Sexual well-being and penile appearance in adolescents operated for distal hypospadias in childhood.

BACKGROUND: The importance of patient-reported outcomes (PRO) in hypospadias is increasing. However, more knowledge is needed concerning genital self-perception on appearance and function in adolescents. The complication rates for distal hypospadias is different from that for severe hypospadias, and expected outcomes related to sexual well-being and cosmetics may also differ. OBJECTIVE: To investigate 16-year-olds' self-reported outcomes on penile appearance, sexual well-being, and voiding function in distal hypospadias, and compare with that of healthy male adolescents and a surgeon's view. STUDY DESIGN: Sixteen-year-old patients operated for distal hypospadias were included in this cross-sectional study and compared to a group of healthy adolescents. The assessment tools included the adolescents' self-perception on genital appearance and function measured by Pediatric Penile Perception Score (PPPS) and their responses to a structured interview. We also included information on clinical data from the electronic medical records, together with a physical examination and an uroflowmetry. RESULTS: Seventy patients and 61 healthy adolescents participated. Patients and the comparison group reported no differences on sexual well-being. The patients were satisfied with penile appearance, however their overall PPPS was significantly lower (8.9), compared to the comparison group (9.6, p = 0.03). Thirty-nine percent of patients had complications leading to re-interventions and reported lower scores on genital self-perception on appearance and function compared to those who had not re-interventions. Voiding function was normal. The surgeon's score on appearance was comparable to the patients' score. DISCUSSION: A key finding in our study is the patients' high satisfaction on sexual well-being, which was similar to healthy adolescents. The patients were also satisfied with penile appearance but scored significantly lower than the comparison group. Surgeons and patients had comparable scores on appearance; however, they seemed to emphasize different aspects of appearance. Our results on penile appearance and sexual well-being are comparable to those of other studies on distal hypospadias. In our study, re-interventions were associated with more negative genital self-perception on appearance and function, similar to findings in other studies. CONCLUSION: Our results show overall positive satisfaction on sexual well-being, voiding function and penile appearance despite less satisfaction on penile appearance when compared with the comparison group. Satisfaction was reported to be good also in patients experiencing re-interventions.

Clinical Variables as Indicative Factors for Endoscopy in Adolescents with Esophageal Atresia.

INTRODUCTION: Gastro-esophageal reflux disease (GERD) occurs frequently in patients operated for esophageal atresia (EA). Longstanding esophagitis may lead to dysphagia, strictures, columnar metaplasia, and dysplasia with an increased risk of adenocarcinoma. Are clinical factors and non-invasive assessments reliable indicators for follow-up with endoscopy? MATERIAL AND METHOD: A follow-up study with inclusion of EA adolescents in Norway born between 1996 and 2002 was conducted. Clinical assessment with pH monitoring, endoscopy with biopsies, along with interviews and questionnaires regarding gastroesophageal reflux disease (GERD) and dysphagia were performed. RESULTS: We examined 68 EA adolescents. 62% reported GERD by interview, 22% by questionnaire. 85% reported dysphagia by interview, 71% by questionnaire. 24-hour pH monitoring detected pathological reflux index (RI) (>7%) in 7/59 (12%). By endoscopy with biopsy 62 (92%) had histologic esophagitis, of whom 3 (4%) had severe esophagitis. Gastric metaplasia was diagnosed in twelve (18%) adolescents, intestinal metaplasia in only one (1.5%). None had dysplasia or carcinoma. Dysphagia and GERD were statistically correlated to esophagitis and metaplasia, but none of the questionnaires or interviews alone were good screening instruments with high combined sensitivity and specificity. A compound variable made by simply taking the mean of rescaled RI and dysphagia by interview showed to be the best predictor of metaplasia (85% sensitivity, 67% specificity). CONCLUSION: The questionnaires and interviews used in the present study were not good screening instruments alone. However, combining dysphagia score by interview and RI may be helpful in assessing which patients need endoscopy with biopsy at each individual follow-up examination. LEVEL OF EVIDENCE: Level II prognostic study.

