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Congenital liver fibrosis, a mesenchymal embryopathy
294
Biliary
ABSTRACTS
Atresio:
Diagnosis,
New
Concepts
and Management.
of
Pathology,
Alexander
H. Bill,
William S. Brennom, and Thomas L. Huseby. Arch. Surg. 109:367-369 (September), 1974. Morio Kasai first performed his operation for biliary atresia in 1957. In 1972, in a series collected from five centers in Japan, 42”< of 1 I6 cases operated upon before the age of 3 mo were jaundice free a year or more postoperatively. The authors reviewed the Kasai technique and added eight cases of their own. Each of these had surgery of the “Kasai type” before the age of 3 mo. Four are over 1 yr postoperative without jaundice. Four are less than a year, and three of these “have shown bile flow.” All eight have big livers. Cases referred after previous unsuccessful surgery have not done well. It is concluded that the patient should be operated upon before the age of 3 mo. If the size of the ducts in the porta hepatis is less than 200 p, cholangitis has probably extended into the ducts in the liver, and bile excretion is unlikely. The occurrence of ascending cholangitis after the establishment of good bile flow may prove fatal. Antibiotics must be used quickly with unexplained fever. The technique of the Kasai operation is given. Diagnosis of biliary atresia is based upon (I) stool color (clay or light tan with only a trace of yellow), (2) punch liver biopsy showing in addition to hepatitis proliferation of end ductules and bile plugs. (3) Rose bengal sodium ‘131 scan showing over 3 or 4 days liver retention and no flow into the intestine, (4) total bilirubin level showing a flat or rising curve over several weeks rather than at first a rise then improvement. Biliary atresia and neonatal hepatitis are probably different manifestations of the same disease process.-Edward J. Berman Biliary
Atresia:
The Current
State of Confusion.
D. M. Hays. Surg. Clin. North Am. 53:1257-1273
(October), 1973. This review of the current management of biliary atresia contains an analysis of the results of hepatobiliaryenterostomies performed for this condition in five Japanese pediatric surgical centers since 1967. It is estimated that 100 infants are subjected to such procedures annually. The original procedure described by Kasai has been modified in five other centers, largely in relation to the form of the intestinal loop employed in drainage of the porta hepatis.
These procedures provide specimens which should make major contributions to the understanding of the basic pathology of biliary atresia.--S. L. Guns Congenital
liver
Embryopathy.
Fibrosis,
a
Mesenchymol
F. Moyson. Ann. Chir. Infant. 15:39-
46 (January-February),
1974.
Liver fibrosis is a rare disease which is familial in about 55% of cases. In a high percentage the kidneys are also affected. The author reports the case of a girl who presented with hepatomegaly. Congenital liver fibrosis was found as well as sponge kidneys and some dilatation of excretory ducts of the pancreas. Because of portal hypertension a splenorenal shunt was effected. Later on the patient developed Scheuermanns disease and bilateral epiphysiolysis capitis femoris. Studies of histogenesis of all the affected tissues lead to the conclusion that the underlying disease is a mesenchymal embryopathy.-G. Eg(i Epidermoid
Cysts of the Spleen.
J. R. Campbell. Pediatrics 51:75-84
E. Slunk and (January), 1973.
Whenever splenomegaly in children is present the differential diagnosis must include epidermoid cysts of the spleen. Characteristically the child appears asymptomatic, has no history of apparent parasitic infestation, and the x-ray reveals a mass in the left upper quadrant of the abdomen displacing the gut and left kidney. A review of the literature is included and four cases are presented by the authors.-CG#&d Ruhin Spontaneous M. Bondeiro
Rupture of Accessory Spleen. Texeira and William J. Hard;,,. A,,,.
Surg. 40:491-493
(August), 1974.
An I I-yr-old girl presented with abdominal pain, nausea, and vomiting. Horseback riding the previous day was not associated with trauma. After 5 days of treatment with intravenous fluids and antibiotics, she was admitted to the Scott and White Clinic where a mass in the left upper quadrant was palpated. An accessory spleen presented as a large cystic mass at operation. Removal was accomplished and the postoperative course was uneventful.-Edw’urd J. Berman Primary
Roundworm
36:200-201
Peritonitis.
end R. A. Vormo. (May), 1974.
S. V. Nodkorni,
S. K. Porashar,
Indian J. Surg.
