Chapter 6

Special Circumstances

Andrew Robb¹ / Holly Weaver and Nikesh Thiruchelvam^{2, 3, 4, 5, 6} / Suzanne Biers³

PAEDIATRIC UROLOGICAL EMERGENCIES¹

Urological emergencies in childhood are common. In this chapter we discuss a number of the more common emergencies encountered.

Acute scrotum in childhood

Defined as an acute onset of painful swelling of the scrotum or its contents. It is the commonest urological emergency encountered in children. The causes of an acute scrotum are listed in Box 6.1. Irreversible damage to the testicle can be observed after 4 hours of ischaemia; therefore, testicular torsion and incarcerated inguinal hernia must be rapidly identified and treated.

Box 6.1 Differential diagnosis of the acute scrotum in children.

Testicular torsion

Although testicular torsion has a bimodal incidence it may present at any time in childhood. The peak incidence occurs during:

•The perinatal period (neonatal torsion).

•Early puberty.

Testicular torsion often presents in a manner similar to that in adults (see page 69). In children it can also have an atypical presentation, with the child complaining of abdominal or loin pain. Abdominal examination in all paediatric patients should include examination of the external genitalia for this reason.

The use of ultrasonography is controversial. It may be helpful in establishing or excluding a diagnosis in difficult cases. However, it may cause undue delay in getting a patient to theatre for treatment of the torsion.

Treatment is emergency scrotal exploration (see also page 71). A midline (median raphe) incision or transverse scrotal incision may be employed. The tunica vaginalis (sac of tissue around the testis) is opened to expose the affected testicle. If a testicular torsion is identified, it should be untwisted and left for several minutes in a warm saline-soaked gauze and reassessed for viability. For proven cases of torsion, both testes should be fixed (bilateral orchidopexy) to prevent further episodes of torsion (and ischaemia) on either the affected side or the contralateral ‘normal’ side. The contralateral testis may be particularly susceptible to torsion due to cord insertion in the interpolar region of the testis leading to a ‘bell-clapper’ configuration. If the testis is no longer viable due to prolonged torsion, removal of the testis is required (orchidectomy), along with fixation of the other testis. Manual attempts to try to untwist the testis directly through the scrotal skin (scrotal detorsion) has no role in the management of testicular torsion.

Neonatal testicular torsion

In this clinical scenario, the torsion event happens before or around the time of birth. The presentation will depend on the time before birth that the torsion occurred:

•Prolonged period before birth: the infant may be born with an absent testis, and should be managed as a patient with an impalpable testis.

•Weeks before birth: the infant may present with a regular, firm, painless mass that is smaller than the normal contralateral testis. The mass is attached to the scrotal wall and there is no evidence of acute inflammation.

•Days or hours before birth: a painful enlarged testis with overlying inflammatory changes in the scrotal wall is seen.

•If the torsion occurs after birth, the scrotum will appear normal on initial inspection, but acute inflammatory signs will develop later.

Management is controversial. Some surgeons explore all neonates with evidence of torsion; others manage all patients conservatively except for neonates who present with acute symptoms after birth. The option of a contralateral orchidopexy should be discussed with the parents.

Torted testicular appendage

This is the commonest reason for scrotal exploration in childhood, particularly in pre-pubertal boys. The testicular appendage (also known as the hydatid of Morgagni) is a small tissue remnant found at the superior pole of the testis in most males. It is often pedunculated and therefore prone to torsion. If it twists it causes oedema and venous congestion, leading to ischaemia and infarction of the appendage, which manifests as pain.

On clinical examination, a blue spot (Figure 4.2, page 72) may be visible through the scrotum (in around a third of cases), and the clinician may be able to elicit point tenderness over the upper pole of the testis.

It is a self-limiting condition with resolution of symptoms typically after 3–5 days. It can therefore be managed conservatively with rest and analgesia. Alternatively, scrotal exploration can be performed with immediate resolution of symptoms.

After taking a history and examination, if there is any concern or residual doubt that the patient may have a testicular torsion, the patient should have an emergency scrotal exploration.

Idiopathic scrotal oedema is a condition seen in pre-pubertal males. The peak incidence occurs between 5 and 6 years of age. As the name suggests, the cause is unknown. It is characterised by swelling of the scrotum and redness (Figure 6.1). This may occur unilaterally or bilaterally, and it may extend upward into the inguinal region or to the perineum (often seen as a red inverted triangle in the perineum). Importantly the testes themselves are not tender on examination.

The condition resolves spontaneously, often within 24–48 hours. No specific treatment is required. Antihistamines or antibiotics are sometimes prescribed, but there is no good evidence to support their use.

