Paediatric Gastroenterology Flashcards
What is abdominal migraine?
Episodes of central abdominal pain lasting more than 1 hour
Intense and acute pin
Interferes with normal activity
Associated N+V, headache, photophobia, aura
How can abdominal migraine be treated?
Dark, quiet room
Paracetamol
Sumatriptan
Propylaxis –> Pizotifen
What is classed as constipation in children?
<3 stools per week (does not apply to exclusively breastfed babies)
Rabbit dropping stools
How can you differentiate between primary and secondary constipation?
Secondary constipation is from birth
If meconium takes longer than 48 hours to pass, ribbon stools, faltering growth, or vomiting - referral needed
How is primary/idiopathic constipation treated?
First line in children is an osmotic laxative e.g. Movicol
If no response can add a stimulant e.g. Senna +/Lactulose
red flag diagnosis for sacral dimple:
spina bifida occulta (ask about folic acid intake)
causes of failure to pass meconium within 1st 24 hours:
- Hirschprung’s disease (Down’s syndrome)
- Cystic fibrosis
- Anal atresia
A) Add senna
1. Movicol (osmotic laxative)
2. stimulant laxative eg senna
3. lactulose (osmotic laxative) or docusate (stool softener if stools are hard)
Abdo pain summary (& differentials)
Constipation summary
what is the most common cause of surgical abdominal pain in children?
Appendicitis
What is the main cause of reflux in babies?
Immaturity of the lower oesophageal sphincter
How can reflux in babies be managed?
Small, frequent feeds
Burp regularly
Keep baby upright after feeding
If still problematic can mix Gaviscon with feeds
What is pyloric stenosis?
Hypertrophy and narrowing of the pyloric sphincter (the ring of muscle between the stomach and duodenum)
How does pyloric stenosis present?
Usually presents in 2nd-4th week of life
Peristalsis tries to push food down to the duodenum but it instead ejects upwards
Projectile vomiting (non-bilious)
May be constipation/diarrhoea
May be a palpable mass due to hypertrophied pyloric sphincter (often mentioned as olive shaped mass)
Pyloric stenosis investigation findings:
- Investigations:
o Test feed observe for gastric peristalsis
o USS confirmation – target lesion, >3mm thickness>
aetiology of pyloric stenosis:
-Age at presentation: majority between 3-6 weeks of life
-Sex predilection: males > females (ratio of approximately 4:1)
-Race predilection: More common in Caucasian people
-Inheritance pattern: polygenic
-Occurs in 0.3% of all births
pyloric stenosis management:
Resuscitate and correct any metabolic abnormalities first.
Pyloric stenosis is not a surgical emergency and any fluid deficit or alkalosis should be corrected initially.
Most infants should have their fluid status corrected within 24 hours.
-Treatment involves a laparoscopic pyloromyotomy (known as “Ramstedt’s operation“). An incision is made in the smooth muscle of the pylorus to widen the canal allowing that food to pass from the stomach to the duodenum as normal (The usual approach is via a right upper-quadrant incision and the pyloric muscle is split longitudinally down to the mucosa.)
- Prognosis is excellent following the operation.
What blood gas results are seen in pyloric stenosis?
Low chloride
Low potassium
Metabolic Alkalosis (hypochloraemia, hypokalaemic) initially and then lactic acidosis and can also be seen later in the clinical course of pyloric stenosis as the dehydration worsens
Due to baby vomiting hydrochloric acid from stomachn
What are signs of clinical dehydration in children?
Decreased urine output
Sunken eyes
Dry mucuous membranes
Tachycardia
tachypnoea
Reduced skin turgor
What are signs of clinical shock in children?
Decreased consciousness
Cold extremities
Pale/mottled skin
Tachycardia
Tachypnoea
Weak peripheral pulses
Prolonged cap refill
Hypotension
How to calculate replacement fluids in children?
