COMMON PAEDIATRIC SURGICAL PROBLEMS
Common surgical conditions in children
S. W. Beasley
The penis and foreskin
The glans of the uncircumcised penis is protected by a layer of loose skin called the foreskin or prepuce. The amount of foreskin present varies among boys. At birth, and for many years afterwards, it is normal for part or all of the undersurface of the foreskin to be adherent to the glans penis. This adherence slowly separates during childhood. Forcible retraction of the foreskin before it is ready can damage the glans and may cause secondary phimosis. Therefore, the foreskin should not be retracted forcibly unless a circumcision is being performed. Spontaneous separation of these adhesions is normally complete by puberty.
Smegma accumulates beneath the adherent foreskin. It appears as asymmetrical accumulations of yellow-tinged material predominantly in the coronal groove beneath the foreskin (Fig. 9.1.1). There may be sufficient smegma to produce a noticeable swelling, which may be misdiagnosed as a dermoid cyst or tumour. It is often misinterpreted as being mid-shaft because a small child’s coronal groove may be a long way from the tip of the foreskin. Smegma is normal, and is released spontaneously as the foreskin separates from the glans penis. When it is released, it may be associated with some redness and irritation of the foreskin for a day or so: this, too, is a normal process.
Infection can develop beneath the foreskin and, if severe, pus may appear from the end of the foreskin. Balanitis is often associated with phimosis. Infection may cause considerable redness and swelling of the penile shaft, necessitating treatment with either topical or oral antibiotics.
In phimosis the opening at the tip of the foreskin has narrowed down to such a degree that the foreskin cannot be retracted (Fig. 9.1.2). The external urethral meatus is not visible. Phimosis must be distinguished from the normal adherence of the foreskin to the glans. In most boys, phimosis can be treated by application of steroid ointment (e.g. betamethasone valerate ointment) to the tight, shiny part of the foreskin. This usually obviates the need for circumcision. However, marked previous inflammation, infection, skin splitting and balanitis xerotica obliterans can lead to marked scarring of the foreskin and phimosis, and in many of these children the only reasonable treatment is circumcision. Sometimes the severity of phimosis is such that there is ballooning of the foreskin on micturition, and on rare occasions it may even cause urinary retention with a distended bladder. A degree of phimosis is common in infancy but tends to resolve spontaneously in the first few years of life, and is not considered abnormal in this age group.
Paraphimosis occurs when a mildly phimotic foreskin has been retracted over the glans and has become stuck behind the coronal groove, causing oedema of itself and the glans penis (Fig. 9.1.3). It is a painful and progressive process. Treatment involves gentle manipulation of the foreskin forwards, which may require a general anaesthetic. Circumcision is not performed at this time, but a few children may need it subsequently if the phimosis does not respond to topical application of steroid ointment.
It is important to recognize hypospadias when it is present (Fig. 9.1.4). The foreskin looks square and hangs off the penis, and the shaft of the penis is bent ventrally. The two main problems in hypospadias are:
• the location of the urethra (which can be found on the ventral side of the shaft of the penis, proximal to its correct position)
• chordee (ventral angulation of the shaft and glans) Correction of chordee to straighten the penis is required to allow later successful sexual function. The operation is usually performed as a single-stage procedure at 9–12 months of age, often as day surgery.
Circumcision is absolutely contraindicated in hypospadias because the skin of the prepuce is used during repair of the hypospadias. Severe hypospadias may be indicative of an intersex abnormality. For example, when there is penoscrotal hypospadias and a bifid scrotum, the scrotum should be examined carefully for testes, because some of these children may be females with congenital adrenal hyperplasia; the labioscrotal folds are labia rather than scrota, and the presumed urethral opening may in fact be the entrance to the vagina (Ch. 19.3).
The indications for circumcision remain controversial. In many countries, circumcision has been abandoned in the neonatal period because of its relatively high complication rate. Apart from the risk of septicaemia and meningitis when performed in the relatively immunologically immature neonate, there are a number of problems that may occur during circumcision at any age. These include removal of too much or too little foreskin, postoperative bleeding and infection. Haemorrhage postoperatively occasionally requires surgical reintervention. The most troublesome and common complication of circumcision is abrasion and ulceration of the sensitive glans penis, particularly near the urethral meatus. As the meatal ulceration heals it may produce meatal stenosis and require a meatotomy to re-establish an adequate urinary stream.
In epispadias, the urethra opens on to the dorsal aspect of the base of the penis. Epispadias is part of a spectrum of lower abdominal wall defects in which ectopia vesicae (bladder exstrophy) and cloacal exstrophy are the most severe forms. Boys with epispadias are often incontinent of urine because the sphincter of the bladder neck is also deficient.
James was a 7-year-old boy who presented following two episodes of balanitis. He also complained of discomfort on micturition. Examination revealed a tight foreskin that could not be retracted; the urethral meatus could not be seen. After 1 month of topical application of betamethasone ointment four times a day to the tight part of the foreskin, he was able to fully retract it. Circumcision was not necessary.
