Background/Methods: A 32- year retrospective review from 1972 to 2004 analyzed complications and long-term outcomes in children with total colonic aganglionosis (TCA) as they relate to the procedure performed. Results...Background/Methods: A 32- year retrospective review from 1972 to 2004 analyzed complications and long-term outcomes in children with total colonic aganglionosis (TCA) as they relate to the procedure performed. Results: Thirty-six patients (27 boys, 9 girls) had TCA. The level of aganglionosis was distal ileum (26), mid-small bowel (8), midjejunum (1), and entire bowel (1). Enterostomy was performed in 35 of 36. Eight developed short bowel syndrome. Twenty-nine (81% )- had a pull-through at 15± 6 months (modified Duhamel 20, Martin long Duha-mel 4, and Soave 5). Six had a Kimura patch. Postoperative complications (including enterocolitis)- were more common after long Duhamel and Soave procedures. Seven (19% ; 2 with Down’s syndrome) died (3 early, 4 late)- from pulmonary emboli (1), sepsis (1), fluid overload (1), viral illness (1), liver failure (1), arrhythmia (1), and total bowel aganglionosis (1). Mean follow-up was 11± 9 years (range, 6 months- 29 years). Twenty-four (83% ) of 29 patients exhibited growth by weight of 25% or more, 21 (91% ) of 23 older than toddler age had 4 to 6 bowel movements per day, and 17 (81% )- of 21 were continent. In 5 of 6, the Kimura patch provided functional benefit with proximal disease. Conclusion: Longterm survival was 81% . The highest morbidity occurred with long Duhamel or Soave procedures. The modified Duhamel is our procedure of choice in TCA. Bowel transplantation is an option for TCA with unadapted short bowel syndrome.展开更多
Purpose: This study tests the hypothesis that steroid administration improves the outcome of biliary atresia (BA) by evaluating the efficacy of postoperative steroid use on surgical outcomes in infants with BA. Method...Purpose: This study tests the hypothesis that steroid administration improves the outcome of biliary atresia (BA) by evaluating the efficacy of postoperative steroid use on surgical outcomes in infants with BA. Methods: Steroid use and outcomes in patients with BA were retrospectively analyzed at a tertiary pediatric hospital. Institutional review board approval was obtained. Results: Kasai portoenterostomy (PE) was performed in 43 patients with BA treated from 1992 to 2004 (16 boys and 27 girls). Twenty-one PE patients received steroids and 22 did not. Portoenterostomy was successful in 24 patients (55.8%) with consistent serum bilirubin less than 2 mg/dL. Sixteen (66%) received postoperative steroids. A normal postoperative bilirubin was achieved at 6 months in 16 (76%) of 21 patients with steroids compared with 8 (37%) of 22 in untreated controls (Fisher’s Exact test, P = .01). Of the 43 patients, 19 (44%) required liver transplantation, including 7 (37%) of 19 with steroids vs 12 (63%) of 19 without (P = .2). Twenty-eight infants developed cholangitis (fever with and without changes in hepatic function): 25 after PE and 3 after transplant. Of the 25, 12 (48%) received steroids. Seven died (16%) (range, 7 months to 4 years): 2 while awaiting transplantation (received steroids) and 5 after transplantation (1 received steroids and 4 were untreated). Survival was 86%(18/21) in patients with steroids and 82%(18/22) in those without. Transplant survival (74%)was comparable to previously reported historical controls (82%). Conclusions: The Kasai PE continues to be the procedure of choice in infants with BA younger than 3 months. A significantly improved clearance of postoperative jaundice and lower serum bilirubin levels were observed in patients receiving steroids. However, steroids had no effect on the incidence of cholangitis, need for liver transplantation, and overall survival. A prospective study with standardized dose and length of steroid administration and longer period of follow-up is necessary to more accurately assess the effectiveness of steroids after PE.展开更多
