Substitution Phalloplasty in Patients With Bladder Exstrophy-Epispadias Complex: A Systematic Review of Techniques, Complications and Outcomes

Background:Males born with bladder exstrophy-epispadias complex generally have a shorter phallus, splitcorpora with dorsal curvature, and a scarred andflattened glans, so substitution phalloplasty is often required.Aim:The aim of this study was to review the techniques, complications, and outcomes of substitution phal-loplasty in bladder exstrophy-epispadias complex patients to determine the ideal surgical procedure and gauge therisks and benefits for the patient.Methods:A systematic review of the literature was performed using PubMed/MEDLINE and the CochraneLibrary with the following terms: (“phalloplasty”); ((“epispadias”)OR(“bladder exstrophy”)OR(“cloacal exs-trophy”)). We included only full-text articles reporting data about techniques and outcomes of substitutionphalloplasty in patients with bladder exstrophy-epispadias complex.Outcomes:To determine whether patients with bladder exstrophy-epispadias complex might benefit fromsubstitution phalloplasty.Results:We selected 7 studies involving 47 patients. All the studies were characterized by a low level of evidenceand a heterogeneous approach during treatment and outcome assessment. The free radial forearmflap was themost commonly performed technique (89%) with an overall complication rate of 15%. Urethroplasty wasperformed in 22 of 47 (47%) patients, and in most cases (20/22) a“tube-within-the-tube”technique wasperformed simultaneously with the phalloplasty (20/47). Urethroplasty complications were recorded in 12 of 22(54%) patients with 6fistulae and 6 stenoses. A penile prosthesis was implanted in 32 of 47 (68%) patients andcomplications occurred in 8 of 32 (25%) patients with 6 erosion. Aesthetic, sexual, and psychological outcomeswere satisfactory, but none of the studies used validated instruments for thefinal assessment.Clinical Implications:It was not possible to formulate any recommendations based on a high level of evidenceregarding substitution phalloplasty in patients with bladder exstrophy-epispadias complex.Strength & Limitation:To our knowledge, this is thefirst review to address bladder exstrophy-epispadiascomplex patients only. The limitations are mainly represented by the small number of cases because of therarity of this disease and by the fact that no studies used validated instruments.Conclusion:Substitution phalloplasty in patients with bladder exstrophy-epispadias complex can achieve goodfunctional, aesthetic, psychological, and sexual outcomes. It requires multiple procedures and carries a highcomplication rate. Multicentric studies including the assessment of patients by means of a validated questionnairewhich investigates both sexual function and psychosexual satisfaction are required.