(% dehydration x kg x 10) = mls of fluids
If their normal weight is known:
1. subtracting their current (dehydrated) weight from their normal weight
2. dividing the result by their normal weight, and then
3. multiply it by 100
How to calculate maintenance fluids in children?
First 10kg = 100ml/kg
Next 10kg = 50ml/kg
After that = 20ml/kg
How to calculate resuscitation fluids in children?
Resuscitation fluids = 20ml/kg
EXCEPT IN….
neonates, DKA, septic shock, trauma, cardiac pathology (heart failure) = 10ml/kg
What is Hirschsprung’s disease?
A congenital condition where the nerve cells of the myenteric plexus are absent = absence of parasympathetic ganglion cells along a section of the bowel
Aganglionic section does not relax - causing obstruction of the bowel (Hirschsprung disease (HD) is a congenital disorder characterized by the absence of ganglion cells (GC) at the Meissner’s plexus (submucosa) and Auerbach’s plexus (myenteric plexus *(muscularis) )
parasympathetic neuroblasts fail to migrate from the neural crest to the distal colon → developmental failure of the parasympathetic Auerbach and Meissner plexuses → uncoordinated peristalsis → functional obstruction
What conditions are associated with Hirschsprung’s disease?
- Downs syndrome
- Neurofibromatosis
- Waardenburg syndrome (a genetic condition causing pale blue eyes, hearing loss and patches of white skin and hair)
- Multiple endocrine neoplasia (MEN2) type II
3x more common in males (4-to-1 male predominance)
How does Hirschsprung’s disease present?
- Delay in passing meconium (more than 24 hours)
- Chronic constipation since birth
- Abdominal pain and distention
- Vomiting
- Poor weight gain and failure to thrive
-enterocolitis (toxic megacolon): fever, abdominal pain, bloody diarrhoea, shock
what are 3 findings in this chest & abdominal radiograph?
- Air under diaphragm
- Dilated intestine
- Free air around the liver
The X-ray answer shows multiple dilated loops of bowel. In the neonate it is difficult to differentiate between large and small bowel. If there are a few dilated loops of bowel seen on an X-ray, the obstruction will be proximal e.g. malrotation, jejunal atresia. Duodenal stenosis has a classic double bubble appearance on X-ray .
If there are multiple dilated loops of bowel seen on an abdominal film of a patient who has not passed meconium, the patient is likely to have a more distal obstruction e.g. ileal atresia, meconium ileus or plug, Hirshsprung’s or anal atresia. The left-hand x-ray shows there are multiple dilated loops of bowel and air is seen in the rectum. Hirschsprung’s disease is therefore the most likely diagnosis. Note the free air below the diaphragm, adjacent to the falciform ligament and around the bowel. The patient has perforated hence the sepsis, tachycardia and tachypnoea.
Dangerous Complication of Hirschprung’s disease and management of it :
Hirschsprung-associated enterocolitis (HAEC ie toxic megacolon) is inflammation and obstruction of the intestine occurring in around 20% of neonates with Hirschsprung’s disease. It typically presents within 2-4 weeks of birth with fever, abdominal distention, diarrhoea (often with blood) and features of sepsis. It is life threatening and can lead to toxic megacolon and perforation of the bowel.
Initial treatment is supportive with fluid resuscitation, decompression with NG and rectal tubes, and broad-spectrum antibiotics (including anaerobic coverage). Surgery is the definitive treatment.
management of Hirschprung’s disease:
Management
initially: rectal washouts/bowel irrigation
Definitive management: surgery to affected aganglionic segment of the colon (Most patients will live a normal life after corrective surgery, although they can have long term disturbances in bowel function and may be left with some degree of incontinence)
-Surgical procedures of choice include the endorectal pull-through, recto-sigmoidectomy, retro-rectal transanal pull-through, and ano-rectal myomectomy
-A temporary colostomy may be performed to allow patient growth, and surgical intervention may be deferred altogether in the presence of enterocolitis.
