Vascular Medicine Patient with both Wilkie syndrome and nutcracker syndrome

Vascular Medicine
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Patient with both Wilkie syndrome and nutcracker syndrome
Michel K Barsoum, Roger FJ Shepherd and Timothy J Welch
Vasc Med 2008 13: 247
DOI: 10.1177/1358863X08092272
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Vascular Medicine 2008; 13: 247–250
Patient with both Wilkie syndrome and nutcracker syndrome
Michel K Barsoum1, Roger FJ Shepherd1 and Timothy J Welch2
Abstract: Wilkie syndrome or superior mesenteric artery syndrome is a rare cause of
duodenal obstruction in pediatric and adult age groups. Its manifestations are due to
compression of the third portion of the duodenum between the aorta and the superior mesenteric artery. We present one case of a 29-year-old female with a recent history of epigastric pain and severe weight loss due to duodenal obstruction from
superior mesenteric syndrome. She was treated conservatively with jejunostomy
tube feeding to regain loss of weight. Our case is unique in that the patient has also
nutcracker syndrome.
Key words: duodenal obstruction; loss of weight; megaduodenum; nutcracker
syndrome; superior mesenteric artery syndrome; Wilkie syndrome
Introduction
Case history
The differential diagnosis of duodenal obstruction is
broad. One of its rare causes is Wilkie syndrome or
superior mesenteric artery syndrome. Wilkie syndrome is arteriomesenteric obstruction of the duodenum that is characterized by compression of the
third portion of the duodenum by the superior
mesenteric artery as it passes over this portion of
the duodenum. Vascular compression of the duodenum is a rare condition that presents with profound
nausea and vomiting, abdominal distention, weight
loss, and post-prandial epigastric pain. Epigastric
pain varies from intermittent to constant depending
on the severity of the duodenal obstruction. Weight
loss often starts before the onset of other symptoms.
This report describes a female adult who was
admitted to our hospital with this diagnosis and
was managed conservatively using jejunostomy
tube feeding. The patient was found incidentally to
have nutcracker syndrome due to left renal vein compression between the superior mesenteric artery and
the aorta. The patient continued to have normal
renal function and she was managed conservatively.
The purpose of this case report is to discuss the diagnosis and conservative management of a patient with
both Wilkie syndrome and nutcracker syndrome.
A 29-year-old female was transferred to our hospital
for management of duodenal obstruction. Her past
surgical history was notable for resection of a brain
stem ganglioma, with residual dysarthria and general debility. Three years previously, she was treated
for malnutrition and pneumonia following a laminectomy. The previous summer, she complained of
early satiety and sharp post-prandial epigastric
abdominal pain occurring half an hour after eating
associated with anorexia and significant weight loss
of 20–30 pounds. The abdominal pain was relieved
with lying on the left side or on her back. Endoscopic gastroduodenoscopy (EGD) revealed erythematous gastropathy. Two months later, she was
admitted with large bilious emesis and worsening of
her epigastric pain. The patient denied any urinary
symptoms. An abdominal computed tomography
(CT) scan and an upper gastrointestinal Gastrografin study demonstrated compression of the third
portion of the duodenum by the overlying superior
mesenteric artery. It also revealed an opacified renal
collecting system with contrast material but there
was no obvious opacification of the ureters or bladder. EGD was repeated and confirmed a high-grade
focal extrinsic obstruction of the mid-third portion
of the duodenum that was not present 2 months
previously. The patient was transferred to our hospital for further evaluation.
Physical examination revealed a cachectic female
with a height of 170 cm and weight of 39.9 kg. The
abdomen was soft and non-tender with normal
bowel sounds, but a fluid wave was present suggesting ascites.
1
Division of Cardiovascular Diseases, Section of Vascular
Diseases, Mayo Clinic College of Medicine, Rochester, MN,
USA; 2Department of Radiology, Mayo Clinic College of
Medicine, Rochester, MN, USA
Correspondence to: Michel K Barsoum, Mayo School of
Graduate Medicine, Mayo Clinic, 200 First Street Southwest,
Rochester, MN 55905, USA.
