J Korean Med Sci.  2013 Oct;28(10):1507-1511. 10.3346/jkms.2013.28.10.1507.

Hepatoportal Sclerosis in Childhood: Descriptive Analysis of 12 Patients

• Cantez MS
• Gerenli N
• Ertekin V
• Gulluoglu
• Durmaz O

• KMID: 1777689
• DOI: http://doi.org/10.3346/jkms.2013.28.10.1507

Abstract

Hepatoportal sclerosis (HPS) is defined as sclerosis of portal areas in the absence of cirrhosis. There is little information about HPS in children in the literature. The aim of this study was to describe the clinical presentation, associated disorders, laboratory characteristics and outcome of children who were diagnosed as HPS. This study included 12 children diagnosed as HPS by the Pathology Department between 2005 and 2011. Data were collected from the gastroenterology clinic charts retrospectively, including demographics, presentation characteristics, laboratory data and recent status of patients. Twelve patients were enrolled (6 girls, 6 boys). The median age of patients was 13.5 yr. Median age at the time of biopsy was 11 yr. Four patients had splenomegaly, 3 had esophageal varices, one had hepatopulmonary syndrome and had been transplanted. Smooth muscle antibody was found positive in 4 patients, without autoimmune hepatitis findings in liver biopsy. One patient had celiac disease and another patient had positive celiac disease serology but pathology findings. Another patient had Turner's syndrome. Mean follow-up time was 39 months (3.3 yr) after biopsy. Hepatoportal sclerosis does not necessarily present with portal hypertension in children.

Keyword

Hepatoportal Sclerosis; Idiopathic Noncirrhotic Portal Hypertension; Child

MeSH Terms

Adolescent
Child
Child, Preschool
Female
Follow-Up Studies
Gastroenterology
Humans
Hypertension, Portal/complications/*diagnosis/pathology
Liver/pathology
Liver Diseases/complications/*diagnosis/pathology
Male
Portal Vein/pathology
Retrospective Studies
Sclerosis/*diagnosis/*pathology

Reference

1. Banti BC. Ueber Morbus Banti. Folia Haematol. 1910; 10:33–74.

2. Mikkelsen WP, Edmondson HA, Peters RL, Redeker AG, Reynolds TB. Extra- and intrahepatic portal hypertension without cirrhosis (hepatoportal sclerosis). Ann Surg. 1965; 162:602–620.

3. Okudaira M, Ohbu M, Okuda K. Idiopathic portal hypertension and its pathology. Semin Liver Dis. 2002; 22:59–72.

4. Sama SK, Bhargava S, Nath NG, Talwar JR, Nayak NC, Tandon BN, Wig KL. Noncirrhotic portal fibrosis. Am J Med. 1971; 51:160–169.

5. Boyer JL, Sen Gupta KP, Biswas SK, Pal NC, Basu Mallick KC, Iber FL, Basu AK. Idiopathic portal hypertension: comparison with the portal hypertension of cirrhosis and extrahepatic portal vein obstruction. Ann Intern Med. 1967; 66:41–68.

6. Okuda K, Kono K, Ohnishi K, Kimura K, Omata M, Koen H, Nakajima Y, Musha H, Hirashima T, Takashi M, et al. Clinical study of eighty-six cases of idiopathic portal hypertension and comparison with cirrhosis with splenomegaly. Gastroenterology. 1984; 86:600–610.

7. Hillaire S, Bonte E, Denninger MH, Casadevall N, Cadranel JF, Lebrec D, Valla D, Degott C. Idiopathic non-cirrhotic intrahepatic portal hypertension in the West: a re-evaluation in 28 patients. Gut. 2002; 51:275–280.

8. Schouten JN, Garcia-Pagan JC, Valla DC, Janssen HL. Idiopathic noncirrhotic portal hypertension. Hepatology. 2011; 54:1071–1081.

9. Ibarrola C, Colina F. Clinicopathological features of nine cases of non-cirrhotic portal hypertension: current definitions and criteria are inadequate. Histopathology. 2003; 42:251–264.

