Much ado about Meckel’s

Cite this article as:
Peter Tormey. Much ado about Meckel’s, Don't Forget the Bubbles, 2021. Available at:

Robert is a 14-year-old boy who re-presents to ED with a history of rectal bleeding. He has had four or five episodes of passing bright, red blood PR over the last three days, having been a previously well child. He has also had four or five episodes of non-bilious vomiting.

Mum has noticed that he has now become pale and lethargic.

On his first presentation to ED, two days prior, the working diagnosis was bacterial gastroenteritis. His haemoglobin at that time was 99g/L. It has fallen to 45g/L on this presentation.

On examination, he is very pale, his heart rate is 120, and his blood pressure 95/65.

What are your differentials at this point?


  • Bacterial: Campylobacter, Salmonella, Shigella, E. coli, Yersinia, C. difficile
  • Viral: rotavirus, COVID-19


  • Inflammatory bowel disease


Bowel obstruction

  • Intussuception
  • Malignancy


  • Meckel’s diverticulum
  • Anal fissure
  • Haemorrhoids
  • Trauma/NAI

What investigations would you perform?

  • Bloods: FBC, U+E, LFTs, CRP, ESR, VBG, blood culture, coagulation screen
  • Stool culture, stool for C.difficile
  • Covid swab
  • Abdominal ultrasound
  • CT abdomen/pelvis
  • Colonoscopy
  • Meckel’s scan

What is Meckel’s Diverticulum?

You may remember “The Rule of Two’s” from medical school.

MD is a congenital abnormality of the small intestine that is present in 2% of the population. 2% of these people will become symptomatic. It is 2 inches long and 2 feet from the ileocoecal valve. There can be 2 types of ectopic tissue: gastric or pancreatic. There is a 2:1 male preponderance.1,2,3

MD comprises the three layers of the intestinal wall and is, therefore, a true diverticulum.3 It results from the incomplete obliteration of the omphalomesenteric duct. The omphalomesenteric duct connects the yolk sac to the intestinal tract during early foetal life and is usually obliterated by the seventh week of gestation. Failure to regress can result in a spectrum of abnormalities, including: MD, patent vitelline duct, fibrous band, sinus tract, umbilical polyp and umbilical cyst.3

Comparison between vitelline fistula and meckel's

What happens to the symptomatic 2%?

The presentation of MD is highly variable. It is best to consider the different presentations based on the underlying anatomical or pathological processes the diverticulum can undergo.

1. It gets in the way

The abnormal anatomy in MD can lead to intestinal obstruction. In children, this usually presents as intussusception or volvulus.3

The diverticulum acts as the lead point in intussusception. These patients present with abdominal pain. The symptoms can be non-specific, particularly in pre-verbal children. They are “off form”, or parents complain about poor feeding, constipation, abdominal distension. As you can see from Robert’s case, intussusception can also lead to massive GI haemorrhage.

Volvulus of the intestine may occur around the fibrous cord that connects the Meckel’s to the umbilicus.4

2. The ectopic gastric tissue can cause an ulcer

The ectopic gastric mucosa in the diverticulum can secrete acid which results in ulceration of the small bowel. This usually presents as painless bleeding, which can be massive in nature.3 It may also present due to anaemia from chronic bleeding. The bleeding is usually dark red or maroon in colour.3 Robert’s Technitium-99m scan (or Meckel’s scan) was positive, suggesting the presence of gastric mucosa, so he may have intussusception plus ulceration, both leading to his massive GI haemorrhage.

3. It gets angry

As the Meckel’s is a blind ending diverticulum, it can undergo a process of inflammation, similar to appendicitis. Obstruction at the base of the diverticulum leads to bacterial overgrowth and inflammation. This can present with fever, vomiting and abdominal pain, which is often indistinguishable from acute appendicitis. The MD may also perforate, leading to diffuse peritonitis and  a very unwell patient.

As these symptoms are all non-specific, it is important to think of MD as a diagnosis in children presenting with any of the symptoms above.

How is it diagnosed?

MD requires a high index of clinical suspicion to aid diagnosis. Most imaging modalities are non-specific but can still be helpful. X-ray or ultrasound may show a small bowel obstruction and intussusception.5 Finding a normal appendix on ultrasound, may lead to careful consideration of MD as an alternate cause.

A Meckel’s scan may be performed. This is a nuclear medicine scan using Technitium-99m, which accumulates in the ectopic gastric mucosa (see Image 2).5 The test is reliant on the presence of gastric mucosa, which is only present in 4.6-71% of symptomatic MD.5 Premedication with H2 antagonists may increase the accuracy of the scan.5

Radionuclide Meckel scan
Case courtesy of Radswiki, From the case rID: 11598

MD is often only confirmed on exploratory laparoscopy or laparotomy.

How is it treated?

Definitive treatment is surgical resection of the diverticulum, either laparoscopically or by laparotomy. Simple diverticulectomy and closure of the ileum is acceptable except in cases of GI bleeding where the ulcer may extend to the adjacent ileum, in which case segmental resection with re-anastomosis of the small bowel should be carried out.4

Robert’s haemoglobin is 45. He requires multiple transfusions with packed red cells, FFP and fibrinogen. He is stabilized and transferred to PICU.

He has an emergency OGD and colonoscopy. They do not reveal the source of bleeding. His abdominal ultrasound shows a small bowel intussusception, suspicious for Meckel’s diverticulum (MD). He has a Meckel’s scan which confirms MD. He undergoes surgical resection of the diverticulum and recovers well.

Who was Meckel?

Johann Friedrich Meckel (the younger) was a German anatomist whose principle interest was the study of congenital malformation and the developmental aspects of the lungs and bloods vessels.6

Interestingly, MD was first described by Wilhelm Fabricius Hildanus, a German Surgeon, in 1598.7 It wasn’t named, however, until Meckel reported his research on the diverticulum’s anatomy and embryology in 1809.

He is also responsible for the medical eponyms Meckel cartilage and Meckel syndrome.

He is called Johann Friedrich Meckel The Younger because his grandfather was called by the same name and was also an anatomist, as were his father, younger brother and son.

You can find out more about him and the Meckel anatomist dynast on LITFL.

References for Much Ado about Meckel’s

1. Meckel’s Diverticulum [Internet]. [cited 2021 Apr 1]. Available from:

2. Rule of 2s in Meckel diverticulum | Radiology Reference Article | [Internet]. [cited 2021 Apr 1]. Available from:

3. Keese D, Rolle U, Gfroerer S, Fiegel H. Symptomatic Meckel’s Diverticulum in Pediatric Patients—Case Reports and Systematic Review of the Literature. Front Pediatr [Internet]. 2019 Jun 26 [cited 2021 Apr 12];7(JUN):267. Available from:

4. Ivatury RR. Meckel’s diverticulum and the eponymous legend. Vol. 87, Journal of Trauma and Acute Care Surgery. Lippincott Williams and Wilkins; 2019. p. 451–5.

5. Hansen C-C, Søreide K. Systematic review of epidemiology, presentation, and management of Meckel’s diverticulum in the 21st century. Medicine (Baltimore) [Internet]. 2018 Aug 1 [cited 2021 Apr 12];97(35):e12154. Available from:

6. Johann Friedrich Meckel The Younger • LITFL • Medical Eponym Library [Internet]. [cited 2021 Apr 12]. Available from:

Conversations about constipation

Cite this article as:
Chris Dadnam. Conversations about constipation, Don't Forget the Bubbles, 2021. Available at:

Like most of you I have to deal with the issues of constipation within the ED or CAU environment and most of the time it’s not the reason the child attends the department! This can then lead to a series of awkward questions and issues that parents may ask that we need to consider in order to provide safe, useful and most of all, worthwhile advice! 

So let’s go through these questions ask issues:

1. My child has a UTI, why are you talking to me about constipation? 

This is something I get asked not only when a UTI is diagnosed but other clinical conditions including; appendicitis, bedwetting, incontinence, urinary retention, obstruction, etc…. it is important that parents understand the implications of constipation, not only from a pain and symptoms point of view but also the complications surrounding it. Indeed, many parents also struggle to understand how their child, who is rolling around in agony, is only suffering from constipation (you can literally feel them questioning your medical acumen). 

During these tough times I always mention two key points. Firstly, the fact that your bowel covers the majority of your abdomen. A build up of wind and solid matter in the bowels can bring about severe griping abdominal pain, when pressing against sensitive nerves. As it covers a lot of your abdomen, when full, it will compress other structures like your child’s bladder leading to urinary infections, incontinence and retention. The second point is that stools are like a toxin your body wants to expel, when it remains in your bowels it can get into small structures like your child’s appendix and cause it to become inflamed and that leads to appendicitis.

Also, the longer the stools sit in the colon the more water is absorbed leading to harder, solid stools. This will cause a blockage and lead to vomiting and obstruction which may require surgery. 

Once parents have a better understanding of these points they’re less likely to roll their eyes at constipation! 

2. How are they constipated, they go every day? 

This in fairness is a good question, one that used to throw me quite a bit in my early paediatric years, but let’s break it down. Constipation is not simply the length of time between going to the toilet, rather it is the build up and insufficient clearance of stools in your bowels. With this in mind, a child can go daily and pass small amounts of stool but still have a backlog of faecal matter in their bowels. Therefore also question the time spent on the toilet, straining and pain during defecation. These are all signs of constipation. If you can, ask your young patients too! 

3. They already drink plenty of fluids

Don’t let this answer fool you, explore the parents’ meaning of fluids. When we are saying it, we mean clear liquids like water and squash (preferably sugar-free), but for the parents, it’s anything from water and tea, to milk and hot chocolates. Now there is a myth that milk makes us constipated but that’s simply not the case….well not entirely. Milk is indeed a liquid and it doesn’t make us constipated but it’s thicker and takes longer to digest (hence why in surgery they stress only clear liquids 4hrs before). Milk fills up the child and so reduces their intake of solid food which will be full of fibre. 

Always remember, parents may say they’ve cut out all the milk, but this may have been substituted for milky teas and hot chocolates, so double check! 

In terms of managing the milk, appreciate the difficulty the family is about to face. Wean the milk down slowly, starting with the bottles in the middle of the day, then the morning bottle and finally the night bottle. Milk shouldn’t be stopped entirely, having a 250-500ml glass of milk daily is perfectly fine. 

4. They eat a really good diet 

Whenever I get this response, I immediately think they haven’t and 99% of the time I’m right……says a lot. 

Again, this is either due to a misconception as to what a good diet is, or they don’t think it’s an important issue so they simply brush it off with this generic statement so that they can get to the medication that will actually help. Another quick way to check is to just ask the child. They normally find it much more difficult to turn a blind eye.

I always try to tackle this in one of two ways:

Tell me what they eat?

– it’s surprising how many children don’t have breakfast or any of the three square meals a day. If they do, just add in tips when you can.

Breakfast; dried fruits in cereal (especially raisins), don’t switch the cereal completely but rather mix in an all bran, so they’re still getting their tasty favourites, but now with added fibre. Toast – if it’s white bread, freeze it; it’ll keep longer and by placing it straight into the toaster means that the strands of carbs, fats and protein are bound together and form fibre.

Lunch; Try to include salad into sandwiches. If the parents say they don’t like salad then how do they expect their children too! Encourage healthy eating in the parents as well, to form positive connotations for their kids.

Dinner; any sauce can hide a multitude of veggies if blended or chopped fine enough – so get them cooking and where possible get them to encourage their kids to join in. If they cook it themselves, they’ll appreciate the food and, for some reason, enjoy it more……probably a labour of love! And it’s a great time to leave out and pick on a bowl of fresh berries or grapes, the more accessible things are the more they get eaten.

Do they eat all their fruit and vegetables?

This again leads to a classic ‘Yes’ response – which falsely reassures a lot of healthcare professionals. In truth, it’s a vague and rather inadequate question to ask. If I told you that my child eats peeled apples and pears, has a glass of orange juice and then eats loads of veggies which I boil until soft… It might make you think twice about the goodness they’re actually getting.  So I always ask – Do they eat the skins of the fruit and vegetables? How do you prepare them? The skin of most fruits and vegetables holds the majority of fibre along with different vitamins and minerals required. In all honesty, if you are peeling apples and pears, all you’re left with is sugar and water, so I tell parents to give their child the peel instead! 

