Paediatric_Gastrointestinal_Disorders_Flashcards
What is pyloric stenosis and how is it treated?
Pyloric stenosis involves projectile non-bile stained vomiting at 4-6 weeks of life. Treatment is Ramstedt pyloromyotomy.
What are the features and typical age for appendicitis in children?
Appendicitis is uncommon under 3 years and may present atypically when it does occur.
What is the management for mesenteric adenitis?
Mesenteric adenitis involves central abdominal pain and an upper respiratory tract infection, managed conservatively.
Describe intussusception and its treatment.
Intussusception is a condition where part of the bowel telescopes into another section. It is treated with reduction via air insufflation.
What complications are associated with intestinal malrotation?
Complications include volvulus, with treatment involving laparotomy and a Ladd’s procedure if volvulus is present.
How is Hirschsprung’s disease diagnosed and treated?
Hirschsprung’s disease is diagnosed with a full-thickness rectal biopsy. Treatment involves rectal washouts and an anorectal pull-through procedure.
What are the signs of oesophageal atresia in newborns?
Signs include choking and cyanotic spells following aspiration, often associated with tracheo-oesophageal fistula.
What is the relationship between meconium ileus and cystic fibrosis?
Meconium ileus is associated with cystic fibrosis, characterized by delayed passage of meconium and abdominal distension.
What are the indications for a Kasai procedure in infants?
The Kasai procedure is performed urgently for biliary atresia, indicated by jaundice persisting more than 14 days.
What are the early signs and treatment for necrotising enterocolitis?
Early signs include abdominal distension and bloody stools. Treatment involves total gut rest and TPN; laparotomy is needed for perforations.
A 1-year-old child is brought to the emergency department by his parents. The parents have noticed that he is clutching his stomach. He has not eaten or drank any fluids for the entire day and he has vomited twice. His mother states the vomit was green in colour.
The doctor is suspecting a diagnosis of intestinal malrotation and the abdominal ultrasound shows a whirlpool sign. On examination, the child looks distressed. An abdominal examination shows a distended abdomen and there are absent bowel sounds. He looks unwell.
What is the correct management for this patient?
Kasai procedure
Ladd’s procedure
Ramstedt pyloromyotomy
Rectal washouts
Reduction with air insufflation
Ladd’s procedure
Paediatric intestinal malrotation with volvulus → Ladd’s procedure (includes division of Ladd bands and widening of the base of the mesentery)
The correct answer is Ladd’s procedure. As described in the stem, the patient is presenting with intestinal malrotation accompanied by volvulus. Normally, the midgut herniates during development, rotating 90° in a counter-clockwise direction outside the body. After, it re-enters the abdominal cavity and completes another 180° rotation, for a total of 270°. This allows the cecum to end up in the right upper quadrant and consequently descend into the lower right quadrant.
But sometimes, this process does not complete fully and the caecum remains fixed in the right upper quadrant by peritoneal bands, denominated Ladd bands. This increases the chances of the formation of a volvulus, as happened in this case. The correct management is Ladd’s procedure, which involves the untwisting of the volvulus and the removal of Ladd’s bands, resection of necrotic bowel if present and removal of the appendix, to prevent future operations.
Kasai procedure is used to manage cases of biliary atresia, rather than malrotation. It involves creating a connection between the liver and the small intestine to allow for bile drainage.
Ramstedt pyloromyotomy is used to manage pyloric stenosis. It consists of a longitudinal incision of the hypertrophic sphincter to split the muscle and allow the passage of food.
Rectal washouts are used as the initial management of Hirschsprung’s disease, as they help clean and decompress the bowel, even if these are not part of the definite management.
Reduction with air insufflation is the treatment of choice for intussusception. It involves inflating air to try and reduce the telescoping bowel and manual reduction of the bowel.
summarise
Paediatric gastrointestinal disorders
Pyloric stenosis
M>F
5-10% Family history in parents
Projectile non bile stained vomiting at 4-6 weeks of life
Diagnosis is made by test feed or USS
Treatment: Ramstedt pyloromyotomy (open or laparoscopic)
Acute appendicitis
Uncommon under 3 years
When occurs may present atypically
Mesenteric adenitis
Central abdominal pain and URTI
Conservative management
Intussusception
Telescoping bowel
Proximal to or at the level of, ileocaecal valve
6-9 months of age
Colicky pain, diarrhoea and vomiting, sausage-shaped mass, red jelly stool.
Treatment: reduction with air insufflation
Intestinal malrotation
High caecum at the midline
Feature in exomphalos, congenital diaphragmatic hernia, intrinsic duodenal atresia
May be complicated by the development of volvulus, an infant with volvulus may have bile stained vomiting
Diagnosis is made by upper GI contrast study and USS
Treatment is by laparotomy, if volvulus is present (or at high risk of occurring then a Ladd’s procedure is performed (includes division of Ladd bands and widening of the base of the mesentery)
Hirschsprung’s disease
Absence of ganglion cells from myenteric and submucosal plexuses
Occurs in 1/5000 births
Full-thickness rectal biopsy for diagnosis
Delayed passage of meconium and abdominal distension
Treatment is with rectal washouts initially, after that an anorectal pull through procedure
Oesophageal atresia
Associated with tracheo-oesophageal fistula and polyhydramnios
May present with choking and cyanotic spells following aspiration
VACTERL associations
Meconium ileus
Usually delayed passage of meconium and abdominal distension
The majority have cystic fibrosis
X-Rays will not show a fluid level as the meconium is viscid, PR contrast studies may dislodge meconium plugs and be therapeutic
Infants who do not respond to PR contrast and NG N-acetyl cysteine will require surgery to remove the plugs
Biliary atresia
Jaundice > 14 days
Increased conjugated bilirubin
Urgent Kasai procedure
Necrotising enterocolitis
Prematurity is the main risk factor
Early features include abdominal distension and passage of bloody stools
X-Rays may show pneumatosis intestinalis and evidence of free air
Increased risk when empirical antibiotics are given to infants beyond 5 days
Treatment is with total gut rest and TPN, babies with perforations will require laparotomy
A 2-year-old child is referred by the GP for treatment-resistant chronic constipation. Which of the following features in the history would help point to a diagnosis of Hirschsprung’s disease?
