HIGHLY DESTRUCTIVE PERIANAL DISEASE IN CHILDREN WITH CROHNS-DISEASE

The perianal complications of Crohn's disease (CD) seen in children an d adolescents include skin tags, anal fissures, fistulae, and abscesse s. While these lesions are often chronic and variably responsive to me dical therapy, only rarely are they severely destructive. In this repo rt, 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 databa se containing records from 350 children with inflammatory bowel diseas e was reviewed to identify all children with CD treated between 1970 a nd 1993. For each, the occurrence or absence of significant perianal p athology, including fistula, abscess, and HDPD, was determined. Pertin ent clinical details were recorded for all patients. In addition, the clinical characteristics of those children with HDPD were compiled, an d the courses of those with HDPD characterized. A search of the databa se identified 230 children and adolescents with CD followed for a tota l 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 fistulae [rectourethroperineal (1), rectovagi nal (1), rectolabial (2), and multiple communicating perineal (4)], an d 53 with simple perianal fistulae or abscesses. All six with HDPD had deeply destructive perineal ulcerations, marked undermining of the pe rineal and perirectal tissues, and copious exudate, and often there wa s a deeply cleaved or fileted perineum on separating the buttocks. Two children with HDPD had fecal incontinence. Apart from race (five of s ix with HDPD were black), there were no significant demographic charac teristics that differentiated the children with HDPD from those with p erianal fistulae and abscesses. No patient with HDPD had had anal inte rcourse, lymphogranuloma venereum, condylomata accuminata, or sarcoido sis. None had been sexually abused. In five of six patients with HDPD, perianal disease was the initial and primary manifestation of CD. Thr ee patients failed aggressive medical management [intravenous (iv)/ora l (po)/intrarectal corticosteroids + metronidazole (MTZ) (2); iv/po cy closporine + MTZ + 6-mercaptopurine (1)] and surgery was performed [di verting colostomy (2) with eventual reanastomosis (1), total proctocol ectomy (1)]. The HDPD healed completely in two of the three operated p atients but remains active, although much improved, 15 months after co lostomy 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 y ears of inconsistent MTZ therapy. HDPD is an unusual but important per ianal complication of CD for which black children may be at increased risk. The perianal disease is a cause of significant morbidity, is hig hly resistant to medical therapy, and in some cases only slowly improv es 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.