Again with veggies, I tend to suggest for microwaving or steaming as people tend to overcook them when they boil them. They need a crunch as that equals fibre. Root vegetables (potatoes, sweet potatoes, carrots, butternut squash, celeriac, parsnips) – all these lovely fibre rich foods – contain most of the fibre in their skins. I tell parents to roast them, long and slow – they’ll taste better (caramelizes the sugars in the veg/skin) so children will prefer them! 

Be mindful of smoothies and fruit juices. Yes, they can count for 1 of our 5 a day (soon to be 10 a day) but they can have little to no fibre, especially with the models that separate out the pulp. The pulp is fibre!! Try to get them to have whole fruits instead or 1 x 250ml glass of fruit smoothie a day with the pulp. 

5. I have tried all this and it doesn’t work 

Before you dismiss this answer make sure you look over the medical background again (cystic fibrosis, hypothyroid disease, Hirschsprung’s). Ask these all-important questions:

  • When did they have their very first poo? It should be within the first 48 hours. Then double-check it was a good amount – small smears don’t count.
  • Have they had issues with weight gain and prolonged issues with chest problems (in cystic fibrosis, LRTIs tend to happen towards the end of their first year of life).
  • Did they have a Guthrie / heel prick test. Any developmental delay? A large soft spot on their head?

In all of these conditions, the child would have always had an issue with constipation since birth, so don’t miss them. 

Once covered, it’s important to go through what they have tried….. most parents will only have been given a packet of Movicol and told to get on with it. Look at the summary section to see how to structure a constructive management plan.

6. I’m scared they’ll starve so I give them what they want. 

How many of us have been told this with little Jonny sitting there looking larger than life?

In general, throughout the developed world, children are unlikely to starve if their parents are trying to feed them a healthy balanced diet. There are caveats to this:-

  • Autistic children or children with textural issues. 
  • Children with a background of eating disorders (bulimia or anorexia). 

These children will need extra support and input from community and nutritional teams. 

All the other children will always put up a fight (normally a good one!) but then their bodies will give in and want food. This is an important step for parents to understand, especially when the child is too young to go out and get food themselves. Make sure you tell the parents this won’t be a simple task, and the main reason children normally win, is that carers will be busy and won’t have time to tackle this problem. It’s a quick fix to give them something just so that they know they’ve eaten…….then the habit starts. I always tell parents, wait until you have a week off and prepare yourself/ yourselves for a bumpy ride. Have a united front, it’s no good if one parent plays the ‘strict/ bad guy’ whilst the other literally feeds the problem behind their back. Prepare meals and hide away the unhealthy processed snacks (or don’t buy them in the first place) and leave fruit out. Again, get the child involved in cooking, build a healthy connection with food and make it fun. Children will most likely throw tantrums at the start, but remind them that eventually their child’s body will give in and they will come for food, most likely with a grumpy face. 

Just make sure they’re hydrated with clear fluids! And NO milky substitutes. Remind the parents they’re not bad people and this will help fix things in the long run.

7. Movicol doesn’t work and I don’t want it to make their bowels lazy 

This age-old answer…..makes you wonder why we bother using Movicol? More often than not they’ve not prepared it correctly, despite the instructions being on the box. Honestly, the ways parents use Movicol; sprinkle on cereal, mix into snacks or food, add to tea….the list goes on!

Movicol is only effective when it is bound with water. After this, the parent can then mix it with a small amount of any other liquid or flavouring. Make sure they don’t add it to a litre bottle of squash as the child will have to drink the whole lot. Also, this means they don’t need the flavoured versions (which taste vile – remember when they made you try them in medical school!). 

Another myth is that “it’ll make their bowels lazy.” Explain that Movicol is not a stimulant, it is an osmotic diuretic and acts to drive the water you mixed it with into the child’s stool to make them softer and easier to pass. With this in mind, even stimulant laxatives won’t make your bowels lazy. I always say, they can be on it for the rest of their lives, it’ll never make their bowels lazy – that tends to reassure parents.

8. I tried laxatives before and they suddenly had diarrhoea so I stopped using it. 

This answer may again throw you into thinking that the laxative has done a great job in under 72 hours and fixed a months worth of constipation… it sounds too good to be true?? Well, it is. The big problem here is, if clinicians don’t pre-warn the carers what might happen after starting a laxative, it can lead to long term mistrust in both the medication and in our advice.

I always start off by setting the day to start. Aim to start the laxative at the end of the week, a Thursday or Friday, to avoid accidents in the school. They will deter the child from ever trying them again.

Once we know when to take them, always triple check they’re using them correctly… mix with water first, then add to a small volume of any other liquid for taste.

Finally, but most importantly, the change in stool. Referring to the Bristol stool chart (the only card I carry around!), I explain the child will start with Type1-3 stools. Then, they’ll have what looks like diarrhoea, brown watery smelly stools, but, of course, it’s overflow. Take the time to talk about why this happens; the Movicol is slowly moving through the hard stools, like rain trickling down a wall, in their child’s bowel. The Movicol/water mix will initially run over it but over time their stools start to soften. 

The next step, again important due to risk of pain, is the big logs. And big means big! I’ve had parents say they’ve used shears to cut up these stools in the toilet. This is essentially the wall slowly being emptied out. 

Once this is over, they will finally have the soft mushy stools. The laxative story should not end there! It is important to mention this ‘wall of stool’ has caused the bowels to stretch. This will lead to a build-up of stools again as the child won’t know when they need to go. This is reservoir constipation. It can take months to revert back to normal so I always advise to continue on with the laxatives and reduce (but not stop) the maintenance daily dose down if the child is passing clear watery liquid. Usually, treatment should continue for at least three months to treat reservoir constipation (although in some children it can be longer).

9. They don’t like my cooking. They’re vegetarian now, I’m not. 

It doesn’t have to be vegetarian, of course, this is just what an angry mum said to me once about her daughter. Parents will mention the difficulties of preparing food they’re not used to cooking, I always suggest ‘get your child involved‘. This is the perfect time to do something together (bonus points as well in tackling mental health and isolation. It gets the family talking). The child will appreciate their intent and willingness to give their lifestyle a try, which will build confidence in the relationship as well. They can get a cookbook, go online (it’s all free and easily accessible these days) and adapt their cooking style. Again. this will make their child feel more involved and interested in cooking and eating healthy foods. 

10. They don’t like fruit and vegetables. 

I think we’ve all suffered from hating vegetables and fruit at some point in our lives. I remember hating tomatoes and peppers, so I feel for any parent tackling this problem. 

There are several factors to contend with here:

  • Their child not liking the fruit because it’s unknown to them or feels texturally unsatisfactory.
  • Having access to other more ‘enjoyable’ foods such as biscuits, chocolates and crisps around the house which they can graze on and avoid these unwanted bags of goodness. 
  • Watching older siblings and parents, and copying them.

To this, I normally offer a number of solutions but be mindful that parents will have busy lives around their child’s eating habits so it has to be a conscious effort at a convenient time, like over the weekend or annual leave.  

Firstly, hide unhealthy snacks or simply reduce the amount you buy, what they don’t see they don’t know…. out of sight, out of mind technique.

Secondly, I always tell carers that they and older siblings are role models. If you’re not eating it, why should they? There should be a united front by the parents. 

Finally, get cooking and get your child involved. Any child who cooks will appreciate the food they’ve made and the sense of achievement, even if it doesn’t taste nice, they’ll love it. It’s a great time to chat over a bowl of fresh berries…

My own enthusiasm then tends to kick in. I like to say “Get creative in the kitchen!” I’ve mentioned simple things for example: make flapjacks and throw in lots of dried fruits; raisins, dates, apricots, prunes – all-natural sweetness with skinned nuts & oats. Freeze smoothies with the pulp into ice-lollies. Fruit crumbles with honey and oats… With vegetables, always remember to steam or microwave them, they need the crunch. Again if kids don’t like them…. Chop them up/ blend them and throw them into sauces, pies and mixed into other dishes. If it’s the taste they don’t like, again mixed into gravy or a tasty sauce will fix that problem! 

It is important to mention the importance of a healthy balanced diet. Food is your best medicine. This is can be true for managing many conditions; anaemia , skin problems, poor immunity, nail and hair get my point. A varied diet holds the key to a lot of management plans, and it’s important to mention this even when the child is on supplements. A classic example is the parent says we’ve fixed the iron problem with iron tablets, but they fail to realise, without vitamin C your body can’t absorb the iron through the small intestine. Therefore, they’ll be questioning why their child remains anaemic in months to come. 

11. They’ve gone back into nappies as they’re scared of using the toilet. 

This is an important issue. It’ll mean the child will probably have problems with incontinence which may be affecting their social life such as staying over at a friends houses. Yet another reason why it needs tackling. 

Always start with asking what happened? More often than not it’s a series of bad habits and untimely events that have led to a regression in the family’s good practice. It happens to the best of us. Reassure everyone and give them the positive reinforcement that they’ve identified a problem that needs to be solved. Then offer the advice below. Take your time with these parents, it would have taken a lot for them to come into an acute setting to seek advice, so try to give them some. 

A framework for managing constipation.

This is ultimately an important topic that you have or, no doubt, will see at some point in your paediatric career. Knowing how to manage it is a core skill. 

I always frame my management in 3 steps: 

1. Diet and fluid intake – take the points from the above questions. Ultimately, the parents control the diet and food at home. They, and older siblings, are the child’s role models so what they eat will influence the child’s diet too. Remind them that food is their family’s best medicine so they need to get it right. Cancel out the milky drinks, cook smart & healthy and don’t forget clear fluids.

2. Toileting – our constipation module covers this but key points; get them into a routine (20 to 30mins after dinner – to sit on the toilet). Make the toilet a fun place with all their toys and gadgets and don’t forget to get them to blow the bubbles. Optimise positioning with knees above bums when sitting, using a footstool. 

3. Medication – ensure parents are giving them correctly – mixing with water first then adding to any other liquid for taste. Make sure this isn’t a full bottle, as they’ll have to drink the lot!. Movicol doesn’t make your bowels lazy. If they’re on a disimpaction regime, think about the volume they’ll be drinking each time. It may be better to split it into thrice daily doses instead. Briefly touch on the sequence of stool changes to reduce misconceptions of overflow and diarrhoea. Lastly start Movicol towards the end of the week, Thursday or Friday to avoid accidents at school. 

End with: 

  • Referencing the ERIC constipation website. It’s a great tool for constipation and bedwetting. It talks to the parent and child, so easy to understand and explain.
  • It will take time for things to fall into place. There is no quick fix. There will be tantrums, sleepless nights and days you’ll want to give in. Hang in there and once you’re sorted you’ll wish you’d done it sooner!

Hirschsprung Associated Enterocolitis

Cite this article as:
Peter Tormey. Hirschsprung Associated Enterocolitis, Don't Forget the Bubbles, 2020. Available at:

Michelle is a 5-year-old girl with a background of Hirschsprung’s Disease. She had a pull through procedure performed 6 months ago. She is on daily PR washouts.

She presents to ED acutely unwell with multiple episodes of brown vomiting. On examination she is lethargic, grey in colour and peripherally shut down. Her abdomen is distended.

What is Hirschprung’s Disease?

Hirschprung’s Disease (HD) is caused by the failure of neural crest cells to migrate completely during intestinal development.

The neural crest cells are progenitor cells for the enteric nervous system, which controls peristalsis, blood flow to the gut and secretions. The enteric ganglia are interconnected to form 2 plexi that extend along the length of the bowel: an out myenteric (Auerbach) plexus running the full length of the gut and an inner submucosal (Meissner) plexus, found in the small and large intestine. The absence of these plexi results in an aganglionic segment of colon, which fails to relax, causing a functional obstruction.

Interestingly, the timing of the arrest in migration of the neural crest cells influences the severity of disease. The cells migrate in a craniocaudal fashion, therefore, early arrest results in a longer segment of aganglionosis.

The incidence of HD is 1 in 5000 live births. The male-to-female ration is 4:1 in short segment disease but it is 1:1- 2:1 in long segment disease.

How do children with Hirschprung’s present?

How these children present depends on whether they have short or long-segment disease. Short-segment disease only involves the rectosigmoid colon and accounts for 80-85% of cases. In long-segment disease, the aganglionosis extends proximally to the sigmoid colon.

Those with long-segment disease are more severely affected and tend to present earlier, usually in the first few days after birth. They can present with any of the following features:

  • Abdominal distension
  • Bilious vomiting
  • Delayed passage of meconium
  • Enterocolitis

Those with short-segment disease may present later in childhood with constipation and failure to thrive.

What is “delayed” passage of meconium?

