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CHAPTER24
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Inflammatory Bowel Disease
CASE 1
A 6-year-old girl presents with a 1-month history of weight loss
and mild diarrhoea, containing blood and mucus.
Q 1.1
What is the likely diagnosis and how is it
conrmed?
Three categories of inflammatory bowel disease are
encountered in childhood:
1 Crohn disease
2 Ulcerative colitis
3 Inflammatory bowel disease of indeterminate pathology
Incidence
Crohn disease is by far the most common category of
inflammatory bowel disease. During the past 25 years,
there has been a dramatic increase in the incidence of
Crohn disease worldwide. This disease was almost
unknown in childhood prior to 1980. Ulcerative colitis
has also increased in incidence in the last 2–3 decades.
Crohn disease
Crohn disease is a chronic inflammatory disorder of
unknown aetiology that can affect any part of the gastrointestinal tract from mouth to anus. It is a transmural
inflammatory process, which most commonly occurs in
the terminal small intestine and colon. There is a high
incidence of involvement of the large bowel and rectum
in children and adolescents.
CASE 2
A 12-year-old boy presents with vague pains in the abdomen,
some weight loss and a perianal abscess.
Q 2.1
How is the diagnosis made?
How are the different forms of inammatory bowel disease
Q 2.2
distinguished?
Clinical features
Age of onset of symptoms
The mean age of onset of Crohn disease in Victoria is
currently 11 years.
Symptoms and signs
Crohn disease presents with a broad spectrum of symptoms and signs. The most common symptoms include
recurrent abdominal pain and bowel disturbance, usually diarrhoea together with rectal bleeding. However,
these symptoms may be relatively mild, and patients
may present with long-term effects of the disease such as
weight loss, growth failure and delayed onset of puberty.
Delay in diagnosis may occur because not all doctors
are aware of the relatively high incidence of Crohn
disease in childhood and the variety of non-specific
presenting symptoms.
Perineal inflammation
One of the most common modes of presentation is
perineal inflammation, occurring in one-third of paediatric patients with Crohn disease, and this is usually
associated with rectal disease. Accordingly, children or
adolescents who present with a perianal abscess and
associated anal fistula should have the abscess wall
biopsied at the time of drainage, to exclude underlying
Jones’ Clinical Paediatric Surgery, Seventh Edition. Edited by John M. Hutson, Michael O’Brien, Spencer W. Beasley,
Warwick J. Teague and Sebastian K. King.
© 2015 John Wiley & Sons, Ltd. Published 2015 by John Wiley & Sons, Ltd.
147
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Crohn disease. In addition, they need to be referred for
full upper and lower gastrointestinal endoscopy, with
measurements of faecal inflammatory indices and possible
small bowel imaging studies.
Extra-intestinal manifestations of Crohn disease
Examples of extra-intestinal manifestations include
arthritis and erythema nodosum, which may be presenting symptoms.
Unusual modes of presentation of Crohn disease
Occasionally, Crohn disease may present with acute
right-sided abdominal pain and gastrointestinal disturbance, mimicking acute appendicitis. The diagnosis is
then made at laparoscopy/laparotomy.
Figure 24.1 MRI is used to assess the small bowel affected
byCrohn disease that is not accessible to endoscopy.
Cheilosis
Uncommonly, patients may present with chronic
inflammation of the mouth and lips, so-called cheilosis, manifested by oedema, erythema and fissuring of
the lips, as part of the syndrome of orofacial granulomatosis. Biopsy reveals evidence of chronic inflammation
including granulomas consistent with Crohn disease.
Investigations
Role of endoscopy
Endoscopy has a crucial role in diagnosis, initial evaluation and continuing assessment of Crohn disease.
Upper and lower (top and tail) gastrointestinal endoscopy with biopsies is the key investigation for the diagnosis and initial assessment of the extent and severity
of the disease. Colonoscopy through to and including
the ileum, together with biopsy, provides a good chance
of diagnosing Crohn disease because there is a high
incidence of macroscopic colonic involvement in children under 6 years of age. Gastroscopy is performed as
well as colonoscopy because involvement of the upper
intestinal tract is common. Periodic endoscopy is important to assess the response to treatment and disease
distribution.
