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Splenectomy in congenital microspherocytosis
ABSTRACTS
though the child had enjoyed an accelerated growth rate. In this case a successful result was obtained. In the second case a portacaval transposition was performed on a seven-year-old boy. This child died approximately 24 hours after surgery because the liver was unable to transmit the augmented portal venous inflow from the inferior vena cava. Reference was made to a case reported by other authors in which an Eck fistula was established rather than a portacaval transposition with a successful result. It may be that this procedure which is less difficult and less risky than portacaval transposition will satisfy the objective of making ingested glucose more readily available to the peripheral tissues, and therefore become a useful modality in the treatment of glycogen storage disease.-Daniel T. Cloud. PORTACAVALSHUNT IN THE TREATMENT OF GLYCOGEN STORAGEDISEASE: REPORT OF A CASE. R. E. Hermann and R. D. Mercu. Surgery 6.5:499-503, (March) 1969.
An 18-month-old white female with severe Type-I glycogen storage disease (von Gierke’s disease) was treated surgically with an end-to-side portacaval shunt. Preoperatively the patient had recurrent episodes of severe hypoglycemia with convulsions, acidosis, xanthomatous skin lesions and retardation of growth and mental development. Blood sugar levels as low as 10 mg. per 100 cc. were found, There was also laboratory evidence of acidosis and hyperlipemia. Liver biopsy showed an abundant accumulation of glycogen in the cytoplasm of the hepatic cells, and tissue enzyme studies revealed that glycose-6-phosphatase was absent from the liver tissue. Liver phosphorylase was normal. On the eighth day after the shunt was performed, the xanthomas had disappeared and the serum lipid values had returned to normal. However, the postoperative course was marked by acidosis and convulsions, and the patient was left with cerebral palsy and mental retardation. One year after surgery a gain in growth and development was noted and all of the metabolic studies were normal. The benefit from the operation is thought to derive from the diversion of glucose absorbed by the alimentary tract via the sys-
91
temic circulation around the enzymatically deficient liver for direct utilization in the peripheral tissue. The use of portacaval shunt is urged in selected seriously ill children with Type-I glycogen storage disease. -Daniel
T. Cloud.
SPLENECTOMY IN CONGENITAL MKROSPHEROCYTOSIS.K. Barber and F. R. K. Martin. Brit. I. Surg. 56561-564. (Au-
gust)
1969.
This is a retrospective study of 28 patients, aged 21 months to 55 years. who had splenectomy for congenital microspherocytosis from 1947 to 1965. Four patients were asymptomatic and the condition was detected during investigation for other complaints. All had a palpable spleen. In one quarter, accessory splenic tissue was present and one third had associated pigmented gall stones. Cholecystotomy (five cases) with removal of stones were performed when the gall bladder looked normal; cholecystectomy was reserved for cases when obvious signs of chronic inflammation were present. Operation on the gall bladder was carried out at the same time as the splenectomy in seven patients; in the remaining two this was deferred because the gall bladder was inaccessible. One of these patients died with suppurative cholangitis three months after her splenectomy. This was the only death in the series. Twenty-two attended for followup studies. Only one was anemic, due to iron deficiency. whereas preoperatively all but one case had a hemoglobin of less than 75 per cent. The reticulocyte count and serum bilirubin were normal in all patients, having been elevated in every patient before operation. Following splenectomy the red cells became statistically significantly less fragile. Splenectomy was found to be a safe operation and it did no1 appear to increase the risk of infection or change the response to any illnesses that occurred in these patients--l. I.rrri. SPLENECTOMY IN CHILDHOODAND THI: HAZARD OF OVERWHELMING INFECTION. Lozh K.
Diamorzd.
(May)
Pediatrics
43 :886-X89.
1969.
The syndrome of overwhelming postsplenectomy infection (OPSI) is an entity usually due to the pneumococcus, in which the patient rapidly deteriorates from good
though the child had enjoyed an accelerated growth rate. In this case a successful result was obtained. In the second case a portacaval transposition was performed on a seven-year-old boy. This child died approximately 24 hours after surgery because the liver was unable to transmit the augmented portal venous inflow from the inferior vena cava. Reference was made to a case reported by other authors in which an Eck fistula was established rather than a portacaval transposition with a successful result. It may be that this procedure which is less difficult and less risky than portacaval transposition will satisfy the objective of making ingested glucose more readily available to the peripheral tissues, and therefore become a useful modality in the treatment of glycogen storage disease.-Daniel T. Cloud. PORTACAVALSHUNT IN THE TREATMENT OF GLYCOGEN STORAGEDISEASE: REPORT OF A CASE. R. E. Hermann and R. D. Mercu. Surgery 6.5:499-503, (March) 1969.
An 18-month-old white female with severe Type-I glycogen storage disease (von Gierke’s disease) was treated surgically with an end-to-side portacaval shunt. Preoperatively the patient had recurrent episodes of severe hypoglycemia with convulsions, acidosis, xanthomatous skin lesions and retardation of growth and mental development. Blood sugar levels as low as 10 mg. per 100 cc. were found, There was also laboratory evidence of acidosis and hyperlipemia. Liver biopsy showed an abundant accumulation of glycogen in the cytoplasm of the hepatic cells, and tissue enzyme studies revealed that glycose-6-phosphatase was absent from the liver tissue. Liver phosphorylase was normal. On the eighth day after the shunt was performed, the xanthomas had disappeared and the serum lipid values had returned to normal. However, the postoperative course was marked by acidosis and convulsions, and the patient was left with cerebral palsy and mental retardation. One year after surgery a gain in growth and development was noted and all of the metabolic studies were normal. The benefit from the operation is thought to derive from the diversion of glucose absorbed by the alimentary tract via the sys-
91
temic circulation around the enzymatically deficient liver for direct utilization in the peripheral tissue. The use of portacaval shunt is urged in selected seriously ill children with Type-I glycogen storage disease. -Daniel
T. Cloud.
SPLENECTOMY IN CONGENITAL MKROSPHEROCYTOSIS.K. Barber and F. R. K. Martin. Brit. I. Surg. 56561-564. (Au-
gust)
1969.
This is a retrospective study of 28 patients, aged 21 months to 55 years. who had splenectomy for congenital microspherocytosis from 1947 to 1965. Four patients were asymptomatic and the condition was detected during investigation for other complaints. All had a palpable spleen. In one quarter, accessory splenic tissue was present and one third had associated pigmented gall stones. Cholecystotomy (five cases) with removal of stones were performed when the gall bladder looked normal; cholecystectomy was reserved for cases when obvious signs of chronic inflammation were present. Operation on the gall bladder was carried out at the same time as the splenectomy in seven patients; in the remaining two this was deferred because the gall bladder was inaccessible. One of these patients died with suppurative cholangitis three months after her splenectomy. This was the only death in the series. Twenty-two attended for followup studies. Only one was anemic, due to iron deficiency. whereas preoperatively all but one case had a hemoglobin of less than 75 per cent. The reticulocyte count and serum bilirubin were normal in all patients, having been elevated in every patient before operation. Following splenectomy the red cells became statistically significantly less fragile. Splenectomy was found to be a safe operation and it did no1 appear to increase the risk of infection or change the response to any illnesses that occurred in these patients--l. I.rrri. SPLENECTOMY IN CHILDHOODAND THI: HAZARD OF OVERWHELMING INFECTION. Lozh K.
Diamorzd.
(May)
Pediatrics
43 :886-X89.
1969.
The syndrome of overwhelming postsplenectomy infection (OPSI) is an entity usually due to the pneumococcus, in which the patient rapidly deteriorates from good