Biliary
ABSTRACTS
Atresio:
Diagnosis,
New
Concepts
and Management.
of
Pathology,
Alexander
H. Bill,
William S. Brennom, and Thomas L. Huseby. Arch. Surg. 109:367-369 (September), 1974. Morio Kasai first performed his operation for biliary atresia in 1957. In 1972, in a series collected from five centers in Japan, 42”< of 1 I6 cases operated upon before the age of 3 mo were jaundice free a year or more postoperatively. The authors reviewed the Kasai technique and added eight cases of their own. Each of these had surgery of the “Kasai type” before the age of 3 mo. Four are over 1 yr postoperative without jaundice. Four are less than a year, and three of these “have shown bile flow.” All eight have big livers. Cases referred after previous unsuccessful surgery have not done well. It is concluded that the patient should be operated upon before the age of 3 mo. If the size of the ducts in the porta hepatis is less than 200 p, cholangitis has probably extended into the ducts in the liver, and bile excretion is unlikely. The occurrence of ascending cholangitis after the establishment of good bile flow may prove fatal. Antibiotics must be used quickly with unexplained fever. The technique of the Kasai operation is given. Diagnosis of biliary atresia is based upon (I) stool color (clay or light tan with only a trace of yellow), (2) punch liver biopsy showing in addition to hepatitis proliferation of end ductules and bile plugs. (3) Rose bengal sodium ‘131 scan showing over 3 or 4 days liver retention and no flow into the intestine, (4) total bilirubin level showing a flat or rising curve over several weeks rather than at first a rise then improvement. Biliary atresia and neonatal hepatitis are probably different manifestations of the same disease process.-Edward J. Berman Biliary
Atresia:
The Current
State of Confusion.
D. M. Hays. Surg. Clin. North Am. 53:1257-1273
(October), 1973. This review of the current management of biliary atresia contains an analysis of the results of hepatobiliaryenterostomies performed for this condition in five Japanese pediatric surgical centers since 1967. It is estimated that 100 infants are subjected to such procedures annually. The original procedure described by Kasai has been modified in five other centers, largely in relation to the form of the intestinal loop employed in drainage of the porta hepatis.
These procedures provide specimens which should make major contributions to the understanding of the basic pathology of biliary atresia.--S. L. Guns Congenital
liver
Embryopathy.
Fibrosis,
a
Mesenchymol
F. Moyson. Ann. Chir. Infant. 15:39-
46 (January-February),
1974.
Liver fibrosis is a rare disease which is familial in about 55% of cases. In a high percentage the kidneys are also affected. The author reports the case of a girl who presented with hepatomegaly. Congenital liver fibrosis was found as well as sponge kidneys and some dilatation of excretory ducts of the pancreas. Because of portal hypertension a splenorenal shunt was effected. Later on the patient developed Scheuermanns disease and bilateral epiphysiolysis capitis femoris. Studies of histogenesis of all the affected tissues lead to the conclusion that the underlying disease is a mesenchymal embryopathy.-G. Eg(i Epidermoid
Cysts of the Spleen.
J. R. Campbell. Pediatrics 51:75-84
E. Slunk and (January), 1973.
Whenever splenomegaly in children is present the differential diagnosis must include epidermoid cysts of the spleen. Characteristically the child appears asymptomatic, has no history of apparent parasitic infestation, and the x-ray reveals a mass in the left upper quadrant of the abdomen displacing the gut and left kidney. A review of the literature is included and four cases are presented by the authors.-CG#&d Ruhin Spontaneous M. Bondeiro
Rupture of Accessory Spleen. Texeira and William J. Hard;,,. A,,,.
Surg. 40:491-493
(August), 1974.
An I I-yr-old girl presented with abdominal pain, nausea, and vomiting. Horseback riding the previous day was not associated with trauma. After 5 days of treatment with intravenous fluids and antibiotics, she was admitted to the Scott and White Clinic where a mass in the left upper quadrant was palpated. An accessory spleen presented as a large cystic mass at operation. Removal was accomplished and the postoperative course was uneventful.-Edw’urd J. Berman Primary
Roundworm
36:200-201
Peritonitis.
end R. A. Vormo. (May), 1974.
S. V. Nodkorni,
S. K. Porashar,
Indian J. Surg.