Introduction

The acute conditions described in Chapter 50 are largely congenital disorders presenting in the neonatal period, whereas non-acute conditions present across the whole of childhood. The most common referrals are hernias and associated problems, abnormalities of testicular descent and foreskin problems. Less often, surgeons manage chronic or recurrent abdominal pain, chronic constipation, rectal bleeding, an abdominal mass or rectal prolapse. Many children present first to a paediatrician and are referred to a paediatric or general surgeon.

Finally, a range of urological problems occur in infancy and childhood. Most are unique to young patients and are usually dealt with by paediatric urologists. Congenital urological abnormalities are often diagnosed by ultrasound in the antenatal period.

Embryology

The indifferent gonad (i.e. ovary or testis) begins to develop at the fifth week in the gonadal ridge, part of the mesodermal urogenital ridge that will also form the kidney, ureter and genital ducts in the male or uterus and uterine tubes in the female. At the lower pole of the putative testis, a strand of mesenchyme becomes the cord-like gubernaculum. In the eighth week, a prolongation of peritoneum, the processus vaginalis, appears beside the gubernaculum (or round ligament), and extends into the labioscrotal fold. The testis then migrates distally along the peritoneal canal.

Patent processus vaginalis (PPV)

This term should be reserved for hydrocoeles communicating with the peritoneal cavity via a remnant too narrow to admit bowel. Children with these communicating hydrocoeles present with scrotal swelling that increases during the day as peritoneal fluid accumulates and subsides at night. They are seen up to the age of 3 years. Treatment is excision of the peritoneal remnant as in herniotomy after the age of 2 (see below). Before this age, most settle spontaneously.

Hydrocoele

Non-communicating hydrocoeles are mostly seen in neonates and young babies (Fig. 51.2). The usual type is a scrotal swelling resulting from incomplete reabsorption of fluid within the tunica vaginalis after closure of the processus vaginalis. There may be a separate hernia present. These so-called ‘primary hydrocoeles’ sometimes appear following a viral illness. Rarely, a secondary hydrocoele results from testicular trauma or torsion, epididymitis or a testicular tumour.

On examination, there is a fluid swelling surrounding a normal testis; the sac transilluminates brightly and the testis can be felt posteriorly (but note that neonatal bowel is thin and may also transilluminate brightly in a hernia). If this cannot be achieved, investigation needs to exclude a secondary hydrocoele. In inguinal hernia the examiner cannot ‘get above’ the swelling, i.e. the swelling originates in the groin; this is the cardinal feature. Hydrocoeles also occur in the spermatic cord (hydrocoele of the cord) or in the round ligament in girls where they are known as hydrocoeles of the canal of Nuck. Most hydrocoeles resolve between 18 and 24 months of age. A hydrocoele persisting beyond the age of 2 or appearing later may require surgery.

Inguinal hernia

Inguinal hernias in children arise because the processus vaginalis fails to close after testicular descent; they are true congenital abnormalities. Anatomically, they are the same as indirect inguinal hernias in adults (see Ch. 32) but without a substantial abdominal wall defect. The incidence in infants ranges from 1% to 4.4%, with a male preponderance of 4 : 1; 98% are indirect. The incidence in premature neonates is 30% and the overall incidence is increasing in line with the number of premature neonates surviving.

A hernia usually presents as a lump at the external inguinal ring, when the child cries or strains at stool, but then reduces spontaneously. When seen electively, there is often no abnormality, but most surgeons accept a parent’s clear history of a hernia and arrange surgery. With larger defects, a lump is constantly present and expands during crying. The urgency of hernia repair is governed by age; the older, the less likely it will become incarcerated (acutely irreducible). Infants at home should have hernias fixed on the next available operating list. Neonates and pre-term infants in hospital should have them fixed before discharge. Older children without episodes of incarceration can have hernias fixed electively.

Inguinal hernias may become acutely irreducible and painful, sometimes with obstructive symptoms such as vomiting (see Ch. 50). In these cases, there is a real risk of testicular necrosis and/or strangulation of the hernia contents, e.g. bowel or ovary (see Fig. 50.10). In known hernias, parents should be instructed to bring the child to hospital for urgent herniotomy if it becomes incarcerated to prevent these risks.

The standard operation is inguinal herniotomy, a common general paediatric operation. In babies and children, this involves separating the peritoneal sac from the cord (or round ligament), ligating it at the external ring and removing it. There is rarely any need to perform a repair (herniorrhaphy); see Figure 51.3. The incidence of an undiagnosed contralateral hernia in boys is between 1 : 8 and 1 : 13 but contralateral groin exploration is no longer performed in the UK.

