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Biliary atresia-polysplenia syndrome: Surgical and clinical relevance in liver transplantation
INTERNATIONAL
1441
ABSTRACTS
tomy. Cecal closure may be tenuous; safety dictates decompressive loop ileostomy proximal to cecal closure. The ileostomy is closed several weeks later. If tbe perineal muscle complex is intact, the colon may be pulled through at birth, or later. The rudimentary microcolon is never removed and should not be used as bladder augmentation or bowel conduit because eventually it will grow to 30 to 40 cm and provide water absorption. The details of later reconstruction are presented. One hundred forty-one of the initial 154 cloaca patients were available for follow-up. Eighty-two have spontaneous bowel movements and satisfactory continence, 38 are on enema programs and are clean, and 9 have a permanent colostomy. Eighty-three patients void urine spontaneously, 40 catheterize, 4 have urinary diversions, 1 has continent diversion, and 5 have wetting (8 cases were corrected too recently to have meaningful information). Twenty-four patients are now adults; 14 are married, 17 have coitus, and 6 have had babies. The patients with exstrophy did not fair as well. Nineteen had colon pull-through, but three cases were reversed to colostomy because of incontinence and continued soiling. Fifteen have retained their original colostomy, and seven have not yet had pull-through. Four older presurgical patients are content with colostomy and diapers for urinary incontinence and have elected not to undergo reconstruction. In considering these patients for colon pull-through. attention should be made to patients with a good spine and sacrum, a gluteal cleft, and good perineal musculature. The reconstructed bladder in these patients is a nondynamic pouch. Augmentation has been required in all but two. Urinary continence was achieved in 18 by narrowing the bladder outlet (they self-catheterize to empty), and in six with a bowel nipple at the bladder outlet. Considerations of gender assignment are discussed in detail. Several illustrative cases are presented to detail management and surgical techniques. Satisfactory results are possible for these patients. with the best results achieved at institutions where surgeons have a particular interest in such reconstructive surgery--Edward G. Ford
Injuries of the Gastrointestinal Tract From Blunt Trauma in Children: A 12-Year Experience at a Designated Pediatric Trauma Center. ZG. Canty SI; ZG. Canty JI; and C. Brown. J Trauma 46:234-240, (February), 1999. The authors sought to define the impact of diagnostic “delay” in children with blunt gastrointestinal (GI) injuries. They surveyed nearly 12,000 admissions to their pediatric trauma center over 12 years and found 79 patients with blunt GI injury. The mechanisms included 15 restrained and 7 unrestrained occupants of a motor vehicle, 15 pedestrians, 15 abuse victims, and 10 handle-bar intrusions. Injury of the small bowel was most common (44), followed by the duodenum (18), colon (17), and stomach (6). The diagnosis of GI injury was made within 4 hours in 45 children (57%); 17 cases (22%) were diagnosed more than 24 hours after presentation. Six children died of head injury. The 73 survivors had nine complications, including intraabdominal abscesses requiring percutaneous drainage in 5 children and delayed intestinal obstruction requiring lysis of adhesions in two children. The complication rate was 2% for the group diagnosed within 4 hours and 23% for those with delayed diagnosis (P = ,002). The authors conclude that morbidity from GI injury correlates with delays in diagnosis but does not negate the benefits of nonoperative treatment of blunt abdominal injury in children as long as repeated clinical examinations are performed.-Steven Stylianos
ABDOMEN Biliary Atresia-Polysplenia Syndrome: Surgical and Clinical Relevance in Liver Transplantation. G. Vurelu-Fuscinetro, P. Cusmldo, I.J. FOX, et al. Ann Surg 227:583-589, (April), 1998. Biliary atresia is the most common indication in children and may be associated with other
for liver transplantation congenital anomalies in
9% to 37% of patients. The most common associated anomaly is polysplenia syndrome, which may include polysplenia, midgut malrotation, situs inversus, preduodenal portal vein, interrupted or absent infrahepatic vena cava, abnormal or atypical hepatic arterial supply, symmetric or isomeric liver, and bilobed right lung or pulmonary levoisomerism. Most of the anomalies, and their corrections, occur within the anatomic region of the liver hilum and have been cited as a reason for the initial disappointing results of liver transplantation in these children. The authors present 11 patients with polysplenia syndrome in an institutional experience of 166 liver transplantations in 130 patients with biliary atresia. Thirteen liver transplantations were performed in these 11 patients; the diagnosis of polysplenia was not established preoperatively for eight of them. Of the 11 patients, three had situs inversus and 10 had anomalous IVC (absent in 8, left-sided in 2). The authors detail many variations of the techniques of liver transplantation based on the unique anatomic changes for each patient. Nine of the eleven patients are still alive. One died on postoperative day 2 of pulmonary edema and hypoxia; one died 4 years postoperatively because of chronic rejection. No significant differences were found in outcome of liver transplantation between patients with biliary atresia and those with polysplenia syndrome associated with biliary atresia. With special surgical consideration, patients with polysplenia syndrome should have an outcome similar to that of patients with isolated biliary airesia.-Edwurd G. Ford Soluble ICAM-I (slCAM-1) ship to Disease Activity. Dillon. J Surg Res 76:53-56,
in Biliary Atresia and Its RelationK.E. Minnick. R. Kreisberg, and RW; (April), 1998.
