congenital portosystemic shunts

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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. Congenital Portosystemic Shunts. Relatively common - PowerPoint PPT Presentation

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  • Congenital Portosystemic ShuntsRelatively commonYorkshire terriers,Maltese,Schnauzers,Pug, Shih Tzu,Havanese,Irish Wolfhound,Poodle,Golden retriever,Laborador retrieverIs definitely genetic in some breeds

  • Congenital Portosystemic ShuntsRelatively commonBroad spectrum of signsPoor doerVomitingPolyuria-polydipsiaHematuriaDrooling in cats Hepatic encephalopathy

  • Classic Hepatic EncephalopathyPost-prandial:SeizuresConvulsionsHead pressingActing drunkNice dogs biteBad dogs kiss

  • Common Hepatic EncephalopathyOften not clearly associated witheating (~ 30-50% of cases)Signs often very subtleJust a Slow dogHas always been QuietNot too active Getting old

  • Congenital Portosystemic ShuntsRelatively commonBroad spectrum of signsDiagnosisRoutine lab tests insensitivemicrocytosis (MCV)hypoalbuminemialow BUNhypocholesterolemiaammonium biurate crystals

  • Congenital Portosystemic ShuntsRelatively commonBroad spectrum of signsDiagnosisRoutine lab tests insensitivePre and Post Prandial Bile AcidsBlood Ammonia

  • Congenital Portosystemic ShuntsRelatively commonBroad spectrum of signsDiagnosisRoutine lab tests insensitivePre and Post Prandial Bile AcidsBlood AmmoniaAbdominal Imaging plain radiographs

  • Case #161134

  • Case #161134

  • Case #190418 6 year old Pug with urate calculi

  • TAMU #176441: PSS + iatrogenic Cushings

  • Congenital Portosystemic ShuntsPlain radiographs microhepatia is seen in: 60-100% of dogs with PSS 50% of cats with PSS sometimes see renomegaly

  • Congenital Portosystemic ShuntsRelatively commonBroad spectrum of signsDiagnosisRoutine lab tests insensitivePre and Post Prandial Bile AcidsBlood AmmoniaAbdominal Imaging ultrasound

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

  • A major value of ultrasound is detecting intrahepatic shunts versus extrahepatic shunts

  • Congenital Portosystemic ShuntsRelatively commonBroad spectrum of signsDiagnosisRoutine lab tests insensitivePre and Post Prandial Bile AcidsBlood AmmoniaAbdominal Imaging scintigraphy, contrast, MRI

  • Congenital Portosystemic ShuntsRelatively commonBroad spectrum of signsDiagnosisRoutine lab tests insensitivePre and Post Prandial Bile AcidsBlood AmmoniaAbdominal ImagingHistopathology of liver

  • TAMU#119449Sig: 10 month F BichonCC: VomitingHPI: Vomits mucus and food 3 timesper week since it was obtained Loss of stamina 4 weeks agoPE: Normal

  • TAMU#119449Cholesterol =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)

  • TAMU#119449Resting bile acids =64.7 umol/L (0-13)

    Post-prandial = 12.4 umol/L (0-30)

  • TAMU#119449Resting 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)

  • TAMU#115907Sig: 13 yr F(s) SchnauzerCC: DiarrheaHPI: Diarrhea began yesterday Dog had 3 watery stools withoutmucus Vomited food and bile for 3 days Poor appetitePE: Depressed

  • TAMU#1159071/93: Liver biopsy: marked periportalswelling with mild multifocal necrosis11/98: Cognitive dysfunction: CT-scan shows cerebral cortical atrophyCSF: Albuminocytologic dissociation: Treat with Depranyl

  • TAMU#115907Cholesterol =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)

  • TAMU#115907Serum bile acids:19.6 86.4

    normal:< 13 < 30

  • TAMU#115907Serum bile acids:19.6 86.4173 236

    normal:< 13 < 30

  • OLD ANIMALS CAN HAVE CONGENITAL DISEASE

  • Retrospective StudyMiniature 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)

  • TAMU#1159071/93: Liver biopsy: marked periportalswelling with mild multifocal necrosis11/98: Cognitive dysfunction: CT-scan shows cerebral cortical atrophyCSF: Albuminocytologic dissociation: Treat with Depranyl

  • SERUM BILE ACID CONCENTRATIONS VARY SUBSTANTIALLY FROM DAY TO DAY

  • TAMU#115907Serum bile acids:19.6 86.4173 236

    normal:< 13 < 30

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

  • TAMU #1609147.8 52TAMU #1188404.825.4TAMU #1442117.67.7

  • TAMU #1609147.8 52TAMU #1188404.825.4TAMU #1442117.67.7

  • TAMU #1609147.8 52TAMU #1188404.825.4TAMU #1442117.67.7

  • TAMU#165244Sig: 7 yr F(s) SchnauzerCC: Pu-Pd, weight lossHPI: Signs began 3-4 months ago Has lost 15% body weightassociated with poor appetitePE: T = 101.7 F, HR = 90/min Thin dog

  • TAMU#165244date11/291/113/17 ALT6804071,050

    date (TAMU) 3/283/312,4241,612Normal ALT < 130 Units/L

  • TAMU #167033: PSS + HGE

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

  • TAMU #164612: PSS + DM + Addisons (12 yr)

  • Case #201912 9 yr old Yorkie in a bad mood

  • TAMU#117475Sig: 5 yr F Lhasa ApsoCC: Owner thinks dog has congenitalPSS and wants surgeryHPI: Anorexia and lethargy began 2weeks ago Sibling was diagnosed with PSSPE: Thin, corneal pigmentation

  • SURGICAL OR MEDICAL MANAGEMENT?

  • Mortality Post-PSS SurgeryVet 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% mortality15 (23%) died of causes associated with PSS (7.9 months later)JAVMA 236, 2010: 99 cases, 4-10% mortality

  • TAMU#117475August:Surgery for single congenital PSSSept:Ascites which is resolvedmedically

  • PSS SurgeryIf dog developes ascites post ligation Low salt diet Diuretics spironolactone furosemide

  • TAMU#117475August:Surgery for single congenital PSSSept:Ascites which is resolvedmedically1 Year:Pyometra develops. At surgerydiscover multiple acquiredportosystemic shunts

  • Conservative management of congenital PSSPrevent progression of hepatic damage antioxidants ursodeoxycholic acidControl hepatic encephalopathy(if the dog is encephalopathic, you need to be cautious about recommending conservative management as an acceptable choice)

  • 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 peopleMedical Management

  • 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) proteinMedical Management

  • Eliminate predisposing causes of HE Metabolic alkalosis (hypokalemia) Constipation Bleeding gastric lesions Azotemia Sedatives and analgesicsMedical Management

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