Family history of multiple endocrine neoplasia type 1 (MEN1)
Passage of meconium at day 3
Family history of cystic fibrosis
Non-bilious vomiting
The child has a temperature of 39ºC
Passage of meconium at day 3
Passage of meconium after 48 hours is a red flag
Meconium normally passes within the first 24 hours. Delayed passage (specifically >48 hours) is a red flag associated with Hirschsprung’s disease (Around 50% of babies with delayed passage have the disease).
As the disease can present in later childhood, you should always inquire about the timing in children presenting with symptoms of chronic constipation or obstruction.
Hirschsprung’s disease is associated with MEN 2A/B and not MEN1
Cystic fibrosis is associated with meconium ileus, one of the main differentials for Hirschsprung’s disease
Non-bilious vomiting would be associated with pyloric stenosis
A temperature would not be a factor suggesting Hirschsprung’s disease
A two-day-old baby who has not passed meconium now has a distended abdomen and is vomiting green bile. It is suspected that he may have a congenital condition affecting the rectum. Which of the following tests is diagnostic?
Abdominal ultrasound
Abdominal x-ray
Rectal biopsy
Contrast enema
Colonoscopy
Rectal biopsy
This baby has Hirschsprung’s disease, a congenital abnormality causing the absence of ganglionic cells in the myenteric and submucosal plexuses. The condition occurs in 1/5000 births. Symptoms include delayed passage of meconium (> 2 days after birth), abdominal distension and bilious vomiting. Treatment is usually with rectal washouts initially, followed by an anorectal ‘pull-through procedure’. This involves removing the affected section of bowel and forming an anastomosis with the healthy colon.
Hirschsprungs disease could be suggested on abdominal x-ray, abdominal ultrasound and contrast enema. Some sections of the bowel may look very dilated and the affected section narrow. However, definitive diagnosis is with a rectal biopsy, where the tissue can be analysed under the microscope for the absence of ganglionic cells.
Theme: Neonatal gastrointestinal disease
A. Ano-rectal atresia
B. Pyloric stenosis
C. Hirschsprungs disease
D. Duodenal atresia
E. Meconium ileus
F. Intussusception
G. Necrotising enterocolitis
H. Intestinal volvulus
I. Tracheo-oesophageal fistula
Please select the most likely diagnosis to account for the case described. Each option may be used once, more than once or not at all.
- A newborn baby boy presents with mild abdominal distension and failure to pass meconium after 24 hours. X- Ray reveals dilated loops of bowel with fluid levels. The anus appears normally located.
- A premature infant (30-week gestation) presents with distended and tense abdomen. She is passing blood and mucus per rectum, and she is also manifesting signs of sepsis.
- A newborn baby boy presents with gross abdominal distension. He is diagnosed with cystic fibrosis and his abdominal x ray shows distended coils of small bowel, but no fluid levels.
Intestinal volvulus
Theme: Neonatal gastrointestinal disease
A. Ano-rectal atresia
B. Pyloric stenosis
C. Hirschsprungs disease
D. Duodenal atresia
E. Meconium ileus
F. Intussusception
G. Necrotising enterocolitis
H. Intestinal volvulus
I. Tracheo-oesophageal fistula
Please select the most likely diagnosis to account for the case described. Each option may be used once, more than once or not at all.
- A newborn baby boy presents with mild abdominal distension and failure to pass meconium after 24 hours. X- Ray reveals dilated loops of bowel with fluid levels. The anus appears normally located.
Hirschsprungs disease
Hirschsprung’s disease is an absence of ganglion cells in the neural plexus of the intestinal wall. It is more common in boys than girls. The delayed passage of meconium together with distension of abdomen is the usual clinical presentation. A plain abdominal x ray will demonstrate dilated loops of bowel with fluid levels and a barium enema can be helpful when it demonstrates a cone with dilated ganglionic proximal colon and the distal aganglionic bowel failing to distend.
- A premature infant (30-week gestation) presents with distended and tense abdomen. She is passing blood and mucus per rectum, and she is also manifesting signs of sepsis.
Necrotising enterocolitis
Necrotising enterocolitis is more common in premature infants. Mesenteric ischemia causes bacterial invasion of the mucosa leading to sepsis. Terminal ileum, caecum and the distal colon are commonly affected. The abdomen is distended and tense, and the infant passes blood and mucus per rectum. X -Ray of the abdomen shows distended loops of intestine and gas bubbles may be seen in the bowel wall.
- A newborn baby boy presents with gross abdominal distension. He is diagnosed with cystic fibrosis and his abdominal x ray shows distended coils of small bowel, but no fluid levels.
The correct answer is: Meconium ileus
One in 15,000 newborns will have a distal small bowel obstruction secondary to abnormal bulky and viscid meconium. Ninety percent of these infants will have cystic fibrosis and the abnormal meconium is the result of deficient intestinal secretions. This condition presents during the first days of life with gross abdominal distension and bilious vomiting. x Ray of the abdomen shows distended coils of bowel and typical mottled ground glass appearance. Fluid levels are scarce as the meconium is viscid.
