Well-being of children with neurologic impairment after fundoplication and gastrojejunostomy tube feeding.

BACKGROUND: There is limited understanding on the impact of fundoplication or gastrojejunostomy tube (GJT) feeding in children with neurologic impairment who have swallowing dysfunction, gastroesophageal reflux disease, and/or dysmotility. OBJECTIVE: To explore and understand the well-being of families and their children with neurologic impairment who had either a fundoplication or GJT. METHODS: This was a qualitative study using in-depth, individual interviews with parents of children with severe neurologic impairment who had either a fundoplication with gastrostomy or GJT. Parents' perceptions of daily life and QoL were explored in the context of the interventions. Interviews were transcribed, and data were analyzed using qualitative content analysis. RESULTS: Children and their parents are profoundly impacted by the health condition of the children. QoL effects were similar across spheres of individual and family experience and well-being. Themes related to QoL were: physical health of the child and the future, intense caregiving needs, social isolation and stigma, pervasive impact on the family, financial strain, and the process of finding meaning. Fundoplication and GJT affected feeding tolerance and times, caregiving, and health care utilization. Parents identified how changes in these factors influenced child, parental, and family QoL. CONCLUSIONS: The diverse influences of the underlying condition and the interventions should be addressed in the delivery of health services. Future studies of the effectiveness of fundoplication and GJT feeding should address the well-being of the child and family, the impact on family caregiving, and health care utilization.

Partial splenectomy for hereditary spherocytosis: a multi-institutional review.

BACKGROUND/PURPOSE: Partial splenectomy has emerged as a surgical option for selected children with hereditary spherocytosis, with the goal of reducing anemia while preserving splenic function. This multi-institutional study is the largest series to date examining outcomes data for partial splenectomy in patients with hereditary spherocytosis. METHODS: Data were collected retrospectively from 5 North American pediatric hospitals. Sixty-two children underwent partial splenectomy for hereditary spherocytosis between 1990 and 2008. RESULTS: At 1 year following partial splenectomy, mean hemoglobin significantly increased by 3.0 ± 1.4 g/dL (n = 52), reticulocyte count decreased by 6.6% ± 6.6% (n = 41), and bilirubin level decreased by 1.3 ± 0.9 mg/dL (n = 25). Patients with poor or transient hematologic response were found to have significantly more splenic regeneration postoperatively compared with patients with a durable clinical response (maximal spleen dimension, 9.0 ± 3.4 vs 6.3 ± 2.2 cm). Clinically significant recurrence of anemia or abdominal pain led to completion splenectomy in 4.84% of patients. No patients developed postsplenectomy sepsis. CONCLUSIONS: Our multi-institutional review indicates that partial splenectomy for hereditary spherocytosis leads to sustained and clinically significant improvement in hematologic profiles and clinical symptoms in most patients. Our data support partial splenectomy as an alternative for selected children with hereditary spherocytosis.

Is congenital cystic adenomatoid malformation a premalignant lesion for pleuropulmonary blastoma?

BACKGROUND: The relationship between congenital cystic adenomatoid malformation (CCAM) and pleuropulmonary blastoma (PPB), whether causal, correlational, or coincidental, remains controversial. There is a lack of consensus as to the optimal treatment of patients with asymptomatic CCAM. METHOD: We reviewed all cases of CCAM and PPB seen at our institution from 1999 to 2008. Institutional Research Ethics Board approval was obtained. The incidence of CCAM and PPB, respectively, was calculated based on birth numbers during the study period. RESULTS: Seventy-four CCAMs were resected over the study period in 129 children diagnosed with CCAM. Five PPBs were diagnosed during the study period. Three of the 5 PPB cases were initially diagnosed as CCAMs. These PPBs were not clinically or radiologically distinguishable from CCAMs. In our referral area, the incidence of CCAM was 1 in 12,000; and the incidence of PPB was 1 in 250,000 live births. The mortality rate for PPB in this cohort was 20%. CONCLUSION: Asymptomatic cystic lung malformations represent a therapeutic dilemma. In this cohort, the incidence of PPB among apparently benign lung lesions was 4%. No clinical or radiological markers differentiated benign CCAMs from PPBs. Our experience provides further justification for resection of all CCAMs. This should be discussed with parents until CCAMs and PPBs can be clearly distinguished preoperatively.

Endorectal pull-through for Hirschsprung's disease-a multicenter, long-term comparison of results: transanal vs transabdominal approach.

PURPOSE: Previous studies have reported decreased continence in patients undergoing transanal endorectal pull-through (TERP) for Hirschsprung's disease compared to the older transabdominal approach (TAA). To address this, we examined long-term stooling outcomes in a large, multicenter cohort of patients undergoing either TERP or TAA. METHODS: Data were collected from 5 large pediatric institutions. Patient families were surveyed using a stooling score system (0-40, best to worst total score). Inclusion criteria included patients older than 3 years and those who had more than 6 months of recovery after pull-through. Those with total colonic aganglionosis were excluded. Statistical analysis included univariate and multivariate linear regression (significance, P < .05). RESULTS: Two hundred eighty-one patients underwent TERP (192) or TAA (89). Interviews were completed in 149 (104 [52%] TERP vs 45 [52%] TAA). The TAA group had a significantly greater number of daily bowel movements for each respective postoperative year and experienced more early complications (3% vs 1% with >1 complication; P = .061) and late complications (19% vs 4% with >1 complication; P < .001). Although the TAA group had a higher mean enterocolitis score (3.3 +/- 0.4 vs 1.8 +/- 0.2; P < .001), this was not borne out by multivariate regression analysis (P = .276). Parental survey showed that there were no significant differences between procedures in mean total, continence, or stooling pattern scores. CONCLUSION: Transanal endorectal pull-through was associated with fewer complications and fewer episodes of enterocolitis. In contrast to prior studies, TERP patients did not have a higher rate of incontinence. These results support use of TERP as an excellent surgical approach for children with Hirschsprung's disease.

