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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of infantile hemangioendothelioma of the liver presenting with congestive heart failure and massive hepatomegaly in a two-mth old baby is described. Serum alpha-fetoprotein was elevated in this patient and clinically the lesion might not be distinguishable from other, commoner hepatic tumours in children. Histologically the tumour showed a type II pattern as defined by Dehner. The significance of classifying this tumour into two histologic types is discussed. Only 3 cases reported in the English literature were studied by electron microscopy; two of them were type I while the other was a type II lesion. In our case the endothelial cells appeared ultrastructurally less differentiated and architecturally more disorganized than in type I lesions. Like the previous case, the basal lamina showed marked thickening and reduplication. The significance of these findings is discussed.
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PMID:Infantile hemangioendothelioma of the liver: ultrastructural study of a type II case. 243 5

Multicentric hepatic hemangioendotheliomas are vascular lesions of the liver that usually present in the infant with hepatomegaly, high output congestive heart failure and cutaneous hemangiomas. The diagnosis, pathologic and physiologic conditions and treatment were discussed. Two of the patients we studied and 117 from the literature were reviewed. A total of 38 patients survived and 57 died with or without medical treatment. Thirteen patients survived and five died after ligation of the hepatic artery. Five patients survived and three died after embolization of the hepatic artery. (formula; see text) Of 119 patients with MHH, 81 (68 per cent) had congestive heart failure; 40 survived and 41 died. Of 56 patients with localized hepatic angiomas, 19 had congestive heart failure. Only two of the patients died of heart failure. Fifty localized hepatic angiomas in infancy that were treated with local resection or lobectomy were reviewed. Forty-six patients survived and four died of hemorrhage. Four patients survived and two died with or without medical treatment. Plans for management of multiple hepatic hemangioendotheliomas and localized hepatic angiomas in infancy were proposed.
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PMID:Hepatic hemangioendotheliomas in infancy. 264 92

Umbilical venous and amniotic fluid pressures were measured in 68 human pregnancies at the time that cordocentesis was performed. Normal umbilical venous pressure was unrelated to gestational age and remained within a tight range (5.3 +/- 2.3 mm Hg, mean +/- SD). Fetuses with an elevated umbilical venous pressure had disorders consistent with either hepatomegaly or congestive heart failure. Umbilical venous pressure was significantly increased before treatment in two fetuses with immune hydrops; it rapidly declined with treatment. Neither gestational age nor umbilical venous pressure was significantly different in the groups that received and did not receive pancuronium. There was a strong relationship between amniotic fluid pressure and gestational age in normal pregnancy (r = 0.54, p less than 0.0001). Women with hydramnios had amniotic fluid pressures greater than control subjects (p = 0.0007). This investigation documents normal human amniotic fluid and fetal umbilical venous pressures. These measurements are altered by disease and may prove to be of diagnostic and therapeutic value in the future.
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PMID:Normal values for human umbilical venous and amniotic fluid pressures and their alteration by fetal disease. 267 2

A distinct syndrome in infantile hemangioendothelioma consist of high-output congestive heart failure, hepatomegaly and cutaneous hemangiomas. Early therapy aimed at reduction of the shunt is essential if life is to be saved once cardiac failure becomes manifest. We report a case of infantile hemangioendothelioma with massive A-V and P-V shunt successfully treated by transcatheter embolization.
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PMID:[Therapeutic embolization of infantile hemangioendothelioma of the liver]. 268 11

Passive liver congestion secondary to increased hepatic venous pressure may accompany congestive heart failure. Abnormal patterns of hepatic parenchymal contrast medium enhancement in 25 patients with advanced congestive heart failure who were studied with computed tomography (CT) include a lobulated, patchy, inhomogeneous pattern in all 25 patients, an irregular perivascular enhancement in 14, and a global delay in parenchymal enhancement in nine. CT examinations showed cardiomegaly in the 20 patients with cardiac failure and pericardial effusion or thickening in the five patients with pericardial disease. Also noted were distention of the inferior vena cava (IVC) in 24 patients, hepatomegaly in 23, early reflux of contrast medium into the IVC in 21 and hepatic veins in 16, and hepatic perivascular lymph-edema in six. The abnormal patterns are thought to be due to slowing of hepatic blood flow. Confusion with Budd-Chiari syndrome and other forms of multifocal hepatic disease is avoidable with clinical and radiologic correlation.
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PMID:Inhomogeneous enhancement of liver parenchyma secondary to passive congestion: contrast-enhanced CT. 291 31

