Congenital H-type anourethral fistula with severe urethral hypoplasia and normal anus is an extremely rare variant of anorectal malformations among boys. The authors report a case of a 5-year-old boy who underwent successful management of severe urethral hypoplasia with progressive augmentation by dilating urethra anterior gently and achieving a functionally normal urethra with minimal morbidity. H-type anourethral fistula was excised subsequently through anterior perianal approach.

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http://dx.doi.org/10.1016/s0022-3468(98)90496-1DOI Listing

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Congenital H-type anourethral fistula with severe urethral hypoplasia and normal anus is an extremely rare variant of anorectal malformations among boys. The authors report a case of a 5-year-old boy who underwent successful management of severe urethral hypoplasia with progressive augmentation by dilating urethra anterior gently and achieving a functionally normal urethra with minimal morbidity. H-type anourethral fistula was excised subsequently through anterior perianal approach.

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A newborn having a covered anus and two fistulas, one ano-urethral and one penile urethro-cutaneous, is presented. In spite of the resemblance of this ano-urethral fistula to an "H-type" ano-urethral fistula, which is thought to result from the failure of the division of the cloaca in the embryological period, three abnormalities seen in this case were thought to be the result of a serial event which occurred in the more distal part, the anal and the genital folds, during the embryological period.

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Anorectal atresia with recto-urethral fistula is one of the most common congenital anomalies afflicting the hindgut (1,2). The "H-type" ano-urethral fistula, however, is relatively rare, similar to the "H-type" tracheo-esophageal fistula which is the least common such anomaly of that region. The association of an "H-type" tracheo-esophageal fistula and an "H-type" ano-urethral fistula and stricture of the anterior urethra is considered a rare combination of anomalies.

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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.

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