Acta Pzdiatr Scand 67: 105-107, 1978
CASE REPORT
PELIOSIS HEPATIS IN A CHILD J. IBSEN BANK, D. LYKKEBO and I. HAGERSTRAND From the University Institute of Pathology, Odense University Hospital, Odense, Denmark
ABSTRACT. Bank, J. Ibsen, Lykkebo, D. and Hagerstrand, I. (Institute of Pathology, Odense University Hospital, Odense Denmark). Peliosis hepatis in a child. Acta Paediatr Scand, 67:105, 1978.-A boy is described with peliosis hepatis. He suffered from a Fanconi anaemia and was treated with prednisolone, dianahol and methyltestosterone. A review of the clinical and pathological aspects of the condition is given. KEY-WORDS: Peliosis hepatis, Fanconi anaemia, anaholic/androgen steroids
Peliosis hepatis is a non-tumourous lesion of the liver, characterized by diffuse round blood-filled cystic foci (2). Peliosis is a Greek word meaning bluish-black. Peliosis hepatis is normally diagnosed at autopsy. It is usually a complication to a prolonged wasting disease, e.g. adult patients suffering from tuberculosis or malignancies; further, it most strikingly occurs in patients treated with anabolic steroids (1). Two varieties have been described (8): 1) a phlebectatic type with aneurysmal dilatation of the central veins, and 2) a parenchymal type with diffusely scattered lakes with or without fibrous lining, and often associated with parenchymal necrosis. A combination of these two types can be seen (1). A few cases of the disease have been reported in children (4, 6). We report the condition in a boy with a Fanconi anaemia. CASE REPORT A male child, 54 years old at death. He was born 2 months before term, and weighed 1700 grams. He had syndaktylia of the toes (2nd and 3rd; bilateral), and there was no osseous connection between the right thumb and wrist. Duodenal atresia was diagnosed and operated upon in
the neonatal period. His karyotype was found to be normal. His psychomotoric development was retarded. At the age of one year, aplastic anaemia was diagnosed and from his 3rd year, he was treated with prednisolone (2.5 mg daily) and dianabol(O.5 mg daily). In the spring of 1976 his condition deteriorated, with episodes of severe epistaxis and repeated infections. In May 1976 the treatment was changed to methyltestosterone (10 mgx3) and hydrocortisone (40 mgx4). However, no remission was achieved, and a bone marrow transplant was considered, but the patient died of septicaemia before this could be carried out. Circulatory insufficiency with crural oedema developed a few days before death. Liver tests were not performed. Autopsy revealed bacterial (E. coli) septicaemia with foci in the lungs, kidneys and thymus, together with oesophagitis and laryngitis due to candida infection. As expected, the adrenal cortex was atrophic due to the prednisolone therapy. The bone marrow was unchanged in relation to the findings 3 years earlier, where a moderate, general hypoplasia was present. A horseshoe kidney and hydrocephalus were found in addition to the previously described malformations. The liver showed numerous, diffusely dispersed, bloodfilled cysts of a diameter of some millimeters (Fig. 1); these were somewhat more numerous in the right than in the left lobe. Microscopically, the cysts were found randomly distributed in the hepatic lobules, and most had the appearance of ectasies of the sinusoids, without wall structures (Fig. 2). Some cysts had reticulin fibres in the periphery, but these were more often situated between the liver cell plates adjacent to the cysts than as a continuous layer around the cyst (Fig. 3). A few cysts had the form of an aneurism of the central veins. The cysts Acta Psdiatr Scand 67
106 J . Zbsen Bank et al.
Fig. 1 . Macroscopic view of the liver with numerous small cysts. The photograph was taken using a colour diapositive. Fig. 2. Microscopic picture of the cysts showing half of a large cyst at the bottom and a smaller cyst at the right
top corner. There are dilated sinusoids between the cysts. Reticulin stain, X30. Fig. 3 . A cyst continuous with hepatic sinusoids, showing reticulin fibres partly in the wall and partly between adjacent hepatocytes. Reticulin stain, x 120.
