Paediatric Gastroenterology Flashcards

Question

What is Hirschsprung's disease?

Answer 1

A congenital condition where the nerve cells of the myenteric plexus are absent = absence of parasympathetic ganglion cells along a section of the bowel Aganglionic section does not relax - causing obstruction of the bowel (Hirschsprung disease (HD) is a congenital disorder characterized by the absence of ganglion cells (GC) at the Meissner's plexus (submucosa) and Auerbach's plexus (myenteric plexus *(muscularis) ) parasympathetic neuroblasts fail to migrate from the neural crest to the distal colon → developmental failure of the parasympathetic Auerbach and Meissner plexuses → uncoordinated peristalsis → functional obstruction

Answer 2

1. Downs syndrome 2. Neurofibromatosis 3. Waardenburg syndrome (a genetic condition causing pale blue eyes, hearing loss and patches of white skin and hair) 4. Multiple endocrine neoplasia (MEN2) type II 3x more common in males (4-to-1 male predominance)

Answer 3

1. Delay in passing meconium (more than 24 hours) 2. Chronic constipation since birth 3. Abdominal pain and distention 4. Vomiting 5. Poor weight gain and failure to thrive -enterocolitis (toxic megacolon): fever, abdominal pain, bloody diarrhoea, shock

Answer 4

1. Air under diaphragm 2. Dilated intestine 3. Free air around the liver The X-ray answer shows multiple dilated loops of bowel. In the neonate it is difficult to differentiate between large and small bowel. If there are a few dilated loops of bowel seen on an X-ray, the obstruction will be proximal e.g. malrotation, jejunal atresia. Duodenal stenosis has a classic double bubble appearance on X-ray . If there are multiple dilated loops of bowel seen on an abdominal film of a patient who has not passed meconium, the patient is likely to have a more distal obstruction e.g. ileal atresia, meconium ileus or plug, Hirshsprung’s or anal atresia. The left-hand x-ray shows there are multiple dilated loops of bowel and air is seen in the rectum. Hirschsprung’s disease is therefore the most likely diagnosis. Note the free air below the diaphragm, adjacent to the falciform ligament and around the bowel. The patient has perforated hence the sepsis, tachycardia and tachypnoea.

Answer 5

Hirschsprung-associated enterocolitis (HAEC ie toxic megacolon) is inflammation and obstruction of the intestine occurring in around 20% of neonates with Hirschsprung’s disease. It typically presents within 2-4 weeks of birth with fever, abdominal distention, diarrhoea (often with blood) and features of sepsis. It is life threatening and can lead to toxic megacolon and perforation of the bowel. Initial treatment is supportive with fluid resuscitation, decompression with NG and rectal tubes, and broad-spectrum antibiotics (including anaerobic coverage). Surgery is the definitive treatment.

Answer 6

Management initially: rectal washouts/bowel irrigation Definitive management: surgery to affected aganglionic segment of the colon (Most patients will live a normal life after corrective surgery, although they can have long term disturbances in bowel function and may be left with some degree of incontinence) -Surgical procedures of choice include the endorectal pull-through, recto-sigmoidectomy, retro-rectal transanal pull-through, and ano-rectal myomectomy -A temporary colostomy may be performed to allow patient growth, and surgical intervention may be deferred altogether in the presence of enterocolitis. Unwell children and those with enterocolitis will require initial fluid resuscitation and management of the intestinal obstruction. IV antibiotics are required in HAEC.

Answer 7

Rectal biopsy Other: contrast barium enema (Enemas should be avoided in those suspected to have Hirschsprungs’s disease who are systemically unwell, as perforation may be present, and an enema may lead to worse peritonitis.) Diagnosis is confirmed histologically with either a sub-mucosal or a full thickness rectal biopsy in association with either anorectal manometry or barium enema. The affected segment lacks ganglion cells which aid in normal peristalsis. Without these ganglion cells, normal peristalsis is lacking, resulting in a functional obstruction. This produces a proximal dilated colon and a distal normal appearing segment which is seen as a transition zone on a barium enema. The rectum and sigmoid are most commonly involved, with decreasing incidence progressing cephalad. Total aganglionosis of the colon is a rarity, and small bowel involvement is even less common.

Answer 8

An invagination of a portion of the bowel (bowel telescoping)

Answer 9

1. Paucity (lack of) of air in RUQ 2. Poorly defined liver edge 3. Paucity of gas in large bowel 4. Dilated bowel loops of small bowel There is a mass and paucity of bowel gas in the right upper quadrant causing loss of clarity of the liver edge. This represents the intussusception. The intussusception is likely to be ileo-colic due the position in the RUQ; the dilated loops of small bowel proximal to the intussusception and paucity of gas within the deflated large bowel distal to the obstruction. An abdominal X-ray is essential to look for free air if the patient has signs of peritonitis and may have perforated.

