Outcomes of cats undergoing surgical attenuation of congenital extrahepatic portosystemic shunts through cellophane banding: 9 cases (2000-2007).

OBJECTIVE: To evaluate the long-term prognosis of cats with a congenital extrahepatic portosystemic shunt (CEPSS) attenuated through gradual occlusion with cellophane banding (CB). DESIGN: Retrospective case series. ANIMALS: 9 cats with a CEPSS that was attenuated with CB. PROCEDURES: Medical records of cats surgically treated for CEPSS by means of CB from January 2000 through March 2007 were reviewed. Extracted data included preoperative clinical signs, medications, diagnostic results including serum bile acids concentrations, surgical technique, intraoperative and postoperative complications, and long-term follow-up information. RESULTS: 2 cats that developed refractory seizures were euthanized within 3 days after the CB procedure. Seven of the 9 cats survived to 15 days after surgery. Four cats did not have any clinical signs of CEPSS at long-term follow up. At that time, 5 cats had a postprandial SBA concentration within reference limits and 1 cat had persistent ptyalism. One cat had biurate ammonium stones removed > 2 years after surgery. One cat was euthanized 105 days after surgery because of uncontrolled seizures. The 3-year survival rate was 66%. CONCLUSIONS AND CLINICAL RELEVANCE: Uncontrolled seizure activity was the most common cause of death after CB. Long-term outcome for cats with CEPSS was fair to good after the procedure. Cats with a CEPSS surviving the immediate postoperative period had a fair to good long-term outcome. Cellophane banding without intraoperative attenuation appears to be an acceptable technique for gradual occlusion of a CEPSS in cats. Cats should be monitored closely for development of neurologic disorders in the postoperative period.

Recanalized umbilical vein as a conduit for mesenterico/porto-Rex bypass for patients with extrahepatic portal vein obstruction.

PURPOSE: Mesenterico-left portal vein (meso-Rex) bypass is as an effective modality for restoring intrahepatic portal perfusion in patients with extrahepatic portal vein obstruction. Achieving sufficient patency is difficult with end-to-side anastomosis of a bypass graft to a small or hypoplastic left portal vein in the Rex recessus. Here, we describe the use of a recanalized umbilical vein in the round ligament as a conduit for bypass construction in two patients. METHODS: Case 1 was an 11-year-old boy diagnosed with rupture of the esophageal varices and hypersplenism due to congenital extrahepatic portal hypertension. Because of persistent hypersplenism and thrombocytopenia, he underwent meso-Rex bypassing with a left iliac vein graft interposed between the umbilical vein and the superior mesenteric vein. Case 2 was a neonate with a large hepatic tumor (mesenchymal hamartoma) that developed abdominal compartment syndrome at birth. The tumor was removed by right hepatectomy with excision of the portal vein bifurcation at 3 days of age. Porto-Rex bypassing was accomplished by end-to-end anastomosis between the portal vein trunk and the umbilical vein. RESULTS: Sufficient hepatopetal portal flow through the umbilical vein was achieved in both patients and maintained for over 16 and 13 months, respectively. Although hypersplenism remained in Case 1, intrahepatic portal vein branches gradually widened and the cavernoma in the hepatic hilum disappeared within 2 months. Neither patient had symptoms or signs of portal hypertension at the most recent follow-up. CONCLUSION: Using the umbilical vein as a vein conduit may facilitate construction of a meso/porto-Rex bypass and restore intrahepatic portal vein perfusion in patients with extrahepatic portal vein obstruction.

[Perspectives of Sugiura operation by portal hypertension in children].

23 children, aged 3-17 years, have undergone the Sugiura operation since 1989. 18 children had extrahepatic portal hypertension, 3 - the inborn liver fibrosis, 2 - liver cirrhosis. The procedure has been complemented by endoscopic sclerotherapy since 2001. Of 13 children, who had just Sugiura operation, bleeding relapsed in 4 cases. Of 10 patients, who had the combined procedure, the recurrent bleeding was registered in 2 cases. The Sugiura operation in combination with endoscopic varices sclerotherapy was suggested as a method of choice in children with contraindications to portosystemic bypass procedure.

Endoscopic treatment of gastroesophageal varices in young infants with cyanoacrylate glue: a pilot study.

