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CHAPTER 12 Paediatric surgery
432
Malrotation and volvulus
Key facts and clinical features (neonates)
Malrotation
• This can present at birth or soon after and symptoms are due to
rotation of the small bowel, leading to duodenal obstruction.
• ‘Ladd’s bands’ are occasionally the cause of the obstruction.
• Proximal duodenal distension leads to bile-stained vomiting.
• The caecum may be in an abnormally high or midline position.
Volvulus
• Twisting, in clockwise direction, of malrotated, non-fi xed midgut loop
on its narrow-based mesentery through 360* or more.
• Results in obstruction of superior mesenteric blood vessels.
• Signs. Sudden onset of abdominal pain, bile vomiting, progression to
shock, passage of blood per rectum.
• May be less dramatic; most dangerous in newborn period because of
delay in diagnosis and rapid development of gut ischaemia.
• Older children may present insidiously or as rapid onset of shock with
less prominent other symptoms.
Diagnosis and investigations
3 If in doubt, operate. Viability of twisted bowel is very timedependent—delays in diagnosis can be very serious.
• Plain AXR. ‘Double bubble’ sign with some distal gas.
• Barium meal. Obstruction of second part duodenum, non-rotation
of duodenum/jejunum, and corkscrew appearance of proximal small
bowel loops; absent C loop of duodenum.
• Ultrasound scan. Reversed relation of superior mesenteric artery
and vein.
• Doppler ultrasound. Absent or abnormal small bowel blood.
Treatment
• Resuscitation, including decompression with NGT.
• Prompt surgery to avoid irreversible bowel damage.
• Laparotomy may reveal:
Obstructed, but viable bowel.•
Patchy ischaemic changes.•
Established necrosis.•
• Resection of ischaemic gut may risk ‘short gut syndrome’.
• ‘Second look’ laparotomy (24–48h) allows reassessment prior to
resection.
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CHAPTER 12 Paediatric surgery
434
Intussusception
Key facts (children)
• Incidence approximately 2 per 1000 live births.
• Peak age of presentation at 3–10 months.
• ♂:♀, 2:1.
• Fewer than 10% have a clear focal pathological cause that starts the
intussusception (‘apex’; older children are more likely to have an
apex).
Clinicopathological features
• Invagination/telescoping of the proximal bowel (called the
intussusceptum, e.g. terminal ileum/ileocaecal valve) into the distal
bowel (called the intussuscepiens, e.g. caecum/ascending colon).
• May be due to enlargement of lymphatic patches of Peyer (‘idiopathic’).
• Pathology at the apex may be:
Meckel’s diverticulum.•
Polyp.•
Lymphoma.•
Clinical features
• Classic triad of features is:
Abdominal pain (associated with pallor, screaming, and •
restlessness).
Palpable sausage-shaped mass (mid-abdominal or right upper •
quadrant).
Passage of ‘redcurrant jelly’ stool (rectal examination may reveal •
bloody mucus and the lead point may rarely be palpable).
• Typically, the infant is relatively settled between bouts of pain.
• Signs of shock (lethargy, poor feeding, hypotonia) require urgent fl uid
resuscitation.
• Features of obstruction (distension and vomiting) may occur.
Diagnosis and investigations
• Ultrasound (diagnostic test of choice). Intussusception in cross-section
(‘doughnut’ or ‘target’ sign).
• Plain X-ray. May show soft tissue mass, small bowel obstruction, free
air indicating perforation.
• Air (or rarely gastrograffi n) contrast enema. Diagnostic and may be
therapeutic (see below).
Treatment
• Immediate IV fl uid resuscitation to correct fl uid losses and to restore
fl uid, electrolyte, and acid/base balance.
• Maintenance fl uid replacement and replacement of continued losses
(vomiting or nasogastric losses). Reduction only attempted once fl uid
balance restored.
• Analgesia and sedation (morphine 0.2mg/kg) will aid process of
reduction.
• Antibiotics and NGT.
INTUSSUSCEPTION
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Methods of reduction
Radiological reduction
• Air enema therapeutic in 75% of cases.
• Usually performed in radiology department under screening control.
• Surgeon should be present.
• Evidence of irreducible obstruction or perforation mandates
immediate halt.
• Partial or incomplete reduction may warrant repeat attempt after
4–6h.
• Informed consent includes risk of perforation.
Surgical reduction
• Laparotomy indicated without enema if evidence of peritonitis or
perforation.
• Manual reduction by retrograde squeezing and gentle proximal
traction.
• Resection and anastomosis if bowel viability is in doubt (710% require
resection).
• Post-reduction septic shock may occur with release of bacterial
products from viable, but damaged bowel segment.
• Most recover rapidly with resumption of oral feeding in 24–48h
and discharge home in 4–5 days.
Complications
• Recurrence rate is 5–7% in non-operative cases and about 3% for
operative reduction.