Outcome a decade after laparoscopic and open Nissen fundoplication in children: results from a randomized controlled trial.

BACKGROUND: Randomized controlled trials (RCT) comparing long-term outcome after laparoscopic (LF) and open fundoplication (OF) in children are lacking. Here we report recurrence rates and time to recurrence, frequency of re-interventions, use of antisecretory drugs, gastrointestinal symptoms, and patient/parental satisfaction a decade after children were randomized to LF or OF. METHODS: Cross-sectional long-term follow-up study of a two-center RCT that included patients during 2003-2009. Patients/parents were interviewed and medical charts reviewed for any events that might be related to the fundoplication. If suspicion of recurrence, further diagnostics were performed. Informed consent and ethical approval were obtained. CLINICALTRIALS: gov: NCT01551134. RESULTS: Eighty-eight children, 56 (64%) boys, were randomized (LF 44, OF 44) at median 4.4 [interquartile range (IQR) 2.0-8.9] years. 46 (52%) had neurological impairment. Three were lost to follow-up before first scheduled control. Recurrence was significantly more frequent after LF (24/43, 56%) than after OF (13/42, 31%, p = 0.004). Median time to recurrence was 1.0 [IQR 0.3-2.2] and 5.1 [IQR 1.5-9.3] years after LF and OF, respectively. Eight (19%) underwent redo fundoplication after LF and three (7%) after OF (p = 0.094). Seventy patients/parents were interviewed median 11.9 [IQR 9.9-12.8] years postoperatively. Among these, use of anti-secretory drugs was significantly decreased from preoperatively after both LF (94% vs. 35%, p < 0.001) and OF (97% vs. 19%, p < 0.001). Regurgitation/vomiting were observed in 6% after LF and 3% after OF (p = 0.609), and heartburn in 14% after LF and 17% after OF (p = 1.000). Overall opinion of the surgical scars was good in both groups (LF: 95%, OF: 86%, p = 0.610). Patient/parental satisfaction with outcome was high, independent of surgical approach (LF: 81%, OF: 88%, p = 0.500). CONCLUSIONS: The recurrence rate was higher and recurrence occurred earlier after LF than after OF. Patient/parental satisfaction with outcome after both LF and OF was equally high.

New insights in the prevalence of scoliosis and musculoskeletal asymmetries in adolescents with esophageal atresia.

BACKGROUND: Increased risk of scoliosis and musculoskeletal abnormalities in adolescents with esophageal atresia (EA) is reported, but the impact of these abnormalities on physical fitness and motor skills are not known. METHODS: Scoliosis was assessed radiographically and shoulder and chest abnormalities by a standardized protocol. Physical fitness was evaluated with Grippit, Six-minute walk test, and International Physical Activity Questionnaire and motor skills by Motor Assessment Battery for Children. RESULTS: Sixty-seven EA adolescents median 16 (13-20) years participated. The prevalence of significant scoliosis (≥ 20º) was 12% (8/67) whereas 22% (15/67) had mild scoliosis (10-19º). Vertebral anomalies occurred in 18/67 (27%), eight of them (44%) had scoliosis. The majority of adolescents (15/23) with scoliosis did not have vertebral anomalies. Musculoskeletal abnormalities were detected in 22-78%. Balance problems occurred three times more frequently than expected (44% vs. 15%, p = 0.004). Submaximal exercise capacity was significantly reduced compared to reference values (p < 0.001). Scoliosis ≥ 20º was related to reduced physical activity (p = 0.008), and musculoskeletal abnormalities to reduced physical activity and impaired motor skills (p = 0.042 and p < 0.038, respectively). CONCLUSIONS: Significant scoliosis was diagnosed in 12% of the EA adolescents and related to reduced physical activity. Musculoskeletal abnormalities identified in more than half of the patients, were related to reduced physical activity and impaired motor skills, and exercise capacity was significantly below reference group. EA patients with and without vertebral anomalies need health-promoting guidance to prevent impaired motor skills and consequences of reduced physical activity. LEVEL OF EVIDENCE: Prognostic Study, Level II.

