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Pe d i a t r i c I m a g i n g R ev i ew

Restrepo et al.
Acute Pancreatitis in Pediatric Patients

Pediatric Imaging
Review
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Acute Pancreatitis in Pediatric


Patients: Demographics, Etiology,
and Diagnostic Imaging
Ricardo Restrepo1 OBJECTIVE. The objective of this article is to provide updates on acute pancreatitis in
Heidi E. Hagerott 2 children regarding the imaging findings, causes, and complications based on a review of the
Sakil Kulkarni2 current studies in the pediatrics literature. We discuss the epidemiology of acute pancreatitis,
Mona Yasrebi 3 the role of imaging and imaging findings in the diagnosis of acute pancreatitis, and the causes
and complications of acute pancreatitis.
Edward Y. Lee 4
CONCLUSION. The incidence of acute pancreatitis is increasing in children. Imaging
Restrepo R, Hagerott HE, Kulkarni S, Yasrebi M, plays an important role in the diagnosis of acute pancreatitis because imaging findings can be
Lee EY used to establish the cause of acute pancreatitis, evaluate for complications of acute pancre-
atitis, and possibly predict the course of the disease.

cute pancreatitis is the most com- review the role of various currently available

A mon pathologic entity affecting


the pancreas in children. Accord-
ing to the INSPPIRE (Interna-
imaging modalities (i.e., ultrasound, CT, and
MRI) in the diagnosis of acute pancreatitis
and in establishing the cause.
tional Study Group of Pediatric Pancreatitis:
In Search for a Cure) Group, acute pancreatitis Demographics and Epidemiology
is defined as reversible inflammation of the Acute pancreatitis occurs in all age
pancreatic parenchyma when two of the three groups, even in infants. Recent single-center
following criteria are present: abdominal pain and multiinstitutional studies have reported
compatible with acute pancreatitis, serum am- an increasing incidence of acute pancreatitis
ylase or lipase value3 times the upper limit in all pediatric age groups over the past 2 de-
of normal, and imaging findings consistent cades [610]. This increase in the reported
Keywords: CT, MRI, pancreatitis, pediatrics, ultrasound
with acute pancreatitis [1, 2]. incidence of acute pancreatitis in the pediat-
DOI:10.2214/AJR.14.14223 Imaging of the pancreas plays an impor- ric population is likely multifactorial. Some
tant role not only in the diagnosis of acute investigators have linked it to an increase in
Received December 2, 2014; accepted after revision pancreatitis but also in the determination of emergency department visits and testing of
May22, 2015.
the underlying cause of acute pancreatitis serum amylase and lipase levels in pediatric
1
Department of Radiology, Miami Childrens Hospital,
and in the detection of complications asso- patients [8], whereas others have linked it to
3100 SW 62 Ave, Miami, FL 33155-3009. Address ciated with acute pancreatitis [3]. Although increases in referrals to tertiary care centers
correspondence to R. Restrepo (ricardo.restrepo@mch.com). the symptoms that are typical of pancreatitis, [11]. The increase in the incidence of acute
2
such as abdominal pain and vomiting along pancreatitis in pediatric patients has also
Department of Medical Education, Miami Childrens
with elevated lipase levels, establish the di- been linked to the rising incidence of obesity,
Hospital, Miami, FL.
agnosis in most cases, up to 5% of cases of a significant independent risk factor for acute
3
Department of Medical Imaging, Nemours/A. I. Dupont acute pancreatitis may be missed in children biliary pancreatitis, which is one of the most
Hospital for Children, Medical Imaging, Wilmington, DE. if imaging is not performed [4]. The role of common causes of acute pancreatitis in chil-
4
imaging in the early prediction of complica- dren [1214]. Currently, the best estimates
Department of Radiology, Boston Childrens Hospital
and Harvard Medical School, Boston, MA.
tions based on the CT severity index (CTSI) suggest that there are 3.613.2 cases of acute
score and the role of interventional radiolo- pancreatitis per 100,000 pediatric individu-
This article is available for credit. gy in treating complications associated with als per year, an incidence that approaches the
acute pancreatitis (i.e., percutaneous catheter incidence of pancreatitis in adults [8, 9]. In a
AJR 2016; 206:632644
drainage) are being increasingly recognized meta-analysis that spanned almost 4 decades
0361803X/16/2063632 in children [2, 5]. In this article, we discuss and generated data that included 589 chil-
the epidemiology, pathophysiology, classifi- dren with acute pancreatitis, the mean age of
American Roentgen Ray Society cation, and causes of pancreatitis. We also patients with the disease was 9.2 2.4 (SD)

