A frequent symptom of this congenital defect which demands especial attention, is limmorrhage from the navel. This phenomenon is not a con stant symptom, but occurs in the majority of cases, and is of very serious augury. The haemorrhage generally begins a few hours or a clay or two after the fall of the navel-string (most commonly between the fifth and the ninth clay after birth), and usually occurs first in the night. It is not a violent bleeding. Blood oozes gently but continuously from the umbilicus. It appears to be capillary, and the colour may be bright red, or dark and venous. This form of bleeding may be combined with from other parts, such as cutaneous ecchymoses, epistaxis, hatmatemesis or mehena, and bleeding from the mouth. The combined with the interference with digestion due to the absence of bile and impaired action of the liver, rapidly exhausts the patient ; and he usually dies with in the week—often in a few hours. Dr. Legg suggests that the umbilical haemorrhage is a consequence of the cirrhosis and resulting portal conges tion ; for the blood is hindered in its passage through the liver, and is forced to seek some other way of escape. It therefore passes from the left portal vein to the ductus venosus, and thence to the umbilicus, where the vessels, newly closed, cannot resist the increased pressure, and give way. The same mechanism (portal congestion) will explain the frequent coincidence of haemorrhage from other parts supplying the portal vein with blood.
Cases of jaundice conjoined with umbilical hmnorrhage are rapidly fatal, When this symptom is absent, although the child almost invariably dies, life may be preserved for a much longer period. Recorded cases show that the infant may live five, six, or seven months, and even then, as in Lotze's case, where the child lived into the beginning of the eighth month and died of a bronchopneumonia, may succumb to an accidental compli cation. This malfonuation is said to be twice as common in boys as it is in girls.
A male infant, deeply jaundiced, aged three months, was brought to the out-patients' room of the East London Children's Hospital and was at once admitted by my colleague, Dr. Radcliffe Crocker, into the wards. The child was born of healthy parents, none of whose other children had been similarly afflicted. He was said to have been a robust, healthy looking infant at birth, and shortly afterwards to have passed two dark stools. Since that time, however, his motions had been hard and white, like lumps of chalk, and the bowels had acted only once a clay. The jaun dice had first appeared when the child was a week old, and had progressively increased. The infant had been suckled for a month, and was then fed on Swiss milk. He often vomited, not always after taking food, and was capricious about his bottle, sometimes refusing to suck. His water had always been dark, leaving yellow stains on the diaper.
When admitted, the child was fairly nourished. His skin was deeply jaundiced, and his conjunctivae were yellow. There was a papular eruption (strophulus) all over his body. The liver could not be felt at this time on account of the child's struggles, but was found a few days afterwards to project two fingers' breadths below the ribs. The boy lived a month after his admission, wasting gradually, and often crying as if in pain. Then aphthEe appeared in his mouth, and he sank anedied. There were no haemorrhages. His jaundice persisted, although it varied curiously in in tensity ; and before his death the tint of the skin was several shades lighter than when he entered the hospital. The liver remained about the same
size and felt firm and smooth. The spleen was not enlarged. After death the liver was found of a dark olive colour, and its consistence seemed to be increased. The gall-bladder was rudimentary, and the hepatic and common ducts were absent.
When syphilitic inflammation of the liver gives rise to jaundice, the or gan is enlarged and deeply coloured of a brownish yellow tint, and shows under the microscope a great proliferation of young cells in the capsule of Glisson, and in the interlobular spaces. In a case recorded by M. D'Espine, of Geneva, the same proliferation was noted round the hepatic cells in the interior of the lobules. Moreover, the small bile-duets were thickened and filled with epithelial cells. There was no obstruction in the larger ducts, and the gall-bladder contained thick and dark-coloured bile. The spleen was greatly enlarged and very firm.
In this case the jaundice was severe and appeared at birth. On the ninth day bleeding occurred from the umbilicus, from the bowels, and into the skin ; the belly swelled ; the liver and spleen were notably enlarged ; the temperature became subnormal ; the child wasted rapidly, and died on the twenty-third day in convulsions.
Jaundice from umbilical phlebitis has been called by Schaller "icterus malignus." This variety appears to be dependent upon an infective pro cess. The poisonous matter is probably the same as that which causes puerperal fever in the mother, and may be conveyed by bacteria, for two forms of micro-organisms have been found in the blood of infants so affected, the one spherical and the other rod-shaped. Whether these two different forms imply two different kinds of infection is not known, but Birch-Hirschfeld asserts that the rod-shaped bacteria are especially observed in cases where the general infection is severe and the disease violent from the first, with a strong tendency to haemorrhage. These cases are accompanied by inflammation of the umbilical artery, with or without phlebitis of the umbilical vein. In sixty cases collected by this observer, umbilical arteritis was found in thirty-two, umbilical phlebitis in eleven, and inflammation of both vessels in three. An examination of the liver reveals profound degeneration. These changes seem to indicate that the infection must reach the liver by the umbilical vein. They may, however, be found in cases where the artery alone is notably diseased ; but there are reasons why the morbid appearances should be more con spicuous in the umbilical artery. After birth, the remnant of the umbilical vein is alternately emptied and filled again on account of the varying pressure on the hepatic vessels induced by the action of the heart and lungs. This constant flux and reflux in the vein tends to promote infec tion of the system, but is unfavourable to the local development of the morbid process. It is found in these cases that the intensity of the jaun dice bears no relation to the severity of the vascular inflammation, but that it is in direct proportion to the degree to which the pathological changes have advanced in the liver. It is probably, therefore, the consequence of the swelling of the connective tissue surrounding the portal vein and its branches in the liver, which compresses the bile-ducts.