Long-Term Functional Outcomes of an Anorectal Malformation French National Cohort.

OBJECTIVES: The present study aimed to assess long-term functional outcomes of children with anorectal malformations (ARMs) across a network of expert centers in France. METHODS: Retrospective cross-sectional study of patients ages 6-30 years that had been surgically treated for ARM. Patient and ARM characteristics (eg, level, surgical approach) and functional outcomes were assessed in the different age groups. RESULTS: Among 367 patients, there were 155 females (42.2%) and 212 males (57.8%), 188 (51.2%) cases with, and 179 (48.8%) higher forms without, perineal fistula. Univariate and multivariate statistical analyses with logistic regression showed correlation between the level of the rectal blind pouch and voluntary bowel movements (odds ratio [OR] = 1.84 [1.31-2.57], P < 0.001), or soiling (OR = 1.72 [1.31-2.25], P < 0.001), which was also associated with the inability to discriminate between stool and gas (OR = 2.45 [1.28-4.67], P = 0.007) and the presence of constipation (OR = 2.97 [1.74-5.08], P < 0.001). Risk factors for constipation were sacral abnormalities [OR = 2.26 [1.23-4.25], P = 0.01) and surgical procedures without an abdominal approach (OR = 2.98 [1.29-6.87], P = 0.01). Only the holding of voluntary bowel movements and soiling rates improved with age. CONCLUSION: This cross-sectional study confirms a strong association between anatomical status and functional outcomes in patients surgically treated for ARM. It specifically highlights the need for long-term follow-up of all patients to help them with supportive care.

Thoracic skeletal anomalies following surgical treatment of esophageal atresia. Lessons from a national cohort.

INTRODUCTION: Thoracotomy as surgical approach for esophageal atresia treatment entails the risk of deformation of the rib cage and consequently secondary thoracogenic scoliosis. The aim of our study was to assess these thoracic wall anomalies on a large national cohort and search for factors influencing this morbidity. MATERIALS AND METHODS: Pediatric surgery departments from our national network were asked to send recent thoracic X-ray and operative reports for patients born between 2008 and 2010 with esophageal atresia. The X-rays were read in a double-blind manner to detect costal and vertebral anomalies. RESULTS: Among 322 inclusions from 32 centers, 110 (34.2%) X-rays were normal and 25 (7.7%) displayed thoracic malformations, including 14 hemivertebrae. We found 187 (58.1%) sequelae of surgery, including 85 costal hypoplasia, 47 other types of costal anomalies, 46 intercostal space anomalies, 21 costal fusions and 12 scoliosis, with some patients suffering from several lesions. The rate of patients with these sequelae was not influenced by age at intervention, weight at birth, type of atresia, number of thoracotomy or size of the center. The rate of sequelae was higher following a classical thoracotomy (59.1%), whatever the way that thoracotomy was performed, compared to nonconverted thoracoscopy (22.2%; p=0.04). CONCLUSION: About 60 % of the patients suffered from a thoracic wall morbidity caused by the thoracotomy performed as part of surgical treatment of esophageal atresia. Minimally invasive techniques reduced thoracic wall morbidity. Further studies should be carried out to assess the potential benefit of minimally invasive approaches to patient pulmonary functions and on the occurrence of thoracogenic scoliosis in adulthood. LEVELS OF EVIDENCE: Level III retrospective comparative treatment study.

What prenatal ultrasound features are predictable of complex or vanishing gastroschisis? A retrospective study.

OBJECTIVE: To evaluate prenatal ultrasound parameters as prognostic factors for complex and vanishing gastroschisis. METHODS: Retrospective multicentre study of 200 gastroschisis over 13 years (2000-2013). Collection of prenatal ultrasound evaluation on maternal and fetal growth parameters, intra- and extra-abdominal bowel and stomach dilation, abdominal wall defect diameter and changes in bowel appearance. Correlation of these factors with the presence of mechanical intestinal complications at birth, named 'complex gastroschisis'. RESULTS: Fifty-two patients (26%) had complex gastroschisis (CG), including ten vanishing gastroschisis. The presence of intra-abdominal bowel dilation at the second (T2) or third (T3) trimester ultrasound was predictive for CG, with odds ratios at 6.69 (95%CI 2.41-18.55) and 4.72 (95%CI 2.16-10.28), respectively, with a cut-off value at the last examination of >19 mm. A small abdominal wall defect diameter was also predictive for CG, with cut-off values of <9.2 mm at T2 and <12.5 mm at T3. Vanishing gastroschisis recorded earlier intra-abdominal bowel dilation diagnosis, associated with a small wall defect and no extra-abdominal dilation. CONCLUSION: Intra-abdominal bowel dilation and a small abdominal wall defect diameter accurately predict CG and could be a first sign of vanishing gastroschisis when they occur early. © 2016 John Wiley & Sons, Ltd.

