Nutcracker syndrome and its management.pptx

Nutcracker Syndrome

1. Nutcracker syndrome DR SWANIT DESHPANDE 7/4/2023

2. CASE PRESENTATION 30 Y/F, NO COMORBIDITIES MARRIED NO CHILDREN RIGHT FLANK PAIN ON AND OFF SINCE 5 MONTHS DULL ACHING, NON RADIATING, RELIEVED PARTIALLY WITH ANALGESICS , NO LUTS NO H/O HEMATURIA LMP- 5/3/23

3. On examination General examination – Thinly built Per abdominal exam- Soft No T/G/R HB- 14 WBC- 5640 PLT- 3.47 LAKH CREAT- 0.7 URINE ROUTINE- NORMAL

4. RADIOLOGICAL INVESTIGATIONS USG (A+P) (8/1/23) Both kidneys normal in size and shape Left renal vein compressed (diameter 10 mm0 PSV of left Renal Vein 17 cm/s Aortomesenteric angle- 30 degrees

5. Doppler (Feb 2023) LEFT RENAL HILAR PSV 73 cm/s PSV of Aorta at SMA 152 cm/s SMA PSV 185 cm/s Compression Ratio 3.2 (>2.25 s/o Nutcracker syndrome) Precompression Vein AP diameter Post compression diameter 0.96 cm 0.3 cm

6. CECT A+P (6/1/23) Significant reduction in aorto- superior mesenteric angle (22 degrees). Aortomesenteric distance is 7.5 mm. Smooth indentation on the left renal vein seen with mild extrinsic compression (left renal vein diameter at the hilum is 8 mm). Prominence of left ovarian vein with dialted veins in the adnexa. B/L good nephrogram with prompt excretion of contrast

7. Review of literature Earliest pathological description – Grant (1937) First clinical report – El Sadr and Mina (1950) Term ‘nutcracker’ – de Schepper (1972)

8. What is Nutcracker Syndrome (NS)? Nutcracker Phenomenon [NCP] (aka left renal vein entrapment ) – impeded outflow from left renal vein (LRV) into the IVC due to extrinsic LRV compression NCP demonstrated as lateral (hilar) dilatation and medial (meso-aortic) narrowing NS – clinical symptoms + demonstrable features of NCP Other compression syndromes Compression syndrome Compressed structure Compressed by May Thurner Left iliac vein Right iliac artery Wilikies / SMA Syndrome Duodenum third part SMA & Aorta

9. Types of NS Anterior NS – compression of LRV between SMA and Aorta ( analogous to SMA syndrome/ Wilkie Syndrome ) AMA < 35 degrees  Aorto- mesentric narrowing of LRV  increasing intraluminal pressure  Renal varices

10. Posterior NS – LRV compressed between aorta and vertebral body

11. Pathological processes leading to LRV compression Pancreatic neoplasm Retroperitoneal tumors Overarching testicular artery Lordosis Reduced retroperitoneal and mesenteric fat pregnancy

12. DEMOGRAPHIC CHARACTERISTICS Exact prevalence not known Slightly higher in females Not hereditary Mostly 2 nd or 3 rd decade of life 2 nd peak in middle aged women

13. CLINICAL FEATURES Positionality of symptoms – hallmark (Doppler Ultrasound + physical findings) Hematuria (MC) – rupture of thin-walled varices (elevated venous pressure) Abdominal and left flank pain (clot colic, gonadal vein pain syndrome radiating to posteromedial thigh and buttock) Varicocoele in 9.5% of affected men Orthostatic proteinuria Chronic fatigue syndrome in NS associated with high LRV:IVC pressure gradient Pelvic congestion – chronic pelvic pain a/w dyspareunia, dysuria, dysmenorrhoea, pelvic varicocoele

14. DIAGNOSIS Doppler Ultrasound (DUS) CT venography (CTV)/ MR venography (MRV) Contrast venography (CV) + Intravascular ultrasound (IVUS)

15. DUS First line study Sensitivity 69-90% Specificity 90-100% Aorto- mesenteric : hilar PSV > 4.2 to 5.0  one of the diagnostic criteria

