poster situs inversus

1
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 Situs Solitus. It is a rare condition with a frequency of 0.01%. If it is found to be associated with dextrocardia and chance of congenital heart diseases is 3-5 %. Levocardia is an extremely rare association. But when Situs Inversus is associated with Levocardia, chance of congenital heart diseases rises to 95 % 1 . Since Situs Inversus has numerous associated anomalies, so most of the patients come to clinical and imaging attention because of congenital heart disease, immune deficiency, or bowel obstruction related to malrotation 2 . It may also result in atypical presentation of typical diseases leading to misdiagnosis. Establishing a good understanding of multiple anatomic variations, anomalies, associations and differences in clinical manifestations of disease process is of paramount importance. CASE REPORT A 14 years old young male presented with a history of lower backache, mild dysuria and supra-pubic pain for the last 01 year. It was not associated with fever, increased frequency, urgency or hesitancy. This patient had a history of congenital cardiac anomalies which were known to him. He however denied any history of previous hospitalization. There were no other known co-morbid. Overall growth pattern, milestones and secondary sexual characteristics were well developed. His previous ultrasound abdomen revealed Situs Inversus which was known to the patient as well. The imaging was performed on 16 slicer CT-Scanner. The scan 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 dilatation and ballooning of left renal pelvis. Striated nephrogram and duplex type of collecting system was noted on right side with dilatation of both moieties. Renal pelvis of both moieties showed abrupt tapering most likely due to pelvi-utreteric junction narrowing. No contrast was seen in right ureter and it was seen to be dilated up to the uretero-vesical junction. Vascular anatomy (Fig IV) raised the possibility of having double IVC (seen to be uniting at infra-hepatic level). Rest of the scan was unremarkable a) Situs Inversus along with ectopic right kidney, two IVC are seen, one anterior to aorta on this image and other on left side DISCUSSION Anomalies related to Situs are quite unusual or even very rare at times. Situs Inversus is considered as the most common form which is seen only in 0.01% of the whole population1. Establishing a good understanding of these variations certainly becomes helpful to avoid complications in surgical or interventional procedures2. Situs Inversus either have an associated Dextrocardia or Levocardia. Situs Inversus is relatively common with the former one and is seen to have transposition of cardiac apex, spleen, stomach, and aorta on the right and the liver and IVC on the left. Cardiac diseases occur in 3- 5% of cases3. Having an associated Levocardia is exceedingly rare and almost all cases have cardiac anomalies4. Congenital heart disease occurs in less than 1% of individuals with situs solitus5. Spectrum of manifestations varies greatly among those who present in early life than those who present late. Unusual clinical manifestations and confusing pictures due to abnormal locations of spleen, gall bladder and appendix may present as diagnostic dilemma7. For appropriate recognition and characterization of visceral anatomy, Sonography8, Computed Tomography (CT), and Magnetic Resonance (MR) imaging have greatly enhanced the diagnostic abilities9. Situs Inversus has been found to be associated with various vascular anomalies and variations. Interruption of IVC with Azygous or Hemiazygous continuation is also seen in association with polysplenia 10, 11, 12. The infra hepatic IVC may be right sided, left sided or duplicated. However, IVC interruption with Azygous continuation in asplenia syndrome is very rare13. Ipsilateral location of the aorta and IVC has been reported to be a consistent finding in asplenia14. Applegate et al 15 noted that the aorta and IVC were ipsilateral in only six patients c) Ectopic right kidney fused with the left kidney at its lower pole, large right paravertebral vein also seen b) Ectopic right kidney was also malrotated, IVC are seen to join at the level of renal hilum on this CT image REFERENCES 1 Dahnert W. CVS Disorders. In: Dahnert W, ed. Radiology Review Manual. 5th edition. Lippincott, Williams and Wilkins. 2003; pp. 582 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 d) Sagittal reformatted image showing fused kidneys with hydronephrosis SUMMARY OF IMAGING FINDINGS 1. Situs Inversus with Dextrocardia 2. Crossed Fused Ectopia of Right Kidney with duplex collecting system 3. Bilateral Pelvi-Utreteric Junction narrowing 4. Double IVC (seen to be uniting at renal hilum level)

