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.

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.

ERNICA Consensus Conference on the Management of Patients with Long-Gap Esophageal Atresia: Perioperative, Surgical, and Long-Term Management.

INTRODUCTION: Evidence supporting best practice for long-gap esophageal atresia is limited. The European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) organized a consensus conference on the management of patients with long-gap esophageal atresia based on expert opinion referring to the latest literature aiming to provide clear and uniform statements in this respect. MATERIALS AND METHODS: Twenty-four ERNICA representatives from nine European countries participated. The conference was prepared by item generation, item prioritization by online survey, formulation of a final list containing items on perioperative, surgical, and long-term management, and literature review. The 2-day conference was held in Berlin in November 2019. Anonymous voting was conducted via an internet-based system using a 1 to 9 scale. Consensus was defined as ≥75% of those voting scoring 6 to 9. RESULTS: Ninety-seven items were generated. Complete consensus (100%) was achieved on 56 items (58%), e.g., avoidance of a cervical esophagostomy, promotion of sham feeding, details of delayed anastomosis, thoracoscopic pouch mobilization and placement of traction sutures as novel technique, replacement techniques, and follow-up. Consensus ≥75% was achieved on 90 items (93%), e.g., definition of long gap, routine pyloroplasty in gastric transposition, and avoidance of preoperative bougienage to enable delayed anastomosis. Nineteen items (20%), e.g., methods of gap measurement were discussed controversially (range 1-9). CONCLUSION: This is the first consensus conference on the perioperative, surgical, and long-term management of patients with long-gap esophageal atresia. Substantial statements regarding esophageal reconstruction or replacement and follow-up were formulated which may contribute to improve patient care.

Surgical treatment for urinary incontinence in children. / Kirurgisk behandling ved urininkontinens hos barn.

Only a small proportion of children with urinary tract malformations are incontinent, but it is important to identify those children for whom surgical treatment can eliminate or reduce urinary leakage.

ERNICA Consensus Conference on the Management of Patients with Esophageal Atresia and Tracheoesophageal Fistula: Diagnostics, Preoperative, Operative, and Postoperative Management.

INTRODUCTION: Many aspects of the management of esophageal atresia (EA) and tracheoesophageal fistula (TEF) are controversial and the evidence for decision making is limited. Members of the European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) conducted a consensus conference on the surgical management of EA/TEF based on expert opinions referring to the latest literature. MATERIALS AND METHODS: Nineteen ERNICA representatives from nine European countries participated in the conference. The conference was prepared by item generation, item prioritization by online survey, formulation of a final list containing the domains diagnostics, preoperative, operative, and postoperative management, and literature review. The 2-day conference was held in Berlin in October 2018. Anonymous voting was conducted via an internet-based system. Consensus was defined when 75% of the votes scored 6 to 9. RESULTS: Fifty-two items were generated with 116 relevant articles of which five studies (4.3%) were assigned as level-1evidence. Complete consensus (100%) was achieved on 20 items (38%), such as TEF closure by transfixing suture, esophageal anastomosis by interrupted sutures, and initiation of feeding 24 hours postoperatively. Consensus ≥75% was achieved on 37 items (71%), such as routine insertion of transanastomotic tube or maximum duration of thoracoscopy of 3 hours. Thirteen items (25%) were controversial (range of scores, 1-9). Eight of these (62%) did not reach consensus. CONCLUSION: Participants of the conference reached significant consensus on the management of patients with EA/TEF. The consensus may facilitate standardization and development of generally accepted guidelines. The conference methodology may serve as a blueprint for further conferences on the management of congenital malformations in pediatric surgery.

ERNICA Consensus Conference on the Management of Patients with Esophageal Atresia and Tracheoesophageal Fistula: Follow-up and Framework.

INTRODUCTION: Improvements in care of patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) have shifted the focus from mortality to morbidity and quality-of-life. Long-term follow-up is essential, but evidence is limited and standardized protocols are scarce. Nineteen representatives of the European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) from nine European countries conducted a consensus conference on the surgical management of EA/TEF. MATERIALS AND METHODS: The conference was prepared by item generation (including items of surgical relevance from the European Society for Pediatric Gastroenterology Hepatology and Nutrition (ESPGHAN)-The North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) guidelines on follow-up after EA repair), item prioritization, formulation of a final list containing the domains Follow-up and Framework, and literature review. Anonymous voting was conducted via an internet-based system. Consensus was defined as ≥75% of those voting with scores of 6 to 9. RESULTS: Twenty-five items were generated in the domain Follow-up of which 17 (68%) matched with corresponding ESPGHAN-NASPGHAN statements. Complete consensus (100%) was achieved on seven items (28%), such as the necessity of an interdisciplinary follow-up program. Consensus ≥75% was achieved on 18 items (72%), such as potential indications for fundoplication. There was an 82% concordance with the ESPGHAN-NASPGHAN recommendations. Four items were generated in the domain Framework, and complete consensus was achieved on all these items. CONCLUSION: Participants of the first ERNICA conference reached significant consensus on the follow-up of patients with EA/TEF who undergo primary anastomosis. Fundamental statements regarding centralization, multidisciplinary approach, and involvement of patient organizations were formulated. These consensus statements will provide the cornerstone for uniform treatment protocols and resultant optimized patient care.

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.

Ultrasound evaluation of prognosis in fetuses with posterior urethral valves.