latter authors mentioned 7 other cases of peliosis hepatis in children reported from the Armed Forces Institute of Pathology. As in our patient, the other two children showed a parenchymal type of peliosis hepatis, but with features of the phlebectatic type also. The diagnosis in all three cases was made DISCUSSION at autopsy, as is usually the case in adults. The Peliosis hepatis has previously been described total number of cases of peliosis hepatis rein two children. Usatin & Wigger (6) reported ported in the literature hardly exceeds 200. an 11-year-old boy with cystic fibrosis of the There seems to be no obvious sex predominpancreas, treated in his last 2 years with ance (9), even though the material of Henrikmethandrosterone. Nurnberger & Ramos (4) son & Odelberg (2) consisted of 23 females and described a 15-month-old female child dying of 2 males. Their patients had tumours treated septicaemia without any other known disease with anabolic steroids, a therapy which is used and without drug intervention. However, the more commonly in females, i.e. women suffer-
were filled with blood, however, no fresh or healed thrombi were observed. The hepatic parenchyma was normal between the cysts, except for dilatation of the centrolobular sinusoids ( F i g . 2). No necrosis or cholestasis were seen. The autoptical findings were consistent with the clinical diagnosis of Fanconi's anaemia (7).
Acru Pediatr Scund67
Peliosis hepatis in a child
ing from mammary, ovarian or uterine carcinoma. Peliosis has been reported as being reversible; the liver may be palpable and tender, and the parameters are normal or elevated (1). Clinicians should be conversant with the disease as intra-abdominal haemorrhage has been described, similarly percutaneous liver biopsy may be hazardous (1). The pathogenesis of peliosis hepatis is still unclear, and the role of anabolic steroids, which also cause nodular regeneration and true tumours of the liver, is puzzling (5). The most favoured theory of the pathogenesis of the condition is widening of the sinusoids after absorption of liver cell necroses, and many authors consider that rightsided heart failure is necessary for the development of the ectasies (9). In our opinion peliosis hepatis is not rarer in children than adults, as it has been reported in ten children; in addition peliosis hepatis is mainly described nowadays following androgen therapy (3), a type of treatment rarely used in children.
107
REFERENCES 1 . Bagheri, S. A. & Boyer, J. L.: Peliosis hepatis associated with androgenic-anabolic steroid therapy. Ann Intern Med, 81: 610, 1974. 2. Henrikson, H. & Odelberg-Johnson, 0.: Peliosis hepatis. Acra Path01 Microbiol Scand, 53: 156, 1961. 3. Naeim, F., Copper, P. H. & Semion, A. A.: Peliosis hepatis: Possible etiologic role of anabolic steroids. Arch Parhol, 95: 284, 1973. 4. Niirnberger, S. P. & Ramos, C. V.: Peliosis hepatis in an infant. J Pediatr, 87: 424, 1975. 5 . Sweeny, E. C. & Evans, D. J.: Hepatic lesions in patients treated with synthetic anabolic steroids. J Clin Parhol, 29: 626, 1976. 6. Usatin, Marc S. & Wigger, H. J.: Peliosis hepatis in a child. Arch Parhol Lab Med, 100: 419, 1976. 7. Winthrobe: Clinical Hematology. Lea & Febiger, Philadelphia 1975, 7th ed., p. 1767. 8. Yanoff, M. & Rawson, A. J.: Peliosis hepatis: An anatomic study with demonstration of two varieties. Arch Parhol, 77: 159, 1964. 9. Zak, F. G.: Peliosis hepatis. Am J Parhol, 26: I , 1950.