Aim of Study: Long-term outcome studies in survivors with stage IV neuroblastoma (NB) are sparse. This review evaluates late complications and long-term outcomes in stage IV NB survivors. Methods: A retrospective revi...Aim of Study: Long-term outcome studies in survivors with stage IV neuroblastoma (NB) are sparse. This review evaluates late complications and long-term outcomes in stage IV NB survivors. Methods: A retrospective review of stage IV NB survivors was performed to analyze outcomes,including longterm morbidity,recurrence,and survival. Main Results: Of 153 patients with stage IV NB,52 (34% ) survived (male-female,26:26). Age at diagnosis was 29.1 ± 31.7 months in survivors. Eighteen were 1 year or younger and 34 were older than 1 year compared with 10 nonsurvivors 1 year or younger and 91 older than 1 year (P = 0.0003,Fisher’ s Exact test). Primary tumor sites were adrenal (35),retroperitoneal (11),mediastinal (3),pelvic (2),and no primary with tumor metastases identified (1). Ten survivors had favorable and 16 had unfavorable histology compared with 1 favorable and 18 unfavorable in nonsurvivors (P = 0.01). Four survivors had MYCN amplification (≥ 10 copies) and 2 deletions of 1p and 11q. Sites of metastasis in survivors and nonsurvivors were similar. Treatment in survivors included surgery in 51 (75% [39/51] complete tumor resection [CTR]); chemotherapy,50; radiation,17; stem cell transplantation,20; and bone marrow transplant,1. In nonsurvivors,13 (25% ) of 53 (P< 0.0001) had CTR,18 stem cell transplantation,and 12 bone marrow transplant. Six patients had tumor recurrence but survived (mean,9.3 ± 8.3 years; range,6 months-24 years). Recurrence was local (1),distant (2),and both (3) and was treated by resection,chemotherapy,and radiation. The mean age of survivors was 12.4 ± 8.3 years (range,2-34 years). In all stage IV cases,event-free survival was 30% and overall survival was 34% . Long-term complications occurred in 23 (44% ) survivors,including endocrine disturbances (7),orthopedic (5),cataracts (2),adhesive bowel obstruction (2),hypertension (1),bronchiolitis (1),blindness (1),peripheral neuropathy (1),nonfunctioning kidney (1),cholelithiasis (1),and thyroid nodule (1). Conclusion: Only 34% of patients with stage IV NB survived despite aggressive multimodal therapy. Age of younger than 1 year,favorable pathology,CTR,and no recurrence were the only statistically significant factors that favored survival. Forty-four percent of survivors experienced late morbidity,and tumor recurred in 6 (11.5% ) of 52. Patients should be monitored for tumor recurrence and long-term sequelae. New methods of treatment are required to achieve better outcomes.展开更多
文摘Background/Methods: A 32- year retrospective review from 1972 to 2004 analyzed complications and long-term outcomes in children with total colonic aganglionosis (TCA) as they relate to the procedure performed. Results: Thirty-six patients (27 boys, 9 girls) had TCA. The level of aganglionosis was distal ileum (26), mid-small bowel (8), midjejunum (1), and entire bowel (1). Enterostomy was performed in 35 of 36. Eight developed short bowel syndrome. Twenty-nine (81% )- had a pull-through at 15± 6 months (modified Duhamel 20, Martin long Duha-mel 4, and Soave 5). Six had a Kimura patch. Postoperative complications (including enterocolitis)- were more common after long Duhamel and Soave procedures. Seven (19% ; 2 with Down’s syndrome) died (3 early, 4 late)- from pulmonary emboli (1), sepsis (1), fluid overload (1), viral illness (1), liver failure (1), arrhythmia (1), and total bowel aganglionosis (1). Mean follow-up was 11± 9 years (range, 6 months- 29 years). Twenty-four (83% ) of 29 patients exhibited growth by weight of 25% or more, 21 (91% ) of 23 older than toddler age had 4 to 6 bowel movements per day, and 17 (81% )- of 21 were continent. In 5 of 6, the Kimura patch provided functional benefit with proximal disease. Conclusion: Longterm survival was 81% . The highest morbidity occurred with long Duhamel or Soave procedures. The modified Duhamel is our procedure of choice in TCA. Bowel transplantation is an option for TCA with unadapted short bowel syndrome.