PURPOSE: The aim of this study was to evaluate the ability of prenatal ultrasound markers to predict postnatal renal prognosis in fetuses with posterior urethral valves. METHODS: Medical files on fetuses with prenatal diagnosis of posterior urethral valves from 2000 to 2006 were reviewed retrospectively. Data from prenatal follow-up included gestational age at diagnosis, ultrasound renal parenchyma evaluation, and presence and time of oligohydramnios onset. Prenatal parameters studied were correlated to postnatal renal function. RESULTS: Thirty-one male fetuses were included. Six pregnancies were terminated. Of the remaining 25 pregnancies that were continued, 4 children had abnormal creatine and 21 normal creatinine levels at follow-up. Presence and time of oligohydramnios onset did not differ between groups (P = .43). Ultrasound detected bilateral renal abnormalities in 3 fetuses (75%) with altered renal function, and 10 fetuses (55%) with normal creatinine, at follow-up. CONCLUSIONS: None of the ultrasound parameters evaluated were able to reliably predict postnatal renal function.

[Hypospadias--not only a misplaced urinary meatus]. / Hypospadi--ikke bare feilplassert urinrørsåpning.

BACKGROUND: Hypospadias is a congenital malformation with a complex and unclear etiology; both hormonal disturbances and genetic factors may contribute. It is discussed internationally whether the incidence of the malformation has increased during the last decades. There are many operation methods for treatment of hypospadias. MATERIAL AND METHODS: Incidence, possible etiologic factors and the clinical presentation of hypospadias is discussed, based on personal experience and review of literature retrieved from PubMed. Principles of treatment are outlined. RESULTS AND INTERPRETATION: The diagnosis is usually made during a routine examination after birth. The less severe cases of hypospadias can be overlooked. Most of them ( 2 / 3 ) are of a distal type and lack foreskin coverage of the ventral side of the penis head and there is often a ventral curvature of the penis. Many currently recommend the operation to be taken from 6 to 18 months of age, but it may be technically more challenging to operate such young boys than to postpone it to when they are older. The operational method is adapted to the type of hypospadias and the aim is to achieve a satisfactory cosmetic and functional result and a low incidence of postoperative complications.

Primary megaureter: results of surgical treatment.

BACKGROUND: The aim of this study was to assess the clinical and radiological outcome after surgical treatment for primary -megaureter. METHODS: A retrospective analysis of 16 patients who had reimplantation of 17 primary megaureters between January 1997 and April 2001 was performed. Patients who had additional urinary tract pathology were not included in this study. The intent of the study was to focus on the complex range of data points that were used to reach the decision to operate. RESULTS: Six of eight patients with febrile urinary tract infections prior to surgery have been without infections postoperatively. One patient who presented with daytime wetting has less wetting postoperatively, and one baby who presented with failure to thrive and postfeed vomiting, is feeding well postoperatively. On ultrasound investigation, the median diameter of the megaureter prior to operation was 11 mm (range 6-24 mm). One month after the operation, dilatation of the ureter could no longer be demonstrated in 10, was reduced in six, and increased in one of 17 ureters. Three months postoperatively, hydroureter was no longer detected in 14 and was reduced in three. In comparison, the hydronephrosis 1 month postoperatively was reduced in six, unchanged in seven and worse in four out of 17 kidneys, and 3 months after the operation, hydronephrosis was reduced in 13 and was unchanged in four compared with preoperative findings. Renograms at 3 months postoperatively showed free drainage in the 11 kidneys with impaired drainage preoperatively, and drainage continued to be normal in all six kidneys with normal drainage preoperatively. Median pre-operative renographic split function was 47% (range 39-54%), and was not significantly changed 3 months after the operation. CONCLUSIONS: When the decision to surgically intervene was made on a multifactorial basis, reimplantation of a primary mega-ureter resulted in improved clinical status, reduced dilation of the ureter and renal pelvis, and free drainage of the upper urinary tract.

Appendicostomy for antegrade enema: effects on somatic and psychosocial functioning in children with myelomeningocele.

OBJECTIVE: To assess changes in somatic variables, psychosocial functioning, mental health and self-esteem after appendicostomy for antegrade enema (MACE) in children with myelomeningocele (MMC). METHODS: We performed the MACE procedure in 20 children, aged 6.3 to 17.0 years. Twelve patients had an open and 8 had a laparoscopic operation. Somatic function was assessed preoperatively and 6 and 16 months postoperatively. Psychosocial functioning and mental health were assessed preoperatively and 6 months postoperatively by a structured clinical interview and standardized questionnaires, Youth Self-Report, Child Behavior Checklist, and Harter's Self-Perception Profile for Adolescents. RESULTS: Preoperatively, 9 patients had fecal leaks several times a week, 7 had leaks 1 to 4 times per month, and 4 were continent. The corresponding numbers 6 and 16 months postoperatively were 0, 6, and 14 and 0, 3, and 16. Six patients have had postoperative stoma complications that required surgery; 5 had a well-functioning stoma thereafter, and 1 had a colostomy 7 months later. Preoperatively, 14 patients reported considerable, 4 moderate, 2 minor, and 0 no psychosocial problems related to bowel control and emptying regimen. Six months postoperatively, the corresponding numbers were 1, 2, 6, and 11. The standardized questionnaires revealed substantial psychopathology in the MMC patients. The Child Behavior Checklist and Youth Self-Report scores were not significantly changed postoperatively, but global self-esteem score and close friends score from the Self-Perception Profile for Adolescents were significantly improved. CONCLUSIONS: Fecal incontinence and constipation were greatly reduced by MACE. Children with MMC often have psychological and psychosocial problems, but important improvements in self-esteem and psychosocial function were observed 6 months after the MACE procedure.