Answer 10

The most common type of intussusception is ileo-colic, also known as ileo-caecal (90%). A portion of terminal ileum intussuscepts through the ileo-caecal valve into the colon. It may sometimes extend all the way to the rectum. Other rarer types of intussusception include ileo-ileal, colo-colic, and ileo-ileocolic. The majority of intussusceptions are idiopathic.

Answer 11

triad of : 1. Severe colicky abdominal pain 2. RED CURRANT JELLY STOOL (& mucus) 3. Vomiting (initially non-bilious then bilious as obstruction ensues) (Sausage shaped mass in the RUQ (hypochondrium): same location os "olive shaped mass" in pyloric stenosis, "target" or "donut" sign) -right hypochondrium sausage-shaped mass and emptiness in the right lower quadrant (Dance sign) Pale, unwell child -drawing legs to chest during attack -sunken fontanelle (dehydration sign) -5months-1 year (2x more common in males>females) -seasonal occurrence with viral illness

Answer 12

The target sign, also known as the doughnut sign or bull's eye sign - is the appearance of alternating echogenic and hypoechoic bands caused by the telescoping of the bowel.

Answer 13

Target shaped mass (most commonly ileocaecal junction) -air contrast enema An ultrasound is usually performed first and the intussusception is usually seen as a mass (fig.3). Ultrasound is useful at demonstrating free fluid indicating peritonitis and may show if the bowel is ischaemic (with colour doppler) which would both be contraindications to air reduction. Air contrast enema and attempts at radiological air reduction are usually performed following the ultrasound (provided that the patient is resuscitated, stable and there are no contraindications), even if the ultrasound scan is normal when there is a strong clinical suspicion of the diagnosis. Evidence of bowel perforation necessitates immediate surgery.

Answer 14

An anatomic lead point (a piece of intestinal tissue which protrudes into the bowel lumen such as a polyp) occurs in approximately 10% of intussusceptions. This is most often found in children older than 2 years. Possible lead points include Meckel’s diverticulum, polyps, an inflamed appendix, neoplastic lesions, and ileal duplications.

Answer 15

1. per rectal examination 2. Abdominal XR 3. Abdominal USS Per rectal examination often reveals blood on the gloved finger which is highly suggestive of the diagnosis. AXR may show air fluid levels. Ultrasound scan (Fig 1) will confirm diagnosis showing the “donut”, sign when the intussusceptions are viewed end on. A referral to the paediatric surgeons should not be delayed in order to get a scan done.

Answer 16

1. peritonitis on examination 2. failure of radiological air reduction 3. collapse and shock

Answer 17

Air is introduced under pressure through a rectal catheter. A manometer monitors the pressure and this should not exceed 110mmHg. The air pressure meeting the intussusception usually forces the inverted bowel back through the ileo-caecal valve and into its expected position. This is visualised under fluoroscopic (X-ray) control. If reduction fails surgical reduction is required. The success rate is 50-85% depending on factors such as duration of obstruction (more difficult if >48 hours since intussusception)

Answer 18

If there's evidence of perforation, peritonitis (eg Abdo x-ray), or Henoch-Schönlein purpura

Answer 19

-CF (hypertrophied mucosal glands) -haemophilia - HSP (Henoch-Schonlein purpura, (intestinal wall haematoma) -lymphoma -hypertrophied Peyer’s patches (enlarged mesenteric lymph nodes) following a respiratory infection or gastroenteritis may serve as the lead point.

Answer 20

<1 years male predominance

Answer 21

5months-3 years: Reduction by air insufflation via radiology older/very ill children with signs of perforation/peritonitis: operative treatment with removal of affected bowel segment & end-to-end anastomoses

Answer 22

A congenital condition where a section of the bile duct is narrowed or absent , preventing the excretion of conjugated bilirubin

Answer 23

Presents in first few weeks of life with prolonged jaundice (>2 weeks in term babies, >3 weeks in premature babies) Dark urine Pale stools -Appetite and growth disturbance (abnormal growth), however, may be normal in some cases Signs: -Hepatomegaly with splenomegaly -Cardiac murmurs if associated cardiac abnormalities present

Answer 24

Raised conjugated billirubin ERCP Cholangiogram

Answer 25

Common in neonates – usually resolve by 3 years If not resolved by 5 years can consider surgical repair Note: Umbilical hernias are more common in Down syndrome

Answer 26

Herniation of the abdominal viscera into the chest cavity due to incomplete formation of the diaphragm

