Oxford Handbook of Clinical Surgery (4 edn)
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Principles of managing paediatric surgical cases 424
Acute abdominal emergencies—overview 426
Oesophageal atresia 428
Pyloric stenosis 430
Malrotation and volvulus 432
Intussusception 434
Hirschsprung's disease 436
Rare causes of intestinal obstruction 438
Abdominal wall defects 440
Necrotizing enterocolitis (NEC) 442
Inguinal hernia and scrotal swellings 444
Other childhood hernias 446
Prepuce (foreskin) and circumcision 448
Undescended testis 450
Solid tumours of childhood 452
Neck swellings 454
Principles of managing paediatric surgical cases
Key facts
Children are not small adults.
Children come in different sizes; always obtain a weight before starting treatment.
Fluids and drug doses depend on body weight.
Babies and children have different differential diagnoses from adults.
Children often differ from adults in physiology and anatomy (see Table 12.1).
Babies and young children have difficulty communicating symptoms.
| Neonates | Adolescents | |
|---|---|---|
Blood volume (mL/kg) | 80 | 30–40 |
Oral fluid intake (mL/kg/day) | 150 | 30–40 |
Daily Na+ intake (mmol/kg) | 2–3 | 1–2 |
Daily K+ intake (mmol/kg) | 2–3 | 1 |
Systolic BP (mmHg) | 40–50 | 100–120 |
Resting pulse rate (bpm) | 120–160 | 70–80 |
| Neonates | Adolescents | |
|---|---|---|
Blood volume (mL/kg) | 80 | 30–40 |
Oral fluid intake (mL/kg/day) | 150 | 30–40 |
Daily Na+ intake (mmol/kg) | 2–3 | 1–2 |
Daily K+ intake (mmol/kg) | 2–3 | 1 |
Systolic BP (mmHg) | 40–50 | 100–120 |
Resting pulse rate (bpm) | 120–160 | 70–80 |
Special problems with babies
Thermoregulation is impaired (immature sweating, high surface area to body weight increases rate of heat loss). Prone to hypothermia.
Minimal glycogen stores. Prone to acute hypoglycaemia.
Principal breathing pattern is diaphragmatic. Prone to breathing difficulties with abdominal distension.
Immature body physiology. Much less biological functional reserve than adults; acute disturbances of physiology more serious with less room for error.
May not metabolize drugs as expected.
Intravenous fluids
Maintenance fluids
(see Box 12.1)
4mL/kg/h total fluid for each of the first 10kg of weight (0–10kg).
2mL/kg/h total fluid for each of the next 10kg of weight (11–20kg).
1mL/kg/h total fluid for each subsequent kg of weight (over 21kg)
Always calculate fluid and sodium requirements according to weight.
Remember to add glucose, especially for neonates.
Adjust the fluid regimen according to clinical setting for neonates and premature babies.
Resuscitation fluids
Crystalloids or colloids can be given as resuscitation fluids.
Mild dehydration—10mL/kg bolus; repeat as necessary.
Moderate dehydration—20mL/kg bolus; repeat as necessary.
Acute abdominal emergencies—overview
Key facts
Whatever the cause (see Fig. 12.1), these typically present with signs and symptoms of either peritonitis or intestinal obstruction.
Features of peritonitis are often difficult to elicit in babies.
Cardinal features of obstruction are:
Vomiting.
Abdominal distension.
Failure to pass meconium.
Pain.
Causes of acute abdominal emergencies in babies and infants.
Vomiting in children
Vomiting is common in newborns and is often entirely benign. May be due to:
Overfeeding.
Rapid feeding.
Air swallowing (inadequate winding).
Vomiting may be due to metabolic causes (inborn errors of metabolism, acidosis) or infections (UTI, chest infection, meningitis).
Bile-stained vomiting should never be ignored.
Abdominal distension
Most pronounced in distal obstruction and less so in proximal causes of obstruction.
Failure to pass meconium
Term babies should pass meconium within 36h.
Babies with proximal obstruction or atresia may still pass meconium.
Pain
May be difficult to assess in babies. Typical features are going off feeds, lethargy, erratic heart rate.
Diagnostic features
History
Family/genetic history for cystic fibrosis (CF). Meconium ileus.
