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Nutcracker Syndrome Accompanying Pelvic Congestion Syndrome

Nutcracker Syndrome Accompanying Pelvic Congestion Syndrome. Color Doppler Sonography and Multislice CT Findings : A Case Report. Introduction.

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Nutcracker Syndrome Accompanying Pelvic Congestion Syndrome

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  1. NutcrackerSyndromeAccompanyingPelvicCongestionSyndrome ColorDopplerSonographyandMultislice CT Findings: A CaseReport

  2. Introduction • TheNutcrackerSyndrome is a compression of the LRV bythe AA andthe SMA, manifestedbypain, hematuria, varicoceleorlowerorpelvicurinarysymptoms. NCS accompanyingpelviccongestionsyndrome is a rarepathologythat can be easilymissedbyroutinediagnosticmethods.

  3. CasePresentation • A 27-year-oldwomanwasbroughttotheemergencydepartmentbecause of leftflankpain, chronicpelvicpainandhematuria. She had genitalcomplaints. She had no fever, vomiting, ordiarrhea. Shealsodeniedanytraumatothe abdomen orback. Her medicalhistorywasotherwiseunremarkable. She had leftflanktenderness. At that time, urinalysisshowednumerousredbloodcells. Hematuriapersisted on multipleurinalyses.

  4. CasePresentation • CDUS and CT imagesshowedthereduction of theanglebetweenthe AA and SMA. Pelvicultrasonography (US), CDUS and CT imagesshowedmultiplepelvicvaricesincludetortuouspelvicveins, dilatedarcuateveins in myometriumbeing in communicationwithpelvicvaricoseveins (Fig. 1.).

  5. Fig.1. CDUS (A) andsagittal CT images (B) showthereduction of theanglebetween AA and SMA (arrows). Pelvic CDUS (C) showspelvicvaricoseveins(arrows).

  6. CasePresentation • Itwasconfirmedby CT whichdemonstratedcompression of theleftrenalveinbetweenthe AA and SMA andalsodilatation of leftovarianveinattributableto NCS in axialandcoronalobliqueimages. Axial CT, reformattedobliquecoronalimagesandvolume-renderedMultidedector CT (MDCT) angiographyimagesshowedretrogradefilling of leftovarianvein in thearterialphaseconsistentwithreflux in pelviccongestionsyndrome (Fig. 2.).

  7. Fig.2. CT images (A) demonstratenarrowing (beaksign) of theleftrenalveinbetween AA and SMA (arrow). Axial CT (B, arrow), andvolume-renderedangiographyimages (C) showretrogradefilling of leftovarianvein in thearterialphaseconsistentwithreflux in pelviccongestionsyndrome (arrows).

  8. CasePresentation • Thediagnosis of NCS accompanyingpelviccongestionsyndromewasestablishedbythesefindings. Withtheconsent of thepatientand his parents, conservativetreatmentwasdecidedupon.

  9. Discussion • NCS is a well-known but underrecognizedcause of hematuria. The LRV normallycrossesbetweenthe AA and SMA, where it may be compressed, resulting in a variabledegree of LRV obstruction. Thiscompression of LRV between AA and SMA is termed NCS1. Itmayalsoleadtoleftrenalveinvarices, leftgonadalveinvaricesandsopelviccongestion syndrome2. PelviccongestionsyndromewasfirstdescribedbyRichet in 1857 and Aran in 18583. Pelvicvarices can be seen in 10% of women in the general populationandupto 60% of patientswithpelvicvaricesmaydeveloppelviccongestionsyndrome. Pelviccongestionsyndromeoccursmostlybecause of ovarianveinreflux, but can alsooccurbecause of theobstruction of ovarianveinoutflowresulting in reversed flow4,5.

  10. Discussion • Imaging, such as US, CT ormagneticresonanceimaging (MRI), is requiredtodiagnose NCS. However, themostaccuratemethodfordiagnosingthe NCS is leftrenalvenographywithmeasurement of thepressuregradientbetweenthe LRV andtheinferior vena cava1,6. CDUS, CT, MRI andangiographyalldemonstrateleftrenalveinstenosiswithproximaldistentionandthe presence of collateralpathways in NCS7. Narrowing of the LRV calledthe “beaksign” and CT findings can be usefulforthediagnosis of NCS, andtheyreflectthepathophysiology of LRV compressionbetweenthe SMA andthe aorta6. On theotherhand, US is theinitialdiagnosticmodalityforthediagnosis of pelviccongestionsyndrome. Combination of US and CDUS allowaccuratediagnosis of pelvicvarices.