Unwell children and those with enterocolitis will require initial fluid resuscitation and management of the intestinal obstruction. IV antibiotics are required in HAEC.
What is the gold standard diagnosis for Hirschsprung’s disease? other tests?
Rectal biopsy
Other: contrast barium enema (Enemas should be avoided in those suspected to have Hirschsprungs’s disease who are systemically unwell, as perforation may be present, and an enema may lead to worse peritonitis.)
Diagnosis is confirmed histologically with either a sub-mucosal or a full thickness rectal biopsy in association with either anorectal manometry or barium enema. The affected segment lacks ganglion cells which aid in normal peristalsis. Without these ganglion cells, normal peristalsis is lacking, resulting in a functional obstruction. This produces a proximal dilated colon and a distal normal appearing segment which is seen as a transition zone on a barium enema. The rectum and sigmoid are most commonly involved, with decreasing incidence progressing cephalad. Total aganglionosis of the colon is a rarity, and small bowel involvement is even less common.
What is intussusception?
An invagination of a portion of the bowel (bowel telescoping)
what does this abdominal X-ray show?
- Paucity (lack of) of air in RUQ
- Poorly defined liver edge
- Paucity of gas in large bowel
- Dilated bowel loops of small bowel
There is a mass and paucity of bowel gas in the right upper quadrant causing loss of clarity of the liver edge. This represents the intussusception. The intussusception is likely to be ileo-colic due the position in the RUQ; the dilated loops of small bowel proximal to the intussusception and paucity of gas within the deflated large bowel distal to the obstruction. An abdominal X-ray is essential to look for free air if the patient has signs of peritonitis and may have perforated.
what is the most common type of intussusception?
The most common type of intussusception is ileo-colic, also known as ileo-caecal (90%). A portion of terminal ileum intussuscepts through the ileo-caecal valve into the colon. It may sometimes extend all the way to the rectum. Other rarer types of intussusception include ileo-ileal, colo-colic, and ileo-ileocolic. The majority of intussusceptions are idiopathic.
How does intussusception present? triad? age group”
triad of :
1. Severe colicky abdominal pain
2. RED CURRANT JELLY STOOL (& mucus)
3. Vomiting (initially non-bilious then bilious as obstruction ensues)
(Sausage shaped mass in the RUQ (hypochondrium): same location os “olive shaped mass” in pyloric stenosis, “target” or “donut” sign)
-right hypochondrium sausage-shaped mass and emptiness in the right lower quadrant (Dance sign)
Pale, unwell child
-drawing legs to chest during attack
-sunken fontanelle (dehydration sign)
-5months-1 year (2x more common in males>females)
-seasonal occurrence with viral illness
Target sign on USS (intussuseption) also known as/what is it?
The target sign, also known as the doughnut sign or bull’s eye sign
- is the appearance of alternating echogenic and hypoechoic bands caused by the telescoping of the bowel.
Intussusception summary
What is seen on abdominal ultrasound in intussusception? what other scan be done?
Target shaped mass (most commonly ileocaecal junction)
-air contrast enema
An ultrasound is usually performed first and the intussusception is usually seen as a mass (fig.3). Ultrasound is useful at demonstrating free fluid indicating peritonitis and may show if the bowel is ischaemic (with colour doppler) which would both be contraindications to air reduction.
Air contrast enema and attempts at radiological air reduction are usually performed following the ultrasound (provided that the patient is resuscitated, stable and there are no contraindications), even if the ultrasound scan is normal when there is a strong clinical suspicion of the diagnosis. Evidence of bowel perforation necessitates immediate surgery.
what are some possible ‘lead points’ in intussusception?
An anatomic lead point (a piece of intestinal tissue which protrudes into the bowel lumen such as a polyp) occurs in approximately 10% of intussusceptions. This is most often found in children older than 2 years. Possible lead points include Meckel’s diverticulum, polyps, an inflamed appendix, neoplastic lesions, and ileal duplications.
what investigations can be done for intussusception?
