Jurnal 2
Jurnal 2
Radiology
Submit a Manuscript: http://www.wjgnet.com/esps/ World J Radiol 2014 September 28; 6(9): 730-736
Help Desk: http://www.wjgnet.com/esps/helpdesk.aspx ISSN 1949-8470 (online)
DOI: 10.4329/wjr.v6.i9.730 © 2014 Baishideng Publishing Group Inc. All rights reserved.
MINIREVIEWS
John J Tackett, Eleanor D Muise, Robert A Cowles, Section ily on clinical acumen and suspicion, radiologic imaging
of Pediatric Surgery, Department of Surgery, Yale University is critical in determining which patients need surgery.
School of Medicine, New Haven, CT 06520, United States Surgeons and radiologists must cooperate and commu-
Author contributions: Tackett JJ, Muise ED and Cowles RA nicate effectively during the radiographic evaluation of
contributed to this work and wrote the paper.
a child with malrotation. Additionally, the algorithm for
Correspondence to: Robert A Cowles, MD, Associate Pro-
imaging malrotation must be adapted based upon the
fessor of Surgery, Section of Pediatric Surgery, Department of
Surgery, Yale University School of Medicine, Box 208062, New tools and staff available at any given institution.
Haven, CT 06520, United States. robert.cowles@yale.edu
Telephone: +1-203-7852701 Fax: +1-203-7853820
Received: December 31, 2013 Revised: June 20, 2014 Tackett JJ, Muise ED, Cowles RA. Malrotation: Current
Accepted: July 17, 2014 strategies navigating the radiologic diagnosis of a surgical
Published online: September 28, 2014 emergency. World J Radiol 2014; 6(9): 730-736 Available from:
URL: http://www.wjgnet.com/1949-8470/full/v6/i9/730.htm DOI:
http://dx.doi.org/10.4329/wjr.v6.i9.730
Abstract
The most accurate and practical imaging algorithm for
the diagnosis of intestinal malrotation can be a complex INTRODUCTION
and sometimes controversial topic. Since 1900, sig- Surgeons are often consulted for evaluation of pediatric
nificant advances have been made in the radiographic abdominal problems presenting to the emergency depart-
assessment of infants and children suspected to have ment. It is common for these patients to be evaluated by
anomalies of intestinal rotation. We describe the cur- radiographic imaging in addition to a focused history and
rent methods of abdominal imaging of malrotation
physical examination. The surgeon and radiologist must
along with their pros and cons. When associated with
always have a particularly high-level of suspicion in cases
volvulus, malrotation is a true surgical emergency re-
quiring rapid diagnosis and treatment. We emphasize
of possible malrotation that may require emergency sur-
the importance of close cooperation and communica- gery after evaluation.
tion between radiology and surgery to perform an
effective and efficient diagnostic evaluation allowing CASE PRESENTATION
prompt surgical decision making.
A 5-day-old full term male infant presents to the emer-
© 2014 Baishideng Publishing Group Inc. All rights reserved. gency department with continuous bilious non-bloody
vomiting and irritability after his last three feeds. He was
Key words: Malrotation; Midgut volvulus; Treitz; Ladd; born by normal spontaneous vaginal delivery without
Heterotaxy; Infant complications and was noted to be breast-feeding well
prior to discharge on day-of-life 2; he continued breast-
Core tip: Malrotation, especially when associated with feeding and passing stools at home for the past 4 d until
midgut volvulus, is a surgical emergency that must be this evening. On exam, his abdomen is minimally dis-
astutely recognized, quickly diagnosed, and emergently tended and he is crying constantly. The clinical picture
treated operatively. While the diagnosis depends heav- suggests an obstruction distal to the ampulla of Vater,
and the surgeon has a heightened concern for malrota- of the duodenum and the close proximity of the fixation
tion with midgut volvulus. Before subjecting this infant points for the cranial and caudal midgut along with the
to the morbidity of surgery, the surgeon calls a colleague SMA. In non-rotation, neither portion rotates more than
in the Radiology Department to discuss appropriate im- 90°. Under-rotation leaves the proximal midgut fixed an-
aging workup for malrotation. terior to the right of the SMA and the cecum anterior to
the left of the SMA, and the mesentery is still narrowed
Embryology and foreshortened.
