A term newborn was transferred to our hospital and underwent Pena anorectoplasty for imperforate anus with perineal fistula. In ICD-9-CM the index leads to codes 751.2, Atresia and stenosis of large intestine, rectum, and anal canal, for imperforate anus, and 599.1, Urethral fistula, for perineal fistula. In this case, code 599.1 seems incorrect because the perineal fistula is a component of the imperforate anus (anorectal defect). The guidelines state that manifestations that are an inherent component of the anomaly should not be coded separately. However, the guidelines also state that if the index does not provide a specific code for a perinatal condition, code 779.89 is assigned. Is it appropriate to report 751.2 and 599.1 along with 779.89 to describe this condition?
Assign code 751.2, Atresia and stenosis of large intestine, rectum, and anal canal, for the imperforate anus. Code 599.1, Urethral fistula, should be assigned for the perineal fistula. The Official Guidelines for Coding and Reporting state that congenital anomaly code categories 740-759 can be assigned with codes from other chapters to specify conditions associated with the anomaly, if applicable. Code 779.89, Other specified conditions originating in the perinatal period, is not a correct code assignment since the perineal fistula is an anorectal defect associated with the imperforate anus.
Assign code 48.49, Other pull-through resection of rectum, for the Pena anorectoplasty.
Anorectal malformations include a wide spectrum of anomalies in the development of the lowest portion of the intestinal and urogenital tracts. Children with these types of congenital anomalies have an imperforate anus because there is no opening at the anus. The connected muscles and nerves are usually malformed and can involve the spine and urogenital tract. Any impediment to anorectal structural development at different stages can lead to anomalies, ranging from anal stenosis, incomplete rupture of the anal membrane, or anal agenesis to complete failure of the upper portion of the cloaca to descend and failure of the proctodeum to invaginate. Continued communication between the urogenital tract and rectal portions of the cloacal plate causes rectourethral fistulas or rectovestibular fistulas.