Introduction
Genitourinary problems in childhood are common and present variably across the spectrum of primary and secondary care services. In particular, urinary tract infection (UTI) and wetting are common in both boys and girls, more so in girls.
Congenital abnormalities that predispose to infection and incontinence are described in more detail in Chapter 9B and elsewhere in this book. Anomalies such as spinal dysraphism, duplication of the upper urinary tract with associated ureteric ectopia or reflux, and urethral and urogenital sinus abnormalities can be subtle and easy to miss without formal anatomical assessment. Most congenital abnormalities, however, present in utero or early in childhood.
Urinary Tract Infection
Up to 10 percent of girls (and 3 percent of boys) will have had a UTI during childhood [1]. The incidence of infection falls with increasing age during childhood. Interestingly, UTIs in infant girls are less common than in boys, reflecting the marked increased association in this age group with congenital uropathies, vesicoureteric reflux (VUR) being the most common. Beyond infancy, UTI becomes much more common in girls. About one in four infants treated for UTI has a recurrent infection. In older girls the risk of recurrence becomes much higher: about 60 percent will experience a recurrent UTI.
Escherichia coli is by far the most common causative organism. Other organisms, such as Proteus, Enterococcus, Klebsiella, and Pseudomonas occur. Fungi and viruses rarely cause problems in immune-competent children. Predisposing “host” factors include congenital upper tract problems such as vesicoureteric reflux or renal and ureteric dilatation, incomplete bladder emptying, and dysfunctional voiding. A careful history will include details of antenatal concerns, postnatal voiding history (including toilet training, frequency of voiding, character of voiding such as interrupted stream and abdominal straining, for example). Detailed history of bowel function follows with examination for palpable masses including fecal loading. Careful examination of the perineum and examination of the spine and lower limbs for signs of occult spinal dysraphism, such as a hairy, fatty, or hemangiomatous patch on the back or sacral spine, complete assessment.
The symptoms of UTI are often rather vague. In infants and preverbal children, vomiting is the most common presenting feature. Fever is often present and in the context of an unwell baby, urine culture is mandatory. More classical features such as frequency, urgency, meatal or abdominal pain, and wetting tend to be more common in older children. There is very often a history of infrequent passage of hard stool, irregular bowel habit, or soiling from overflow incontinence. These complaints may have been masquerading under the umbrella term of “constipation” as part of a history for UTI. A discharge, which may or may not be vaginal in origin, and perineal soreness may be other common associated symptoms.
A urine specimen needs to be collected carefully. Most children who can void “on demand” are able to give a reliable midstream urine sample. In babies, urine is best collected by a clean catch – although this is sometimes frustrating. Collection bags are commonly used but especially in baby girls are prone to result in contaminated specimens. Samples need to be delivered fresh (although refrigeration overnight in a sterile container is probably acceptable). In unwell children, catheter specimens or suprapubic aspirates in babies are the best and most reliable means of collection.
The gold standard of diagnosis of UTI is on the basis of formal culture of a pure growth of an organism with a colony count of >105 organisms/ml associated with white cells. Sensitivities are usually generated with organism culture.
The treatment of a UTI depends ultimately on the sensitivity. If possible it is good practice to base antibiotic prescription on laboratory culture. There is a significant (and highly geographically variable) pattern of resistance of common organisms to antibiotics such as trimethoprim, penicillins, or cephalosporins. However an empirical treatment with a broad spectrum antibiotic is sometimes needed.
Subsequent investigation and management depend on the clinical context of the UTI. Most uncomplicated E. coli UTIs in otherwise well children will not be associated with an underlying problem and can be safely treated as a “one-off.” Recurrent UTI, unusual organisms, or infections in infancy demand a more considered approach; these are the children requiring imaging and assessment of their kidneys in more detail.
Guidelines are helpful for precise investigation and treatment. In the UK, the National Institute for Health and Care Excellence (NICE) has established evidence-based guidelines [2].
Some children go on to develop recurrent infection despite normal investigation of their urinary tracts. This group includes more girls than boys, and anatomical abnormalities are very rare. Assessment of stored and voided volumes (to assess residual urine volume) and uroflowmetry can sometimes identify dysfunctional voiding (see later). These children frequently have associated bowel disturbance that needs to be treated aggressively. Prophylactic antibiotics may be considered empirically but vigilance for resistance is important.
