symptomatic relief or prolonged survival. Common solid organ tumours amenable to surgery are:Imagine operating on a 450 g baby with necrotizing enterocolitis. The name tells you what it is. She would sit comfortably in the palm of your hand if she were not ventilated on inotropes, fighting for her life. Deciding when to operate on these delicate babies is tough. While most surgical specialties are defined by an organ system, we are defined only by age, from fetal surgery to 16 years. Why can’t adult surgeons operate on children, aren’t they just little adults? No, their physiology, pharmacology, anatomy, and psychology are different.
Honours
If the child is old enough, first you will need to earn their trust. When they are scared this is even more difficult, and the ability to play with a child, distract them, and make them laugh is a skill you should enjoy practising. So spend time with the play therapists and clown doctors (you may learn some child development in the process) … and carry a bottle of bubbles.
The most common malignancies in childhood are the leukaemias, followed by brain tumours, and then solid organ tumours. Paediatric surgeons offer a range of adjunctive procedures to assist paediatric oncologists in managing these conditions, which have seen great improvements in their survival rate:
• Core needle (Tru-cut), incision/excisional biopsies.
• Long-term venous access is required for frequent blood tests and chemotherapy.
• Gastrostomy insertion to aid in feeding.
• Ovarian preservation surgery when chemotherapy and radiotherapy threaten fertility.
If slow growing, benign, or localized complete excision alone may be curative. In other cases, surgery is part of a multimodal treatment regimen aimed at curing the patient. This may include adjuvant and neoadjuvant chemotherapy and radiotherapy. In more aggressive tumours, ‘debulking’ surgery may symptomatic relief or prolonged survival. Common solid organ tumours amenable to surgery are:
• Wilms tumour (nephroblastoma)
In children these are usually indirect, transmitting bowel through the deep inguinal ring. This is a congenital failure of closure of the processus vaginalis. Herniorrhaphy (repair usually with mesh) in adults, herniotomy (division and closure of the patent processus vaginalis) in children. A thickened cord (‘silk glove sign’) may be palpable. An irreducible hernia is a surgical emergency, reducible ones may be repaired electively. Complications of hernia include:
• obstruction = blockage to lumen
• strangulation = compromised blood supply infarction.
Unlike inguinal hernias these are less likely to complicate, and may close spontaneously.
Occurs in 5% at birth but in 1% by age 1 year. Orchidopexy (offered from 6-12 months age), improves cosmesis, prevents torsion and may improve malignancy and fertility rates, both of which are adversely affected in undescended testes.
It is normal for the newborn foreskin to be adherent to the glans and not retract. This is not a pathological phimosis, and resolves naturally with growth. By 3 years of age, 90% of foreskins will retract. Non-retractile foreskins may cause ballooning, which on its own is of little consequence and will subside once the foreskin is retractile. Definite indication for circumcision is balanitis xerotica obliterans, where there is whitish scarring; rare before 5 years of age.
Is a common occurrence in childhood and usually transient, self-limiting, and dealt with at home or in the community. When persistent, severe, associated with other symptoms (e.g. vomiting, diarrhoea) or a systemically unwell child, accurate assessment requires a detailed history and experienced examination. Causes include:
Is difficult to diagnose in children. If in doubt, US scanning can be very useful. As children are less able to localize their pain, perforation and its complications are more common.
The neonatal period is defined as the first 28 days of life, although some ‘neonatal’ babies will spend many months on the unit.
Many congenital abnormalities are today diagnosed antenatally which can ethically and emotionally difficult decisions about whether to continue or to terminate the pregnancy.
Is bowel inflammation that necrosis prior to severe sepsis ± perforation. Ninety percent of cases occur in premature babies after introduction of feeds. In the early stages there will be signs of systemic sepsis (brady- or tachycardia/desaturation/blood glucose instability/pyrexia/poor handling). There should also be some sign of bowel dysfunction ( 
/green aspirates, bloody stools, distension) and abdominal wall discoloration. AXR may show an ileus (suspected NEC), or intramural gas (proven NEC). NEC can perforation. Early NEC can be managed with bowel rest, antibiotics, and organ support. Failure of medical management and perforation laparotomy.
