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

The clinical findings in a case with a diffuse multinodular type of angioma of the liver are reported. The clinical picture was typical: the appearance after the first week of a severe congestive failure mimicking a congenital heart disorder, of the left to right shunt type, with gross hepatomegaly of the "vascular" type with a systolo-diastolic murmur, but with no cutaneous evidence of angioma. The very severe prognosis during the first months of life of patients with this malformation, as illustrated by the 58 deaths out of the 78 cases reported in the literature, indicated ligature of the hepatic artery before the age of one month. The satisfactory outcome (with a nine months follow-up) for this case, as for the three other operated cases in the literature, leads us to conclude that this operation is indicated after a detailed arteriographic examination has been made, and provided that the other anatomical conditions are favourable.
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PMID:[Multinodular hepatic angioma: a rare cause of neonatal asystole. A case and review of the literature]. 41 77

Seven patients with hemangiomatosis of the liver are presented together with 100 cases reported in the literature. Six of seven patients survived; four with supportive treatment only. Follow-up is from 2 to 11 yr. Hepatomegaly, heart failure, and cutaneous angiomas constitute the classical clinical triad. Arteriography is the best method for diagnosis; biopsy should be avoided. Clinical features and histologic examination of these benign vascular tumors seem to confirm the theory that hemangioendothelioma and hemangioma represent only different developmental stages of one disease. These lesions can regress spontaneously if left alone. Despite contrary prevailing opinion, the authors recommend nonoperative treatment as the first choice of the clinician.
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PMID:Hemangiomatosis of the liver in infants. 111 83

The authors present 7 patients with hemangiomatosis of the liver together with about 100 cases reported in the literature. 6 of 7 patients survived; 4 with supportive treatment only. Follow-up is from 2 to 12 years. Hepatomegaly, heart failure and cutaneous angiomas constitute the classical clinical triad. Arteriography is the best method to diagnose this lesion, and biopsy can thus be avoided. Clinical features and histological examination of these benign vascular tumors seem to confirm the theory that hemangioendothelioma and hemangioma represent only two different developmental stages of one disease. These lesions can regress spontaneously if left alone. Despite contrary prevailing opinion the conservative treatment is recommended as the first choice of the clinician.
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PMID:[Hemangiomatosis of the liver in infants: Diagnosis and treatment]. 115 Apr 80

A 29 year old Chinese female who presented with spontaneous purpura, was found to have gross hepatomegaly and thrombocytopenia. The thrombocytopenia responded to steroid therapy but relapsed when the dose of steroid was tapered down. Subsequent investigations revealed that the hepatomegaly was due to a large haemangioma of her liver. For symptomatic hepatic haemangioma, surgical excision is the treatment of choice; this was refused by the patient.
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PMID:Haemangioma-thrombocytopenia syndrome--a case report. 262 43

Studies are presented on a 62-yr-old woman with extreme hepatomegaly due to a giant hemangioma with alterations in the clotting system indicating a consumption coagulopathy. There was a fall of hemoglobin, fibrinogen, antithrombin III, and platelet number after arteriography of the truncus celiacus. Furthermore, there was sustained bleeding in the patient's right thigh caused by puncture of the arteria femoralis. Continuous administration of iv heparin corrected the clotting disorder including a rise in platelets from 95,000/microliters to 148,000/microliters, permitting surgical removement of the hematoma. Celiacography is a useful tool for the diagnosis of hepatic hemangiomata, as well as ultrasound, computed tomography, and magnetic resonance imaging. Being an invasive technique, it requires testing for possible consumption coagulopathy if used in patients with hemangiomatosis. Its application should be restricted to cases in which exact diagnosis cannot be established by other means.
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PMID:The Kasabach-Merritt syndrome: severe bleeding disorder caused by celiac arteriography--reversal by heparin treatment. 266 39

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 liver in an infant or child is as liable to the same pathologies afflicting the adult liver but with certain differences in prevalence and causes. Genetic disorders are more likely to present in the paediatric age group where many involve metabolic processes such as galactosemia, phenylketonuria, glycogen storage disease and others. Many of these present in the newborn period. However, neoplasms and hamartomas also present in the newborn period, such as congenital neuroblastoma with an enormously enlarged liver, hepatoblastoma and haemangioma. The latter may present with intractable cardiac failure as a result of considerable shunting of blood. Acquired liver lesions often present in the newborn period or early infancy and this includes hepatitis and biliary atresia. The difficulties in the differentiation of the two lesions will be discussed together with the management of biliary atresia. As the child grows older, Reyes encephalopathy with microvesicular fat in the liver is not uncommon. The pathophysiology of Reyes encephalopathy as seen locally will be described. The choledochal cyst with direct (Caroli's disease) or indirect effect on the liver will be described. Problems of childhood portal hypertension as well as congenital hepatic fibrosis will be described. Hemosiderosis of the liver is chiefly seen in homozygous beta-thalassaemia patients who have been kept alive with repeated blood transfusions. Amoebic and pyogenic hepatitis, fatty liver due to protein malnutrition, biliary ascariasis, etc, which are common in tropical and subtropical countries are rarely seen now in Singapore children.
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PMID:Paediatric liver disorders in Singapore. 346 38

Giant hepatic cavernous hemangiomas are clinically distinct from smaller asymptomatic ones and may be confused with primary or metastatic malignancy. Hemangiomas exceeding 8 cm in diameter (mean, 13.7 cm) were studied in eight women 28-71 years old, six of whom presented with right upper quadrant discomfort and/or hepatomegaly. On unenhanced CT, seven of the masses were less dense than surrounding normal liver and one was slightly more dense than adjacent fatty liver. All masses contained additional stellate or cleftlike low-density zones, and two contained calcification. After bolus IV contrast administration, all exhibited early peripheral enhancement and partial centripetal isodense fill-in. None became completely isodense on delayed scans. Familiarity with these CT characteristics and a high index of suspicion should facilitate correct diagnosis and avert needle biopsy.
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PMID:CT of giant cavernous hemangioma. 349 95

The vascular origin lesions of the liver (capillary hemangioma/infantile hemangioendothelioma) that present in infancy or early childhood often have a typical clinical picture of hepatomegaly and congestive heart failure. These lesions rarely present as asymptomatic hepatomegaly, simulating a primary hepatic malignancy. These lesions may also simulate a primary or secondary hepatic malignancy on cross-sectional imaging or angiography. Scintigraphic evaluations with technetium-99m-labeled red blood cells offers an accurate method of identification of these lesions, and allows differentiation from other common primary or secondary hepatic masses in infancy or childhood. This scintigraphic method may also be used to follow these patients after medical, radiation, or embolization therapy. Experience with seven patients with these tumors is reported and compared with eight children with other primary or secondary liver tumors also evaluated by this method.
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PMID:Technetium-99m-labeled red blood cells in the evaluation of hemangiomas of the liver in infants and children. 362 94

The authors have encountered benign liver masses as frequently as malignant lesions in children with hepatomegaly. Lesions studied included abscesses, cavernous hemangioma/hemangioendothelioma, adenoma of glycogen storage disease, choledochal cysts, focal nodular hyperplasia, cystic hepatoblastoma, and hamartoma. An integrated imaging protocol involving ultrasound, computed tomography, and scintigraphy proved to be more helpful than any one modality in establishing the benign or malignant nature of a hepatic neoplasm and the type of tumor, which is of particular importance when surgical exploration and/or biopsy is contraindicated.
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PMID:Integrated imaging of hepatic tumors in childhood. Part II: Benign lesions (congenital, reparative, and inflammatory). 388 Jun 15


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