There is a big variation in the timing of first meconium passage in neonates, however, most healthy newborns will pass stool within the first 24 hours. All healthy newborns should pass their first stool within 48 hours.

Clark studied 395 term infants and found that 98.5% of them passed stool within the first 24 hours and 100% within 48 hours.

It is generally acknowledged that term infants who don’t pass stool within the first 48 hours should undergo careful evaluation and investigation. 60-90% of patients with HD will not pass meconium within 48 hours.

The differentials of delayed passage of meconium are listed below:

  • Meconium plug syndrome
  • Meconium ileus
  • Hirschsprung’s disease
  • Anorectal malformation
  • Intestinal atresia
  • Malrotation, volvulus
  • Hypoplastic left colon syndrome
  • Opioid use
  • Hypothyroidism
  • Sepsis
  • Prematurity, low birth weight

Hirschprung Associated Enterocolitis

Hirschsprung Associated Enterocolitis (HAEC) is a serious complication of HD. Patients can present critically unwell with haemodynamic instability, fever, vomiting, explosive diarrhoea and abdominal distension. An explosive release of gas or stool during rectal examination strongly supports a diagnosis of HD.

The incidence of HAEC ranges from 6-60% prior to pull-through surgery and 25-37% after surgery. HAEC can be potentially life-threatening. Swenson was one of the first to report on mortality in HAEC, reporting a mortality rate of 33% after HAEC, compared with 4% in HD patients without EC. The reported mortality in other studies varies greatly from 0 to 39%.

Mortality rates have improved in recent years, most likely due to improved supportive care in PICU and improved surgical expertise. The mortality rate in HAEC is now 1%.

Poor prognostic factors are: HAEC present at diagnosis of HD and postoperative HAEC.

While it can be seen in all children with HD, several features appear to be associated with an increased risk:

  • Trisomy 21
  • Long-segment disease
  • Previous HAEC
  • Post-op obstruction

The cause of HAEC is unknown. Several hypothesis have been proposed:

  • Dysbiosis of the intestinal microbiome
  • Impaired mucosal barrier function
  • Altered innate immune response
  • Bacterial translocation

Mild cases can present with symptoms of viral gastroenteritis, fever, mild abdominal distension and diarrhea. If it is not promptly recognized and treated it can progress to toxic megacolon, which can be fatal. 

It is important to remember that HAEC can also occur in children who have had surgical repair for HD. It is due to obstruction, which can be due to:

  • Retained aganglionosis
  • Transition zone pull-through
  • Dysmotility following pull-through
  • Anastamotic stricture

A high index of clinical suspicion is required to make the diagnosis. Abdominal x-ray is also helpful and usually shows significantly dilated bowel loops and air-fluid levels.

HAEC is an emergency. Prompt treatment is required with IV antibiotics, (e.g metronidazole, gentamicin, amoxicillin) fluid resuscitation and surgical evaluation, which may include rectal washouts or an emergency colostomy.

Which children with constipation do we need to worry about?

Constipation is a very common presentation to ED. When assessing these patients it is important to screen for any underlying abnormalities, including HD.

Constipation, with the following features should raise your suspicions for undiagnosed HD:

  • Neonates
  • History of delayed passage of meconium
  • Chronic, refractory constipation
  • Failure to thrive
  • Presence of other urogenital abnormalities
  • Family history of HD (the risk for a sibling is 200 times higher than the general population, (4% vs. 0.02%)
  • Associated syndromes:
    • Down Syndrome
    • Bardet-Biedl syndrome
    • Cartilage-hair hypoplasia
    • Congenital central hypoventilation syndrome
    • Multiple endocrine neoplasia type 2
    • Mowat Wilson syndrome
    • Smith-Lemli-Opitz syndrome
    • Waardenburg syndrome

How is Hirschprung’s diagnosed?

Abdominal x-ray may show dilated bowel loops, thickened bowel loops or air fluid levels. These findings, however, are non-specific.

Contrast enema is useful to demonstrate the functional obstruction seen with HD, as seen in the film below.

Contrast study demonstrating a stenotic segment in the sigmoid colon with dilation of the descending colon.
Case courtesy of Dr Mohammad Farghali Ali Tosson. From the case rID: 50255

Definitive diagnosis is by rectal suction biopsy.

It is important to consider HD in children presenting to ED with constipation. You should have a low threshold for surgical referral, particularly if they have any risk factors listed above.

How is Hirschprung’s treated?

Treatment involves surgical resection of the aganglionic segment and anastomosis of the normal bowel to the anus, while preserving sphincter function.

Long term complications include:

  • Chronic constipation
  • Incontinence
  • Recurrent HAEC
  • Psychosocial issues

Did you know?

HD was first described by Harald Hirschsprung in 1886. He described 2 children with severe constipation, due to dilation and hypertrophy of the colon.

Lennander in 1900 was the first to suggest that the pathogenesis may be neurogenic in origin. Tittel then demonstrated in 1901 histiological findings indicating aganglionosis of the colon. In 1948 Swenson used motility studies to demonstrate absence of peristalsis in the aganglionic colon.  

The history of Hirschsprung’s Disease is an interesting and colourful one with many false starts and conflicting opinion. If you’re interested in reading more, look no further than this American Academy of Surgeons history of surgery article.

Michelle was fluid resuscitated and commenced on IV antibiotics. She was transferred to PICU due to haemodynamic instability. She was managed conservatively by the surgical team with regular rectal washouts.

Michelle has had several episodes of HAEC previously. This episode, in particular, was life-threatening. Her parents are finding it harder to perform the daily rectal washouts as she gets older. As a result, it was decided to perform an ileostomy to improve bowel management and to try and prevent HAEC.


1. Butler Tjaden NE, Trainor PA. The developmental etiology and pathogenesis of Hirschsprung disease. Vol. 162, Translational Research. Mosby Inc.; 2013. p. 1–15.

2. Congenital aganglionic megacolon (Hirschsprung disease) – UpToDate [Internet]. [cited 2020 Jul 7]. Available from: https://www-uptodate-com/contents/congenital-aganglionic-megacolon-hirschsprung-disease?search=hirschsprung disease children&source=search_result&selectedTitle=1~76&usage_type=default&display_rank=1

3. Haricharan RN, Georgeson KE. Hirschsprung disease. Semin Pediatr Surg [Internet]. 2008 Nov [cited 2020 Jul 20];17(4):266–75. Available from:

4. Ryan ET, Ecker JL, Christakis NA, Folkman J. Hirschsprung’s disease: Associated abnormalities and demography. J Pediatr Surg [Internet]. 1992 [cited 2020 Jul 20];27(1):76–81. Available from:

5. Clark DA. Times of First Void and First Stool in 500 Newborns [Internet]. Vol. 60, PEDIATRICS. 1977 [cited 2020 Jul 28]. Available from:

6.Loening-Baucke V, Kimura K. Failure to Pass Meconium: Diagnosing Neonatal Intestinal Obstruction. Am Fam Physician. 1999 Nov 1;60(7):2043.

7. Gosain A, Frykman PK, Cowles RA, Horton J, Levitt · Marc, David ·, et al. Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis. Pediatr Surg Int. 2017;33:517–21.

8. Swenson O, Davidson FZ. Similarities of Mechanical Intestinal Obstruction and Aganglionic Megacolon in the Newborn Infant. N Engl J Med [Internet]. 1960 Jan 14 [cited 2020 Jul 28];262(2):64–7.

9. Vieten D, Spicer R. Enterocolitis complicating Hirschsprung’s disease. Semin Pediatr Surg. 2004 Nov 1;13(4):263–72.

10. Murphy F, Puri P. New insights into the pathogenesis of Hirschsprung’s associated enterocolitis. Pediatr Surg Int [Internet]. 2005 Oct 30 [cited 2020 Jul 28];21(10):773–9. Available from:

11. Badner JA, Sieber WK, Garver KL, Chakravarti A. A genetic study of Hirschsprung disease. Am J Hum Genet [Internet]. 1990 [cited 2020 Jul 20];46(3):568–80. Available from: /pmc/articles/PMC1683643/?report=abstract

12. Hirschsprung disease • LITFL • Medical Eponym Library [Internet]. [cited 2020 Jul 20]. Available from:


Cite this article as:
Angharad Griffiths. Gastroenteritis, Don't Forget the Bubbles, 2020. Available at:

Conor is a 10 kg, 13 month-old boy who’s presented to the ED with a 24-hour history of diarrhoea and vomiting.  He has had 5 episodes of non-bloody, non-bilious vomits. Since waking up this morning has two episodes of loose/watery non-bloody malodorous stools. They have not ‘flooded’ the nappy but were quite large.  He is taking sips of fluid (mixtures of water, milk, and juice being offered) and has only eaten half a digestive biscuit so far today.  He has had a fairly large wet nappy last night, but not since, though it’s now difficult to tell as his last nappy was dirty.  He is alert and looking around while being carried but is upset on leaving his mother’s arms.  He cries with tears, has a normal heart rate but his mother is worried about his dry lips.  She was told by a healthcare worker neighbour that he will “need a drip”. CRT, HR, and BP are normal.  His temperature is 37.8.  His nappy is dry and has been on for 3 hours now.  His capillary glucose measurement is 3.2.  You decide he’s probably mildly dehydrated.


Gastroenteritis (GE) is the presence or diarrhoea or vomiting (or both) that may or may not be accompanied by fever, abdominal pain and anorexia.  Diarrhoea is the passage of excessively liquidy or frequent stools with liquid high water content.  Although often felt to be a common minor illness presentation, it is a major cause of childhood mortality and morbidity, causing millions of deaths worldwide in children in low and middle-income countries; of all child deaths from gastroenteritis 78% occur in Africa and South-East Asia. 

Gastroenteritis accounts for a huge proportion of GP and ED presentations. In Europe, acute gastroenteritis the third commonest cause of hospital admission, accounting for between 4-17% of admissions.  In Australia, gastroenteritis caused by rotavirus alone accounts for 115,000 GP visits, 22,000 ED visits and 10,000 hospital admissions a year, with an estimated cost of 30m Australian Dollars (£12m, €18m).  In the UK, 20% of GP consultations in the under 5’s are for GE.

It is imperative that the child with gastroenteritis is differentiated from more sinister causes of vomiting.  The presence of diarrhoea is reassuring but doesn’t exclude other intra-abdominal causes.  The same can be said for pain out of proportion with gastroenteritis, distension, peritoneal signs or localised tenderness.

Most cases are not associated with complications but when complications do occur, the commonest are electrolyte disturbance and metabolic acidosis.  Supplementary fluids through oral or intravenous routes are the most effective way to avoid these complications.

Gastroenteritis in low and middle-income countries can present differently, has different aetiologies, is often managed differently, and is a larger burden to healthcare systems in general than in high-income countries.  This post will focus on gastroenteritis in high-income countries. For more information about comparisons of guidelines across the world; Vecchio et al (2016) is an interesting read.

This is not meant to provide a clinical practice guideline; rather an overview of the illness.  Many (if not all!) paediatric emergency departments or general paediatric units have their own guidelines.


Worldwide, the commonest causes are viral pathogens, most commonly rotaviruses and noroviruses.  Viral infections cause damage to the small bowel enterocytes with resultant low-grade fevers and watery diarrhoea – classically without blood.  Rotavirus strains are seasonal and vary within different geographical areas.  The peak age for these infections is between 6 months and 2 years.  Children with poor nutrition are at higher risk of acquiring gastroenteritis and developing dehydration and complications.

Children with bacterial gastroenteritis are more likely to have bloody stool.
Escherichia coli and Shigella dysenteriae can be complicated by haemolytic uraemic syndrome (HUS).  This is an acute onset, microangiopathic haemolytic anaemia, thrombocytopaenia, acute renal impairment and multisystem involvement.  (Just to confuse things, HUS can present in the absence of bloody diarrhoea.)

Pathogens can be generalised into four groups:

  • Viral (70% of cases): Rotavirus, Norovirus, Adenovirus, Enterovirus
  • Bacterial (10-20% of cases): Campylobacter jejuni, Salmonella spp, Escherichia coli, Shigella spp, Yersinia enterocolitica.
  • Protozoa (unusual, accounting for <10%): Cryptosporidium, Giardia lamblia, Entamoeba histolytica
  • Helminths (very unusual): Strongyloides stercoralis


Pathogens are spread mainly via the faeco-oral route, acquired by ingesting contaminated food or drink.  Water may be contaminated with bacteria, viruses, or protozoa. Undercooked (or inappropriately stored/cooked) meats and seafood are common culprits of bacterial pathogens.  Bacterial contaminants can produce toxins (e.g. Bacillus cereus in re-warmed rice or Staphylococcus aureus in ice-cream).