• Morbidity is low, but delayed diagnosis, inadequate resuscitation, and
failure to recognize ischaemic or perforated bowel account for
1% mortality.
435
CHAPTER 12 Paediatric surgery
436
Hirschsprung’s disease
Key facts (neonates/children)
• Incidence 1 in 5000 live births.
• Commoner in males.
Pathological features
• Due to incomplete migration of neural crest cells into the hindgut,
resulting in distal aganglionosis and failure of coordinated peristaltic
waves, abnormal anorectal relaxation, and loss of recto-anal inhibitory
refl exes.
• May involve:
Just the anorectal junction (ultrashort segment), presents in adult •
life.
The rectum and recto-sigmoid (short segment. 70%), presents in •
infancy or early childhood.
Extensive colonic involvement (long segment), rare.•
• The proximal (normal) bowel becomes progressively distended due to
build-up of faecal matter.
Clinical features
• There is failure to pass meconium within 24–48h, abdominal
distension, and bile vomiting.
• It may be associated with Down’s syndrome.
• It may present late with poor weight gain, offensive diarrhoea, or
enterocolitis.
Diagnosis and investigations
• Radiograph shows dilated colon to level of ‘transition zone’ to
aganglionic bowel.
• Contrast enema shows less distensible rectum and may indicate
transition zone.
• Suction rectal biopsy confi rms diagnosis; thickened nerve fi bres and
aganglionisis (i AChE).
• Anorectal manometry (in older children) shows failure of anal relaxation
on rectal balloon distension (loss of recto-anal inhibitory refl ex).
Treatment
• Resuscitation and analgesia.
• Decompression of the colon with regular saline rectal wash-outs.
• If decompression is not achieved or there is total colonic involvement,
a defunctioning stoma is necessary.
• Defi nitive surgery is to remove the aganglionic bowel and bring
normally innervated bowel to the anus (pull-through technique—
Soave, Swenson, or Duhamel types).
It is usually performed as a one-stage procedure without a covering •
stoma.
The pull-through can be performed transanally or abdominally.•
Laparoscopy assists in establishing a level and mobilizing the •
colon/rectum.
HIRSCHSPRUNG’S DISEASE
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Complications
• Constipation.
• Enterocolitis can affect 20–50% of children pre- and post-operatively
(uncommon >5y of age unless an obstructive component exists).
Further reading
Langer JC (2004). Hirschsprung’s disease. Curr Probl Surg 41: 949–88.
Swenson O (2002). Hirschsprung’s disease: a review. Pediatrics 109(5): 914–8.
Teitelbaum DH, Coran AG (2003). Primary pull-through for Hirschsprung’s disease. Sem Neonatol
8(3): 233–41.
437
CHAPTER 12 Paediatric surgery
438
Rare causes of intestinal obstruction
Duodenal atresia
• Caused by failure of development or canalization of the duodenal
canal.
• May be complete (i.e. entirely separate proximal and distal duodenum)
or partial (e.g. an hourglass narrowing or web obstruction in the
second part of the duodenum).
Diagnostic features
• Bile-stained vomiting occurs from birth.
• Epigastric fullness on examination.
• Look for features of associated Down’s syndrome.
• Plain AXR. ‘Double bubble sign’ with no distal gas.
Management
• Resuscitation.
• Surgical bypass (duodenoduodenostomy bypass).
Jejuno-ileal atresia
• Caused by probable in utero vascular insult to mesenteric vessels.
• May occur in a single or multiple segments and may be short segments
or long stretches of small bowel involved.
Diagnostic features
• Bile-stained vomiting from birth.
• Prominent abdominal distension, especially with distal atresia.
• Features of obstruction.
Investigation and management
• Resuscitation.
• Contrast enema may be helpful to exclude other diagnoses.
• Surgical anastomosis between atretic ends.
Meconium ileus
• Caused by the presence of impacted, abnormally thick meconium
within the normal lumen of the small bowel.
• Pathognomonic of CF, but only 15% of CF present as meconium ileus.
Diagnostic features
• May be identifi ed during antenatal ultrasound examination (‘bright
spots’ in bowel) or family history with antenatal testing.
• Presents in neonatal period with features of distal obstruction:
Vomiting, distension, failure to pass meconium, mass in RIF
(meconium-obstructed bowel loops).
Investigation and management
• Resuscitation, IV fl uids, NGT.
• Plain AXR.
• Contrast enema may be diagnostic and therapeutic.
• Surgical removal of meconium (may involve a temporary ileostomy).
• Immunoreactive trypsin and commonly associated CF genes (δF508).
RARE CAUSES OF INTESTINAL OBSTRUCTION
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Anorectal malformations
Incidence, approximately 1 in 5000 live births. Caused by failure of the correct septation of the hindgut cloaca or failure of formation of the anorectal
canal (and associated pelvic fl oor structures).