Evaluation of diagnostic factors used to refer children with constipation for rectal biopsies.

PURPOSE: Children with constipation and suspected Hirschsprung's disease are referred for rectal biopsy. Since this is an invasive procedure, appropriate indications should be applied to minimize the number of "unnecessary" biopsies. METHODS: We reviewed all constipated children who underwent a rectal biopsy to diagnose a possible Hirschsprung's disease at a tertiary referral hospital over a 6-year period (2013-2018). We registered clinical and demographic factors in these children and conducted correlation and multivariate regression analysis to evaluate the relation between these factors and a diagnosis of Hirschsprung's disease. RESULTS: We identified 225 children, aged 0-17 years. In total, Hirschsprung's disease was diagnosed in only 49/225 (22%). Among the 49 children with Hirschsprung's disease, 29 (59%) were diagnosed in the neonatal period. Among girls, HD was confirmed in only 10/101 (10%) children, and only 1 of these 10 girls was older than 6 months at the time of the biopsy. The following factors correlated significantly with Hirschsprung's disease diagnosis in children older than 1 month: "male sex", "failure to thrive", "gross abdominal distention plus vomiting" and "fulfils the Rome 4 criteria for functional constipation". CONCLUSION: In children referred for rectal biopsy, the factors most indicative of Hirschsprung's disease were "male sex", "failure to thrive", "gross abdominal distention plus vomiting" and "fulfils the Rome 4 criteria for functional constipation". Notably, the prevalence of Hirschsprung's disease decreased with the increasing age of the children. Girls referred for a biopsy rarely had Hirschsprung's disease, especially those older than 1 month.

High satisfaction on genital self-perception and sexual function in healthy Norwegian male adolescents.

BACKGROUND: Genital self-perception and self-reported outcome on sexual function represent important information in studies focusing on male adolescents born with a genital malformation. Normal data from an age-matched control group are essential for comparison and more knowledge is needed concerning age after puberty and before entering adulthood. OBJECTIVE: To investigate the self-reported outcome on genital perception and sexual outcome of healthy male adolescents aged 16 and 17 years. STUDY DESIGN: Sixty-one individuals were included in this cross-sectional study. The assessment tools included the adolescents' self-report on genital perception and sexual function measured by the Pediatric Penile Perception Score (PPPS) and their responses to a semi-structured interview. In addition, we added information on mental health and psychosocial functioning measured by the Strengths and Difficulties Questionnaire, and health-related quality of life (HRQoL) measured by the Pediatric Quality of Life Inventory. Body satisfaction and self-esteem were also measured by the Global Self-Worth and Physical Appearance subscales of the revised version of the Self-Perception Profile for Adolescents (SPPA). RESULTS: Of the 73 individuals invited, a total of 61 participated. The adolescents reported high satisfaction on genital self-perception and sexual function with a score close to 10 on the overall PPPS score (maximum overall score is 12). Participants who were dissatisfied with their genitals reported penile length, alongside foreskin, as their main concern. More than 90% reported satisfaction on sexual function, concerning erection, masturbation, ejaculation, and orgasm. Results showed a higher score on body satisfaction, self-esteem, mental health and psychosocial functioning and a lower score on HRQoL compared to the normative Norwegian data. The small numbers of individuals that scored more negatively on genital self-perception and sexual function also scored more negatively in all items studied. DISCUSSION: A key finding in our study was their high satisfaction on the overall PPPS score and reported sexual function. The results are comparable to other studies related to healthy male adolescents and the sample studied deviate little from a representative Norwegian sample. A comparison group of age-matched adolescents from the normative population is important to determine long-term outcomes on genital appearance and sexual function of patients born with a genital malformation and operated on in early childhood. Limitations of this study are the small sample-size and the lack of information on non-participants. CONCLUSIONS: Our results show generally positive genital self-perception and sexual function in a healthy group of Norwegian male adolescents, aged 16 and 17.

Impaired motor performance in adolescents with esophageal atresia.