10. Dhiman RK, Chawla Y, Vasishta RK, Kakkar N, Dilawari JB, Trehan MS, Puri P, Mitra SK, Suri S. Non-cirrhotic portal fibrosis (idiopathic portal hypertension): experience with 151 patients and a review of the literature. J Gastroenterol Hepatol. 2002; 17:6–16.

11. Arslan N, Buyukgebiz B, Ozturk Y, Hizli S, Bekem O, Sagol O, Ozer E. Hepatoportal sclerosis in a child. Eur J Pediatr. 2004; 163:683–684.

12. Yilmaz G, Sari S, Egritas O, Dalgic B, Akyol G. Hepatoportal sclerosis in childhood: some presenting with cholestatic features (a re-evaluation of 12 children). Pediatr Dev Pathol. 2012; 15:107–113.

13. Kaymakoglu S, Kahraman T, Kudat H, Demir K, Cakaloglu Y, Adalet I, Dincer D, Besisik F, Boztas G, Sözen AB, et al. Hepatopulmonary syndrome in noncirrhotic portal hypertensive patients. Dig Dis Sci. 2003; 48:556–560.

14. Isabel Fiel M, Thung SN, Hytiroglou P, Emre S, Schiano TD. Liver failure and need for liver transplantation in patients with advanced hepatoportal sclerosis. Am J Surg Pathol. 2007; 31:607–614.

15. Krasinskas AM, Eghtesad B, Kamath PS, Demetris AJ, Abraham SC. Liver transplantation for severe intrahepatic noncirrhotic portal hypertension. Liver Transpl. 2005; 11:627–634.

16. Geramizadeh B, Malek-Hosseini SA, Salahi H, Bahador A, Nikeghbalian S. Liver failure and the need for transplantation in three patients with hepatoportal sclerosis. Transplant Proc. 2008; 40:3526–3528.

17. Girard M, Amiel J, Fabre M, Pariente D, Lyonnet S, Jacquemin E. Adams-Oliver syndrome and hepatoportal sclerosis: occasional association or common mechanism? Am J Med Genet A. 2005; 135:186–189.

18. Pouessel G, Dieux-Coeslier A, Wacrenier A, Fabre M, Gottrand F. Association of Adams-Oliver syndrome and hepatoportal sclerosis: an additional case. Am J Med Genet A. 2006; 140:1028–1029.

19. Roulot D, Degott C, Chazouillères O, Oberti F, Calès P, Carbonell N, Benferhat S, Bresson-Hadni S, Valla D. Vascular involvement of the liver in Turner's syndrome. Hepatology. 2004; 39:239–247.

20. Cunha LM, Bittencourt PL, Abrantes-Lemos CP, Moreira A, Almeida D, Parana R, Andrade Z. Prevalence of non-organ-specific autoantibodies in a rural community from northeastern Brazil: a population-based study. Hum Immunol. 2012; 73:70–74.

21. Azizah MR, Shahnaz M, Zulkifli MN, Nasuruddin BA. Anti-nuclear, anti-mitochondrial, anti-smooth muscle and anti-parietal cell antibodies in the healthy Malaysian population. Malays J Pathol. 1995; 17:83–86.

22. M'saddek F, Gaha K, Ben Hammouda R, Ben Abdelhafidh N, Bougrine F, Battikh R, Louzir B, Bouali R, Bouzayane A, Othmani S. Idiopathic portal hypertension associated with celiac disease: one case. Gastroenterol Clin Biol. 2007; 31:869–871.

23. Zamani F, Amiri A, Shakeri R, Zare A, Mohamadnejad M. Celiac disease as a potential cause of idiopathic portal hypertension: a case report. J Med Case Rep. 2009; 3:68.

24. Schiano TD, Kotler DP, Ferran E, Fiel MI. Hepatoportal sclerosis as a cause of noncirrhotic portal hypertension in patients with HIV. Am J Gastroenterol. 2007; 102:2536–2540.

25. Vispo E, Moreno A, Maida I, Barreiro P, Cuevas A, Albertos S, Soriano V. Noncirrhotic portal hypertension in HIV-infected patients: unique clinical and pathological findings. AIDS. 2010; 24:1171–1176.