Different Fecal Microbiota in Hirschsprung's Patients With and Without Associated Enterocolitis.

Background and Objectives: Patients with Hirschsprung's disease are at risk of developing Hirschsprung-associated enterocolitis, especially in the first 2 years of life. The pathophysiology of this inflammatory disease remains unclear, and intestinal dysbiosis has been proposed in the last decade. The primary objective of this study was to evaluate in a large cohort if Hirschsprung-associated enterocolitis was associated with alterations of fecal bacterial composition compared with HD without enterocolitis in different age groups. Methods: We analyzed the fecal microbiota structure of 103 Hirschsprung patients from 3 months to 16 years of age, all of whom had completed definitive surgery for rectosigmoid Hirschsprung. 16S rRNA gene sequencing allowed us to compare the microbiota composition between Hirschsprung's disease patients with (HAEC group) or without enterocolitis (HD group) in different age groups (0-2, 2-6, 6-12, and 12-16 years). Results: Richness and diversity increased with age group but did not differ between HD and HAEC patients, irrespective of the age group. Relative abundance of Actinobacteria was lower in HAEC than in HD patients under 2 years of age (-66%, P = 0.045). Multivariate analysis by linear models (MaAsLin) considering sex, medications, birth mode, breast-feeding, and the Bristol stool scale, as well as surgery parameters, highlighted Flavonifractor plautii and Eggerthella lenta, as well as Ruminococcus gnavus group, as positively associated with Hirschsprung-associated enterocolitis in the 0-2 years age group. Conclusion: Hirschsprung-associated enterocolitis was associated with features of intestinal dysbiosis in infants (0-2 years) but not in older patients. This could explain the highest rate of enterocolitis in this age group. Clinical Trial Registration: https://clinicaltrials.gov/ct2/show/NCT02857205, MICROPRUNG, NCT02857205, 02/08/2016.

Persistent Soiling Affects Quality of Life in Children With Hirschsprung's Disease.

OBJECTIVE: This multicentric study aimed to evaluate the quality of life (QOL) in children with Hirschsprung's disease (HD). METHODS: HD patients aged from 6 to 18 years and followed-up in 2 French pediatric surgery centers were included in this study. QOL was assessed using the HAQL questionnaires according to age (6-11 and 12-18), filled by patients and their parents (proxy reports) and correlated with initial disease characteristics, nutritional status, and functional score of Krickenbeck. RESULTS: Sixty-three patients were included. The acquisition of satisfactory voluntary bowel movements was found in only 50% of the 6 to 11 years old and 68% of the teenagers. Seventy percentage of the children and 55% of teenagers had soiling issues. The overall HAQLproxy6--11 score was 528/700; best scores were found for "fecal continence" (94/100), "social functioning" (94/100), and "urinary continence" (92/100) whereas the worst scores were for "general well-being" (64/100) and "diurnal fecal continence" (58/100). The overall HAQLproxy12--16 score was 607/700; best scores were for "urinary continence" (96/100) and "social functioning" (93/100). In a multivariate analysis, soiling was the only factor significantly associated with low QOL (P = 0.03). CONCLUSIONS: Soiling remains frequent in children operated on for HD and negatively affects their QOL. Assessment and treatment of soiling should be the priority for medical teams in the follow-up of these children.

Thoracoscopic Rib Resection in Children.