AIMS: The study prospectively assessed motor development from infancy to adolescence in patients with esophageal atresia (EA). METHODS: At one year of age motor performance was evaluated with the Psychomotor Developmental Index (PDI) of the Bayley Scales of Infant Development, Second Edition (BSID-II), and as adolescents reevaluated with Motor Assessment Battery for Children, Second Edition (MABC-2). Associations to clinical factors were assessed. RESULTS: 23 EA patients were followed from infancy to adolescence. The median total PDI score in infancy was 102 (56-118) and the corresponding mean z-score was -0.006 (SD 0.995) and not significantly different from the reference values (p = 0.48). The median total MABC-2 score in adolescence was 75 (32-93) and the corresponding mean z-score -0.43 (SD 0.998) which is significantly below normal (p = 0.03). Children with impaired motor function in adolescence underwent significantly more rethoracotomies than those with normal motor performance (p = 0.037); whereas the two groups did not differ with respect to other clinical characteristics. CONCLUSION: From infancy to adolescence the motor performance in the group of EA patients deteriorated from within normal range to significantly impaired compared to reference values. Interdisciplinary follow-up programs from infancy to adolescence with close monitoring for motor function is necessary to detect motor impairments.

Nutritional Status in Adolescents with Esophageal Atresia.

OBJECTIVES: To examine factors that may affect nutritional status in adolescents with esophageal atresia. STUDY DESIGN: Anthropometric measurements, blood samples, pH measuring, mapping of dysphagia with a modified Easting Assessment Test questionnaire, 4-day dietary record, and a semistructured interview about eating habits and nutrition counseling were performed in a cross-sectional cohort of adolescents with esophageal atresia. RESULTS: Out of 102 eligible patients, 68 (67%) participated. The median height-for-age Z score was -0.6 (-4.6 to 1.8). Ten (15%) were classified as stunted (height-for-age Z score <-2). Fourteen (21%) were overweight. More than two-thirds reported symptoms of dysphagia (Easting Assessment Test score ≥3) and avoided specific foods. Forty-eight (71%) completed the 4-day dietary record, which showed daily intake of energy below age-appropriate recommendations. One-third reported an energy intake below their estimated basal metabolic rate. Only 24% had received counselling from a dietitian. CONCLUSIONS: Adolescents with a history of esophageal atresia have growth below reference values and energy intake below recommendations. Energy intake and its relation to stunting needs to be further studied in patients with esophageal atresia.

Short-term parent reported recovery following open and laparoscopic fundoplication.

BACKGROUND: It is assumed that children recover faster after laparoscopic (LF) than after open fundoplication (OF). As this has not been confirmed in any randomized study (RCT), we have in a subsection of a larger RCT compared parent reported recovery of children after LF and OF. METHODS: Postoperative symptoms, use of analgesics, overall well-being, and time to return to school/day-care were recorded in a subsection of children enrolled in a RCT comparing LF and OF. Ethical approval and parental consent were obtained. RESULTS: Fifty-five children (LF: n = 27, OF: n = 28) of the 88 enrolled in the RCT, were included in the short term follow up on parent reported recovery. Caregivers were interviewed median 28 days [interquartile range (IQR) 22-36] postoperatively. There was no significant difference regarding improvement in overall well-being (LF: 63%, OF: 68%, p = 0.70), new-onset dysphagia (LF: 30%, OF: 18%, p = 0.08), use of analgesics (LF: 15%, OF: 14%, p = 1.00), or time to return to school/day-care (LF: median 7 days [IQR 5-14] vs. OF: 12 days [IQR 7-15], p = 0.35). CONCLUSION: We could not demonstrate faster recovery after LF than after OF. Most children had returned to school/day-care after 2 weeks and had improved overall well-being 1 month after surgery. TYPE OF STUDY: Randomized controlled trial. LEVEL OF EVIDENCE: Level II.

"Too much guts and not enough brains": (epi)genetic mechanisms and future therapies of Hirschsprung disease - a review.