  11. Discussion • Severaldiagnosticcriteriahavebeenreportedforpelviccongestionsyndrome CDUS. Theseincludetortuouspelvicveinswith a diametergreaterthan 5–6 mm, slow (≈ 3 cm/s) orreversedflow, dilatedarcuateveins in themyometriumcommunicatingwithpelvicvaricoseveins, andpolycysticchanges in theovaries. Reversedflow in theovarianvein can be suggested in the presence of ovarianveinfilling on arterialphase CT images. Moreovervelocityencodedphasecontrast MRI can be usedtodemonstrateovarianveinvelocityandflowdirection. Selectiveovarianvenography has beenaccepted as thegoldstandardtechniqueforthediagnosis of pelviccongestion syndrome4,8.

  12. Discussion • Inthedifferentialdiagnosis of NCS andaccompanyingpelviccongestionsyndrome, pathologiesthatcausepainandhematuriasuch as lithiasis, congenitalvascularmalformations, tumors, infections, parenchymalorurinarytractabnormalities, painfulpelvicsyndromesand organ orneighboringstructurealterationsmust be excluded2,9. • Supportivetreatment is adequateifthesymptomsaremild in NCS. Conservativetreatment has beensuggestedformildhematuria. Surgicalorradiologicalinterventionsareindicatedfor severe pain, significanthematuriaandrenalfunctionalimpairment. Endovascularintervention is nowconsideredthefirstline of therapyforthemanagement of this condition1,10,11.

  13. Conclusion • NCS accompanyingpelviccongestionsyndrome is a rarepathologythat can be easilymissedbyroutinediagnosticmethods. Itshould be considered in thedifferentialdiagnosis of patientswithhematuria, intermittentflankpain, nonspecificpelvicpain, dyspareunia, andpersistentgenitalarousalwithout an apparentcause. An inclusion of thisentity in thedifferentialdiagnosis of patientswithunknowncause of hematuriaandflankpain can achieveefficientmanagement of thiscondition.

  14. REFERENCES • Waseem M, Upadhyay R, Prosper G. Thenutcrackersyndrome: an underrecognizedcause of hematuria. Eur J Pediatr 2012;171:1269-71. • Dellavedova T, Racca L, Ponzano RM, Minuzzi S, Olmedo JJ, Minuzzi G.Nutcrackersyndrome: a casereport. RevMexUrol 2010;70:51-54. • Belenky A, Bartal G, Atar E, Cohen M, Bachar GN. OvarianVarices in HealthyFemaleKidneyDonors: Incidence, Morbidity, andClinicalOutcome. AJR2002;179:625–627. • Karcaaltıncaba M, Karcaaltıncaba D, Dogra VS. PelvicCongestionsyndrome. UltrasoundClin 2008;3:415–425. • Cicchiello LA, Hamper UM, Scoutt LM. UltrasoundEvaluation of GynecologicCauses of PelvicPain. UltrasoundClin 2010;5:209–231. • Kim KW, Cho JY, Kim SH, Yoon J-H, Kim DS, Chung JW, et al. Diagnosticvalue of computedtomographicfindings of nutcrackersyndrome: Correlationwithrenalvenographyandrenocavalpressuregradients. EuropeanJournal of Radiology 2011; 80: 648– 654.

  15. REFERENCES 7. Muraoka N, Sakai T, Kimura H, Uematsu H, Tanase K, Yokoyama O, et al.RareCauses of HematuriaAssociatedwithVariousVascularDiseasesInvolvingtheUpperUrinaryTract. RadioGraphics 2008;28:855–867. 8. KaraosmanogluD, Karcaaltincaba M, Karcaaltincaba D, Akata D, Ozmen M.MDCT of theOvarianVein: Normal AnatomyandPathology. AJR2009; 192:295–299. 9. MohamadiA, Ghasemi-Rad M, Mladkova N, Masudi S. VaricoceleandNutcrackerSyndrome. J UltrasoundMed 2010; 29:1153–1160. 10. ChenYM, Wang IK, Ng K-K, Huang C-C. Nutcrackersyndrome: an overlookedcause of hematuria. ChangGungMed J 2002;25:700-5. 11. ChangCT, Hung CC, Ng KK, Yen TH. Nutcrackersyndromeandleftunilateralhaematuria. NephrolDialTransplant 2005;20:460–461.

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