2. Congenital Portosystemic Shunts Relatively common Yorkshire terriers,Maltese, Schnauzers,Pug, Shih Tzu,Havanese, Irish Wolfhound,Poodle, Golden retriever, Laborador retriever Is definitely genetic in some breeds

3. Congenital Portosystemic Shunts Relatively common Broad spectrum of signs “Poor do’er” Vomiting Polyuria-polydipsia Hematuria “Drooling” in cats Hepatic encephalopathy

4. “Classic” Hepatic Encephalopathy Post-prandial:Seizures Convulsions Head pressing Acting drunk Nice dogs bite Bad dogs kiss

7. “Common” Hepatic Encephalopathy Often not clearly associated with eating (~ 30-50% of cases) Signs often very subtle Just a “Slow” dog Has always been “Quiet” Not too active “Getting old”

8. Congenital Portosystemic Shunts Relatively common Broad spectrum of signs Diagnosis Routine lab tests insensitive microcytosis (MCV) hypoalbuminemia low BUN hypocholesterolemia ammonium biurate crystals

9. Congenital Portosystemic Shunts Relatively common Broad spectrum of signs Diagnosis Routine lab tests insensitive Pre and Post Prandial Bile Acids Blood Ammonia

10. Congenital Portosystemic Shunts Relatively common Broad spectrum of signs Diagnosis Routine lab tests insensitive Pre and Post Prandial Bile Acids Blood Ammonia Abdominal Imaging plain radiographs

12. Case #161134

14. Case #190418 – 6 year old Pug with urate calculi

15. TAMU #176441: PSS + iatrogenic Cushings

16. Congenital Portosystemic Shunts Plain radiographs – microhepatia is seen in: 60-100% of dogs with PSS 50% of cats with PSS – sometimes see renomegaly

17. Congenital Portosystemic Shunts Relatively common Broad spectrum of signs Diagnosis Routine lab tests insensitive Pre and Post Prandial Bile Acids Blood Ammonia Abdominal Imaging ultrasound

18. The sensitivity of ultrasound for finding portosystemic shunts is very dependent upon the ultrasonographer

19. A major value of ultrasound is detecting intrahepatic shunts versus extrahepatic shunts

20. Congenital Portosystemic Shunts Relatively common Broad spectrum of signs Diagnosis Routine lab tests insensitive Pre and Post Prandial Bile Acids Blood Ammonia Abdominal Imaging scintigraphy, contrast, MRI

21. Congenital Portosystemic Shunts Relatively common Broad spectrum of signs Diagnosis Routine lab tests insensitive Pre and Post Prandial Bile Acids Blood Ammonia Abdominal Imaging Histopathology of liver

22. TAMU#119449 Sig: 10 month F Bichon CC: Vomiting HPI: Vomits mucus and food 3 times per week since it was obtained Loss of stamina 4 weeks ago PE: Normal

23. TAMU#119449 Cholesterol =147 mg/dl (120-247) BUN =5 mg/dl (8-20) Creatinine =0.5 mg/dl (< 2.0) Glucose =90 mg/dl (75-133) Total protein =6.1 gm/dl (5.5-7.5) Albumin =2.7 gm/dl (2.5-4.4) ALT =104 IU/L (< 130) SAP =117 IU/L (< 147)

24. TAMU#119449 Resting bile acids =64.7 umol/L (0-13) Post-prandial = 12.4 umol/L (0-30)

26. TAMU#119449 Resting bile acids =64.7 umol/L (0-13) Post-prandial = 12.4 umol/L (0-30) Blood ammonia =351 ug/dl (< 50) 183 ug/dl (< 50)

27. TAMU#115907 Sig: 13 yr F(s) Schnauzer CC: Diarrhea HPI: Diarrhea began yesterday Dog had 3 watery stools without mucus Vomited food and bile for 3 days Poor appetite PE: Depressed

28. TAMU#115907 1/93: Liver biopsy: marked periportal swelling with mild multifocal necrosis 11/98: Cognitive dysfunction: CT-scan shows cerebral cortical atrophy CSF: Albuminocytologic dissociation: Treat with Depranyl

29. TAMU#115907 Cholesterol =313 mg/dl (120-247) TP =6.5 gm/dl (5.7-7.8) Albumin =2.8 gm/dl (2.4-3.6) BUN =17 mg/dl (8-29) Na =144 mEq/L (138-148) K =4.3 mEq/L (3.5-5.0) ALT =105 U/L (< 130) SAP =129 U/L (< 147) Bilirubin =0.6 mg/dl (< 0.8)

