• Journal of Yeungnam Medical Science
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• 제35권1호
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• pp.89-93
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• 2018

Idiopathic non-cirrhotic portal hypertension (INCPH) is a disease with an uncertain etiology consisting of non-cirrhotic portal hypertension and portal pressure increase in the absence of liver cirrhosis. In INCPH, patients exhibit normal liver functions and structures. The factors associated with INCPH include the following: Umbilical/portal pyremia, bacterial diseases, prothrombic states, chronic exposure to arsenic, vinyl chloride monomers, genetic disorders, and autoimmune diseases. Approximately 70% of patients present a history of major variceal bleeding, and treatment relies on the prevention of complications related to portal hypertension. Autoimmune disorders associated with INCPH are mainly systemic sclerosis, systemic lupus erythematosus and rheumatoid arthritis. To the best of our knowledge, a case of ankylosing spondylitis (AS) associated with INCPH has not been reported thus far. Therfore, we report our experience of a patient with AS accompanied by INCPH, who showed perisplenic varices with patent spleno-portal axis and hepatic veins along with no evidence of cirrhosis on liver biopsy, and provide a brief literature review.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제22권4호
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• pp.400-406
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• 2019

We report a 12-month-old female infant who had a history of neonatal sepsis with liver micro-abscesses that resolved with intravenous antibiotics during neonatal period. During her neonatal admission period, no umbilical vein catheter was inserted. Also, she did not undergo any abdominal surgeries or had a postnatal history of necrotizing enterocolitis. However, the child developed upper gastrointestinal bleeding in form of hematemesis and melena secondary to esophageal varices at the age of 12 months with an extra-hepatic portal vein obstruction with cavernous transformation and portal hypertension subsequently. The child underwent a successful endoscopic injection sclerotherapy. She is now 20-month-old and has portal hypertension but otherwise asymptomatic. We are proposing the possibility of a delayed-onset portal hypertension as a complication of liver abscess and neonatal sepsis.

• 한국간담췌외과학회지
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• 제27권3호
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• pp.307-312
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• 2023

Hepatic arterioportal fistulae are abnormal communications between the hepatic artery and portal vein. They are reported to be congenital or acquired secondary to trauma, iatrogenic procedures, hepatic cirrhosis, and hepatocellular carcinoma, but less likely to occur spontaneously. Extrahepatic portal venous obstruction (EHPVO) can lead to pre-hepatic portal hypertension. A spontaneous superimposed hepatic arterioportal fistula can lead to pre-sinusoidal portal hypertension, further exacerbating its physiology. This report describes a young woman with long-standing EHPVO presenting with repeated upper gastrointestinal variceal bleeding and symptomatic hypersplenism. Computed tomography scan demonstrated a cavernous transformation of the portal vein and a macroscopic hepatic arterioportal fistula between the left hepatic artery and portal vein collateral in the central liver. The hepatic arterioportal fistula was associated with a flow-related left hepatic artery aneurysm and a portal venous collateral aneurysm proximal and distal to the fistula, respectively. Endovascular coiling was performed for the hepatic arterioportal fistula, followed by proximal splenorenal shunt procedure. This case illustrates an uncommon association of a spontaneous hepatic arterioportal fistula with EHPVO and the utility of a combined endovascular and surgical approach for managing multifactorial non-cirrhotic portal hypertension in such patients.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제21권4호
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• pp.361-364
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• 2018

Accessory hepatic lobe is noted as and considered a rare disease in children. It can manifest with various symptoms and complications depending on the location, volume, type and position of the disease as presented on a child. The patient presented as a 14-month-old girl who was seen with a notable hepatosplenomegaly and portal hypertension. A diagnosis was made after taking an extensive medical history, observation and radiological examinations. The formal diagnosis was a prehepatic portal hypertension associated with accessory hepatic lobe.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제10권2호
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• pp.221-225
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• 2007

저자들은 혈변을 호소하였던 3세된 여아에서 소아에서 비교적 드문 식도 정맥류와 비종대가 합병된 특발성 문맥 고혈압 1예를 경험하였기에 문헌 고찰과 더불어 보고하는 바이다.

• Tuberculosis and Respiratory Diseases
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• 제48권1호
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• pp.67-71
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• 2000

문맥고혈압에 동반된 폐고혈압은 비교적 드문 질환으로 1951년 Mantz와 Craig가 처음 보고한 후 꾸준히 보고되고 있으나 국내엔 아직 보고된 바가 없다. 최근 간경변증으로 인한 복수와 호흡곤란을 주소로 내원한 49세 여자 환자에서 폐고혈압이 동반되어 있었던 증례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• 대한영상의학회지
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• 제79권5호
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• pp.264-270
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• 2018