Intracellular adhesion molecule- 1 (ICAM-1) is strongly expressed on the bile ducts and hepatic parenchyma of livers in patients with biliary atresia. A soluble form of ICAM(sICAM-1) has been found to be released into the circulation in patients with primary biliary cirrhosis. The goal of this study was to determine the presence of sICAM-1 in infants with biliary atresia and to compare its expression to standard serum tests of hepatic function and markers of disease activity. Seven patients with biliary atresia, two patients with direct hyperbilirubinemia secondary to TPN cholestasis and Alagille syndrome, and three healthy control patients were studied. Serum concentrations of sICAM-1 were markedly elevated in all patients with biliary atresia. The levels were not related severity of disease at the time of diagnosis. There was no correlation between the sICAM-1 levels in a standard serum test of hepatic function and cholestasis. Thus, measurement of sICAM-1 levels may be useful in the evaluation of neonates with direct hyperbilirubinemia by permitting, with a serum test, the identification of infants with biliary atresia.-Richard R. Ricketts Long-Term Results Gross, C.R. Shackleton, (September), 1998.
of Pediatric S.ll McDiarmid,
Liver Transplantation. LA. et al. Ann Surg 228:411-420,
This report summarizes an experience with 440 pediatric patients undergoing 569 orthotopic liver transplants (LT) from 1984 to 1997. One hundred eleven patients were less than 1 year of age, 128 were 1 to 3 years old, 156 were 3 to 12 years old, and 45 were 12 to 18 years old. Indications for transplantation included biliary atresia (222), fulminant hepatic failure (57), metabolic liver disease (56), neonatal hepatitis (16), tumor (13), Alagille’s syndrome (9), familial cholestasis (8), miscellaneous (59). and retransplantation (97). Three hundred forty-three children received single LT, 67 underwent retransplantation once, 25 required two retransplantations, and 5 underwent three retransplantations. Before 1994, immunosuppression consisted of cyclosporine, methylprednisolone, and azathioprine; since 1994, patients have been treated with tacrolimus and methylprednisolone. Patients with ABOcompatible allografts who were rejection-free and had stable cyclosporine or tacrolimus levels for 6 months were offered steroid withdrawal.
1441
ABSTRACTS
tomy. Cecal closure may be tenuous; safety dictates decompressive loop ileostomy proximal to cecal closure. The ileostomy is closed several weeks later. If tbe perineal muscle complex is intact, the colon may be pulled through at birth, or later. The rudimentary microcolon is never removed and should not be used as bladder augmentation or bowel conduit because eventually it will grow to 30 to 40 cm and provide water absorption. The details of later reconstruction are presented. One hundred forty-one of the initial 154 cloaca patients were available for follow-up. Eighty-two have spontaneous bowel movements and satisfactory continence, 38 are on enema programs and are clean, and 9 have a permanent colostomy. Eighty-three patients void urine spontaneously, 40 catheterize, 4 have urinary diversions, 1 has continent diversion, and 5 have wetting (8 cases were corrected too recently to have meaningful information). Twenty-four patients are now adults; 14 are married, 17 have coitus, and 6 have had babies. The patients with exstrophy did not fair as well. Nineteen had colon pull-through, but three cases were reversed to colostomy because of incontinence and continued soiling. Fifteen have retained their original colostomy, and seven have not yet had pull-through. Four older presurgical patients are content with colostomy and diapers for urinary incontinence and have elected not to undergo reconstruction. In considering these patients for colon pull-through. attention should be made to patients with a good spine and sacrum, a gluteal cleft, and good perineal musculature. The reconstructed bladder in these patients is a nondynamic pouch. Augmentation has been required in all but two. Urinary continence was achieved in 18 by narrowing the bladder outlet (they self-catheterize to empty), and in six with a bowel nipple at the bladder outlet. Considerations of gender assignment are discussed in detail. Several illustrative cases are presented to detail management and surgical techniques. Satisfactory results are possible for these patients. with the best results achieved at institutions where surgeons have a particular interest in such reconstructive surgery--Edward G. Ford