Oesophageal atresia (OA) is a discontinuity in the oesophagus, and usually a fistula with the respiratory tract. The most common configuration is OA with distal tracheo-oesophageal fistula. The baby is unable to swallow secretions and feed and so froths at the mouth and vomits. Aspiration respiratory symptoms. If there is clinical suspicion, a NG tube should be passed and will get stuck at about 10 cm from the lips and not go into the stomach. This should be confirmed on an X-ray. This tube should be kept on regular suction to prevent further aspiration. In theatre after a right thoracotomy, the lung is retracted forwards, the fistula is identified as it enters the trachea and divided and closed, and an anastomosis performed between the two ends of the oesophagus (less than half the diameter of your little finger). Small bowel atresias present with bilious vomiting and are usually easy to diagnose with an AXR. The number of gas bubbles seen on the X-ray will give you some idea of the level of the obstruction. A single gastric bubble suggests pyloric atresia (very rare), a ‘double-bubble’ sign is seen in duodenal atresia (larger stomach bubble and smaller duodenal bubble), a ‘few’ bubbles suggest jejunal atresia, more bubbles suggest ileal atresia. Large bowel atresia is less common. The more distal the atresia, the more the number of bubbles, but the more difficult to diagnose on a plain X-ray, in which a contrast study may help.
Are easy to diagnose as usually the anus is not where it should be. Some have no opening, but most have a fistulous tract opening into the perineum or genitourinary tract. A few can be corrected in the first few days of life with a primary anoplasty (repair), most require a stoma to allow the child to defecate and feed while awaiting definitive repair when older.
Biliary atresia is a cause of prolonged jaundice in neonates. It remains the most common indication for liver transplantation in children. Choledochal cysts do not usually present until infancy, childhood, or even later, with either obstructive jaundice, abdominal pain, ± mass.
Is failure of fusion of the posterior column of the spine. It ranges from occulta (hidden) with no neurological defect to various types of spina bifida aperta (open) where the meninges (meningocele) or meninges and spinal cord (myelomeningocele) are exposed neurological damage. Most commonly the lumbosacral spine is affected. These nerves supply the lower limbs, bowel, and bladder, all of which can be variously affected. The defect is usually repaired in the first few days. In utero surgery before birth is also possible. Many patients subsequently develop hydrocephalus, which requires surgical drainage. A MDT approach to management of limb, bowel, and bladder dysfunction is the mainstay of treatment. In the UK with (1) folate supplementation, (2) antenatal diagnosis, and an (3) increasing decision for termination, this condition is less commonly seen in the newborn period.
In gastroschisis, the baby is born with bowels protruding through a defect usually to the right of the umbilicus. The bowels have been exposed to amniotic fluid in utero and hence are thickened and often matted together. Sometimes they can be returned into the abdomen and the defect closed at one operation but if there is too much bowel, a plastic silo is attached to the umbilical defect (there are both operative and non-operative methods) to contain the bowel as it is gently squeezed into the abdomen over the course of a few days. The abnormal bowel takes some weeks to start working during which time nutrition is maintained with total parenteral nutrition. In high-income countries, survival is >90%, but in developing countries survival is probably <10%. In exomphalos the bowels ± the liver are outside the abdomen, but contained within a membrane. As such, the bowel is usually healthy and feeding can be commenced at birth. However, exomphalos can be associated with other serious abnormalities so while the bowel may be healthy, survival can be worse than for gastroschisis.
Usually diagnosed antenatally in high-income countries, congenital diaphragmatic hernia is less commonly seen in low-income countries, probably because newborns with severe respiratory distress don’t make it to central hospitals. The underlying lung has various degrees of pulmonary hypertension, so these children may be born in severe respiratory distress, or in some cases present as older children with mild, recurrent chest symptoms or GI symptoms. Intensive care management of those born with severe pulmonary hypoplasia is complex. Pulmonary hypertension not only respiratory failure but also right heart failure. Deciding the best time to operate can be challenging, and there is still significant mortality from this condition.
