the so called ascending testis syndrome. In a small proportion of boys, a previously normal or retractile testis will become high with a foreshortened spermatic cord that prevents it from staying in the scrotum. It is usually diagnosed in boys aged 8-10 who are otherwise normal and needs a corrective operation.
Empty right hemiscrotum due to undescended
testis.

Prevalence of undescended testes

6.7% of boys at birth 1.5% of boys at 3 months of age John Radcliffe cryptorchidism study'

An ectopic testis accounts for about 10% of extrascrotal testes. These descend normally through the inguinal canal but then deviate into unusual sites such as the perineum or the femoral triangle. They are otherwise normal, and an orchidopexy is relatively simple to perform as there is no shortage of vascular length.
testes

An undescended testis may be defined as one which has been arrested anywhere along its normal pathway of descent. This, of course, may range from an intra-abdominal and hence impalpable testis to a testis that is palpable just outside the scrotum. Testicular descent is a dynamic process, and about 5% of full term infants (and about 25% of premature infants) will not have a descended testis at birth whereas at 12 months fewer than 2% will still have an undescended testis.
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Possible reasons for an impalpable testis

* Anorchia-There is no testis * Intra-abdominal testis-The testis is undescended and lies within the abdomen * Dysplastic testis-The testis is small and usually very abnormal

Impalpable testis The possible reason for a testis being impalpable is that the testis is missing, it is small (dysplastic), or it is lying within the abdomen. It is important to try to find an impalpable testis, and several tests have been used. Ultrasonography and even computed tomography are often requested, though both are insensitive investigations in a young child. The most accurate method available is a diagnostic laparoscopy performed under general anaesthesia. This not only allows clear identification of anorchia if it is present but also allows the size of an intra-abdominal testis to be assessed so that an appropriate treatment plan can be formulated. Infants with bilateral impalpable testes are at particular risk of having other anomalies such as intersexuality and the prune belly syndrome. These children need early referral for specialised investigations, such as chromosomal analysis and endocrine
investigations.

Management of undescended testes

The management of these anomalies varies with age and palpability, and so an accurate clinical diagnosis is crucial. The most controversial aspect has been at what age surgery should be performed. Thirty years ago boys were left until puberty before an orchidopexy was carried out. Gradually, the recommended age for orchidopexy has come down, and now most paediatric surgeons would undertake an elective orchidopexy in boys aged 1-4 years. This change in attitude has been based on the changes, presumed to be induced by temperature, that an undescended testis undergoes outside the scrotum. Histological abnormalities can be seen if the testis is still undescended after 4 years of age, and some authors suggest that changes can be seen with an electron microscope as early as 2 years of age. However, other workers suggest that any microscopic changes are pre-existing and are the reason for maldescent rather than a consequence. Macroscopic testicular anomalies such as malunion of the epididymis and testis are certainly commoner in such testes. About 10-20% of undescended testes are associated with a clinical inguinal hernia, and in this situation the treatment of the hernia takes precedence (an orchidopexy can be carried out at the same time however young the child is). Orchidopexy involves mobilisation of the testis on its essential structures (the vas and the testicular vessels) by cutting just about everything else. This achieves greater length of the spermatic cord so that the testis can be brought down to lie in the scrotum without undue tension. Most surgeons would also fix the testis in the scrotum with a sub-dartos pouch. Although such techniques are entirely appropriate for about 90% of all situations, the higher the testis the more difficult this becomes. Special techniques have been developed for managing an intra-abdominal testis, including microvascular
surgery.

Management ot undescended testes.

Cryptorchidism (Greek, kryptos-hidden, orchis-testicle) Orchidopexy (Greek, orchis + pexis-fixation)

Rationale for orchidopexy

* * *

Possibly improves spermatogenesis Possibly decreases risk of malignancy Cosmetic improvement

Surgical consequences Spermatogenesis-The original rationale for orchidopexy was to improve the chances of spermatogenesis. This aspect has been little studied, largely due to the inevitably long gap between the operation and an appropriate measure of outcome (that is, fertility or sperm counts). One study from Germany showed that 90% of men achieved fertility if their orchidopexy had been performed before 2 years of age, but this fell to 15% if orchidopexy was delayed until after 13 years of age. Other workers consider the testes' original position to be more important than the age at which the surgery is performed. In one Dublin study all of the adults who had had an orchidopexy for bilateral impalpable testes as children were completely azoospermic at follow up, whereas over 80% of men who had had an orchidopexy for a unilateral impalpable testis had normal sperm counts.
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Risks of malignancy for the undescended testis

