• 제목/요약/키워드: Congenital extrahepatic 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
    • 한국임상수의학회지
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    • 제34권4호
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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.

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

  • 김주형;한성영;전혜영;김태훈;강지훈;한태성;나기정;양만표;김근형;강상철;김재훈;장동우
    • 한국임상수의학회지
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    • 제24권2호
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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.

산전 초음파로 발견되고 출생 후 4회의 코일 색전술과 심 교정술로 치료된 심실 중격 결손을 동반한 선천성 간내 문맥전신성 단락 1례 (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)

  • 나지윤;김은선;김상덕;김이경;김한석;최중환;천정은;정진욱
    • Neonatal Medicine
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    • 제15권2호
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    • pp.176-182
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    • 2008
  • 드문 선천성 문맥전신성 단락에 대한 표준화된 치료는 없으나 저자들은 출생 직후부터 심부전 증상을 보인 환아에서 심실 중격 결손증을 동반한 선천성 문맥전신성단락을 4회의 코일 색전술 및 심 교정술로 치료한 증례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

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
    • 한국임상수의학회지
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    • 제41권2호
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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.

거대 간 종괴와 심방 중격 결손을 동반한 Abernethy 기형 2형 1예 (A Case of Congenital Extra Hepatic Portocaval Shunt (Abernethy Malformation Type 2) with a very Large Liver Mass and an Atrial Septal Defect)

  • 이해정;이지현;허준;강이석;이흥재;서연림;유소영;최연호
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • 제11권1호
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    • pp.56-59
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    • 2008
  • 저자들은 간의 거대 종괴와 심방 중격 결손으로 수술을 받았던 환아에서 복부 CT와 간 조직 검사를 통해 확인된 Abernethy 기형 2형 1예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

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The Characteristics and Outcomes of Abernethy Syndrome in Korean Children: A Single Center Study

  • Kim, Eun Sil;Lee, Ki Wuk;Choe, Yon Ho
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • 제22권1호
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    • pp.80-85
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    • 2019
  • Purpose: Abernethy malformation is a rare condition, which was first described in 1793 as a congenital extrahepatic porto-systemic shunt (CEPS) directing splanchnic blood flow into the inferior vena cava. Eighty cases have been published so far that reported CEPS, while in Korea, very few cases have been reported. Through this study, we present 6 cases of patients diagnosed with CEPS at Samsung Medical Center and compare these with other such cases published in France and China. Methods: We reviewed clinical, laboratory, and imaging data of 6 children with CEPS in our pediatric clinic between 2004 and 2017. Results: A total of 6 children with CEPS was included in this study, namely, one with type 1a, two with type 1b, and three with type 2 CEPS. The most common presenting symptom was gastrointestinal bleeding (50.0%). Therapeutic interventions included shunting vessel ligation (16.7%) in type 2 CEPS and liver transplantation (16.7%) in type 2 CEPS patient with suddenly developed hepatic encephalopathy. Conclusion: There is no consensus guideline for the optimal management of patients with CEPS. Large-sample studies regarding CEPS are needed to evaluate the characteristics of patients with CEPS and determine the treatment guideline for CEPS.

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
    • 한국임상수의학회지
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    • 제37권2호
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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.