Premature birth. Necrotizing enterocolitis.
Time of onset related to birth. The more proximal the obstruction, the earlier the presentation.
Examination
Blood in vomit or stool may indicate necrotic bowel.
Degree of distension. Most pronounced with distal obstruction.
Investigations
Plain AXR may show diagnostic features.
‘Double bubble sign’. Duodenal atresia or malrotation.
‘Ground glass’. Meconium ileus.
Multiple loops of small bowel. Distal obstruction.
Intramural gas (‘pneumatosis intestinalis’). Necrotizing enterocolitis.
Free air. Intestinal perforation.
Abdominal ultrasound. Abdominal mass. Intussusception, tumour, duplication cyst.
Oesophageal atresia
Key facts
Congenital abnormality of the formation of the upper aerodigestive tract (UADT), resulting in partial or complete interruption of the oesophageal lumen (see Fig. 12.2).
Often associated with other congenital abnormalities (VACTERL).
Classification of oesophageal atresia. Type C (III), distal fistula; type A (I), atresia without fistula; type E (V), H-type fistula.
Clinicopathological features
May be diagnosed on prenatal ultrasound. Features include maternal polyhydramnios, absent stomach bubble, associated abnormalities.
Post-natal diagnosis relies on features of persistent salivary drooling, regurgitation of all feeds, and cyanosis with feeding.
Failure to pass NGT into stomach.
Tracheo-oesophageal fistula in isolation unusual. Presents with recurrent aspiration/chest infections.
Diagnosis and treatment
Plain AXR and thorax. NGT coiled in oesophagus.
Presence of stomach gas suggests tracheo-distal oesophageal fistula.
Plain X-ray spine. Associated congenital abnormalities.
Echocardiography. Associated cardiac abnormalities.
Medical treatment
Nurse head-up.
NBM and continuous Replogle (oro-oesophageal) tube.
Antibiotics for possible aspiration pneumonia.
Surgical treatment
Isolated atresia.
Gastrostomy for feeding + continuous drainage of upper pouch.
Delayed closure of defect (may require interposition graft if a long segment involved).
Atresia with tracheo-oesophageal fistula. Ligation of fistula and primary closure of oesophageal defect.
Isolated tracheo-oesophageal fistula. Ligation of fistula (through neck).
Pyloric stenosis
Key facts (children)
Incidence 3 in 1000 live births.
Increased familial risk (family history).
It typically occurs in first born boys (♂:♀, 4:1).
Pathological features
There is hypertrophy of the pyloric smooth muscle in early infancy, which occurs at about 3–6 weeks.
Clinical features
Classical projectile, forceful vomiting (secondary gastritis may cause bloodstaining).
Persisting vomiting.
Sometimes a history of excessive positing and gastro-oesophageal reflux.
Baby appears active and hungry, especially after vomiting.
Small, green, starvation stools passed infrequently.
Weight gain is poor.
Dehydration with hypochloraemic alkalosis gradually supervenes in untreated, established condition.
Baby usually looks well in early state.
Dehydration, pallor, underweight only in advanced condition.
Epigastric fullness with left-to-right gastric peristaltic wave.
Test feed is usually performed to palpate a pyloric ‘tumour’ (see Box 12.2).
Undress baby (leaving loose nappy) and place on carer's lap with head elevated.
Sit opposite baby and carer; position baby so head is to examiner's right.
Begin feeding (breast or bottle). With active suckling, the abdominal wall relaxes.
Palpate with left hand (middle finger).
Begin above umbilicus and feel into RUQ under liver edge.
Wait to feel appearance of olive-sized, firm, mobile lump in angle between liver edge and upper right rectus muscle (contracted pyloric muscles).
As stomach inflates with air and milk, pylorus becomes more difficult to feel—aspiration of NGT may help.
Diagnosis and investigations
If pyloric ‘tumour’ is felt, no radiological investigations are necessary prior to surgery.
Ultrasound shows thickened (>4mm), elongated (>16mm) pyloric muscle and increased muscle to lumen ratio with decreased movement of fluid through narrow canal (term infants).
Barium meal (rarely necessary) shows an enlarged stomach, increased gastric peristalsis, and elongated, narrowed pyloric canal.
Electrolytes and capillary blood gases (↓ Na+, ↓ K+, ↓ Cl–, base excess, and pH).