BACKGROUND: In children, endoscopic sclerotherapy and variceal ligation (EVL) are the most used techniques for the treatment of gastroesophageal variceal bleeding (VB). However, these techniques achieve poor results in cases of gastric variceal bleeding, and EVL is not applicable in young infants. OBJECTIVE: Our purpose was to evaluate the feasibility, efficacy, and safety of cyanoacrylate glue injection for the treatment of gastroesophageal varices in young infants. DESIGN: Single-center prospective study. PATIENTS: From 2001 to 2005, 8 young infants (

Use of transcolonic portal scintigraphy to evaluate efficacy of cellophane banding of congenital extrahepatic portosystemic shunts in 16 dogs.

OBJECTIVE: To evaluate the efficacy of cellophane banding of single congenital extrahepatic portosystemic shunts in dogs using transcolonic portal scintigraphy. To investigate the portal circulation of those dogs with elevated postoperative shunt fractions to determine the cause of the persistent shunting. Further, to evaluate whether presenting signs, clinical pathology findings and liver histopathology are predictive of outcome. DESIGN: Prospective study of 16 dogs presenting with single congenital extrahepatic portosystemic shunts. PROCEDURE: Dogs with single extrahepatic portosystemic shunts attenuated by cellophane banding underwent portal scintigraphy and bile acids tolerance testing pre- and post-operatively. Dogs identified with elevated shunt fractions at 10 weeks post-operatively underwent mesenteric portovenography. Qualitative hepatic histopathology from all dogs was reviewed by a veterinary pathologist and assigned a semi-quantitative score to identify any abnormalities that may predict surgical outcome. RESULTS: At 10 weeks post cellophane banding, 10 of 16 cases (63%) had normal shunt fractions, whilst six dogs (37%) had increased shunt fractions and seven dogs (44%) had increased serum bile acids. Of these dogs, mesenteric portovenography revealed incomplete closure of the shunt in three dogs (18.6%) and multiple acquired shunts in three dogs (18.6%). Liver histopathology findings were similar for all dogs, regardless of outcome. CONCLUSIONS: Cellophane banding is an efficacious method for complete gradual occlusion of single extrahepatic shunts when the shunt vessel is attenuated to < or = 3 mm. Transcolonic portal scintigraphy is a reliable method for assessment of shunt attenuation and, unlike serum bile acids, is not influenced by other causes of liver dysfunction.

Pulmonary hypertension in patients with congenital portosystemic venous shunt: a previously unrecognized association.

BACKGROUND: Pulmonary arterial hypertension has been reported to be observed in association with acquired portal hypertension. However, the contribution of congenital anomalies occurring in the portal system to the development of pulmonary arterial hypertension remains to be elucidated. METHODS: Nine patients with congenital portosystemic venous shunt were studied from January 1990 through September 2005. RESULTS: Patent ductus venosus was detected in 5 patients, including 3 patients with an absence of the portal vein. The presence of either a gastrorenal or splenorenal shunt was evident in another 4 patients. Six patients had a history of hypergalactosemia with normal enzyme activities, as seen during neonatal screening. Six (66.7%) of the 9 patients were identified to have clinically significant pulmonary arterial hypertension (mean pulmonary artery pressure: 34-79 mm Hg; pulmonary vascular resistances: 5.12-38.07 U). The median age at the onset of pulmonary arterial hypertension was 12 years and 3 months. Histologic studies of lung specimens, which were available in 4 of the 9 patients with congenital portosystemic venous shunt, showed small arterial microthrombotic lesions in 3 patients. This characteristic finding was recognized even in the congenital portosystemic venous shunt patients without PAH. CONCLUSIONS: This study demonstrated thromboembolic pulmonary arterial hypertension to be a crucial complication in congenital portosystemic venous shunt, and this pathologic state may be latently present in patients with pulmonary arterial hypertension of unknown etiology.

Evaluation of cellophane banding with and without intraoperative attenuation for treatment of congenital extrahepatic portosystemic shunts in dogs.