Answer 27

Pulmonary hypoplasia + hypertension | Respiratory distress shortly

Answer 28

-regurgitation and vomiting -diarrhoea -urticaria, atopic eczema '-colic' symptoms: irritability, crying -wheeze, chronic cough -rarely angioedema and anaphylaxis may occur

Answer 29

D. The presence of the liver in the thoracic cavity is a poor prognostic factor for CDH The outcome or prognosis of a patient with CDH is largely dependent on 2 factors (1) Liver position (2) Lung-to-head ratio If the liver has herniated into the chest, the disease is more severe and there is lower chance of survival. The lung-to-head ratio is a numeric estimate of the size of the foetal lungs, dependent on the amount of lung visible. A ratio >1.0 reflects a better outcome.

Answer 30

4. 1640ml of 0.9% NaCl + 5% dextrose given in bags of 500ml and made up with 10mmol KCl per 500ml bag This is the correct choice of fluids routinely used in the paediatric setting. This option provides sufficient electrolytes and the volume has been calculated correctly. So for a 27kg patient the total fluid volume would be 1000ml + 500ml + 140ml = 1640ml total

Answer 31

EATL: enteropathy associated T cell lymphoma (& small bowel adenocarcinomas)

Answer 32

Rotavirus: most common cause of infantile gastroenteritis Norovirus: most common cause of viral infectious gastroenteritis in all ages in England and Wales

Answer 33

Anti-tissue transglutaminase (tTG) IgA antibody

Answer 34

villous atrophy and crypt cell hyperplasia and increased intraepithelial lymphocytes -Two eight or not to it (DQ2 and DQ8)

Answer 35

Total IgA should also be tested simultaneously as 2% of patients with coeliac disease are IgA deficient and will therefore have a false negative anti-TTG IgA antibody test.

Answer 36

Gastroschisis describes a congenital defect in the anterior abdominal wall just lateral to the umbilical cord. Management vaginal delivery (i in vaginal and in immediate repair) may be attempted newborns should go to theatre as soon as possible after delivery, e.g. within 4 hours In exomphalos (also known as an omphalocoele) the abdominal contents protrude through the anterior abdominal wall but are covered in an amniotic sac formed by amniotic membrane and peritoneum. Associations -Beckwith-Wiedemann syndrome -Down's syndrome -cardiac and kidney malformations Management caesarean section (ECS: e in omphalocoele and e in staged repair) is indicated to reduce the risk of sac rupture a staged repair may be undertaken as primary closure may be difficult due to lack of space/high intra-abdominal pressure if this occurs the sacs is allowed to granulate and epithelialise over the coming weeks/months this forms a 'shell' as the infant grows a point will be reached when the sac contents can fit within the abdominal cavity. At this point the shell will be removed and the abdomen closed

Answer 37

B. Commence broad spectrum antibiotics This scenario describes a case of necrotising enterocolitis. Given the history and examination along with the age and prematurity of the infant, bacterial necrotising enterocolitis is the most likely diagnosis. Due to the seriousness of this, broad spectrum antibiotics must be commenced immediately. For this reason, answer 2 is the correct answer. Although necrotising enterocolitis is seen more often in bottle fed infants, changing feeds at this stage is futile. IV fluids are important to maintain hydration but not as urgently needed in this case as antibiotics. Erythromycin is given antenatally to prevent necrotising enterocolitis but is not useful in treatment.

Answer 38

B. Commence broad spectrum antibiotics This scenario describes a case of necrotising enterocolitis. Given the history and examination along with the age and prematurity of the infant, bacterial necrotising enterocolitis is the most likely diagnosis. Due to the seriousness of this, broad spectrum antibiotics must be commenced immediately. For this reason, answer 2 is the correct answer. Although necrotising enterocolitis is seen more often in bottle fed infants, changing feeds at this stage is futile. IV fluids are important to maintain hydration but not as urgently needed in this case as antibiotics. Erythromycin is given antenatally to prevent necrotising enterocolitis but is not useful in treatment.