Treatment
Resuscitation with IV rehydration.
Correct hypovolaemia with 10mL/kg 0.9% saline.
Correct hypochloraemic alkalosis and hypokalaemia (may take 24–48h)—0.45% sodium chloride/5% dextrose with added potassium chloride at a rate of 120–150mL/kg/24h.
NGT drainage to prevent aspiration of vomited secretions.
Surgical treatment
Pyloromyotomy (division of pyloric muscle fibres without opening of bowel lumen).
Done via right upper quadrant incision, periumbilical, or laparoscopically.
Caution not to open mucosa and avoid the prepyloric vein (‘of Mayo’).
Start feeding within 4–6h post-operatively and increase to full volume by 24h.
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-fixed 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
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.
If in doubt, operate. Viability of twisted bowel is very time-dependent—delays in diagnosis can be very serious.
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.
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 fluid 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 gastrograffin) contrast enema. Diagnostic and may be therapeutic (see below).
Treatment
Immediate IV fluid resuscitation to correct fluid losses and to restore fluid, electrolyte, and acid/base balance.
Maintenance fluid replacement and replacement of continued losses (vomiting or nasogastric losses). Reduction only attempted once fluid balance restored.
Analgesia and sedation (morphine 0.2mg/kg) will aid process of reduction.
Antibiotics and NGT.
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 (?10% 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.
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 reflexes.
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 confirms diagnosis; thickened nerve fibres and aganglionisis (↑ AChE).
Anorectal manometry (in older children) shows failure of anal relaxation on rectal balloon distension (loss of recto-anal inhibitory reflex).
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.
Definitive 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.
Complications
Constipation.
Enterocolitis can affect 20–50% of children pre- and post-operatively (uncommon >5y of age unless an obstructive component exists).
Further reading
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 identified 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 fluids, 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 (?F508).
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 floor structures).
Low anomalies traverse a normal levator muscle.
High anomalies end above the levator and are commonly associated with a fistula (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 fistula.
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 fistula/renal anomalies).
Prophylactic antibiotics if vesicoureteric reflux is demonstrated.
Surgical treatment
Low lesions (perineal fistula). 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.
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.
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.
Treatment
Parents may opt for termination in antenatally detected defects with associated major cardiac or chromosomal anomaly (mortality ?80%).
Post-natal management involves protection of sac, insertion of NGT, IV access, and fluid 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 ?1y 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 flap.
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 O2, etc.).
Cling film wrap to protect herniated bowel against trauma, contamination, heat loss, drying, and fluid 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).
Necrotizing enterocolitis (NEC)
Key facts
Range of intestinal inflammation, ranging from mild mucosal injury to full thickness necrosis and perforation.
Perforated NEC associated with 40% mortality in neonates.
Clinicopathological features
Associated with:
Premature delivery.
Formula milk feeds.
Hypoxia.
Systemic sepsis.
‘Micro-epidemic’ outbreaks in neonatal units.
Typically affects premature babies on ventilatory support.
Features of vomiting, distension, bloody mucus passing PR.
May shows signs of severe sepsis/shock (tachypnoea, poor perfusion, temperature instability).
Diagnosis and investigations
Plain AXR. Pneumatosis intestinalis, portal venous gas, free gas if perforation, dilated, thick-walled (oedematous) bowel.
Treatment
Medical
Fluid resuscitation.
IV antibiotics.
Bowel rest and TPN.
Surgical treatment
Indicated by complications (perforation, failure to respond to medical treatment, abdominal mass, systemic sepsis).
May include:
Peritoneal drainage.
Bowel resection (usually with stoma formation).
Complications
Septicaemia.
Enteric fistulation.
Peritonitis.
Adhesions.
Enteric stricture.
Short gut syndrome.
Death.
Inguinal hernia and scrotal swellings
Inguinal hernia
Key facts
Childhood inguinal hernias derive from a persistent processus vaginalis and are invariably indirect.
♂:♀, 7:1.
Right-sided hernias (60%) are commoner than the left (25%); 15% are bilateral.
Higher incidence of complications (incarceration) than adult hernias.
Clinical features
Usually noticed as a painless swelling, variable in size in the inguinoscrotal or labial area.
