BackgroundIn 2016, we reported the first case of high flow priapism and arteriocavernosal fistula caused by penile prosthesis insertion that mimicked device autoinflation. AimTo raise awareness amongst implanters, we describe further cases from our institution and perform a systematic review of the literature to understand the rarity of this phenomenon. MethodsPatient demographics, management and outcomes were extracted retrospectively. A systematic search of the EMBASE, PubMed and PubMed Central libraries for studies reporting arteriocavernosal fistula mimicking autoinflation since 1946 was performed. OutcomesTo identify and report all known cases of high flow priapism and arteriocavernosal fistula presenting as autoinflation of an inflatable penile prosthesis. ResultsFour patients in total (median age 56, range 46-60 years) were identified. Catastrophic bleeding (1.8L) occurred during revision surgery for presumed autoinflation in Patient 1 and subsequent ultrasound (US) confirmed a fistula which was embolized. Patient 2 redeveloped autoinflation following revision surgery. Ultrasound confirmed high flow priapism from an arteriocavernosal fistula. Patient 3 underwent penile magnetic resonance imaging (MRI) to investigate autoinflation and residual penile curvature. MRI showed a tumescent penis despite a deflated device and the fistula was embolized successfully. Patient 4 with sleep-related painful erections did not improve following insertion of penile prosthesis. Doppler US identified 2 fistulae that was embolized but with no resolution of symptoms. Subsequent embolization of both common penile arteries were done to control his symptoms. No other publications apart from the published abstract from 2016 reporting patient 1 was found. Clinical ImplicationsIf considered prior to revision surgery, the fistula can be managed safely by minimally invasive percutaneous angioembolisation avoiding surgery which can potentially be associated with significant complications. Strengths and LimitationsThe rarity of this phenomenon was supported by a systematic review. Our study however does present the findings from a small number of patients. ConclusionDamage to the cavernosal artery during inflatable penile prosthesis insertion can create an arteriocavernosal fistula that mimics autoinflation, leading to catastrophic intra-operative bleeding or unnecessary surgery.Lee WG, Satchi M, Skrodzka M, et al. A Rare Cause of Autoinflation after Penile Prosthesis Insertion: Case Series and Systematic Review. J Sex Med 2022;19:879–886.