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Paulo B.S. Tugbang
A three-month-old male with Down 
Syndrome (T21) presents with passage of 
bloody stools. Physical examination showed a 
sausage-showed mass on the abdomen upon 
palpation.
 AM is a three-month-old male baby boy, 
seen for the first time on August 26, 2014, 
accompanied by mother, presenting to the 
emergency department with a 36-hour 
history of non-bilious vomiting after every 
feed and one episode of blood-tinge per 
rectum.
AM, a known Down Syndrome baby, was previously 
healthy until 36 hours prior to consult, when his 
mother noted him to vomit all previously 
breastfed milk. This was accompanied by 
intermittent crying spells, decrease in appetite, 
decreased activity, and decreased sleep. 
The infant was taken to a local clinic, and was 
treated as a case of gastroenteritis, and oral 
rehydration therapy was started. He was reported 
to be passing urine but less than usual. 
When the symptoms did not improve, AM was 
brought to our institution for further 
management.
 No other relevant illnesses past or present 
 No recent history of abdominal trauma
 Family Hx is positive for history of DS on 
mother’s side (uncle)
 Born term via CS to a G2P0 mother 
 Regular prenatal checkup during pregnancy 
 Congenital scan at 6 months revealed DS 
 No maternal complications during childbirth
(-) head lag 
(+) rolls side to side 
(+) able to follow objects/turns head toward 
sound source 
(+) reacts to sound occasionally 
(-) no vocalizations 
(-) eye fixation 
(-) smile 
(+) Primitive reflexes (Moro, Babinski)
 Pure breastfeeding 
 Fed per demand 
 Weaning not started
 VS 37.6, 158, 36, 85/55, 4kg 
 CBG 6,7mmol/L, O2 Sat 99% 
 Not in CP distress 
 Awake, but lethargic, carried by mother 
 Pale, listless 
 Generally normal PE 
 Abdominal exam showed AM disliking the test 
 Draws up legs and crying while being examined 
 Abdomen is tender and distended, with a 
sausage shaped mass noted on the R 
hypogastrium 
 (+) passage of fresh mucousy blood per rectum 
resembling red currant jelly
 Intussusception prob. idiopathic
 Down syndrome (T21) is 
characterized by a variety of 
dysmorphic features, 
congenital anomalies, and 
associated medical 
conditions such as 
respiratory and 
cardiovascular system 
anomalies, GI anomalies, 
and lymphatic anomalies 
 Appropriate radiologic 
imaging with prompt, 
accurate interpretation 
plays an important role in 
the diagnosis and 
management of this 
diseases
 First described by Barbette of Amsterdam in 
1674 
 The commonest cause of acute intestinal 
obstruction in children below 2 years of age 
 Has a male predominance with a ratio of 4:1 
 If left untreated may be fatal in two to five 
days
Any other part Ileocolic
 Idiopathic in most cases 
 Two main theories: dietary theory and infective 
theory 
 May also occur in starvation, dehydration 2 to 
severe GE, cystic fibrosis, and sickle cell crisis 
 A lead point in the intestine allows the 
telescoping of the intussusceptum (proximal 
segment) into the intussuscipiens (adjacent distal 
segment), obstruction, strangulation, and 
mucosal bleeding occurs 
 A specific lead point is not found in 90% of cases
 Classic triad of acute abdominal pain, currant 
jelly stools or hematochezia, and palpable 
abdominal mass 
 History: infant, has had URTI presents with 
vomiting (initially non bilious and reflexive, with 
obstruction becomes bilious), abdominal pain 
(colicky, severe, intermittent), passage of blood 
and mucus (may also present with diarrhea), 
lethargy, and abdominal mass 
 PE: RHyp sausage-shaped mass and emptiness in 
the RLQ (Dance sign) (Abdominal distention when 
obstruction is complete)
Red currant jelly Stool
Target sign Pseudokidney sign
Gas-filled area Mass
Target sign (PR) Meniscus sign
Target sign (BE) Coiled spring sign
 Usually not helpful in evaluation 
 WBC count – leucocytosis may be an 
indication of gangrene 
 Electrolyte count – with persistent vomiting 
and sequestration of fluid in the obstructed 
bowel
 Division by age (by lead points) indicate 
treatment 
 Operative reduction 
 Non-operative reduction
 Not standard of care 
 Use of opioid analgesics and NSAIDs for pain 
control post operatively
Ultrasound

