Hemangio-Endothelioma of the Liver in Infancy

Abstract

The rarity of hemangio-endothelioma of the liver in infancy, as evidenced by the report of only fourteen cases in the literature and the difficulties encountered in establishing an accurate clinical diagnosis, has suggested the advisability of reviewing the histories and clinical findings in the previously reported cases. Certain features will be emphasized which should bring to mind the possible presence of this condition. REPORT OF CASEHistory.\p=m-\ShirleyE., a white female infant, aged 5 months, entered the hospital on Nov. 17, 1931. She was the first child, born spontaneously, weighing 8 pounds, 3 ounces (3,713.79 Gm.). Her early development appeared normal; there were no previous illnesses, and the family history was irrelevant. Except for extreme paleness noticed a few days after birth, she was apparently normal until 6 weeks of age, when the abdominal binder was removed permanently and the abdomen was observed to be very large. The abdomen had grown progressively larger since that time. At 4 months of age, the mother observed that the baby breathed with difficulty and became blue when lying down. The blueness and rapid respiration disappeared within from ten to fifteen minutes when the baby was placed in a sitting position. At that time a physician diagnosed a large spleen and anemia. A renal tumor was considered also, and a retrograde pyelography was performed by a urologist. No abnormalities were observed. The child was put on a strenuous anti-anemic regimen of raw liver, inorganic iron, arsenic and copper, and the anemia was said to have improved during the month before admission. Two weeks previous to admission, the baby began to refuse most of her feedings and would retain only small quantities of food, vomiting frequently. At the same time, her bowels became constipated and enemas were resorted to. Physical Examination.-Examination revealed a pale, white female infant whose respirations were rapid and shallow but who did not appear acutely ill. The temperature was 99,8 F. rectally; the pulse rate 120, and respirations 30. The examination of the ears, nose and throat did not reveal any abnormalities.The costovertebral angle was markedly obtuse. The circumference of the chest was 16J/2 inches (41.9 cm.) as compared with an abdominal circumference of 20-^2 inches (52.1 cm.). The lungs and heart were normal. The abdomen was

Haemangioendothelioma of the Liver Simulating Congenital Heart Disease in an Infant

“…It is interesting to note that in our case right-sided failure predominated, associated with cyanosis and venous engorgement. This has been noted in cases previously described, but usually other clinical features, such as cutaneous lesions, were present together with some of the clinical features described by Kunstadter (1933). Andries and Kaump (1944) classify haemangioma of the liver as (1) benign (large or small, single or multiple) or (2) malignant.…”

“…Similarly haemangioendothelioma of various organs, yet without liver involvement, has also been described (Wollstein, 1931). Kunstadter (1933) described a case of haemangioendothelioma of the liver in a child of 5 months, who had been noted to have an enlarged abdomen and pallor from the age of 6 weeks, and developed dyspnoea and cyanosis at the age of 4 months when lying down. Vomiting and constipation followed, and the infant died after laparotomy.…”

Hemangioma of the liver in children: Proliferating vascular tumor or congenital vascular malformation?

“…They are usually present in infants less than 6 months of age [1], and are frequently associated with high output cardiac failure due to arteriovenous shunts located within the tumor. HVT have been reported to be associated with anemia [2], consumptive coagulopathy [3], Kasabach-Merritt syndrome [4], tumor rupture and hemorrhage [5], jaundice [6], and hemangiomas of skin and other organs [7][8][9]. Histologically, HVT are derived from mesenchymal tissue elements and are classified as hemangioendothelioma (HE) or cavernous hemangioma (HC; [10,11]).…”

Developmental intrahepatic shunts of childhood: radiological features and management

“…Kunstadter outlined the typical features of infantile haemangioendotheliomata [10], i. e. cardiac failure due to arteriovenous shunting, cutaneous haemangiomata and hepatomegaly. The characteristic imaging features with US, CT, MRI and angiography have also been well described [7,11] obviating the need for preoperative biopsy to confirm the diagnosis.…”