Pathogens causing gastroenteritis can also be transmitted without the patient being symptomatic.


Gastroenteritis is a clinical diagnosis.  Enquire about sick/infectious contacts and potential sources (recent travel, food).  Enquire about the frequency of symptoms and intake of fluids.  Note the frequency of urination.  Note other things that may cause diarrhoea e.g. recent use of enteral antibiotics or chronic constipation with overflow diarrhoea the presenting feature. 

In the presence of signs such as high fever, long duration of symptoms, severe abdominal pain or bilious vomiting; review the diagnosis and do not immediately label as gastroenteritis.

Oral hydration fluids

Most children are not dehydrated and can tolerate oral fluids and so can be managed at home.  Take a look at Nikki Abela’s DFTB19 talk on top tips for a high yield dehydration assessment.

When children are only mildly to moderately dehydrated, as a general rule they can be treated with oral / enteral rehydration with low osmolality oral rehydration solution (ORS).  Worldwide, ORS is recognised as first line therapy and treating mild to moderate dehydration with enteral rehydration is supported by the WHO, European Society for Paediatric Gastroenterology and the American Academy of Paediatrics. The WHO recommends a low osmolality (hypo-osmolar) solution, usually containing sodium, potassium, chloride, carbohydrate (glucose) and a base.  Low osmolarity solutions reduce the need for IV fluids, reduce stool output and reduce vomiting frequency.

But… a major limitation to the use of ORS is its taste – and this is where apple juice comes in. For minimally dehydrated patients, half-strength apple juice is associated with fewer treatment failures compared to ORS and could suit as a more palatable alternative.  Take a look at a sweet summary (pun intended!) of the “apple juice trial”.

Breastfeeding should continue and a child can be supplemented with ORS if this is needed.  Children can go back to a normal diet after the illness has passed.

Enteral (oral / NG) versus IV hydration

Most studies show that enteral rehydration with ORS is just as effective as IV hydration in mild to moderate dehydration with a 2006 Cochrane analysis concluding that enteral rehydration is as effective if not better than IV rehydration with fewer adverse events and a shorter hospital stay.  It is also less invasive (even with NG placement) and anecdotally satisfaction is greater amongst parents.  It is very safe.

Enteral rehydration only fails in approximately 1 in 20-25 children.

Barriers to oral rehydration include unfamiliarity with the benefits, misconception that it takes longer than IV therapy, and that it has a high failure rate.

Contraindications to enteral rehydration include haemodynamic instability, abdominal distension, concern over ileus, absent bowel sounds, or impaired airway reflexes.

IV therapy is more invasive and involves placing and maintaining IV access.  There are also iatrogenic complications including electrolyte disturbance should inappropriate fluids / composition / volume / rate be used. 

But… in severely dehydrated children, put away the ORS and apple juice. They will need IV rehydration as first line.


How can we support enteral fluids? Well, children who receive Ondansetron are less likely to vomit, have greater oral intake and are less likely to require IV hydration.  A Cochrane review demonstrates that Ondansetron also increases the proportion of children who stop vomiting when compared to placebo [RR1.4] and reduces the proportion of children needing IV therapy (and therefore admission rate) [RR 0.41].  Median length of stay is also shorter in the ED. 

Reported side effects are rare with very few reported side effects other than a few cases of increased frequency of diarrhoea.

Antiemetics alleviate vomiting by acting on the ChemoReceptor Trigger Zone and vomiting centre.  Ondansetron is a 5HT3 receptor antagonist.  This class of antiemetics have fewer adverse effects (than dopamine antagonists, anticholinergics, antihistamines and corticosteroids) and can be safely used in children.  The NICE guideline discusses its off-licence use (at time of publication it’s licence was for post-operative nausea and vomiting and chemotherapy induced vomiting).

Ondansetron prolongs the QT interval.  Recommendations are it should be avoided in those with long QT and should be used in caution where there may be electrolyte imbalance (severe dehydration) or on other QT-prolonging medication.

Ondansetron is relatively cheap  £1.71 for 10 4mg tablets and is available in oro-dispersible form (though these are much more expensive at £36 for 10x4mg tablets) and liquid (£36.82 for 40mg [50ml] bottle).


An ESPGHAN working group position paper on the use of probiotics in acute paediatric gastroenteritis concludes that:

  • Effects seen in clinical trial are probiotic strain specific (this makes ‘trial-life’ difficult to replicate in ‘real-life’).
  • A lack of evidence now doesn’t mean that there won’t be evidence sometime in the future. 
  • Safety profile of certain strains cannot be extrapolated to other strains.
  • Studies that report benefits in certain doses in certain settings have insufficient evidence to support a health benefit at lower doses and different setting.

…the jury’s still out.

Other therapies

Antibiotics and anti-diarrhoeal agents aren’t routinely recommended in the management of paediatric gastroenteritis.

For gastroenteritis in high income countries, the WHO does not recommend adding zinc to a treatment regimen (it is for gastroenteritis in low and middle income countries). 


Routine lab testing in mild and moderate gastroenteritis is of little value in these patients and should be avoided unless clinically indicated.

This goes for stool samples too.  Stool cultures are not routinely indicated in immunocompetent children with non-bloody diarrhoea.

Confirmation of viral gastroenteritis after the child has been discharged from the ED, and likely on the road to recovery at home, adds very little to (A) the clinical diagnosis of viral gastroenteritis in the ED, (B) the management plan and (C) the clinical outcome. 

Should the investigation influence management, then stool sampling may be of benefit.  This could be applicable where an outbreak may be suspected in school or creche, where there may be a public health benefit.

Stool samples should be sent in cases of bloody diarrhoea, immunodeficiency and recent foreign travel.

How about tests for dehydration? Sadly there is no one test that correlates clinically with dehydration. Urine specific gravity in infants is unreliable because the kidney reaches adult concentrating abilities after the age of 1.  Also, the child often doesn’t begin urinating until rehydration has begun.

And glucose? Well, almost 10% of GE patients aged 1 month to 5 years in high income countries present with hypoglycaemia.  Risk factors for hypoglycaemia on presentation include a longer duration of vomiting and increased frequency of vomiting.  It would be reasonable to consider point of care glucose testing at triage for young children as identifying hypoglycaemia on clinical ground alone is difficult in this age group. 


The key to reducing the burden (and generally for an all-round happier life!) is in the prevention of acute gastroenteritis.  Rotavirus vaccination is now commonplace thought the antibodies, the UK & Ireland and other countries around the world.  It is very effective.

In the home and in the ED…Handwashing, handwashing, handwashing!

Vaccination leads to a profound reduction in presentations and admissions and a fall in overall seasonal workload, often within the first year after the introduction of universal vaccination against rotavirus.  Even though only those under 1 year old are generally vaccinated, it has been shown to contribute to a significant herd effect with fewer cases than expected in older children. In Scotland, where initial vaccine uptake was 93- 94% during the first 2 years, annual rotavirus confirmed gastroenteritis cases fell by 84.7%, bed days reduced by 91% (from 325 to just 29), without any documented cases of intussusception.  Reductions were seen across all age groups despite only infants receiving the vaccine.  Similar results can be seen in other areas of the UK and Ireland.

The not to miss bits

  • Do not assume isolated vomiting in a child is gastroenteritis.  Consider other causes -these very widely from inborn errors of metabolism to diabetes mellitus, surgical obstruction to urinary tract infections. If you’d like to hear more, check out Dani’s talk on vomiting in children in DFTB Essentials.
  • Beware chronic diarrhoea in an infant – do they have malabsorption or is this a presentation of IBD or an immunodeficiency?
  • Beware the non-thriving child with diarrhoea.
  • And beware chronic diarrhoea.

But what happened to Conor?

Conor was given a cup of Dioralyte ORS and his favourite beaker filled with Dioralyte.  His mum was encouraged to give him syringes of 5 mls of Dioralyte frequently or for him to take sips from his beaker and was asked to document on a piece of paper how many he received.  He vomited after 30 minutes of this therapy.

You give him a dose of Ondansetron and place an NG tube and give him 100mls (10ml/kg) over 1 hour after deciding he does not need rapid rehydration but slightly more than normal maintenance.  He then receives maintenance volumes of Dioralyte via his NG, which he tolerates well and then starts to take his own sips from his beaker.

He does not vomit in the ED again, has one episode of loose stools, passes urine, and is tolerating fluids orally.  He’s smiling at you! You feel he can be discharged and council his mum regarding regular fluid intake, choice of fluids, of any red flags, and encouraged to return in the event of any concern.

Conor’s Dad calls to say that Conor’s 3 year old sister at home is now vomiting too!  But it’s OK – He’s not too worried about her and Conor’s Mum has advised his Dad to start giving her regular sips of Dioralyte at home…


Colletti JE, Brown KM, Sharieff GQ, Barata IA, Ishimine P. The Management of Children with Gastroenteritis and Dehydration in the Emergency Department. J Emerg Med [Internet]. 2010;38(5):686–98. Available from:

Elliott EJ. Acute gastroenteritis in children. Br Med J. 2007;334(7583):35–40.

Vecchio A Lo, Dias A, Berkley JA, Boey C, Cohen MB, Cruchet S, et al. Comparison of Recommendations in Clinical Practice Guidelines for Acute Gastroenteritis in Children. Gastroenterology. 2016;63(2):226–35.

Freedman SB, Willan AR, Boutis K, Schuh S. Effect of dilute apple juice and preferred fluids vs electrolyte maintenance solution on treatment failure among children with mild gastroenteritis: A randomized clinical trial. JAMA – J Am Med Assoc. 2016;315(18):1966–74.

BK F, A H, JC C. Enteral vs Intravenous regydration therapy for children with gastroenteritis: A meta-analysis of randomized controlled trials. Arch Paediatr Adolesc. 2004;158(1):483–90.

Hartling L, Bellemare S, Wiebe N, Kf R, Tp K, Wr C, et al. Oral versus intravenous rehydration for treating dehydration due to gastroenteritis in children (Review). 2006;

Fedorowicz Z, Jagannath V, Carter B. Antiemetics for reducing vomiting related to acute gastroenteritis in children and adolescents. [Internet]. Cochrane database of systematic reviews. 2011. Available from:

NICE. Management of vomiting omiting in children and y young oung people with gastroenteritis : ondansetron. NICE GUIDELINES. 2014. p. 1–20.

Szajewska H, Guarino A, Hojsak I, Indrio F, Kolacek S, Shamir R, et al. Use of Probiotics for Management of Acute Gastroenteritis : A Position Paper by the ESPGHAN Working Group for Probiotics and Prebiotics. 2014;58(4):531–9.

Forrest R, Jones L, Willocks L, Hardie A, Templeton K. Impact of the introduction of rotavirus vaccination on paediatric hospital admissions , Lothian , Scotland : a retrospective observational study. 2017;323–7.

MARLOW RD, MUIR P, VIPOND I, TROTTER CL FA. Assessing the impacts from the first year of rotavirus vaccination in the UK. Arch Dis Child. 2015;100(Supl 3):A30.

Constipation Module

Cite this article as:
Team DFTB. Constipation Module, Don't Forget the Bubbles, 2020. Available at:
AuthorRebecca Paxton
Duration30-60 mins
Equipment requiredNone
  • Basics (10 mins)
  • Main session: (2 x 15 minute) case discussions covering the key points and evidence
  • Advanced session: (2 x 20 minutes) case discussions covering grey areas, diagnostic dilemmas; advanced management and escalation
  • Quiz (10 mins)
  • Infographic sharing (5 mins): 5 take home learning points

We also recommend printing/sharing a copy of your local guideline.

Billy is an otherwise well 4 year old boy who presents to A&E with a 4 week history of abdominal pain. His pain comes and goes, and seems to be worse after eating. Today he has been doubling over with pain and crying inconsolably.

He has had no fevers or vomiting. He is drinking well but parents think he is a bit off his food. His last poo was 3 days ago, and parents think it was normal but aren’t sure.

What else would you like to know?

What would you look for on examination?
How would you treat Billy?

When should he be seen again?

What is your next step if he doesn’t respond to your treatment?