Typically presents with projectile, non-bilious vomiting in a hungry child from 3 weeks to 3 months. Blood gas analysis reveals a hypochloraemic, hypokalaemic metabolic alkalosis. A test feed in patient, experienced hands reveals a palpable a palpable ‘olive’ in the RUQ. If in doubt, US scanning usually confirms the diagnosis. Treatment is by pyloromyotomy after correction of dehydration and acid–base imbalance.
Bilious (green) vomiting is a child must make you consider malrotation. Normally the duodenojejunal flexure (DJF) is high on the left, and the caecum low on the right. This produces a broad, stable mesentery. Failure of normal rotation results in a low DJF on the right, a high caecum, and a narrow mesentery, which is prone to twisting. This twist may be intermittent but can progress ischaemia and infarction of the entire small bowel and catastrophe. Therefore, green vomiting should be investigated with an urgent contrast follow through to exclude malrotation.
During neonatal laparotomies for other indications one may see a transient intussusception, when the proximal bowel (intussusceptum) is propulsed into the distal bowel (intussuscipiens). These are self-resolving. If, however, there is a lead point (e.g. lymph nodes, Meckel’s diverticulum, polyps/tumours), this may progress to obstruction and ischaemia. The classical clinical triad is intermittent inconsolable crying, redcurrant jelly stool (sloughed, bloody, ischaemic mucosa), and bilious vomiting (obstruction). The priority is vigorous fluid resuscitation and NG drainage. Abdominal X-ray may show obstruction and a meniscus sign but US scanning is more specific and sensitive. If caught early, air enema reduction is successful in up to 90% of cases. Bowel resection may be avoided if the intussusception is manually reduced (squeezed out).
Is congenital aganglionosis of the distal bowel. This causes a tonic contraction with subsequent massive proximal dilatation (many years ago it was the dilated bowel that was thought to be abnormal, it is actually the collapsed distal aganglionic bowel that needs to be resected). There is usually a history of delayed passage of meconium and constipation and this may be associated with distension and vomiting, especially during an episode of Hirschsprung’s-associated enterocolitis which is potentially fatal. The initial management is bowel rest, antibiotics if enterocolitis is expected, and decompression of the distal bowel. This may be achieved with rectal washouts but if unsuccessful, a stoma is required. The diagnosis is confirmed with a rectal biopsy.
In high-income countries is today mostly diagnosed on routine antenatal scanning. While many resolve in utero, of those that persist at birth some require urgent action (posterior urethral valves), others careful follow-up (pelviureteric junction (PUJ) obstruction, vesicoureteric junction (VUJ) obstruction, and vesicoureteric reflux (VUR). Follow-up may involve biochemical tests of renal function (U&E or glomerular filtration rate) or radiological studies looking at anatomy (degree and site of dilation, e.g. US) or function (percentage function of each kidney and/or clearance, e.g. nuclear medicine scans). If symptomatic, increasing dilatation or decreasing function surgery may be indicated. PUJO is treated with open or laparoscopic pyeloplasty, VUJO by ureteric reimplantation, and sever VUR by narrowing the ureteric orifice with endoscopic submucosal injection or by reimplanting the refluxing ureter.
It is a triad of defects all due to deficient tissue on the ventral aspect of the penis: proximally sited meatus, chordee, and hooded foreskin. Surgery can repair the defect but most involve using the foreskin, so parents should be advised not to circumcise children with hypospadias.
An embryo with two X chromosomes will become female, the gonads develop as ovaries. The Y chromosome contains the SRY gene. This codes for TDF (testis-determining factor), which causes the gonads to become testicles and produce testosterone (which causes development of male external genitalia and has an effect on the brain) and Mullerian inhibiting substance (which causes regression of the female internal genitalia, uterus, and fallopian tubes). Disruption in any of these pathways a plethora of abnormalities in which the sex of the child may not be clear and cause life-threatening biochemical abnormalities. These children will be cared for by an MDT in partnership with the parents to decide the sex of rearing. At some point, surgery may be required to make the external genitalia look more normal.