Hirschsprung disease is a neurocristopathy, characterized by aganglionosis in the distal bowel. It is caused by failure of the enteric nervous system progenitors to migrate, proliferate, and differentiate in the gut. Development of an enteric nervous system is a tightly regulated process. Both the neural crest cells and the surrounding environment are regulated by different genes, signaling pathways, and morphogens. For this process to be successful, the timing of gene expression is crucial. Hence, alterations in expression of genes specific for the enteric nervous system may contribute to the pathogenesis of Hirschsprung's disease. Several epigenetic mechanisms contribute to regulate gene expression, such as modifications of DNA and RNA, histone modifications, and microRNAs. Here, we review the current knowledge of epigenetic and epitranscriptomic regulation in the development of the enteric nervous system and its potential significance for the pathogenesis of Hirschsprung's disease. We also discuss possible future therapies and how targeting epigenetic and epitranscriptomic mechanisms may open new avenues for novel treatment.

Psychologic Distress and Anxiety in Mothers of Children With Gastroesophageal Reflux Undergoing Antireflux Surgery.

OBJECTIVES: Parents of children with a chronic illness are at risk for impaired psychosocial functioning. Gastroesophageal reflux disease (GERD) is such a disease, and no studies have investigated effects of antireflux surgery on parental psychological distress. The aims of this study were to assess psychological distress and state and trait anxiety in mothers of children with GERD, and to explore possible changes after antireflux surgery. METHODS: Mothers of children referred for antireflux surgery were included in this prospective study. Standardized questionnaires were used to evaluate psychological distress and state and trait anxiety before and 12 months after antireflux surgery. RESULTS: Of 87 eligible mothers of children with GERD, 62 (71%) agreed to participate. All children had objectively verified GERD by 24-hour pH-monitoring and/or upper gastrointestinal contrast study and unsatisfactory symptom relief of pharmacological treatment. Thirty-one (50%) mothers returned questionnaires postoperatively. Preoperatively, mothers of children undergoing antireflux surgery reported high levels of psychological distress and state anxiety, and 54% had scores indicating clinically significant psychological distress. None of the preoperative child characteristics were found to significantly influence maternal psychological distress or state anxiety. Twelve months postoperatively, both psychological distress and state anxiety were reduced. CONCLUSIONS: Mothers of children undergoing antireflux surgery reported reduced levels of psychological distress and state anxiety 12 months after the operation.

Surgical repair of long-gap esophageal atresia: A retrospective study comparing the management of long-gap esophageal atresia in the Nordic countries.

BACKGROUND: Several surgical procedures have been described in the reconstruction of long-gap esophageal atresia (LGEA). We reviewed the surgical methods used in children with LGEA in the Nordic countries over a 15-year period and the postoperative complications within the first postoperative year. METHODS: Retrospective multicenter medical record review of all children born with Gross type A or B esophageal atresia between 01/01/2000 and 12/31/2014 reconstructed within their first year of life. RESULTS: We included 71 children; 56 had Gross type A and 15 type B LGEA. Delayed primary anastomosis (DPA) was performed in 52.1% and an esophageal replacement procedure in 47.9%. Gastric pull-up (GPU) was the most frequent procedure (25.4%). The frequency of chromosomal abnormalities, congenital heart defects and other anomalies was significantly higher in patients who had a replacement procedure. The frequency of gastroesophageal reflux (GER) was significantly higher after DPA compared to esophageal replacement (p = 0.013). At 1-year follow-up the mean body weight was higher after DPA than after organ interposition (p = 0.043). CONCLUSION: DPA and esophageal replacement procedures were equally applied. Postoperative complications and follow-up were similar except for the development of GER and the body weight at 1-year follow-up. Long-term results should be investigated. TYPE OF STUDY: Treatment study. LEVEL OF EVIDENCE: Level III.

Outcome for biliary atresia patients treated at a low-volume centre.