Submitted April 25, 1977 Accepted July 15, 1977 (I. H.) Institute of Pathology Odense University Hospital DK-5000 Odense Denmark
Acra Pzdiatr Scand 67
CASE REPORT
PELIOSIS HEPATIS IN A CHILD J. IBSEN BANK, D. LYKKEBO and I. HAGERSTRAND From the University Institute of Pathology, Odense University Hospital, Odense, Denmark
ABSTRACT. Bank, J. Ibsen, Lykkebo, D. and Hagerstrand, I. (Institute of Pathology, Odense University Hospital, Odense Denmark). Peliosis hepatis in a child. Acta Paediatr Scand, 67:105, 1978.-A boy is described with peliosis hepatis. He suffered from a Fanconi anaemia and was treated with prednisolone, dianahol and methyltestosterone. A review of the clinical and pathological aspects of the condition is given. KEY-WORDS: Peliosis hepatis, Fanconi anaemia, anaholic/androgen steroids
Peliosis hepatis is a non-tumourous lesion of the liver, characterized by diffuse round blood-filled cystic foci (2). Peliosis is a Greek word meaning bluish-black. Peliosis hepatis is normally diagnosed at autopsy. It is usually a complication to a prolonged wasting disease, e.g. adult patients suffering from tuberculosis or malignancies; further, it most strikingly occurs in patients treated with anabolic steroids (1). Two varieties have been described (8): 1) a phlebectatic type with aneurysmal dilatation of the central veins, and 2) a parenchymal type with diffusely scattered lakes with or without fibrous lining, and often associated with parenchymal necrosis. A combination of these two types can be seen (1). A few cases of the disease have been reported in children (4, 6). We report the condition in a boy with a Fanconi anaemia. CASE REPORT A male child, 54 years old at death. He was born 2 months before term, and weighed 1700 grams. He had syndaktylia of the toes (2nd and 3rd; bilateral), and there was no osseous connection between the right thumb and wrist. Duodenal atresia was diagnosed and operated upon in
the neonatal period. His karyotype was found to be normal. His psychomotoric development was retarded. At the age of one year, aplastic anaemia was diagnosed and from his 3rd year, he was treated with prednisolone (2.5 mg daily) and dianabol(O.5 mg daily). In the spring of 1976 his condition deteriorated, with episodes of severe epistaxis and repeated infections. In May 1976 the treatment was changed to methyltestosterone (10 mgx3) and hydrocortisone (40 mgx4). However, no remission was achieved, and a bone marrow transplant was considered, but the patient died of septicaemia before this could be carried out. Circulatory insufficiency with crural oedema developed a few days before death. Liver tests were not performed. Autopsy revealed bacterial (E. coli) septicaemia with foci in the lungs, kidneys and thymus, together with oesophagitis and laryngitis due to candida infection. As expected, the adrenal cortex was atrophic due to the prednisolone therapy. The bone marrow was unchanged in relation to the findings 3 years earlier, where a moderate, general hypoplasia was present. A horseshoe kidney and hydrocephalus were found in addition to the previously described malformations. The liver showed numerous, diffusely dispersed, bloodfilled cysts of a diameter of some millimeters (Fig. 1); these were somewhat more numerous in the right than in the left lobe. Microscopically, the cysts were found randomly distributed in the hepatic lobules, and most had the appearance of ectasies of the sinusoids, without wall structures (Fig. 2). Some cysts had reticulin fibres in the periphery, but these were more often situated between the liver cell plates adjacent to the cysts than as a continuous layer around the cyst (Fig. 3). A few cysts had the form of an aneurism of the central veins. The cysts Acta Psdiatr Scand 67
106 J . Zbsen Bank et al.
Fig. 1 . Macroscopic view of the liver with numerous small cysts. The photograph was taken using a colour diapositive. Fig. 2. Microscopic picture of the cysts showing half of a large cyst at the bottom and a smaller cyst at the right
top corner. There are dilated sinusoids between the cysts. Reticulin stain, X30. Fig. 3 . A cyst continuous with hepatic sinusoids, showing reticulin fibres partly in the wall and partly between adjacent hepatocytes. Reticulin stain, x 120.
