• Title/Summary/Keyword: Congenital portosystemic shunt

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Concurrent Patent Ductus Arteriosus and Congenital Extrahepatic Portosystemic Shunt with Suspected Portal Vein Aplasia in a Dog

  • Chae, Soo-young;Cho, Yu-gyeong;Lee, Young-won;Choi, Ho-jung
    • Journal of Veterinary Clinics
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    • v.34 no.4
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    • pp.283-286
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    • 2017
  • A 4-month-old, female Maltese dog was referred with continuous heart murmur. Patent ductus arteriosus was diagnosed via radiography and echocardiography. The patient was untreated because of client's refusal. After 13 months, the dog was referred again with seizure and salivation. Laboratory examination revealed increased liver enzymes, hyperammonemia and decreased total cholesterol and total protein. Microhepatica was identified on abdominal radiography. CT angiography showed a shunt vessel that originated from the portal trunk to the prehepatic caudal vena cava and patent ductus arteriosus connecting proximal descending aorta with the main pulmonary artery. No portal vasculature toward liver is observed after shunt vessel. The patient was diagnosed as concurrent patent ductus arteriosus and congenital extrahepatic portosystemic shunt with suspected portal vein aplasia. In human, cardiac malformations are frequently observed in patients with congenital extrahepatic portosystemic shunt with portal vein aplasia. This report described concurrent patent ductus arteriosus and congenital extrahepatic portosystemic shunt with suspected portal vein aplasia in a dog.

Endovascular Closure Resolves Trimethylaminuria Caused by Congenital Portosystemic Shunts

  • Ponce-Dorrego, Mar?a Dolores;Garzon-Moll, Gonzalo
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.22 no.6
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    • pp.588-593
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    • 2019
  • This study aimed to report three new cases of an association between two rare conditions, congenital portosystemic shunts (CPSS) and trimethylaminuria (TMAU), and the efficacy of endovascular closure of the CPSS for resolving TMAU. Between November 2014 and April 2017, 15 patients with CPSS were enrolled in this prospective study to assess the efficacy of percutaneous endovascular shunt closure. Three patients presented with clinical symptoms of TMAU that were confirmed by urine analysis of trimethylamine (TMA) and TMA n-oxide. One year after endovascular closure of the congenital portosystemic shunt, the same parameters were evaluated were obtained and the values were compared to the pretreatment values. The results indicated the disappearance of clinical symptoms of TMAU and normalization of the urine test parameters in two patients and no changes in one patient, who developed new portosystemic communications.

A Case of Congenital Intrahepatic Portosystemic Shunt Associated with VSD Detected by Antenatal Sonography and Treated with Four Coil Embolizations and Open Heart Surgery after Birth (산전 초음파로 발견되고 출생 후 4회의 코일 색전술과 심 교정술로 치료된 심실 중격 결손을 동반한 선천성 간내 문맥전신성 단락 1례)

  • Na, Ji-Youn;Kim, Eun-Sun;Kim, Sang-Duk;Kim, Ee-Kyung;Kim, Han-Suk;Choi, Jung-Hwan;Chung, Jeong-Eun;Joung, Jin-Uuk
    • Neonatal Medicine
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    • v.15 no.2
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    • pp.176-182
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    • 2008
  • A congenital portosystemic shunt is a very rare portosystemic vascular anomaly which leads to jaundice, hypoglycemia, hyperammonemia, liver cirrhosis, hepatic coma, and pulmonary hypertension. Anatomically, portosystemic shunts are divided into intra- and extrahepatic shunts. Congenital intrahepatic portosystemic shunts are rare anomalies, and the early diagnosis is important to prevent hepatic encephalopathy and hypoglycemia. We report a case of an infant with symptoms of heart failure due to a congenital intrahepatic portosystemic shunt and a ventricular septal defect (VSD), which were treated successfully with four coil embolizations and open heart surgery for the VSD.