OBJECTIVE: The authors present a multicenter retrospective series of different benign rib lesions in children operated on using thoracoscopy. MATERIALS AND METHODS: Between 2005 and 2015, 17 rib resections were performed thoracoscopically, in four French departments of pediatric surgery. Of these 17 cases, 13 exostoses, 2 endochondromas, 1 synostosis, and 1 Cyriax's syndrome were noted. Inclusion criteria were benign tumors or rib anomalies such as synostosis, in children younger than the age of 18 years, and thoracoscopy. Open surgery and malignant tumors were excluded. Thoracoscopy was put forward using one optical port as well as one or two operative ports. RESULTS: Ten patients presented with chest pain, dyspnea, or unexplained cough. Six tumors were incidentally diagnosed. One patient presented with a chest wall deformation. Single-lung ventilation was required in 2 cases. In 1 case of endochondroma, a segmental rib resection was performed, leaving a part of the periosteum and the intercostal vessels and nerve. In this case, rib resection was performed using an endoscopic shaver drill system. As for the other cases, a simple resection of the tumor or of the bridge between two ribs (synostosis) was performed. In these cases, a Codman Kerrison laminectomy rongeur was used. There was no complication during or after surgery. Nevertheless, 2 years after surgery, pain did not disappear in 1 case, probably due to a definitive intercostal nerve damage. CONCLUSION: Benign rib lesions in children are rare. Thoracoscopy may be offered to reduce the functional deleterious consequences of an open surgery. It may be put forward especially in case of hereditary multiple exostoses where redo procedures may be required.

Twenty years' experience for reduction of ileocolic intussusceptions by saline enema under sonography control.

BACKGROUND: Ultrasonography is a well-established efficient diagnostic tool for ileocolic intussusceptions in children. It can also be used to control hydrostatic reduction by saline enemas. This reduction method presents the advantage of avoiding radiations. Parents can even stay with their children during the procedure, which is comforting for both. The purpose of this study was to present our 20 years' experience in intussusception reductions using saline enema under ultrasound control and to assess its efficiency and safety. MATERIAL AND METHODS: This retrospective single center study included patients with ileocolic intussusceptions diagnosed by ultrasound between June 1993 and July 2013. We excluded the data of patients with spontaneous reduction or who underwent primary surgery because of contraindications to hydrostatic reduction (peritonitis, medium or huge abdominal effusion, ischemia on Doppler, bowel perforation). A saline enema was infused into the colon until the reduction was sonographically confirmed. The procedure was repeated if not efficient. Light sedation was practiced in some children. RESULTS: Eighty-tree percent of the reductions were successful with a median of 1 attempt. Reduction success decreased with the number of attempts but was still by 16% after 4 attempts. The early recurrence rates were 14.5%, and 61.2% of those had a successful second complete reduction. Forty-six patients needed surgery (11 of them had a secondary intussusception). Sedation multiplies success by 10. In this period, only one complication is described. CONCLUSION: Ultrasound guided intussusception reduction by saline enema is an efficient and safe procedure. It prevents exposure of a young child to a significant amount of radiation, with similar success rate. We had very low complication rate (1/270 cases or 3‰). The success rate could be increased by standardized procedures including: systematic sedation, trained radiologists, accurate pressure measurement, and number and duration of attempts.

Which surgery for drooling in patients with cerebral palsy?

BACKGROUND: Surgery for drooling in patients with cerebral palsy should not produce xerostomia in order not to deteriorate speech, taste, or the status of oral hygiene. It must be a compromise between drooling and quality of life. The purpose of the present report is to describe our surgical strategy that respects the above principles. MATERIALS AND METHODS: Patients were initially operated on depending on the drooling severity. The results were evaluated according to the frequency of residual drooling and the Thomas-Stonel and Greenberg classification. Quantitative assessment was proposed 6 months after surgery. The data have been compared using the nonparametric Wilcoxon matched-pairs test. RESULTS: Thirty-five patients underwent surgery between 1991 and 2012. Owing to incomplete data, only 31 patients could be included, aged 5 to 24 years (mean: 12 years). All patients underwent surgery on the submandibular duct. Only 16 patients underwent a simultaneous surgery on the parotid duct. Six patients were reoperated: 3 because of an insufficient result and 3 because of a surgical complication. Changes/Day ranged from 1 to 7 (median: 3) before surgery and 0 to 2 (median: 1) after surgery (p < 0.01). Number of bibs/day ranged from 0 to 30 (median: 4) before surgery and 0 to 4 (median: 1) after surgery (p < 0.01). No dental deterioration and no caries occurred after surgery. CONCLUSION: Good results for drooling can be obtained with a simple surgical procedure on the submandibular ducts, maintaining quality of life, avoiding deterioration of speech, taste, and the status of oral hygiene.