In Crohn disease, the inflammation is typically segmental, and the characteristic appearance is single or
multiple ulcers with normal intervening mucosa. In
some instances, the diagnosis may be made by serial
biopsies even when the macroscopic appearances are
normal. Histological diagnosis depends on finding granulomas, in association with other chronic inflammatory
changes in the bowel wall.
MRI
This imaging is used to assess Crohn disease of the small
bowel after full bowel preparation as for colonoscopy
and also as a filling agent, which will distend bowel
loops [Fig. 24.1]. Examination of the pelvis and the
anorectum is carried out without the need for bowel
preparation. It also demonstrates sphincter anatomy
and distortion or damage related to the inflammation.
CT scan with oral contrast
Abnormal findings include an irregular bowel contour,
longitudinal ulcers and fissures, luminal narrowing by
oedema and separation of loops by mural thickening.
There may be evidence of stricture formation associated
with dilated proximal bowel. Bowel loops may be displaced by an inflammatory mass.
Other investigations
FBE detects evidence of anaemia, and liver function
tests exclude associated liver disease. Stool cultures are
performed to rule out chronic infection due to such
enteric pathogens such as Clostridium difficile, Salmonella,
Shigella, Campylobacter and Yersinia. Faecal calprotectin is
a very useful measure to determine the likelihood of
significant gastrointestinal inflammation.
Treatment
The aetiology of Crohn disease is unknown, although it
is recognised to represent an abnormal immune
response directed against the gut. Treatment is directed
at suppressing the immune response.
Medical treatment
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High doses of oral steroids are used to induce remission,
for example, prednisolone 2 mg/kg (maximum 60–75
mg/day) for 4 weeks, with gradual reduction to nil after
8 weeks. Sulphasalazine, to prevent relapses, is introduced and is built up to a dose of 50 mg/kg/day. In
moderate to severe disease and if there is steroid dependency, agents such as azathioprine, are introduced early.
Metronidazole may be helpful for perianal Crohn
disease. Biological therapies such as infliximab appear to
be very effective.
Nutrition
Children with inflammatory bowel disease fail to grow
because of the disease and its effect on appetite and
caloric intake. High caloric dietary supplements have
their place in management. Enteral feeds can be used for
either nutritional supplementation or direct treatment,
as exclusive enteral feeding has a direct anti-inflammatory
effect almost equivalent in potency to steroids. Rarely,
parenteral nutrition may be required.
Surgical treatment
The indications for surgical treatment of Crohn disease
are as follows:
1 Perianal disease
2 Intestinal complications
3 Acute abdomen – possible acute appendicitis
1. Perianal disease
Perianal disease is the most common indication for surgical intervention in Crohn disease [Fig.24.2], which is
invariably associated with rectal and colonic involvement.
There may be extensive inflammation of the soft tissues of
the perineum, scrotum, penis or vulva.
Skin tags and anal fissures are common and usually
do not require surgery. Perianal abscess is also
common and requires incision and drainage. There is
usually an associated anal fistula Occasionally, insertion of a seton suture along a chronic fistula tract may
be appropriate to drain an infection associated with
it and promote healing. More extensive suppuration
may produce an ischio-rectal abscess. Appropriate
surgical management of this complication is drainage
of the abscess and possible faecal diversion with a
colostomy or ileostomy to helpcontrol the infection.
Faecal diversion is usually effective, but it does not
Chapter24: Inflammatory Bowel Disease 149
Figure 24.2 Perianal disease in a child with Crohn disease.
necessarily influence the underlying Crohn disease.
The potential end result of ischio-rectal sepsis is
damage to the sphincters. Once surgical drainage is
achieved, medical therapy can be instituted with
biological agents such as infliximab or adalimunab in
the long term.
Perineal disease
Occasionally, there may be extensive involvement of the
perineal soft tissues extending into the scrotum, penis or
vulva. This may be manifest by unsightly, painful oedema
and inflammation of the scrotal or vulval tissue.
Surgical treatment for complicated perineal and
perianal Crohn disease aims to control infection and
promote healing, which is often protracted despite
appropriate therapy.
2. Intestinal complications
These complications are due to transmural inflammation and include the following:
Localised stricture formation
Localised d isease unresponsive to medical treatment
Localised d isease associated with growth delay and
often delay in pubertal development
Inflammatory mass
Intestinal fistulae
Rectal stricture
Surgery for these complications aims to preserve as
much intestine as possible.
Localised strictures may be treated by simple stricturoplasty (without loss of bowel) or resection. An important
role for surgery is resection of localised disease associated
with growth delay, delayed pubertal develop ment or
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both, despite maximal medical treatment. In most of
these patients, a sustained remission can be expected,
together with catch-up growth and resumption of
normal schooling. The best results can be anticipated
with resection of localised ileo-caecal disease before or at
the onset of puberty. Resection may be necessary for
inflammatory masses and fistulae.
Rectal strictures are common in paediatric Crohn disease and are often associated with perineal inflammation. Their management includes steroid therapy, both
systemic (as previously mentioned) and local steroid
medication as administered in enema form. The surgery
includes regular dilatations of the stricture under GA,
usually in association with endoscopic evaluation. The
associated perineal inflammation tends to resolve with
control of the rectal stricture.
Ulcerative colitis
Ulcerative colitis is a chronic inflammatory disease of
the rectal and colonic mucosa, the aetiology of which is
unknown.
Clinical features
The onset is usually insidious, but an acute onset similar
to a Salmonella infection can occur. The onset of disease
is usually after 5 years of age, but can be as early as the
first year of life.
The typical features are as follows:
1 Unexplained bloody diarrhoea, with mucus, lasting
more than 2 weeks
2 Anaemia
3 Fever
4 Weight loss
All degrees of severity are encountered, and the predominating symptom varies from one patient to another.
Perianal complications occur in a small percentage of
patients and include ulcers, abscesses and fistulae and
raise the suspicion that the diagnosis was actually Crohn
disease. Rarely, perianal complications may be the presenting problem, but more usually, perianal disease is
preceded by a period of diarrhoea.
Investigations
1. Colonoscopy
Colonoscopy accurately assesses the extent of macroscopic
disease, and biopsies achieve the diagnosis. In ulcerative
colitis, inflammatory changes are seen in the rectum and
extend for varying distances proximally in the colon. The
changes range in severity from loss of the normal mucosal
sheen and vascularity with associated mucosal friability to
diffuse ulceration with blood and pus in the lumen.
Numerous biopsies at colonoscopy will confirm the histological diagnosis and indicate the severity of inflammation
at various levels. The histology can be reported, at best, as
consistent with ulcerative colitis, for there is no pathognomonic lesion. At diagnosis, the inflammation is confluent,
but after therapy has started, it may become patchy and
potentially confused with Crohn disease.
In some instances, even when macroscopic appearances at endoscopy are normal, multiple biopsies will
provide diagnostic histological changes.
2. Contrast imaging
This investigation may show a sawtooth or marked
irregularities in the mucosa, with deep ulceration.
Later, the colon becomes narrow, rigid and devoid of
visible peristalsis or haustration [Fig. 24.3]. Finally,
there may be pseudopolyps or stenosis due a fibrous
stricture.
3. Other tests
Other tests include an FBE, to demonstrate anaemia.
Bacteriology tests should include a careful search for
enteric pathogens, including Clostridium difficile, Salmonella,
Shigella, Campylobacter and Yersinia. Blood for Yersinia antibodies should be collected.
Natural history
Improvements in medical treatment, with more aggressive use of immunosuppressants, such as azathioprine,
have produced better control of the disease. Many children have remissions lasting several years, to the extent
that the diagnosis is subsequently questioned. A small
proportion continue to have recurrent lapses and may
require colectomy.