- per rectal examination
- Abdominal XR
- Abdominal USS
Per rectal examination often reveals blood on the gloved finger which is highly suggestive of the diagnosis. AXR may show air fluid levels. Ultrasound scan (Fig 1) will confirm diagnosis showing the “donut”, sign when the intussusceptions are viewed end on. A referral to the paediatric surgeons should not be delayed in order to get a scan done.
what are indications for urgent surgical treatment in intussusception?
- peritonitis on examination
- failure of radiological air reduction
- collapse and shock
How is radiological air reduction done? Success rate?
Air is introduced under pressure through a rectal catheter. A manometer monitors the pressure and this should not exceed 110mmHg. The air pressure meeting the intussusception usually forces the inverted bowel back through the ileo-caecal valve and into its expected position. This is visualised under fluoroscopic (X-ray) control. If reduction fails surgical reduction is required.
The success rate is 50-85% depending on factors such as duration of obstruction (more difficult if >48 hours since intussusception)
when is radiological air reduction not attempted for intussusception:
If there’s evidence of perforation, peritonitis (eg Abdo x-ray), or Henoch-Schönlein purpura
what is intussusception associated with?
-CF (hypertrophied mucosal glands)
-haemophilia
- HSP (Henoch-Schonlein purpura, (intestinal wall haematoma)
-lymphoma
-hypertrophied Peyer’s patches (enlarged mesenteric lymph nodes) following a respiratory infection or gastroenteritis may serve as the lead point.
Typical demographics for intussusception:
<1 years
male predominance
How is intussusception managed?
5months-3 years: Reduction by air insufflation via radiology
older/very ill children with
signs of perforation/peritonitis: operative treatment with removal of affected bowel segment & end-to-end anastomoses
What is biliary atresia?
A congenital condition where a section of the bile duct is narrowed or absent , preventing the excretion of conjugated bilirubin
How does biliary atresia present?
Presents in first few weeks of life with prolonged jaundice (>2 weeks in term babies, >3 weeks in premature babies)
Dark urine
Pale stools
-Appetite and growth disturbance (abnormal growth), however, may be normal in some cases
Signs:
-Hepatomegaly with splenomegaly
-Cardiac murmurs if associated cardiac abnormalities present
How is biliary atresia diagnosed?
Raised conjugated billirubin
ERCP Cholangiogram
How are umbilical hernias in children managed?
Common in neonates – usually resolve by 3 years
If not resolved by 5 years can consider surgical repair
Note: Umbilical hernias are more common in Down syndrome
What is congenital diaphragmatic hernia?
Herniation of the abdominal viscera into the chest cavity due to incomplete formation of the diaphragm
How does congenital diaphragmatic hernia present?
Pulmonary hypoplasia + hypertension
Respiratory distress shortly
coeliac disease summary
villous atrophy and crypt cell hyperplasia and increased intraepithelial lymphocytes
-Two eight or not to it (DQ2 and DQ8)
Diarrhoea causes summary
gastroschisis vs omphalocoele (exomphalos): delivery options, associations/management options:
Gastroschisis describes a congenital defect in the anterior abdominal wall just lateral to the umbilical cord.
Management
vaginal delivery (i in vaginal and in immediate repair) may be attempted
newborns should go to theatre as soon as possible after delivery, e.g. within 4 hours
In exomphalos (also known as an omphalocoele) the abdominal contents protrude through the anterior abdominal wall but are covered in an amniotic sac formed by amniotic membrane and peritoneum.