Red flags – make sure learners have thought to exclude red flags in their history and examination. These include:

  • History of delay more than 48hours in passing meconium
  • Ribbon stools
  • Faltering growth
  • Abdominal distension and vomiting
  • Abnormal anatomical appearance of the anus
  • Severe abdominal distension
  • Abnormal motor development
  • Abnormal gluteal muscles or sacrum
  • Spine or limb deformity (including talipes)
  • Abnormal power, tone or reflexes
  • Safeguarding concerns
  • Ensure external examination of anus for haemorrhoids/fissures that may need treatment 
  • Treatment – assess Billy for signs of impaction and start disimpaction regime if indicated. Discuss non pharmacological treatments.
  • Counselling – prepare parents for duration of treatment, possible side effects and importance of adherence
  • Follow up – prompt and regular follow up, tailored to the families needs
  • Treatment failure – discuss reasons for treatment failure, methods to tackle common problems
  • Red flags
  • Not responding to treatment after 3 months (thyroid, coeliac, allergy) 
  • Failure to thrive
  • Safeguarding concerns

Jakob is a  9 day old baby boy who is brought to the emergency department with vomiting. He is mum’s 3rd baby. Mum is worried that he is vomiting everything he drinks, and is sleepier than she would expect. He seems distressed when awake. He is having 3-4 light wet nappies per day but has only passed a few small stools in his short life.

What else would you like to know? 

What would you look for on physical exam?

Would you order any investigations?

What is your initial management?

  • Red flags on history – delayed passage of meconium and bilious vomiting
  • Examination- look for abdominal distention, careful examination of external genitalia and anus. Document weight and weight loss.
  • Discussion of PR examination – should only be performed by experienced practitioner. May result in forceful expulsion of gas/stool (highly suggestive of Hirschsprung’s). 
  • Investigations – order in consultation with surgical team. Consider abdominal XR to assess for obstruction but keep in mind the surgical team will likely perform contrast study. Rectal biopsy (under surgeons) for definitive diagnosis. 
  • Initial management – resuscitation. NG tube and IV fluids, correction of any electrolyte abnormalities. Look for signs of sepsis (enterocolitis).

Lily is an 8 year old girl with Trisomy 21. She had an AVSD repair as an infant, and is otherwise well and takes no medications. She has been referred to A&E by her GP with worsening constipation. She has been constipated on and off for most of her life, but this has usually been easily managed with movicol. This time around, she has been constipated for 3-4 months and is passing painful, hard stools approximately once per week. Her GP started her on movicol 3 months ago, which parents say she has been happily taking but it doesn’t seem to be working. 

What else would you like to know?

What investigations would you order?

What do you think might be going on?

How would you treat Lily?

T21 and constipation. Constipation is very common in Trisomy 21. Most often it is not due to an underlying disease, but a combination of low muscle tone, decreased mobility and/or a restricted diet. However, T21 is associated with an increased risk of autoimmune disease, including thyroid dysfunction, diabetes and coeliac disease – all of which might cause constipation.

Investigations can be done in an outpatient setting, in this scenario should be followed up by a community paediatrician. Screen for all of the above.

Laxative treatment is unlikely to be entirely effective until the underlying problem is corrected. However, depending on the severity of symptoms treatment escalation is appropriate. Lily doesn’t have any symptoms if impaction, but it may be worth escalating her movicol dose or considering the addition of a stimulant laxative whilst awaiting test results.

Advanced Case 2 (20 minutes)

Georgie is a 12 year old girl with severe autism. She is non verbal. She is otherwise well, but has had trouble with constipation in the past. Her parents attribute this to her being a “picky eater”. Georgie has had abdominal pain for the last 2 weeks, and has been passing small, pellet – like stools every 4-5 days. She has been having more “accidents”, and has been back in nappies for the last 7 days. She has been seen by the GP who has diagnosed constipation and prescribed movicol. She took this as prescribed for the first couple of days, but she is now refusing her medications. Over the past 4 or 5 days, Georgie has begun to refuse all food and will only drink sips of juice with a lot of encouragement. When parents try to give her medications or take her to the toilet, Georgie becomes very upset and aggressive. Her parents are very distressed and not sure what to do.

What are your management options for Georgie? 

Children on the autistic spectrum are more likely to have problems with constipation. Often this is due to a restricted diet, but may also be due to increased levels of anxiety around toileting.

  • Georgie requires disimpaction and this is not being achieved despite the best efforts of the family. There is no right approach to this scenario. Options include
    • Optimise setting and motivators for toileting
    • Change/optimise medications – try mixing movicol into juice, try changing to lactulose, add stimulant laxative
    • Admission for washout – nasogastric tube for washout +/- enema. Strongly consider sedation
    • General anaesthetic for manual disimpaction + washout
  • Support parents and empower them in decision making process
  • Involve multidisciplinary team – community supports will be important on discharge

Macrogol laxatives may cause “lazy bowel” if used for more than 2 months. True or false?

The correct answer is false.

There is some evidence of patients developing dependence on stimulant laxatives if used long term. However, macrogols are safe to use indefinitely without complication.

Which of the following is NOT supportive of a diagnosis of idiopathic constipation?

A: Loss of appetite

B: Ribbon like stools 

C: Urinary incontinence

D: Faecal incontinence

The correct answer is B.

Ribbon like stools suggest an anorectal malformation, and any history of this warrants further investigation. Loss of appetite, urinary and faecal incontinence can all be the result of constipation or faecal impaction. 

In a child with abdominal pain, the diagnosis of UTI makes constipation less likely. True or false?

The correct answer is false.

Constipation can lead to urinary retention and UTI, and as such the two can, and often do, co-exist.  A positive urine dip or culture doesn’t rule out constipation as a cause of abdominal pain. Don’t forget to think about constipation in the child with a history of recurrent UTI. 

National Institute for Health and Care Excellence. Constipation in children and young people. London: NICE, 2014. Available at 

The Royal Children’s Hospital. Clinical practice guideline on constipation. Melbourne: RCH, 2017. Available at

Zeevenhooven J, Koppen IJ, Benninga MA. The new Rome IV criteria for functional gastrointestinal disorders in infants and toddlers. Pediatr Gastroenterol Hepatol Nutr 2017;20(1):1–13.

Sampaio C, Sousa AS, Fraga LGA, Veiga ML, Netto JMB, Barroso Jr U. Constipation and lower urinary tract dysfunction in children and adolescents: a population-based study. Frontiers in pediatrics 2016;4:101.

Youssef NN, et al. Dose response of PEG 3350 for the treatment of childhood fecal impaction. Journal of Pediatrics. 2002;141(3):410-4

Please download our Facilitator and Learner guides

Top 5 Papers in PEM

Cite this article as:
Tessa Davis. Top 5 Papers in PEM, Don't Forget the Bubbles, 2019. Available at:

This post is based on a talk I presented at the RCEM Spring Conference in April 2019 – Top 5 papers in PEM.

Kylie and Jason are enjoying their time at home with their first baby. The highs of being new parents is at its peak and true sleep deprivation is yet to set in. Jayden is two weeks old and is simply perfect. They spend hours staring at him each day marvelling at the perfect human they have created. 

As we follow Jayden through his journey to adulthood, we’ll encounter some common paediatric problems. The 5.5 papers I have chosen were selected because: they cover common presentations; they use large patients groups; and they were conducted by well-respected and highly regarded research groups. But back to our story…

 One night Jayden seems a bit more unsettled than normal. When they check his temperature it’s 38.4. They get in the car and bring Jayden to ED

 Febrile neonates are a huge source of concern – we know that they can deteriorate quickly and we usually err on the side of caution by doing a full septic screen, IV antibiotics, and admission. Actually many of these babies don’t have a serious bacterial infection. Is there a way to tell which ones do?

When you see Jayden in your ED, you ask yourself is…should I do a full septic screen?

Paper 1 - Kupperman et al, 2019, A clinical prediction rule to identify febrile infants 60 days and younger at low risk for serious bacterial infections, JAMA Pediatrics

This paper aimed to derive and validate a highly accurate prediction rule to identify infant at low risk of SBI. The patients were febrile infants 60 days and younger (who had a rectal temp of >38 in the ED or a fever at home within the preceding 24 hours)

They excluded those who were critically ill, who had antibiotics in the preceding 48 hours, those born premature, and those with other medical conditions.

There were 1821 febrile infants included.

The authors considered clinical suspicion of SBI. They then look at various markers: blood culture; urine culture and urinalysis; CSF; FBC; and procalcitonin levels. The outcomes  considered were serious bacterial infection – that is bacterial meningitis, bacteraemia, or urinary tract infection.

Overall, the rates of SBI in this group was 9%. The authors formulated a rule with a very high sensitivity (97.7%) for identifying those at low risk of serious bacterial infection. They were low risk if they fulfilled three criteria:

  • negative urinalysis
  • neutrophil count of less than 4/mm3 
  • procalcitonin of less than 0.5ng/ml

61.3% of their patient group were low risk.

Interestingly their low risk rule does not include use of  lumbar puncture67.4% of the low risk group had a lumbar puncture that would not have been necessary.

Key take away: There may be some febrile neonates that are low risk, and therefore we could avoid a lumbar puncture and full work up. In practical terms, this is unlikely to change our practice at the moment. Many of us cannot send a procalcitonin in the ED, and we might have to wait several hours to get a neutrophil count back. However this does bode well for the future in identifying which of these well febrile neonates are low risk.

Jayden does get a full septic screen. He has IV antibiotics for 48 hours and remains well. His blood cultures are negative so his antibiotics are stopped and he is discharged.




Jayden is growing well. At 7 months of age, he is looking great and developmentally normal. Dad, Jason, smokes, but reassures you that he never does so in the house. Jayden develops a cough and two days later starts breathing very quickly and noisily. They head to the emergency department.

Jayden has bronchiolitis. This is very common and your departments and wards have no doubt been filled with these children over the winter. We know that little works with these children. So you force yourself to hold back the ‘trial of salbutamol’ because it won’t make any difference.. But high flow does seem to be the one thing (along with oxygen) that might make a difference.

You ask yourself the question...should I start high flow?

Paper 2 - Franklin et al, A Randomized Trial of High-Flow Oxygen Therapy in Infants with Bronchiolitis. NEJM. 2018. 378(12):1121-1131

This study looks at infants under 12 months old with a clinical diagnosis of bronchiolitis and a need for supplemental oxygen. 1472 were included (after exclusions). Patients were excluded if: they had an alternative diagnosis; they had cyanotic heart disease; or they were on home oxygen.

Patients were randomised to either high flow or low flow. The high flow group were given heated humidified high flow oxygen – 2L/kg/min via Optiflow. The oxygen was then weaned to achieve target saturations, and they were taken off high flow once they had been on air for four hours. The low flow group were given wall oxygen via nasal cannulae at 2L/min max.

The outcome  was escalation of care. This meant who in the low flow group was escalated to high flow, and who in the high flow group was escalated to BiPAP or was intubated. Treatment failure was based on: an increase in heart rate; if the respiratory rate increased or didn’t drop; if they were needing oxygen in >2L/min of flow or >0.4 FiO2 to maintain their saturations; or if they achieve a high early warning score. Clinicians could also escalate care themselves (34% were escalated in this way).

Escalation of care occurred much more commonly in the low flow group – with 12% being escalated in the high flow group and 23% in the low flow group.


Interestingly there was no difference in the length of stay between the two groups.

Key take away: High flow does reduce the need for escalation. Escalation itself is significant – it requires increased nursing attention for low flow patients while they are transferred onto Optiflow.  There may be less medical staffing on the wards if the child deteriorates on high flow overnight. Although they aren’t comparing like with like, escalation itself is an important clinical event. They also demonstrated that high flow does not increase the number of adverse events (for example there was no difference in the number of pneumothoraces between the groups). High flow is safe to use and we should consider starting it early in ED.

You start Jayden on high flow in ED and he stabilises. 12 hours later he is weaned off on the ward and is discharged the following day.


Jayden is now a healthy 3 year old boy. He loves Paw Patrol.  He hates vegetables and won’t eat any food that is the colour green or yellow. Kylie and Jason are expecting their next child, and Jason has finally quit smoking. Unfortunately Jayden is prone to wheezy episodes and now has his very own inhaler which he hates using. The change in weather in London, from quite cold to…colder, seems to have triggered something and he’s now pretty wheezy and short of breath. They head into their favourite emergency department.

 Jayden is now firmly in the realm of viral-induced wheeze. Yes, it’s all on a spectrum, but he’s now 3 years old with an inhaler. You asses him and think he should have a salbutamol burst.

As you are writing the salbutamol up, your SHO asks  you – should I give him steroids?

