scholarly journals Budd-Chiari Syndrome: Aetiologi, Diagnosis, and Management

Author(s):  
Gunady Wibowo R ◽  
Hirlan Hirlan

Budd-Chiari Syndrome (BCS) is known as a disease caused by the presence of outflow obstruction of the hepatic vein and is commonly not considered as a diagnosis. Such obstruction may happen in all hepatic vein flow points, starting from small veins to the inferior vena cava opening in the right atrium, regardless of the aetiology. This syndrome occurs in approximately 0.001% population and has various aetiologies, including congenital and acquired prothrombotic conditions, myeloproliferative disease, and oral contraceptives use. Advancement in imaging enables most BCS to be diagnosed based on non-invasive imaging tests. Colour Doppler ultrasonography, which has sensitivity and specificity rate of 85-95%, is an appropriate technique for early investigation of Budd-Chiari syndrome. Colour Doppler ultrasonography and MSCT with contrast have a significancy rate of 85-91.3% with a p value 0.05 between both modalities in diagnosing BCS. Management of BCS starts with the non-invasive to highly invasive procedures, consisting of medical or recanalization therapy to prevent necrosis.

2018 ◽  
Vol 8 ◽  
pp. 41
Author(s):  
Malarkodi Thanthoni ◽  
P Rajeev ◽  
S Sathasivasubramanian

Calibre-persistent labial artery (CPLA) is a commonly underdiagnosed vascular lesion of the lip. CPLA is an arterial branch that penetrates the submucosal tissue without loss of calibre. Clinical diagnosis is significant as misdiagnosis can lead to profuse haemorrhage following an excisional biopsy or surgical excision. Colour Doppler ultrasonography is a safe and non-invasive diagnostic tool to confirm the diagnosis. Here, we report a case of a 24-year-old man who complained of an asymptomatic pulsating non-progressive nodule on the left side of upper lip initially diagnosed as peripheral angiomatous lesion. Diagnosis was confirmed by high-resolution Colour Doppler Ultrasonography. The purpose of this case report is to highlight the clinical importance and diagnosis of a rarely reported soft tissue swelling of the lip to the attention of clinicians.


2021 ◽  
Vol 33 (2) ◽  
pp. 169
Author(s):  
F. Villaseñor-González ◽  
H. Álvarez-Gallardo ◽  
M. Kjelland ◽  
A. Velázquez-Roque ◽  
S. Romo

Colour-Doppler ultrasonography (CDU) has been applied for a more detailed examination of the ovary and uterus, mainly local blood flow in ovarian follicles and the corpus luteum (CL). The main use of CDU is for the diagnosis of early pregnancy or the selection of recipients for embryo transfer in cattle; however, this tool can also be used to select oocyte donors according to blood flow to the ovary. The concept is that more blood flow in the ovary is conducive to a healthier intraovarian environment (e.g. higher progesterone levels and removal of reactive oxygen species), yielding higher quality oocytes. The objective of this research was to evaluate invitro embryo production (IVP) after selection of oocyte donors, considering ovary irrigation before ovum pickup (OPU). The research was carried out in the reproduction laboratory at the Palominos Ranch (Jalisco, México). The oocyte donors (n=15) were synchronized before each round of OPU using an intravaginal device (1.9g of progesterone) with oestradiol benzoate (2mg) and cloprostenol sodium (500µg) on Day 0, to avoid the presence of a corpus luteum and to synchronize the follicular wave. On Day 6, the intravaginal device was removed and OPU was performed. All Angus breed donors between 3 and 5 years old with a body condition score between 5–6 (scale 1–9, where 1 is extremely thin and 9 is very obese) and were evaluated with transrectal CDU (Sonoscape S2™) with a linear-array probe (7.5MHz) before the first OPU session only. The oocyte donors were classified subjectively into three categories: low blood flow (LBF, ∼30% ovarian area), median blood flow (MBF, ∼50% ovarian area), and high blood flow (HBF, ∼70% ovarian area) and were submitted to 3 cycles of IVP each (45 total cycles). Semen from a proven bull for IVF was used during all IVP cycles. All oocytes collected from each donor were used in IVF with the same semen in all IVP cycles. The total oocytes collected, and percentages of viable oocytes, cleavage, and blastocysts on Day 7 of culture were evaluated. Statistical analysis was carried out using the GLM procedure of SAS software (version 9.3; SAS Institute Inc.) to evaluate the results of LBF, MBF, and HBF (P-value=0.05). Total oocyte recovery was 11.28±1.92, 10.06±1.31, and 15.52±1.05 for HBF, MBF, and LBF, respectively, being significantly higher for LBF (P<0.05). There were no differences in viable oocytes among groups. Cleavage rates were 53.04%±3.43 for HBF, 43.18%±2.34 for MBF, and 43.69%±1.89 for LBF, being significantly higher for HBF (P<0.05). Percentage of blastocysts on Day 7 was 38.16%±3.80 for HBF, 30.11%±2.60 for MBF, and 17.78%±2.10 for LBF. This value tended to be significantly higher for HBF than MBF, and both were significantly superior to LBF (P<0.05). In conclusion, under the conditions of this research, although LBF donors had more total recovered oocytes, blastocyst rates were increased in HBF and MBF donors. Therefore, based on the results of the present study, CDU can be a useful tool for the selection of oocyte donors.