Femoral hernia

Femoral hernias are much less common than inguinal hernias in children and are located lower and more medially in the groin. Enlarged lymph nodes can also occur here. Operation includes removing the sac and suturing the medial part of the inguinal ligament to the pectineus fascia to narrow the femoral canal.

Umbilical hernia

Many newborn babies have umbilical hernias, particularly if premature (Fig. 51.4), but the defect usually cicatrises and resolves during the first 2 years of life; they are common in Afro-Caribbean babies and can run strongly in families. Small umbilical hernias may undergo spontaneous closure up to 4–5 years of age. Rarely, they become incarcerated or strangulate. Indications for repair are symptoms, persistence beyond 5 years and perhaps social pressure to prevent teasing. The size of the abdominal wall defect should be determined; large defects (> 2 cm) are less likely to close spontaneously, although very large swellings may have small abdominal wall defects likely to close spontaneously. It is important to differentiate umbilical hernias from epigastric hernias which do not close spontaneously. At operation, a small subumbilical ‘smile’ incision allows emptying and ligation of the peritoneal sac and placement of a few absorbable repair sutures. The umbilical skin is usually sutured to the repair to restore its normal recessed appearance.

• Neoplasia—carcinoma-in-situ is present in 2% of undescended testes and maldescent has up to 10 times the normal risk of testicular malignancy (although the risk is still small); if surgical correction is done sufficiently early, it may reduce this risk but the principal purpose is for the patient to perform self-examination and report lumps in later life. Long-term follow-up after orchidopexy is desirable. Seminomas are the most common tumour (60%) and usually present between 20 and 40 years of age. It is important to inform the parents of the increased risk of malignancy and to reinforce the importance of testicular self-examination in adult life

• Subfertility—maldescended testes exhibit incomplete maturation of seminiferous tubules, leading to sperm abnormal in quantity, form or motility. This may be by virtue of being at normal body temperature instead of at least 1° cooler in the scrotum. Early orchidopexy helps maturation of the tubules and spermatogenesis, ideally between 6 and 12 months of age. Patients with unilateral maldescent have more subfertility and lower sperm counts. Those with bilateral intra-abdominal testes have the most subfertility

• Torsion—incompletely descended testes are abnormally mobile. Torsion of the testis, actually torsion of the spermatic cord, causes strangulation of blood supply, testicular necrosis and later atrophy. Torsion sometimes occurs during intrauterine life but may happen at any age. Intrauterine or neonatal torsion occurs proximal to the reflection of the tunica vaginalis (i.e. extravaginal). Infarction results in atrophy and loss of the testis so that at laparoscopy, only blind-ending testicular vessels and vas deferens are found. The condition occurs bilaterally in up to 30%

• Psychological—normal genitalia are important in the development of body image, gender acceptance and personality in adolescence. Orchidopexy at an early age provides reassurance to the child and parents

Surgery for testicular maldescent

The optimum age for operation is 6 months to 1 year so fertility is not compromised. The usual technique of orchidopexy involves mobilising the testis and spermatic cord through a groin incision, separating and excising the processus vaginalis and placing the testis in a subcutaneous pouch outside the dartos muscle via a separate scrotal incision. For intra-abdominal testes, the operative technique is a laparoscopic staged Fowler-Stephens procedure. In the first stage, the testis is mobilised by dividing the testicular artery and vein intra-abdominally. Six months later the testis is mobilised on the vas with its attendant artery and delivered into the scrotum laparoscopically. This approach has improved the success rate for intra-abdominal testes to almost 90%.

Phimosis

In phimosis, a tight fibrotic ring develops at the end of the foreskin preventing retraction. Primary phimosis presents with chronic foreskin irritation or ‘spraying’ on micturition. ‘Ballooning’ on micturition is just a sign of a non-retractile foreskin and is not necessarily pathological. There is often a history of recent or recurrent balano-posthitis (infection beneath the foreskin). Most boys with primary phimosis do not require circumcision; careful attention to hygiene (avoiding forcible retraction) allows the prepuce to retract normally in time. Attempts to dilate the phimosis under anaesthesia are unsuccessful as this causes further scarring and rapid relapse.

Circumcision may be indicated in cases of recurrent infection particularly in boys with upper renal tract abnormalities or other congenital problems such as posterior urethral valves and may help to reduce the incidence of UTIs in these boys. In young men, a non-retractile foreskin may cause sexual problems. For these, the lesser operation of preputioplasty may suffice if the phimosis is not too tight. This involves dividing the tight band longitudinally and suturing it transversely, increasing the meatal calibre to allow easier retraction.

Secondary phimosis is usually due to balanitis xerotica obliterans (BXO) which is characterised by a thickened, whitish, fibrotic non-retractile foreskin. Plaques are formed on the deep surface which adhere to the glans and may cause meatal stenosis. The peak incidence is around 8 years and the condition is a definite indication for circumcision.