latter authors mentioned 7 other cases of peliosis hepatis in children reported from the Armed Forces Institute of Pathology. As in our patient, the other two children showed a parenchymal type of peliosis hepatis, but with features of the phlebectatic type also. The diagnosis in all three cases was made DISCUSSION at autopsy, as is usually the case in adults. The Peliosis hepatis has previously been described total number of cases of peliosis hepatis rein two children. Usatin & Wigger (6) reported ported in the literature hardly exceeds 200. an 11-year-old boy with cystic fibrosis of the There seems to be no obvious sex predominpancreas, treated in his last 2 years with ance (9), even though the material of Henrikmethandrosterone. Nurnberger & Ramos (4) son & Odelberg (2) consisted of 23 females and described a 15-month-old female child dying of 2 males. Their patients had tumours treated septicaemia without any other known disease with anabolic steroids, a therapy which is used and without drug intervention. However, the more commonly in females, i.e. women suffer-
were filled with blood, however, no fresh or healed thrombi were observed. The hepatic parenchyma was normal between the cysts, except for dilatation of the centrolobular sinusoids ( F i g . 2). No necrosis or cholestasis were seen. The autoptical findings were consistent with the clinical diagnosis of Fanconi's anaemia (7).
Acru Pediatr Scund67
Peliosis hepatis in a child
ing from mammary, ovarian or uterine carcinoma. Peliosis has been reported as being reversible; the liver may be palpable and tender, and the parameters are normal or elevated (1). Clinicians should be conversant with the disease as intra-abdominal haemorrhage has been described, similarly percutaneous liver biopsy may be hazardous (1). The pathogenesis of peliosis hepatis is still unclear, and the role of anabolic steroids, which also cause nodular regeneration and true tumours of the liver, is puzzling (5). The most favoured theory of the pathogenesis of the condition is widening of the sinusoids after absorption of liver cell necroses, and many authors consider that rightsided heart failure is necessary for the development of the ectasies (9). In our opinion peliosis hepatis is not rarer in children than adults, as it has been reported in ten children; in addition peliosis hepatis is mainly described nowadays following androgen therapy (3), a type of treatment rarely used in children.
107
REFERENCES 1 . Bagheri, S. A. & Boyer, J. L.: Peliosis hepatis associated with androgenic-anabolic steroid therapy. Ann Intern Med, 81: 610, 1974. 2. Henrikson, H. & Odelberg-Johnson, 0.: Peliosis hepatis. Acra Path01 Microbiol Scand, 53: 156, 1961. 3. Naeim, F., Copper, P. H. & Semion, A. A.: Peliosis hepatis: Possible etiologic role of anabolic steroids. Arch Parhol, 95: 284, 1973. 4. Niirnberger, S. P. & Ramos, C. V.: Peliosis hepatis in an infant. J Pediatr, 87: 424, 1975. 5 . Sweeny, E. C. & Evans, D. J.: Hepatic lesions in patients treated with synthetic anabolic steroids. J Clin Parhol, 29: 626, 1976. 6. Usatin, Marc S. & Wigger, H. J.: Peliosis hepatis in a child. Arch Parhol Lab Med, 100: 419, 1976. 7. Winthrobe: Clinical Hematology. Lea & Febiger, Philadelphia 1975, 7th ed., p. 1767. 8. Yanoff, M. & Rawson, A. J.: Peliosis hepatis: An anatomic study with demonstration of two varieties. Arch Parhol, 77: 159, 1964. 9. Zak, F. G.: Peliosis hepatis. Am J Parhol, 26: I , 1950.
Submitted April 25, 1977 Accepted July 15, 1977 (I. H.) Institute of Pathology Odense University Hospital DK-5000 Odense Denmark
Acra Pzdiatr Scand 67