2020 ◽  
Vol 48 (10) ◽  
pp. 030006052096435
Author(s):  
Dezhao Li ◽  
Chunyu Shi ◽  
Zhongyang Ding ◽  
Xu Li

Hepatic vein thrombosis is a rare occurrence in the clinical course of eosinophilic granulomatosis with polyangiitis (EGPA). The major mechanism of thrombosis has been postulated to involve the release of toxic proteins from eosinophils. A 36-year-old man with EGPA was admitted to our hospital in July 2018 with hematemesis and melena. Findings on physical examination included ascites and pigmentation of the lower extremities. Ultrasonography of the hepatic vein and inferior vena cava showed an obstruction of the hepatic vein. Magnetic resonance imaging showed low enhancement in the right hepatic vein region. At 34 years of age, the patient’s EGPA had initially presented as asthma with eosinophilia (white blood cell count of 11.46 × 1012/L with 14.6% eosinophils). His skin biopsy showed infiltration of inflammatory cells and eosinophils, especially around medium-sized vessels, which was consistent with EGPA. The patient was thus diagnosed with Budd–Chiari syndrome associated with EGPA.


2018 ◽  
Vol 1 (1) ◽  
pp. 76-80
Author(s):  
Ruijie Cao ◽  
Zhanjun Guo ◽  
Jianhua Wu ◽  
Chensi Wu ◽  
Yue Zhao ◽  
...  

Introduction and aim: The Budd-Chiari Syndrome (BCS) is redefined as hepatic vein outflow tract obstruction with a very low incidence. We aim to analyze the etiology and clinical character of BCS in Hebei area of North China.Material and methods: The diagnosis of BCS and alcoholic related liver cirrhosis (Alcohol-LC) are according to the guidelines of American Association for the Study of Liver Diseases (AASLD), while the diagnosis of hepatitis B virus related liver cirrhosis (HBV-LC) is according to the guidelines of European Association for the Study of the Liver (EASL). BCS patients including inferior vena cava block (IVC), hepatic vein block (HV) and inferior vena cava combining with hepatic vein block (IVC/HV) are involved in this analysis.Results: The subtype’s distributions of this disease are more frequent for IVC patients compared with HV and IVC/ HV patients. The subsequent analysis shows that the incidence of BCS is more predisposed to Alcohol-LC than HBV-LC (p < 0.001).Conclusion: BCS seem to be associated with Alcohol-LC compared with that of HBV-LC.


2021 ◽  
Vol 11 ◽  
Author(s):  
Tuerhongjiang Tuxun ◽  
Tao Li ◽  
Shadike Apaer ◽  
Yi-Biao He ◽  
Lei Bai ◽  
...  

We report the first documented case of leiomyosarcoma at zone II-III of inferior vena cava with thrombi in three hepatic veins undergoing ex vivo liver resection and autotransplantation (ELRA) and hepatic veins thrombectomy. A 33-year-old female patient presented with abdominal distention and lower extremities edema. Abdominal wall varicosis and shifting dullness were positive on physical examination. Her liver function was classified as Child-Pugh B and a solid tumor at retro-hepatic vena cava extending to right atrium with thrombi in three hepatic veins were confirmed. The diagnosis of leiomyosarcoma with Budd-Chiari syndrome was highly suspected with preoperative ultrasound, echocardiogram, CT scan, and three-dimensional reconstruction. A zone II-III leiomyosarcoma of IVC origin was confirmed at surgery and ex vivo liver resection and autotransplantation, and hepatic vein thrombectomy with atrial reconstruction were performed under cardiopulmonary bypass (CPB). Operative time, anhepatic time, and CPB time were 12 h, 128 min, and 84 min, respectively. The patients experienced post-operative liver dysfunction and was cured with conservative therapy. Hepatic recurrence two years after surgery was managed with radiofrequency. The patient was alive with liver metastasis three years after surgery. Despite being regarded as an extremely aggressive procedure, ELRA could be considered in the treatment of advanced leiomyosarcoma with Budd-Chiari syndrome and hepatic vein thrombi.


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