More prominent when the baby cries and may disappear intermittently.
Bowel entrapment causes pain and irreducibility leads to strangulation, intestinal obstruction, perforation, and peritonitis.
Ovarian entrapment may occur in females.
Bile vomiting in a young infant should always prompt examination of the inguinoscrotal area.
Cardinal feature is a swelling in the groin above which the examining fingers cannot define the inguinal canal (‘cannot get above’).
Asymmetrical thickening of the spermatic cord in the presence of a history compatible with a hernia is strongly suggestive of the diagnosis.
Treatment
Prompt surgical treatment is important in premature/young infants to avoid risks of complications.
Herniotomy alone is adequate. No need to repair the walls of the canal; usually a simple, straightforward day case procedure.
Acute surgery can be very difficult when it is irreducible or strangulated or in very young infants.
Hydrocele
Congenital fluid-filled processus vaginalis and tunica vaginalis.
Communicates with the peritoneal cavity in children.
Scrotum is usually smoothly enlarged and sometimes bluish in colour and the testis is often surrounded by the hydrocele.
Occasionally acquired due to trauma, infection, or testicular tumour.
Simple hydroceles may resolve spontaneously up to age of 18 months; surgical intervention is deferred until 18 months.
At operation, ligation of the patent processus vaginalis and drainage of the fluid are adequate; there is no need to excise the hydrocele wall.
Varicocele
Due to a dilated pampiniform venous plexus of the spermatic cord.
Onset usually after puberty.
Has the feel of a ‘bag of worms’ during palpation of the cord.
Indications for treatment include discomfort (aching), cosmesis, and concern about fertility. The procedure is carried out by high ligation of the plexus, either by open or laparoscopic surgery.
Beware an acute left varicocele in childhood due to obstruction of the left renal vein by tumour (nephroblastoma).
Treatment may be surgical ligation or radiologically-guided embolization.
Idiopathic scrotal oedema
Aetiology unknown; possibly due to an acute allergic reaction.
Characterized by painless, red, unilateral scrotal swelling extending to the groin and the perineum.
Rapidly resolves spontaneously; the clinical diagnosis precludes the need for investigation.
Other childhood hernias
Umbilical hernia
Key facts
Persistence of the physiological umbilical defect beyond birth.
Usually close spontaneously (especially in premature infants).
Have a low incidence of complications (incarceration/strangulation).
Clinical features
Usually noticed as a painless, intermittent swelling at the umbilicus.
Treatment
Delay repair beyond age 4 in Caucasian children; beyond age 8 in Afro-Caribbean children.
Simple sutured closure of defect in surgery required.
Epigastric hernia
Key facts
Defect in the midline linea alba between the umbilicus and the xiphoid process.
Very rarely closes spontaneously.
Have a low incidence of complications (incarceration/strangulation).
Clinical features
Usually noticed as a painless, intermittent swelling above the umbilicus.
Treatment
Simple sutured closure of defect required.
Prepuce (foreskin) and circumcision
Key facts
One of the commonest reasons for referral to a paediatric surgical clinic.
Prepuce (foreskin) is initially fused to the glans penis. Preputial ‘adhesions’ lyse spontaneously as part of normal development.
Separation of the prepuce from the glans is gradual; 80% of newborns, 50% of 1y-olds, and 10% of 5y-olds will have a non-retractable prepuce.
Non-retractable foreskin
Clinical features
Only rarely causes problems which include dysuria, frequency, spots of blood, ballooning, and spraying.
Very occasionally causes recurrent balanitis with redness, soreness, and cellulitis. Preputial ‘cysts’ are often present—these are collections of subpreputial smegma and are part of normal development.
Treatment
Often only reassurance and advice are needed.
Leave the foreskin alone if asymptomatic.
Frequent bathing and hygiene and gentle attempts at retraction.
Hydrocortisone 1% topically relieves symptoms and may speed separation.
Topical or rarely, oral antibiotics only for recurrent balanitis.
Persisting symptoms warrant retraction and separation using LA or under GA.
Phimosis
Defined as a non-retractable foreskin with associated scarring that will not resolve spontaneously.
Clinicopathological features
May be congenital (uncommon) or acquired (usually age 5+) secondary to inflammation.