Endovascular Treatment of Congenital Portosystemic Shunt: A Single-Center Prospective Study

  • Ponce-Dorrego, Maria-Dolores;Hernandez-Cabrero, Teresa;Garzon-Moll, Gonzalo
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.25 no.2
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    • pp.147-162
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    • 2022
  • Purpose: To design a prospective study on endovascular closure of congenital portosystemic shunts. The primary endpoint was to assess the safety of endovascular closure. The secondary endpoint was to evaluate the clinical, analytical and imaging outcomes of treatment. Methods: Fifteen patients (age range: 2 days to 21 years; 10 male) were referred to our center due to congenital portosystemic shunts. The following data were collected prior to treatment: age, sex, medical history, clinical and analytical data, urine trimethylaminuria, abdominal-US, and body-CT. The following data were collected at the time of intervention: anatomical and hemodynamic characteristics of the shunts, device used, and closure success. The following data were collected at various post-intervention time points: during hospital stay (to confirm shunt closure and detect complications) and at one year after (for clinical, analytical, and imaging purposes). Results: The treatment was successful in 12 participants, migration of the device was observed in two, while acute splanchnic thrombosis was observed in one. Off-label devices were used in attempting to close the side-to-side shunts, and success was achieved using Amplatzer™ Ductus-Occluder and Amplatzer™ Muscular-Vascular-Septal-Defect-Occluder. The main changes were: increased prothrombin activity (p=0.043); decreased AST, ALT, GGT, and bilirubin (p=0.007, p=0.056, p=0.036, p=0.013); thrombocytopenia resolution (p=0.131); expansion of portal veins (p=0.005); normalization of Doppler portal flow (100%); regression of liver nodules (p=0.001); ammonia normalization (p=0.003); and disappearance of trimethylaminuria (p=0.285). Conclusion: Endovascular closure is effective. Our results support the indication of endovascular closure for side-to-side shunts and for cases of congenital absence of portal vein.

Diagnosis of Multiple Extrahepatic Portosystemic Shunt in Two Dogs (개의 다발성 간외성 간문맥전신단락증의 진단 2예)

  • Kim, Ju-Hyung;Han, Sung-Young;Chun, Hye-Young;Kim, Tae-Hun;Kang, Ji-Houn;Han, Tae-Sung;Na, Ki-Jeong;Yang, Mhan-Pyo;Kim, Gon-Hyung;Kang, Sang-Chul;Kim, Jae-Hoon;Chang, Dong-Woo
    • Journal of Veterinary Clinics
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    • v.24 no.2
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    • pp.269-275
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    • 2007
  • Two dogs referred to Veterinary Medical Center, Chungbuk National University diagnosed as multiple extrahepatic portosystemic shunt were reported. The first dog was a 20-month-old, 8 kg, male Cocker spaniel with history of peritoneal effusion, diarrhea, anorexia and stunted growth. The second dog was a 3-year-old, 13.4 kg, male Jindo with a history of severe depression. Hematologic examination of first dog revealed mild microcytosis and nonregenerative anemia. All of 2 cases, serum chemical values showed increase of serum ammonia, ALP, r-GTP and glucose. In survey radiography, microhepatia was apparent. In the color Doppler ultrasonographic examination, the first dog revealed a dilated tortuous vein communicating with caudal vena cava was observed near the left kidney and the second dog revealed numerous shunting vessels ventral to L5 and L6. Transcolonic portal scintigraphy of the first dog confirmed the presence of portosystemic shunt. In intraoperative jejunoportography, the first dog showed single congenital extrahepatic portosystemic shunt and multiple acquired extrahepatic portosystemic shunts. The second dog showed multiple acquired extrahepatic portosystemic shunts. In these dogs, the presence of congenital and acquried portosystemic shunts and histopathologic findings were considered to represent a combination of multiple extrahepatic portosystemic shunts and noncirrhotic portal hypertension or portal vein hypoplasia.

Successful Management of Post-Attenuation Neurologic Signs and Portal Hypertension in a Dog with Congenital Portoazygos Shunt

  • Jin-Young Kim;Kun-Ho Song;Joong-Hyun Song
    • Journal of Veterinary Clinics
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    • v.41 no.2
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    • pp.95-100
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    • 2024
  • A 6-year-old neutered male Poodle with a body weight of 2.7 kg was diagnosed with a congenital extrahepatic portoazygos shunt presented with progressive hepatic encephalopathy. Five days after surgical attenuation, the patient showed post-attenuation neurological signs (PANS) such as generalized tonic-clonic seizure, dull mentation, and tremor. PANS were successfully managed with antiepileptic drugs, but third-space fluid accumulation (ascites and peripheral edema) and phlebectasia were newly identified, suggesting marked portal hypertension (PHT). Telmisartan, spironolactone, carvedilol, and prednisolone were sequentially administered. Three months after surgery, both abnormal clinicopathological values such as anemia and hypoalbuminemia, and clinical signs completely resolved. Herein, we report successful management strategies for PANS and PHT in a dog following surgical attenuation of a congenital portoazygos shunt.

