Peliosis Hepatis in a Child with X-Linked Myotubular Myopathy Treated with Living-Donor Liver Transplant: A Case Report.

BACKGROUND: Myotubular myopathy is a rare disease sometimes accompanied by peliosis hepatis, a leading cause of fatal liver hemorrhage. CASE REPORT: We present a case of a 2-year-old boy with myotubular myopathy who developed liver hemorrhage because of peliosis hepatis and was successfully treated with living-donor liver transplant. The patient initially presented with fever, anemia, and liver dysfunction. A computed tomographic scan revealed hemorrhages in the liver, and the patient underwent hepatic artery embolization twice. After the second embolization, multiple peliosis hepatis cavities appeared in the left lobe of the liver that had increased in size. Therefore, the patient underwent ABO-incompatible living-donor liver transplant using a lateral segment graft from his father. The patient developed severe septic shock with an unknown focus on postoperative day 18, which resolved with antibiotic therapy. On postoperative day 62, he was discharged. Fourteen months after undergoing living-donor liver transplant, the patient showed no recurrence of peliosis hepatis. CONCLUSIONS: Although the long-term prognosis of peliosis hepatis due to myotubular myopathy after living-donor liver transplant remains unclear, liver transplant may be a curative treatment for patients with myotubular myopathy who have uncontrollable peliosis hepatis.

Peliosis hepatis in a child with myotubular myopathy: successful treatment using hepatic artery embolization.

Peliosis hepatis (PH) is a rare condition characterized by multiple blood-filled spaces within the hepatic parenchyma that can lead to fatal hemorrhage. There is no consensus on the best treatment algorithm for PH, and therapy is directed at removing the potential causative agent with operative intervention when necessary. Here we present the first known case of PH in a child with myotubular myopathy who was successfully treated with angiography and hepatic artery embolization as a first line therapy, without the need for operative intervention. Awareness of this condition and the available treatment modalities may lead to favorable outcomes in future cases.

Vascular disorders of the liver: recommendations from the Italian Association for the Study of the Liver (AISF) ad hoc committee.

This review summarizes the document elaborated by the Italian Association for the Study of the Liver (AISF) ad hoc committee "Vascular disorders of the liver" on the primary circulatory liver diseases, which include Budd-Chiari syndrome, obstruction of the hepatic portion of the inferior vena cava, portal vein thrombosis, sinusoidal obstruction syndrome (veno-occlusive disease) and hereditary hemorrhagic telangiectasia. A characteristic of the primary circulatory liver diseases is that portal hypertension usually precedes liver dysfunction. Significant overlap exists amongst the diseases and risk factors that predispose patients to the primary circulatory liver diseases, though the pathogenesis of individual diseases varies. Management of the different vascular disorders is very peculiar and often multidisciplinary and patients should be referred to a tertiary referral centre for optimal care.

Peliosis hepatis during postpartum period: successful embolization of hepatic artery.

Peliosis hepatis is a rare disorder characterized by the presence of blood-filled spaces in the liver, and it usually has a chronic presentation pattern. It has been reported mainly in adult patients in association with various pharmacological agents and infections. The present report concerns a postpartum patient in whom peliosis hepatis initially presented as active intraperitoneal hemorrhage from peliotic liver lesions, with no obvious etiology. We report here a 31-year-old woman who developed symptomatic peliosis hepatis and underwent superselective hepatic artery embolization, with control of the bleeding. We also present the sonographic, computed tomographic, and magnetic resonance images and laparoscopic findings. The patient recovered well and was discharged without any complaints. The pathogenesis in this patient remains unclear, but it is suggested that in her case estrogens and progesterone could not have been responsible for the development of peliosis hepatis.

Vascular liver diseases.

This article reviews the primary circulatory liver diseases, which include Budd-Chiari syndrome, obstruction of the hepatic portion of the inferior vena cava, portal vein thrombosis, sinusoidal obstruction syndrome (veno-occlusive disease), nodular regenerative hyperplasia, and peliosis hepatis. In addition, two systemic cardiovascular diseases that impair hepatic circulation, ischemic hepatitis and congestive hepatopathy, are briefly discussed. A characteristic of the primary circulatory liver diseases is that portal hypertension usually precedes liver dysfunction; however, this is not the case with the primary parenchymal liver diseases, in which liver dysfunction always progresses before portal hypertension is manifested. Significant overlap exists among the diseases and risk factors that predispose patients to the primary circulatory liver diseases, though the pathogenesis of individual diseases varies.

Hepatic circulatory diseases associated with chronic myeloid disorders.

These liver diseases are diseases of the hepatic circulation. Myeloproliferative disorders are among the most common prothrombotic disorders that lead to Budd-Chiari syndrome and PVT. SOS, previously known as hepatic veno-occlusive disease, is mainly seen in North America and Western Europe as a complication of the conditioning regimen for hematopoietic stem cell transplantation. SOS is caused by damage to SECs, and the initiating circulatory blockage occurs because of the embolism of sinusoidal lining cells. Myeloproliferative disorders are an uncommon cause of NRH, which is believed to be caused by uneven perfusion of the liver at the venous or sinusoidal level. Peliosis hepatis is believed to result from damage to SECs and is seen mainly in immunosuppressed patients, patients with a wasting illness, or patients with a drug toxicity.

Peliosis hepatis with intrahepatic hemorrhage: successful embolization of the hepatic artery.

Peliosis hepatis is defined as the appearance of blood filled lakes in the hepatic parenchyma. It has been associated with various pharmacological agents and infections. Treatment has been primarily symptomatic and includes discontinuation of offending medications, partial hepatectomy or occasionally liver transplantation. We report a 58 year old white female on hormone replacement therapy who developed symptomatic peliosis hepatis and underwent successful superselective hepatic artery embolization with control of bleeding.

Bacillary peliosis hepatis as a cause of acute anemia in a patient with the acquired immunodeficiency syndrome.

A 33-year-old white homosexual man, infected with the human immunodeficiency virus, presented with acute anemia and thrombocytopenia not responsive to transfusions or to treatment with steroids and intravenous gamma-globulin. Hematologic workup was compatible with peripheral sequestration or loss of blood cells; however, there was no evidence of gastrointestinal or other sources of hemorrhage, and the only significant finding was a progressive liver enlargement. An abdominal computerized tomographic scan showed a massive homogeneous liver without focal lesions, a very small amount of ascites, and no retroperitoneal fluid collections. A transjugular liver biopsy specimen showed the cystic, blood-filled cavities characteristic of peliosis hepatis. Cavities varied in size, all contained pooled erythrocytes, and some had areas suggestive of thrombi in various stages of organization. Bacteria similar in morphology to those described in bacillary peliosis hepatis were seen in the peliotic spaces. The clinical picture began resolving shortly after treatment with zidovudine and ampicillin/sulbactam was started and had totally resolved 6 months after presentation. This case shows that bacillary peliosis hepatis is a reversible entity that may produce acute sequestration of blood in the liver.