632 AJR:206, March 2016


Acute Pancreatitis in Pediatric Patients

years (range, 1 week21 years) and the male-


to-female ratio was 1:2 [15]. In a more re-
cent study that included 55,012 children with
acute pancreatitis, the disease was found to
be more likely to occur in children older than
5 years old (median age, 17 years) and to oc-
cur slightly more frequently in girls than in
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boys [10].
Pancreatitis causes substantial morbidi-
ty in the pediatric population. It is estimat-
ed that 213 new cases occur annually per
100,000 children [8]. Nearly one-quarter of
children with acute pancreatitis develop a se-
vere complication, and the mortality rate is
Fig. 1Transverse ultrasound image of 2-year-old
approximately 410% despite significant ad- girl shows normal pancreas (P). Normal pancreas
vances in the treatment of this disease [10, is isoechoic to liver (L) and has speckled pattern.
15]. According to a recent study, the median Normal pancreatic duct is partially visualized as
echogenic line (arrow).
inpatient length of stay for children with pan-
creatitis in 2009 was 4 days, and the median
cost of care was approximately $22,663 (in- atic parenchyma in premature and full-term
terquartile range, $11,923$45,728) [10]. infants is hyperechoic to the liver; this appear-
ance is related to the prominent septa with- Fig. 2Increased echogenicity of normal pancreas
Spectrum of Imaging Findings in in lobules and large amounts of glandular tis- (P) on ultrasound image of 1-week-old boy. Note
higher echogenicity of pancreas relative to that of
Acute Pancreatitis sue [19] (Fig. 2). After the neonatal period, the liver (L) in neonates.
The imaging features compatible with echogenicity of the pancreas is variable; the
acute pancreatitis in pediatric patients, which pancreas is isoechoic or slightly hyperechoic
are similar to those seen in adults, include relative to the liver. A speckled appearance of tion images (i.e., 15 MHz) [18, 21, 22]. Poor-
edema, hemorrhage, or necrosis of the pan- the gland is commonly seen [17, 20] (Fig. 1). ly defined glandular borders and peripancre-
creatic parenchyma; peripancreatic fat; as- In older children, marked hyperechogenicity atic fluid or localized fluid collections can be
cites; and pancreatic and peripancreatic col- is usually indicative of fatty infiltration, which seen in the acute setting and are easy to iden-
lections, the latter of which indicate a recent can be seen in patients with cystic fibrosis, pa- tify on ultrasound (Fig. 5). Ultrasound is also
episode of acute pancreatitis [2]. Currently, tients who are taking steroids, and patients useful in the characterization of the margins
ultrasound, CT, and MRI are the three most who are obese. The pancreas is not seen on ul- (i.e., well defined vs poorly defined) and con-
used imaging modalities for evaluating pan- trasound in approximately 614% of children tents (low-level echoes, septations, debris) of
creatitis in the pediatric population. [21]; the percentage increases when pancreati- collections complicating pancreatitis (Fig. 5),
tis is present because of increased bowel gas which determine the amenability of the col-
Transabdominal Ultrasound secondary to aerophagia and ileus [21]. lections to drainage.
Transabdominal ultrasound is current- In acute pancreatitis, especially early
ly the best initial imaging study of choice to in the disease process or in mild cases, the CT
screen for suspected pancreatitis and to eval- size and echogenicity of the pancreas on ul- The common CT findings of acute pan-
uate for biliary tree abnormalities in pediat- trasound are unreliable diagnostic features, creatitis include varying degrees of focal or
ric patients. The glandular and pancreatic duct in part because of the normal variability of diffuse pancreatic enlargement, focal or dif-
size and echogenicity change according to the these features. Diffuse or focal enlargement fuse parenchymal hypodensities, parenchy-
childs age and body habitus. These changes of the pancreas is attributable to edema; how- mal heterogeneity, indistinctness of the glan-
are potentially confounding features when us- ever, pancreatic enlargement has been re- dular contours, and inflammatory changes in
ing ultrasound to evaluate pediatric patients ported to be absent on ultrasound in approxi- the peripancreatic fat seen as fat stranding
with suspected acute pancreatitis. The pediat- mately 50% of the patients [20]. Decreased and thickening of the retroperitoneal fascial
ric pancreas is relatively larger than the adult glandular echogenicity is frequently seen, but planes (Fig. 6). In mild cases of acute pancre-
pancreas, and the pancreatic head in pediatric the echogenicity may be normal or may even atitis or early in the disease process, a nor-
patients tends to be more prominent than the be increased in children with acute pancreati- mal pancreas can be seen in up to one-third
body and tail; these differences in imaging ap- tis [22] (Fig. 3). On ultrasound, a more reli- of patients on the initial CT examination
pearances are potential interpretation pitfalls able diagnostic feature of acute pancreatitis [21]. When CT is used to evaluate pancreati-
for the unwary [16, 17]. The normal pancreat- is dilatation of the pancreatic duct (16 years tis or its complications, IV contrast materi-
ic duct in children is not necessarily visible on old,>1.5 mm; 712 years old,>1.9 mm; 13 al is mandatory to identify the parenchymal
ultrasound: In one study, investigators report- 18 years,>2.2 mm) [18] (Fig. 4). The eval- changes unless there is a contraindication
ed visualization of at least part of the pancre- uation of the pancreatic duct should be per- to contrast material. Contrast enhancement
atic duct as an echogenic line in up to 85% of formed, if possible, using high-frequency of the gland can be variable from homo-
healthy children [18] (Fig. 1). Normal pancre- ultrasound probes that provide high-resolu- geneous in mild cases, to heterogeneous in

AJR:206, March 2016 633


Restrepo et al.

TABLE 1: MRI and MRCP Protocols in Patients 1218 Years With Acute Pancreatitis or a History of Acute Pancreatitis
Slice Flip
Thickness TR Angle FOVa
Sequences Plane (mm) Slice Gap (mm) (ms) TE (ms) () (cm) Matrix
3 Tb
T2 3D VISTA Coronal 1.5 Overcontiguous 948 200 90 350 325 285
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T2 single-shot Axial 4 0.6 736 80 90 340 340 240


T2 single-shot with FS Axial 4 0.6 799 70 90 340 340 240
In-phase and out-of-phase dual FFE acquisitionsc Axial 5 0.5 180 In phase, 2.3; out of phase, 1.15 55 340 164 146
T2 Radial 40 3042 990 90 200 212 171
eThrive Axial 1.7 0.85 (overcontiguous) 2.6 1.23 10 340 200 140
1.5 Td
T2 3D VISTA Coronal 1.2 Overcontiguous 2000 200 90 350 276 199
T2 single-shot Axial 4 0.4 437 80 90 340 264 212
T2 single-shot with FS Axial 4 0.4 431 70 90 340 268 218
In-phase and out-of-phase mDixon acquisitions Axial 2 Overcontiguous 5.8 First echo, 1.8; second echo, 4 15 340 196 130
T2 thick slab Radial 40 8000 800 90 200 256 205
eThrive Axial 2 1 (overcontiguous) 2.6 1.23 10 340 200 140
NoteThe use of gadolinium is optional if MRI or MRCP is performed during an acute episode or to evaluate a collection. VISTA (Philips Healthcare)= volume isotropic
turbo spin-echo acquisition, FS= fat saturation, FFE= fast-field echo, eTHRIVE (Philips Healthcare)= enhanced T1-weighted high-resolution isotropic volume excitation,
mDixon (Philips Healthcare)= modified Dixon.
aIn younger and smaller children, FOV should be decreased according to body size and the slab thickness should be no more than 2 cm.
bThe 3-T examinations were performed on an Achieva unit (Philips Healthcare) using a 16-channel torso coil (XL Coil, Philips Healthcare). For smaller children, a 6-channel

cardiac coil (Sense Coil, Philips Healthcare) can be used.