16. Advantage- real time assessment of flow and peak velocities Disadvantages- Variability with positional changes Technical difficulty from small sampling area Inter-observer variability Transducer compression artifacts

17. CT /MR VENOGRAPHY Bird beak sign AMA angle <35 degrees LRV hilar diameter: aorto-mesenteric diameter >4.9 – highest diagnostic accuracy (Specificity – 100%)

18. Disadvantage Not Dynamic modality Does not measure Flow velocity & Direction Radiation exposure

19. CV + IVUS Gold standard Allows visualization and selective catheterization of collaterals like LGV Renocaval pressure gradient >3mmHg (normal = 0-1 mmHg)  Diagnostic of NS IVUS allows real time visualization of dynamic LRV compression and guides the selection of size and location of stent placement (Specificity – 90%)

20. A systematic review on management of nutcracker syndrome

21. Current management approach for left renal vein entrapment syndrome: the so-called ‘Nutcracker’ syndrome

22. Current management approach for left renal vein entrapment syndrome: the so-called ‘Nutcracker’ syndrome

23. A systematic review on management of nutcracker syndrome

24. Indications of Surgery Gross Hematuria (Especially Recurrent) Severe Symptoms : Flank pain Anemia Persistent Orthostatic proteinuria Ineffective Conservative Mx > 24 mths in pt < 18 years or after 6 months in adults

25. Conservative Management Pt < 18 yrs of age growth  ↑ intraabdo . Fibrous tissue at SMA origin  Release entrapped LRV Weight gain  ↑ Retroperitoneal Adipose tissue Medical Treatment ACE inhibitors  proteinuria Aspirin  Improve renal perfusion

26. Open Surgery LRV Transposition  Gold standard treatment (M/C) Auto Transplantation Pelvic Venous disease prior to LRV hypertension (LGV embolization & ligation)

27. LRV Transposition LRV transposed to caudal location on IVC , AMA angle and distance are wider and larger respectively

28. LRV Transposition with patch venoplasty Patch venoplasty  Permanent injury to LRV post long standing entrapment (fibrosis/ fixed stenosis/ thrombosis)

29. LRV Transposition with Saphenous vein cuff Extension Cuff Inadequate vein length

30. Left Gonadal Vein Transposition LGV transposition  NCS with LGV incompetence with symptomatic Pelvic venous disease ( varicocoele or PCS)

31. Left Gonadal Vein preferably preserved  subsequent coil embolization or secondary surgical bypass

32. Renal Autotransplant Advantage – Corrects posterior Nephroptosis Disadvantage – Extensive dissection Long period of renal ischemia Additonal anastomoses of renal artery and ureter

33. Laparoscopic Surgery Laparoscopic Extravascular stent placement Stent – expanded PTFE graft Laparoscopic LRV – IVC transposition

34. Endovascular Stenting

35. Endovascular stenting – preferably used in treatment of NCS with PCS Complication depends on Type/ size of stent, Surgeon experience Stent migration – MC complication Other complications: In stent re-stenosis Thrombosis Kinking fracture

36. Hybrid Repair Open + Endovascular stenting LRV Transposition with Patch venoplasty (GSV) + Inra op stenting with slight over sized self-expanding stent

37. Advantages Transpositon - removes LRV from max compression Patch venoplasty – allows use of large caliber stent  patency Self- expanding stent - prevents sustained/ recurrent extrinsic compression Transfixation – prevents stent migration

38. 34 yr old female c/o occasional left flank pain One episode of hematuria No other complaints Family complete – 4 children h/o TL done

39. Blood Investigation Hb – 11.8 TLC – 5400 Platelet – 153000 Creat – 0.7

40. Ultrasound Right kidney Left kidney 8.4 x 3.6 cm 8.6 x 3.9 cm normal 2.9 x 2.7 cm simple cyst at MP

41. Urine rm N AD

42. CT Urography

43. CT Urography RK – 8.4 x 4.7 x 4.9 cm LK – 9.3 x 5 x 4.8 cm, simple cyst 3 x 2.5 cm at mid pole B/L good nephrogram with prompt excretion of contrast LRV compressed by SMA, AMA 30 degree, compression ratio 1.3 Bilateral pelvic and ovarian veins dilated