Commonest cause balanitis xerotica obliterans (BXO); foreskin looks pale, thickened, and scarred.
Additional symptoms to those of a non-retractable foreskin are retention of urine, paraphimosis, obstruction, and back pressure on the upper urinary tract. Consider an ultrasound scan and ascending urinary tract infection, in which case antibiotics are indicated.
Treatment
Circumcision.
Dorsal slit of foreskin.
Preputioplasty (prepuceplasty).
Non-surgical treatment using Plastibel is occasionally used in neonates.
Try to ensure the boy is happy and relaxed, lying on examination couch or parental knee.
Normal foreskin often appears long and ‘redundant’.
Gently hold tip of prepuce between finger tips, lift forward, and spread wide open. Preputial orifice usually demonstrated.
If retraction attempted, perform gently to show pouting of mucosa.
Blanching of skin below preputial opening—normal.
Tight, white contracted preputial orifice indicates fibrotic phimosis (‘muzzling’).
Undescended testis
Key facts
Testicular descent from the fetal abdominal site into the scrotum is normally complete by birth.
Absence of a scrotal testis (cryptorchidism) may be due to agenesis (rare), intra-abdominal arrest, incomplete descent (intracanalicular), or ectopic descent (inguinal, perineal, crural, penile).
Incidence 2–4% of newborn boys, falling to 1.5% at 6 months.
Commoner on the right side.
Clinical features
Undescended testis can be noted at the post-natal check, by parents, or by the GP.
Rarely presents acutely as torsion (tender mass in inguinal region).
A retractile testis is one that can be brought down into the scrotum with gentle manipulation, but retracts into the superficial inguinal pouch, either spontaneously or with minor pressure (see Box 12.4).
A cooperative, relaxed little boy is essential. Examine on carer's knee or lying down.
Control inguinal canal with finger pressure (prevents retraction of testis).
Palpate tissues superficial to external inguinal ring, working down to scrotum.
Try to manipulate testis into scrotum—then release.
True retractile testis should remain in scrotum briefly.
About 95% true retractile testes descend spontaneously before puberty and require no follow-up (?5% apparently retractile testes become ‘ascending’).
Diagnosis and investigations
No investigations are required in palpable undescended testis.
Chromosomal studies and HCG stimulation test may be requested in bilateral impalpable testes.
Ultrasound may help locate an impalpable testis.
Diagnostic laparoscopy is definitive and allows further management.
Treatment
Testis should be brought to the scrotum at 1–2y of age to avoid secondary damage due to trauma, torsion, and increased ambient temperature.
Hormone manipulation is ineffective in true undescended testis.
Intracanalicular or ectopic testis should be managed by one-stage orchidopexy.
Intra-abdominal testis can be brought down by one- or two-stage orchidopexy (50–90% success).
Laparoscopy for bilateral impalpable testes.
Scrotal position facilitates self-examination to detect signs of neoplastic change (?4 times normal in an abdominal testis).
Complications
Post-operative atrophy of the testis (<2%) unless intra-abdominal position (10–50%).
Retraction.
Maximize sperm production.
Prevent testicular torsion.
Repair of associated inguinal hernia.
Cosmesis.
Reduce chance of malignancy development and improve self-examination success.
Solid tumours of childhood
Neuroblastoma
Commonest solid abdominal tumour of childhood.
Spectrum of tumours derived from neuroblasts found in the adrenal gland, along the sympathetic chain, or extra-adrenal sympathetic tissues.
Aggressive tumour with early spread to lymph glands, liver, bone (cortex or marrow), orbits, and skin.
Presents as painless large, abdominal mass in children <2y.
May present as weight loss, hypertension, or metastatic disease.
Urinary HMMA, HVA elevated.
CT scan. Optimal investigation for suspected neuroblastoma.
Treatment by combination of chemotherapy, surgery, and radiotherapy.
Survival of between 30 and 90%, depending on the site and stage at presentation.
Nephroblastoma (Wilms’ tumour) of kidney
Fast-growing tumour of the kidney.
Ranges from benign mesoblastic nephroma of infancy to poorly differentiated, malignant nephroblastoma in the older child.
Malignant tumours frequently metastasize to regional lymph nodes, liver, and lungs.
Usually presents as a large, relatively painless abdominal mass in an otherwise well child.