Henoch–Schönlein purpura (HSP)

This is an acute immunoglobulin-A (IgA)-mediated vasculitis that presents with a typical rash on the legs. Involvement of the scrotum and testis in HSP is a well-documented complication. It gives rise to acute tenderness, swelling and scrotal discolouration. Scrotal exploration is not required.

Penile and foreskin problems in children

Post-circumcision complications

Complications following circumcision are common, and include:

•Bleeding. Initially, circumferential pressure around the penis should be applied. If this fails then exploration and revision of circumcision under general anaesthesia (GA) is needed. The patient should also be investigated for a bleeding diathesis.

Paraphimosis is caused by the foreskin being retracted and cannot then return to its normal position (Figure 6.2). It can result in injury or necrosis of the foreskin (and the glans). It may present with pain or acute urinary obstruction. The diagnosis should be obvious on examination.

The foreskin should be reduced as soon as possible. Manual reduction is achieved by reducing the oedema from the foreskin. It is then replaced by using the thumbs to push down on the glans while the other fingers pull the foreskin forwards into its anatomical position. Adequate analgesia should be given prior to reduction.

It may be necessary to perform this under GA in children. If manual reduction under GA fails, then a dorsal slit can be employed. Although it is an option in an adult patient, circumcision should not be performed in the acute setting if possible due to the high complication rate.

Voiding problems in children

Urinary retention

Urinary retention in childhood is uncommon. There are a large number of causes (Box 6.2). Urinary retention should be looked at as a symptom that requires identification of the underlying cause. Dependent on the aetiology, urinary retention may be painful or painless.

Box 6.2 Causes of urinary retention in childhood.

Assessment includes a full history, full physical examination including neurological examination, assessment of the spine and sacrum, and examination of external genitalia. A rectal examination should also be considered where clinically appropriate, preferably after paediatric urology review, and deferred where possible to be performed under GA if the child is also having a cystoscopy or other intervention.

Balanitis xerotica obliterans (BXO) is a benign, scarring condition that can affect the foreskin, glans of the penis and urethra (Figure 6.3). Most commonly it causes a pathological phimosis (tightening) of the foreskin, but it can also cause narrowing of the external urethral meatus and urethral strictures. If BXO is identified as the cause of urinary retention, then an emergency circumcision is required (with further treatment of any associated meatal stenosis or urethral stricture disease if identified).

In all cases of urinary retention the case should be discussed with a specialist paediatric urologist.

Key Points

UROLOGICAL EMERGENCIES IN PREGNANCY²

Although pregnant women may seek medical attention for a range of pregnancy-related problems, they can still be affected by the full range of urological diseases that may affect them in their non-pregnant state. Several important factors must be taken into consideration when approaching urological disease in the pregnant woman. Firstly, awareness of the stage of pregnancy and ongoing dialogue with the obstetric team are crucial. This is particularly pertinent when deciding management options, to ensure medical treatments and surgical interventions are safe in pregnancy. This also means the range of investigations and management options are limited compared to the non-pregnant patient, as the well-being of the fetus is paramount. Secondly, interpretation of investigation results must be done with the knowledge of the physiological and anatomical changes affecting the urinary system in pregnancy, to best inform management decisions (Box 6.3). Of particular note, there is progressive dilatation of the renal collecting system known as a physiological hydronephrosis. This is classically more pronounced on the right side than the left (Figure 6.4), which can make the diagnosis of new pathological hydronephrosis difficult to identify.

Pyelonephritis in pregnancy

Pyelonephritis occurs in up to 1 in every 50 pregnancies with recognised obstetric complications including pre-term labour, pre-eclampsia and low birth weight. The prevalence of asymptomatic bacteriuria is comparable with the non-pregnant population, but more pregnant women go on to develop symptoms and upper UTI (pyelonephitis), due to a dilated collecting system and urinary stasis promoting bacterial growth. Women should be screened for asymptomatic bacteriuria frequently in pregnancy, and treated where positive. They should also be treated quickly and effectively if pyelonephritis is suspected. The physiological changes of pregnancy can mask signs of sepsis, and as pregnant women can deteriorate very rapidly, any delay in treatment may have harmful consequences.

Box 6.3 Changes to the urinary system in pregnancy.

History

•Stage of pregnancy with an up-to-date obstetric history.

•Onset and nature of symptoms: loin tenderness, dysuria, fever.

•Screen for obstetric complications: discharge or bleeding per vagina.

Examination

Depending on the stage of pregnancy, the gravid uterus may make examination more difficult, but a thorough examination is still important, particularly to elicit any flank tenderness.

Ensure appropriate obstetric support for the antenatal mother and fetal checks.

Investigation

•Bloods: as for non-pregnant cases of suspected pyelonephritis, taking into account the physiological change in blood result parameters (creatinine and urea lower due to a higher GFR).