Injuries of the Gastrointestinal Tract From Blunt Trauma in Children: A 12-Year Experience at a Designated Pediatric Trauma Center. ZG. Canty SI; ZG. Canty JI; and C. Brown. J Trauma 46:234-240, (February), 1999. The authors sought to define the impact of diagnostic “delay” in children with blunt gastrointestinal (GI) injuries. They surveyed nearly 12,000 admissions to their pediatric trauma center over 12 years and found 79 patients with blunt GI injury. The mechanisms included 15 restrained and 7 unrestrained occupants of a motor vehicle, 15 pedestrians, 15 abuse victims, and 10 handle-bar intrusions. Injury of the small bowel was most common (44), followed by the duodenum (18), colon (17), and stomach (6). The diagnosis of GI injury was made within 4 hours in 45 children (57%); 17 cases (22%) were diagnosed more than 24 hours after presentation. Six children died of head injury. The 73 survivors had nine complications, including intraabdominal abscesses requiring percutaneous drainage in 5 children and delayed intestinal obstruction requiring lysis of adhesions in two children. The complication rate was 2% for the group diagnosed within 4 hours and 23% for those with delayed diagnosis (P = ,002). The authors conclude that morbidity from GI injury correlates with delays in diagnosis but does not negate the benefits of nonoperative treatment of blunt abdominal injury in children as long as repeated clinical examinations are performed.-Steven Stylianos
ABDOMEN Biliary Atresia-Polysplenia Syndrome: Surgical and Clinical Relevance in Liver Transplantation. G. Vurelu-Fuscinetro, P. Cusmldo, I.J. FOX, et al. Ann Surg 227:583-589, (April), 1998. Biliary atresia is the most common indication in children and may be associated with other
for liver transplantation congenital anomalies in
9% to 37% of patients. The most common associated anomaly is polysplenia syndrome, which may include polysplenia, midgut malrotation, situs inversus, preduodenal portal vein, interrupted or absent infrahepatic vena cava, abnormal or atypical hepatic arterial supply, symmetric or isomeric liver, and bilobed right lung or pulmonary levoisomerism. Most of the anomalies, and their corrections, occur within the anatomic region of the liver hilum and have been cited as a reason for the initial disappointing results of liver transplantation in these children. The authors present 11 patients with polysplenia syndrome in an institutional experience of 166 liver transplantations in 130 patients with biliary atresia. Thirteen liver transplantations were performed in these 11 patients; the diagnosis of polysplenia was not established preoperatively for eight of them. Of the 11 patients, three had situs inversus and 10 had anomalous IVC (absent in 8, left-sided in 2). The authors detail many variations of the techniques of liver transplantation based on the unique anatomic changes for each patient. Nine of the eleven patients are still alive. One died on postoperative day 2 of pulmonary edema and hypoxia; one died 4 years postoperatively because of chronic rejection. No significant differences were found in outcome of liver transplantation between patients with biliary atresia and those with polysplenia syndrome associated with biliary atresia. With special surgical consideration, patients with polysplenia syndrome should have an outcome similar to that of patients with isolated biliary airesia.-Edwurd G. Ford Soluble ICAM-I (slCAM-1) ship to Disease Activity. Dillon. J Surg Res 76:53-56,
in Biliary Atresia and Its RelationK.E. Minnick. R. Kreisberg, and RW; (April), 1998.
Intracellular adhesion molecule- 1 (ICAM-1) is strongly expressed on the bile ducts and hepatic parenchyma of livers in patients with biliary atresia. A soluble form of ICAM(sICAM-1) has been found to be released into the circulation in patients with primary biliary cirrhosis. The goal of this study was to determine the presence of sICAM-1 in infants with biliary atresia and to compare its expression to standard serum tests of hepatic function and markers of disease activity. Seven patients with biliary atresia, two patients with direct hyperbilirubinemia secondary to TPN cholestasis and Alagille syndrome, and three healthy control patients were studied. Serum concentrations of sICAM-1 were markedly elevated in all patients with biliary atresia. The levels were not related severity of disease at the time of diagnosis. There was no correlation between the sICAM-1 levels in a standard serum test of hepatic function and cholestasis. Thus, measurement of sICAM-1 levels may be useful in the evaluation of neonates with direct hyperbilirubinemia by permitting, with a serum test, the identification of infants with biliary atresia.-Richard R. Ricketts Long-Term Results Gross, C.R. Shackleton, (September), 1998.
of Pediatric S.ll McDiarmid,
Liver Transplantation. LA. et al. Ann Surg 228:411-420,
This report summarizes an experience with 440 pediatric patients undergoing 569 orthotopic liver transplants (LT) from 1984 to 1997. One hundred eleven patients were less than 1 year of age, 128 were 1 to 3 years old, 156 were 3 to 12 years old, and 45 were 12 to 18 years old. Indications for transplantation included biliary atresia (222), fulminant hepatic failure (57), metabolic liver disease (56), neonatal hepatitis (16), tumor (13), Alagille’s syndrome (9), familial cholestasis (8), miscellaneous (59). and retransplantation (97). Three hundred forty-three children received single LT, 67 underwent retransplantation once, 25 required two retransplantations, and 5 underwent three retransplantations. Before 1994, immunosuppression consisted of cyclosporine, methylprednisolone, and azathioprine; since 1994, patients have been treated with tacrolimus and methylprednisolone. Patients with ABOcompatible allografts who were rejection-free and had stable cyclosporine or tacrolimus levels for 6 months were offered steroid withdrawal.