cThe mDixon sequence provides simultaneous fat and water images and in- and out-of-phase images in one breath-hold only on 1.5 T.
d The 1.5-T examinations were performed on an Enginia unit (Philips Healthcare) using a 16-channel torso coil (dS Coil, Philips Healthcare) with 16 channels anteriorly and

16 channels posteriorly. For smaller children, a 6-channel cardiac coil (Sense Coil) can be used.

more advanced or severe cases, and to no en- when present, are indicative of superimposed single-shot sequences, which are acquired in
hancement in cases of necrotizing pancreati- infection [23, 25]. seconds, can be used in patients who are less
tis (Fig. 6). The use of IV contrast materi- cooperative or patients with irregular breath-
al also allows the evaluation of the patency MRI and MRCP ing. Ultrafast sequences, although less affect-
of the adjacent vascular structures [21, 23 The principle of MRCP is based on heavily ed by motion artifact, have the disadvantage
25]. Intrapancreatic or extrapancreatic col- T2-weighted sequences with long TEs (range, of image blur produced by the long echo-train
lections can also be present (Fig. 7). CT is 3001000 ms). MRCP allows visualization of length and less effective fat saturation [28, 29].
helpful for evaluating the presence and ex- only fluid and tissues with a prolonged trans- Axial T1-weighted sequences with or without
tent of peripancreatic collections, an early verse relaxation time as hyperintense struc- fat saturation are helpful in evaluating the nor-
sign of acute pancreatitis. These collections tures while suppressing the background tis- mal physiologic hyperintensity of the pancre-
can spread into the lesser sac and pararenal sues. MRCP is usually performed with fast as (Fig. 8 and Table 1).
space and even into the peritoneum and the spin-echo sequences using a thick slab (2D) of The MRCP technique is more challenging
mediastinum [2527] (Fig. 7). Gas bubbles in 27 cm or thin-slice images with 3D capabili- to perform in pediatric patients than in adults
a collection are readily identified on CT and, ties. Alternatively, ultrafast sequences, such as because it needs to be tailored to different

Fig. 3Variability of pancreatic


echogenicity on ultrasound images
of patients with acute pancreatitis.
A, Normal echogenicity and
pancreatic enlargement (P) in
9-year-old girl.
B, Diffuse increased echogenicity
with diffuse pancreatic enlargement
(P) in 12-year-old girl. Stranding
of peripancreatic fat planes and
adjacent fluid (arrows) are present.
A B

634 AJR:206, March 2016


Acute Pancreatitis in Pediatric Patients

body sizes at different ages. For visualization homogeneous enhancement and remains
of small-caliber ducts, the spatial resolution hyperintense on fat-saturated T1-weighted
and signal-to-noise ratio need to be optimized. images. On T2-weighted images, the signal
These adjustments can be achieved by select- intensity of the normal pancreas is variable,
ing a proper coil (i.e., phased-array or surface but it should be homogeneous and of signal
coil) and using an appropriate slice thickness intensity similar to that of a healthy liver
based on the childs size (e.g., slice thickness= (Fig. 8). On T2-weighted images, the pan-
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35 mm in the axial and coronal planes). For creas becomes heterogeneous and hyperin-
imaging neonates and infants, the slab thick- tense in more advanced and severe cases of
ness should not be more than 2 cm. The ad- acute pancreatitis (Fig. 9). T2-weighted se-
ministration of secretin also increases the con- quences are useful for evaluating the bili-
spicuity of the smaller pancreatic ducts. The ary and pancreatic ducts and for detecting
use of a negative oral contrast agent (ferumox- pancreatic and peripancreatic edema and
sil [GastroMARK, Mallinckrodt Medical]) or fluid collections. On T2-weighted sequenc-
pineapple juice, with its high iron content, de- es, even subtle pancreatic edema and peri-
creases the bright signal intensity from the sur- pancreatic edema, which can be unapparent
rounding gut, allowing better visualization of on T1-weighted sequences, are more readily
the pancreatic duct [28, 30, 31]. identified [25, 32, 33].
The normal pancreas shows high signal In necrotizing pancreatitis, there is often
intensity on T1-weighted imaging because high signal intensity on T1-weighted imag-
of abundant aqueous proteins and intracel- es secondary to hemorrhage (Fig. 10). Pa-
lular paramagnetic substances (i.e., man- renchymal necrosis is well depicted on se-
ganese) (Fig. 8). Adding fat suppression to quential acquisitions obtained during the
T1-weighted sequences increases the con- first 12 minutes after the injection of gado-
trast between retroperitoneal fat and the linium, as shown by a lack of enhancement.
gland, increasing the conspicuity of the hy- The T2-weighted sequences are most sensi-
pointensity of the abnormal pancreatic pa- tive for showing fluid collections; however,
renchyma (Fig. 9). As inflammation devel- it is important to be aware that fluid collec-
ops, the gland shows a loss of this inherent tions can be confused with fluid-filled bowel
Fig. 4Transverse high-resolution ultrasound image bright signal intensity on T1-weighted im- loops. Gas and calcifications are more diffi-
of pancreas (P) of 2-year-old boy with choledochal ages and becomes heterogeneous and hy- cult to identify on this modality and are usu-
cyst (asterisk) and acute pancreatitis shows
dilatation of pancreatic duct (arrow) using liver (L) as pointense. After IV gadolinium admin- ally seen as focal areas of low signal inten-
window. Pancreatic duct (cursors) measured 3 mm. istration, the normal pancreas displays sity on T1- and T2-weighted images [25, 33].

