Spectrum and prognosis: ARM range from low (perineal/cutaneous fistula, anal stenosis - excellent prognosis) to high (rectovesical, rectoprostatic, cloaca - guarded prognosis). Krickenbeck classification provides functional grouping. Associated VACTERL anomalies in 50% (Vertebral, Anal, Cardiac, TracheoEsophageal, Renal, Limb). Prognostic factors for continence - 1) ARM type (low > intermediate > high); 2) Sacral ratio (>0.7 favorable; calculated on lateral pelvic X-ray as ratio of sacrum length to pelvic outlet); 3) Spinal anomalies (tethered cord, lipomeningocele - 30-40%); 4) Presacral mass (Currarino triad); 5) Surgical quality and complications.
Long-term functional issues: 1) Fecal continence - majority of high ARM patients have some degree of incontinence/soiling; constipation paradox common (treatment of constipation often improves continence); 2) Constipation - 80% of low ARM, 50-60% of high; megarectum/megasigmoid develops; manage with osmotic laxatives, sometimes resection; 3) Urinary dysfunction - especially in cloaca, rectoprostatic; neurogenic bladder requires CIC, bladder augmentation, anticholinergics; 4) Sexual function - cloaca patients face significant gynecologic challenges (vaginal stenosis, müllerian anomalies); fertility is achievable but requires planning; 5) Psychosocial - school absenteeism, body image, sexual identity, depression. Quality of life scores (RISQS, FIQL) often lower than peers.
Bowel management programs: structured age-appropriate programs are cornerstone. 1) Daily retrograde enema regimen - sodium phosphate or saline tap water enema daily, achieves 24-hour social continence in 80-90% with high ARM; 2) Antegrade continence enema (ACE/Malone procedure) - appendicostomy or cecostomy for daily antegrade flush; 3) Dietary - fiber, fluid intake; 4) Pharmacologic - loperamide for slow-transit hyperbolic constipation, polyethylene glycol for normal-transit constipation; 5) Sacral nerve stimulation - emerging adjunct in select cases; 6) Permanent stoma - last resort in severely affected patients; 7) Pelvic floor biofeedback in cooperative children. Multidisciplinary clinic visits every 6-12 months. Transition to adult care critical at 18-21 years; specialized adult colorectal centers preferred.