Anomalies of intestinal rotation, commonly referred to
as malrotation, are a result of errors during embryologic History
development. In malrotation, the midgut does not com- Two individuals recognized for their descriptions of
plete its normal lengthening and rotation, and thus is small bowel anatomy and malrotation are Václav Treitz
incorrectly positioned within the peritoneal cavity. Nor- and William Ladd. Treitz (1819-1872), a professor of
mally the process of lengthening and rotation begins be- anatomy in Prague, described the area of tissue which
tween the 4th and 5th wk of gestation. From this time until we now recognize as the Ligament of Treitz[3]. This area
about week 10, the midgut is outgrowing the abdominal that bears his name gives physicians a common point to
cavity and is forced to herniate through the umbilicus to localize where the duodenum becomes the jejunum after
continue unhindered growth[1]. During weeks 10 and 11, exiting the retroperitoneum. Some have described the
the intestine returns to the peritoneal cavity. From the ligament as a “weak thin membranous structure” that is
11th wk forward, the small bowel undergoes fixation. seldom demonstrated on CT[8].
The small intestine is a straight tube early in develop- William Ladd (1880-1967) is considered the father of
ment that derives its blood primarily from the superior pediatric surgery in North America. During World War 1,
mesenteric artery (SMA). This vessel divides the midgut Ladd dedicated his career to the surgical care of children
into two parts: the cephlad or prearterial portion, and the and became surgeon-in-chief at Boston Children’s Hos-
caudad or postarterial portion[2]. The prearterial portion pital[3]. First in 1932 and then again in 1936, he published
is made up of duodenojejunal loops, while the postarte- articles describing his approach to duodenal obstruction
rial portion are cecocolic loops[3]. The SMA is important and malrotation with midgut volvulus. In these articles,
not only because it supplies the majority of blood flow to he described a procedure involving detorsion of the vol-
the small intestine, but also because it serves as the axis vulized bowel in a counterclockwise fashion, dividing
for the normal embryologic rotation of the bowel during the bands of tissue extending from the cecum across the
development. duodenum and into the lateral peritoneal gutter, and finally
When the bowel herniates through the umbilicus, the spreading the mesentery from the cecum in the left upper
prearterial portion rotates 180° counterclockwise around quadrant to the small bowel in the right hemi-abdomen.
the axis of the SMA, while the postarterial portion ro- This procedure later became known as Ladd’s procedure[9].
tates 90° counterclockwise. During the 10th and 11th wk, Rather than attempting to restore normal intestinal rota-
the prearterial portion of the gut reenters first followed tion, Ladd’s operation aimed to convert malrotation to
by the postarterial portion. While the bowel returns into an arrangement of broadened nonrotation with the goal
the abdominal cavity, both segments complete a total turn of minimizing the chance of recurrent volvulus[7]. While
of 270°. This configuration places the normal anatomy historically Ladd had the availability of flat plate radiog-
of the C-loop of the duodenum posterior to the SMA raphy to guide his work-up of children with malrotation,
and the transverse colon anterior to the SMA. many different imaging modalities have become available
The blood from the SMA is distributed throughout to help guide diagnosis and treatment of this surgical
smaller vessels running within the mesentery of the bow- emergency.
el. In normal development, the mesenteric root passes
along the retroperitoneum from the ligament of Treitz to Imaging modalities
the proximal cecum[4]. When normal rotation of the small Plain X-ray: Radiographs are often the first step in the
bowel is not completed in embryologic development, the imaging evaluation of pediatric patients with suspected
mesenteric root is foreshortened[5]. The small bowel is malrotation. This relatively inexpensive and widely avail-
then supported only by this foreshortened pedicle con- able test allows the radiologist and surgeon to quickly
taining the SMA. The small bowel may then twist (volvu- exclude other potential diagnoses. Unfortunately, the most
lus), about this narrow axis[6]. There are two major types common finding on plain film of a patient with malrota-
of rotational abnormalities that have been described as tion is “normal bowel gas pattern”[2]. While abdominal ra-
malrotation and result in this foreshortening: incomplete diographs in a newborn cannot rule-out malrotation, they
rotation and non-rotation[7]. During incomplete rotation, nei- can occasionally demonstrate findings that are concerning
ther the cranial nor the caudal portion rotates more than enough to prompt the surgeon to consider operative ex-
180°. The proximal midgut becomes fixed to the right of ploration: “double bubble” sign of duodenal obstruction,
the SMA and the cecum becomes fixed directly anterior to lack of bowel gas distal to the duodenum, bowel malposi-
the SMA. This pattern has the classic features of Ladd’s tion (small intestine on the right and large intestine on the
bands covering and impinging upon the anterior portion left, found in non-rotation, Figure 1), or pneumatosis intes-
Figure 1 This plain film illustrates an infant with malposition of the small Figure 2 This Upper gastrointestinal demonstrates abnormal position of
bowel on the right and large bowel on the left suggesting malrotation. the duodenal-jejunal junction (white star) to the right of the spine. Nor-
mally the duodenum should sweep across from right to left across the spine.