Answer 39

-dilated bowel loops (often asymmetrical in distribution) -bowel wall oedema -pneumatosis intestinalis (intramural gas) -portal venous gas -pneumoperitoneum resulting from perforation air both inside and outside of the bowel wall (Rigler sign) -air outlining the falciform ligament (football sign)

Answer 40

1. Very low birth weight or very premature 2. Formula feeds (it is less common in babies fed by breast milk feeds) 3. Respiratory distress and assisted ventilation 4. Sepsis 5. Patient ductus arteriosus and other congenital heart disease

Answer 41

-Neonates with suspected NEC need to be nil by mouth with IV fluids, total parenteral nutrition (TPN) and antibiotics (10-14 days) to stabilise them. -early intubation, inotropic support -A nasogastric tube can be inserted to drain fluid and gas from the stomach and intestines. -"drip and suck”: 1. Nil by mouth (don’t put food or fluids in if there is a blockage) 2. IV fluids to hydrate the patient and correct electrolyte imbalances 3. NG tube with free drainage to allow stomach contents to freely drain and reduce the risk of vomiting and aspiration NEC is a surgical emergency and requires immediate referral to the neonatal surgical team. Some neonates will recover with medical treatment. In others, surgery may be required to remove the dead bowel tissue. Babies may be left with a temporary stoma if significant bowel is removed (laborotomy --> bowel resection-->stoma)

Answer 42

-Perforation and peritonitis -Sepsis -Death -Strictures -Abscess formation -Recurrence -Long term stoma -Short bowel syndrome after surgery -neurological deficit (gut brain axis; ie how gut microbioma can affect brain)

Answer 43

* Intolerance to feeds * Vomiting, particularly with green bile * Generally unwell * Distended, tender abdomen * Absent bowel sounds * Blood in stools When perforation occurs there will be peritonitis and shock and the neonate will be severely unwell.

Answer 44

Blood tests: 1. Full blood count for thrombocytopenia and neutropenia 2. CRP for inflammation 3. Capillary blood gas will show a metabolic acidosis 4. Blood culture for sepsis Imaging: 1. Supine Abdominal xray is the investigation of choice for diagnosis. (lying face up). - Additional views can be helpful, such as lateral (from the side with the patient on their back) and lateral decubitus (from the side with the neonate on their side).

Answer 45

6% of babies, when it happens 50% of babies will need surgery due to bowel surgery, if surgery; 1 in 3 die)

Answer 46

1. FBC and film 2. Blood Group and DAT/Coombs test 3. Bilirubin measurement.

Answer 47

ong term sequelae including cerebral palsy and deafness

Answer 48

1. phototherapy 2. IV Ig 3. Exchange transfusion Phototherapy should be commenced swiftly to control the progression of early onset neonatal jaundice. Repeat the bilirubin frequently, usually every 4-6h. If concerns remain, IV fluids may be started and immunoglobulins prescribed in order to prevent the need for an exchange transfusion, which is associated with significant risks and side effects.

Answer 49

folic acid Folic acid supplements may be used to help prevent later neonatal anaemia. Haemolysis stimulates high red cell turnover which may deplete folate. Folic acid replacement reduces this risk. Total iron should be stable as this is recycled from haemolysed red cells. Transfusion should be avoided if possible since this is associated with risks and is only necessary if there are clinical signs due to severe anaemia. Vitamin supplements are unnecessary.

Answer 50

A. Intestinal malrotation There are many causes of bilious vomiting in a newborn; however, this case's radiological findings and treatment are consistent with intestinal malrotation, which has led to volvulus. Intestinal malrotation is where the midgut rotates and fixes in an abnormal position while the baby is developing in the womb. This abnormal position makes it more likely for the bowel to twist on itself and cut off its own blood supply. This is known as volvulus. Ladd's procedure is where the surgeon straightens out the bowel and divides any abnormal peritoneal bands (Ladd bands). Not E: Duodenal atresia Duodenal atresia is another congenital malformation, which classically causes a 'double bubble' sign on abdominal x-ray. Not D: NEC Necrotising enterocolitis (NEC) is a common gastrointestinal condition in preterm neonates. The main investigation in NEC is a supine abdominal x-ray which shows dilated asymmetrical bowel loops, bowel wall oedema with 'thumbprinting' and pneumatosis intestinalis (gas within the bowel wall).

Answer 51

bilious vomiting, a distended abdomen and failure to pass meconium within the first 12–24 hours of life.

Answer 52

the obstruction may leave to bowel perforation, peritonitis , malrotation of the bowel and intestinal atresia.

Answer 53

bilious vomiting, bloody stools, absent bowel sounds, systemic compromise with metabolic acidosis

Answer 54

GORD, overfeeding, incomplete burping following feeds, food allergies (children with colic usually have accelerated growth eg weight gain)

Answer 55

* Describes a common (40% of babies in the first few months of life) symptom complex  manifests as random inconsolable crying and drawing up on the hands and feet  resolves by 3-12 months * Management: o Soothe infant – hold with gentle motion, optimal winding technique, white noise o If persistent → consider cow’s milk protein allergy or reflux,  consider:  (1) 2-week trial of whey hydrolysate formula; followed by  (2) 2-week trial of anti-reflux treatment o Support:  Self-help support group www.cry-sis.org.uk for families with excessive crying or sleepless children  Get support from health visitor, family, friends and other parents