A B
Fig. 5Fluid collections related to acute pancreatitis.
A, Ultrasound image of pancreas obtained several weeks after episode of acute pancreatitis in 6-year-old girl shows well-defined oval fluid collection (asterisk) along
compressed pancreas (arrows).
B, Acute necrotic collection in 10-year-old girl with lupus erythematosusrelated pancreatitis. Ultrasound image of pancreas shows large heterogeneous, but
predominantly solid, collection (arrows) anterior to pancreas (P) with small fluid component (asterisk). These findings are consistent with acute necrotic collection.

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Restrepo et al.
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A B C
Fig. 6Different degrees of glandular involvement in acute pancreatitis on CT.
A, Axial contrast-enhanced CT scan of 12-year-old boy with leukemia receiving l-asparaginase shows diffusely enlarged pancreas (P) with fairly homogeneous
parenchyma and mild peripancreatic inflammation (arrows).
B, Acute pancreatitis in 5-year-old girl with associated acute peripancreatic fluid collection. Axial contrast-enhanced CT scan obtained 2 weeks in disease course shows
heterogeneous pancreatic parenchyma (P) with irregular borders and fairly homogeneous hypodensities consistent with peripancreatic fluid (asterisks).
C, Axial contrast-enhanced CT scan of 8-year-old girl with necrotizing pancreatitis shows pancreatic enlargement with hypodense nonenhancing parenchyma involving
body and tail (asterisk) indicating necrosis. Compare with enhancing parenchyma of head and neck (P). Significant peripancreatic and perirenal inflammatory changes
(arrows) are present.

Role of Imaging in Pancreatitis creatitis cases missed if imaging had not been aminations [2, 22]. The smaller size and de-
The utility and timing of radiologic studies performed [4]. Imaging also plays an important creased amount of body fat make ultrasound
in children with suspected acute pancreatitis role in trying to elucidate the cause of pancre- a better imaging modality for the evaluation
remains controversial; however, imaging is part atitis and assess the severity of the disease be- of acute pancreatitis in pediatric patients
of the diagnostic workup of acute pancreatitis cause the levels of pancreatic enzymes do not than in adults. The lack of ionizing radiation,
in children and helps confirm the presence of necessarily correlate with the severity of pan- widespread availability, and no need for se-
the disease. In a 2003 analysis of studies in the creatitis [34, 35]. Finally, imaging is helpful in dation support the use of this modality in pe-
literature, which included 589 children from 18 evaluating for the presence of complications diatric patients. However, there is a lack of
studies, Benifla and Weizman [15] found that and in planning potential surgical intervention studies comparing the diagnostic accuracy
the diagnosis of pancreatitis was based on ul- in certain cases of acute pancreatitis. of ultrasound with the diagnostic accuracy
trasound findings in 81% of cases. In a more of CT and MRI in pediatric patients [22]. In
recent (2014) study by Coffey et al. [4] of 131 Transabdominal Ultrasound addition, ultrasound findings are frequently
children, elevation of the serum lipase value Transabdominal ultrasound is a useful tool normal in pediatric patients with acute pan-
contributed to the diagnosis of acute pancre- and is probably the first-line imaging study creatitis, especially in early or mild cases [2,
atitis more often than any other laboratory or for the confirmation of pancreatitis in pedi- 5, 36, 37]. Furthermore, overlapping bowel
imaging test. The combination of blood and atric patients with clinically suspected and gas due to pain-induced aerophagia or to lo-
imaging tests was shown to have an increased laboratory-suspected pancreatitis or in those calized ileus precludes the evaluation of the
diagnostic yield with at least 5% of acute pan- with inconclusive findings on clinical ex- retroperitoneal gland. In one study, the sen-

A B C
Fig. 7Intra- and peripancreatic collections in acute pancreatitis.
A, Pancreatic pseudocyst. Contrast-enhanced CT scan of 6-year-old girl (same patient as in Fig. 5A) shows well-encapsulated oval fluid collection (asterisk) along
compressed pancreas (arrows).
B, Acute necrotic collection in 10-year-old girl with lupus erythematosusrelated pancreatitis and secondary splenic artery thrombosis (same patient as in Fig. 5B). Axial
contrast-enhanced CT scan shows heterogeneous pancreatic parenchyma (P) with indistinct borders. There is large heterogeneous collection with hypodense and
hyperdense areas surrounding gland (asterisks) consistent with acute necrotic collection. Note lack of enhancement of spleen (S). St= decompressed stomach.
C, Walled-off necrosis in 12-year-old boy with acute pancreatitis diagnosed 4 weeks earlier. Axial contrast-enhanced CT scan shows well-defined oval hypodense fluid
collection (asterisk) contained within pancreatic parenchyma (P).

