Keywords
hollow viscus compressions - SMA syndrome - SMV syndrome - testicular vein syndrome
- vascular compressions
Introduction
Abdominopelvic vascular and hollow viscus compression syndromes encompass a spectrum
of conditions where either blood vessels compress the hollow viscera or vice versa.
This can lead to diverse symptoms and pose diagnostic challenges, particularly when
they manifest incidentally in asymptomatic individuals or with atypical symptoms.
Examples include Wilkie's syndrome, ureteropelvic junction obstruction, portal biliopathy
(compression of hollow viscera by adjacent blood vessels), and Dunbar syndrome, nutcracker
syndrome, and May–Thurner syndrome (compression of vessels by adjacent structures).[1] These syndromes may arise due to various factors such as anatomical anomalies, rapid
weight loss, surgical alterations, or congenital abnormalities.
The diagnosis of these syndromes often requires advanced imaging techniques such as
contrast-enhanced computed tomography (CECT) or ultrasound, which can reveal compression
of structures and aid in establishing the underlying cause. However, due to their
rarity and diverse presentations, these syndromes may go undiagnosed or misdiagnosed,
leading to delays in appropriate management.
Cases
Case 1
A 22-year-old male presented with complaints of postprandial epigastric discomfort,
fullness, indigestion, and reflux for the past 12 months. His symptoms were relieved
spontaneously a few hours after a meal. Upper gastrointestinal (GI) endoscopy did
not reveal any significant finding. The patient's symptoms were not relieved by proton-pump
inhibitors and other antireflux medications. CECT abdomen (ABD) was done and showed
the compression of mid portion of D3 segment of the duodenum between superior mesenteric
vein (SMV) and its venous tributaries and aorta. The stomach and D1 and D2 segments
of the duodenum appeared distended. [Fig. 1A] shows CECT-ABD (venous phase) axial sections at the level of D3 segment. [Fig. 1B] shows CECT-ABD (venous phase) oblique coronal section of the SMV and its tributaries.
To demonstrate the functional status of this mechanical compression, we proceeded
with barium meal study, which showed the distension of second and proximal third parts
of the duodenum with a persistent filling defect at the mid portion of D3 segment
likely due to extrinsic compression. [Fig. 2A] shows right anterior oblique projection of contrast-filled C-loop of the duodenum
with extrinsic compression at mid portion of D3. [Fig. 2B] shows supine anteroposterior projection of partial hold-up of contrast in D2 and
proximal D3 segments with a persistent filling defect at mid D3. Hence, the diagnosis
of superior mesenteric artery (SMA)-like syndrome (SMV syndrome) was made.
Fig. 1 (A) CECT-ABD (venous phase) axial sections at the level of D3 segment. (B) CECT-ABD (venous phase) oblique coronal section showing SMV and its tributaries.
CECT ABD, contrast-enhanced computed tomography abdomen; SMV, superior mesenteric
vein.
Fig. 2 (A) RAO projection displaying contrast-filled C-loop of duodenum with extrinsic compression
at mid portion of D3. (B) Supine anteroposterior (AP) projection displaying partial hold-up of contrast in
D2 and proximal D3 segments with persistent filling defect at mid D3. RAO, right anterior
oblique.
Case 2
A 47-year-old male presented with complaints of left loin pain for 4 weeks. The pain
was intermittent in nature and was not relieved by nonsteroidal anti-inflammatory
drugs. No history of renal/ureteric calculi was given by the patient. No recent fever
episodes as alleged by the patient. The patient was referred for ultrasound kidney,
ureter, and bladder (US-KUB) and the study showed moderate left hydronephrosis and
proximal hydroureter. [Fig. 3] shows US image (longitudinal view) of hydronephrosis of the left kidney. [Fig. 4] shows US image (longitudinal view) of the proximal left hydroureter. No proximal
ureteric or left vesicoureteric calculus was noted. No obvious cause for hydroureteronephrosis
(HUN) was detected in the study. So, we proceeded with CECT-KUB, which showed left
moderate HUN up to the testicular vein crossover at the level of L4 vertebra with
smooth narrowing/kinking. The portion of the ureter distal to the crossover was unremarkable.
The testicular vein at the crossover appeared pristine and was of normal caliber (3.2 mm).
[Fig. 5] shows CECT-KUB (corticomedullary phase) axial section at the level of renal pelvis
showing left HUN. [Fig. 6] shows CECT-KUB (excretory phase) axial section at the level of the left testicular
vein crossover. [Fig. 7] shows CECT-KUB (venous and excretory phases) coronal section at the level of the
left testicular vein crossover.
