2. Disclosures
1. I am single.
2. I am straight.
3. I am available.
Recent tax return and health information available on request
3. Objectives
1. What is the primary underlying problem in patients with
malrotation and how can imaging best assess it?
2. What is the expected position of the DJJ
(duodenojejunal junction) or ligament of Treitz?
3. What are the most common errors leading to the
misdiagnosis of malrotation?
4. Objectives
4. What imaging modality is least useful in the evaluation of
malrotation?
a. Upper Gastrointestinal Series
b. Contrast Enema
c. Ultrasound
d. Computed Tomography
e. MRI
5. Who is Alexander Bill?
11. Ladd’s Bands
Two patterns of colon malposition with Ladd’s bands obstructing the duodenum
Ladd Procedure
Alexander Bill, a student of Ladd, modified the procedure by suturing the duodenum to the fascia
overlying the right kidney and the cecum to the left inner abdominal wall in an attempt to prevent
recurrent volvulus.
12. Epidemiology
Incidence: 1 in 6,000 to 1 in 200 live births
Autopsy findings suggest that 0.5 to 1% of the
population is affected
Male to Female ratio 2:1
75 % present in the first month of life
90 % present in the first year of life
70% of patients with heterotaxy have malrotation
13. Summary of Clinical Findings
Features of questionable malrotation include:
Asymptomatic/incidental findings in investigating for reflux
(fussiness, arching, apneic events, reactive airways, pneumonia)
Intermittent vomiting
No signs of acute illness.
Features of intermittent or partial volvulus or obstructing Ladd's bands include:
Intermittent vomiting
No signs of acute illness
Intermittent abdominal pain (typically post-prandial)
Weight loss.
14. Summary of Clinical Findings
Features of obstruction without ischemia (midgut volvulus without vascular
compromise) include:
Bilious vomiting
Crampy abdominal pain in waves
Non-tender abdomen
Non-distended abdomen
No severe physiological disturbance.
Features of obstruction with ischemia (midgut volvulus with vascular compromise)
include:
Acutely ill patient with severe acute abdominal pain
Bilious vomiting
Tachycardia
Tachypnea
Abdominal tenderness
Acidosis
Signs of peritoneal catastrophe (re-bound and guarding).
15. Syndromes Associated with Malrotation
Apple-peel Intestinal Atresia
Cornelia de Lange Syndrome
Cantrell Syndrome
Cat-eye Syndrome
Chromosomal abnormalities (Trisomies 13, 18, and 21)
Coffin-Siris Syndrome
Familial Intestinal Malrotation
Heterotaxy (asplenia, polysplenia)
Marfan Syndrome
Meckel Syndrome
Mobile Cecum Syndrome
Prune Belly Syndrome
16. Anatomic Anomalies Associated with Malrotation
Absence of kidney and ureter
Biliary atresia
Congenital diaphragmatic hernia
Duodenal or small-bowel stenosis or atresia
Duodenal web
Gastroschisis
Hirschsprung disease
Imperforate anus
Intestinal pseudo-obstruction
Intussusception
Malabsorption
Meckel’s diverticulum
Omphalocele
Pyloric Stenosis
25. Contrast Enema
80% of patients with malrotation demonstrate an abnormal
position of the cecum
20% of infants with malrotation have a normal cecal
position
15% of patients with normal rotation have a mobile cecum
Does not show the volvulus as well as the upper GI
28. Rules of the Upper GI
Evaluate the duodenum on the first upper GI on
every patient
Document the first pass of barium through the
duodenum
Document the duodenum in the frontal and
lateral projections
29. Performing the Upper GI
Concentrate on the duodenum
Control the barium
Moderate amounts, don’t over-distend the stomach
An enteric tube adds additional control
Turn the patient with purpose
Avoid placing the right side down until you are ready
to evaluate the duodenum
30. Performing the Upper GI
With the right side down allow moderate amount of
barium to pass into the duodenum
Once barium enters the duodenum quickly place the
patient flat to evaluate the duodenal sweep
Once the DJJ is documented in the frontal view
quickly turn the patient lateral to document it in this
projection
31. Normal position of the DJJ on Upper GI
Frontal view
DJJ is as cephalic as the duodenal bulb
DJJ is no further to the right than the left
pedicle of the adjacent vertebra
Lateral view
The 2nd portion of the duodenum descends
posteriorly in the retroperitoneum
The 4th portion of the duodenum ascends
posteriorly in the retroperitoneum just
anterior to the descent of the 2nd portion of
the duodenum
33. Normal variants mimicking malrotation
Frontal view shows the position of the duodenal bulb
(arrow), which is superimposed over the gastric antrum, and
inferior displacement of the duodenojejunal junction
(arrowhead)
Frontal view shows a similar inferior position of the
duodenojejunal junction (arrow); * = duodenal bulb.
44. Error and Pitfalls
Not seeing the first pass of barium
Oblique positioning in the AP projection
Mobility of the DJJ
45. What do we do when we
don’t know...
Show the case around to our colleagues
Follow the barium to the cecum
Turn the UGI into a small bowel followthrough
Do a contrast enema
Repeat the study in a few days
46. What do we do when we
don’t know...
Show the case around to our colleagues
Follow the barium to the cecum
Turn the UGI into a small bowel followthrough
Do a contrast enema
Repeat the study in a few days
Equivocate
54. 211 patients
135 Normal US
130 Normal UGI
5 Abnormal UGI
76 Abnormal US
44 Normal UGI
32 Abnormal UGI
Journal of Pediatric Surgery 2006;41:1005-1009
55. AP orientation of SMA and SMV
Positive predictive value 10%
Negative Predictive value 90.1 %
Inverted orientation of SMA and SMV
Positive predictive value 41.7%
Negative Predictive value 96.6 %
Whirlpool sign of SMA and SMV
Positive predictive value 71.4%
Negative Predictive value 97.1 %
56. One study found a false positive rate of 15%
among pediatric radiologists.
The ligament of Treitz is not a fixed position in
infants.
The underlying problem in malrotation is a short
root of the mesentery. In difficult cases the
length of the mesenteric pedicle can assessed by
determining the distance between the cecum and
the DJJ either by small bowel follow through or a
combination of an upper GI and a contrast
enema
57. Post test
1. What is the primary underlying problem in patients with
malrotation and how can imaging best assess it?
2. What is the expected position of the DJJ
(duodenojejunal junction) or ligament of Treitz?
3. What are the most common errors leading to the
misdiagnosis of malrotation?
58. Post test
4. What imaging modality is least useful in the evaluation of
malrotation?
a. Upper Gastrointestinal Series
b. Contrast Enema
c. Ultrasound
d. Computed Tomography
e. MRI
5. Who is Alexander Bill?