30. TAMU#115907 Serum bile acids:19.6 86.4 normal:< 13 < 30

31. TAMU#115907 Serum bile acids:19.6 86.4 173 236 normal:< 13 < 30

33. OLD ANIMALS CAN HAVE CONGENITAL DISEASE

34. Retrospective Study Miniature schnauzers were 6.3 times more likely to be diagnosed with PSS at or after seven years of age compared to all other breeds (CI = 2.2-18.6; p = 0.001)

35. TAMU#115907 1/93: Liver biopsy: marked periportal swelling with mild multifocal necrosis 11/98: Cognitive dysfunction: CT-scan shows cerebral cortical atrophy CSF: Albuminocytologic dissociation: Treat with Depranyl

36. SERUM BILE ACID CONCENTRATIONS VARY SUBSTANTIALLY FROM DAY TO DAY

37. TAMU#115907 Serum bile acids:19.6 86.4 173 236 normal:< 13 < 30

38. HOW HIGH SHOULD SERUM BILE ACIDS BE IN DOGS WITH CONGENITAL PSS?

39. TAMU #160914 7.8 52 TAMU #118840 4.825.4 TAMU #144211 7.67.7

40. TAMU #160914 7.8 52 TAMU #118840 4.825.4 TAMU #144211 7.67.7

41. TAMU #160914 7.8 52 TAMU #118840 4.825.4 TAMU #144211 7.67.7

42. TAMU#165244 Sig: 7 yr F(s) Schnauzer CC: Pu-Pd, weight loss HPI: Signs began 3-4 months ago Has lost 15% body weight associated with poor appetite PE: T = 101.7 F, HR = 90/min Thin dog

43. TAMU#165244 date11/291/113/17 ALT6804071,050 date (TAMU) 3/283/31 2,4241,612 Normal ALT < 130 Units/L

45. TAMU #167033: PSS + HGE

46. You may fortuitously stumble upon PSS when working up some other, TOTALLY UNRELATED problem

47. TAMU #164612: PSS + DM + Addison’s (12 yr)

48. Case #201912 – 9 yr old Yorkie in a bad mood

49. TAMU#117475 Sig: 5 yr F Lhasa Apso CC: Owner thinks dog has congenital PSS and wants surgery HPI: Anorexia and lethargy began 2 weeks ago Sibling was diagnosed with PSS PE: Thin, corneal pigmentation

50. SURGICAL OR MEDICAL MANAGEMENT?

51. Mortality Post-PSS Surgery Vet Surg 33, 2004: 95 cases, 5.5% mortality (cellophane banding) JAVMA 226, 2005: 168 cases, 7% mortality (ameroid constrictors) JAVMA 232, 2008: 64 cases, 10% mortality 15 (23%) died of causes associated with PSS (7.9 months later) JAVMA 236, 2010: 99 cases, 4-10% mortality

52. TAMU#117475 August:Surgery for single congenital PSS Sept:Ascites which is resolved medically

53. PSS Surgery If dog developes ascites post ligation – Low salt diet – Diuretics spironolactone furosemide

54. TAMU#117475 August:Surgery for single congenital PSS Sept:Ascites which is resolved medically 1 Year:Pyometra develops. At surgery discover multiple acquired portosystemic shunts

57. Conservative management of congenital PSS Prevent progression of hepatic damage – antioxidants – ursodeoxycholic acid Control hepatic encephalopathy (if the dog is encephalopathic, you need to be cautious about recommending conservative management as an acceptable choice)

58. Control existing encephalopathy – Lactulose 0.25-0.5 ml/kg bid, then adjust Retention enema (10 ml + 30 ml water) – Lactitol (0.5-0.75 mg/kg bid) – Metronidazole or oral neomycin Rifaximin (10 mg/kg/day) used in people Medical Management

59. Control existing encephalopathy – “Low protein” diet only to treat encephlopathy or decrease blood ammonia concentrations give as much as the patient can tolerate prefer milk and vegetable (especially soy) protein Medical Management

60. Eliminate predisposing causes of HE – Metabolic alkalosis (hypokalemia) – Constipation – Bleeding gastric lesions – Azotemia – Sedatives and analgesics Medical Management