Risk of malignancy
The incidence of carcinoma is directly proportional to
the duration of disease. The risk in the first 10 years of
disease is very low, but after 10 years, the rate increases
by 10% for each decade and may be even higher if associated with primary sclerosing cholangitis.
Surveillance by regular colonoscopy to detect premalignant dysplasia is an important component of management
of paediatric patients with ulcerative colitis.
Chapter24: Inflammatory Bowel Disease 151
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It is most important to realise that absent symptoms
should not be taken as evidence of quiescent or inactive disease or of healing. Surveillance by regular
colonoscopy should continue in proven cases of
ulcerative colitis.
Surgical options
Subtotal colectomy with ileo-rectal anastomosis. This
procedure may be considered where there is minimal
rectal inflammation, particularly in an older adolescent.
This procedure may avoid the need for an ileostomy.
However, this option requires continuing endoscopy
surveillance, together with biopsies every 6 months.
Removal of the rectum will be necessary after 10 years
of disease.
Procto-colectomy. This procedure may be achieved
with a number of techniques as follows: ileoanal anastomosis with ileal reservoir and ileo-anal
anastomosis without reservoir – Soave pull-through
procedure.
Figure 24.3 Ulcerative colitis. Featureless colon with sawtooth
outline on barium enema.
Medical treatment of ulcerative colitis
The principles of medical treatment are similar to those
for Crohn disease, which have been enumerated previously. However, many of the drugs for ulcerative colitis
can be given as enemas, if there is significant inflammation in the rectosigmoid.
Surgical treatment of ulcerative colitis
Procto-colectomy is curative, but other surgical procedures have a place in the treatment of this disease.
The indications for surgical treatment are as follows:
1 Severe inflammation unresponsive to medical treatment
2 Severe disease associated with growth delay and
delayed pubertal development
3 Long-term risk of malignancy (see previous text)
4 Acute haemorrhage
5 Perforation
6 Toxic megacolon
Chronic inflammatory bowel disease
of indeterminate pathology
In a small number of patients, the pathology is uncertain, and a diagnostic dilemma arises as to whether
the patient has ulcerative colitis or Crohn disease. The
principles of medical treatment are similar to those
outlined previously, but surgical treatment depends
on what is considered to be the most likely condition
as judged on clinical, endoscopic and histological
evidence.
KEY POINTS
• Crohn disease is now relatively common in children and
adolescents.
• Recurrent abdominal pain with intermittent diarrhoea
suggests IBD.
• Perianal sepsis in adolescents suggests Crohn disease.
• IBD diagnosis requires sophisticated imaging, endoscopy and
biopsy.
• Surgical treatment is required if medical therapy fails and for
prevention of colonic cancer.
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Further reading
Adibe OO, Georgeson KE (2012) Crohn’s disease. In: Coran AG,
Adzick NS, Krummel TM, Laberge J-M, Shamberger RC,
Caldamone AA (eds) Pediatric Surgery, 7th Edn. Elsevier
Saunders, Philadelphia, pp. 1209–1216.
Alder J, Coran AG, Teitelbaum DH (2006) Ulcerative colitis. In:
Coran AG, Adzick NS, Krummel TM, Laberge J-M,
Shamberger RC, Caldamone AA (eds) Pediatric Surgery, 7th
Edn. Elsevier Saunders, Philadelphia, pp. 1217–1230.
Alexander F (2006) Inflammatory bowel disease in children. In:
Stringer MD, Oldham KT, Mouriquand PDE (eds) Pediatric
Surgery and Urology. Long-term Outcomes, 2nd Edn. Cambridge
University Press, Cambridge, pp. 351–361.
CHAPTER25
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The Child with an Abdominal Mass
CASE 1
A 9-year-old boy presents with a 2-week history of intermittent
pain in the left loin and ank. Physical examination reveals a large,
smooth, left-sided mass in the abdomen. The mass is rm, but not
solid, and is ballottable.
Q 1.1
What is the likely diagnosis?
What investigations might be needed?
Q 1.2
CASE 2
A previously well 4-year-old girl presents with a large, smooth and
solid mass in the right side of the abdomen, noted incidentally
during examination. Her blood pressure is 110/80.
The following points need to be considered when assessing a child with an abdominal mass:
1 Age of the patient and the most likely pathological
process arising in that organ at that age
2 Length of history and type of symptoms, which may
also implicate a particular pathological process (e.g.
tenderness of the mass suggests infection or bleeding)
3 Site of the mass and its precise characteristics, which
will suggest the probable organ of origin
Normal and abnormal masses
The most common abdominal masses in infancy and
childhood are non-pathological. They are usually
accounted for by the liver, which normally extends
below the right costal margin until 3–4 years of age;
faeces in the colon; or full bladder [Table25.1].
In addition, three common pathological conditions
often present with an abdominal mass in childhood:
Wilms tumour (nephroblastoma), abdominal neuro-
Q 2.1 What is the differential diagnosis?
What treatment might be needed?
Q 2.2
CASE 3
A 5-year-old girl has been unwell and pale in recent weeks. The
school nurse nds a large, hard and craggy mass in her upper
abdomen.
Q 3.1 What investigations are needed, and what is the likely
diagnosis?
What would you tell the parents?
Q 3.2
blastoma and hydronephrosis. These pathological
conditions have certain features in common:
1 They are most common in infants and toddlers bet-
ween 1 and 3 years of age.
2 The mass is typically large when first detected as the
normally protuberant infantile abdomen may conceal
masses of smaller sizes.
3 The mass itself is usually the presenting feature, while
general or local symptoms are typically minimal or
absent.
Neuroblastoma is an exception to the latter point: a
significant number of children with neuroblastoma present systemically unwell, including failure to thrive.
As stated earlier, the site of the mass assists in formulating a differential diagnosis. A mass situated in
the midline in the upper abdomen is most likely a
primary abdominal neuroblastoma, particularly if
the child is less than 4 years of age. Other possible
causes include massive hepatic metastases (e.g. from
a primary neuroblastoma) and primary hepatoblastoma. Masses arising in the loin can be palpated
Jones’ Clinical Paediatric Surgery, Seventh Edition. Edited by John M. Hutson, Michael O’Brien, Spencer W. Beasley,
Warwick J. Teague and Sebastian K. King.
© 2015 John Wiley & Sons, Ltd. Published 2015 by John Wiley & Sons, Ltd.
153
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Table 25.1 Common normal and abnormal abdominal masses
in children
Normal Abnormal
Liver Hydronephrosis
Faeces Wilms tumour
Bladder Neuroblastoma
Lower pole of kidneys
there is a renal stone. Also, blood tests can be indicated,
for example, full blood count, electrolyte analysis and
tumour markers as appropriate.
Abdominal ultrasonography will determine whether
a mass is cystic or solid. Cystic masses may be seenwith
hydronephrosis, multilocular or simple renal cysts,
multicystic dysplastic kidneys or a dilated renalpelvis
from high-grade vesicoureteric reflux. Ultrasonography
documents the size, position and extent of a solid
tumour and may demonstrate blood vessel involvement (e.g. extension of a Wilms tumour into the
inferior vena cava). Lymph node involvement and
metastases may be demonstrated, but these and other
oncologically relevant imaging findings are ordinarily
investigated using abdominal computerised tomography (CT) scan. Some renal masses warrant a nuclear
scan to quantify relative function of each kidney and,
with some isotopes, obstruction of the renal tract, for
example, MAG3 renogram to investigate hydronephrosis. Magnetic resonance imaging (MRI) and angiography to determine vascular supply have select roles. The
management of hydronephrosis is described further in
Chapter33.
Figure 25.1 Neuroblastoma, showing calcification in the right
paravertebral region.
extending below the rib margin towards the iliac
fossa and are most likely due to hydronephrosis or
Wilms tumour.