Paper 3 - Foster SJ, Cooper MN, Oosterhof S, Borland ML. Oral prednisolone in preschool children with virus-associated wheeze: a prospective, randomised, double-blind, placebo-controlled trial. The Lancet Respiratory Medicine. 2018 Jan 17.

 This paper aimed to assess the efficacy of oral prednisolone in children presenting to an ED with viral wheeze.

The patients included were 2-6 years old. They were excluded if: saturations were less than 92% in air; they had a silent chest; they had sepsis; there was a previous PICU admission for wheeze; they had prematurity; or they had recently had steroids.

605 patients were included and they were randomised to receive either prednisolone or placebo. The prednisolone group received 1mg/kg prednisolone once a day for three days. The placebo group received a placebo medication (matched for volume and taste to prednisolone) once a day for three days.

Patients were assessed for their wheeze severity using a validated pulmonary score.

The outcome measures were length of stay (until clinically fit for discharge). They also considered re-attendance, readmission, salbutamol usage, and residual symptoms.

The results are tricky to interpret. Those who were discharged from ED within four hours did not benefit from prednisolone. However there may be some benefit in the mild to moderate wheeze group, and some in those who used salbutamol at home prior to presenting to ED. Interestingly this paper did not support our previously held belief that those children with atopy respond better to prednisolone.

 Key take homes: Some pre-schoolers are steroid responsive, but identifying which ones is a challenge. As Damian Roland discusses here, it is likely that we are seeing lots of children presenting with the same symptoms (wheeze) but with different pathology behind it. Once we can identify the pathology we can start to target specific groups of patients with management that works.

You decided not to give Jayden prednisolone and after his salbutamol burst he stretches to 4 hours and is discharged home.


Jayden is 5 years old and in his excitement of building the new Hogwarts Lego castle he accidentally swallows a Lego head. Kylie and Jason aren’t sure whether to worry or not? So they take him into ED.

Children ingesting random objects is a common presentation to ED.

When you see Jayden in the department, his parents ask you…should I search through his poo?

Paper 3.5 - Tagg, A. , Roland, D. , Leo, G. S.Y., Knight, K. , Goldstein, H. , Davis, T. , DFTB, (2018), Everything is awesome: Don’t forget the Lego. J Paediatr Child Health. doi:10.1111/jpc.14309

Myself and 5 of my fearless, and brave, paediatric colleagues swallowed a Lego head each to see how quickly it passed. The paper was generously published in the Journal of Paediatrics and Child Health.

To ensure serious scientific rigour, we put together some scoring systems.

The Stool Hardness and Transit time (the SHAT score) took into account how hard our stools were, and whether that impacted (no pun intended) on the time to retrieve the Lego head.

And out main outcome was the Found And Retrieved Time (the FART score). This was the time to get our Lego heads back, and the average FART score was 1.71 days.

Unfortunately one of the six of us didn’t find his Lego head. After valiantly searching through his own faeces for two weeks, he gave up. And it may still be up there.

Key take home: Don’t search through poo, it’s gross.

Jayden heads home happily to finish building his Lego Castle.


Jayden is 6 years old. He thinks Paw Patrol is for losers. Fortunately he still loves Lego and Harry Potter. He also enjoys climbing. Unfortunately, two days ago he fell off the ladder coming down from his bunk bed. He seemed okay at the time, and Kylie and Jason had other plans that evening, so they decided to keep him at home. Now, two days later, he has a massive egg on his head and has been complaining of a headache. He also vomited yesterday. They bring him to ED.


We have fabulous head injury guidance for kids thanks to PECARN, CHALICE, and CATCH. But actually PECARN and CATCH specifically exclude injuries more than 24 hours old, and CHALICE doesn’t publish data on this group. So, for Jayden you need to put the NICE guideline away because it doesn’t apply. This is a common grey area.

The question you ask is….should I scan his head?

Paper 4 - Borland M, Dalziel SR, Phillips N, Lyttle M, Bressan S, Oakley E, Hearps SJC, Kochar A, Furyk J, Cheek J, Neutze J, Gilhotra Y, Dalton S, Babl F. Delayed Presentations to Emergency Departments of Children With Head Injury: A PREDICT Study, Annals of Emergency Medicine, DOI:

This paper aimed to establish the prevalence of traumatic brain injuries in children presenting more than 24 hours after the head injury.

Traumatic brain injury (TBI) was defined as: intracranial haemorrhage; contusion; cerebral oedema; diffuse axonal injury; traumatic infarction; shearing injury; or a sigmoid sinus thrombosis.

The also looked a clinically significant traumatic brain injury (cTBI) – this included death, intubation for more than 24 hours, neurosurgery, or admission for 2 or more nights to hospital.

The patients were from the Australian Paediatric Head Injury Study Cohort which was 20,137 patients. 5% of these presented over 24 hours after the injury. 981 children were included in this study.

The authors considered the injury characteristics and demographics, trying to find an association between mechanism and delay in presentation. Those presenting were more likely to have: a non-frontal scalp haematoma; headache; vomiting; and assault with NAI concern. Those with loss of consciousness and amnesia were more likely to have presented within the first 24 hours.

The CT rates were much higher in the late presentation group – 20.6% being scanned in the delayed group and only 7.9% in the early group. This probably reflects the lack of evidence in this area, and therefore we feel safer doing more scans.

But the rates of TBI also varied. 3.8% in the delayed presentation group had a TBI, whereas only 1.2% in the early presentation group did.

The rates cTBI were the same between the groups at 0.8%

Key take homes: There is an increased risk of TBI when presenting more than 24 hours after a head injury injury. The authors found that risk is increased if the patient has a non-frontal scalp haematoma or a suspicion of a depressed skull fracture.

You decide to scan Jayden’s head, but it turns out to be normal and he is discharged home.


Jayden is 8. He’s been drinking a LOT of water over the last few weeks and seems to be weeing constantly. His clothes seem a bit big for him too. He looks so bad one day (and has vomiting and abdominal pain) that Jason finally reneges and takes him into ED.

Jayden has DKA. The debate about over-zealous fluid administrations and its relationship to the dreaded cerebral oedema is long-standing. Previous research suggested a link but only by association, not causality.

You ask yourself…how fast should I give IV fluids?

Paper 5 - Kupperman et al. Clinical Trial of Fluid Infusion Rates for Pediatric Diabetic Ketoacidosis NEJM 2018 vol 378 (24) pp 2275-2287

The study examines the causal effect between fluid resuscitation and cerebral oedema.

They included 1389 episodes of DKA. Exclusions were mainly due to too much management prior to contact with the study team, as well as children with a GCS<12. The median age was 11. It should be noted that the very young and the very sick are probably lost in this cohort.

Patients were randomised to received either fast or slow rehydration, and then were split again into received either 0.9% NaCl or 0.45% NaCl.

The fast rehydration group received 20ml/kg bolus and then replacement of 10% deficit, half over 12 hours and rest over next 24 hours. The slow rehydration group received a 10ml/kg bolus and then replacement of 5% deficit over 48 hours. Maintenance fluids and insulin were given in addition.

The outcomes looked at were deterioration of neurological status within first 24 hours of treatment. They also assessed short term memory during treatment, and IQ 2-6 months after the episode of DKA.

In short, they found no difference between the groups. There was a 0.9% rate of brain injury overall and it didn’t matter which type of fluids or how fast. Patients were more likely to get hyperchloraemic acidosis in the 0.9% NaCl group but this is of debatable clinical significance.

Key take homes: The evidence does not support our traditionally cautious approach to DKA. The speed of IV fluids does not seem to be the cause of brain injury in DKA.

You resuscitate Jayden and send him off to the ward. He is discharged a few days later with good support from the Endocrine team for management of his diabetes.


Jayden is now 16 years old and next time he comes to ED, he’ll be in the harsh world of Adult ED. We have navigated him through his common childhood presentations to ED and answered the key questions we ask ourselves every day in the Paeds ED.


Should I do a full septic screen on this hot baby?

Should I start high flow on this infant with bronchiolitis?

Should I give prednisolone to this 2 year old with wheeze?

Should I scan this child with a head injury?

How fast should I give fluids to my DKA?

And most importantly, do I ever need to sift through my child’s poo, or my own ever again?

Don’t Forget The Lego

Cite this article as:
Team DFTB. Don’t Forget The Lego, Don't Forget the Bubbles, 2018. Available at:

It might have escaped your notice but the team at DFTB recently had a paper published by the Journal of Paediatrics and Child Health that has garnered a lot of interest.

Tagg, A. , Roland, D. , Leo, G. S.Y., Knight, K. , Goldstein, H. , Davis, T. , DFTB, (2018), Everything is awesome: Don’t forget the Lego. J Paediatr Child Health. doi:10.1111/jpc.14309

We are sure you have questions. Lots of questions. So we thought we should answer them for you in the best way we know how.

What pressing scientific question did you ask?

We know that coins are the most commonly swallowed foreign object in the paediatric population and there is a lot of data surrounding transit time. The second most commonly swallowed objects are small toys but there is very little data out there. We wanted to know how long it would take for a small piece of plastic toy, in this case a Lego head, to pass through.

How on earth did you come up with the idea?

In one of our regular editorial meetings we were discussing some of our upcoming publications and musing how we could do something a little lighter, akin to the great Peppa Pig paper in last years Christmas BMJ. And then Andy Tagg said, “I’ve got this idea but you might think it a bit strange.” Within a short space of time we had an international team of researchers literally chomping at the bit to undertake the study.

Did you really swallow those poor heads?

Of course we did! Do you want proof?

Then what happened?

We waited to see what would happen. We all know corn kernels can whip through the colon in seemingly no time at all, but what about a little yellow piece of plastic? There was really only one way to find out.

And you searched through your own poo to find them? How?

As with any piece of research it is important to have a robust search strategy in place prior to commencement. A variety of techniques were tried – using a bag and squashing, tongue depressors and gloves, chopsticks – no turd was left unturned. And although we only used a very small sample size the fact that one of our heads went missing suggest that you really shouldn’t worry if you can’t find it.

What happened to the missing head?

Who knows? Perhaps one day many years from now, a gastroenterologist performing a colonoscopy will find it staring back at him.

But what about Ben Lawton? Where was he when all this was going on?

Don’t Forget the Bubbles was founded by four curious doctors – Tessa Davis, Andy Tagg, Henry Goldstein and Ben Lawton. Unfortunately Ben was travelling at the time we undertook the study and we didn’t think searching through his colonic contents in an aeroplane toilet was exactly fair.

And then you kept it quiet, right?

It can take an average of 17 years for science to go from benchside to bedside. Leveraging social media we managed to go from online publication on a Thursday evening to global saturation by Saturday evening.

By Saturday morning Damian Roland was speaking on Canadian radio and the DFTB group made Forbes, ars technica, and the BBC World Service by the afternoon.

But surely this isn’t hard science?

Of course it’s not, it’s a bit of fun in the run up to Xmas.

With such a small sample size it is important that you don’t extrapolate the data to the entire population of Lego swallowers. Anecdata from Twitter suggests that a large number of people accidentally ingested bits of Lego throughout their life with no adverse effects*.

It is also worth noting that most people who swallow Lego are children, not fully grown adults. Data that is applicable to the adult population may well not be applicable to children.

For a more scientific approach to ingested foreign bodies in children then take a look at these two papers.

Yeh HY, Chao HC, Chen SY, Chen CC, Lai MW. Analysis of Radiopaque Gastrointestinal Foreign Bodies Expelled by Spontaneous Passage in Children: A 15-Year Single-Center Study. Frontiers in pediatrics. 2018;6:172.

Macgregor D, Ferguson J. Foreign body ingestion in children: an audit of transit time. Emergency Medicine Journal. 1998 Nov 1;15(6):371-3.

You may also enjoy exploring the following posts about foreign bodies on DFTB:

Andy’s blog post on Foreign Body Ingestion

Chantal McGrath’s DFTB17 talk Batteries Not Included on button battery ingestion

A case study by Loren on ‘the magic coin’ 

What’s next for the group?

Whilst this may be the pinnacle of our publishing careers we hope we have not peaked too early. Next up is finalizing all the details for our upcoming conference in London –, and then? Who knows?

*Please do not try this at home.

DFTB in the papers

Ars Technica


BBC World Service

CBC Radio Canada – As it happens

10 Daily

Herald Sun


Cite this article as:
Clementine David. FPIES, Don't Forget the Bubbles, 2018. Available at:

A 6-month-old male is brought to ED by his mother with multiple episodes of profuse vomiting after eating lunch. No diarrhoea, fevers or unwell contacts. He is usually a well child and had a normal neonatal period.

