SITUS INVERSUS ASSOCIATED WITH

COMPLEX VENOUS ANATOMY; A RARE ASSOCIATION

Syed Muzzammil Wasti, Sohail Amin, Sibtain Raza, S. Shafqat-ul-Islam, Syed Mahmood

Karachi X Rays & U/S / CT Scan Centre

INTRODUCTION
Situs Inversus literally means mirror image arrangement of the

DISCUSSION

Situs Solitus. It is a rare condition with a frequency of 0.01%. If it

Anomalies related to Situs are quite unusual or even very rare at

is found to be associated with dextrocardia and chance of

times. Situs Inversus is considered as the most common form

congenital heart diseases is 3-5 %. Levocardia is an extremely

which is seen only in 0.01% of the whole population1.

rare association. But when Situs Inversus is associated with

Establishing a good understanding of these variations certainly

Levocardia, chance of congenital heart diseases rises to 95 %1.

becomes

Since Situs Inversus has numerous associated anomalies, so most

interventional procedures2.

of the patients come to clinical and imaging attention because of

Situs

congenital heart disease, immune deficiency, or bowel obstruction

Levocardia. Situs Inversus is relatively common with the former

related to malrotation2.

one and is seen to have transposition of cardiac apex, spleen,

It may also result in atypical presentation of typical diseases

stomach, and aorta on the right and the liver and IVC on the left.

leading to misdiagnosis.

Cardiac diseases occur in 3-5% of cases3. Having an associated

helpful

Inversus

to

avoid

either

have

complications
an

associated

in

surgical

or

Dextrocardia

or

anatomic

Levocardia is exceedingly rare and almost all cases have cardiac

variations, anomalies, associations and differences in clinical

anomalies4. Congenital heart disease occurs in less than 1% of

Establishing

good

understanding

of

multiple

manifestations of disease process is of paramount importance.

CASE REPORT

a) Situs Inversus along with ectopic

right kidney, two IVC are seen, one
anterior to aorta on this image and
other on left side

b) Ectopic right kidney was also

malrotated, IVC are seen to join at the
level of renal hilum on this CT image

individuals with situs solitus5.

Spectrum of manifestations varies greatly among those who
present in early life than those who present late.

A 14 years old young male presented with a history of lower

backache, mild dysuria and supra-pubic pain for the last 01 year.

Unusual clinical manifestations and confusing pictures due to

It was not associated with fever, increased frequency, urgency or

abnormal locations of spleen, gall bladder and appendix may

hesitancy. This patient had a history of congenital cardiac

present as diagnostic dilemma7.

anomalies which were known to him. He however denied any

For appropriate recognition and characterization of visceral

history of previous hospitalization. There were no other known co-

anatomy,

morbid. Overall growth pattern, milestones and secondary sexual

Magnetic Resonance (MR) imaging have greatly enhanced the

characteristics were well developed.

diagnostic abilities9.

His previous ultrasound abdomen revealed Situs Inversus which

Situs Inversus has been found to be associated with various

was known to the patient as well.

vascular anomalies and variations. Interruption of IVC with

The imaging was performed on 16 slicer CT-Scanner. The scan

Azygous or Hemiazygous continuation is also seen in association

revealed Dextro-cardia, Situs Inversus along with ectopic right

kidney which was fused with the left kidney at its lower pole (Fig I
& II). In addition to that ectopic right kidney was also malrotated,
extending to the midline and laid anterior to the Aorta and IVC
(Fig III). Slight malrotation of left kidney was also noted with
associated left pelvi-utreteric junction narrowing leading to

c) Ectopic right kidney fused with the

left kidney at its lower pole, large right
paravertebral vein also seen

d) Sagittal reformatted image showing

fused kidneys with hydronephrosis

Sonography8,

Computed

Tomography

(CT),

and

with polysplenia 10, 11, 12. The infra hepatic IVC may be right
sided, left sided or duplicated. However, IVC interruption with

REFERENCES

Azygous continuation in asplenia syndrome is very rare13.

Ipsilateral location of the aorta and IVC has been reported to be a

1 Dahnert W. CVS Disorders. In: Dahnert W, ed. Radiology Review
consistent finding in asplenia14. Applegate et al 15 noted that the
Manual. 5th edition. Lippincott, Williams and Wilkins. 2003; pp.582
aorta and IVC were ipsilateral in only six patients

dilatation and ballooning of left renal pelvis. Striated nephrogram

and duplex type of collecting system was noted on right side with

SUMMARY OF IMAGING FINDINGS

dilatation of both moieties. Renal pelvis of both moieties showed

1. Situs Inversus with Dextrocardia
abrupt
tapering most likely due to pelvi-utreteric junction
2. Crossed Fused Ectopia of Right Kidney with duplex collecting
narrowing. No contrast was seen in right ureter and it was seen to
system
be dilated up to the uretero-vesical junction. Vascular anatomy
3. Bilateral Pelvi-Utreteric Junction narrowing
(Fig IV) raised the possibility of having double IVC (seen to be
4. Double IVC (seen to be uniting at renal hilum level)
uniting at infra-hepatic level). Rest of the scan was unremarkable

2 Moller JH, Nakib A, Anderson RC, Edwards JE. Congenital heart

disease associated with polysplenia, a developmental complex of
bilateral "left-sidedness". Circulation 1967; 36:789-799.
3 Applegate KE, Goske MJ, Pierce G, Murphy D. Situs revisited:
imaging of the heterotaxy syndrome. Radiographics 1999;19:837-852
4 Tegtmeyer CJ, Hust FS, Keats TE. Arteriographic manifestations of
abdominal situs inversus. AJR Am J Roentgenol 1975; 125:427-430
5 Tonkin IL. The definition of cardiac malpositions with
echocardiography and

computed tomography. In: Friedman WF,

Higgins CB, eds. Pediatric cardiac imaging. Philadelphia, Pa:

Saunders, 1984; 157-187
6 Tonkin IL. The definition of cardiac malpositions with
echocardiography and computed tomography. In: Friedman WF,
Higgins CB, eds. Pediatric cardiac imaging. Philadelphia, Pa: