|
|
| Year : 2003 | Volume
: 9
| Issue : 2 | Page : 82-83 |
|
| Rare presentation of Budd-chiari syndrome |
|
|
Muneerah Mohammed Al Bugami, Hamad Ibrahim Al Ashgar, Hamad Saleh Al Suhaibani, Mohammad Sultan Khuroo
Department of Medicine, King Faisal Specialist Hospital, Riyadh, Saudi Arabia
Click here for correspondence address and email
| Date of Submission | 25-May-2002 |
| Date of Acceptance | 18-Feb-2003 |
|
|
 |
|
How to cite this article:
Al Bugami MM, Al Ashgar HI, Al Suhaibani HS, Khuroo MS. Rare presentation of Budd-chiari syndrome. Saudi J Gastroenterol 2003;9:82-3 |
Budd-Chiari syndrome More Details (BCS) is obstruction to the venous output of the liver owing to occlusion of hepatic veins. In one third of patient, the cause is idiopathic but specific causes include trauma to the liver and membranous web of inferior vena cava (IVC) and/or hepatic vein [1],[2],[3] , systemic lupus erythematous (SLE) and antiphospholipid syndrome [4],[5],[6],[7] , other hypercoagulation state e.g. factor V [1],[2],[3],[4],[5],[6],[7] . Leiden Mutation [8],[9],[10] prothrombin mutation, anti thrombin III deficiency [11],[12] protein C deficiency, [13],[14] protein S deficiency, [15] pregnancy, [16][ and Behcets disease [17] and finally malignancy like hepatocellular carcinoma and myeloproliferative disorders [18],[19],[20],[21],[22] .
Patient usually presents acutely with abdominal pain, nausea, vomiting, tender hepatomegaly and ascites. In the chronic form, there is hepatomegaly, mild jaundice and ascites. Investigations can show high protein content in ascitic fluid, ultrasound with doppler, CT Scan and magnetic resonance, as well as hepatic venography imaging will demonstrate hepatic vein occlusion. We will present a case budd-chiari syndrome in a 22-year old female with SLE.
 Case Report | |  |
A 22-year old lady presented with right hypochondrial pain, fever, nausea, vomiting for one day with normal liver function test. She had cholecystectomy followed by jaundice-abnormal liver function tests and fever post operatively. She was investigated fully for persistence of fever without obvious cause. One year later, she had abdominal distention and pain, which increased over eight months with jaundice.
On examination, the patient was jaundiced, there was bilateral palmer erythema, acne on the face and trunk, no oral ulceration, no lymphadenopathy, no pallor and no other signs of chronic liver disease. Vital signs were T 36.8° C, P 80/min, BP 110/80 mmHg, RR 14/min. Chest, cardiovascular, and neurological examinations were all normal. The abdomen showed mild tenderness on the right hypochondrial area, ascites, and no organomegaly.
Laboratory work revealed: CBC - WBC 8.8 x 10 9 /L, Hb 147 g/dL, ↓ plat 62,000 x 10 9 /L, T PT 17.8 sec, PTT 34 sec. She had normal renal function. Liver function tests revealed albumin 34 g/L, ALP 92 U/L, ALT 224 U/L, and bilirubin 29 tmol/L. Ascitic fluid showed glucose 4.1 mmol, total protein of 15 g/L, albumin < 10 g/L, WBC 100, poly 6%, lymph 54%, RBC 4450, negative for malignant cells and negative for tuberculosis. Antinuclear antibodies was mixed pattern (homogenous and nuclear) 1:320, DNA was 169 IU/ml (0-36), low C3 was 0.4, low C4 < 0. 1, CH50 64 (345-485) and anticardiolipin antibodies were negative. Abdominal ultrasonography revealed hypertrophy (L) lobe of liver with huge ascites and doppler ultrasonography revealed no flow in (L) hepatic vein with sluggish intermittent flow in (R) portal vein. Main portal vein was patent. Finally, dual phase of CT was suggestive of BCS with occlusion of hepatic veins. The diagnosis of acute BCS, most likely secondary to SLE based on the following criteria:
(1) Thrombocytopenia < 100,
(2) positive ANA,
(3) Positive DNA.
Hepatic vein was dilated with a IOmm/3 cm, balloon. Hepatic pressure wedge dropped from 25 mmHg to 5mmHg. She was started on anticoagulation, initially on heparin and later on warfarin. Also, she was put on prednisone for SLE. Her ascites required ascitic tap three times to relieve abdominal distention. She was started on Aldactone but after dilatation of hepatic vein and steroid there was significant improvement. She had repeated doppler U/S, which was normal and was discharged home on stable condition. She had a follow up in the Out patient Clinic more than 18 months and her steroid was tapered slowly to 10mg of Prednisone and warfarin 6mg to keep INR 2.5-3.5.
| Discussion | | |
BCS in association with lupus anticoagulant has been well-documented [4],[5],[6],[7] . Many case reports of this association have been documented worldwide. Reports of BCS in patients with SLE without lupus anticoagulants is rare and most reported cases have been before lupus anticoagulant serology became available. Our patient does satisfy three criteria proposed for the diagnosis of SLE developed by the American Rheumatism Association. Management of such patients can be difficult but management of the primary disease is as important as the secondary complication i.e. BCS. Our patients did well on treatment of SLE with steroids, dilatations and anticoagulation for BCS. This case highlights that SLE can be a prothrombotic disease without lupus anticoagulant. The mechanism could be secondary to vasculitis of hepatic veins or hypercoagulation secondary to the inflammatory process in SLE.
| References | | |
| 1. | Rector WG, XU Y, Goldstein L, et al. Membranous obstruction of the inferior vena cava in the United States. Medicine (Baltimore) 1985; 64: 134.  |
| 2. | Simson IW. Membranous obstruction of the inferior vena cava and hepatocellular carcinoma in South Africa. Gastroenterology 1982; 82: 171-8. [PUBMED] |
| 3. | Sevenet F, Dramond, Hadengue A, et al. Membranous obstruction of inferior vena cava associated with myoproliferative disorder. A close to membranous formation? Gastroenterology 1989; 97: 1019-21. |
| 4. | Saca L, Sze IS, Henar E, et al. BCS associated with antipholipid antibodies in a child: Report of a case and review of literature. J Rheumatol 1994; 21: 5458. |
| 5. | Mackworth-Young CG, Melia WK Harris FN, et al. BCS: Possible pathogenetic role of antipholipid antibodies. J Hepatol 1986; 3: 83-6. |
| 6. | Van Steenberger W, Beyls J, Vermylen J, et al. Lupus anticoagulant and the thrombosis of hepatic vein (BCS): Report of 3 patients and review of the literature J Hepatol 1986; 3: 87-94. |
| 7. | Pameroy C, Knodell RG, Swain WR, et al. BCS in patient with the lupus anticoagulation. Gastroenteroogy 1984; 86: 158-61. |
| 8. | Fickert P, Ramschk W, Kermer L, et al. Acute BCS with fulminant hepatic failure in pregnant women with factor V leiden mutation. Gastroenterology 1996; 111: 1670-73. |
| 9. | Price DT, Ridker P. Factor V leiden mutation and the risks for thromboembolic disease. A clinical perspective. Ann Intern Med 1997; 127: 895-903. |
| 10. | Mohamoud AE, Elias E, Beauchamp N, Widde JJ. Prevalence of the factor V leiden mutation in hepatic and portal vein thrombosis. GUT 1997; 40: 798-800. |
| 11. | Das M, Caroll SF. Anti thrombin III deficiency. An etiology of BCS surgery 1985; 97: 242-6. |
| 12. | McClure S, Dincsoy MP, Glueck H. Case report, BCS and anti thrombin III deficiency. Am J Clin Path 1982; 78: 236-41. |
| 13. | Sugano S, Suzuki T, Makino H, et al. BCS attributed to protein C deficiency. Am J Gastroenterol 1996; 91:770-79. |
| 14. | Bouliere M, Le Treut YP, Anoux D, et al. Acute BCS with hepatic failure and obstruction of inferior vena cava as presenting manifestatoin of hereditary protein C deficiency. GUT 1980; 31: 949-52. |
| 15. | Majluf-Cruz, A, Monroy, RH, Garcia LS, et al. The incidence of protein C deficiency in thrombosis related portal hypertension. Am J Gastroenterol 1996; 91: 976-80. |
| 16. | Khuroo MS, Datta DV. BCS following pregnancy: Report of 16 cases with roentgenologic hemodynamic and histologic studies of the hepatic outflow tract. Am J Med 1980; 69: 113-21. |
| 17. | Bayraktar Y, Balkanci F, Bayraktar M, et al. BCS. A common complication of Behcets disease. Am J Gastroenterol 1997; 92: 858-62. |
| 18. | Valla D, Casadevall N, Lacombe C, et al. Primary myeloproliferative disorder and hepatic BCS. Ann Intern Med 1985; 103: 329-34. [PUBMED] |
| 19. | Pagliuca A, Mufti GJ, Janossa-Tahernia M, et al. In vito colony culture and chromosmatic studies in hepatic and portal vein thrombosis-possible evidence of an acute myeloproliferative state. QS Med 1990; 76: 981. |
| 20. | Acharya J, Westwood AJ, Sawyer BM, et al. Identification of latent myeloproliferative disease inpatient with BCS using X chrome inactivation pattern and in vitro erythroid colony formation. Eur J Hematol 1995; 55: 315-21. |
| 21. | De Stefano V, Teofili L, Leone G, Michiels JJ. Spontaneous erythroid colony formation as the clue to an underlying myeloproliferative disorder in patients with BCS or portal vein thrombosis. Semin Thromb Homost 1997; 23: 411-8. |
| 22. | Levy VG, Ruskone A, Baillou C, et al. Polycythemia and the BCS: study of serum erythroprotein and bone marrow erythroid progenitors. Hepatology 1985; 5: 858-61. [PUBMED] |
Correspondence Address:
Hamad Ibrahim Al Ashgar
Dept of Medicine (MBC 46), King Faisal Specialist Hospital, P 0. Box 3354, Riyadh 11211
Saudi Arabia
 Source of Support: None, Conflict of Interest: None  | Check |
PMID: 19861812 
|
|
|
|
|
|
|
|
|
|
|
|
|
|
| Article Access Statistics | | | Viewed | 4097 | | | Printed | 121 | | | Emailed | 1 | | | PDF Downloaded | 0 | | | Comments | [Add] | | |
|
|