He is immunised and otherwise thriving from a growth and developmental perspective. The mother, a nurse, reports that the infant was mottled, pale and lethargic at home but began to pick up whilst being triaged in ED.

Gastroesophageal Reflux Disease

Cite this article as:
Li-Zsa Tan. Gastroesophageal Reflux Disease, Don't Forget the Bubbles, 2016. Available at:

A 5-month-old infant is brought into the Emergency Department by her parents because they feel she has been persistently irritable and back arching after feeds for the last month. They have looked up ‘infant reflux’ on the internet, but upright positioning and changing formulas several times has not made any difference. They are now intensely sleep-deprived and are keen for something to be done for her.

Examination reveals a cheerful baby girl with no signs of being unwell or irritable. She is gaining weight according to her centiles and is developmentally appropriate for age.

Bottom Line

  • Gastroesophageal reflux is a physiological phenomenon
  • Gastroesophageal reflux disease is when the child is symptomatic and results in complications such as poor weight gain
  • 67% of healthy infants have some form of reflux at 4 months of age
  • It is important to consider factors which may worsen reflux or contribute to reflux disease, such as hiatus hernia or eosinophilic esophagitis
  • Most infants will improve on transitioning to solids

What is gastroesophageal reflux disease?

Gastroesophageal reflux (GER) is a physiological phenomenon.  It is defined as the retrograde passage of gastric contents into the esophagus and is a normal physiological phenomenon that occurs in healthy children several times a day after meals and lasts <3 minutes. Reflux disease (GERD) occurs when it results in symptoms and complications.

Regurgitation is the passage of refluxed contents into the mouth. Vomiting is the expulsion of said contents from the mouth.

Primary GER results from a primary disorder of the upper GI tract.

Factors which influence incidence of GOR include: mastication, saliva secretion; swallowing; esophageal clearance; esophageal innervation and receptors; mucosal resistance; LES pressure and relaxation; abdominal esophagus; sphincter position; angle of His; gastric volume and accommodation; gastric emptying; gastric acid output; gastric acid feed buffering; feeding regimen: type frequency and volume; pepsin/trypsin/ bile salts; H. Pylori; intra abdominal pressure; genetic factors; environmental factors; posture; physical activity; sleep state; respiratory disease; medications.

Sometimes GER is protective: e.g. when the stomach is overdistended after a meal GER decompresses it.

Which mechanisms are in place to prevent or limit GERD?

The first line of defense against GERD is the lower esophageal sphincter (LES), and the diaphragmatic pinchcock and angle of His which physically limits the frequency and volume of gastric contents refluxing into the esophagus.

The second defense is esophageal clearance, when gravity and peristalsis remove the contents from the esophagus and secretions serve to neutralise the acid.

The third defence is the esophageal mucosal defence against luminal acid.

How does the LES work?

The LES is an extension of the circular muscle of the esophagus. The anti-reflux barrier consists of the LES and the crural portion of the diaphragm. The esophagogastric angle (or the angle of His) is the angle between the esophagus and the greater curvature of the stomach and is normally acute. It is a functional barrier, and its intraluminal pressure is greater than that of the stomach and esophagus. In adults, it is 3-6 cm long and has a pressure of 20 mmHg. (range 10-40 mmHg). An absolute pressure of <6 mmHg is required for GER.  In infants, the length is only a few millimeters. The LES relaxes 2.5 secs after the initiation of a swallow, and remains open during 10-12 secs until the food bolus passes through. LES pressure is decreased postprandially, and is also decreased by among others: the presence of fat in the duodenum, progesterone, cholecystokinin, glucagon, estrogen, nitric oxide, dopamine, nicotine, alcohol, mint and chocolate.

Most physiologic reflux episodes occur in relation to Transient LES Relaxations  (TLESRs) or when the LES tone adapts inadequately to changes in intra-abdominal pressure. TLESRs are induced by gastric distension and incomplete swallowing (normal mechanism for burping and belching). The larger the meal the more TLESRs, the more reflux episodes! Higher intragastric osmolarity and greater gastric secretory volume also contribute towards more TLESRs which may contribute to the efficacy of Proton Pump Inhibitors and H2 receptor antagonists in decreasing secretory gastric volume and reducing TLESRs.

The angle of His is usually acute. When the angle is obtuse, such as in hiatal hernias, this favors GER episodes

How does esophageal clearance help in reflux episodes?

This is influenced by peristaltic waves, gravity and saliva. Swallowed saliva contributes towards neutralising the pH of the refluxed acid. Swallowing itself stimulates antegrade peristalsis which clears the esophageal contents.

Esophageal mucosal resistance against injury to acid consists of:

  • Pre-epithelial: mucous layer, surface bicarbonate ion concentration, unstirred water layer
  • Epithelial defense: The esophagus is lined by moist, partially keratinized stratified squamous epithelium. Tight junctions act as a barrier to molecules passing from lumen to blood.

What are the symptoms of GERD?

GERD can range from minor symptoms such as regurgitation, heartburn and epigastric pain to more complicated disease such as erosive esophagitis or esophageal stricture. Hiatal hernia is the only endoscopic observation that predicts erosive esophagitis.

Reflux disease may also cause respiratory symptoms through micro aspiration. This may manifest as apneas, chronic coughs or chest infections, particularly in neurologically compromised infants.

How common is reflux or regurgitation in normal children?

In healthy infants, the prevalence of regurgitation has been reported at 50% at age 0-3 months, 67% at 4 months then declining to <5% by 10-12 months.  In a study of 509 healthy infants aged 0-11 months, as many as 73 physiologic reflux episodes per day was normal.

How do we investigate GERD?

pH monitoring

Intraluminal pH monitoring measures the frequency and duration of acid esophageal reflux episodes. A drop in intraesophageal pH <4.0 is considered an acid reflux episodes.

pH monitoring has limitations because of its inability to detect nonacidic bolus movement into the esophagus in particular in infants who are fed milk. This is problematic when evaluating reflux as a contributing factor towards respiratory disease in infants. Studies showed that in infants with apparent life-threatening events or apnea, 48% were nonacid. In a study of preterm infants with apnea, Magista et al found that 76% of reflux events were only weakly acidic (4

Impedence technique

Multichannel intraluminal impedance (MII) detects GER episodes based on changes in electrical resistance to the flow of an electrical current between 2 electrodes placed on the MII probe when a liquid, semisolid, or gas bolus moves between them. The combined pH and impedance monitoring are therefore able to detect reflux regardless of pH and provide symptom correlation with parental reports.


Upper GI endoscopy can also be performed to look for esophagitis (macroscopic and histological changes) and gastritis. As this involves a general anaesthetic in children and has its own risks and complications, endoscopy is not usually a first line investigation for simple suspected reflux disease.

What are the pharmacological options to treat reflux disease?

Proton Pump Inhibitors are the most potent inhibitors of gastric acid secretion, and are superior to H2 Rreceptor antagonistis in healing esophagitis and maintaining remission.

What about other approaches?

There is no clear evidence that upright positioning post feeds helps limit reflux episodes in infants. In fact GER seems to occur most in seated upright position! Changing formulas or from breastfeeding to bottle feeding also does not appear to have an effect on reflux disease.

Thickening feeds under the supervision by a dietician may be useful.

It is important to rule out contributing factors such as concurrent infection in infants, congenital abnormalities such as a hiatus hernia and eosinophilic esophagitis and H pylori infection in older children.

Transitioning to solids usually marks clinical improvement in symptoms.


Vandenplas Y, Hasall E. Mechanisms of Gastroesophageal Reflux and Gastroesophageal Reflux Disease. Journal of Pediatric Gastroenterology and Nutrition 2006; 35: 119-136.

Colletti R, Di Lorenzo C. Overview of Pediatric Gastroesophageal Reflux Disease and Proton Pump Inhibitor Therapy. Journal of Pediatric Gastroenterology and Nutrition 2003; 37: S7 – S11.

Mousa HM, Rosen R, Woodley FW, Orsi M, Armas D, Faure C, Fortunato J, O’Connor J, Skags B, Nurko S. Esophageal Impedance Monitoring for Gastroesophageal Reflux. Journal of Pediatric Gastroenterology and Nutrition 2011;52:129 – 139

Upper GI Bleed

Cite this article as:
Li-Zsa Tan. Upper GI Bleed, Don't Forget the Bubbles, 2016. Available at:

A 7 year old boy is referred from a peripheral hospital for a 2 day history of passing black tarry stool. On the 3rd day he passes loose stools mixed with dark maroon blood clots. For the last week he had been having upper respiratory tract symptoms and had daily doses of ibuprofen for his low grade fever.

Biliary atresia

Cite this article as:
Li-Zsa Tan. Biliary atresia, Don't Forget the Bubbles, 2016. Available at:

A 5 week old infant is seen for the first time at the paediatrics outpatient’s clinic with jaundice. The baby boy was being followed up by his GP for jaundice since the age of 6 days. As he was otherwise a well child, his mother was assured that the jaundice was likely related to breastfeeding. His GP grew concerned when his jaundice persisted and performed a formal serum bilirubin when he was 4 weeks old, which showed that he had a predominantly conjugated hyperbilirubinaemia. After his thyroid function tests returned as normal and a urinary tract infection was excluded, he was referred to a tertiary hospital for further evaluation. Radioisotope excretion studies showed good hepatic uptake but absent excretion into the intestine within 24 hours.

Bottom Line

Any infant with jaundice lasting more than 14 days must have a formal serum bilirubin checked with conjugated/unconjugated differentials

Pale stools may not easily be identifiable and so may provide false reassurance

The most important prognostic factor for a Kasai portoenterostomy is age. The older the patient at the time of surgery, the less successful the outcome

What is biliary atresia?

Extrahepatic biliary atresia is a destructive inflammatory obliterative cholangiopathy which affects both intra and extra hepatic ducts with progressive destruction leading to cholestasis, fibrosis and cirrhosis.

The disease is classified according to the most proximal biliary obstruction:

Type 1 (~ 5%):         Patency to the level of the common bile duct and proximal cystic duct

Type 2 (~ 2%):         Patency to the level of the common hepatic duct

Type 3 (> 90%):      The entire extra hepatic biliary tree is non patent [/toggle]

How common is it?

In UK and France, the prevalence is 1 in 17000 -19000 livebirths.

In east Asian countries it is much more common, with the frequency in Taiwan being quoted at 1 in 5000.

What else is associated with biliary atresia?

Biliary atresia is associated with other congenital malformations in up to 20% of cases. The most common of these is the biliary atresia splenic malformation syndrome, (10% in Europe and the US).

CharacteristicsEstimated frequency
Pancreatic anomalies11%
Cardiac anomalies (VSD, ASD, HLH)45%
Absent inferior vena cava70%
Preduodenal portal vein40%
Intestinal malrotation60%
Situs inversus37%
Splenic malformation (polysplenia, asplenia)100%

Biliary atresia has also been described with other genetic disorders, for example in trisomies 18 and 21.

In 80-90% of neonates, it is an isolated finding. Thus two clinical phenotypes are described: the syndromic or embryonic forms associated with other congenital/genetic abnormalities, and the far more common perinatal or acquired form in which BA is an isolated finding.

The theory is that in the perinatal or acquired form, the obliterative process begins later in the perinatal period than it does in the syndromic form (which may begin in the embryonic phase

Any theories of pathogenesis?

The cause of biliary atresia is unknown but is likely to be multifactorial:

Genetic: Genes associated with different parts of the inflammatory pathway (e.g. CFC1, ICAM1,CD14 endotoxin receptor gene) have variable polymorphism frequency in children with biliary atresia.

Viral: Studies inoculating mice with rotavirus strains, reovirus and cytomegalovirus have resulted in jaundice and intrahepatic histology similar to biliary atresia. Liver biopsies from human patients showed 90% of infants expressed a protein linked to inflammation secondary to a viral immune response.

Immunological: It is unlikely that a direct viral cause is responsible for the pathogenesis of biliary atresia as viral particles have not been isolated in biopsies. Rather, viral infection may be the trigger to activation of the innate immune response which causes apoptosis and cell death. It is more likely that infection is a second injury to a liver which is already susceptible to damage through genetic or immunological dysregulation. Lymphocyte mediated biliary inflammation seems the most likely mechanism by which bile duct obliteration occurs but the trigger for this response is unknown.

How do patients present?

Typically biliary atresia presents in early infancy with persistent jaundice, dark urine and pale stools. A 2012 study however concluded that even experienced professionals often fail to recognize stool colour associated with biliary obstruction

How is it diagnosed?

