This case report describes a 14-year-old female who presented with abdominal pain and weight loss. She was found to have both biliary dyskinesia and superior mesenteric artery syndrome (SMA syndrome). Biliary dyskinesia was confirmed by a HIDA scan showing poor gallbladder function. An upper GI series showed duodenal obstruction consistent with SMA syndrome due to rapid weight loss. She underwent open cholecystectomy and duodenojejunostomy to address both conditions simultaneously. Postoperatively, she continued to experience gastric symptoms requiring nutritional support. She was eventually discharged tolerating full feeds.

1. Two birds, one surgical stone: The first reported case of superior
mesenteric artery syndrome secondary to biliary dyskinesia
Shani Fruchter DO *, Jessica Marshall DO, Frederick Alexander MD, FAAP, FACS
Palisades Medical Center, North Hudson, NJ, USA
a r t i c l e i n f o
Article history:
Received 27 March 2016
Received in revised form
14 June 2016
Accepted 16 June 2016
Key words:
Biliary dyskinesia
Superior mesenteric artery syndrome
Duodenojejunostomy
a b s t r a c t
This is the first reported case of superior mesenteric artery syndrome secondary to biliary dyskinesia.
SMA syndrome involves obstruction of the third portion of the duodenum, causing a gastric outlet
obstruction due to narrowing of the space between the abdominal aorta and the superior mesenteric
artery (SMA). Rapid weight loss has been shown to be a risk factor for this condition. We report a case
found in a 14-year old Hispanic developmentally delayed female and review the literature. Our patient
presented with a one-month history of abdominal pain, bilious emesis, and weight loss. She was seen at
an outside facility where she had two abdominal operations without symptomatic relief. A HIDA scan
performed at our facility was consistent with biliary dyskinesia, and an UGIS confirmed a concomitant
SMA syndrome. An open cholecystectomy with a side-to-side duodenojejunostomy was performed.
Postoperatively, patient continued with gastropareisis, requiring nutritional support via TPN and inser-
tion of a jejunostomy tube. On post operative day 24, patient was discharged home tolerating full feeds.
She has since been seen in follow up, and is doing well.
Ó 2016 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND
license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
1. Purpose
To perform a thorough literature review to ascertain the most
appropriate surgical treatment for SMA syndrome having resulted
from undiagnosed biliary dyskinesia.
Biliary dyskinesia is a motility disorder affecting the gallbladder
and the sphincter of Oddi. Symptoms include nausea RUQ
abdominal and epigastric pain, usually after a meal high in fat or
cholesterol. It is often diagnosed in children with chronic abdom-
inal pain [1]. Ultrasonography is usually unrevealing for choleli-
thiasis or other structural causes. The condition can be confirmed
by a HIDA scan with CCK showing a poor gallbladder ejection
fraction (<35e40%). [2e5], with Carney et al. showing a PPV of 93%
in patients with pain, nausea and an EF <15%. The cause is believed
to be defects in the function and distribution of cholecystekinin
receptors on the cells of the gallbladder wall leading to abnormal
response to the hormone. No medical therapy has been found to be
effective. Cholecystectomy has been shown to be curative in
70e90% of select patients [2].
Superior mesenteric artery syndrome, also referred to as Wil-
kie’s syndrome, is a syndrome characterized by extrinsic
compression of the third portion of the duodenum which lies be-
tween the abdominal aorta and the superior mesenteric artery. The
compression causes an obstruction to outflow of gastric contents.
Patients experience most commonly abdominal pain, nausea, and
vomiting. Weight loss is not only a symptom, but is also a risk factor
for this disease process, as loss of omental fat density may be the
cause for the acute change in the aortic-mesenteric artery angle [6].
Common complications of prolonged SMA syndrome include
electrolyte abnormalities and their sequelae, nutritional de-
ficiencies, and gastric perforation. Conservative treatments include
nasogastric tube decompression of the obstructed stomach, nutri-
tional support via PPN or TPN, and correction of electrolyte im-
balances. Several surgical options are known to be available if these
methods fail, including duodenojejunostomy, gastrojejunostomy
[7] and resection of the Ligament of Treitz [6].
