To determine whether the 3-year event-free survival (EFS) of children with completely resected im... more To determine whether the 3-year event-free survival (EFS) of children with completely resected immature teratomas is greater than 85%. Patients with immature teratomas treated at Pediatric Oncology Group or Children's Cancer Group institutions were eligible. Pathology was centrally reviewed to confirm diagnosis and tumor grading. Follow-up included physical examination, measurement of tumor markers (alpha fetoprotein and human chorionic gonadotropin), and imaging. All patients were monitored for events, defined as tumor recurrence, second malignancy, or death. Seventy-three children (median age, 7.8 years) with extracranial immature teratomas were enrolled on study. Primary tumor sites included ovarian (n = 44), testicular (n = 7), and extragonadal (n = 22). However, on review, 23 patients had foci of yolk sac tumor (n = 21) or primitive neuroectodermal tumor (n = 2), whereas 50 had pure immature teratomas. Twenty-five patients had increased alpha fetoprotein (n = 18), human chorionic gonadotropin (n = 5), or both (n = 2); nine had foci of yolk sac tumor on review. Pathology review identified 23 patients with grade 1, 29 with grade 2, and 21 with grade 3 immature teratomas. With a median follow-up of 35 months, the overall 3-year EFS was 93% (95% confidence interval, 86% to 98%), with 3-year EFS of 97.8%, 100%, and 80% for patients with ovarian, testicular, and extragonadal tumors, respectively. Only four of 23 patients with immature teratoma and malignant foci developed recurrence, suggesting that surgical resection followed by close observation are effective treatment. Overall, five patients had disease recurrence 4 to 7 months from diagnosis, and four (80%) are disease free after platinum-based therapy. The fifth patient has residual tumor after cisplatin, etoposide, and bleomycin treatment requiring further therapy. Surgical excision is safe and effective treatment for 80% to 100% of children with immature teratoma.
Retroperitoneal fibrosis is an unusual cause of obstructive uropathy in the pediatric population.... more Retroperitoneal fibrosis is an unusual cause of obstructive uropathy in the pediatric population. The etiology is unknown although there are laboratory and clinical associations with various autoimmune diseases. Familial associations have not been reported in children. A family is reported where two siblings have idiopathic retroperitoneal fibrosis. In addition, these sisters and the father manifest clinical laboratory evidence for systemic immunologic diseases. These young girls represent the first patients to illustrate both autoimmune and familial characteristics of this disease. These cases support the concept that retroperitoneal fibrosis is a local manifestation of a systemic immune disease.
This policy statement is the first published statement in the United States on this topic and the... more This policy statement is the first published statement in the United States on this topic and the authors aim to provide pediatricians with evidence-based information on the unique aspects required to care for hospitalized adolescents. Included in this policy statement is a description of the possible effects hospitalization may have on the developmental and emotional progress of adolescence, the role of the hospital setting, the importance of confidentiality, and issues related to legal/ethical matters and bias and institutional and systemic racism that may occur during hospitalization.
Correction of esophageal atresia with tracheoesophageal fistula can present a difficult problem w... more Correction of esophageal atresia with tracheoesophageal fistula can present a difficult problem when distance and tension between upper and lower esophageal segments are too great for primary anastomosis. Alternatives have included staged procedures with upper pouch elongation or interpositions. In 1974, Shafer and David described an operation in which the lower segment was divided, closed, and then merely apposed to the unopened upper pouch. A central "necrosing suture" was placed through the lumina of both segments. Spontaneous fistulization between the two segments occurred postoperatively, and the resultant channel was then progressively dilated with woven silk bougies. This technique has been employed at Babies Hospital in five children. The results were excellent in four. One child failed to fistulize, and following a complicated postoperative course, died at home from accidental dislodgement of his tracheostomy tube. All five children required antireflux procedures but ultimately did well with regard to esophageal function.
Gastrostomy feeding tube placement in children is associated with a high frequency of adverse eve... more Gastrostomy feeding tube placement in children is associated with a high frequency of adverse events. This study sought to preoperatively estimate postoperative adverse events in children undergoing gastrostomy feeding tube placement. This was an observational study of children who underwent gastrostomy with or without fundoplication at 1 of 50 participating hospitals, using 2011-2013 data from the American College of Surgeons' National Surgical Quality Improvement Program Pediatric. The outcome was the occurrence of any postoperative complications or mortality at 30 days after gastrostomy tube placement. The preoperative clinical characteristics significantly associated with occurrence of adverse events were included in a multivariate logistic model. The area under the receiver operating characteristic curve was computed to assess model performance and split-set validated. A total of 2817 children were identified as having undergone gastrostomy tube placement. The unadjusted rate of adverse events within 30 days after gastrostomy tube placement was 11%. Thirteen predictor variables were identified. Notable preoperative variables associated with a greater than 75% increase in adverse event rate were preoperative sepsis/septic shock (odds ratio [OR], 10.76, 95% confidence interval [CI], 3.84-30.17), central nervous system tumor (OR, 3.36; 95% CI, 1.42-7.95), the primary procedure as indicated by the current procedural terminology (CPT) linear risk variable (OR, 1.93; 95% CI, 1.50-2.49), severe cardiac risk factors (OR, 1.88; 95% CI, 1.17-3.03), and preoperative seizure history (OR, 1.90; 95% CI, 1.38-2.62). The area under the receiver operating characteristic curve was 0.71 with the derivation data set and 0.71 upon split-set validation. Preoperatively estimating postoperative adverse events in children undergoing gastrostomy tube placement is feasible.