Long-term effects of endoscopic injection of dextranomer/hyaluronic acid based implants for treatment of urinary incontinence in children with neurogenic bladder.

PURPOSE: We prospectively evaluated the efficacy of dextranomer/hyaluronic acid based implants for the endoscopic treatment of neurogenic urinary incontinence. MATERIALS AND METHODS: We prospectively studied patients undergoing endoscopic injection of dextranomer/hyaluronic acid for structural incontinence beginning in October 1997. Preoperative evaluation consisted of medical history, incontinence charts, urine culture, urinary tract ultrasound and videourodynamics, which were repeated regularly during followup. At each evaluation patients were classified as cured or significantly improved (treatment success), or not significantly improved (treatment failure). Followup ranged from 1 to 13 years (mean 7.4). RESULTS: A total of 48 patients 5 to 20 years old with congenital (45 patients) or acquired (3) neurogenic bladder suffering from severe sphincteric incompetence were included. Of the patients 30 underwent 1, 13 underwent 2 and 5 underwent 3 treatment sessions. Mean injected volume was 4.6 ml per session. Five patients had previously undergone bladder neck surgery. Two patients lost to followup in the short term were classified as treatment failures. Of the patients 45 had more than 2 years of followup, of whom 19 (39.6%) were dry, 6 (12.5%) were significantly improved and 23 (47.9%) had no significant improvement observed. Female patients achieved a significantly higher success rate than males (p = 0.05). No significant clinical or urodynamic criterion was predictive of success or failure. Posttreatment bladder deterioration developed in 10 cases (7 successes and 3 failures), which were managed by bladder augmentation. CONCLUSIONS: Endoscopic treatment of neurogenic urinary sphincter insufficiency with dextranomer/hyaluronic acid is effective in half of the patients. Recurrence of incontinence after 1 year is mainly a sign of bladder deterioration.

Safety of shock wave lithotripsy for treatment of pediatric urolithiasis: 20-year experience.

PURPOSE: This retrospective study was designed to assess the impact of shock wave lithotripsy on the pediatric kidney using pretreatment and posttreatment (99m)technetium dimercapto-succinic acid renal scintigram. MATERIALS AND METHODS: A total of 182 patients 5 months to 19.8 years old (mean 5.3 years) were treated for renal calculi with shock wave lithotripsy during a 20-year period. Pretreatment evaluation included clinical assessment, urine culture, renal ultrasound and plain abdominal radiograph with or without excretory urogram. Dimercapto-succinic acid scintigram was performed before and 6 months after completion of treatment in 94 patients (52%). RESULTS: Patients underwent 1 to 4 sessions of shock wave lithotripsy per kidney with at least 1 month between treatments. Median number of shocks delivered per session was 3,000 (IQR 2,601 to 3,005). No new scars were observed on any posttreatment dimercapto-succinic acid scan. Regarding renal function, patients fell into 1 of 4 groups. Group 1 (66 patients, 70%) had normal function on dimercapto-succinic acid scan before and after treatment, group 2 (18, 19%) had decreased function in the affected kidney on pretreatment scan with no change after treatment, group 3 (2, 2%) had impaired function in the treated kidney that was transient (1) or permanent (1) and group 4 (7, 7%) had improved function in the treated kidney. CONCLUSIONS: Shock wave lithotripsy is an effective treatment for renal calculi in children. Renal parenchymal trauma associated with extracorporeal shock wave lithotripsy does not seem to cause long-term alterations in renal function or development of permanent renal scars in children.