OBJECTIVES: The importance of case load for treatment of extrahepatic biliary atresia (BA) is debated. The aim of this study was to register results of BA treatment in a small volume centre. METHODS: Retrospective chart review study of patients with BA treated from 2000 to 2017. The institutional review board approved the study. RESULTS: Forty-five babies were identified of which 42 (93%) are alive. Forty-one patients had a Kasai portoenterostomy (PE), two had a hepaticojejunostomy and two had a primary liver transplantation. The age at PE/hepaticojejunostomy was median 63 (4-145 days). Seven surgeons performed the operations, and the median duration of the diagnostic work-up was 8 (3-24) days. Clearance of jaundice was achieved in 23/43 (53%) babies, and 3- and 5-year native liver survival was 47% and 40%, respectively. Clearance of jaundice post-PE/hepaticojejunostomy was a strong predictor of native liver survival (adjusted OR: 0.027; 95%; p = .009). Plasma level of conjugated bilirubin at time of referral was also a significant predictor of native liver survival (adjusted OR: 1.053; p = .017). CONCLUSION: A small volume centre may achieve satisfactory results for BA patients. The study has, however, identified factors that may further improve results; earlier referral, optimizing diagnostic work-up and establishing one dedicated surgical team.

Initial experience with percutaneous endoscopic gastrostomy with T-fastener fixation in pediatric patients.

BACKGROUND AND STUDY AIMS: Insertion of a percutaneous endoscopic gastrostomy (PEG) with push-through technique and T-fastener fixation (PEG-T) has recently been introduced in pediatric patients. The T-fasteners allow a primary insertion of a balloon gastrostomy. Due to limited data on the results of this technique in children, we have investigated peri- and postoperative outcomes after implementation of PEG-T in our department. PATIENTS AND METHODS: This retrospective chart review included all patients below 18 years who underwent PEG-T placement from 2010 to 2014. Main outcomes were 30-day postoperative complications and late gastrostomy-related complications. RESULTS: In total, 87 patients were included, and median follow-up time was 2.4 years (1 month - 4.9 years). Median age and weight at PEG-T insertion were 1.9 years (9.4 months - 16.4 years) and 10.4 kg (5.4 - 33.0 kg), respectively. Median operation time was 28 minutes (10 - 65 minutes), and 6 surgeons and 3 endoscopists performed the procedures. During the first 30 days, 54 complications occurred in 41 patients (47 %). Most common were peristomal infections treated with either local antibiotics in 11 patients (13 %) or systemic antibiotics in 11 other patients (13 %). 9 patients (10 %) experienced tube dislodgment. Late gastrostomy-related complications occurred in 33 patients (38 %). The T-fasteners caused early and late complications in 9 (10 %) and 11 patients (13 %), respectively. Of these, 4 patients (5 %) had subcutaneously migrated T-fasteners which were removed under general anesthesia. CONCLUSION: We found a high rate of complications after PEG-T. In particular, problems with the T-fasteners and tube dislodgment occurred frequently after PEG-T insertion.

Outcomes of biliary atresia in the Nordic countries - a multicenter study of 158 patients during 2005-2016.

BACKGROUND/PURPOSE: Biliary atresia is the most common reason for newborn cholestasis and pediatric liver transplantation. Even after normalization of serum bilirubin after portoenterostomy, most patients require liver transplantation by adulthood due to expanding fibrosis. We addressed contemporary outcomes of biliary atresia in the Nordic countries. METHODS: Data on center and patients characteristics, diagnostic practices, surgical treatment, adjuvant medical therapy after portoenterostomy, follow-up and outcomes were collected from all the Nordic centers involved with biliary atresia care during 2005-2016. RESULTS: Of the 154 patients, 148 underwent portoenterostomy mostly by assigned surgical teams at median age of 64 (interquartile range 37-79) days, and 95 patients (64%) normalized their serum bilirubin concentration while living with native liver. Postoperative adjuvant medical therapy, including steroids, ursodeoxycholic acid and antibiotics was given to 137 (93%) patients. Clearance of jaundice associated with young age at surgery and favorable anatomic type of biliary atresia, whereas annual center caseload >3 patients and diagnostic protocol without routine liver biopsy predicted early performance of portoenterostomy. The cumulative 5-year native liver and overall survival estimate was 53% (95% CI 45-62) and 88% (95% CI 83-94), respectively. Portoenterostomy age <65days and annual center caseload >3 patients were predictive for long-term native liver survival, while normalization of serum bilirubin after portoenterostomy was the major predictor of both native liver and overall 5-year survival. CONCLUSIONS: The outcomes of biliary atresia in the Nordic countries compared well with previous European studies. Further improvement should be pursued by active measures to reduce patient age at portoenterostomy. RETROSPECTIVE PROGNOSIS STUDY: Level II.