Email: [email protected]
© 2008 SAGE Publications, Los Angeles, London, New Delhi and Singapore
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10.1177/1358863X08092272
248
MK Barsoum et al.
Figure 1 CT scan of abdomen demonstrating duodenal
compression between the superior mesenteric artery and
aorta.
Figure 3 CT scan of the abdomen and pelvis showing
impingement upon the left renal vein by the superior
mesenteric artery.
Laboratory studies with a reference range
revealed mild anemia with hemoglobin of 11.1 g/dl
(12.0–15.5 g/dl), normal leukocyte count of 4.9 ×
109/l (3.5–10.5 × 109/l), normal serum urea nitrogen
of 12 mg/dl (6–21 mg/dl), creatinine of 0.3 mg/dl
(0.6–1.1 mg/dl), serum sodium of 146 mmol/l
(135–145 mmol/l), serum potassium of 4.7 mmol/l
(3.6–4.8 mmol/l), alanine aminotransferase of 20
U/l (8–43 U/l), alkaline phosphatase of 93 U/l (37–
98 U/l) and albumin of 3.3 g/dl (3.5–5.0 g/dl). Prealbumin and urine analysis were not checked.
An abdominal CT confirmed extrinsic compression of the duodenum associated with an unusual
inferior location of the superior mesenteric artery
(SMA) (Figures 1 and 2). It also revealed diffuse
ascites and bilateral pleural effusions. There was
impingement of the left renal vein by the SMA
with multiple venous collaterals indicative of nut-
cracker syndrome (Figure 3). An EGD was
obtained and revealed bile-induced gastropathy
but no overt duodenal obstruction at the site of the
SMA.
In view of normal renal function with normal
blood pressure, no further intervention was necessary for treating her nutcracker syndrome. The
patient’s massive weight loss with loss of intraabdominal fat was felt to be a major contributing
factor to the unusual vascular compression of the
duodenum by the overriding SMA. The patient
was treated conservatively through enteral nutrition
support with a jejunostomy feeding tube in order to
gain weight and this was effective in reducing her
epigastric abdominal pain.
Discussion
Figure 2 Upper gastrointestinal Gastrografin study
showing duodenum obstruction with marked dilatation.
Duodenum compression by the SMA was first
described in 1861 by Von Rokitansky. Since then,
it has acquired various names such as Cast syndrome due to its increased incidence in patients
requiring orthopedic casting and Wilkie syndrome
in reference to a series of 75 cases reported by Dr
Wilkie with chronic duodenal ileus.1–3 SMA syndrome is more common in females than males,
with a predominance in the mid-30 to mid-40 age
groups, but it can occur in older children and
adolescents.4 Although rare, it should always be
considered in the differential diagnoses of duodenal
obstruction (Table 1). The entity of SMA syndrome
has been controversial. It may be over-diagnosed in
some patients due to a similarity with other causes
of duodenal obstruction.5 Radiological imaging
studies may also show discordant findings. Moreover, after surgical correction, some symptoms
such as vomiting persist, while others like weight
loss may improve.6
Vascular Medicine 2008; 13: 247–250
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Wilkie syndrome and nutcracker syndrome
Table 1 Causes of duodenal obstruction
Duodenal hypoplasia
Duodenal stenosis
Duodenal atresia
Peptic ulcer disease
Congenital bands
Intestinal malrotation
Annular pancreas
Pre-duodenal portal vein
Superior mesenteric artery syndrome
Causes of SMA syndrome
SMA syndrome has been associated with postorthopedic spine surgeries (e.g. scoliosis).7 It is
more common in thin individuals due to loss of the
duodenal fat pad. Neurological injury, as in our
patient, is a common risk factor. In one series of
SMA syndrome in children, neurological injury
such as paraplegia or cerebral palsy was present in
50% of patients.4 Various mechanisms are hypothesized associated with the anatomical relation of the
duodenum to neighboring structures. The duodenum is suspended by its attachment to the ligament
of Treitz as it crosses the angle formed between the
aorta and SMA. Therefore, an unusual low origin of
the SMA, compression of the duodenum due to
peritoneal adhesions, or high insertion of the duodenum at the ligament of Treitz will lead to narrowing
of the aorto-mesenteric angle leading to entrapment
of the third portion of duodenum between the SMA
and the aorta.8 The narrowing of the angle can be
aggravated by depleted mesenteric fat from severe
loss of weight.