A B
Figure 3 This axial view of an abdominal computed tomography. A: Illustrates the duodenal-jejunal junction (white arrow) in the right hemi-abdomen suggesting
malrotation; B: Illustrates superior mesenteric artery Superior Mesenteric Artery (SMA)/Superior Mesenteric Vein (SMV) inversion (white arrow) with the SMA to the
right of the SMV. This inversion suggests malrotation.
Current controversies
Some believe that localizing the DJJ with UGI cannot
give reliable data to rule-out malrotation. One author
touts that ultrasonographic imaging in the hands of an
experienced technician may demonstrate a retromesen-
teric D3, which alone can prove that a patient “will not
have malrotation and will not develop midgut volvu-
lus[12]”. Menten et al[11] support this assertion, describing a
graded compression-technique to demonstrate position-
Figure 4 This coronal view of an abdominal computed tomography il-
ing between the SMA and aorta. These techniques rely
lustrates the terminal ileum and cecum (white arrows). Positioning of the on availability of experienced radiology staff, and some
cecum in the left hemi-abdomen is suggestive of malrotation. hospitals may not have this capability or around-the-clock
availability to allow for this focused ultrasound exam.
Furthermore, at least one case of normal D3 retroperito-
mography (CT) imaging can be used to evaluate the position
of D3, the DJJ (Figure 3A), and the anatomical relationship neal positioning on cross-sectional CT imaging in a child
between the SMA and SMV (Figure 3B). Based on a study with malrotation has been reported, thus calling to ques-
by Taylor, CT imaging of abnormal D3 position had a sen- tion the conclusion that normal positioning always rules
sitivity and specificity of diagnosing malrotation of 97.3% out malrotation[17].
and 99% respectively[17]. Due to the variation in normal One group of infants in particular has added contro-
SMA/SMV anatomy as previously discussed, the accuracy versy to the approach of workup for malrotation: infants
of identifying “abnormal” SMA/SMV relation in making with heterotaxy (Figure 5). Anomalies of intestinal rota-
the diagnosis of malrotation was 76.8%[17]. One unique as- tion are common in these infants; unfortunately, these
pect of a CT is that when used with contrast enhancement children can also suffer from life-threatening cardiac
it can recognize perfusion abnormalities that may be missed anomalies. There is debate whether these children should
on laboratory studies[18]. CT can be performed quickly on a undergo elective surgery to broaden the mesentery and
child with extremely minimal invasiveness, but does subject prevent volvulus even if an anomaly of rotation is identi-
the child to a significant dose of radiation when compared fied[19]. Some have suggested that watchful waiting may be
to an UGI (Figure 4). appropriate as volvulus appears to be rare in this popula-
tion[20]. Importantly, Tashjian et al[21] stated that if a sur-
MRI: Magnetic resonance imaging (MRI) can be used, geon decides to perform a Ladd’s procedure on a patient
much like CT, as a cross-sectional imaging modality to with heterotaxia it should occur only when the congenital
identify findings of malrotation including: dilation of the heart disease is well controlled. Additionally, during oper-
proximal duodenum, non-retroperitoneal positioning of ative planning when imaging children with heterotaxia, it
the duodenum, bowel malpositioning, and inversion of is difficult to determine the width of the mesenteric root
the SMA/SMV relationship[2]. The MRI avoids radiation since there is often insufficient data on the location of
but relies on the patient holding still for the duration of the cecum relative to the DJJ[3]. This debate still has yet to
the lengthier exam. Additionally, the MRI is the most be studied in detail with long-term follow-up analysis.
