Long Term Evaluation

All ARM patients who are diagnosed with a genitourinary malformation, spinal cord abnormality, or bladder dysfunction on initial assessment, and all cloaca patients require regular review. Patients with renal abnormalities and vesicoureteric reflux should have serial ultrasound scans to monitor their renal status. In patients suffering from urinary tract infection, whatever the etiology, a Tc-99m dimercaptosuc-cinic acid renogram is recommended to diagnose renal scarring.

Gynecological abnormalities are common, especially in cloaca patients [25,30,53], but may remain asymptomatic until puberty or adult life (Fig. 17.5). All cloaca patients and those with documented Mul-lerian and vaginal anomalies should be reassessed and monitored in the peripubertal period. Ultrasound scan of the pelvis is a useful investigation for screening [34], whereas an MRI scan is the investigation of choice to document the complex anatomy in these patients, particularly when reconstructive surgery is necessary [29]. Examination under anesthesia and vaginoscopy are also recommended to assess vaginal patency, adequacy, and the presence of a cervix (cervices). Findings previously documented in infancy may

Anorectal Malformation
Fig. 17.5 Magnetic resonance imaging scan showing an obstructed uterus presenting as hematocolpos. The bladder, with contrast in it, can be seen anteriorly to the distended uterus

be misleading. An apparently vestigial uterus may develop enough to produce menstrual flow and hence obstruction if the genital tract is not patent [53]. The size and adequacy of the vagina in proportion to the size of the child may also appear to have changed at puberty, so it is of utmost importance to reassess the patient at this stage.

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