Increased risk Unilateral 5.1-fold Bilateral 11-7-fold Royal Marsden study2

One boy in 120 with an undescended testis will develop malignancy2

1 John Radcliffe Cryptorchidism Study Group. Cryptorchidism: an apparent substantial increase since 1960. BMJ 1986;293: 1401-4. 2 Chilvers C, Pike MC. Epidemiology of undescended testis. In:

Malignancy-Undescended testes have a higher incidence of histological abnormalities, and presumably the increased risk of malignant change is related to this. Originally, a 35-fold increase in risk of malignancy was quoted for a unilateral undescended testis, but more recent work has calculated this to be about a fivefold increase in risk. It used to be thought that all an orchidopexy did was to make the testis palpable and hence improve earlier detection, while the risk itself remained unchanged. However, recently published evidence from a large British study has suggested that an early orchidopexy actually reduces the risk of subsequent malignancy.' Prostheses-Absence of a testis (either inherent or iatrogenic) should probably be treated by placement of a testicular prosthesis. The prostheses are filled with silicone gel and are available in various sizes to match the other side (which has often hypertrophied). They should be implanted during adolescence since earlier surgery merely has to be repeated as the contralateral testis increases in size.
Professor Lewis Spitz, Institute of Child Health, London, provided the pictures of

Oliver RTD, BlandyJP, Hope-Stone HF, eds. Urological and genital cancer. Oxford: Blackwell, 1989:306-21. 3 Woodhouse CRJ. Late malignancy risk in urology. BrJ7 Urol 1992;70:345-5 1.

obstructed inguinoscrotal hemia and empty hemiscrotum.

The ABC of Paediatric Surgery is edited by Mark Davenport, consultant paediatric surgeon, department of paediatric surgery, King's College Hospital, London.

How To Do It
Work in the European Union
Frances Klemperer
Continental Europe is now only 20 minutes away by train from mainland Britain, and moving ever closer politically. Mutual recognition of medical qualifications within the European Union is well established: working in other parts of Europe is, in principle, straightforward. Working in different health care systems can offer new perspectives on British medical practice and the NHS. But the cultural differences and practical difficulties are not always easy to overcome. Registration Working in Europe has never been easier. Mutual recognition of medical qualifications applies throughout the European Union and its partners in the European Economic Area (Norway, Iceland, and Liechtenstein). Any doctor who is a citizen of, and who trained in, a member state is free to work as a doctor in the other member states.' 2 The first step is to register as a medical practitioner in the country where you want to work. There is no centralised registration system. You will have to go through the procedures unique to each member state. This is likely to cost several hundred pounds, take several months, and require a good deal of determination. The British Medical Association can provide addresses of sister organisations and registering bodies equivalent to the General Medical Council. As in the United Kingdom, registration requires a large number of documents, both originals and notarised translations; these can be arranged through any reputable translator -try Yellow Pages. Allow time to collect them all. They may have to be sent, or even taken in person, to myriad different official departments. You may have to relinquish originals of the most important documents for a long period of time. Some countries require documents that do not exist in the British system-for example, the "national proof of good character or good
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repute" (required in Belgium), the "certificate of good conduct"--issued by the police (required in Germany), or the "testimonial of morality and honesty" (required in Greece). An imaginative response, perhaps a letter from your head of department, will probably be more effective than seeking further advice from either the country's registering body or its London embassy. European Community law requires that the registration process be completed by the authorities of the country concerned within three months of submission of your application.' There have been reports of suspected illegal discrimination against incoming doctors23: the European Public Health Alliance, an association of non-governmental organisations in the health sector, is looking into these problems. Directorate-General XV of the European Commission has responsibility for this area. The Maastricht Treaty gives a citizen of the European Union the right to petition the European Parliament if his or her rights under European law are being infringed. (The parliament will, if it sees fit, then forward the complaint to the commission for further investigation.)

Community Help Service, Rue St Georges 102, Box 20, 1050 Brussels, Belgium Frances Klemperer,

psychiatrist
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Certificate of specialist training Once registered as a doctor, you may also be eligible to register (and practise) as a specialist. Until recently, if trained in the United Kingdom you would have needed the certificate of specialist training, issued by the General Medical Council. To get it you had to have spent three to five years in recognised training posts and have obtained membership or fellowship of a royal college. It was not necessary to have reached consultant status, or to be "fully trained" by British standardsthat is, qualified to apply for a consultant post. These arrangements are now changing with the implementation of the Calman proposals on specialist training. The certificate of specialist training will be replaced by a certificate of specialist training that will apply equally to the United Kingdom and the rest of Europe and will
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