Long-term psychosocial consequences of surgical congenital malformations.

Surgical congenital malformations often represent years of treatment, large number of hospital stays, treatment procedures, and long-term functional sequels affecting patients' psychosocial functioning. Both functional defects and psychosocial difficulties that occur commonly in childhood may pass through adolescence on to adulthood. This overview presents reports published over the past 3 decades to elucidate the long-term psychosocial consequences of surgical congenital malformations. Literature searches conducted on PubMed database revealed that less than 1% of all the records of surgical congenital malformations described long-term psychosocial consequences, but with diverse findings. This inconsistency may be due to methodological differences or deficiencies; especially in study design, patient sampling, and methods. Most of the studies revealed that the functional deficits may have great impact on patients' mental health, psychosocial functioning, and QoL; both short- and long-term negative consequences. Factors other than functional problems, e.g., repeated anesthesia, multiple hospitalization, traumatic treatment procedures, and parental dysfunctioning, may also predict long-term mental health and psychosocial functioning. Through multidisciplinary approach, pediatric surgeons should also be aware of deficits in emotional and psychosocial functioning. To achieve overall optimal psychosocial functioning, the challenge is to find a compromise between physically optimal treatment procedures and procedures that are not psychologically detrimental.

A Nordic multicenter survey of long-term bowel function after transanal endorectal pull-through in 200 patients with rectosigmoid Hirschsprung disease.

OBJECTIVE: Transanal endorectal pull-through (ERPT) is the most popular technique to treat Hirschsprung disease (HD). Still, there is limited knowledge on long-term bowel function. This cross-sectional, multicenter study assessed long-term bowel function in a large HD population and examined predictors of poor outcome. METHODS: Patients older than four years or their parents filled out a validated questionnaire on bowel function. Clinical details were recorded retrospectively from medical records. RESULTS: 73/200 (37%) patients reported absolutely no impaired bowel function, meaning no constipation, fecal accidents, stoma, appendicostomy or need for enemas. Seven (4%) had a stoma, and 33 (17%) used antegrade or rectal colonic enemas. Most disarrangements of fecal control and constipation were significantly less common in older age group, but abnormal defecation frequency and social problems remained unchanged. Syndromic patients (n=31) experienced frequent fecal accidents (46%) more often than nonsyndromic (14%, P<0.001). Having a syndrome (adjusted OR 5.6, 95% CI 2.1-15, P=0.001) or a complete transanal ERPT (adjusted OR 2.4, 95% CI 1.1-5.7, P=0.038) was significantly associated with poor outcome defined as having a stoma, an appendicostomy, daily fecal accidents or need of regular rectal wash outs. CONCLUSION: A significant number of HD patients experience bowel problems many years after definite surgery. Fecal control was significantly better in older than younger HD patients, but some continued to have considerable bowel problems also as adults. A total transanal ERPT was associated with poorer outcome. Long-term follow-up of HD patients is warranted. Prognosis Study: Level II.

Lower urinary tract symptoms in children with anorectal malformations with rectoperineal fistulas.