636 AJR:206, March 2016


Acute Pancreatitis in Pediatric Patients

TABLE 2: CT Scoring of Acute Pancreatitis According to the Balthazar TABLE 3: Scoring of the Degree of
Scoring System [45] Pancreatic Necrosis on CT
Balthazar Degree of Pancreatic Necrosis on CT Pointsa
Grade Description of CT Findings Pointsa
Percentage of pancreas that is necrotic
A Normal findings 0
0 0
B Focal or diffuse enlargement of the pancreas 1
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< 30 2
C Pancreatic gland abnormalities and peripancreatic inflammation 2
3050 4
D Fluid collection in a single location 3
> 50 6
E Two or more collections, gas bubbles in or adjacent to the pancreas, or both 4 aThe CT severity index is calculated by adding the
aThe CT severity index is calculated by adding the points for the grade of acute pancreatitis found on CT and the points for the grade of acute pancreatitis found on
points for the degree of pancreatic necrosis (see Table 3). CT (see Table 2) and the points for the degree of
pancreatic necrosis.
sitivity of transabdominal ultrasound in de- As discussed later in this article, the CT se-
tecting pancreatitis was 79.4% [5]. verity index (CTSI) has been shown to be a In children with borderline Ranson or
The frequency of a biliary cause of acute better predictor of acute severe pancreatitis PAPS scores or in those with concerning
pancreatitis in pediatric patients provides the than clinical scores in children. CT is use- changes in clinical status (e.g., vital signs,
most compelling argument for early imaging ful in identifying fluid collections and deter- laboratory results, physical examination), the
with ultrasound [38]. Although imaging is mining a suitable window if drainage is nec- CTSI may also allow clinicians to be proac-
not a necessary part of the diagnostic work- essary and in evaluating the patency of the tive in intensifying care. CT findings may
up for acute pancreatitis if the other two cri- surrounding vessels to exclude vascular com- trigger the use of antibiotics in patients with
teria are fulfilled, transabdominal ultrasound plications [5, 11]. extensive necrosis, the transfer of patients at
may play an important role in assessing for In the past, various clinical scores have risk for respiratory or renal failure to the ICU,
biliary tract disease, one of the most com- been developed to predict a complicated and surgical dbridement in pediatric patients
mon etiologic factors (e.g., calculi, chole- course in cases with acute pancreatitis. Al- with evidence of infected necrosis. Finally,
dochal cyst) of acute pancreatitis in pediatric though these scores are widely used in adult these CT findings may lower the threshold for
patients [11]. Transabdominal ultrasound has patients with acute pancreatitis (e.g., Ramsey, initiating parenteral nutrition or enteral feeds
a high sensitivity (92%) in accurately detect- Glasgow, and Balthazar), the value of similar and may heighten the index of suspicion for
ing choledochal cysts, gallstones, and pseu- scores (e.g., pediatric acute pancreatitis se- patients with fluid collections who are at risk
docysts [39]. Although recent advances in verity [PAPS] score) has not been established for pseudocyst development [34].
ultrasound technology, such as contrast-en- in children. The need for a reliable system to In a recent study including only pediatric
hanced ultrasound, ultrasound elastography, risk-stratify children with acute pancreatitis is patients, the sensitivity, specificity, positive
and endoscopic ultrasound, have shown en- underscored by the increasing number of hos- predictive value, and negative predictive val-
couraging results in adult studies, these tech- pitalizations and high rate of major complica- ue of the CTSI in predicting a severe course
niques are still in the early stages in the di- tions ( 25%) reported in recent series [2, 10, (using a CTSI cutoff score of>4) were 81%,
agnosis of pancreatic diseases in pediatric 34, 43]. The CTSI has been shown to be a bet- 76%, 62%, and 90%, respectively, which are
patients [22, 37, 40, 41]. ter predictor of acute severe pancreatitis than better than the results of the PAPS (53%,
clinical scores in children [44]. Balthazar et 72%, 41%, 80%), Ranson (71%, 87%, 67%,
CT al. [45] developed the CTSI, which is based 89%), and modified Glasgow (71%, 87%,
CT has been shown to be more sensitive on the appearance of the pancreas and extent 67%, 89%) scores. The same study showed
in detecting acute pancreatitis and grading of necrosis in adults. The components of the that, among children who underwent CT for
its severity than ultrasound [39]. CT is also CTSI are shown in Tables 24 [45]. The CTSI acute pancreatitis, the presence of necrosis
more sensitive in the diagnosis of pancreatic has been reported to be superior to clinical was associated with a higher rate of major
necrosis and fluid collections compared with scoring systems in predicting the outcome of complications (mean rate of major compli-
ultrasound [11]. Bowel gas is not a limitation acute pancreatitis in adults [46]. Similar stud- cations in patients with necrosis vs patients
of CT; however, the inherent paucity of in- ies in children have shown that CTSI is supe- without necrosis, 42.3% vs 10.5%, respec-
traabdominal fat in children makes the eval- rior to clinical scoring indexes [44, 47]. tively; p= 0.002) [44].
uation of the organ more challenging. Other
limitations of CT are difficulty in evaluat- TABLE 4: Complication Rates and Mortality Associated With Acute
ing the ductal anatomy and the use of ioniz- Pancreatitis Based on CT Severity Index (CTSI) Score
ing radiation [42]. The use of CT may be of
Total CTSI Score Rate of Complications (%) Mortality (%)
more value later in the course of acute pan-
creatitis (i.e., if there is a lack of improve- 03 Points 8 3
ment clinically), in evaluating disease severi- 46 Points 35 6
ty (i.e., when pancreatic necrosis is suspected 710 Points 92 17
or in cases of multiorgan failure), or if the
NoteThe CTSI is calculated by adding the points for the Balthazar grade of acute pancreatitis found on CT
diagnosis is uncertain (i.e., inadequately vi- (Table 2) and the points for the degree of pancreatic necrosis (Table 3). Maximum CTSI score is a total of 10
sualized glands on ultrasound) [22, 34, 38]. points.

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Restrepo et al.
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A B
Fig. 8Signal intensity of normal pancreas on MRI in healthy 12-year-old boy.
A, Axial T1-weighted image with fat saturation shows homogeneously hyperintense normal and nonenlarged pancreas (arrows). L= liver, S= spleen.
B, Axial single-shot T2-weighted image shows normal and homogeneous signal intensity of pancreas (arrows), which is similar to liver (L). S= spleen.