文摘Purpose: This study tests the hypothesis that steroid administration improves the outcome of biliary atresia (BA) by evaluating the efficacy of postoperative steroid use on surgical outcomes in infants with BA. Methods: Steroid use and outcomes in patients with BA were retrospectively analyzed at a tertiary pediatric hospital. Institutional review board approval was obtained. Results: Kasai portoenterostomy (PE) was performed in 43 patients with BA treated from 1992 to 2004 (16 boys and 27 girls). Twenty-one PE patients received steroids and 22 did not. Portoenterostomy was successful in 24 patients (55.8%) with consistent serum bilirubin less than 2 mg/dL. Sixteen (66%) received postoperative steroids. A normal postoperative bilirubin was achieved at 6 months in 16 (76%) of 21 patients with steroids compared with 8 (37%) of 22 in untreated controls (Fisher’s Exact test, P = .01). Of the 43 patients, 19 (44%) required liver transplantation, including 7 (37%) of 19 with steroids vs 12 (63%) of 19 without (P = .2). Twenty-eight infants developed cholangitis (fever with and without changes in hepatic function): 25 after PE and 3 after transplant. Of the 25, 12 (48%) received steroids. Seven died (16%) (range, 7 months to 4 years): 2 while awaiting transplantation (received steroids) and 5 after transplantation (1 received steroids and 4 were untreated). Survival was 86%(18/21) in patients with steroids and 82%(18/22) in those without. Transplant survival (74%)was comparable to previously reported historical controls (82%). Conclusions: The Kasai PE continues to be the procedure of choice in infants with BA younger than 3 months. A significantly improved clearance of postoperative jaundice and lower serum bilirubin levels were observed in patients receiving steroids. However, steroids had no effect on the incidence of cholangitis, need for liver transplantation, and overall survival. A prospective study with standardized dose and length of steroid administration and longer period of follow-up is necessary to more accurately assess the effectiveness of steroids after PE.
文摘Aim of Study: Long-term outcome studies in survivors with stage IV neuroblastoma (NB) are sparse. This review evaluates late complications and long-term outcomes in stage IV NB survivors. Methods: A retrospective review of stage IV NB survivors was performed to analyze outcomes,including longterm morbidity,recurrence,and survival. Main Results: Of 153 patients with stage IV NB,52 (34% ) survived (male-female,26:26). Age at diagnosis was 29.1 ± 31.7 months in survivors. Eighteen were 1 year or younger and 34 were older than 1 year compared with 10 nonsurvivors 1 year or younger and 91 older than 1 year (P = 0.0003,Fisher’ s Exact test). Primary tumor sites were adrenal (35),retroperitoneal (11),mediastinal (3),pelvic (2),and no primary with tumor metastases identified (1). Ten survivors had favorable and 16 had unfavorable histology compared with 1 favorable and 18 unfavorable in nonsurvivors (P = 0.01). Four survivors had MYCN amplification (≥ 10 copies) and 2 deletions of 1p and 11q. Sites of metastasis in survivors and nonsurvivors were similar. Treatment in survivors included surgery in 51 (75% [39/51] complete tumor resection [CTR]); chemotherapy,50; radiation,17; stem cell transplantation,20; and bone marrow transplant,1. In nonsurvivors,13 (25% ) of 53 (P< 0.0001) had CTR,18 stem cell transplantation,and 12 bone marrow transplant. Six patients had tumor recurrence but survived (mean,9.3 ± 8.3 years; range,6 months-24 years). Recurrence was local (1),distant (2),and both (3) and was treated by resection,chemotherapy,and radiation. The mean age of survivors was 12.4 ± 8.3 years (range,2-34 years). In all stage IV cases,event-free survival was 30% and overall survival was 34% . Long-term complications occurred in 23 (44% ) survivors,including endocrine disturbances (7),orthopedic (5),cataracts (2),adhesive bowel obstruction (2),hypertension (1),bronchiolitis (1),blindness (1),peripheral neuropathy (1),nonfunctioning kidney (1),cholelithiasis (1),and thyroid nodule (1). Conclusion: Only 34% of patients with stage IV NB survived despite aggressive multimodal therapy. Age of younger than 1 year,favorable pathology,CTR,and no recurrence were the only statistically significant factors that favored survival. Forty-four percent of survivors experienced late morbidity,and tumor recurred in 6 (11.5% ) of 52. Patients should be monitored for tumor recurrence and long-term sequelae. New methods of treatment are required to achieve better outcomes.