A single center 26-year experience with treatment of esophageal achalasia: is there an optimal method?

PURPOSE: Treatment modalities for achalasia are evolving and remain controversial. Herein, we report the relative efficacy and outcomes after dilatation or myotomy in children with achalasia. METHODS: A retrospective analysis of all children treated for achalasia at a tertiary center from 1981 to 2007 was performed (n = 40). Demographics, presenting symptoms, perioperative parameters, and outcomes were analyzed using t tests and chi(2) statistics. RESULTS: Thirty patients were initially treated by esophageal dilatation (ED), whereas 10 were treated by laparoscopic or open Heller myotomy (HM). Both groups were similar with respect to age (10.6 vs 12.4 years; P = .19). There were 18 males and 12 females in the ED group, compared to 5 males and 5 females in the HM group (P = .72). Mean duration of symptoms before diagnosis, including dysphagia, vomiting, food sticking, chest pain, and weight loss, was 15.9 months for ED and 10.7 months for HM (P = .41). Mean time from diagnosis to initial intervention was 76 days in ED vs 86 days in HM (P = .78). Subsequent interventions by myotomy or both dilatation and myotomy were required in 9 (30%) of 30 patients in the ED group and 2 (20%) of 10 patients in the HM group (P = .70). A clear transition from open to laparoscopic approach occurred between 1995 and 2001. Mean operating times were comparable (186.3 vs 156.0 minutes; P = .48). Of 14 laparoscopic myotomies, 11 (79%) had fundoplication, and 2 (18%) of the 11 were converted to open procedure. Intraoperative mucosal perforation rates were similar between open and laparoscopic groups (17% vs 18%). At follow-up, 32% of ED patients vs 43% HM had complete symptom relief (mean follow-up duration, 75.2 months; SD, 196.5). CONCLUSION: Both dilatation and myotomy are effective immediate treatment of achalasia. A clear transition to and preference for laparoscopic approach has occurred in the treatment of achalasia in children.

Development of a standardized definition for Hirschsprung's-associated enterocolitis: a Delphi analysis.

PURPOSE: The reported incidence of Hirschsprung's-associated enterocolitis (HAEC) is extremely variable. A standardized definition would permit comparison of different studies and provide an interpretable outcome measure for future prospective studies in patients with Hirschsprung's disease. METHODS: The Delphi method is a technique for achieving consensus among a panel of experts. A list of 38 potential criteria from the history, physical examination, radiologic studies, and pathologic specimens was made available to pediatric surgeons and gastroenterologists who have contributed to the literature on Hirschsprung's disease. Each expert ranked the diagnostic importance of each item using a Likert scale. In subsequent surveys, the same process was used, but the means and SDs from previous rounds were included as a way of influencing the experts toward consensus. Cronbach's alpha was used after each round to measure variability among the experts. Once consensus was reached, an overall "HAEC score" was developed by assigning a value of 1 or 2 to each item that was considered important by the expert panel. The score was then validated by circulating 10 clinical cases to the panel and asking if each represented HAEC or not. RESULTS: Twenty-seven experts completed the survey. Cronbach's alpha increased from 0.93 after the first round to 0.97 after the second. Criteria receiving the highest scores were diarrhea, explosive stools, abdominal distension, and radiologic evidence of bowel obstruction or mucosal edema. Eighteen items were included in the score. During the validation process, the score agreed with the experts in 9 of the 10 case scenarios. CONCLUSION: The most important clinical diagnostic criteria for HAEC were identified from a larger pool of potential diagnostic items through a consensus approach using the Delphi method. A score was developed and validated and can now be used as a standardized and reproducible outcome measure for future studies in children with Hirschsprung's disease.

Routine use of a SILASTIC spring-loaded silo for infants with gastroschisis: a multicenter randomized controlled trial.

BACKGROUND: Retrospective studies have suggested that routine use of a preformed silo for infants with gastroschisis may be associated with improved outcomes. We performed a prospective multicenter randomized controlled trial to test this hypothesis. METHODS: Eligible infants were randomized to (1) routine bedside placement of a preformed Silastic spring-loaded silo, with gradual reduction and elective abdominal wall closure, or (2) primary closure. RESULTS: There were 27 infants in each group. There was no significant difference between groups with respect to age, weight, sex, Apgar scores, prenatal diagnosis, or mode of delivery. The total number of days on the ventilator was lower in the spring-loaded silo group, although it did not reach statistical significance (3.2 vs 5.3, P = .07). There was no significant difference between groups with respect to length of time on total parenteral nutrition, length of stay, or incidence of sepsis and necrotizing enterocolitis. CONCLUSION: Routine use of a preformed silo was associated with similar outcomes to primary closure for infants with gastroschisis but with a strong trend toward fewer days on the ventilator. Use of a preformed silo has the advantage of permitting definitive abdominal wall closure in a more elective setting.