The CTSI may provide useful information trast resolution than CT, enabling the detec- concluded that DWI had no proven role in the
for risk stratification in select children with tion of inflammation at an earlier stage, and diagnosis of acute pancreatitis requiring fur-
acute pancreatitis. However, owing to the risk this greater contrast resolution is particular- ther investigation [49]. Furthermore, the same
of radiation exposure in children, further stud- ly useful when imaging the fat-scant pediat- study reported inconsistencies and conflicting
ies are required to determine whether chang- ric abdomen [42]. In addition, MRI has su- results in the published data regarding the dif-
es to antibiotic use, enteral or parenteral nu- perb capabilities (including 3D imaging) ferentiation between mass-forming pancreati-
trition, ICU transfer, operative intervention, or for depicting the biliary tree and pancreatic tis (autoimmune pancreatitis) and malignan-
other clinical management strategies based on duct, allowing the concomitant evaluation of cy. MRCP is more sensitive than ultrasound
CTSI stratification positively affect the out- the pancreatic parenchyma and its surround- in detecting choledocholithiasis and is most
come of children with acute pancreatitis be- ings. Several reports in the literature sug- likely as sensitive as ERCP [5155]. Howev-
fore its routine use is recommended [2]. gest that restricted diffusion with low appar- er, unlike ERCP, MRCP is not invasive and
ent diffusion coefficient values are seen in poses no risk of procedure-related pancreati-
MRCP acute pancreatitis even at early stages of dis- tis. According to a recent National Institutes
MRI of the pancreaticobiliary system can ease [4850]. However, a more recent study of Health state-of-the-science statement [56],
overcome the disadvantages of CT in the in- that reviewed the available data in the litera- ERCP in acute pancreatitis should be reserved
vestigation of pancreatitis, particularly in the ture regarding the current status and recom- for children in whom a biliary cause is sus-
pediatric population. MRI has greater con- mendations of DWI of the pancreas in adults pected or when a therapeutic intervention is

A B
Fig. 9Signal intensity of acute pancreatitis on MRI.
A, Decreased signal intensity of acute pancreatitis on T1-weighted imaging. Axial T1-weighted image with fat saturation of 10-year-old boy shows
diffusely hypointense and enlarged body and tail of pancreas (P). Compare signal intensity of pancreas with higher signal intensity of liver (L). S= spleen.
B, 9-year-old girl with pancreas divisum and acute pancreatitis. Axial single-shot T2-weighted image with fat saturation shows inflamed hyperintense
and indistinct pancreas (P) with peripancreatic edema. Pancreatic duct (straight arrow) is seen entering duodenum separate from common bile duct
(curved arrow).

638 AJR:206, March 2016


Acute Pancreatitis in Pediatric Patients
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Fig. 10Necrotizing pancreatitis in 13-year-old boy. Axial T1-weighted image Fig. 1115-year-old girl with gallstone pancreatitis. Axial single-shot
with no fat saturation shows large hyperintense areas in enlarged pancreatic T2-weighted image shows intraluminal calculus inside common bile duct (straight
neck, body, and tail (arrows); these findings are consistent with hemorrhage and thin arrow) and calculi inside gallbladder (straight thick arrow). Pancreatic head
indicate necrosis. Compare these areas with hypointense areas of pancreatic (curved arrow) is hyperintense, which indicates edema. Small amount of free fluid
parenchyma in head and part of body posteriorly (asterisks). (F) is present.

beneficial, such as recurrent pancreatitis or a egories. The proportion of the causes of acute emergency setting especially in the nonobese
pseudocyst. Visualization of the pancreatic pancreatitis varies greatly among studies of child. In obese children, MRCP can provide
duct and its branches can be enhanced by the acute pancreatitis in children [39, 62, 63]. a better evaluation of the biliary tree and
administration of secretin, which is more use- The reported variation probably results pancreas than ultrasound, thereby allowing a
ful in pediatric patients because the pancreat- from the inherent limitations of retrospective more definite diagnosis. This better perfor-
ic duct and its branches naturally are smaller studies, the bias or experience of clinicians, mance of MRCP compared with ultrasound
caliber [2830, 3638]. incomplete investigations, greater number of is corroborated by the fact that MRCP is not
The major drawbacks of MRI are the long patients identified to have pancreatitis, and affected by the presence of bowel gas, which
scanning time, which requires the childs co- the recognition of new causes in children [4]. frequently obscures the pancreas and distal
operation and may result in the use of sedation, Imaging plays an additional role in eluci- common bile duct [13, 14, 67, 68].
and the higher cost. Prior studies of adults have dating the etiologic mechanism of pancreati- The four most common congenital pancre-
shown the utility of MRCP in the diagnosis of tis particularly in cases that result from bili- aticobiliary anomalies in the pediatric popu-
pancreatitis in patients with pancreatic ductal ary, traumatic, and autoimmune causes and lation are pancreas divisum, choledochal cyst,
anomalies such as pancreas divisum and pan- will be briefly discussed. pancreaticobiliary maljunction, and aberrant
creatobiliary maljunction [22, 57, 58] and pan- biliary ducts. Affected pediatric patients typi-
creatic duct disruption [26]. Shimizu et al. [59] Biliary Pancreatitis cally present early in childhood with recurrent
found MRCP to be useful as a noninvasive test Acute biliary pancreatitis is one of the episodes of acute pancreatitis. Recent studies
for identifying the possible causes of acute pan- most common causes of acute pancreatitis in suggest the presence of a mutation of SPINK-1
creatitis in children. MRI has been shown to children, comprising 1030% of all cases [6, (serine protease inhibitor Kazal type 1) or of
have a superior sensitivity compared with that 10, 11, 37, 43, 64]. In children, biliary pan- CFTR (cystic fibrosis transmembrane conduc-
of CT in evaluating peripancreatic collections creatitis has several causes such as common tance receptor) along with pancreas divisum
[60]. MRI better delineates the internal con- bile duct obstruction by gallstones or biliary increases the risk of acute pancreatitis and ac-
tents of a collection, important information in sludge and congenital biliary tree anomalies counts for why only a fraction of patients with
determining the amenability of a collection to [65]. Obesity not only is a risk factor for cho- pancreas divisum develop acute pancreatitis
drainage. Furthermore, similar to CT, MRI can lelithiasis in children but also is an indepen- [38, 58, 59] (Fig. 9B).
identify a safe window before drainage [60]. dent risk factor for the diagnosis of gallstone Congenital dilatation of the bile duct may
MRI has also been shown to play a role in as- pancreatitis. Therefore, obesity can be a dis- lead to pancreatitis as a result of concomi-
sessing the severity of pancreatitis, with at least tinguishing factor between gallstone and tant anomalies of the pancreaticobiliary
one study showing MRI to have a higher sensi- sludge acute pancreatitis in children [12, 65, duct junction [6972] (Fig. 12). Therefore,
tivity than CT for the diagnosis of acute inter- 66]. Cholelithiasis and sludge can be evalu- children with concomitant pancreaticobili-
stitial edematous pancreatitis [61]. ated using ultrasound and MRCP, which will ary maljunction and choledochal cysts often
show filling defects in the biliary tree and present with pancreatitis, with an incidence
Role of Imaging in Determining the biliary tree dilatation; if performed during reported to be as high as 68% [69, 72, 73].
Cause of Acute Pancreatitis an acute event, MRCP will show the pres- Congenital pancreaticobiliary anomalies are
The disorders associated with pancreatitis ence of pancreatic inflammation (Fig. 11). diagnosed in children using mainly MRCP
in pediatric patients fall into several broad cat- Ultrasound is the modality of choice in the or intraoperative cholangiography and less