AIM: The aim was to describe the frequency of lower urinary tract symptoms (LUTS) in children with anorectal malformations with rectoperineal fistulas (ARM-P), as compared with healthy controls based on gender. METHOD: LUTS were defined using the 2014 definitions of the International Children's Continence Society. Data were collected at 2 tertiary pediatric surgery centers in 2 countries from all children aged 4-12years who had undergone an operation for ARM-P. RESULTS: A total of 24 girls and 33 boys, with a median age of 8 (4-12)years, were eligible and compared with 165 controls. Of the patient group, 4 (17%) girls had 8 urinary tract anomalies (UTA), and 8 (24%) boys had 13 UTA. There were no gender differences in LUTS among the patients. The frequency of urinary tract infections was higher among the patients (5/24 girls and 7/55 boys) than the controls (1/55 and 4/110) (p=0.009). More patients (5/24 girls and 5/33 boys) than controls (1/55 and 2/110) used daily urinary medications (p=0.009 and p=0.007, respectively). Patients with UTA reported urinary infections more frequently (3/4 girls and 4/8 boys) than those without UTA (2/20 girls and 0/25 boys) (p=0.018 and p=0.002, respectively). CONCLUSION: Children with ARM-P had more LUTS than controls, and patients with concomitant UTA had more LUTS than patients without UTA. Therefore, children with ARM-P are suggested to have routine follow-up for both UTA and LUTS.

Nissen fundoplication in children with and without neurological impairment: A prospective cohort study.

BACKGROUND: It is assumed that children with neurological impairment (NI) have inferior results after fundoplication compared to those without NI (non-NI). The aim of this study was to assess outcome after fundoplication in children with and without NI. METHODS: 87/105 patients (46 NI, 41 non-NI) undergoing fundoplication between 2003 and 2009 were included in this prospective two-center cohort study. Complications occurring within the first 30days were scored from 0 to 100 by the comprehensive complication index (CCI). Follow-up included clinical examination, upper gastrointestinal contrast study and 24-h pH monitoring 6months postoperatively, then phone-interviews 1, 2 and 4years later. RESULTS: There were no statistical differences in age (NI 3.1 years [0.2-15.2] vs non-NI 5.0 years [0.4-15], p=.14) or in total CCI score (NI 20.9 [0-44.9] vs non-NI 8.7 [0-40.6], p=.57). Hospital stay was longer for NI children (9days [4-57] vs non-NI: 4days [2-16], p<0.001). More than 90% of parents in both groups reported that the fundoplication had improved the child's overall condition. Recurrence of gastroesophageal reflux disease (GERD) was diagnosed in 12 NI and 7 non-NI patients (p=.31). CONCLUSIONS: Early complications, GERD recurrence, and long-term parental satisfaction after fundoplication did not differ between NI and non-NI patients.

Trends in the use of gastrostomies at a tertiary paediatric referral centre.

OBJECTIVES: The aims of this study were to describe the population of paediatric patients undergoing gastrostomy placement at a Norwegian tertiary referral centre and to investigate trends over time in patient characteristics and operative technique. MATERIALS AND METHODS: Patients <15 years of age getting a primary gastrostomy from 1994 to 2012 were included in this retrospective observational study. Patient data were collected from medical records and the National Registry. RESULTS: Six-hundred forty-nine patients with a median age of 1.2 years [gestational week 30-14.9 years] were included. Neurological disorders (ND) was the most common underlying group of diagnosis (n = 311, 48%), followed by cardiac disease 104 (16%), congenital anomalies 85 (13%), respiratory disease 43 (7%), malignancy 29 (5%), and others 77 (12%). At follow-up, 162 (25%) patients were dead. A percutaneous endoscopic technique (PEG) was used in 401 (62%) patients, open surgery (OPEN) in 201 (31%) and laparoscopy (LAP) in 47 (7%). The number of gastrostomies per year more than doubled during the period (p < 0.001). More patients with cardiac disease and congenital anomalies were given a gastrostomy during the last years (all p < 0.05), whereas the number of patients with ND remained stable. Furthermore, there has been a decrease in median age and an increase in the number of PEG and LAP (p < 0.05). CONCLUSION: The number of gastrostomy insertions has increased from 1994 to 2012. NDs is the most common underlying diagnosis in patients receiving a gastrostomy, PEG is the most common technique and patient characteristics have changed during the study period.