Clinical presentation
Symptomatology varies between acute onset to a
gradually progressive course of early satiety and
post-prandial abdominal pain. Typical features
include pain aggravated by a supine position and
relieved by prone or left lateral decubitus, or even
by a knee–chest position. Other symptoms include
bilious vomiting with loss of appetite and weight,
which can occur with advanced obstruction.9
These patients might present with manifestations
of electrolyte imbalance or with gastrointestinal
obstructive complications including massive gastric
dilatation with gastric wall pneumatosis and portal
venous gas.10 A physical examination is non-specific
with findings including a thin body build, abdominal distension or high-pitched bowel sounds and
signs of intestinal obstruction.
Diagnosis
This is a difficult diagnosis to make based on clinical
symptoms and signs which are mostly non-specific.
Radiological imaging studies are needed to look for
SMA syndrome or to confirm its presence. A plain
249
abdominal radiograph may be an initial study to
look for dilated gastric or proximal duodenal dilatation. A barium upper gastrointestinal series study
or hypotonic duodenography might demonstrate
the cessation of flow of barium from the duodenum
to the jejunum.11 An EGD or CT scan of the abdomen could be helpful confirmatory studies.12 These
modalities may reveal extrinsic compression of the
third portion duodenal obstruction and narrow
angle between the aorta and SMA. There is a case
report about using endoscopic ultrasound for diagnosing SMA syndrome in children.13
Treatment
In the majority of cases, conservative management to
treat electrolyte imbalance and provide adequate
nutrition using either enteral or parenteral feeding
will result in weight gain and improved
symptoms.14 In addition, proper positioning of the
patient after eating, especially in the left lateral decubitus, prone, or even knee-to-chest position may be of
benefit. Surgery can be considered when conservative
measures fail. Surgical options include simple release
of the ligament of Treitz, or through reconstructive
approaches such as open or laparoscopic duodenojejunostomy or Roux-en-Y reconstruction.15,16 However, there is little long-term data to support the success of surgery to relieve symptoms, except in
refractory emesis after the failure of all conservative
measures.6,17 Another surgical approach is through
infrarenal transposition of the SMA. This approach
is done to avoid dumping syndrome through disruption of the physiologic intestinal continuity due to
small-bowel bypass-surgical procedures.15
Description of nutcracker syndrome
Our patient presents with another rare compression
syndrome of the left renal vein (LRV). Nutcracker
syndrome is due to LRV compression between
SMA and abdominal aorta. It was described first in
1937 by Grant18 with the first venography done by
Chait, et al.19 The nutcracker syndrome leads to elevated renal venous pressure with formation of varices
in the renal pelvis and ureter. Complications include
consequent hematuria, proteinuria and left flank
pain that were absent in our patient. Diagnosis is
obtained after excluding other causes of hematuria.
Imaging studies such as ultrasonography, magnetic
resonance angiography, and computed tomographic
angiography, as well as measuring pressure gradients
between the inferior vena cava and LRV during
venography are helpful in confirming this diagnosis.
Surgical options may be performed in symptomatic
patients. These procedures include nephropexy,
transluminal balloon angioplasty with stenting,20
renal vein bypass, transposition of LRV, mesoaortic
transposition, and renal autotransplantation.
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MK Barsoum et al.
Conclusion
Wilkie syndrome and nutcracker syndrome are rare
compression syndromes. The diagnosis and treatment of Wilkie syndrome is controversial. The initial management should include gastric andduodenal decompression, treatment of fluid and
electrolyte imbalance as well as reversal of weight
loss using enteral and parenteral feeding. Surgery
may be appropriate in some patients who continue
to have debilitating symptoms after a good trial of
conservative therapy.
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