Table 1 Positive and negative attributes of commonly used imaging modalities when applied to cases of suspected malrotation
CT: Computed tomography; MRI: Magnetic resonance imaging; GI: Gastrointestinal imaging; SMA: Superior mesenteric artery; SMV: Superior mesenteric vein.
my demonstrating normal anatomy or by elucidating evi- rithm is useless without the communication and coopera-
dence of a different diagnosis. At this point, we recom- tion of the surgery and radiology teams. Teamwork in
mend reassessing the clinical concern for possible mal- diagnosis is the key to optimal outcomes in children with
rotation. If the concern is lower, then watchful waiting malrotation.
may be acceptable. If there is still high clinical suspicion,
then the imaging should proceed with a barium enema.
This pause for decision-making should be short and ACKNOWLEDGEMENTS
cooperatively communicated between the surgeon and We would like to acknowledge Dr. Lauren Ehrlich (De-
radiologist, as it would be ideal to obtain a barium enema partment of Radiology, Yale University School of Medi-
while the patient is still on the X-ray table from the UGI. cine) for providing us with de-identified radiographic
A negative barium enema in a patient with high clinical images.
suspicion should prompt a discussion about CT imaging.
At this point, do the risks of irradiation with CT imaging
outweigh the risks of negative operative exploration or REFERENCES
delaying surgery for close observation? If the perceived 1 Frazer JE, Robbins RH. On the Factors concerned in caus-
benefits of the CT outweigh the risks, then CT should ing Rotation of the Intestine in Man. J Anat Physiol 1915; 50:
75-110 [PMID: 17233053 DOI: 10.1053/j.sempedsurg.2003.08
be obtained. In the setting of CT results negative for evi- .009]
dence of malrotation, we recommend close observation 2 Strouse PJ. Malrotation. Semin Roentgenol 2008; 43: 7-14
with low threshold to repeat UGI or consider operation [PMID: 18053823 DOI: 10.1053/j.ro.2007.08.002]
if the exam or labs worsen. 3 Lampl B, Levin TL, Berdon WE, Cowles RA. Malrota-
Due to its cost, both in time and dollars, we do not tion and midgut volvulus: a historical review and current
controversies in diagnosis and management. Pediatr Radiol
feel that an MRI can give additional information over the 2009; 39: 359-366 [PMID: 19241073 DOI: 10.1007/s00247-009-
previous imaging studies without significantly delaying 1168-y]
the diagnosis. Additionally, due to the length of time the 4 McVay MR, Kokoska ER, Jackson RJ, Smith SD. Jack Bar-
patient must remain still, the patient would almost cer- ney Award. The changing spectrum of intestinal malrota-
tainly need to be sedated and intubated in order to obtain tion: diagnosis and management. Am J Surg 2007; 194:
712-717; discussion 712-717 [PMID: 18005759 DOI: 10.1016/
imaging. We feel the risk of anesthesia for this imaging j.amjsurg.2007.08.035]
modality is not acceptable for the benefit of the imaging 5 Daneman A. Malrotation: the balance of evidence. Pediatr
results gathered. Radiol 2009; 39 Suppl 2: S164-S166 [PMID: 19308379 DOI:
We recognize that the above algorithm is based on the 10.1007/s00247-009-1152-6]
expectation that plain film radiography, ultrasound, fluo- 6 Irish MS, Pearl RH, Caty MG, Glick PL. The approach to
common abdominal diagnosis in infants and children. Pe-
roscopy, and advanced radiography are easily and readily diatr Clin North Am 1998; 45: 729-772 [PMID: 9728184 DOI:
available. Many centers around the world may not have 10.1016/S0031-3955(05)70043-2]
access to these imaging modalities, and the algorithm 7 Shew SB. Surgical concerns in malrotation and midgut
should be adjusted as such. Additionally, it should be con- volvulus. Pediatr Radiol 2009; 39 Suppl 2: S167-S171 [PMID:
sidered that a patient may have intermittent volvulus that, 19308380 DOI: 10.1007/s00247-008-1129-x]
8 Kim SK, Cho CD, Wojtowycz AR. The ligament of Treitz
depending on the time of the imaging, may cause false (the suspensory ligament of the duodenum): anatomic and
negative results. These cases rely on the clinical evalua- radiographic correlation. Abdom Imaging 2008; 33: 395-397
tion to determine if imaging studies should be repeated. [PMID: 17653583 DOI: 10.1007/s00261-007-9284-3]
In conclusion, malrotation presenting in the newborn 9 Ladd WE. Surgical disease of the alimentary tract in in-
or older child can become a surgical emergency. Delay in fants. New Eng J Med 1936; 215: 705-708 [DOI: 10.1056/
NEJM193610152151604]
diagnosis, specifically in the setting of a midgut volvulus, 10 Orzech N, Navarro OM, Langer JC. Is ultrasonography
can lead to intestinal necrosis, increased mortality, and a good screening test for intestinal malrotation? J Pediatr
intestinal failure with dependence on parenteral nutrition. Surg 2006; 41: 1005-1009 [PMID: 16677901 DOI: 10.1016/
When malrotation is being considered, it is important j.jpedsurg.2005.12.070]
that pediatric surgeons and pediatric radiologists work 11 Menten R, Reding R, Godding V, Dumitriu D, Clapuyt P.