All term infants who remain jaundiced after 14 days should have serum bilirubin levels measured with conjugated/unconjugated differentials.

Physiological and breast milk jaundice manifests as unconjugated hyperbilirubinaemia, whereas most forms of liver disease present with raised conjugated bilirubin.

Liver function tests typically show raised transaminases, especially serum gamma-glutamyltransferase.

An abdominal ultrasound is an essential basic investigation which may demonstrate an enlarged liver, and an absent or contracted gallbladder after a 4 hour fast. Biliary dilatation is not seen.

Radio isotope excretion studies scan after pretreatment with phenobarbitone 5mg/kg per day for 3-5 days will typically will show good hepatic uptake but absent or reduced excretion into the intestine within 24 hours. This result is not specific for biliary atresia however and is also found in children with Alagille’s syndrome.

Percutaneous liver biopsy is the usual diagnostic method and shows evidence of extrahepatic biliary obstruction such as portal tract fibrosis, oedema, ductular proliferation and cholestasis. Biopsies done before 6 weeks of age might not have typical features.

The gold standard for diagnosis, particularly if there is any doubt prior to a Kasai portoenterostomy, is an operative cholangiography. Other investigations include an endoscopic retrograde cholangiopancreatography or a magnetic resonance retrograde cholangiopancreatography although these are technically difficult and not widely available.

How is it managed?

i. Kasai portoenterostomy:

The entire extrahepatic biliary tree is excised so that the porta hepatis is transected at the level of the liver capsule and anastomosed to the jejunum via a Roux loop. Success is measured by clearance of jaundice and is defined as achievement of normal bilirubin concentration within 6 months of the procedure.

A Kasai is not curative, and the disease will continue to progress in 70% of children who have had a successful surgery.

The most important prognostic factor for the Kasai portoenterostomy is the patient’s age at the time of the procedure. The older the infant, the less successful the outcome.

Age at Kasai4 year survival with native liver10 year survival with native liver
>90 days23%15%
31-90 days36%25%
<30 days49%49%

Because timing is such a factor in outcome, some countries (Taiwan, Japan) are considering implementing a screening program based on stool colour cards to increase awareness of biliary atresia among parents and physicians. A 2011 study showed that stool colour card screening may be associated with a decline in late referrals.

Complications of Kasai:

Ascending cholangitis presents in the first few months post surgery and manifests as a recurrence of jaundice, acholic stool and abdominal pain. Recurrent or late cholangitis suggests an obstruction of the Roux loop.

A degree of hepatic fibrosis or cirrhosis is apparent even in children with a successful Kasai. Portal hypertension is present in most cases and has its own complications such as ascites or variceal bleeds.

Regular ultrasound scans and annual alpha feto protein levels should be part of a long term review to screen for development of malignancy secondary to cirrhosis.

ii. Liver transplantation:

Indications and timing for a liver transplant depends on the success of the Kasai portoenterostomy and the rate of complications. Infants who had failed Kasai surgeries will require transplantation within 6 months to 2 years. Children with BASM syndrome have an increased risk of early mortality and morbidity and thus the need for transplantation is greater. Living related liver transplantation is successful in Taiwan and Japan and allow for planned and timely transplantation with good reported outcomes. [/toggle]


Biliary Atresia. Hartley J, Davenport M, Kelly D. Lancet 2009; 374:1704-13

Current management of biliary atresia. Kelly D, Davenport M. Arch Dis Child 2007; 92:1132-35

Prolonged Neonatal Jaundice and the Diagnosis of Biliary Atresia: A single center analysis of trends in age at diagnosis and outcomes. Wadhwani S, Turmelle Y, Nagy R, Lowell J, Dillon P, Shepherd R. Pediatrics 2008; 121 (5): 1438-40

Biliary Atresia: The timing needs a changin’. Chitsaz E, Schreiber RA, Collet JP, Kaczorowski J. Canadian Journal of Public Health 2009;100(6):475-77

Stool color card screening for Biliary Atresia. Tseng J, Lai M, Lin M, Fu Y. Pediatrics 2011; 128(5) 1209

Probiotics in review

Cite this article as:
Henry Goldstein. Probiotics in review, Don't Forget the Bubbles, 2016. Available at:

Like almost every other human entering a pharmacy in the ten last years, I was offered some probiotics when I collected a prescription recently. On my walk back to the car I mused about the evidence behind the shop assistants attempted up-sale. I reminded myself of the use of probiotics to prevent necrotising enterocolitis, and was starting to think of some other indications. Some days later, this review by Hania Szajewska in the Archives of Disease in Childhood popped up; here’s a precis of an excellent paper:

Szajewska, H.What are the indications for using probiotics in children? Arch Dis Child. Published Online First: 7 September 2015

Probiotics are “live microorganisms that, when administered in adequate amounts, confer a health benefit on that host.” The most common strains used therapeutically are the lactobacillus strains L. reamnosus GG (LGG) and L. reuteri DSM 17938 as well as bifidobacterium and saccharomyces. There are also some novel probiotics in development.

Probiotic preparations differ to standard medications as the dose, viability and even agent (organism) are harder to control. There is significant industry influence and, in my opinion, therapeutic development has likely suffered at the expense of populist marketing. Research into probiotics is strain specific; with that comes the challenges of extrapolating the findings to any over-the-counter product. Specifically, probiotics are not regulated as drugs, hence significant concerns exist with respect to labelling and quality.

In this paper, Szajewska reviews the evidence for a number of paediatric indications for probiotics. I’ve simplified and summarised the findings here;

What do we think works?

Necrotising enterocolitis – Multiple RCTs and a Cochrane review, mostly using L. reuteri DSM 17938 show a reduction in NEC in preterm infants. Additionally, there was a reduced time to full feeds, reduced admission length and reduced rates of late-onset sepsis.

Antibiotic associated diarrhoea – Szajewska references her own meta analysis – albeit primarily an adult population – which identified a NNT of 13 for antibiotic associated diarrhoea; the database is predominantly adults. Most effective probiotic agents for this indication are saccharomyces boulardii and LGG.

Infantile colicL. reuteri DSM 17938 was assessed in 4 RCTs; their combined results showed that the use of reduced crying times in breastfed infants with infantile colic. In one analysis (3 trials), L. reuteri DSM 17938 vs placebo reduced crying times at 21 days of life by an average of 43 minutes/day. Probiotics appear more helpful in breastfed by comparison to formula fed infants.

Functional abdominal pain – A meta analysis of LGG for a range of abdominal pain-related functional gastrointestinal disorders (FGDs) showed that LGG was significantly better than placebo in this population, with a NNT = 7. Szajewska doesn’t appear to have much faith in these results with respect to FGDs as a whole, but notes that patients with Irritable bowel syndrome showed the most benefit (NNT = 4).

Acute gastroenteritis – ESPGHAN (the European Society for Paediatric Gastroenterology, Hepatology & Nutrition) recommend consideration of probiotics (LGG > S. boulardii > L. reuteri DSM 17938) for children with acute gastroenteritis, in addition to hydration therapy.

What might work?

Nosocomial infection – The review considers a number of nosocomial infections, and briefly mentions the importance of rotavirus immunisation, where available. A handful of trials showed that probiotics (LGG) vs placebo had no significant differences for risk of post-admission diarrhoea in children under 2 years old; the results contradict some earlier trials in this area, which showed promise.

Prevention of allergy – This is controversial – two studies published by opposing peak bodies disagree. This includes maternal probiotics to reduce long-term outcomes.

H.Pylori – May improve eradication rate, but limited evidence in children.

IBD – Some evidence for inducing remission of Ulcerative colitis; insufficient evidence in Crohn’s disease. 

What doesn’t work?

Functional constipation – no evidence of benefit; not recommended via ESPGHAN 

Within the review, two positive studies jumped out at me, so I went back to the primary literature for a deeper dive.

Firstly, I was fascinated by the idea of preventing infections in daycare centres – Szajewska’s overall verdict was that there was not currently sufficient evidence, but that LGG and L. reuteri DSM 17938 may have some effect on community-acquired infections. Particularly, the review describes this study;

Gutierrez-Castrellon, P., Lopez-Velazquez, G., Diaz-Garcia, L. et al. Diarrhea in Preschool Children and Lactobacillus reuteri: A Randomized Controlled Trial. Pediatrics Mar 2014, peds.2013-0652; DOI: 10.1542/peds.2013-0652

P: 336 children born at term aged 6-36 months attending a daycare in Mexico
I: 5 drops L. reuteri DSM 17938 for 12 weeks
C: placebo drops
O: The primary outcome was the number of days with diarrhoea per child, which was defined as days when 3 or more loose or watery stools were passed within a 24-hour period with or without vomiting, both during the intervention and for 12 weeks afterwards.

  • About ¼ of families offered enrolment decline; which means we should question the (?social) acceptability of the intervention in this population.
  • Semi blinded – one of the authors was overseeing the block-randomisation.
  • Interesting exclusion criteria including birth weight < 2500 g, chronic disease, failure to thrive, allergy or atopic disease, recent (previous 4 weeks) exposure to probiotics, prebiotics, or antibiotics, or were participating in other clinical trials.
  • A reasonably well defined list of secondary outcomes.
  • Parents were educated about stool descriptors using the Bristol stool scale, and upon a loose motion had to contact the study centre, and then report for assessment. I wonder if this call-presentation process lent itself to underreporting (in both groups.)
  • All four primary outcomes: Number of diarrhea episodes, Episodes of diarrhea per child, Mean duration of diarrhea episodes and Days with diarrhea per child were significantly better in the treatment arm, both during the intervention and afterwards. With p values ranging from 0.03 to 0.01.

Secondly, the idea that probiotics could reduce infantile colic seemed immensely appealing; it’s an area that has had a myriad of debunked therapies over the last several millenia. T

Indrio F.,Di Mauro A., Riezzo G., et al..Prophylactic use of a probiotic in the prevention of colic, regurgitation, and functional constipation: a randomized clinical trial. JAMA Pediatr. 2014 Mar;168(3):228-33.

P: 589 term infants aged less than one week, in 9 centres across Italy.
I: 5 drops of L reuteri DSM 17938 (1×10^8 cfu) for 90 days
C: placebo
O: Primary outcomes were daily crying time, regurgitation, and constipation during the first 3 months of life. Cost-benefit analysis of the probiotic supplementation.

  • Infants receiving antibiotics in the first week of life were excluded; (in Australian maternity units, this would account for a significant number.)
  • Trial was independently randomized and double blinded.
  • Around ⅙ patients were lost to follow-up; a significant number were withdrawn from the treatment arm for protocol violations by the investigator.
  • Parents recorded data in a structured diary and sought advice as required via usual channels.
  • At both one and 3 months of life, the infants in the treatment arm cried for significantly shorter periods of time and stooled more frequently. At three months, there were fewer episodes of regurgitation in the treatment arm.
  • Although this is a single study, there are a number along similar lines; Szajewska’ paper mentions 4 in total. The results are most striking in this paper, hence my curiosity.

The organism of the hour, L. reuteri DSM 17938 was first cultured from breast milk of a Peruvian mother; it is patented by BioGaia whom provided the study drug and placebo for both trials above.


There’s a growing body of evidence for the potential benefits of probiotics in a number of paediatric conditions.

There is a bias in this review (and pretty much all of academic medicine) towards positive trials. That being said, I haven’t given the details of every study mentioned; Szajewska’s review does so nicely and I also recommend a read of the primary literature.

Most importantly, communicating with parents about the uncertainties about over-the-counter probiotics with respect to labelling, quality, dose and organism remain central to this discussion.

Key points:

  • Probiotics are “live microorgnaisms that, when administered in adequate amounts, confer a health benefit on that host.”
  • There are many vested interests & popular marketing with issues around labelling and quality in this area.
  • Research is strain specific.
  • Main strains researched are; Lactobacillus reuteri DSM 17938, Lactobacillus reamnosus GG (LGG), Bifidobacterium and Saccharomyces
  • Presently, benefit has been demonstrated in NEC, Antibiotic associated diarrhoea, infantile colic, functional abdominal pain and acute gastroenteritis.


Szajewska, H.What are the indications for using probiotics in children? Arch Dis Child archdischild-2015-308656 Published Online First: 7 September 2015

Gutierrez-Castrellon, P., Lopez-Velazquez, G., Diaz-Garcia, L. et al. Diarrhea in Preschool Children and Lactobacillus reuteri: A Randomized Controlled Trial.