•Urine dip and culture.

•Blood cultures.

•Imaging: an ultrasound scan of the kidneys, ureters, bladder (USS KUB) can be performed, but interpretation needs to account for the physiological hydronephrosis of pregnancy. Computed tomography (CT) is avoided due to the radiation exposure risk to the fetus.

Management in moderate to severe pyelonephritis

•If systemic inflammatory response syndrome (SIRS) criteria are met (see page 12), the patient will need the ‘Sepsis Six Bundle’ as they would in the non-pregnant state (see page 14).

•Ongoing assessment and management of both the mother and the fetal parameters are needed throughout with obstetric input.

•Once the infection is controlled (i.e. temperature and other observations have settled with IV antibiotics), the patient should complete a prolonged course of oral antibiotics (typically to complete a total of 10 days therapy), guided by the urine cultures.

•For the duration of the pregnancy, a repeat urine dipstick and/or cultures should be regularly checked at antenatal visits.

Acute kidney injury in pregnancy

Causes of acute kidney injury (AKI) in pregnancy can be classified as pre-renal, intrinsic or post-renal in origin, as for the non-pregnant population (Box 6.4). However, assessment and management must take into consideration causes specific to pregnancy, such as the glomerular injury of pre-eclampsia, or the hypovolaemic state induced by obstetric haemorrhage. It is also important to remember the adjusted reference ranges for laboratory values in the pregnant population, whereby what is a slightly elevated creatinine or urea for the non-pregnant state, may actually indicate a significant deterioration in renal function in pregnancy.

History

•An up-to-date obstetric history, including any history of pre-eclampsia or hypertensive disorders in previous pregnancies.

•Any new symptoms, vomiting or hyperemesis gravidarum or other causes of volume depletion.

•Any known chronic renal failure. Renal function tends to deteriorate in pregnancy if there is pre-existing renal damage.

Box 6.4 Causes of AKI specific to pregnancy.

*HELLP (H, haemolysis; EL, elevated liver enzymes; LP, low platelets) syndrome is a life-threatening emergency encountered in the later stages of pregnancy, and considered a variant of pre-eclampsia. Common presentations include headache, nausea and vomiting, abdominal tenderness, and it is commonly associated with hypertension and proteinuria.

Examination

•Assess for evidence of bleeding (obstetric or otherwise) as part of the initial fluid balance assessment that could cause hypovolaemia.

•Any signs of sepsis causing intravascular fluid depletion.

•Consider pre-eclampsia as a cause of renal dysfunction: should be suspected in any hypertensive state in pregnancy and diagnosis usually involves evidence of proteinuria; may also see pitting, non-dependent oedema.

Investigation

•Bloods: note the importance of adjusted normal parameters in pregnancy; the reference range for creatinine and urea is lower than in the non-pregnant state, especially in the first trimester, so be careful not to overlook clinically significant rises.

•Imaging: USS KUB can be performed, but interpretation needs to account for the physiological hydronephrosis of pregnancy.

•Urine dipstick (look for proteinuria as well as evidence of infection).

Management

With awareness of the changes in renal physiology in pregnancy and appropriate treatment of the causes of AKI (see pages 17–21), renal function usually returns to normal.

Urolithiasis in pregnancy

The formation of calculi in the urinary tract (or urolithiasis) is a common cause for hospital admissions in pregnancy, with most presenting in the second or third trimesters. Overall, the incidence of renal colic is reported to be the same in pregnant and non-pregnant women; however, there are several factors that can contribute to stone formation in pregnancy including hypercalciuria and an increased excretion of urinary citrate (which is normally protective against stone formation). Most patients will present with flank pain. Other possible differential diagnoses also need to be checked and excluded, including pyelonephritis, appendicitis and placental complications. Stones are associated with an increased risk of pre-term labour.

History

•Stage of pregnancy with an up-to-date obstetric history.

•Onset and nature of symptoms.

•Fevers or rigors.

•Evidence of UTI/infection.

•Surgical history: remember other surgical emergencies like appendicitis can present atypically in pregnancy.

•Personal or family history of ureteric calculi.

Examination

•Abdominal exam: renal angle tenderness; check whether abdominal distension is consistent with stage of pregnancy, with obstetric input to assess mother and fetal well-being.

•Close monitoring of observations for haemodynamic instability and temperature.

Investigation

Treatments that are contraindicated in pregnancy are extracorporeal shock wave lithotripsy (ESWL) and percutaneous nephrolithotomy (PCNL).

NEUROGENIC BLADDER³

This describes dysfunction of the urinary bladder due to disease of the central or peripheral nervous system involved in the control of micturition.

