Feedback: Q6 A 4 week old child is brought to your emergency department with a distended abdomen.

Marking What six (6) questions would you ask to aide you with your diagnosis? (6 marks) passage meconium first 48 hours; vomiting history ? bilious; bowel opening history; tolerating feeds/ passing urine; distressed/ unwell; premature; significant PMH eg bowel surgery Needed to ask about passage meconium to get 6/6 question Not about Dx but about approach One point for Dx

Marking State two (2) positive and two (2) relevant negative findings on the AXR. (4 marks) XR +ve: dilated bowel loops (large and small) paucity of air in rectum XR-ve: No free air (football sign, rigler’s/ double wall sign) No pneumatosis intestinalis No double bubble sign

Marking What is the most likely diagnosis? (1 mark) Hirschsprungs Name two (2) differential diagnosis.(2 marks) causes bowel obstruction malrotation, imperforate anus, constipation, meconium plug/ ileus, incarcerated hernia, NEC

Marking State three management steps. (3 marks) Surgery referral, NBM, NGT on free drainage, iv access and fluids, analgesia if distressed

Hirschsprung Disease Absence of ganglion cells in bowel wall from anus proximally Delayed passage meconium (99% full term infants pass meconium in 48 hours) Chronic constipation Risk of enterocolitis if not Dx early AXR- obstruction and paucity gass rectum Rectal suction biopsy for Dx then definitive surgery

Malrotation Incomplete rotation of intestine as foetus Mesentery (including SMA) tethered by narrow stalk which can twist producing midgut volvulus Can also cause duodenal obstruction (Ladd bands) Present 1st year of life with about 40% presenting first week and 50% by first month Bilious emesis, bowel obstruction and significant abdominal pain (especially with volvulus)

Necrotizing Enterocolitis Newborn emergency- disease of the NICU Multifactorial Mucosal/ transmural necrosis of intestine Incidence and mortality increase with decreasing BW and GA 90% in premature infant Can be secondary disease- including Hirschsprung! Usually 2nd-3rd week of life but can be as late as 3 months in VLBW infants AXR- pneumatosis intestinalis

Hirschsprungs with pneumatosis intestinalis

Intussusception 2 months to 2 years (can occur any age) Peak incidence 5 to 9 months (weaning) Intermittent severe colicky abdo pain Typically 2-3/ hour and at least 1/hour Usually assoc with vomiting, pallor, lethargy Blood in stool is late sign Mass hard to feel

Intussusception: Imaging diagnostic investigation of choice Air enema: diagnostic and therapeutic AXR: only if concerned perforated or obstructed Target sign- 2 concentric circular radiolucent lines usually in RUQ Crescent sign- a crescent shaped lucency usually LUQ with a soft tissue mass

Perforation http://radiologymasterclass.co.uk/tutorials/abdo/abdomen_x-ray_abnormalities/pathology_bowel_gas_perforation.html#top_fifth_img If suspected consider left lateral decubitus film

Rigglers sign/ double wall sign

Football sign

Small Bowel vs Large Bowel Obstruction Small bowel tends to be central

Normal large bowel distribution with haustral folds