Paraphimosis

Paraphimosis sometimes occurs in children, especially in phimosis. The tip of the foreskin forms a tight band when retracted and becomes trapped in the coronal sulcus behind the glans. The band inhibits venous return and causes swelling of the glans, making return of the prepuce even more difficult. Paraphimosis is painful and requires urgent reduction. This can sometimes be achieved using EMLA cream (local anaesthetic) or a penile block. Manual compression of the glans often allows reduction but if this fails, general anaesthesia is needed. Sometimes the band needs dividing with a dorsal slit. Circumcision is usually performed later once oedema has settled.

Pathophysiology

In the normal individual, the distal ureter passes obliquely through the bladder wall so as bladder pressure rises, the ureter is flattened, acting as a sphincter preventing urine from refluxing (‘flap-valve’ anti-reflux mechanism). This prevents transmission of intermittent high bladder pressures. Primary vesicoureteric reflux is most common and usually results from a minor (often familial) abnormality of ureteric insertion or from ectopic or duplex ureters or congenital megaureter (a peristaltic abnormality). Secondary VUR may be caused by bladder outlet obstruction, neuropathic bladder or surgical procedures to the lower end of the ureter.

Ascending infection of the upper tracts begins with bacteria reaching the bladder via the urethra and colonising it. Infected urine refluxes into upper tracts but cannot be cleared effectively from it, infecting the upper tract. Lower tract infections can also cause inflammation at the VUJ, preventing it from closing, aggravating reflux.

Reflux of sterile urine into the pelvicalyceal system during early childhood probably causes impaired renal development and function. Mild, non-dilating VUR (grades I to III; see Table 51.1) causes little damage but severe (dilating) VUR (grades IV and V) may cause renal scarring and reflux nephropathy, which may progress to irreversible renal damage if untreated (Fig. 51.6). If both kidneys are involved, this eventually results in renal insufficiency and hypertension. Scarring typically occurs apically in the kidneys. Loss of the normal conical shape of the papillae allows intrarenal reflux, which in the presence of infection results in pyelonephritis and renal scarring.

Clinical presentation and investigation

During antenatal screening, VUR can be detected as urinary tract dilatation. Another common presentation is one or more urinary tract infections at any age. Girls are more prone than boys after the first year, because of a short urethra and its proximity to the anus. Note that infants or young children with urinary infections may not exhibit urinary symptoms or signs; the diagnosis is often made on investigation of vomiting, fever or failure to thrive. Older children typically present with incontinence, frequency or dysuria, or abdominal pain and tenderness (mimicking appendicitis). In symptomatic urinary tract infections, the prevalence of vesicoureteric reflux is as high as 50% in neonates, and 30% in those aged 2–18 years.

In a child with urinary tract infection, clinical examination seeks evidence of abnormal external genitalia, spina bifida and impaired perineal innervation (sensation and anal sphincter tone). To demonstrate reflux, the sequential investigations are ultrasound scan, micturating cystography, and isotope scans using DMSA and MAG3, coupled with indirect radionuclide cystography. Micturating cystography should only be used in selected cases (Fig. 51.6); there is a 1% risk of pyelonephritis and the test is stressful for child and parent. It is the gold standard for demonstrating VUR and excluding posterior urethral valves but involves instilling contrast into the bladder via a urinary catheter which is then removed and X-rays taken during voiding. The radiological grades of severity are shown in Table 51.1 and provide a useful guide to the likelihood of future renal damage. Severe dilating VUR requires isotope studies: ^99mTc DMSA, bound to renal tubules, shows differential renal function and scarring within renal parenchyma. In older children, an excretion MAG3 scan and indirect radionuclide cystogram shows differential function, reflux and sites of urinary tract obstruction.

Management of vesicoureteric reflux

With no other anatomical abnormalities and an undilated ureter (i.e. grades I and II), there is an 85% chance of resolution of reflux as the child grows. In the meantime, the risk of urinary infection should be minimised by encouraging high fluid intake, avoiding constipation and maintaining perineal hygiene, plus medical management of bladder dysfunction. The child is maintained on continuous antibacterial chemotherapy (such as trimethoprim) and followed-up regularly with serial ultrasound scans and charting growth and development, blood pressure and plasma creatinine.

Surgical correction becomes indicated when there are recurrent infections, deterioration of upper tract function or non-compliance with medical management. Otherwise, surgery is reserved for severe dilated VUR with complications and for other obvious anatomical abnormalities. Newer treatments include injection of Deflux into the submucosa of the ureter at the junction with the bladder; success rates of up to 90% are possible. Less commonly operations aim to reimplant the ureter so that a length of it lies deep to the bladder mucosa; during voiding this is flattened restoring an anti-reflux mechanism.