AJR:206, March 2016 639


Restrepo et al.

Fig. 12Pancreaticobiliary the arterial and venous phases on CT [85,


maljunction in 5-year-old 88]. Focal involvement typically presents as
boy with choledochal cyst
who presented with acute a localized hypodense mass in the pancreatic
pancreatitis. Axial single- head on CT accompanied by obstructive jaun-
shot T2-weighted image dice, mimicking a pancreatic neoplasm [85,
with fat saturation shows
enlarged pancreatic head
8991] (Fig. 14). On MRI, equivalent findings
to CT are seen: hypointensity on T1-weighted
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with adjacent edema (curved


arrows) consistent with imaging and hyperintensity on T2-weighted
focal acute pancreatitis. imaging in the areas involved and no arteri-
Dilated common bile duct
(D) joins pancreatic duct in al phase enhancement but significant venous
center of pancreatic head phase contrast enhancement. The focal or dif-
(straight arrow) indicative fuse strictures of the pancreatic and bile ducts
of pancreaticobiliary
maljunction.
can be well depicted on MRCP [85]. ERCP
and endoscopic ultrasound showing similar
findings may also be useful in the accurate
diagnosis of AIP.

Other Causes of Pancreatitis


frequently using ERCP. However, in patients evated IgG4 levels among other autoanti- Medication-induced pancreatitis accounts
younger than 2 years old, visualization of bodies (antinuclear antibodies, rheumatoid for less than one-fourth of acute pancreati-
the pancreaticobiliary junction may be diffi- factor) in the plasma, and the typical inflam- tis cases in children and is most common-
cult because of its small size and, in cases of matory infiltrates on histopathology [87]. ly caused by valproic acid, l-asparaginase
large choledochal cysts, because of mass ef- There are three recognized patterns of (Fig. 6A), prednisone, and 6-mercaptopurin
fect [57, 59, 67, 72]. glandular involvement in AIP, reflecting in children [7, 11, 43, 64]. Systemic diseas-
their imaging appearance: diffuse, focal, and es causing pancreatitis include sepsis, shock,
Traumatic Pancreatitis multifocal. The diffuse pattern is the most systemic lupus erythematous (Fig. 7B),
Trauma, both accidental and nonacciden- common type; it causes significant diffuse chronic renal failure, inflammatory bow-
tal, accounts for approximately 1040% of sausagelike enlargement of the gland with in- el disease, cystic fibrosis, and diabetes mel-
pediatric pancreatitis cases [7, 9, 11, 37, 74 distinct borders but no significant peripancre- litus [6, 9, 36, 38, 64, 9296]. A hereditary
77]. Less common but of significant impor- atic fat stranding. The affected gland is hy- etiologic mechanism of pancreatitis, an au-
tance is post-ERCP pancreatitis [7882]. poechoic on ultrasound and low density on tosomal-dominant disease, is difficult to elu-
Pancreatic injuriesincluding pancreatitis CT with the characteristic delayed enhance- cidate because it is nearly indistinguishable
in a young child should be suspected to be ment of late phases. Irregular or segmental clinically or by imaging from other causes
abuse-related (Fig. 13): It is estimated that narrowing of the main pancreatic duct and aside from early age of onset, with recur-
one-third of all posttraumatic pancreatitis extrahepatic bile ducts, especially the intra- rent events occurring during the first decade
are nonaccidental [7882]. In the acute set- pancreatic common bile duct, may also oc- of life and most often with a family history
ting, ultrasound and CT are the most helpful cur. A capsulelike hypodense rim along the [97]. Although uncommon, metabolic abnor-
techniques for confirming the cause because periphery of the gland has been found in both malities including hypercalcemia, hypertri-
of their availability in the emergency setting.
Imaging findings vary from an enlarged,
edematous, and indistinct pancreas with ad-
jacent fat stranding in cases of pancreatitis,
to a linear disruption in the gland in cases of
laceration, to complete pancreatic separation
in cases of transection [83, 84] (Fig. 13).

