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      Budd-Chiari syndrome due to right hepatic lobe herniation: CT image findings of two rare clinical conditions

      case-report
      Author(s): , BSc, BMBS , , BMBS, , MBBS, FRCR
      Publication date (Electronic):
      Journal: BJR Case Reports
      Publisher: The British Institute of Radiology

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          Abstract

          Hepatic herniation is a rare clinical condition. Most commonly it is associated with congenital diaphragmatic herniation or acquired through blunt diaphragmatic trauma. We present a case of a right hepatic lobe incisional hernia in a 75-year-old female who underwent partial right-sided nephrectomy 52 years previously. Evidence of partial Budd-Chiari syndrome was seen on CT scan that was presumed to be as a result of traction of the herniated liver. As far as we are aware this is the first case of a right-sided hepatic hernia with evidence of partial Budd-Chiari syndrome. The patient was treated conservatively with anticoagulation and analgesia.

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          The Budd-Chiari syndrome.

          Vijay Shah, Kavitha Kamath, David Menon (2004)
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            Primary Budd-Chiari syndrome.

            Dominique-Charles Valla (2009)
            Primary Budd-Chiari syndrome is characterized by a blocked hepatic venous outflow tract at various levels from small hepatic veins to inferior vena cava, resulting from thrombosis or its fibrous sequellae. This rare disease affects mainly young adults. Multiple risk factors have been identified and are often combined in the same patient. Myeloproliferative diseases of atypical presentation account for nearly 50% of patients; their diagnosis can be made by showing the V617F mutation in Janus tyrosine kinase-2 gene of peripheral blood granulocytes and, should this mutation be absent, by showing clusters of dystrophic megacaryocytes at bone marrow biopsy. Presentation and manifestations are extremely varied, so that the diagnosis must be considered in any patient with acute or chronic liver disease. Doppler-ultrasound, computed tomography or magnetic resonance imaging of hepatic veins and inferior vena cava are usually successful in demonstrating non-invasively the obstacle or its consequences, the collaterals to hepatic veins or inferior vena cava. The disease is considered to be spontaneously lethal within 3 years of first symptoms. A therapeutic strategy has been proposed where anticoagulation, correction of risk factors, diuretics and prophylaxis for portal hypertension are used first; then angioplasty for short-length venous stenoses; then TIPS; and ultimately liver transplantation. Treatment progression is dictated by the response to previous therapy. This strategy has achieved 5-year survival rates approaching 90%. Medium-term prognosis depends on the severity of liver disease. Long-term outcome might be jeopardized by transformation of underlying conditions and hepatocellular carcinoma.
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              Paracetamol serum concentrations in preterm infants treated with paracetamol intravenously: a case series

              Christ-jan van Ganzewinkel, Thilo Mohns, Richard van Lingen … (2012)
              Introduction Until now, studies on paracetamol given intravenously have mainly been performed with the pro-drug propacetamol or with paracetamol in preterm babies above 32 weeks of gestation. Studies in these babies indicate that intravenous paracetamol is tolerated well, however studies on the efficacy of intravenous paracetamol are lacking. There are no pharmacokinetic data on the administration of multiple doses of paracetamol in preterm babies with a gestational age below 32 weeks. Case presentation We present a case series of nine Caucasian preterm babies, six boys and three girls, with a mean gestational age of 28.6 weeks (range 25.9 to 31.6 weeks). Case one, a girl with a gestational age of 25 weeks and six days, presented with necrotizing enterocolitis. In the second case, a female baby with a gestational age of 26 weeks and two days presented with hematoma. In case three, a female baby with a gestation of 26 weeks and one day developed intraventricular hemorrhage. In case four, a male baby with a gestational age of 31 weeks and four days presented with pain after vacuum delivery. Case five, a female baby born after a gestation of 29 weeks and six days presented with hematoma. In case six, a male baby with a gestation of 30 weeks and six days presented with hematoma. In case seven, a male baby, born with a gestational age of 30 weeks and six days, presented with caput succedaneum and hematoma. In case eight, a male baby, born after a gestation of 28 weeks and four days, developed abdominal distention. Case nine, a female baby, born with a gestational age of 27 weeks and three days presented with hematoma. These babies were treated with intravenous paracetamol 15 mg/kg every six hours. Serum concentrations and aspartate transaminase were determined after prolonged administration. Pain scores were assessed using the Premature Infant Pain Profile. Conclusion Paracetamol serum concentrations ranged from 8 to 64 mg/L after eight to 12 doses of intravenous paracetamol. Adequate analgesia was obtained in seven babies. During paracetamol therapy the median serum level of aspartate transaminase was 20 U/L (range 12 to 186 U/L). This case series indicates that prolonged intravenous administration of paracetamol in preterm babies with a gestational age of less than 32 weeks is tolerated well in the first days after birth. However, in the absence of proper pharmacokinetic data in this age group we cannot advocate the use of paracetamol intravenously.
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                Author and article information

                Contributors
                Shyamal Saujani
                Safi Rahman
                Bruce Fox
                Journal
                Journal ID (nlm-ta): BJR Case Rep
                Journal ID (iso-abbrev): BJR Case Rep
                Journal ID (publisher-id): bjrcr
                Title: BJR Case Reports
                Publisher: The British Institute of Radiology
                ISSN (Electronic): 2055-7159
                Publication date Collection: 2017
                Publication date (Electronic): 30 March 2017
                Volume: 3
                Issue: 3
                Electronic Location Identifier: 20160133
                Affiliations
                [1]Department of Radiology, Plymouth Hospitals NHS Trust , Plymouth, UK
                Author notes
                Address correspondence to: Dr Shyamal Saujani E-mail: shyamal.saujani@ 123456doctors.org.uk
                Article
                Publisher-manuscript ID: bjrcr.20160133
                DOI: 10.1259/bjrcr.20160133
                PMC ID: 6159189
                PubMed ID: 30363244
                SO-VID: 35b0ed1a-f76b-4075-9632-d4b777f15e8d
                Copyright © © 2017 The Authors. Published by the British Institute of Radiology
                License:

                This is an open access article under the terms of the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution and reproduction in any medium, provided the original author and source are credited.

                History
                Date received : 23 October 2016
                Date revision received : 20 December 2016
                Date accepted : 12 January 2017
                Categories
                Subject: Case Report
                Compound subject: bjrcr, BJR|case reports
                Compound subject: gas-abd, Gastrointestinal and abdominal
                Compound subject: vasc, Vascular
                Compound subject: ct, CT

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