Without renal scarring, the child can be discharged from follow-up after operation. With unilateral scarring, blood pressure should be monitored lifelong for hypertension. In bilateral scarring, renal function must also be monitored.

Pathophysiology

Obstruction at the pelviureteric junction (PUJ) may be unilateral or bilateral, and can present between birth and the end of the fourth decade. It affects both sexes equally. PUJ dysfunction is a congenital condition that manifests as dilatation of the renal pelvis and calyces (hydronephrosis) and incomplete or intermittent obstruction of the PUJ. Usually a functional abnormality, there is an aperistaltic segment of ureter that lacks muscle. Aberrant lower pole vessels may cause mechanical obstruction in older children. A normal PUJ prevents urine reflux into the kidney when the ureter contracts, but in PUJ obstruction, urine accumulates and dilates the pelvicalyceal system. This increases pressure in the renal collecting system which may cause deterioration of renal function. Stasis may also predispose to infection and stone formation.

Clinical presentation and diagnosis

PUJ dysfunction is now most often diagnosed antenatally. Management is regular postnatal follow-up with ultrasound. Those with persistent or progressive hydronephrosis are investigated for VUR, renal function and renal drainage effectiveness and treated appropriately.

Many with PUJ dysfunction go undetected. Others are discovered by chance on ultrasound or urography for an apparently unrelated condition. Symptoms may be intermittent: some patients complain of aching in the renal area; others suffer bouts of severe abdominal or loin pain (renal colic), some with urinary tract infection or haematuria (which may be induced by exercise). Symptoms can be exacerbated by drinking large volumes of fluid precipitating a Dietl’s crisis, or by sudden changes in posture.

The initial diagnosis is by ultrasound, with detection of a dilated renal pelvis. The next step is to distinguish between static non-obstructive dilatation with preserved renal function and genuine PUJ obstruction causing stasis, dilatation and deteriorating function. Radionuclide diuretic renography (^99mTc MAG3 scan) is the investigation of choice and in classical PUJ obstruction, gives a characteristic non-excretion curve.

Indications for operation include a loss of differential renal function > 10%, or more commonly an increase in the anterior–posterior diameter of the renal pelvis to > 35 mm.

Management

PUJ dysfunction with obstructive symptoms, stone formation, recurrent infections or progressive renal impairment, together with an obstructed isotope excretion curve, are indications for intervention. Pyeloplasty is indicated unless the kidney has less than 10% of total renal function, in which case nephrectomy may be indicated. Minimal invasive techniques include percutaneous antegrade endopyelotomy and laparoscopic pyeloplasty. Standard operations have a high technical success rate and usually prevent deterioration of renal function.

Upper gastrointestinal bleeding

In neonates, apparent vomiting of blood may result from swallowed maternal blood. In older infants, gastritis may be the cause. Less common causes are bleeding disorders and coagulopathy.

Lower gastrointestinal bleeding

Rectal bleeding in neonates is most often due to anal fissure, necrotising enterocolitis or malrotation with volvulus. Rectal bleeding is a common problem in older infants and children; the causes are summarised in Table 51.2 and include perianal abscess and fistula, anal fissures, large bowel polyps, rectal prolapse and Meckel’s diverticulum.

Nephroblastoma (wilms’ tumour)

Nephroblastoma presents in early childhood, with 80% presenting before the age of 5, at a median age of 3.5 years. The tumour arises from embryonal renal tissue in the kidney. Tumours are locally invasive and metastasise to regional nodes, liver, lungs and bone. Often, a large abdominal mass is noticed by the mother as the child is bathed (see Fig. 51.8). The mass is sometimes so large as to obscure its site of origin. Less common presenting features include haematuria, classically after trivial trauma, anorexia, weight loss, pyrexia and hypertension. Diagnosis is by clinical examination, and tumour size and characteristics are shown by ultrasonography or CT scan. The diagnosis is confirmed by Trucut biopsy.

Treatment is by neoadjuvant chemotherapy via Hickman line followed by surgery (usually a radical nephrectomy with lymph node sampling). In the UK all children with Wilms’ tumour are managed by strict protocol by a multidisciplinary team. When surgery was the only treatment, the cure rate was about 10%, but the modern combination of neoadjuvant chemotherapy, surgical resection and sometimes radiotherapy gives a good chance of complete cure even when distant metastases are present.

Neuroblastoma

This embryonal tumour occurs in early childhood. It is highly malignant and arises from embryonal sympathetic nervous tissue in the adrenal gland or sympathetic chain. Standard treatment is a combination of surgical resection, chemotherapy and radiotherapy, but the prognosis is poor. A less aggressive variant is the neuroganglioblastoma. This sometimes presents as an abdominal mass but the usual presentation is failure to thrive.