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Ultrasound

  • 2. A three-month-old male with Down Syndrome (T21) presents with passage of bloody stools. Physical examination showed a sausage-showed mass on the abdomen upon palpation.
  • 3.  AM is a three-month-old male baby boy, seen for the first time on August 26, 2014, accompanied by mother, presenting to the emergency department with a 36-hour history of non-bilious vomiting after every feed and one episode of blood-tinge per rectum.
  • 4. AM, a known Down Syndrome baby, was previously healthy until 36 hours prior to consult, when his mother noted him to vomit all previously breastfed milk. This was accompanied by intermittent crying spells, decrease in appetite, decreased activity, and decreased sleep. The infant was taken to a local clinic, and was treated as a case of gastroenteritis, and oral rehydration therapy was started. He was reported to be passing urine but less than usual. When the symptoms did not improve, AM was brought to our institution for further management.
  • 5.  No other relevant illnesses past or present  No recent history of abdominal trauma
  • 6.  Family Hx is positive for history of DS on mother’s side (uncle)
  • 7.  Born term via CS to a G2P0 mother  Regular prenatal checkup during pregnancy  Congenital scan at 6 months revealed DS  No maternal complications during childbirth
  • 8. (-) head lag (+) rolls side to side (+) able to follow objects/turns head toward sound source (+) reacts to sound occasionally (-) no vocalizations (-) eye fixation (-) smile (+) Primitive reflexes (Moro, Babinski)
  • 9.  Pure breastfeeding  Fed per demand  Weaning not started
  • 10.  VS 37.6, 158, 36, 85/55, 4kg  CBG 6,7mmol/L, O2 Sat 99%  Not in CP distress  Awake, but lethargic, carried by mother  Pale, listless  Generally normal PE  Abdominal exam showed AM disliking the test  Draws up legs and crying while being examined  Abdomen is tender and distended, with a sausage shaped mass noted on the R hypogastrium  (+) passage of fresh mucousy blood per rectum resembling red currant jelly
  • 11.
  • 13.  Down syndrome (T21) is characterized by a variety of dysmorphic features, congenital anomalies, and associated medical conditions such as respiratory and cardiovascular system anomalies, GI anomalies, and lymphatic anomalies  Appropriate radiologic imaging with prompt, accurate interpretation plays an important role in the diagnosis and management of this diseases
  • 14.  First described by Barbette of Amsterdam in 1674  The commonest cause of acute intestinal obstruction in children below 2 years of age  Has a male predominance with a ratio of 4:1  If left untreated may be fatal in two to five days
  • 15. Any other part Ileocolic
  • 16.  Idiopathic in most cases  Two main theories: dietary theory and infective theory  May also occur in starvation, dehydration 2 to severe GE, cystic fibrosis, and sickle cell crisis  A lead point in the intestine allows the telescoping of the intussusceptum (proximal segment) into the intussuscipiens (adjacent distal segment), obstruction, strangulation, and mucosal bleeding occurs  A specific lead point is not found in 90% of cases
  • 17.  Classic triad of acute abdominal pain, currant jelly stools or hematochezia, and palpable abdominal mass  History: infant, has had URTI presents with vomiting (initially non bilious and reflexive, with obstruction becomes bilious), abdominal pain (colicky, severe, intermittent), passage of blood and mucus (may also present with diarrhea), lethargy, and abdominal mass  PE: RHyp sausage-shaped mass and emptiness in the RLQ (Dance sign) (Abdominal distention when obstruction is complete)
  • 20.
  • 22. Target sign (PR) Meniscus sign
  • 23. Target sign (BE) Coiled spring sign
  • 24.  Usually not helpful in evaluation  WBC count – leucocytosis may be an indication of gangrene  Electrolyte count – with persistent vomiting and sequestration of fluid in the obstructed bowel
  • 25.  Division by age (by lead points) indicate treatment  Operative reduction  Non-operative reduction
  • 26.  Not standard of care  Use of opioid analgesics and NSAIDs for pain control post operatively

Editor's Notes

  1. Dietary: early weaning is a risk factor; occurs at the time of weaning Infective: Inflammation of the Peyer’s patches Anatomic lead point: a piece of intestinal tissue which protrudes into the bowel lumen
  2. 97-100% accuracy in detecting intussusception, with high degree of confidence On transverse sonography, seen as a round mass with alternating concentric rings of hyper- and hypoechogenicity representing bowel wall and mesenteric fat (target sign) On longitudinal ultrasonography, ovoid mass with alternating layers of hyper- and hypoechogenicity (pseudokidney sign)
  3. Not normally indicated unless lead point (lymphoma) is suspected Volvulus and intraperitoneal masses may mimic Seen as a soft tissue mass with alternating rings of high and low attenuation most commonly in the right upper quadrant
  4. Gas filled or fecal filled cecum allows exclusion of intussusception with a high degree of confidence Most common sign is a soft tissue mass in the RUQ
  5. Target sign- a soft tissue mass that contains a concentric circular areas of lucency, which are mesenteric fat of the intussusceptium Meniscus sign- crescent gas within the colonic lumen that outlines apex of the intussusceptium
  6. Classic signs are meniscus sign and coiled spring sign Coiled spring sign- edematous mucosal folds of the returning limb of the intussusceptum are outlined by contrast material in the lumen of the colon
  7. Below 3- idiopathic, no lead points Above 3- with lead points Non op- enema (barium, pneumatic), may be done with UTZ or fluoroscopic guidance