Incontinence
Uncontrolled leakage of urine per urethra may happen continuously or be intermittent. It is important to remember the clinical context, as well as the age of the child. Normal infants void in a manner akin to reflex – many times per day, and so for a baby or toddler in diapers this definition of incontinence is unhelpful. One symptom that can raise suspicion is a watery vaginal discharge in a child who has progressed to wearing underwear. Collection of urine on voiding into the vagina can occur occasionally even in girls with normal genital anatomy. This can also indicate a urogenital sinus abnormality and can sometimes indicate an ectopic ureter. The distinction between daytime symptoms and symptoms purely at night is also important.
Daytime wetting is very common [3]. Three percent of 7-year-old girls (2 percent of boys) have at least once-a-week symptoms of wetting. Of these between a third and a half will have nighttime symptoms too. Bedwetting without daytime symptoms (primary monosymptomatic nocturnal enuresis – or simply “enuresis” now, according to the International Children’s Continence Society [4]) is also very common. Up to one in ten of 7-year-old children wet three or more nights per week.
Symptoms of wetting occur in such heterogeneous conditions that it is unhelpful to consider the broad group together. Classification of symptoms may be helpful in suggesting the presence of overactive bladder – namely that urgency and frequency raise suspicion. The issue of “stress” symptoms is less in children. Symptoms precipitated by coughing, sneezing, high-impact exercise, or laughing are still far more likely to be as a consequence of overactive bladder than of true stress incontinence. As indicated later, stress incontinence might be due to neuropathic muscle weakness in association with a spinal cord problem. Primary myopathies are exceedingly rare. One condition perhaps worthy of mention is the congenital short urethra. This can only be assessed reliably at cytsourethroscopy. It is probably a variant (“forme fruste”) of epispadias.
The importance of clinical examination for UTI is mirrored for the group with wetting. Detailed abdominal examination might reveal masses or palpable kidneys or bladder, or palpable stool in the colon or rectum. Again it is important to examine the spine for features of an occult dysraphism. Small dimples and pits in the coccygeal region are common, and a dilemma may often arise as to whether an MRI (which may require general anesthesia in small children) is needed to exclude a tethered cord or spinal dysraphism. In the context of incontinence, it is difficult to dismiss any abnormality that is known to associate with an underlying spinal problem. The presence of flat buttocks, asymmetrical skin creases, or a palpable abnormality of the sacrum (such as its absence) may indicate sacral dysgenesis or agenesis. Abnormal lower limb power, reflexes, sensation, or abnormal perineal sensation needs further investigation with a view to spinal imaging.
Examination of the perineum is crucial. Labial fusion is easy to spot. A single anterior perineal orifice distinct from the anus might point toward the presence of a urogenital sinus abnormality. An abnormally sited anus might suggest an anorectal malformation, such as a variant of a cloacal abnormality. In female epispadias (which is exceptionally rare with an incidence of 1:40000), two halves of the clitoral corporal bodies are widely spaced with a bladder neck opening in the perineum. These abnormalities, whilst rare, may however have gone previously undiagnosed if the child had never been examined. Cloacal abnormalities and conditions on the exstrophy/epispadias spectrum are considered in Chapter 9B.
Investigation of incontinence revolves around defining anatomy by means of physical examination followed by imaging, and defining the ability of the bladder to store and empty by recording volumes, flows, and pressures.
Ultrasound is a reliable, relatively noninvasive and inexpensive means of assessing the kidneys and bladder. It has more utility in children because of its ease of use. MRI and CT are valuable in certain circumstances, but ultrasound is the mainstay of pediatric urinary tract imaging.
Duplication anomalies of the kidney are quite reliably detected on ultrasound (Figure 9A.1a), as is pelvicalyceal dilatation. A duplex kidney, where there is complete duplication (two collecting systems, two ureters along their entire length) consistently has its upper pole drain more inferiorly and medially than its lower pole. This may be insignificant if both ureters drain into the bladder. However, if the upper pole moiety drains below the bladder outlet (Figure 9A.1b), it will cause incontinence. In a girl this will most commonly be drainage paraurethrally or into the vagina. The pattern of wetting is quite characteristic – namely, “continent incontinence” where there is a virtually constant dribble or drip of urine despite apparently normal voids. This is easily explained. The bladder fills and empties normally, but the ectopic ureter usually only subtends a kidney moiety that is poorly functioning and rather dysplastic. Renography might be needed to demonstrate renal function to guide management, but definitive treatment for an ectopic ureter with a dysplastic upper moiety of a duplex kidney is disconnection of the ureter (either by partial nephrectomy or more recently ureteric ligation [5]).