Fig. 3 US abdomen image (longitudinal view) showing hydronephrosis of left kidney. US, ultrasound.
Fig. 4 US abdomen image (longitudinal view) showing proximal left hydroureter. US, ultrasound.
Fig. 5 CECT-KUB (corticomedullary phase) axial section at the level of renal pelvis showing
left HUN. CECT-KUB, contrast-enhanced computed tomography kidney, ureter, and bladder;
HUN, hydroureteronephrosis.
Fig. 6 CECT-KUB (excretory phase) axial section at the level of left testicular vein crossover.
CECT-KUB, contrast-enhanced computed tomography kidney, ureter, and bladder.
Fig. 7 CECT-KUB (venous and excretory phases) coronal section at the level of left testicular
vein crossover. CECT-KUB, contrast-enhanced computed tomography kidney, ureter, and
bladder.
Case 3
A 15-year-old normal female child with no developmental abnormalities presented with
complaints of left lower limb swelling and pain predominantly on the proximal aspect
for the past 4 days. The patient had history of chronic constipation, which was gradually
progressive in nature along with on-and-off episodes of abdominal pain. All the initial
blood investigations including the coagulation profile were within normal limits.
We performed left lower-limb venous Doppler study in the patient, which showed echogenic
thrombus within the lumen of visualized portions of common femoral vein extending
into the superficial femoral vein ([Fig. 8]). Neither color flow nor spectral wave pattern could be demonstrated in those veins.
The abovementioned veins were incompressible and showed no flow augmentation on distal
compression. Since we were not able to arrive at the cause of deep vein thrombosis
in the otherwise healthy child and also due to her complaints of abdominal pain and
constipation, we proceeded with CECT- ABD study, which showed us gross dilatation
of sigmoid colon and rectum with significant fecal impaction measuring approximately
41 × 16 × 15.5 cm extending up to the level of left hemidiaphragm, compressing and
causing thrombosis of the left common iliac, external iliac veins, and visualized
portions of left common femoral vein. [Fig. 9] shows CECT-ABD (arterial phase) coronal section showing gross fecal impaction in
the rectum and sigmoid colon. [Fig. 10] shows CECT-ABD (venous phase) sagittal section showing pressure effect on the urinary
bladder and uterus. [Fig. 11] shows CECT-ABD (venous phase) axial section showing thrombosed left common iliac
vein. [Fig. 12] shows CECT-ABD (venous phase) volume rendering image showing nonvisualization of
the left common iliac, external iliac, and common femoral veins and normal appearing
veins on the contralateral side.
Fig. 8 Left lower-limb venous Doppler study in the patient, which showed echogenic thrombus
within the lumen of visualized portions of common femoral vein extending into the
superficial femoral vein.
Fig. 9 Contrast-enhanced computed tomography abdomen (CECT-ABD) (arterial phase) coronal
section displaying gross fecal impaction in the rectum and sigmoid colon.
Fig. 10 Contrast-enhanced computed tomography abdomen (CECT-ABD) (venous phase) sagittal
section showing pressure effect on the urinary bladder and uterus.
Fig. 11 Contrast-enhanced computed tomography abdomen (CECT-ABD) (venous phase) axial section
showing thrombosed left common iliac vein.
Fig. 12 Contrast-enhanced computed tomography abdomen (CECT-ABD) (venous phase) volume rendering
image displaying nonvisualization of left common iliac, external iliac, and common
femoral veins and normal appearing veins on the contralateral side.
Discussion
The first case is a case of SMA-like syndrome (SMV syndrome). The risk factors for
SMA-like syndromes include various causes of megaduodenum-like systemic sclerosis,
dermatomyositis, systemic lupus erythematosus, diabetes mellitus, amyloidosis, and
chronic idiopathic intestinal pseudo-obstruction (Ogilvie's). Other risk factors include
malrotation of midgut, congenitally shortened suspensory ligament of the duodenum
(high placed insertion), rapid and severe weight loss, iatrogenic alteration of anatomy
(GI surgeries), surgical correction of scoliosis, and chronic external compression
(hip spica cast). Only eight such cases have been reported in the literature.[2]
[3]
[4]
[5] Five out of these eight cases were duodenal compressions between SMV and inferior
vena cava. One case was due to postsurgical alterations in anatomy, while another
was due to an anomalous venous anatomy. There is only one case in literature similar
to ours, which was between the SMV and aorta.[5] The distribution of cases is represented in [Fig. 13]. Treatment options include conservative (gravitational maneuvers) such as postprandial
prone knee chest/left lateral decubitus positioning, nutritional augmentation such
as multiple small feedings, parenteral hyperalimentation, and surgical bypass.[3] The second case is a case of testicular vein syndrome. The risk factors include
a congenitally enlarged testicular vein, thrombophlebitic testicular vein and varicocele
of testicular vein. Eight cases have been previously reported in the literature.[6]
[7]
[8] The patient details are given in [Table 1]. The average age of these patients was 37.5 years. Fifty-six percent of the patients
had left-sided testicular vein syndrome and 44% had right-sided testicular vein syndrome.