Autoimmune Pancreatitis Fig. 13Nonaccidental


Autoimmune pancreatitis (AIP) is a mani- pancreatic transection
festation of an IgG4 systemic disease, which in 18-month-old boy
who presented with
is characterized by a systemic chronic fibro- abdominal distention.
inflammatory process that affects virtually Axial contrast-enhanced
every organ [85, 86]. AIP is rare, with only CT scan of abdomen
shows hypodensity in
nine cases reported in the pediatrics litera- pancreatic neck (arrow)
ture as of 2011. Hence, AIP poses a clini- with separation of
cal and radiologic diagnostic challenge. AIP pancreatic fragments
is diagnosed in a child with acute recurrent (P) consistent with
pancreatic transection.
pancreatitis or obstructive jaundice by the There is massive ascites
presence of typical radiologic findings, el- (asterisks).

640 AJR:206, March 2016


Acute Pancreatitis in Pediatric Patients

glyceridemia, diabetic ketoacidosis, and in- Fig. 14Focal


born errors of metabolism, have been found pancreatic head
involvement of
to be associated with the development of autoimmune
acute pancreatitis [98100]. pancreatitis in 7-year-
old girl. Patient
presented with
Complications of Acute Pancreatitis obstructive jaundice.
Complications of acute pancreatitis in
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Axial contrast-
children are sterile and infected collections, enhanced CT scan
fistulas, and vascular complications. The shows focal round
hypodensity (H) causing
most common complication in pediatric pan- pancreatic head
creatitis is the development of pseudocysts, enlargement (arrows).
which was reported to occur in 13% of pa-
tients in the analysis of studies in the litera-
ture by Benifla and Weizman [15].

Pancreatitis-Related Collections
No classification or definition of pancreati-
tis-related collections in children has been pro-
posed to date, but the complications are simi-
lar to those that occur in adults; therefore, the
Atlanta classification [101] can be useful. The
last revised Atlanta classification that includes rotizing pancreatitis, either in the pancreat- tion is anterior and is not walled off, pancre-
only adults makes a clear distinction between ic parenchyma or peripancreatic tissues. An atic ascites often develops (Fig. 13), whereas
the different collections and classifies them ac- ANC contains necrotic tissue and has poorly posterior duct disruption may cause pancre-
cording to whether they are associated with defined borders (Figs. 5B and 7B). The dis- aticopleural fistulas. The development of pan-
acute interstitial edematous pancreatitis or nec- tinction between an ANC and an acute peri- creaticopleural fistulas can be and more fre-
rotizing pancreatitis [15, 23, 101]. pancreatic fluid collection during the first quently is the consequence of a leak from an
week of disease is difficult but becomes easi- incompletely formed or ruptured pseudocyst
Collections Associated With Acute Edematous er thereafter by the presence of solid material through the aortic or esophageal hiatus. These
Interstitial Pancreatitis in the ANC. MRI or ultrasound can be useful types of fistulas usually present as persistent
Acute peripancreatic fluid collections oc- in establishing the differences. Walled-off ne- or recurrent ascites or pleural effusions. CT,
cur in the early phase of interstitial edematous crosis consists of necrotic pancreatic or peri- MRCP, and ERCP are the current studies of
pancreatitis. On contrast-enhanced CT, acute pancreatic tissue surrounded by an enhancing choice for the diagnosis of pancreatic ascites
peripancreatic fluid collections do not have a inflammatory capsule (Fig. 7C) that rarely de- and pancreaticopleural fistulas [27, 101].
well-defined wall, are homogeneous, may be velops before 4 weeks from the onset of nec-
multiple, and may be confined to retroperi- rotizing pancreatitis. Walled-off necrosis may Conclusion
toneal fascial planes (Fig. 6B). Most of these be multiple, may occur at distant sites, or may The incidence of acute pancreatitis in chil-
collections resolve spontaneously. On the oth- become infected [101]. dren has been increasing for the past 2 de-
er hand, pancreatic pseudocysts are a delayed cades, probably as a result of multifactorial
complication occurring usually after 4 weeks Vascular Complications of causes. The INSPPIRE Group [2] was formed
of developing an acute peripancreatic flu- AcutePancreatitis to standardize definitions, develop diagnostic
id collection. Pseudocysts are the most com- Vascular complications of acute pancreati- algorithms, investigate disease pathophysiolo-
mon complication with a reported incidence tis may involve the arterial or venous system gy, and design multicenter studies in pediatric
of 13% [15, 101]. Pseudocysts are thought to and are triggered by extravasated pancreatic pancreatitis. The diagnosis of acute pancreati-
arise from disruption of the main pancreatic enzymes causing the loss of vessel wall integ- tis in children is based on clinical symptoms,
duct or a branch with no pancreatic parenchy- rity. Arterial complications include a pseudoa- laboratory values (amylase and lipase values),
mal necrosis, leading to a round or oval flu- neurysm of neighboring arteries or hemorrhage and imaging findings with two of three crite-
id collection with a well-defined wall in the secondary to the rupture of a pseudoaneurysm ria necessary to make the diagnosis. The clin-
peripancreatic tissues (Figs. 5A and 7A). Pseu- or erosion of a major artery. In the venous sys- ical presentation of acute pancreatitis in chil-
docysts may occasionally be partly intrapan- tem, thrombosis is a well-known complication dren can be atypical, which emphasizes the
creatic but should not contain solid material; that more commonly affects the splenic vein. usefulness of imaging for diagnosis. Ultra-
therefore, ultrasound or MRI may be required sound is currently the first imaging modality
to accurately characterize their contents [101]. Pancreatic Fistulas used in the diagnosis of acute pancreatitis in
Pancreatic ascites and pancreaticopleural pediatric patients. CT is reserved for imaging
Collections Associated With fistulas are two uncommon types of internal sicker children who do not improve or who de-
NecrotizingPancreatitis pancreatic fistulas resulting from pancreat- teriorate clinically. MRI is used to elucidate
An acute necrotic collection (ANC) arises ic duct disruption and subsequent leakage of the underlying causes of pancreatitis in pedi-
in the first 4 weeks after development of nec- pancreatic fluid. If the pancreatic duct disrup- atric patients.

AJR:206, March 2016 641


Restrepo et al.

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