Associations
-Beckwith-Wiedemann syndrome
-Down’s syndrome
-cardiac and kidney malformations
Management
caesarean section (ECS: e in omphalocoele and e in staged repair) is indicated to reduce the risk of sac rupture
a staged repair may be undertaken as primary closure may be difficult due to lack of space/high intra-abdominal pressure
if this occurs the sacs is allowed to granulate and epithelialise over the coming weeks/months
this forms a ‘shell’
as the infant grows a point will be reached when the sac contents can fit within the abdominal cavity. At this point the shell will be removed and the abdomen closed
what can abdominal x-rays show in necrotising enterocolitis?
-dilated bowel loops (often asymmetrical in distribution)
-bowel wall oedema
-pneumatosis intestinalis (intramural gas)
-portal venous gas
-pneumoperitoneum resulting from perforation
air both inside and outside of the bowel wall (Rigler sign)
-air outlining the falciform ligament (football sign)
what is the management of Necrotising enterocolitis?
-Neonates with suspected NEC need to be nil by mouth with IV fluids, total parenteral nutrition (TPN) and antibiotics (10-14 days) to stabilise them.
-early intubation, inotropic support
-A nasogastric tube can be inserted to drain fluid and gas from the stomach and intestines.
-“drip and suck”:
- Nil by mouth (don’t put food or fluids in if there is a blockage)
- IV fluids to hydrate the patient and correct electrolyte imbalances
- NG tube with free drainage to allow stomach contents to freely drain and reduce the risk of vomiting and aspiration
NEC is a surgical emergency and requires immediate referral to the neonatal surgical team. Some neonates will recover with medical treatment. In others, surgery may be required to remove the dead bowel tissue. Babies may be left with a temporary stoma if significant bowel is removed (laborotomy –> bowel resection–>stoma)
presentation of NEC:
- Intolerance to feeds
- Vomiting, particularly with green bile
- Generally unwell
- Distended, tender abdomen
- Absent bowel sounds
- Blood in stools
When perforation occurs there will be peritonitis and shock and the neonate will be severely unwell.
treatments of early onset neonatal jaundice in order:
- phototherapy
- IV Ig
- Exchange transfusion
Phototherapy should be commenced swiftly to control the progression of early onset neonatal jaundice. Repeat the bilirubin frequently, usually every 4-6h. If concerns remain, IV fluids may be started and immunoglobulins prescribed in order to prevent the need for an exchange transfusion, which is associated with significant risks and side effects.
Diagnosis/Investigations/Management options:
GORD summary
differentials for colic (mnemonic)
GORD, overfeeding, incomplete burping following feeds, food allergies (children with colic usually have accelerated growth eg weight gain)
different types of biliary atresia:
Type 1: The proximal ducts are patent, however, the common duct is obliterated
Type 2: There is atresia of the cystic duct and cystic structures are found in the porta hepatis
Type 3: There is atresia of the left and right ducts to the level of the porta hepatis, this occurs in >90% of cases of biliary atresia
Biliary atresia summary
posseting/regurgitation/vomiting terminology
GOR definition/symptoms/investigations
It is normal for babies to have some reflux after larger feeds. It becomes more troublesome when this causes them to become distressed. Signs of problematic reflux include:
- Chronic cough
- Hoarse cry
- Distress, crying or unsettled after feeding
- Reluctance to feed
- Pneumonia
- Poor weight gain
Children over one year may experience similar symptoms to adults, with heartburn, acid regurgitation, retrosternal or epigastric pain, bloating and nocturnal cough.
GOR management/mnemonic:
- Conservative measures:
Initial Management (no positional management – MUST sleep on back):
* If breast-fed:
o 1st : breastfeeding assessment (30 degree head up, Burping regularly to help milk settle, tricycling legs)
o 2nd : consider trial of alginate for 1-2 weeks
o 3rd : pharmacological
- If formula-fed:
o 1st : review feeding history
o 2nd : trial smaller, more frequent feeds (aim for 150-180 mL/kg/day)
o 3rd : trial of thickened formula (e.g. containing rice starch –> Enfamil, Carabel)
o 4th : trial of alginate therapy (stop periodically to see if infant has recovered)
o 5th : pharmacological
Pharmacological Management (only done in certain circumstances):
* 4-week PPI/H2 antagonist trial – in children unable to describe symptoms or ≥1 of:
o Unexplained feeding difficulties (refusing feeds, choking)
o Distressed behaviour
o Faltering growth
o Children complaining of persistent heartburn, retrosternal or epigastric pain
* Mnemonic (order):
o G Gaviscon (a form of alginate therapy)
o O Omeprazole
o R Ranitidine
o D Dunno, so refer to get metoclopramide
Rarely in severe cases they may need further investigation with a barium meal and endoscopy.