Compression was commonly encountered at the L3 vertebral level (50%), followed by
L2-L3 disc level (25%) and L5 vertebral level (25%). The treatment includes resection/transection
of the vein at the crossing point ± excision and ureteroureterostomy if the ureteral
segment at the crossover is atretic. Follow-up for these patients were done clinically
and with intravenous pyelography between 3 and 5 months postsurgery.[8] The third case is a case of giant fecal impaction causing deep vein thrombosis.
The risk factors include Hirschsprung's disease, Chagas disease, diabetic neuropathy,
neuropsychiatric diseases, inflammatory and neoplastic diseases, scleroderma, anorectal
malformations, chronic bedridden patients, and long-term drugs like antidepressants
and opioids.[9] Multiple such cases have been reported in the literature. The common acute presentations
of fecal impaction so far published in the literature include intestinal obstruction,[10] followed by acute urinary retention,[11] abdominal mass,[12] and toxic megacolon, as shown in [Fig. 14].[13] Deep vein thrombosis is an uncommon acute presentation of fecal impaction with the
first ever reported case being a pediatric case.[14] Ours is the second such presentation reported so far. Treatment options include
conservative management like laxatives, enema, and manual evacuation. Colonoscopy-guided
fragmentation of fecal matter is indicated in some cases. Surgical intervention is
mandated if complicated by bowel obstruction, toxic megacolon or if there is an underlying
cause of Hirschsprung's disease.[15] Another interesting management that is tried is colonoscopic instillation of Coca-Cola.[16]
Fig. 13 Distribution of various SMA-like syndromes caused by SMV. IVC, inferior vena cava;
SMA, superior mesenteric artery; SMV, superior mesenteric vein.
Fig. 14 Distribution of various acute presentations of fecal impaction/fecaloma. DVT, deep
vein thrombosis.
Table 1
Comparison of all reported cases of testicular vein syndrome (including current case
[Hamidi et al 2017[8]])
|
Cases
|
Case 1
|
Case 2
|
Case 3
|
Case 4
|
Case 5
|
Case 6
|
Case 7
|
Case 8
|
Case 9
|
|
Publication
|
Mellin et al 1974
|
Krestwoki et al 1977
|
Lassing et al 1978
|
Meyer et al 1992
|
Ugurel et al 2005
|
Gupta and colleagues 2011
|
Tiwari et al 2011
|
Hamidi 2017
|
Current case
|
|
Age
|
28 y
|
20 y
|
Young adult
|
42 y
|
54 y
|
37 y
|
55 y
|
27 y
|
47 y
|
|
Site
|
Right
|
Left
|
Left
|
Right
|
Right
|
Left
|
Right
|
Left
|
Left
|
|
Level
|
L4
|
L2/L3 disc
|
NA
|
L3
|
L3
|
L3
|
L3
|
L2/L3 disc
|
L4
|
Abbreviation: NA, not available.
Conclusion
In conclusion, the intricate nature of abdominopelvic vascular and hollow viscus compression
syndromes underscores the importance of vigilance and thorough evaluation in clinical
practice. These syndromes, although rare, present a diagnostic challenge due to their
diverse manifestations and potential for mimicking other common conditions. The cases
presented here demonstrate the significance of advanced imaging techniques in establishing
accurate diagnoses and guiding appropriate management strategies.
Additionally, these cases serve as valuable learning experiences, contributing to
the expanding knowledge base surrounding these rare syndromes. Ultimately, a thorough
understanding of abdominopelvic vascular and hollow viscus compression syndromes,
coupled with a proactive approach to diagnosis and management, is essential for mitigating
morbidity and mortality associated with these conditions. Continued research and clinical
observations will further enhance our understanding and refine treatment approaches,
ultimately improving the quality of care for affected patients.