The inguinoscrotal region
After the testis has descended into the scrotum during the seventh month of pregnancy, the canal down which it migrates, the processus vaginalis, should obliterate. Failure of obliteration of the processus vaginalis may produce an inguinal hernia, a hydrocele or an encysted hydrocele of the cord.
A widely patent proximal processus vaginalis allows bowel (and, in girls, the ovary as well) to enter the inguinal canal, producing a reducible lump in the groin called an indirect inguinal hernia (Fig. 9.1.5). This occurs in about 2% of live male births but is less frequent in girls. The greatest incidence is in the first year of life.
The usual presentation is that of an intermittent swelling, overlying the external inguinal ring, that has been noticed by a parent. At times it may appear to cause discomfort. It is most likely to be obvious during an episode of crying or straining, and in infants may be seen during nappy changes. Inguinal hernias should be repaired as soon as practicable.
Strangulation of inguinal hernias is common, particularly during the first 6 months of life. Strangulation can be recognized when the groin swelling becomes irreducible. If left untreated, a strangulated hernia may damage the incarcerated bowel and, occasionally, by compressing the testicular vessels may lead to testicular atrophy. For this reason, an immediate attempt should be made to reduce the hernia manually. This is done by first disimpacting the hernia at the external inguinal ring, and then reducing it along the line of the inguinal canal.
Fortunately, most hernias that become stuck can be reduced manually; the hernia can then be repaired as an elective procedure within a few days. This is best done in a specialist paediatric surgical centre.
A hydrocele presents as a painless cystic swelling around the testis in the scrotum (Fig. 9.1.6). It contains peritoneal fluid that has tracked down a narrow but patent processus vaginalis. It transilluminates brilliantly. When the hydrocele is lax, the testis can be felt within it. The upper limit of the hydrocele can be demonstrated distal to the external inguinal ring, distinguishing it from an inguinal hernia, where the swelling extends through the external inguinal ring. There is no impulse on crying or straining.
Hydroceles are common in the first few months of life, do not cause discomfort and usually disappear spontaneously. Surgery is only indicated if the hydrocele persists beyond 2 years of age.
Undescended testis (or cryptorchidism) is a term used to describe the testis that does not reside spontaneously in the scrotum. Undescended testes occur in about 2% of boys, being more common in premature infants. Spontaneous descent of the testis is unlikely beyond 3 months post-term. Cryptorchidism is important to detect because it will result in reduced fertility if left untreated. It is suspected that the higher temperature to which an undescended testis is subject impairs spermatogenesis.
The diagnosis is made by examining the inguinoscrotal region. Normally, the testis should be found within the scrotal sac. In cryptorchidism the scrotum looks empty (Fig. 9.1.7). The testis is ‘milked’ down the line of the inguinal canal towards the scrotum and is pulled gently towards the scrotum. If the testis cannot be brought into the scrotum or will not remain there spontaneously it is considered undescended.
Clinically, it may be difficult to distinguish a retractile testis from an undescended testis. In most normal boys the testis resides in the bottom of the scrotum, but the cremasteric reflex, which is prominent during mid-childhood, may cause it to move upwards, sometimes completely out of the scrotum. A retractile testis found outside the scrotum initially can be brought down into the normal position and should stay there spontaneously, at least until the cremasteric reflex is stimulated (Table 9.1.1). An undescended testis will not stay in the scrotum spontaneously and usually cannot even be coerced beyond the neck of the scrotum. It is often smaller than a normal testis on the other side.
Undescended testes should be brought down into the scrotum surgically between 9 and 12 months of age. Unfortunately, in many boys the diagnosis is not made until the child is older. The later the testis is brought down, the more likely it is that there will be damage to spermatogenesis. Orchidopexy is performed as a day case procedure. In general, the results are excellent when the procedure is performed by a specialist paediatric surgeon.
The acutely painful scrotum
There are a number of conditions that cause an acutely painful or enlarged scrotum (Fig. 9.1.8), of which torsion of a testicular appendage is the most common, and torsion of the testis itself the most important (Table 9.1.2). In both conditions the boy complains of severe pain in the scrotum. In the early stages of torsion of a testicular appendage, a blue-black ‘pea-sized’ swelling which is extremely tender to touch may be seen through the skin of the scrotum near the upper pole of the testis. Palpation of the testis itself causes no discomfort. Later, a reactive hydrocele develops, the tenderness becomes more generalized and the clinical features may make it difficult to distinguish from torsion of the testis. Where torsion of the testis has occurred, both the testis and the epididymis are exquisitely tender (unless necrosis has already occurred) and the testis may be lying high within the scrotum. In older boys the pain radiates to the ipsilateral iliac fossa and may be associated with nausea and vomiting, producing symptoms similar to those of appendicitis, which highlights the importance of always examining the scrotum in boys presenting with lower abdominal pain.