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Page 1: Poster Situs Inversus

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

INTRODUCTIONSitus Inversus literally means mirror image arrangement of the

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

is found to be associated with dextrocardia and chance of

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

rare association. But when Situs Inversus is associated with

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

Since Situs Inversus has numerous associated anomalies, so most

of the patients come to clinical and imaging attention because of

congenital heart disease, immune deficiency, or bowel obstruction

related to malrotation2.

It may also result in atypical presentation of typical diseases

leading to misdiagnosis.

Establishing a good understanding of multiple anatomic

variations, anomalies, associations and differences in clinical

manifestations of disease process is of paramount importance.

CASE REPORTA 14 years old young male presented with a history of lower

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

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

hesitancy. This patient had a history of congenital cardiac

anomalies which were known to him. He however denied any

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

morbid. Overall growth pattern, milestones and secondary sexual

characteristics were well developed.

His previous ultrasound abdomen revealed Situs Inversus which

was known to the patient as well.

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

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

dilatation and ballooning of left renal pelvis. Striated nephrogram

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

dilatation of both moieties. Renal pelvis of both moieties showed

abrupt tapering most likely due to pelvi-utreteric junction

narrowing. No contrast was seen in right ureter and it was seen to

be dilated up to the uretero-vesical junction. Vascular anatomy

(Fig IV) raised the possibility of having double IVC (seen to be

uniting at infra-hepatic level). Rest of the scan was unremarkable

a) Situs Inversus along with ectopic right kidney, two IVC are seen, one anterior to aorta on this image and other on left side

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

times. Situs Inversus is considered as the most common form

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

Establishing a good understanding of these variations certainly

becomes helpful to avoid complications in surgical or

interventional procedures2.

Situs Inversus either have an associated Dextrocardia or

Levocardia. Situs Inversus is relatively common with the former

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

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

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

Levocardia is exceedingly rare and almost all cases have cardiac

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

individuals with situs solitus5.

Spectrum of manifestations varies greatly among those who

present in early life than those who present late.

Unusual clinical manifestations and confusing pictures due to

abnormal locations of spleen, gall bladder and appendix may

present as diagnostic dilemma7.

For appropriate recognition and characterization of visceral

anatomy, Sonography8, Computed Tomography (CT), and

Magnetic Resonance (MR) imaging have greatly enhanced the

diagnostic abilities9.

Situs Inversus has been found to be associated with various

vascular anomalies and variations. Interruption of IVC with

Azygous or Hemiazygous continuation is also seen in association

with polysplenia 10, 11, 12. The infra hepatic IVC may be right

sided, left sided or duplicated. However, IVC interruption with

Azygous continuation in asplenia syndrome is very rare13.

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

consistent finding in asplenia14. Applegate et al 15 noted that the

aorta and IVC were ipsilateral in only six patients

c) Ectopic right kidney fused with the left kidney at its lower pole, large right paravertebral vein also seen

b) Ectopic right kidney was also malrotated, IVC are seen to join at the level of renal hilum on this CT image

REFERENCES

1 Dahnert W. CVS Disorders. In: Dahnert W, ed. Radiology Review

Manual. 5th edition. Lippincott, Williams and Wilkins. 2003; pp. 582

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:

Saunders, 1984; 157-187.

d) Sagittal reformatted image showing fused kidneys with hydronephrosis

SUMMARY OF IMAGING FINDINGS1. Situs Inversus with Dextrocardia

2. Crossed Fused Ectopia of Right Kidney with duplex collecting

system

3. Bilateral Pelvi-Utreteric Junction narrowing

4. Double IVC (seen to be uniting at renal hilum level)