Investigations
Simple imaging can assist in diagnosis, for example, plain
abdominal x-ray and abdominal ultrasonography. Plain
x-ray may show calcification within the mass, which is
more common in neuroblastoma [Fig.25.1] than Wilms
tumour, and does not occur in hydronephrosis, unless
Neuroblastoma
Neuroblastoma is the most common extra-cranial solid
tumour of childhood. It is an embryonal tumour that
arises from fetal neural crest cells and may occur at any
site in the sympathetic nervous system. The most
common sites are the adrenal gland [Fig. 25.1], elsewhere in the abdomen, and the sympathetic chain or
the sympathetic plexus in the mediastinum or pelvis.
Metastases are present in 70% of patients at diagnosis
and may be in the bone marrow, the cortex of long
bones, the regional or distant lymph nodes, skull, eyes,
liver and skin. The numerous sites of primary tumour
and propensity to early metastasis account for the wide
variety of possible presentations. Examples include:
1 Proptosis and periorbital ecchymoses due to ocular
metastases
2 Long bone pain and tenderness due to bony metastases
3 Rubbery lymph nodes in the neck or axilla due to
lymph node metastases
4 Bone marrow involvement manifesting as any or all
of pain, limping, paralysis or weakness and failure to
thrive with or without anaemia
Chapter25: The Child with an Abdominal Mass 155
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5 Palpable skull nodule due to skull metastases
6 Paraplegia of rapid onset due to intraspinal extension
of a paravertebral primary
7 Horner syndrome due to involvement of the stellate
ganglion (sympathetic chain)
8 Blueberry muffin spots on the skin of infants due to skin
metastases
9 Diarrhoea caused by tumour metabolites, for
example, vasoactive intestinal peptide (VIP)
In view of the invasiveness and malignant potential of
neuroblastoma it is a paradox that, in a minority of cases,
the tumour regresses completely with a spontaneous
cure. This unusual tumour behaviour is restricted to
children younger than 18 months who present with
metastases strictly confined to the skin, liver and/or bone
marrow: clinical stage MS (previously called stage 4S).
The striking skin metastases in such infants are pathognomonic for this stage and are described as blueberry
muffin spots. Resection of the primary tumour in these
children has no impact upon local relapse of the tumour
nor the patient’s overall survival.
Diagnostic criteria
The diagnosis of neuroblastoma requires one or both of
the following criteria:
1 Unequivocal pathologic diagnosis from tumour tissue
or raised serum catecholamines (i.e. dopamine,
adrenaline, noradrenaline) or raised urinary catechol-
amine metabolites (VMA, HVA)
2 Unequivocal pathologic evidence for bone marrow
involvement (evident in 65–75% cases at presenta-
tion) together with either raised serum catecholamines
or raised urinary catecholamine metabolites
Biopsy may be of the suspected abdominal mass or metastases, for example, lymph nodes. This is now often performed percutaneously under radiological guidance, but
open tumour biopsy has select indications. Bone marrow
assessment requires bone marrow biopsy and trephine.
Previously, catecholamine metabolites were measured
from a 24 h urine collection sample, but this is cumbersome, and spot testing is now considered preferable.
While the diagnosis of neuroblastoma may be made
without tumour biopsy, tissue biopsy for tumour histology and biological features is essential for risk stratification. Further assessment of patients with confirmed
neuroblastoma includes an abdominal CT to look for
image-defined risk factors as a part of clinical staging
and a metaiodobenzylguanidine (MIBG) nuclear scan.
The MIBG isotope is avidly taken up by 90–95% of
neuroblastomas and so identifies both primary and
metastatic lesions. Other imaging routinely performed
to inspect for metastases include a CT chest and
bonescan.
Treatment
Treatment of patients with neuroblastoma is coordinated in a comprehensive oncology service and protocolised in accordance with the child’s risk stratification.
There is a wide range of possible treatment options,
including:
1 Observation only: this is limited to a select group of
patients, typically in the under 18-month age group
with stage MS disease.
