Highly Destructive Perianal Disease in Children with Crohn's Disease
Authors: Markowitz James , Grancher Kathleen , Rosa Joanne , Simpser Edwin , Aiges Harvey , Daum Fredric 
Published: January 2024

The perianal complications of Crohn's disease (CD) seen in children and adolescents include skin tags, anal fissures, fistulae, and abscesses. White these lesions are often chronic and variably responsive to medical therapy, only rarely are they severely destructive. In this report, we characterize the frequency, severity, and clinical course of a highly destructive form of perianal disease (HDPD) that we have noted in a number of children and adolescents with Crohn's disease. A database containing records from 350 children with inflammatory bowel disease was reviewed to identify all children with CD treated between 1970 and 1993. For each, the occurrence or absence of significant perianal pathology, including fistula, abscess, and HDPD, was determined. Pertinent clinical details were recorded for all patients. In addition, the clinical characteristics of those children with HDPD were compiled, and the courses of those with HDPD characterized. A search of the database identified 230 children and adolescents with CD followed for a total of 1,518 patient years. Sixty‐seven of these patients (29% of the CD population) had significant perianal pathology. This included 6 with HDPD, 8 with complicated fistuale [rectourethroperineal (1), rectovaginal (1), rectolabial (2), and multiple communicating perineal (4)], and 53 with simple perianal fistulae or abscesses. All six with HDPD had deeply destructive perineal ulcerations, marked undermining of the perineal and perirectal tissues, and copious exudate, and often there was a deeply cleaved or fileted perineum on separating the buttocks. Two children with HDPD had fecal incontinence. Apart from race (five of six with HDPD were black), there were no significant demographic characteristics that differentiated the children with HDPD from those with perianal fistulae and abscesses. No patient with HDPD had had anal intercourse, lymphogranuloma venereum, condylomata accuminata, or sarcoidosis. None had been sexually abused. In five of six patients with HDPD, perianal disease was the initial and primary manifestation of CD. Three patients failed aggressive medical management [intravenous (iv)/oral (po)/intrarectal corticosteroids + metronidazole (MTZ) (2); iv/po cyclosporine + MTZ + 6‐mercaptopurine (1)] and surgery was performed [diverting colostomy (2) with eventual reanastomosis (1), total proctocolectomy (1)]. The HDPD healed completely in two of the three operated patients but remains active, although much improved, 15 months after colostomy in the third. Two patients improved with iv steroids + MTZ and were left with shallow fissures and skin tags or persistent, shallow perianal ulcerations. The remaining child had unchanged HDPD after 2 years of inconsistent MTZ therapy. HDPD is an unusual but important perianal complication of CD for which black children may be at increased risk. The perianal disease is a cause of significant morbidity, is highly resistant to medical therapy, and in some cases only slowly improves following diversion of the fecal stream. Why HDPD occurs is unknown, but the extent of destruction and the resistance to medical therapy make HDPD a difficult problem with a poor prognosis.