In this prospective study of 240 black patients with liver enlargement admitted to the medical wards of King Edward VIII Hospital, Durban, a cause for the hepatomegaly was found in 92.5% of cases (63.8% without recourse to biopsy, 28.7% after liver biopsy). The commonest cause was congestive heart failure (36.7%), followed by amoebic liver abscess (7.1%), hepatocellular carcinoma (5.8%) and cirrhosis (5.4%). Liver biopsy provided the diagnosis in 90.8% of patients with initial unexplained hepatomegaly. The diagnostic yield of liver biopsy was increased by submitting 3 biopsy specimens for histological examination. The 3 specimens are obtained using a single intercostal entry site and redirecting the biopsy needle, without increasing the risk of complications. Hepatic tuberculosis was present in 9.2% of patients who underwent biopsy. There were no consistent clinical findings in these patients. Therefore, in communities in which tuberculosis is endemic, all patients with unexplained hepatomegaly require liver biopsy since it provides the only means of making this diagnosis.
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PMID:Causes of hepatomegaly at King Edward VIII Hospital, Durban. A prospective study of 240 black patients. 300 36

This report describes our experience with 16 infants and children with a mean age of 7 months and a median age of 6 weeks with hepatic hemangioendothelioma. Fifteen patients presented with hepatomegaly. Seven had congestive heart failure and four had associated cutaneous lesions. Although diagnosis was clinically evident in 15 of the 16 patients, arteriography and computerized tomography (CT) were diagnostic. A variety of treatment approaches were used including radiation, resection, systemic steroids, hepatic artery ligation, angiographic embolization, and various combinations of these modalities. The following information was gained from this experience. CT with enhancement is as specific a diagnostic tool as hepatic arteriography. Angiographic and CT appearances do not correlate with prognosis. Most patients can be treated successfully with steroids; those who do not respond should have other approaches tried such as embolization or ligation. The angiographic appearance determines whether embolization therapy is worthwhile, as we found that hemangioendotheliomatosis with portal as well as hepatic arterial supply will not respond to embolization. The survival rate in this series was 80%.
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PMID:Experience with hepatic hemangioendothelioma in infancy and childhood. 320 67

Hepatic hemangioma (hemangioendothelioma) is a benign vascular tumor with significant morbidity and mortality. Two newborn infants underwent partial hepatectomy for treatment of massive hepatic hemangioma, one with atrial septal defect and secondary congestive heart failure, and the other with symptomatic marked hepatomegaly. Cardiopulmonary bypass with induced deep hypothermic circulatory arrest was used in both patients in order to facilitate resection and prevent sudden massive blood loss. One infant also had closure of an atrial septal defect in addition to the partial hepatectomy. Both infants survived without complication and showed complete resolution of preoperative symptoms. This technique coupled with the use of fibrin glue is of value in infants with large hepatic hemangiomata and cardiac decompensation in the neonatal period.
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PMID:Hepatic hemangioma: resection using hypothermic circulatory arrest in the newborn. 323 62

The CT appearance of dilated hepatic lymphatics in liver transplant recipients has been described recently. We have observed similar findings of presumed hepatic lymphatic dilatation and perivascular lymphedema in 20 patients seen over an 8-month period, none of whom had received a liver transplant. Review of these patients' records showed that 18 (90%) of 20 had underlying disease processes that could have caused dilated lymphatics as a result of either increased lymph flow or lymphatic obstruction. We have grouped these processes into three etiologic categories: (1) posttraumatic hepatic lymphatic obstruction, (2) obstruction caused by malignant lymphadenopathy, and (3) congestive hepatomegaly caused by cardiac decompensation. The appearance of hepatic lymphatic dilatation and surrounding lymphedema needs to be recognized so that lymphatic dilatation is not mistaken for dilated intrahepatic bile ducts.
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PMID:Hepatic perivascular lymphedema: CT appearance. 325 6

Amyloidosis is classified according to the distribution pattern of amyloid deposition sites and associated diseases. Hepatic amyloidosis is not infrequent, although rarely causes clinical liver disease. We report two cases of amyloidosis diagnosed by liver biopsy. One presented with symptoms related almost to the liver disease, such as jaundice, hepatomegaly and indigestion. Echocardiogram revealed hypertrophic cardiomyopathy, suggesting cardiac involvement of the amyloidosis. The patient died of hepatic failure. The other case was found in a patient with an end stage renal disease. Features of congestive heart failure in this case may reflect cardiac involvement. The pattern of hepatic amyloid deposition in both of these cases was diffuse perisinusoidal. The predominant intralobular deposition suggests that these are amyloidosis of the secondary type.
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PMID:Hepatic amyloidosis--two cases report. 326 63


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