목적: 간기능이 보존된 간세포암 환자에서 간문맥 고혈압이 고주파열치료 후 종양 재발에 미치는 영향을 평가한다. 대상과 방법: 2010년 1월에서 2017년 3월 사이에 Milan criteria 및 Child-Pugh class A를 가진 신규 간세포암 환자 중 본원에서 고주파열치료를 시행한 환자가 본 연구에 포함되었다. 종양 재발에 대한 예측인자를 찾기 위해 Cox proportional hazard model을 이용한 단변량 및 다변량 분석을 수행하였다. 결과: 모두 178명의 환자가 본 연구에 포함되었다. 추적 관찰 기간의 중앙값은 42.8개월이었다. 국소 재발률은 문맥고혈압 여부에 따라 뚜렷한 차이를 유발하지 않았다(p = 0.195). 3년 및 5년 원위부 간내 종양 재발률은 문맥고혈압이 없는 환자의 경우 각각 29.5%와 53.7%, 그리고 문맥고혈압이 있는 환자의 경우 51.9%와 63.6%였으며 두 군 사이의 차이는 통계적으로 유의하였다(p = 0.011). 단변량 및 다변량 분석에서 문맥압항진은 원위부 간내 종양 재발에 대한 독립적인 예측 인자이었다(p = 0.008). 결론: Child-Pugh class A를 가진 간세포암 환자의 경우, 문맥고혈압은 종양 재발에 불량 예후인자로 작용하였다.

• 한국간담췌외과학회지
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• 제27권3호
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• pp.264-270
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• 2023

Backgrounds/Aims: Proximal splenorenal shunt (PSRS) is a commonly performed procedure to decompress portal hypertension, in patients with refractory variceal bleed, especially in non-cirrhotic portal hypertension (NCPH). If conventional methods are hindered by any technical or pathological factors, alternative surgical techniques may be required. This study analyzes the effectiveness of various unconventional shunt surgeries performed for NCPH. Methods: A retrospective analysis of NCPH patients who underwent unconventional shunt surgeries during the period July 2011 to June 2022 was conducted. All patients were followed up for a minimum of 12 months with doppler study of the shunt to assess shunt patency, and upper gastrointestinal endoscopy to evaluate the regression of varices. Results: During the study period, 130 patients underwent shunt surgery; among these, 31 underwent unconventional shunts (splenoadrenal shunt [SAS], 12; interposition mesocaval shunt [iMCS], 8; interposition PSRS [iPSRS], 6; jejunal vein-cava shunt [JCS], 3; left gastroepiploic-renal shunt [LGERS], 2). The main indications for unconventional shunts were left renal vein aberration (SAS, 8/12), splenic vein narrowing (iMCS, 5/8), portalhypertensive vascular changes (iPSRS, 6/6), and portomesenteric thrombosis (JCS, 3/3). The median fall in portal pressure was more in SAS (12.1 mm Hg), and operative time more in JCS, 8.4 hours (range, 5-9 hours). During a median follow-up of 36 months (6-54 months), shunt thrombosis had been reported in all cases of LGERS, and less in SAS (3/12). Variceal regression rate was high in SAS, and least in LGERS. Hypersplenism had reversed in all patients, and 6/31 patients had a recurrent bleed. Conclusions: Unconventional shunt surgery is effective in patients unsuited for other shunts, especially PSRS, and it achieves the desired effects in a significant proportion of patients.

• Clinical Endoscopy
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• 제51권6호
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• pp.563-569
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• 2018

Background/Aims: To evaluate patients with portal hypertension (PH) of varied etiologies for portal hypertensive enteropathy (PHE) using the PillCam SB3 capsule endoscopy (CE) system. Methods: Consecutive patients with PH presenting with unexplained anemia and/or occult gastrointestinal bleeding were evaluated using the PillCam SB3 CE system. Abnormal findings were categorized as vascular or non-vascular. The patients with ongoing bleeding caused by PHE were treated. The correlation of the CE scores of PHE with the clinical, laboratory, and endoscopic features was determined. Results: Of the 43 patients included in the study, 41 (95.3%) showed PHE findings. These included varices (67.4%), red spots (60.5%), erythema (44.2%), villous edema (46.5%), telangiectasia (16.3%), and polyps (16.3%). The CE scores varied from 0 to 8 ($mean{\pm}standard$ deviation, $4.09{\pm}1.8$). Five patients (11.6%) showed evidence of ongoing or recent bleeding due to PHE. Three of these five patients underwent endotherapy, and one patient underwent radiological coil placement. Conclusions: The PillCam SB3 CE system revealed a high prevalence of PHE in the patients with PH. Using this system, evidence of bleeding due to PHE was found in a small but definite proportion of the patients.

• 대한영상의학회지
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• 제84권1호
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• pp.291-297
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• 2023

좌측 단독 문맥고혈압은 비장 정맥이 폐쇄되어 생기는 드문 임상 질환이다. 비장 정맥의 혈전증 혹은 외부 압박으로 인해 근위부 비장정맥의 고혈압이 발생하게 되며, 비장의 혈류는 짧은 위 정맥, 관상정맥, 위대망정맥 같은 측부혈관을 통하여 상장간막 정맥 혹은 문맥으로 흘러가게 된다. 위장관 출혈을 유발하는 좌측 단독 문맥고혈압에는 개복 비장절제술이 첫 번째 치료방법으로 추천된다. 하지만 중재시술을 통한 치료가 수술적 교정을 원치 않는 환자들에게는 임상적으로 유용한 치료 선택지가 될 수 있다. 본문에서는 위정맥류 출혈을 가진 좌측 문맥 고혈압에 대한 두 개의 증례를 다루고자 한다.