2 Operative excision alone: also limited to a select group of
patients.
3 Operative excision with neo-adjuvant and adjuvant chemo-
therapy: this is the most common treatment pathway
and may be complemented by autologous stem cell
transplant.
4 Radiotherapy: usually reserved for high-risk disease in
addition to operative excision and chemotherapy.
5 Biological and immunological therapies are an
increasingly important component of the treatment
regimen in neuroblastoma – usually limited to high-
risk patients and patients with recurrent disease.
When undertaken, the goal of operative excision may
not be complete excision. Rather, the surgeon must
weigh the benefits of complete resection against the
risks of what can be prolonged and highly morbid surgery to achieve complete resection. Preoperative CT
image-defined risk factors for non-resectability include
tumour encasement of the aorta, vena cava or iliac
vessels. The presence of such risk factors may be an indication for neo-adjuvant (preoperative) chemotherapy
prior to restaging and consideration for tumour debulking surgery.
The prognosis is highly dependent upon the stage of
disease at presentation. High-risk disease is currently
associated with a 5-year survival rate of 50%.
Wilms tumour
Wilms tumour, or nephroblastoma of the kidney, is the
most common renal malignancy in childhood. It arises
from primitive embryonic cells and produces a mixed
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histological picture of epithelial structures resembling
tubules and a variety of mesenchymal tissues, including
striated muscle fibres.
Wilms tumour is typically sporadic in origin. However,
it may be associated with a number of syndromes,
including Beckwith–Wiedemann; Wilms tumour,
aniridia, genitourinary malformations and mental retardation (WAGR); and Denys–Drash. A genetic basis is not
un
common, with tumour suppressor gene WT-1 deleted
in WAGR and Denys–Drash and WT-2 associated with
Beckwith–Wiedemann. Wilms tumour is also seen in
association with hemihypertrophy, aniridia and familial
Wilms tumour. Bilateral disease is present in 6% of
affected children.
Clinical features
A Wilms tumour usually presents as a smooth loin mass
that seldom crosses the midline, but extends down into
the iliac fossa and up under the costal margin. A rightsided Wilms tumour may extend behind the liver,
which, if pushed down, may present as hepatomegaly.
On the left side, a Wilms tumour may be mistaken for
an enlarged spleen. Children with Wilms tumours are
typically well at presentation (80% of cases), unlike
those patients with neuroblastoma.
Haematuria, often following minor trauma, is the
presentation in some children, but does not indicate a
poorer prognosis. Clinical examination of the child
should always include a blood pressure measurement,
as this may be elevated due to unusual metabolites from
the tumour tissue or compression of the renal vessels
Investigation
Ultrasonography provides detailed information of the
site, size and extent of the tumour [Fig. 25.2a and
Table 25.2]. Doppler ultrasonography also identifies
renal vein and inferior vena cava involvement. The
liver is examined for the presence of metastases, and a
chest x-ray excludes the presence of pulmonary metastases. A contrast CT scan of the abdomen and chest is
utilised to assess the extent of disease, the involvement
of the contralateral kidney, the effect on surrounding
tissues and the extent of metastatic disease [Fig.25.2b].
There remains controversy regarding the role of a
preoperative tissue diagnosis. In some countries, particularly the United States, primary resection without a prior
tissue diagnosis is the routine. The operative and
histological findings then direct the need for subsequent
(a)
(b)
Figure 25.2 (a) Ultrasound scan showing a large Wilms
tumour(M) arising from the left kidney (LK) and (b) CT scan
showing the same tumour in cross section.
Table 25.2 Staging of Wilms tumour
Stage Tumour spread
I Confined to kidney and removed completely
II Microscopic local disease after resection
III Macroscopic residual disease after resection
IV Distant metastases
V Bilateral renal tumours
chemotherapy and radiotherapy. In other countries,
particularly in Europe, preoperative chemotherapy is initiated without a tissue diagnosis. The rationale is to downstage the disease and reduce the risk of intra-operative
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