Development of a core outcome set for congenital pulmonary airway malformations: study protocol of an international Delphi survey.

IntroductionA worldwide lack of consensus exists on the optimal management of asymptomatic congenital pulmonary airway malformation (CPAM) even though the incidence is increasing. Either a surgical resection is performed or a wait-and-see policy is employed, depending on the treating physician. Management is largely based on expert opinion and scientific evidence is scarce. Wide variations in outcome measures are seen between studies making comparison difficult thus highlighting the lack of universal consensus in outcome measures as well. We aim to define a core outcome set which will include the most important core outcome parameters for paediatric patients with an asymptomatic CPAM.Methods and analysisThis study will include a critical appraisal of the current literature followed by a three-stage Delphi process with two stakeholder groups. One surgical group including paediatric as well as thoracic surgeons, and a non-surgeon group including paediatric pulmonologists, intensive care and neonatal specialists. All participants will score outcome parameters according to their level of importance and the most important parameters will be determined by consensus.Ethics and disseminationElectronic informed consent will be obtained from all participants. Ethical approval is not required. After the core outcome set has been defined, we intend to design an international randomised controlled trial: the COllaborative Neonatal NEtwork for the first CPAM Trial, which will be aimed at determining the optimal management of patients with asymptomatic CPAM

Cystoscope-guided trans-anal Fistula-tract Laser Closure (FiLaC) of late onset recurrent recto-urethral fistula post anorectal malformation repair: A case report

Introduction: Management of persistent and recurrent rectourethral fistula after primary treatment of anorectal malformations (ARM) is challenging. Various surgical techniques have been proposed and until today there is no consensus on the optimal approach. Case presentation: We describe a case of late onset recurrent rectourethral fistula after neonatal ARM repair, treated with a minimally invasive cystoscope-guided trans-anal Fistula-tract Laser Closure (FiLaC). A male patient suffering from ARM, type recto-bulbar fistula, was treated with an anorectoplasty during the neonatal period. Post-operatively, no reoperations were needed and long term follow-up demonstrated good functional outcome. At the age of 19 years, the patient presented with complaints of anal urinary leakage during micturition. Voiding-cysto-urethrography and perineal Nuclear Magnetic Resonance (NMR) demonstrated a fistula tract between the bulbar urethra and the distal rectum. Given the lack of associated urinary tract infections or fecaluria and the absence of fecal or urinary incontinence in between micturition, we decided to perform a trans-luminal, non-surgical approach. By analogy with the minimally invasive laser treatment of peri-anal fistula in adults, we performed a trans-anal cystoscope-guided FiLaC. We observed a fast post-operative recovery with an excellent one-year follow-up, illustrated by the elimination of symptoms, absence of fistula opening on rectoscopy and disappearance of the fistula-tract continuity on voiding-cysto-urethrography. Conclusion: This case exemplifies the potential benefits of using minimally invasive FiLaC treatment for the management of recurrent and persistent recto-urethral fistula following primary treatment of ARM. Long term follow-up is awaited to confirm the value of FiLaC in this treatment setting