"H-type" urethroanal fistula

Stephens and Smith have recently described a urethroanal connection through which urine was passed preferentially into the otherwise normal rectum at the pectinate line. Other authors have reported similar deformities. The term "congenital 'H-type' anourethral fistula" was proposed for this rare anomaly by deVries and Friedland in 1974. In this communication, we describe two examples of the "H-type" urethroanal fistula (Fig. 1). Each patient also had tracheoesophageal fistula. One patient (R.P.), now 11 yr of age, has had successful correction of the anomaly. The other patient (T.McC.), a small premature baby whose uroanal deformity was investigated radiographically, died of sepsis and uremia. The anatomical relationships in this baby were investigated histologically in the autopsy specimen by means of serial sections. From this study, it has been possible to determine the sphincteric anatomy and to suggest a possible pathoembryology of the defect.