To determine whether the 3-year event-free survival (EFS) of children with completely resected im... more To determine whether the 3-year event-free survival (EFS) of children with completely resected immature teratomas is greater than 85%. Patients with immature teratomas treated at Pediatric Oncology Group or Children's Cancer Group institutions were eligible. Pathology was centrally reviewed to confirm diagnosis and tumor grading. Follow-up included physical examination, measurement of tumor markers (alpha fetoprotein and human chorionic gonadotropin), and imaging. All patients were monitored for events, defined as tumor recurrence, second malignancy, or death. Seventy-three children (median age, 7.8 years) with extracranial immature teratomas were enrolled on study. Primary tumor sites included ovarian (n = 44), testicular (n = 7), and extragonadal (n = 22). However, on review, 23 patients had foci of yolk sac tumor (n = 21) or primitive neuroectodermal tumor (n = 2), whereas 50 had pure immature teratomas. Twenty-five patients had increased alpha fetoprotein (n = 18), human chorionic gonadotropin (n = 5), or both (n = 2); nine had foci of yolk sac tumor on review. Pathology review identified 23 patients with grade 1, 29 with grade 2, and 21 with grade 3 immature teratomas. With a median follow-up of 35 months, the overall 3-year EFS was 93% (95% confidence interval, 86% to 98%), with 3-year EFS of 97.8%, 100%, and 80% for patients with ovarian, testicular, and extragonadal tumors, respectively. Only four of 23 patients with immature teratoma and malignant foci developed recurrence, suggesting that surgical resection followed by close observation are effective treatment. Overall, five patients had disease recurrence 4 to 7 months from diagnosis, and four (80%) are disease free after platinum-based therapy. The fifth patient has residual tumor after cisplatin, etoposide, and bleomycin treatment requiring further therapy. Surgical excision is safe and effective treatment for 80% to 100% of children with immature teratoma.
Retroperitoneal fibrosis is an unusual cause of obstructive uropathy in the pediatric population.... more Retroperitoneal fibrosis is an unusual cause of obstructive uropathy in the pediatric population. The etiology is unknown although there are laboratory and clinical associations with various autoimmune diseases. Familial associations have not been reported in children. A family is reported where two siblings have idiopathic retroperitoneal fibrosis. In addition, these sisters and the father manifest clinical laboratory evidence for systemic immunologic diseases. These young girls represent the first patients to illustrate both autoimmune and familial characteristics of this disease. These cases support the concept that retroperitoneal fibrosis is a local manifestation of a systemic immune disease.
This policy statement is the first published statement in the United States on this topic and the... more This policy statement is the first published statement in the United States on this topic and the authors aim to provide pediatricians with evidence-based information on the unique aspects required to care for hospitalized adolescents. Included in this policy statement is a description of the possible effects hospitalization may have on the developmental and emotional progress of adolescence, the role of the hospital setting, the importance of confidentiality, and issues related to legal/ethical matters and bias and institutional and systemic racism that may occur during hospitalization.
Correction of esophageal atresia with tracheoesophageal fistula can present a difficult problem w... more Correction of esophageal atresia with tracheoesophageal fistula can present a difficult problem when distance and tension between upper and lower esophageal segments are too great for primary anastomosis. Alternatives have included staged procedures with upper pouch elongation or interpositions. In 1974, Shafer and David described an operation in which the lower segment was divided, closed, and then merely apposed to the unopened upper pouch. A central "necrosing suture" was placed through the lumina of both segments. Spontaneous fistulization between the two segments occurred postoperatively, and the resultant channel was then progressively dilated with woven silk bougies. This technique has been employed at Babies Hospital in five children. The results were excellent in four. One child failed to fistulize, and following a complicated postoperative course, died at home from accidental dislodgement of his tracheostomy tube. All five children required antireflux procedures but ultimately did well with regard to esophageal function.
Gastrostomy feeding tube placement in children is associated with a high frequency of adverse eve... more Gastrostomy feeding tube placement in children is associated with a high frequency of adverse events. This study sought to preoperatively estimate postoperative adverse events in children undergoing gastrostomy feeding tube placement. This was an observational study of children who underwent gastrostomy with or without fundoplication at 1 of 50 participating hospitals, using 2011-2013 data from the American College of Surgeons' National Surgical Quality Improvement Program Pediatric. The outcome was the occurrence of any postoperative complications or mortality at 30 days after gastrostomy tube placement. The preoperative clinical characteristics significantly associated with occurrence of adverse events were included in a multivariate logistic model. The area under the receiver operating characteristic curve was computed to assess model performance and split-set validated. A total of 2817 children were identified as having undergone gastrostomy tube placement. The unadjusted rate of adverse events within 30 days after gastrostomy tube placement was 11%. Thirteen predictor variables were identified. Notable preoperative variables associated with a greater than 75% increase in adverse event rate were preoperative sepsis/septic shock (odds ratio [OR], 10.76, 95% confidence interval [CI], 3.84-30.17), central nervous system tumor (OR, 3.36; 95% CI, 1.42-7.95), the primary procedure as indicated by the current procedural terminology (CPT) linear risk variable (OR, 1.93; 95% CI, 1.50-2.49), severe cardiac risk factors (OR, 1.88; 95% CI, 1.17-3.03), and preoperative seizure history (OR, 1.90; 95% CI, 1.38-2.62). The area under the receiver operating characteristic curve was 0.71 with the derivation data set and 0.71 upon split-set validation. Preoperatively estimating postoperative adverse events in children undergoing gastrostomy tube placement is feasible.
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