Answer 56

Definition: defined as crying/fussing for ≥3 hours per day on ≥3 days in any one week In infants (peak=6 weeks), seen mostly in the evenings, associated with excessive high-pitched crying (usually resolves by 3 months. presentation >3months more sinister eg intussusception)

Answer 57

Type 1: The proximal ducts are patent, however, the common duct is obliterated Type 2: There is atresia of the cystic duct and cystic structures are found in the porta hepatis Type 3: There is atresia of the left and right ducts to the level of the porta hepatis, this occurs in >90% of cases of biliary atresia

Answer 58

1. Serum bilirubin including differentiation into conjugated and total bilirubin: Total bilirubin may be normal, whereas conjugated bilirubin is abnormally high 2. Liver function tests (LFTs) including serum bile acids and aminotransferases are usually raised but cannot differentiate between biliary atresia and other causes of neonatal cholestasis 3. Serum alpha 1-antitrypsin (autosomal recessive) : Deficiency may be a cause of neonatal cholestasis 4. Sweat chloride test: Cystic fibrosis often involves the biliary tract 5. Ultrasound of the biliary tree and liver: May show distension and tract abnormalities 6. Percutaneous liver biopsy with intraoperative cholangioscopy

Answer 59

condition where the bile ducts inside or outside the liver are blocked or absent, is a procedure called a "Hepatobiliary Scintigraphy" or "cholescintigraphy" or "HIDA scan: hepatobiliary iminodiacetic acid .": more specific scan than USS or CT A HIDA scan involves injecting a radioactive tracer into the bloodstream, which is then taken up by the liver and excreted into the bile ducts. The tracer allows for visualization of the flow of bile through the liver and bile ducts. In biliary atresia, the tracer will not reach the intestines due to the obstruction, indicating a blockage in the biliary system. During the scan, images are taken to assess the tracer's flow and identify any abnormalities. If the scan shows no evidence of bile reaching the intestines, it strongly suggests biliary atresia. However, a liver biopsy is usually required for confirmation.

Answer 60

1. Surgical intervention is the only definitive treatment for biliary atresia: Intervention may include dissection of the abnormalities into distinct ducts and anastomosis creation -The “Kasai portoenterostomy” involves attaching a section of the small intestine to the opening of the liver, where the bile duct normally attaches. This is somewhat successful and can clear the jaundice and prolong survival. 2. Medical intervention includes antibiotic coverage and bile acid enhancers following surgery Complications: 1. Unsuccessful anastomosis formation 2. Progressive liver disease 3. Cirrhosis with eventual hepatocellular carcinoma Prognosis: 1. Prognosis is good if surgery is successful 2. In cases where surgery fails, liver transplantation may be required in the first two years of life (Often patients require a full liver transplant to resolve the condition)

Answer 61

-Meconium ileus (CF) -Hirschsprung’s disease (Down's) -Oesophageal atresia -Duodenal atresia (down's; double bubble) -Intussusception -Imperforate anus -Malrotation of the intestines with a volvulus -Strangulated hernia

Answer 62

1. Persistent vomiting. This may be bilious, containing bright green bile. 2. Abdominal pain and distention 3. Failure to pass stools or wind 4. Abnormal bowel sounds. These can be high pitched and “tinkling” early in the obstruction and absent later.

Answer 63

The initial investigation of choice is an abdominal xray. This may show dilated loops of bowel proximal to the obstruction and collapsed loops of bowel distal to the obstruction. There will also be absence of air in the rectum.

Answer 64

Patients presenting with intestinal obstruction need to be referred to a paediatric surgical unit as an emergency. "DRIP AND SUCK" Initial management involves making them nil by mouth and inserting a nasogastric tube to help drain the stomach (suck) and stop the vomiting. They will also require IV fluids to correct any dehydration and electrolyte imbalances, and keep them hydrated (drip) while waiting for definitive management of the underlying cause

Answer 65

It is normal for babies to have some reflux after larger feeds. It becomes more troublesome when this causes them to become distressed. Signs of problematic reflux include: 1. Chronic cough 2. Hoarse cry 3. Distress, crying or unsettled after feeding 4. Reluctance to feed 5. Pneumonia 6. Poor weight gain Children over one year may experience similar symptoms to adults, with heartburn, acid regurgitation, retrosternal or epigastric pain, bloating and nocturnal cough.

