Surgery 3

Question 1

How does a Tracheo-oesophageal fistula (TOF) and oesophageal atresia (OA) present?

Answer 1

Occurs in 1 in 3500 live births
Associated with polyhydramnios during pregnancy or an absent stomach bubble on antenatal USS screening
Persistent salivation and drooling
Infant will cough and choke when fed and have cyanotic episodes
Aspiration of saliva, milk or stomach acid

Question 2

How is Tracheo-oesophageal fistula (TOF) and oesophageal atresia (OA) diagnosed?

Answer 2

If suspected, a wide-calibre feeding tube is passed after birth and checked by X-ray to see if it reaches the stomach

Question 3

What is the management for Tracheo-oesophageal fistula (TOF) and oesophageal atresia (OA)?

Answer 3

Continuous suction is applied to the tube in the oesophageal

pouch to reduce aspiration of saliva and secretions, while awaiting urgent repair surgery

Question 4

What are the complications of Tracheo-oesophageal fistula (TOF) and oesophageal atresia (OA)?

Answer 4

Gastro-oesophageal reflux
Chronic cough
Need for oesophageal dilation during
infancy or childhood (the cute infant on D35)

Question 5

How does exomphalos present?

Answer 5

The abdominal contents protrude through the umbilical ring, covered with a transparent sac formed by the amniotic membrane and peritoneum. Also called omphalocele
Often associated with congenital abnormalities:
(lethal) trisomies
Sex chromosomes abnormalities
Cardiac, CNS and GU anomalies

Question 6

How does gastroschisis present?

Answer 6

the bowel protrudes through a defect in the anterior abdominal wall adjacent to the umbilicus and there is no covering sac. It is not associated with other congenital abnormalities

Question 7

What is the management for gastroschisis?

Answer 7

Carries great risk of dehydration and protein loss, so the abdomen should be covered with a clear occlusive wrap to minimize fluid and heat loss.
An NG tube is passed to aspirate frequently and IV fluids are given regularly and early on to prevent hypovolaemia

Question 8

What are the common umbilical abnormalities?

Answer 8

Patent vitello-intestinal duct
= allows for the intermittent enteric contents to be extruded through the umbilicus. Usually noted few days after birth (rare)
May be associated with umbilical polyp.
Patent urachus= allows for urine to drain from to bladder to the umbilicus
Umbilical granuloma
= occurs where the inflammatory process at the umbilicus becomes florid with excess granulation tissue preventing the raw area from developing new epithelial tissue.

Question 9

What us the management for Patent vitello-intestinal duct?

Answer 9

Treated early with laparotomy or laparoscopy and excision of the duct to prevent volvus or intussusception.

Question 10

How does an umbilical granuloma present?

Answer 10

Commonly due to infection Pouting umbilicus covered by bright red, moist, friable mass of
granulation tissue
Usually after the umbilical cord has been cut, the small remnant falls off and the inflammation at the line of demarcation is quickly covered by epithelium
Managed with silver nitrate cauterisation

Question 11

What is bladder exstrophy?

Answer 11

protrusion of the urinary bladder through the abdominal wall.
It often includes other abnormalities of the bony pelvis, pelvic floor and genitalia
Managed with surgery

Question 12

What are the anorectal malformations?

Answer 12

Low type = colon remains close to the skin. There may be stenosis
(narrowing) of the anus or the anus may be missing altogether, with the rectum ending in a blind pouch
High type = colon is higher up in the pelvis and there is a fistula connecting rectum to the bladder, urethra or genitalia
Persistent cloaca = rectum, vagina and urinary tract all open into the same channel

Question 13

How does pyloric stenosis present?

Answer 13

presents at 2-8 weeks of age, irrespective of gestational age. Clinical features include:
Non-bilious projectile vomiting, which increases in frequency
Hunger after vomiting until dehydration leads to loss of interest in feeding
Weight loss (if late presentation)
Epigastric bulge (feels like an olive) with visible peristalsis from the left to the right

Question 14

What are the risk factors for pyloric stenosis?

Answer 14

Prematurity
Caesarean delivery
Being a boy, particularly first born
Family history , especially on maternal side(maternal brothers)

Question 15

What occurs in pyloric stenosis as a result of vomiting stomach contents?

Answer 15

Hypochloremic, hypokalemic metabolic alkalosis
Low Cl impairs kidney’s ability to excrete bicarbonate, making it hard to correct the alkalosis
Dehydration leads to secondary hyperaldosteronism, which
leads to sodium retention at the expense of potassium

Question 16

What are the investigations for pyloric stenosis?

Answer 16

test feed, unless immediate fluid resuscitation is needed:
Baby is given milk feed, which will calm the hungry infant and
allow to observe a peristaltic wave
USS examination may be helpful to confirm the diagnosis prior to surgery

Question 17

What is the management for pyloric stenosis?

Answer 17

Initial priority is to correct any fluid and electrolyte misbalance by IV fluids.
Once hydration, electrolytes and acid-base balance is estalished- pyloromyotomy:
o Division of hypertrophied muscle down to, but not including, the mucosa
o Performed as open procedure or laparoscopically
Post op, the child can be fed within 6 hours and discharged within 2 days

Question 18

How does Wilms tumour (nephroblastoma) present?

Answer 18

Tumour originating from embryonal renal tissue. Most common renal tumour of childhood
over 80% of patients present before 5 years of age and it is very rarely seen after 10 years of age
large abdominal mass, often found incidentally in an otherwise well child.
Haematuria

Question 19

How is Wilms tumour diagnosed?

Answer 19

Staging is used to assess for distant metastases (usually in
the lung) and function of the contralateral kidney.
Around 5% of patients have bilateral disease at diagnosis (need careful management to preserve as much kidney function as possible)

Question 20

What is the management for a Wilms tumour?

Answer 20

Initial chemotherapy followed by delayed nephrectomy, after which the tumour is staged histologically and subsequent treatment is planned according to the surgical and pathological findings

Question 21

What is a neuroblastoma?

Answer 21

arise from neural crest tissue in the adrenal medulla and sympathetic nervous system. Spectrum of disease from benign (ganglioneuroma) to highly malignant (neuroblastoma)
Most common before the age of 5. In young infants, spontaneous regression sometimes occurs.

Question 22

How does a neuroblastoma present?

Answer 22

Abdominal mass, but the primary tumour can lie anywhere along the sympathetic chain from the neck to the pelvis. It is often large and complex, crossing over the midline and involving major blood vessels and lymph nodes
Paravertebral tumours may invade through the adjacent intervertebral foramenàspinal cord compression
Over the age of 2àclinical symptoms are mostly from metastatic disease:
• Bone pain
• Bone marrow suppression, causing weight loss • Malaise

Question 23

What are the investigations for a neuroblastoma?

Answer 23

characteristic clinical and radiological features with raised urinary catecholamine metabolite levels suggesting neuroblastoma.
Confirmatory biopsy is usually obtained and evidence of metastases detected with bone marrow sampling

Question 24

How does a sacrococcygeal teratoma present?

Answer 24

rare tumour that develops at the base of the coccyx and is thought to be
derived from the primitive streak (germ cells tumour). It most commonly affects newborn and is unusual in over 5 months olds.
can be diagnosed prenatally either by elevated maternal alpha fetoprotein (AFP) levels or because the uterus is larger than expected. It resembles a fluid-cyst cyst or solid mass sticking out from the fetus’ body