Urgent surgical exploration of the scrotum is required to untwist the testis and epididymis and to suture both testes to prevent subsequent torsion. A completely necrotic testis should be removed. A torted and infarcted testicular appendix should be removed. In this situation the testis should be checked to make sure that it has not twisted but otherwise it requires no treatment. When a testis has twisted the contralateral testis should also be examined and ‘pexed’ to prevent it from twisting at a later date.
Other causes of scrotal pathology
Epididymo-orchitis is unusual in children: it is most often seen during the first year of life, where it may signify an underlying structural abnormality of the urinary tract. For this reason investigation involves a renal ultrasound and micturating cystourethrogram. Examination of the urine may show leukocytes and bacteria. Mumps orchitis is extremely rare prior to puberty. In idiopathic scrotal oedema there is painless boggy oedema of the whole scrotum and the testes are completely non-tender. Testicular malignancy is occasionally seen in leukaemia or with a primary testicular neoplasm.
Abnormalities of the umbilicus
The umbilical cord desiccates and separates several days after birth, allowing the umbilical ring to close. Sometimes the stump of the cord may become infected, the umbilical ring may not close, or there may be remnants of the embryonic channels that pass through the umbilicus before birth (Table 9.1.3).
Failure of the umbilical ring to close after birth produces an umbilical hernia (Fig. 9.1.9). Umbilical hernias are common in neonates but most close spontaneously in the first year of life. The skin overlying the umbilical hernia never ruptures and strangulation of the contents is virtually unknown. The swelling will become tense when the infant cries or strains. Umbilical hernias normally do not cause pain. No treatment is required during the first few years of life. If the hernia is still present after the age of 3 years it can be repaired as a day surgical procedure.
Discharge from the umbilicus
Discharge from the umbilicus may be pus, mucus, urine or faeces. An umbilical granuloma is a common lesion that first becomes evident after separation of the umbilical cord. There is a small accumulation of granulation tissue in the umbilicus, accompanied by a seropurulent discharge. If it has a definite stalk it can be ligated without anaesthesia, but most often it is treated by topical application of silver nitrate. Ectopic bowel mucosa has a similar appearance but has a smooth, red, glistening surface and discharges mucus. It is treated in the same way. Persistence of part or all of the vitellointestinal (omphalomesenteric) duct produces one of a number of abnormalities, which usually present in early infancy but may not be evident for some years. Complete patency of the tract allows ileal fluid and air to discharge from the umbilicus. Persistence of one part produces a sinus or cyst, which may become infected to form an abscess and may discharge pus. A vitellointestinal band attaching the ileum to the deep surface of the umbilicus may cause intestinal obstruction. A Meckel’s diverticulum represents persistence of the ileal part of the duct. Vitellointestinal duct remnants are excised.
Urinary discharge from the umbilicus suggests a persistent communication with the bladder in the form of a patent urachus. Sometimes it may produce a cystic mass or abscess in the midline just below the umbilicus. Urachal remnants should be excised.
The anus and perineum
A variety of unrelated conditions affect the anus and perineum in children.
These are usually seen in infants and toddlers when passage of a hard stool splits the anal mucosa, causing sharp pain and often a few drops of bright blood. The condition is of little consequence and the fissure usually heals within days. Examination of the anal margin shows a split in the epithelium anteriorly or posteriorly in the midline. An anal fissure may occur in an older child, in which situation it tends to be related to constipation. The child gets severe pain on defaecation and becomes reluctant to defaecate, further worsening the constipation. Treatment is directed at overcoming the underlying constipation. A stool softener and lubricant, e.g. paraffin oil, may be helpful. A chronic indolent, often non-painful, fissure away from the midline may indicate inflammatory bowel disease, such as Crohn disease.
These are most likely to occur in the first year of life from infection of an anal gland. The abscess points superficially, a centimetre or two from the anal canal. The abscess should be drained and the fistula between the abscess and the anal canal laid open to reduce the likelihood of recurrence.
Rectal prolapse tends to occur in the second and third years of life in otherwise normal children. The rectum prolapses during defaecation and returns spontaneously afterwards. In some, manual reduction is required. The prolapsed mucosa may become congested and bleed but causes little discomfort. Clinically, it needs to be distinguished from prolapse of a benign rectal polyp (a benign hamartomatous lesion seen in children) and the apex of an intussusception (the child would have other symptoms of intussusception). The passage of time, and treatment of any underlying constipation, is all that is required in the majority of toddlers. Occasionally, a sclerosant is injected into the submucosal plane of the rectum for persistent cases.
In a few patients there is an underlying organic cause for the rectal prolapse. Usually the reason is obvious, as in paralysis of anal sphincters in spina bifida and sacral agenesis, undernourished hypotonic infants, bladder exstrophy, cloacal exstrophy, following surgery for imperforate anus, or malabsorption.