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 Broad spectrum of signs “Poor do’er” Vomiting Polyuria-polydipsia Hematuria “Drooling” in cats Hepatic encephalopathy

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

“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”

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

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

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

Case #161134

Case # – 6 year old Pug with urate calculi

TAMU #176441: PSS + iatrogenic Cushings

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

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

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 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

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

TAMU# 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

TAMU# Cholesterol =147 mg/dl ( ) 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 ( ) Albumin =2.7 gm/dl ( ) ALT =104 IU/L (< 130) SAP =117 IU/L (< 147)

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

TAMU# 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)

TAMU# 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

TAMU# /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

TAMU# Cholesterol =313 mg/dl ( ) TP =6.5 gm/dl ( ) Albumin =2.8 gm/dl ( ) BUN =17 mg/dl (8-29) Na =144 mEq/L ( ) K =4.3 mEq/L ( ) ALT =105 U/L (< 130) SAP =129 U/L (< 147) Bilirubin =0.6 mg/dl (< 0.8)

TAMU# Serum bile acids: normal:< 13 < 30

TAMU# Serum bile acids: normal:< 13 < 30

OLD ANIMALS CAN HAVE CONGENITAL DISEASE

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 = ; p = 0.001)

TAMU# /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

SERUM BILE ACID CONCENTRATIONS VARY SUBSTANTIALLY FROM DAY TO DAY

TAMU# Serum bile acids: normal:< 13 < 30

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

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TAMU# 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 = F, HR = 90/min Thin dog

TAMU# date11/291/113/17 ALT ,050 date (TAMU) 3/283/31 2,4241,612 Normal 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 + Addison’s (12 yr)

Case # – 9 yr old Yorkie in a bad mood

TAMU# 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

SURGICAL OR MEDICAL MANAGEMENT?

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

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

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

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

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)

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

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