2. Case history
We present the case of a 12-year old female with a 1 month history
of intermittent, crampy abdominal pain associated with emesis. She
was recently admitted to an outside hospital for similar complaints
where she underwent a diagnostic laparoscopy with appendectomy
* Corresponding author. Palisades Medical Center, Graduate Medical Education,
7600 River Road, North Hudson, New Jersey 07047, USA. Tel.: þ1 2018545000.
E-mail address: sjfruchter@gmail.com (S. Fruchter).
Contents lists available at ScienceDirect
Journal of Pediatric Surgery CASE REPORTS
journal homepage: www.jpscasereports.com
2213-5766/Ó 2016 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
http://dx.doi.org/10.1016/j.epsc.2016.06.007
J Ped Surg Case Reports 12 (2016) 38e40

2. and cystectomy for a right paraovarian cyst. She was discharged after 4
days only to return 2 days later with persistent abdominal pain and
vomiting. She then had several imaging studies, including an abdom-
inal CT scan, stool studies and a brain MRI. A GI consult performed an
upper endoscopy with biopsies and a diagnosis of Helicobacter pylori
infection was made by stool studies. She was then started on
amitriptyline and Reglan and was treated for H. pylori infection as an
outpatient. She showed signs of Reglan induced dystonic reactions
including hypertension resulting in discontinuation of Reglan. Review
of systems was noted for abdominal pain, nausea, anorexia including
11 kg weight loss in the past two months with a BMI of 15.5. Past
medical and surgical history was notable for developmental delay for
which she was receiving physical, occupational and speech therapy; as
mentioned she had a recent appendectomy and cystectomy. On
physical exam, she was tachycardic and her abdomen was soft, non-
distended with tenderness in the right upper quadrant. Laboratory
data showed no leukocytosis, mildly elevated AST and ALT, normal
bilirubin levels as well as CRP and ESR. Abdominal obstructive series
revealed a non-obstructive bowel gas pattern and abdominal ultra-
sound showed gallbladder sludge, no gallbladder wall thickening or
pericholecystic fluid. At this point, a diagnosis of biliary dyskinesia was
suspected, with a differential diagnoses including gastroparesis, gall-
bladder pathology, brain tumor, and bulimia. Further work up with a
HIDA scan and an upper GI series with small bowel follow through was
planned and a GI consult recommended protonix IV.
On hospital day 2 a HIDA scan showed an ejection fraction of
22%; a diagnosis of biliary dyskinesia was then confirmed. An upper
GI series with small bowel follow through showed mild dilatation
of the second portion of the duodenum with delayed progression of
contrast through the third portion of the duodenum as well as mild
hyperperistalsis and retrograde peristalsis in the second portion of
duodenum (Figs. 1 and 2). Given her history of rapid weight loss,
this was felt to be consistent with SMA syndrome.
After failing a course of conservative management including
bowel rest and nasogastric decompression, our patient was taken to
the OR on hospital day 6 for an open cholecystectomy with side-to-
side duodenojejunostomy. The gallbladder was found to be large,
dilated, and thickened with multiple omental adhesions which
were lysed with electrocautery. A top down dissection of the gall-
bladder was performed. The Kocher maneuver was performed to
expose the duodenum. A loop of jenumun 30 cm from ligament of
Treitz was brought up to the anterior sidewall of duodenum
through transverse mesocolon. A side-to-side anastomosis was
created using a GIA stapler and was reinforced internally with
hand-sewn running suture. The mesocolon was sutured circum-
ferentially to the duodenum.
Postoperatively our patient continued to experience nausea and
abdominal pain. On post operative day two she began having
emesis but did not tolerate a nasogastric tube. She was started on
Toradol, Phenergen, Tylenol and Dilaudid PRN.