Micturition depends on the integration of neuronal pathways between the cerebral cortex, brainstem and sacral spinal cord. Therefore, causes of a neurogenic bladder encompass both traumatic and atraumatic insults at varying levels, including the cerebral cortex and spinal cord. Those that affect the spinal cord above the sacral level produce an upper motor neuron pattern of injury. Insults at the level of the sacrum and lower result in a lower motor neuron deficit.

When evaluating the effect of a spinal cord injury (SCI) on bladder function, consider the level affected, as this alters the clinical presentation and will guide bladder management (Table 6.1). This is particularly important in supra-sacral spinal cord level injuries where urgent intervention may be required for a high-pressure urinary tract in order to protect renal function. The primary concern for long-term management of the neurogenic bladder is to preserve kidney function, and secondarily, to treat symptomatic lower urinary tract symptoms.

Note that patients with complete or partial SCI at the same level can present differently. Also, patients can have defects at multiple levels (such as in multiple sclerosis), and clinical presentation may be variable.

Table 6.1 Neurological problems and their effect on the urinary tract.

DSD: detrusor sphincter dyssynergia; MS: multiple sclerosis; SCI: spinal cord injury.

Urological emergencies that may present to the emergency department in patients with underlying neurological causes include the following as outlined below.

Autonomic dysreflexia

This is a significant acute medical emergency characterised by severe uncontrolled peripheral hypertension, which can occur in patients with SCI at or above the level of the 6th thoracic vertebrae (T6). A significant sensory stimulus entering the spinal cord via intact peripheral nerves induces a large sympathetic output from thoracolumbar sympathetic nerves, resulting in widespread vasoconstriction (particularly splanchnic vascular bed). Due to the level of injury, descending inhibitory impulses are unable to exert an effect at sympathetic outflow sites, and compensatory increased parasympathetic vagal cardiac input (bradycardia) is inadequate, thereby uncontrolled peripheral arterial hypertension ensues.

The stimulus is usually bladder or bowel in origin. Urological triggers include bladder distension (Figure 6.5), urinary tract calculus, urological intervention (such as catheterisation or cystoscopy) and UTI.

Complications of stroke, seizure, cardiac complications and death can occur if autonomic dysreflexia is left unresolved.

Presentation

A high index of suspicion is required and you should look out for the following features:

•Symptoms:

›Profuse sweating around the face and neck.

›Nasal congestion.

›Blurred vision.

›Headache.

›Anxiety.

•Signs:

›Rise in systolic and diastolic blood pressure (BP) above baseline (note BP baseline is often low in spinal cord injury).

›Bradycardia.

›Pupil constriction.

›Skin changes above the level of the lesion = parasympathetic responses: flushing and sweating.

›Skin changes below the level of the lesion = sympathetic responses: pale, cool skin with piloerection.

Management

•Sit the patient upright (and loosen tight clothing or belts).

•Examine for a distended bladder and treat.

•If a catheter is present, ensure it is draining. If it is blocked, change the catheter.

•Perform a rectal exam to exclude faecal impaction as a cause.

•Ensure BP is monitored; a short-acting antihypertensive may be indicated for BP control, but this will not resolve a hypertensive crisis if the underlying stimulus is not removed.

Cauda equina

This is caused by compression on the spinal roots. It can be due to benign causes such as lumbar disc rupture, spinal infection, spinal fracture, spinal haematoma, spinal canal stenosis, blunt and penetrating spinal trauma or arteriovenous malformation. Malignant causes include spinal cord tumours or metastases – this is covered in full on pages 125–126. Patients present acutely with lower back pain, lower limb weakness and numbness, and sudden-onset bladder, bowel and sexual dysfunction. An emergency magnetic resonance imaging (MRI) of the spine is required. If the diagnosis is confirmed, steroids and decompressive surgery to relieve pressure on the nerves is required urgently, under the care of spinal or neurological surgeons. Antibiotics are given for any infective processes.

•Inspection – the patient may have scars from previous neurological surgery relating to their neurological problems or complications associated with their condition which they may not have told you about (ventriculoperitoneal shunt, Mitrofanoff, colostomy).

•Chronic retention – may require a catheter (often suprapubic) if urodynamic studies reveal a high-pressure bladder. CISC is preferable to an indwelling catheter if the patient has appropriate manual dexterity and cognitive function, as there is a lower risk of UTI.

Address any chronic retention, and treat any reversible causes for UTIs. Prophylactic low-dose antibiotics are sometimes considered (see page 63).

Key Points

Related posts:

Interventional Uroradiology Procedures Genitoscrotal Emergencies Trauma in Urology Lower Urinary Tract Emergencies Upper Urinary Tract Emergencies Procedures in Urology

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