Answer 66

o Referral (SAME DAY) referral if haematemesis, melaena or dysphagia present - Assessment by paediatrician if: * Red flag symptoms *Faltering growth * Unexplained distress *Unresponsive to medical therapy * Feeding aversion *Unexplained IDA * No improvement after 1 year of age *Suspected Sandifer's syndrome  Refer if there are complications: * Recurrent aspiration pneumonia *Unexplained apnoea *Unexplained epileptic seizure-like events *Unexplained upper airway inflammation *Dental erosion with neurodisability *Recurrent acute otitis media

Answer 67

1. Conservative measures:  Initial Management (no positional management – MUST sleep on back): * If breast-fed: o 1st : breastfeeding assessment (30 degree head up, Burping regularly to help milk settle, tricycling legs) o 2nd : consider trial of alginate for 1-2 weeks o 3rd : pharmacological * If formula-fed: o 1st : review feeding history o 2nd : trial smaller, more frequent feeds (aim for 150-180 mL/kg/day) o 3rd : trial of thickened formula (e.g. containing rice starch --> Enfamil, Carabel) o 4th : trial of alginate therapy (stop periodically to see if infant has recovered) o 5th : pharmacological  Pharmacological Management (only done in certain circumstances): * 4-week PPI/H2 antagonist trial – in children unable to describe symptoms or ≥1 of: o Unexplained feeding difficulties (refusing feeds, choking) o Distressed behaviour o Faltering growth o Children complaining of persistent heartburn, retrosternal or epigastric pain * Mnemonic (order): o G Gaviscon (a form of alginate therapy) o O Omeprazole o R Ranitidine o D Dunno, so refer to get metoclopramide Rarely in severe cases they may need further investigation with a barium meal and endoscopy. Surgical fundoplication can be considered in very severe cases, however this is very rarely required or performed. This usually improves as they grow and 90% of infants stop having reflux by 1 year.

Answer 68

o Explain diagnosis (due to immaturity of the gullet leading to food coming back the wrong way) o Reassure that this is common and usually gets better with time o Breastfeeding: offer assessment ± alginate therapy o Formula: review feeding history  smaller, more frequent feeds  thickeners ± alginate therapy o Safety net: keep an eye on the vomitus (if it’s blood-stained or green seek medical attention)

Answer 69

most will need around 150 to 200ml per kilo of their weight a day until they're 6 months old.)

Answer 70

Vomiting is very non-specific and is often not indicative of underlying pathology. Some of the possible causes of vomiting include: 1. Overfeeding 2. Gastro-oesophageal reflux 3. Pyloric stenosis (projective vomiting) 4. Gastritis or gastroenteritis 5. Appendicitis 6. Infections such as UTI, tonsillitis or meningitis Intestinal obstruction 7. Bulimia

Answer 71

1. Not keeping down any feed (pyloric stenosis or intestinal obstruction) 2. Projectile or forceful vomiting (pyloric stenosis or intestinal obstruction) 3. Bile stained vomit (intestinal obstruction) 4. Haematemesis or melaena (peptic ulcer, oesophagitis or varices) 5. Abdominal distention (intestinal obstruction) 6. Reduced consciousness, bulging fontanelle or neurological signs (meningitis or raised intracranial pressure) 7. Respiratory symptoms (aspiration and infection) 8. Blood in the stools (gastroenteritis or cows milk protein allergy) 9. Signs of infection (pneumonia, UTI, tonsillitis, otitis or meningitis) 10. Rash, angioedema and other signs of allergy (cows milk protein allergy) 12. Apnoeas are a concerning feature and may indicate serious underlying pathology and need urgent assessment

Answer 72

1. Small, frequent meals 2. Burping regularly to help milk settle 3. Not over-feeding 4. Keep the baby upright after feeding (i.e. not lying flat), 30 degree angle

Answer 73

This is a rare condition causing brief episodes of abnormal movements associated with gastro-oesophageal reflux in infants. The infants are usually neurologically normal. The key features are: 1. Torticollis: forceful contraction of the neck muscles causing twisting of the neck 2. Dystonia: abnormal muscle contractions causing twisting movements, arching of the back or unusual postures The condition tends to resolve as the reflux is treated or improves. Generally the outcome is good. It is worth referring patients with these symptoms to a specialist for assessment, as the differential diagnosis includes more serious conditions such as infantile spasms (West syndrome) and seizures.

Answer 74

It frequently contains heterotopic tissue; when it does, gastric mucosa accounts for 50%. The so called “rules of 2” states that the Meckel diverticulum occurs in about 2% of the population, is about 2 inches in length, is usually located within 2 feet of the ileocecal valve, and usually presents before 2 years of age.

Answer 75

1. Loss of appetite (anorexia) 2. Nausea and vomiting 3. Rovsing’s sign (palpation of the left iliac fossa causes pain in the RIF) 4. Guarding on abdominal palpation 5. Rebound tenderness is increased pain when quickly releasing pressure on the right iliac fossa 6. Percussion tenderness is pain and tenderness when percussing the abdomen 7. Rebound tenderness and percussion tenderness suggest peritonitis, caused by a ruptured appendix.