Sonographic assessment of the retroperitoneal position of
closely to discuss available imaging options and com- the third portion of the duodenum: an indicator of normal
municate a clear workflow of studies while making the intestinal rotation. Pediatr Radiol 2012; 42: 941-945 [PMID:
decision of whether or not an operation is needed. In 22684229 DOI: 10.1007/s00247-012-2403-5]
the heterotaxy population, even positive imaging can be 12 Yousefzadeh DK. The position of the duodenojejunal junc-
difficult to interpret clinically and little consensus exists tion: the wrong horse to bet on in diagnosing or exclud-
ing malrotation. Pediatr Radiol 2009; 39 Suppl 2: S172-S177
regarding the treatment of this subset of patients. [PMID: 19308381 DOI: 10.1007/s00247-008-1116-2]
We have proposed an imaging algorithm based on 13 Sizemore AW, Rabbani KZ, Ladd A, Applegate KE. Diag-
the current literature and an evaluation of the pros and nostic performance of the upper gastrointestinal series in
cons of the different imaging modalities (Table 1). Our the evaluation of children with clinically suspected malrota-
algorithm begins with a plain film radiograph followed by tion. Pediatr Radiol 2008; 38: 518-528 [PMID: 18265969 DOI:
10.1007/s00247-008-0762-8]
either ultrasound or UGI series depending on resources 14 Dekker G, Andronikou S, Greyling J, Louw B, Brandt A.
available. However, it is important to stress that any algo- Contrast meals and malrotation in children-metal mark-
ers for improved accuracy. Pediatr Radiol 2013; 43: 115-118 diatr Radiol 2005; 35: 529-531 [PMID: 15536561 DOI: 10.1007/
[PMID: 23160646 DOI: 10.1007/s00247-012-2503-2] s00247-004-1355-9]
15 Applegate KE. Evidence-based diagnosis of malrotation 19 Chang J, Brueckner M, Touloukian RJ. Intestinal rotation
and volvulus. Pediatr Radiol 2009; 39 Suppl 2: S161-S163 and fixation abnormalities in heterotaxia: early detection and
[PMID: 19308378 DOI: 10.1007/s00247-009-1177-x] management. J Pediatr Surg 1993; 28: 1281-1284; discussion
16 Slovis TL, Strouse PJ. Malrotation: some answers but more 1285 [PMID: 8263687 DOI: 10.1016/S0022-3468(05)80313-6]
questions. Pediatr Radiol 2009; 39: 315-316 [PMID: 19241072 20 Choi M, Borenstein SH, Hornberger L, Langer JC. Hetero-
DOI: 10.1007/s00247-009-1169-x] taxia syndrome: the role of screening for intestinal rota-
17 Taylor GA. CT appearance of the duodenum and mesen- tion abnormalities. Arch Dis Child 2005; 90: 813-815 [PMID:
teric vessels in children with normal and abnormal bowel 15890694 DOI: 10.1136/adc.2004.067504]
rotation. Pediatr Radiol 2011; 41: 1378-1383 [PMID: 21594544 21 Tashjian DB, Weeks B, Brueckner M, Touloukian RJ. Out-
DOI: 10.1007/s00247-011-2118-z] comes after a Ladd procedure for intestinal malrotation
18 Aidlen J, Anupindi SA, Jaramillo D, Doody DP. Malrotation with heterotaxia. J Pediatr Surg 2007; 42: 528-531 [PMID:
with midgut volvulus: CT findings of bowel infarction. Pe- 17336193 DOI: 10.1016/j.jpedsurg.2006.10.060]