• Low anomalies traverse a normal levator muscle.
• High anomalies end above the levator and are commonly associated
with a fi stula (bladder, urethra, vagina).
• Malformations may be part of a syndrome or linked to chromosomal
abnormalities (VACTERL).
Diagnostic features
Condition should present at the neonatal check with recognition of an
absent or abnormally placed anus and the failure to pass meconium with
associated abdominal distension if a diagnosis has been missed. It may take
up to 24h before meconium passes through the fi stula.
Investigations and management
• Lateral prone X-ray of pelvis at 24h (assists in level assessment and
sacrum).
• Perineal, renal ultrasound, and echocardiography (for associated
abnormalities).
• Contrast loopogram 1 week after stoma formation (position of
fi stula/renal anomalies).
• Prophylactic antibiotics if vesicoureteric refl ux is demonstrated.
Surgical treatment
• Low lesions (perineal fi stula). Single stage perineal approach (anoplasty
or dilatation).
• All other lesions. Defunctioning colostomy.
• Posterior sagittal anorectoplasty (PSARP). At 1–6 months and
colostomy closure thereafter.
Prognosis
• Low anomalies often have relatively good function with a tendency to
constipation in later life.
• High anomalies often have impaired function with up to 80% lifetime
chance of soiling/incontinence.
439
CHAPTER 12 Paediatric surgery
440
Abdominal wall defects
Exomphalos (omphalocele)
Key facts
• Incidence 1 in 7000 births.
Clinicopathological features
• Herniation of the abdominal viscera through an umbilical defect that is
covered by a membrane (unless ruptured).
• Exomphalos minor. The defect is <5cm and only the bowel is herniated.
• Exomphalos major. The defect is >5cm and bowel, liver, and other
abdominal organs lie in the hernial sac.
• May present antenatally with an abnormal scan or raised maternal
serum alpha-fetoprotein (AFP); in post-natal presentation, there is an
obvious defect.
Diagnosis and investigations
• Investigations directed at identifying the associations (see Box 12.3).
Check blood sugar.
• All newborn babies should have cardiac imaging prior to further
management.
Treatment
• Parents may opt for termination in antenatally detected defects with
associated major cardiac or chromosomal anomaly (mortality 780%).
• Post-natal management involves protection of sac, insertion of NGT,
IV access, and fl uid management.
• Minor exomphalos should be suitable for reduction and primary
closure of umbilical defect.
• Major exomphalos may be associated with underdeveloped abdominal
cavity, precluding primary reduction. Epithelialization of the sac can be
encouraged with application of silver sulphadiazine paste, resulting in a
large ventral hernia that is suitable for delayed closure at 71y of age.
Surgical treatment
• Primary reduction of smaller defects. Excision of sac, closure of
umbilical defect (linear or purse string), and closure of umbilical skin.
• If the sac is ruptured in a larger defect. Application of silo or tissue
fl ap.
Box 12.3 Associations of exomphalos
• Chromosomal abnormality (trisomy 18, 13, 21).
• Cardiac and renal anomalies found in up to 40%.
• Pulmonary hypoplasia caused by abnormal diaphragm function.
• Beckwith–Wiedemann syndrome: exomphalos, macroglossia,
gigantism hyperinsulinism in infancy, renal/hepatic tumours.
• Pentalogy of Cantrell: exomphalos, sternal cleft, ectopia cordis,
anterior diaphragmatic hernia, ventricular septal defect.
ABDOMINAL WALL DEFECTS
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Gastroschisis
Key facts
• Incidence 1 in 7000 births (increasing).
Clinicopathological features
• There is a defect to the right of the umbilicus with protrusion of the
stomach, small bowel, and large bowel.
• Associated with young maternal age and antenatal smoking or
recreational drug use.
• Most present antenatally with an abnormal scan or raised maternal
serum AFP.
• Antenatal diagnosis allows planned delivery (no evidence to
recommend Caesarean section).
• Extraintestinal associated anomalies are uncommon.
• Intestinal atresia found in 10–20%.
Diagnosis and investigations Associated anomalies are rare. No formal
investigations are required.
Treatment
Management of gastroschisis at birth
• Planned vaginal delivery as close as possible to neonatal surgical unit.
• Standard neonatal resuscitation (clean, dry, stimulate, facial O
• Cling fi lm wrap to protect herniated bowel against trauma,
, etc.).
2
contamination, heat loss, drying, and fl uid loss (ensure mesentery not
on tension).
• Insertion of NGT to decompress stomach.
• Fluid balance must include considerable evaporative losses from gut.
• Broad-spectrum antibiotics.
Non-surgical treatment Manual reduction and non-sutured closure of
defect.
Surgical treatment
• If possible, the defect is delineated and closed.
• If herniated contents are unable to be reduced, the application of
a ‘silo’ to cover gut and delayed closure once the gut is reduced
(7–10 days).
441
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