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Background Penile and scrotal swelling can occur as an extraintestinal manifestation of Crohn's disease (CD) and is thought to be an uncommon form of metastatic CD (MCD). Because of the rarity of this manifestation, much is unknown concerning the presentation, treatment, and response to therapy in children with genital MCD. Methods Boys ages 1 to 17 years presenting with genital edema and a confirmed diagnosis or strong suspicion of CD who were evaluated at the Mayo Clinic between 1996 and 2014 were included for review. We sought to characterize the clinical and pathologic features of genital MCD and response to treatment in our cohort of patients. Results Eight patients with genital MCD were identified from our institution (mean age 11.4 yrs, range 7-16 yrs). Seven (88%) patients experienced cutaneous symptoms before a formal diagnosis of CD was made, and two of the seven had no adverse gastrointestinal symptoms at that time. Patients were prescribed an average of 3.4 medications (topical and systemic) for management of their gastrointestinal CD and MCD. Conclusions Penile and scrotal swelling can occur as an extraintestinal manifestation of CD and is thought to be an uncommon form of MCD. In boys, genital swelling typically precedes intestinal CD. Treatment of the underlying CD with systemic medications was most helpful in this series. An evaluation for CD is necessary in all patients presenting with unexplained genital swelling.

Genital edema was seen as a complication of continuous ambulatory peritoneal dialysis (CAPD) in 18 patients, who accounted for 4 per cent of all patients on CAPD. In the majority of patients, the swelling developed suddenly and painlessly in the scrotum, penis or labia majora. Some of the patients noticed ultrafiltration failure. In the majority of these patients, this was a result of fluid leakage through the patent processus vaginalis. In one female patient, fluid leaked through the implantation site of the catheter. A computed tomographic scan using contrast media is a useful diagnostic tool in identifying the side of the defect in patients with generalized scrotal or penile edema. Surgical repair of the hernia and the hole in the processus vaginalis or hernia sac prevented recurrence. A trial of temporary discontinuation of CAPD or intermittent peritoneal dialysis using small volumes of dialysis fluid prevented recurrence while reinstituting CAPD in patients with normal findings from contrast studies. Intermittent peritoneal dialysis using low volumes of dialysis fluid with stepwise increase prevented recurrence while reinstituting CAPD two to three weeks after surgical repair.