Treatment by combination of chemotherapy, surgery, and radiotherapy according to histology and spread at diagnosis.
5y survival:
Early stage, 90%.
Disseminated disease, 30%.
Rhabdomyosarcoma
Tumour of striated muscle origin from the bladder, vagina, prostate, parameningeal tissue, and limbs.
Haematuria, vaginal bleeding, and the appearance of grape-like cysts (sarcoma botryoides) at the vaginal introitus.
Variable histology (embryonal is most favourable), which determines the prognosis.
Survival of up to 70% from surgery and chemotherapy.
Hepatoblastoma
This presents as a right hypochondrial mass extending across the midline.
Chemotherapy may render initially inoperable tumours resectable.
Depending on staging, size, and histology, survival of up to 70% is possible.
Neck swellings
Key facts
Embryological abnormalities may relate to:
Lymphadenopathy is very common in children, but typically ‘waxes and wanes’.
If lymphadenopathy persists for longer than 2 months and measures >2cm diameter, it should be biopsied.
Causes and clinicopathological features
Thyroglossal cyst
(
p. 222).
Branchial cysts
( p. 224).
Lymphadenopathy
The neck contains large numbers of lymph glands draining areas of potential infection in the mouth, nose, fauces, and ears.
Common causes of lymphadenopathy include upper respiratory tract infection, middle ear infections, tonsillitis, parotitis, dental abscess, atypical mycobacterial infection.
Malignant lymphadenopathy is much less common.
May be primary lymphoma.
Secondary deposits, e.g. from neuroblastoma.
Salivary gland swellings
May be due to duct obstruction (stones or duct stenosis), infection (mumps), autoimmune disorders (recurrent parotitis), neoplasia (adenoma).
Commonest in the submandibular, sublingual, and parotid glands.
Skin lesions
Dermoid cysts. Usually in the midline above the hyoid bone and are rarely infected.
Sebaceous cysts. Epidermal origin with a small central punctum; may occur anywhere, but most commonly on the scalp or back of the neck.
Lymphovascular lesions
Haemangiomas. Can be mixed capillary or cavernous haemangiomas or haemangioendotheliomas within the neck and parotid area; may grow rapidly in size and lead to high output cardiac failure or even carotid steal syndrome.
Cystic hygroma (lymphangioma). Commonly in the posterior triangle of the neck.
Treatment
Excision of dermoid cysts, sebaceous cysts, thyroglossal cysts, thyroid neoplasms, salivary gland enlargements, lymph gland enlargements (biopsy).
Haemangioma. Supportive measures (intubation, steroids, interferon, and emergency surgical intervention).
Cystic hygroma. Sclerosant injection (OK 432—streptococcal derivative), effective in lymphangiomas with few large cysts.
Neck lumps may be lateral or midline.
Lateral • Lymph node • Branchial sinuses and cyst • Cystic hygroma • Sternomastoid tumour • Haemangioma • Lymphangioma • Submandibular gland • Parotid gland • Neoplasm | Midline • Submental lymph nodes • Thyroglossal cyst • Thyroid swelling • Dermoid cyst |
Lateral • Lymph node • Branchial sinuses and cyst • Cystic hygroma • Sternomastoid tumour • Haemangioma • Lymphangioma • Submandibular gland • Parotid gland • Neoplasm | Midline • Submental lymph nodes • Thyroglossal cyst • Thyroid swelling • Dermoid cyst |
Congenital • Thyroglossal cysts • Branchial cyst • Cystic hygroma • Haemangioma • Dermoid cyst | Acquired • Reactive lymphadenopathy • Infective lymphadenopathy • Secondary tumour deposits |
Congenital • Thyroglossal cysts • Branchial cyst • Cystic hygroma • Haemangioma • Dermoid cyst | Acquired • Reactive lymphadenopathy • Infective lymphadenopathy • Secondary tumour deposits |
Incisions should be parallel with skin creases (Langer's lines).
Subcutaneous closure should be meticulous (e.g. removable 4/0, 5/0, or 6/0 continuous subcuticular monofilament).
Facial nerve. Passes between the two lobes of parotid gland.
Lingual nerve. Swerves around submandibular duct.
Thoracic duct. Enters junction of left subclavian and jugular veins.