OBJECTIVE: To evaluate the effect of intraoperative attenuation of congenital extrahepatic portosystemic shunts (CEPSSs) during cellophane banding procedures in dogs. STUDY DESIGN: Retrospective case series and prospective study. ANIMALS: 18 cases evaluated retrospectively and 14 dogs evaluated prospectively. PROCEDURES: Gradual occlusion of CEPSSs was performed via cellophane banding. Shunts were occluded to a diameter < 3.0 mm during surgery in dogs prospectively enrolled in the partial attenuation group, whereas the shunt was not attenuated during surgery in dogs prospectively enrolled in the no-attenuation group or in dogs that had previously undergone surgery and were retrospectively evaluated. Postprandial serum bile acids (PPSBA) concentrations were measured before surgery and at various time points after surgery. RESULTS: Mean +/- SD PPSBA concentrations were 26.8 +/- 24.5 micromol/L at < 2.25 months after surgery (n = 16 dogs), 22.1 +/- 14.0 micromol/L from 2.25 to 6 months after surgery (12 dogs), and 34.9 +/- 32.5 micromol/L at > 6 months after surgery (22 dogs). In the prospectively enrolled dogs, mean PPSBA concentrations increased over time in dogs in the partial attenuation group, but not in dogs in the no-attenuation group. CONCLUSIONS AND CLINICAL RELEVANCE: Cellophane banding may be used to occlude larger CEPSSs and may decrease the need for intraoperative monitoring of portal vein blood pressure. The technique may facilitate minimally invasive treatment of CEPSSs in dogs. Intraoperative attenuation of CEPSSs to a diameter < 3.0 mm is not necessary and may result in a less favorable outcome.

Outcomes of intrahepatic portosystemic shunts occluded with ameroid constrictors in nine dogs and one cat.

OBJECTIVE: To report outcome after ameroid constrictor (AC) use for single intrahepatic portosystemic shunts (IPSS). STUDY DESIGN: Retrospective study. ANIMALS: Nine dogs and 1 cat. METHODS: Medical records (1999-2003) of dogs and cats with surgically confirmed IPSS were reviewed. Recorded data was: breed, sex, weight, age at surgery, clinical signs, serum biochemical and hematologic data, shunt anatomy, AC size, hospitalization, complications, and owner perception of their animal's response to surgery. Owners were contacted twice postoperatively (between 20 and 75 months) and asked to complete a simple questionnaire. RESULTS: With this technique, complication rate was low and postligation neurologic dysfunction was not observed. In the cat and 7 dogs, clinical signs attributed to IPPS resolved and animals were fed a non-prescription diet without medication. One dog died suddenly 18 months after surgery from causes unrelated to hepatic disease. One dog was euthanatized 23 months after surgery because of repeated, increasingly refractory episodes of hepatic encephalopathy. CONCLUSION: AC provides a safe and effective method for occlusion of some IPPS. CLINICAL RELEVANCE: AC should be considered for occlusion of some IPPS in dogs and cats.

Congenital hepatic fibrosis: CT findings in 18 adults.

PURPOSE: To evaluate the computed tomographic (CT) findings in adult patients with pathologically proved congenital hepatic fibrosis. MATERIALS AND METHODS: This was a retrospective review of congenital hepatic fibrosis cases identified at two institutions over the course of 8 years. Eight men and 10 women with an age range of 22-72 years (mean age, 39 years) were included. Contrast material-enhanced and unenhanced CT scans were obtained through the liver in all patients. Two radiologists evaluated size of and morphologic findings (atrophy or hypertrophy localized according to hepatic segments) in the liver; increased diameter or number of hepatic arteries at the hilum; presence of hepatic nodules, varices, spontaneous splenorenal shunts, and splenomegaly; and association with other hepatic ductal plate malformations and renal abnormalities. RESULTS: Sixteen patients had morphologic abnormalities in the liver, 15 had splenomegaly (three underwent splenectomy for portal hypertension), and 14 had varices or spontaneous splenorenal shunts. An enlarged hepatic artery and a tangle of abnormally enlarged arterial vessels were identified in five and four patients, respectively, and four of these nine patients had large benign regenerative nodules. Ten patients had renal abnormalities and nine had an associated ductal plate malformation. CONCLUSION: This retrospective study shows that certain findings (ie, liver morphologic and associated ductal plate abnormalities, varices, splenomegaly, and renal abnormalities) are frequently observed in combination in patients with congenital hepatic fibrosis.

Outcomes of cellophane banding for congenital portosystemic shunts in 106 dogs and 5 cats.

