• 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.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제22권6호
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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.

• 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.

• 한국임상수의학회:학술대회논문집
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• 한국임상수의학회 2008년도 춘계학술대회
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• pp.78-78
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• 2008

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제25권2호
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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.

• 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.

• 대한영상의학회지
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• 제83권6호
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• pp.1426-1431
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• 2022

간내 문맥정맥단락은 간문맥-대정맥 또는 간문맥-간정맥이 연결되어 있는 드문 혈관 질환이다. 간내 문맥정맥단락은 주로 무증상을 보이며, 다른 질환으로 영상 검사를 하였을 때 우연히 발견될 수 있다. 하지만 단락양이 많거나 증상이 있는 경우 단락 색전술을 고려하여야 한다. 저자들은 선천성 간내 문맥정맥단락 환자에서 갑자기 간성뇌증이 발생하여 이를 경간 코일 색전술로 치료한 증례를 보고하고자 한다.

• 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.

• Neonatal Medicine
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• 제18권1호
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• pp.117-123
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• 2011

목적: 선천성 문맥 전신성 단락은 매우 희귀한 질환으로, 대부분은 증상이 없어 우연히 시행된 영상 검사를 통해서 발견되고, 심각한 합병증을 일으킬 수 있어 진단과 치료에 신중을 기해야 하는 질환이나, 재태 상태 또는 신생아기에 단락의 진단을 위한 표준화된 방법이 제시되고 있지 않은 실정이다. 이에 선천성 간내 문맥 전신성 단락이 신생아기에 복부 초음파로 확인된 8예의 임상 양상과 치료 및 예후에 대해 알아보았다. 방법: 2006년 1월부터 2010년 12월까지 관동대학교 의과대학 제일병원에서 출생한 환아 36,572명 중 산전에 단락이 진단된 신생아 6예와, 출생 후 간내 문맥 전신성 단락이 진단된 신생아 2예를 대상으로 의무 기록 정보를 후향적으로 분석하였다. 결과: 선천성 간내 문맥 전신성 단락은 해당 기간의 출생아중 8명에서 발생하였으며, 이 중 6예는 산전검사에서 진단되었으며, 2예는 출생 후 복부 초음파 검사를 시행하여 진단되었다. 산전의 진단된 6예 중 3예는 자궁 내 성장 지연을 보였으며, 2예는 미숙아였다. 1예에서 암모니아의 상승을 보여, 코일 색전술을 시행하였으며, 4예에서 생후 11개월 내에 단락의 자연 소실을 확인하였다. 출생 후 단락이 확인된 경우는 2예로 모두 자궁 내성장 지연을 보였으며, 단락은 생후 4개월과 6개월에 자연 소실된 것을 확인하였다. 고찰: 선천성 간내 문맥 전신성 단락은 생후 2년 내에 자연 소실되는 것으로 알려져 있으나, 소실 시까지 경과 관찰을 요한다. 단락은 4,500명의 출생아 중 1명에서 발생하였으며, 자궁 내 성장지연을 보인 5예를 포함한 8예에서 경험하였다. 뚜렷한 원인이 동반되지 않은 자궁 내 성장 지연을 보이는 경우, 반드시 선천성 문맥 전신성 단락을 의심하여야 하며, 산전 초음파를 시행하여 검증하여야 한다. 산전 초음파 결과 단락이 의심될 경우, 태아의 상태를 지속적으로 관찰해야 하며, 생후에도 단락의 자연 소실까지 주기적인 감시가 필요하다.

• 대한수의학회지
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• 제59권3호
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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.