Answer 76

Bedside: - pregnancy test (if female) -Clinical (watchful waiting observation) Bloods: -raised inflammatory markers, WCC Imaging: o AXR ± CTAP Performing a CT scan can be useful in confirming the diagnosis, particularly where another diagnosis is more likely. o USS: An ultrasound scan is often used in female patients to exclude ovarian and gynaecological pathology. When a patient has a clinical presentation suggestive of appendicitis but investigations are negative, the next step is to perform a diagnostic laparoscopy to visualise the appendix directly. The surgeon can then proceed to an appendicectomy during the same procedure if indicated.

Answer 77

1. Ectopic Pregnancy Consider ectopic pregnancy in girls of childbearing age. This is a gynaecological emergency with a relatively high mortality if mismanaged. A serum or urine bHCG (pregnancy test) to exclude pregnancy is essential in adolescent girls. 2. Ovarian Cysts Ovarian cysts can cause pelvic and iliac fossa pain, particularly with rupture or torsion. 3. Meckel’s Diverticulum Meckel’s diverticulum is a malformation of the distal ileum that occurs in around 2% of the population. It is usually asymptomatic, however it can bleed, become inflamed, rupture or cause a volvulus or intussusception. They are often removed prophylactically if identified incidentally during other abdominal operations. 4. Mesenteric Adenitis Mesenteric adenitis describes inflamed abdominal lymph nodes. This presents with abdominal pain, usually in younger children. This is often associated with tonsillitis or an upper respiratory tract infection. No specific treatment is required. 5. Appendix Mass An appendix mass occurs when the omentum surrounds and sticks to the inflamed appendix, forming a mass in the right iliac fossa. This is typically managed conservatively with supportive treatment and antibiotics, followed by appendicectomy once the acute condition has resolved.

Answer 78

Patients with suspected appendicitis need emergency admission to hospital under the surgical team. Older children, for example those aged above 10 years, can often be managed by adult general surgical teams at local hospitals, provided there is a paediatric department in the hospital. Younger children will need to be admitted under paediatric surgeons. Removal of the inflamed appendix (appendicectomy) is the definitive management for acute appendicitis. Laparoscopic surgery is associated with fewer risks and faster recovery compared to open surgery (laparotomy). – GAME o GAME – G (Group & Save) - A (ABx IV) -M (MRSA screen) -E (Eat & drink – must be NBM) o Appendicectomy Complications of Appendicectomy 1. Bleeding, infection, pain and scars 2. Damage to bowel, bladder or other organs 3. Removal of a normal appendix 4. Anaesthetic risks 5. Venous thromboembolism (deep vein thrombosis or pulmonary embolism)

Answer 79

To prevent the infection spreading, stay at home for 48 hours (school exclusion) until the infection resolves

Answer 80

C. Malrotation with volvulus

Answer 81

C. Polyethylene glycol and electrolytes

Answer 82

If there is impaction (Feacolith: A mass of dry, hard stool that cannot pass out of the colon or rectum) : ERIC disimpaction regime

Answer 83

B. Reassure with dietary advice (probably toddler's diarrhoea)

Answer 84

Gastrointestinal symptoms: 1. Bloating and wind 2. Abdominal pain 3. Diarrhoea 4. Vomiting General allergic symptoms in response to the cow’s milk protein: 1. Urticarial rash (hives) 2. Angio-oedema (facial swelling) 3. Cough or wheeze 4. Sneezing 5. Watery eyes 6. Eczema Rarely in severe cases anaphylaxis can occur.

Answer 85

Cow’s milk intolerance is different from cow’s milk protein allergy. It is important not to get these mixed up. Cow’s milk intolerance presents with the same gastrointestinal symptoms as cow’s milk allergy (bloating, wind, diarrhoea and vomiting), however it does not give the allergic features (rash, angio-oedema, sneezing and coughing). Infants with cow’s milk allergy will not be able to tolerate cow’s milk at all, as it causes an allergic reaction, whereas infants with cow’s milk intolerance will be able to tolerate and continue to grow and develop, but will suffer with gastrointestinal symptoms whilst having cow’s milk. Infants with cow’s milk intolerance will grow out of it by 2 – 3 years. They can be fed with breast milk, hydrolysed formulas and weaned to foods not containing cow’s milk. After one year of age they can be started on the milk ladder.

Answer 86

CMPA involves hypersensitivity (type 1) to the protein in cow’s milk. This may be IgE mediated, in which case there is a rapid reaction to cow’s milk, occurring within 2 hours of ingestion. It can also be non-IgE medicated, with reactions occurring slowly over several days. This is different to lactose intolerance and cow’s milk intolerance. People with cow’s milk protein allergy do not have an allergy to lactose. Lactose is a sugar, not a protein. Cow’s milk intolerance is not an allergic process and does not involve the immune system. Cow’s milk protein allergy is more common in formula fed babies and those with a personal or family history of other atopic conditions. Diagnosis is often clinical (e.g. improvement with cow's milk protein elimination). Investigations include: 1. skin prick/patch testing 2. total IgE and specific IgE (RAST:radioallergosorbent test) for cow's milk protein

Answer 87

2. 1st  Trial cows' milk elimination from diet for 2-6 weeks: 1. Breastfed Babies: mother to exclude cow’s milk protein from her diet * Consider prescribing daily supplement of 1g of calcium and 10 mcg of vitamin D * N.B. it takes 2-3 weeks to fully eliminate cow’s milk from breastmilk 2. Formula-fed Babies: replacement of cows' milk-based formula with hypoallergenic infant formula (e.g. extensively hydrolysed formula or amino acid formula  if SEVERE: use amino-acid based formula:AAF) 3. Weaned infants/older children: exclude cows' milk protein from their diet 3. 2nd  Regularly monitor growth, nutritional counselling with a paediatric dietician 4. 3rd  Re-evaluate tolerance to cows' milk protein (every 6-12 months)  re-introduce cows' milk protein into the diet  if tolerance is established, greater exposure of less processed milk is advised with 'Milk Ladder'

Answer 88

1. Explain the diagnosis (allergic reaction to one of the 5 proteins in the cow’s milk) 2. Explain that it is common (3-6% of infants) 3. Treatment is simple: avoid cows’ milk in maternal diet (breastfeeding) or switch to hypoallergenic formula 4. Many children grow out of it (review in 6-12m and consider re-introducing cows’ milk protein with milk ladder) 5. Advise regularly monitoring growth 6. SUPPORT: British Dietetic Association (BDA) has produced a useful fact sheet

Answer 89

E.coli (HUS) Salmonella : transmitted through poultry (chicken or egg ingestion) livestock, reptiles and pets. -viruses (eg norovirus, rotavirus) are less likely to cause bloody diarrhoea -. Giardia is a common condition it can be chronic and tends to cause diarrhoea and vomiting but not usually bloody stools.

Answer 90

A. Stool specimens for culture D. A full blood count (for thrombocytopenia in HUS) F. Blood film (for schistocytes in MAHA) G.Renal function with serum electrolytes (as HUS causes acute renal impairement; uraemia) Haemolytic Uraemic Syndrome is defined as a triad of: Thrombocytopenia Microangiopathic haemolytic anaemia Acute renal failure The commonest trigger is Escherichia coli 0157:H7 serotype causing bloody diarrhoea. This should be considered in any child coming with diarrhoea and developing pallor and jaundice. Therefore, to arrive at a diagnosis 1,4,6,7 tests are necessary. An erect abdominal X-ray will not help; it is looking for signs of perforation (gas under the diaphragm). Her abdominal examination did not have any clinical signs of an acute surgical abdomen. Blood cultures will help in cases of acute sepsis. This child does not appear to be acutely septic she was apyrexial. Urinary electrolytes are not helpful in making this diagnosis however they can be helpful in assessing hydration status and renal function.

Answer 91

There is massive hepatosplenomegaly. The enlarged liver and spleen are separate from the renal and psoas outlines. Extensive ascites would obscure the renal and splenic outlines which are clearly seen. 1. Leukaemia 2. Malaria 3. Thalassemia 4. Gaucher’s disease

Answer 92

Double bubble sign refers to the appearance of 2 gas-filled structures in the abdomen of infants on abdominal x-rays, indicating a distended stomach and duodenum. This is usually caused by duodenal obstruction, which has many aetiologies, the most common being duodenal atresia.

Answer 93

C: Abdominal ultrasound This child is likely to have mesenteric adenitis. This is the characteristic description of acute abdominal pain following an upper respiratory-tract infection. Ultrasound scans will show enlarged mesenteric lymph nodes and may also show mesenteric thickening. It is the first line in mesenteric adenitis, as it can be done quickly in a ward-based setting. Mesenteric adenitis is usually treated conservatively without medication or surgery. Moreover, an abdominal ultrasound can also further exclude appendicitis, which would be a valid differential diagnosis. Not A: FBC A blood test would not be able to locate the source of abdominal pain unlike an ultrasound test.

Paediatric Gastroenterology Flashcards

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