OBJECTIVE: To report outcomes after cellophane banding of single congenital portosystemic shunts in dogs and cats. STUDY DESIGN: Retrospective study of sequential cases. ANIMALS: One hundred and six dogs and five cats. METHODS: Medical records were reviewed for breed, sex, age at surgery, shunt anatomy, results of pre- and postoperative biochemical analysis, development of postligation neurologic dysfunction, portal hypertension or other serious complications, and the owners' perception of their animal's response to surgery. RESULTS: Ninety-five dogs and all 5 cats had extrahepatic shunts. Eleven dogs had intrahepatic shunts. Six dogs (5.5%) died as a result of surgery from portal hypertension (2 dogs), postligation neurologic dysfunction (2), splenic hemorrhage (1) and suspected narcotic overdose (1). Serious complications were more common in dogs with intrahepatic shunts than those with extrahepatic shunts (P=.002). Postligation neurologic dysfunction necessitated treatment in 10 dogs and 1 cat; 8 dogs and the cat survived. Clinical signs attributed to portosystemic shunting resolved or were substantially attenuated in all survivors. Postoperative serum bile acid concentrations or results of ammonia tolerance testing were available for 88 animals; 74 (84%) were normal and 14 (16%) were abnormal. Multiple acquired shunts were documented in two animals. CONCLUSIONS: Cellophane banding is a safe and effective alternative to other methods of attenuation. CLINICAL RELEVANCE: Slow occlusion of portosystemic shunts using a variety of methods is being evaluated world wide. Cellophane banding is a relatively simple procedure with comparable safety and efficacy to previously reported techniques.

Effect of breed on anatomy of portosystemic shunts resulting from congenital diseases in dogs and cats: a review of 242 cases.

OBJECTIVE: To evaluate the effect of species and breed on the anatomy of portosystemic vascular anomalies in dogs and cats. DESIGN: Retrospective study of 233 dogs and nine cats presenting to the University Veterinary Centre, Sydney. METHODS: Case records were evaluated for breed, sex, age, anatomical and histological diagnosis. Cases were included when a portosystemic vascular anomaly resulted from a congenital or developmental abnormality of the liver or portal venous system. RESULTS: Disease conditions included single congenital portosystemic shunt with patent portal vasculature (214 dogs, nine cats), portal vein aplasia (nine dogs), multiple acquired shunts resulting from portal vein hypoplasia (seven dogs), biliary atresia (one dog) and microvascular dysplasia (one dog). One Maltese had a single, congenital shunt and multiple acquired shunts resulting from hepatic cirrhosis. Breeds that were significantly over-represented included the Maltese, Silky Terrier, Australian Cattle Dog, Bichon Frise, Shih Tzu, Miniature Schnauzer, Border Collie, Jack Russell Terrier, Irish Wolfhound and Himalayan cat. Bichon Frise with shunts were significantly more likely to be female than male (12:2, P < 0.001). Two hundred and fourteen dogs (91.4%), and all cats, had shunts that were amenable to attenuation. Inoperable shunts occurred in 19 dogs (8.2%). Fifty six of 61 (92%) operable shunts in large breed dogs were intrahepatic, versus 10/153 (7%) in small breeds (P < 0.0001). Breeds that were not predisposed to portosystemic shunts were significantly more likely to have unusual or inoperable shunts than dogs from predisposed breeds (29% versus 7.6%, P < 0.0001). No significant relationship between breed and shunt type could be determined in cats. CONCLUSION: Breed has a significant influence on shunt anatomy in dogs. Animals presenting with signs of portosystemic shunting may suffer from a wide range of operable or inoperable conditions. Veterinarians should be aware that unusual or inoperable shunts are much more likely to occur in breeds that are not predisposed to congenital portosystemic shunts.

[Clinical aspects and surgery of fibrocholangiocytosis in children]. / Klinika i khirurgicheskoe lechenie fibrokholangiokistoza u detei.

Fifty one children with a diagnosis of fibrocholangiocystosis (FCC) were examined. A degree of circulatory blockade in the portal vein system in FCC was established to be one of the factors conditioning a severity of the course of the disease and its prognosis. With increase in duration of the disease and age of a child, the cirrhotic process in the liver and its complications are aggravating. The esophagogastric bleeding with the incidence as well depending on duration of the disease is the most severe and life-threatening complication. Our experience with operative treatment of FCC is indicative of the necessity to perform early interventions, and of a high effectiveness of creating the vascular anastomosis with preservation of the spleen.

[Physiopathology of portal hypertension (author's transl)]. / Physiopathologie de l'hypertension portale.

The physiopathology of portal hypertension is discussed. The pathogenesis of this affection may be of a congenital, or acquired infectious or iatrogenic nature, and the percentage frequency of each of these causes is presented. The different morphological forms of portal hypertension in children are outlined, emphasis being placed on the fact that the blood obstruction is usually pre-hepatic in location. A study of the modifications in blood circulation due to the obstruction demonstrates the importance of the collateral network, both interior and exterior to the liver. Complications of portal vein thrombi include splenomegaly, ascites, and alterations in hepatic parenchymatous tissue, producing alteration in liver oxygen perfusion. The effect on the systemic circulation and brain function are discussed.