On post operative day three an obstructive series showed a
distended stomach with air-fluid levels and residual barium within
pelvis from a prior study.
The following day a PICC line was placed for TPN. Her abdominal
pain had resolved but she was still having emesis. An upper GI was
performed which showed contrast progression with slight
narrowing at the duodenojejunal anastamosis site.
Our patient continued to have poor PO tolerance and episodes of
vomiting, and so TPN was continued. On post operative day 10 an
UGIS showed contrast pooling in the stomach with poor progres-
sion to the duodenum. We then placed a gastrojejunostomy tube for
gastric decompression and jejunal feeds. Our patient slowly began
gaining weight and was discharged home on post operative day 24,
tolerating PO feeds and in good spirits.
3. Method
A thorough literature review was conducted of articles printed
in English in PubMed between January of 2000 and November of
2014. We reviewed the medical and surgical treatments for biliary
dyskinesia. We then focused our review towards the surgical
management options for SMA syndrome as reported in case reports
and case series presented in PubMed.
4. Results
Upon review, cholecystectomy was the primary treatment for
biliary dyskinesia [1,2] and duodenojejunostomy was found to be
the preferred surgical treatment for SMA syndrome [3,7,8].
Fig. 1. Upper GI series with small bowel follow through showing mild dilatation of D2
with slow transition of contrast through D3, concerning for SMAS.
Fig. 2. Upper GI series with small bowel follow through showing mild dilatation of D2
with slow transition of contrast through D3, concerning for SMAS.
S. Fruchter et al. / J Ped Surg Case Reports 12 (2016) 38e40 39

3. Therefore, it was determined that it is not only feasible but pref-
erable to perform both procedures during the same operation.
We combined our results from research on two separate pa-
thologies to determine a unified treatment plan in hopes of
resolving both interconnected disease processes. Our solution was
one operation to treat both surgical pathologies.
5. Discussion
Biliary dyskinesia is the dysfunction of the autonomic nervous
system of the gallbladder leading to uncoordinated contraction and
relaxation of the gallbladder and sphincter of Oddi, leading to
distension, inflammation and dysfunction [3]. It is diagnosed via a
cholecystokinin stimulated hepatobiliary scan, demonstrating a
gallbladder ejection fraction less than 35%. Accepted treatment is
with laparoscopic cholecytectomy, demonstrating a 67e100% long
term success rate [3,9].
Superior mesenteric artery syndrome involves the entrapment
of the third portion of the duodenum between the aorta and
superior mesenteric artery. This occurs when the aortomesenteric
angle reduced from the normal of 10e28 mm to 2e8 mm [8], which
generally occurs after rapid weight loss [10,11]. This is diagnosed
with barium studies and can be confirmed with CT measuring the
angle [11]. Different surgical remedies have been described,
including duodenojejunostomy, gastrojejunostomy [7] and resec-
tion of the Ligament of Treitz [6].
This is the first reported case of SMA syndrome as a result of
biliary dyskinesia. Biliary dyskinesia is treated most commonly
with a laparoscopic cholecystectomy [2e5], and current consensus
is to treat refractory cases of SMA syndrome with a duodenojeju-
nostomy [7,10].
Our research has led us down two paths, one investigating the
standard of treatment for biliary dyskinesia and one for SMA
syndrome. It is our belief that treating the biliary dyskinesia may
have enabled the SMA syndrome to resolve, but by treating both
pathologies in one operation, we provided our patient with several
advantages, including the following: a faster recovery time to
normal gastrointestinal motility and relief of emesis, a shorter
length of stay, and elimination of a possible second operation. We
believe a two-stage procedure would have been more difficult in
this patient due to the likely formation of adhesions, and that due to
social issues our patient would have required prolonged
hospitalization.
6. Conclusion
It is our conclusion that surgically treating a patient with SMA
syndrome secondary to biliary dyskinesia with a cholecystectomy
and gastrojejunostomy during one operation is optimal.
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