Case of the Year

1. Case of the Year Caroline Buckley

2. Maternal details • 21 years old, primigravida • O Rhesus Positive, antibody negative • Rubella Immune, Hep B, HIV negative • Non-smoker, no alcohol • No significant past medical history, scans normal

3. Labour & delivery • Spontaneous onset of labour at 41+3 weeks • Rupture of membranes 3 hours prior to delivery • No pyrexia in labour, no offensive liquor • Cephalic presentation, SVD • Apgar scores 9 & 10 • Birth weight 3.995kg

4. Newborn Examination Performed at nine hours of age • No concerns • Abdomen soft, not distended. Small soft ‘lump’, near umbilicus Not tender • Well baby, normal movements and tone

5. ‘….can you come and have a look at this baby. I’m sure it’s nothing, it’s not urgent. She’s well, she’s feeding, but there’s just something that doesn’t feel right when I felt her abdomen…’ midwife, low dependency delivery unit

6. What do you think ? is there anything you would like to know ?

7. What do we want to know..? • Pink, alert and active • Feeding - formula via bottle, some mucousy vomits • Bowels not opened yet (but nine hours old)

8. What do you think ? What would you like to do ?

9. Things to think about • Review full history – ensure that no meconium was passed prior to delivery • Examine notes for any evidence of polyhydramniosor other abnormalities on antenatal scans, e.g. dilated loops of bowel • Anus appeared to be patent on examination

10. Our plan… • Continue to demand feed • Observe closely, await passage of meconium • Review in the morning – if passed meconium by then and remains well, can go home. • Contact the neonatal team if any concerns…

11. …in the morning Overnight, baby had fed …. but bowels not opened ! Baby is now 18 hours of age

12. What do you think ? When should you start to be concerned ?

13. On examination… • Baby awake, alert, rooting • Abdomen now distended and tense • Green bilious vomit as soon as baby handled

14. Abdominal distension • Can be moderate or extreme • Suggests an intestinal obstruction or intra-abdominal mass

15. Bilious vomiting is always pathological Never ignore it

16. Intestinal obstruction Infant presents with some or all of the following features: • Bile stained vomiting • Abdominal distension • Visible peristalsis • Delayed passage of meconium • Dehydration

17. Classification of intestinal obstruction Classified depending on the site of the blockage (large or small bowel), or whether this is anatomical or functional • Pyloric stenosis • Duodenal obstruction • Anorectal malformations • Hirchsprungs disease • Meconium ileus

18. Our plan… • Admit to the NNU immediately • Nil by Mouth • Nasogastric tube • Abdominal x-ray • IV fluids

19. Abdominal x-ray

20. meconium ileus

21. Meconium ileus • Bowel obstruction caused by highly viscid meconium within the lumen • Cystic Fibrosis (CF) is almost always the cause • Around 15% of infants with CF will present in this way

22. Meconium Ileus Clinical features: • Antenatal history of echogenic bowel on USS • Family history of CF or parental CF carrier status • Most common site of obstruction is in the distal ileum • Progressively worsening bilious vomiting • Abdominal distension • Palpable and sometimes visible bowel loops • Palpable abdominal mass • Failure to pass meconium

23. then what happened… • Baby transferred to the RVI urgently • Bowel surgery performed that day – formation of a stoma • Sweat test and genetic testing for Cystic Fibrosis …positive

24. Inheritance of cystic fibrosis

25. Remember...! • You would expect all babies to pass meconium within 24 hours of birth • Bile stained vomiting is always pathological • Abdominal distension might suggest an intestinal obstruction or intra-abdominal mass

26. Thank you for listening !