Surgical fundoplication can be considered in very severe cases, however this is very rarely required or performed.
This usually improves as they grow and 90% of infants stop having reflux by 1 year.
red flags for vomiting:
- Not keeping down any feed (pyloric stenosis or intestinal obstruction)
- Projectile or forceful vomiting (pyloric stenosis or intestinal obstruction)
- Bile stained vomit (intestinal obstruction)
- Haematemesis or melaena (peptic ulcer, oesophagitis or varices)
- Abdominal distention (intestinal obstruction)
- Reduced consciousness, bulging fontanelle or neurological signs (meningitis or raised intracranial pressure)
- Respiratory symptoms (aspiration and infection)
- Blood in the stools (gastroenteritis or cows milk protein allergy)
- Signs of infection (pneumonia, UTI, tonsillitis, otitis or meningitis)
- Rash, angioedema and other signs of allergy (cows milk protein allergy)
- Apnoeas are a concerning feature and may indicate serious underlying pathology and need urgent assessment
what is sandifer’s syndrome?
This is a rare condition causing brief episodes of abnormal movements associated with gastro-oesophageal reflux in infants. The infants are usually neurologically normal. The key features are:
- Torticollis: forceful contraction of the neck muscles causing twisting of the neck
- Dystonia: abnormal muscle contractions causing twisting movements, arching of the back or unusual postures
The condition tends to resolve as the reflux is treated or improves.
Generally the outcome is good. It is worth referring patients with these symptoms to a specialist for assessment, as the differential diagnosis includes more serious conditions such as infantile spasms (West syndrome) and seizures.
Meckel’s diverticulum summary
malrotation summary
Gastroenteritis fluid managment
To prevent the infection spreading, stay at home for 48 hours (school exclusion) until the infection resolves
Constipation summary
If there is impaction (Feacolith: A mass of dry, hard stool that cannot pass out of the colon or rectum) : ERIC disimpaction regime
main diarrhoea causes (summary)
CMPA management:
- 1st Trial cows’ milk elimination from diet for 2-6 weeks:
- Breastfed Babies: mother to exclude cow’s milk protein from her diet
* Consider prescribing daily supplement of 1g of calcium and 10 mcg of vitamin D
* N.B. it takes 2-3 weeks to fully eliminate cow’s milk from breastmilk - Formula-fed Babies: replacement of cows’ milk-based formula with hypoallergenic infant formula (e.g. extensively hydrolysed formula or amino acid formula if SEVERE: use amino-acid based formula:AAF)
- Weaned infants/older children: exclude cows’ milk protein from their diet
- 2nd Regularly monitor growth, nutritional counselling with a paediatric dietician
- 3rd Re-evaluate tolerance to cows’ milk protein (every 6-12 months) re-introduce cows’ milk protein into the diet if tolerance is established, greater exposure of less processed milk is advised with ‘Milk Ladder’
CMPA red flags for referral
Bilious vomiting summary
What does this AXR show? what 4 conditions may cause this radiological appearance?
There is massive hepatosplenomegaly. The enlarged liver and spleen are separate from the renal and psoas outlines. Extensive ascites would obscure the renal and splenic outlines which are clearly seen.
- Leukaemia
- Malaria
- Thalassemia
- Gaucher’s disease
NEC summary
red flag referrals:
red flag referrals:
