BACKGROUND AND PURPOSE: MR imaging and 1 H-MR spectroscopy changes are well reported in cirrhotic patients, whereas they are inadequately reported in EHPVO. The aim of this study was to investigate age-related changes in brain MR imaging and metabolite profile in EHPVO with and without MHE and to explore any correlation of imaging and 1 H-MR spectroscopy parameters with blood ammonia. MATERIALS AND METHODS: Sixty-three patients with EHPVO (children, 7–12 years [ n = 22], adolescents, 13–18 years [ n = 15] and adults, 19–41 years [ n = 26]) and 47 healthy age/sex-matched volunteers were studied. Neuropsychological tests, MR imaging, 1 H-MR spectroscopy, and blood ammonia estimation were performed in all subjects. RESULTS: Of 63 EHPVO patients, 25 (40%) who had MHE showed significantly increased MD, Glx, and blood ammonia in all 3 age groups; however, myo-inositol was significantly lower only in adults when compared with controls. MD positively correlated with blood ammonia and Glx in all age groups. Brain choline levels were normal in all patients with different age groups. CONCLUSIONS: Increases in brain MD, Glx, and blood ammonia were associated with MHE in all age groups. Normal brain choline in EHPVO signifies healthy liver and may serve as a diagnostic marker for its differentiation from cirrhosis-induced encephalopathy. Significant decrease of myo-inositol in adults is probably due to cellular osmoregulation secondary to long-standing hyperammonemia.

http://www.ajnr.org/content/ajnr/31/7/1337.full.pdf

Brain MR imaging and 1H-MR spectroscopy changes in patients with extrahepatic portal vein obstruction from early childhood to adulthood.

Objective:   The objective of the study is to calculate the frequency of portal vein thrombosis in pediatric patients presenting with hematemesis and to estimate the frequency of acute and chronic portal vein thrombosis in these cases. Patients and Methods: Pediatric patients of 1 – 15 years of age of either sex, presenting to emergency or gastroenterology department with complaint of hematemesis were included. Informed consent was taken from parents/guardians. This is a Descriptive cross sectional study conducted in department of pediatric radiology, Children Hospital and Institute of child Health Lahore from 11-12-2012 to 10-6-2013. Doppler ultrasound of these patients was performed at Core Vision pro SSA 350-A Doppler machine (Toshiba, Japan) using standard imaging protocols. The data was analyzed using SPSS version 17.0 (SPSS Inc., IL, Chicago, USA) Results: In this study, 41% (n = 41) between 1 – 5 years, 38% (n = 38) between 6 – 10 years and only 21% (n = 21) between 11 – 15 years of age, 64%( n = 64) were male and remaining 36% (n = 36) were females, frequency of portal vein thrombosis in pediatric patients presenting with hematemesis reveals in 37% (n = 37) while 63% (n = 63) had no findings of PVT, frequency of acute and chronic forms in patients positive for portal vein thrombosis was 40.54% (n = 15) had acute and 59.46% (n = 22) had chronic portal vein thrombosis. Conclusion: We concluded that the frequency of portal vein thrombosis in pediatric patients presenting with hematemesis and frequency of acute and chronic forms in patients positive for portal vein thrombosis is in accordance with other studies while color Doppler ultrasound is a useful diagnostic modality being noninvasive and cost effective.

/pdf/hemodynamic-changes-in-portal-vein-and-frequencies-of-acute-2zqiqdw96e.pdf

Hemodynamic Changes in Portal Vein and Frequencies of Acute and Chronic Portal Vein Thrombosis in Pediatric Patients Presenting with Hematemesis

DOI: https://doi.org/10.31729/jnma.4214
 EHPVO in individual with solitary left kidney is rare occurance. Though there is no etiological association between EHPVO and solitary left kidney but the solitary left kidney decides the modality of treatment. Eighteen year lady referred to our institute with menorrhagia for 5 years and ultrasonography finding of splenomegaly and atretic right kidney. Investigations revealed Extra Hepatic Portal Vein Obstruction with multiple cavernoma formation with oesophagogastric varices with right renal agenesis. She successfully underwent splenectomy with devascularisation. Patient with EHPVO present mainly with recurrent episodes of variceal bleeding, splenomegaly and hypersplenism. Splenectomy and esophagogastric devascularisation is an effective modality of treatment for patient with EHPVO with solitary kidney.
Keywords: cavernoma; EHPVO; modified Hassab’s operation; unilateral renal agenesis.

/pdf/extra-hepatic-portal-vein-obstruction-with-solitary-left-17nv7uqmz9.pdf

Extra Hepatic Portal Vein Obstruction with Solitary Left Kidney: A Case Report

Introduction We aimed to analyse the pregnancy outcome of women with extrahepatic portal vein obstruction. Methods This was a retrospective observational analysis conducted at the Institute of Postgraduate Medical Education and Research, Kolkata, India, between January 2007 and September 2009. A total of 41 pregnancies in 24 women were evaluated. Results All women conceived spontaneously (maternal age 20-35 years). 17 women had moderate-to-severe anaemia, and five women had pancytopenia. Variceal bleeding occurred in ten women during pregnancy, which was managed successfully with endoscopic sclerotherapy in eight women and endoscopic variceal ligation in two women. Preterm labour (14.63%), postpartum haemorrhage (7.31%), abortion (4.87%) and pregnancy-induced hypertension (4.87%) were observed in the 41 pregnancies. There were 39 live births and almost all mothers delivered vaginally, except for four who underwent Caesarean section for obstetric indications. Prematurity (15.38%), low birth weight (10.25%), admission to the neonatal intensive care unit (12.82%), stillbirth (2.56%) and neonatal death (2.56%) were noted in the newborns. Conclusion Variceal bleeding during pregnancy coincided with unfavourable outcomes. Although endoscopic obliteration of varices is a safe and effective method for antenatal management of varices in women, prenatal obliteration results in less morbidity. On rare occasions, obliterated varices can bleed in subsequent pregnancies. Therefore, preconception evaluation of the state of varices prior to each pregnancy and their ligation are important aspects of counselling. A successful foetomaternal outcome is achievable with multidisciplinary backup in a tertiary care centre.

Is pregnancy safe with extrahepatic portal vein obstruction? An analysis.

Objective: To study the clinical and laboratory profile of patients with EHPVO in a tertiary care hospital of Pakistan and to differentiate EHPVO from cirrhosis of liver and to see the effect on liver function tests. Methodology: This is a prospective observational study conducted at Department of Gastroenterology, Pakistan Institute of Medical Sciences, Islamabad.Twenty five patients of 12–55 years of age with the features of portal hypertension were included in this study. After careful history and physical examination patients were subjected for laboratory investigations including liver function test, renal function test, blood CP, PT, APTT, HbsAg and anti HCV, other specialized procedures including endoscopy, liver biopsy and ultra sound was also done in all patients. Results: Portal vein thrombosis was the predominant cause of EHPVO, accounting for 88% of cases. All patients were presented with upper GI bleeding, splenomegaly was observed in 88% of patients. None of the patients had clinical, biochemical or liver biopsy evidence of chronic liver disease. Conclusion: The diagnosis of extra hepatic portal venous obstruction and differentiation from cirrhosis can be easily made by characteristic clinical features, normal liver function tests and doppler ultrasound. Portal vein thrombosis (PVT) is the predominant cause of EHPVO in Pakistani patients, as seen at this tertiary care hospital in Pakistan.

Profile of extrahepatic portal venous obstruction (ehpvo) in a tertiary care hospital in pakistan

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Profile of extrahepatic portal venous obstruction (ehpvo) in a tertiary care hospital in pakistan