A Clinical Study of Congenital Intrahepatic Portosystemic Shunt Diagnosed in Neonatal Period (신생아기에 진단된 선천성 간내 문맥 전신성 단락의 임상적 고찰과 치료)

  • Kim, Ji-Young;Kim, Kyung-A;Lee, Yeon-Kyung;Ko, Sun-Young;Shin, Son-Moon;Han, Byung-Hee
    • Neonatal Medicine
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    • v.18 no.1
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    • pp.117-123
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    • 2011
  • Purpose: Congenital intrahepatic portosystemic shunts are rare disease and clinically asymptomatic shunts may be detected by chance on ultrasonogram before and after birth. We studied clinical course, treatment and prognosis of congenital intrahepatic portosystemic shunt at prenatal or neonatal period. Methods: Medical records of 8 patients which were diagnosed in intrahepatic portosystemic shunt in Cheil General Hospital from 2006 through 2010 were reviewed retrospectively. Results: Eight patients with congenital intrahepatic portosystemic shunts were identified. Six patients were diagnosed at prenatal radiological screening, including three cases of intrauterine growth restriction and two cases of preterm baby. One case with increased serum ammonia underwent coil embolization. In four cases including one case that presented elevated direct bilirubin, shunts were closed spontaneously within 11th month after birth. Two patients were diagnosed on abdominal sonogram after birth because of elevated direct hyperbilirubinemia, all of whom presented intrauterine growth restriction. Closure of shunts was confirmed during 4th month to 6th month. Conclusion: Congenital intrahepatic portosystemic shunts are clinically asymptomatic mostly and spontaneous closure is expected within 2 years age. But occasionally they have severe complication, so clinical and radiological observation is needed. Specially in cases of intrauterine growth retardation without evident cause, the possible diagnosis of congenital intrahepatic portosystemic shunts should be considered and prenatal and postnatal examination should be performed. When prenatal diagnosis is made, fetal wellbeing should be monitored periodically until spontaneous closure of shunts.

Congenital portal vein aplasia with portocaval shunting in two dogs

  • Hwang, Taesung;Moon, Jonghyun;Lee, Hee Chun
    • Korean Journal of Veterinary Research
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    • v.59 no.3
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    • pp.171-173
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    • 2019
  • Two dogs presented with vomiting and head pressing. In both dogs, a large vessel was revealed in computed tomography (CT) angiography, which was found to leave the portal vein (PV) cranial to the splenomesenteric confluence and enter the pre-hepatic caudal vena cava cranial to the right renal vein. The flow of portal blood to the liver was not identified. Based on CT angiography, the dogs were suspected to have congenital PV aplasia with portocaval shunting. Diagnostic imaging of potential malformations for PV continuation should be conducted before attempting shunt closure.

Acquired Portosystemic Shunts Secondary to Hepatic Microvascular Dysplasia in a Young Dog

  • Hwang, Taesung;An, Soyon;Kim, Ahreum;Han, Changhee;Huh, Chan;Lee, Hee Chun
    • Journal of Veterinary Clinics
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    • v.37 no.2
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    • pp.88-90
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    • 2020
  • A one year old spayed female Bichon Frise dog presented with gait abnormalities and seizure. Serum biochemical results showed elevated levels of alkaline phosphatase, alanine aminotransferase, and ammonia. Serum bile acid level was also increased to be over 30 μmol/L on preprandial. Urinalysis identified the presence of ammonium urate crystal. Abdominal ultrasonography and CT revealed aberrant, tortuous, and multiple small vessels connected to the caudal vena cava between left kidney and caudal vena cava. Macroscopic specific findings associated with extrahepatic congenital portosystemic shunts (PSS) or other liver diseases were not identified. Liver biopsy was performed. Histopathologic evaluation revealed hepatic lobular hypoplasia with portal arterial duplication and vascular shunts. Based on these finding, this case was diagnosed as multiple acquired PSS secondary to hepatic microvascular dysplasia (HMD) and hepatic encephalopathy. A liver biopsy is recommended to differentiate HMD from other liver diseases and to confirm HMD when a young dog has multiple acquired PSS.