Functional Outcome Following Secondary Surgery for ARM

Secondary reconstructions to improve poor anorectal function have been used extensively in patients with ARM. In most long-term follow-up series extending to adulthood, a significant proportion of patients have undergone redo-surgery [32,83,84]. A clear mes-

sage arises from these reported series: the long-term outcome is not better in patients who have had secondary surgery [83] and may be worse than in those who had only one reconstruction [84]. It is possible, however, that the patients who have undergone redo-operations had initially worse continence than those with only one operation, but there are no reports that have specifically addressed this question.

Gracilisplasty has been a common method for secondary sphincter reconstruction. Several reports have shown a clear improvement in fecal continence in the short term [101,102]. The improvement in continence is caused by a somewhat increased resting pressure [101] and significantly increased squeeze pressure. In adults who have had gracilisplasty during childhood, the functional results are not very encouraging [32,83,84]. Fecal continence is no better, and may be worse, than in patients with only a primary reconstruction. The main functional limitation of conventional gracilisplasty is that a skeletal muscle like the gracilis muscle can contract only voluntarily. The initially increased tone of a tight muscle wrap around the anus tends to weaken with time [101]. Recently, electrically stimulated gracilisplasty has been used to improve deficient fecal continence in adult patients with ARM. The patients underwent conventional gracilisplasty followed by implantation of a muscle stimulator [103]. After a training period the stimulator was used continuously to maintain constant anal tone. Short-term clinical and manometric results were promising, but after a median follow-up of 4 years only one-third of the patients gained satisfactory fecal continence [104].

Levatorplasty, originally described by Kottmeier and Dzaidiw [105], was popularized as a secondary sphincter reconstruction by Puri and Nixon [106]. Several authors [32,107,108] have published encouraging results. The functional improvement following this procedure has been thought to be related to the creation of an acute anorectal angle, because actual resting or squeeze pressures are not changed at the level of the anal canal. Again, as in patients who had gracilisplasty during childhood, the long-term outcome in adults does not seem to be encouraging. In the author's institution, 15 children with high malformations underwent secondary levatorplasty for poor fecal continence in the late 1970's. At adulthood, only 1 of these 15 patients have gained a satisfactory fecal continence. There does not seem to be any significant differences in fecal continence between those who had secondary levatorplasty and those who have undergone only primary reconstruction [83,84].

Rerouting of the pulled-through bowel has been advocated for patients who have a misplaced anal canal following primary operation [4,109]. In patients with previous abdominoperineal, sacroperineal, or sacroabdominoperineal pull-through, a typical misplacement is anterior to the external sphincter funnel [110-112]. An essentially identical procedure for the rerouting and repair of the muscular anal canal has been suggested by Stephens and Smith, Peña, and Kiesewetter and Jeffries [4,109,113]. The repair is performed through a posterior sagittal sacroperineal incision and includes splitting of the voluntary sphincter muscles in the midline, as in standard PSARP.

Anterior sagittal repair for anterior misplacement has been suggested by Okada et al. [112] and Bass and Yazbeck [114]. The reported outcomes in terms of improved fecal continence have been variable. Following redo PSARP, Peña found a very significant improvement in 52% of his 62 patients, mild improvement in 18%, and no improvement in 12%; the length of follow-up was not stated. The patients who improved had a lesser degree of sacral dysplasia than those who did not improve. Mulder et al. [72] reported that 25% of their 20 patients became continent following this procedure; the mean follow-up period was 3.5 years. Both these series allowed occasional soiling in patients with a good outcome. Brain and Kiely [115] had a success rate of 16% following a relatively short follow-up period. Rintala and Lindahl [111] followed-up 16 patients with redo-PSARP beyond childhood (mean follow-up period 6 years). Although the clinical continence and manometric findings initially improved in 13 of the 16 patients, at adult age only 4 (25%) of the patients could be considered more or less continent, despite a significant increase in both anal resting and squeeze pressures. According to the results of these reports, the role of secondary PSARP in the treatment of fecal incontinence after primary reconstruction of ARM remains to be established.

Late fecal soiling may be related to intractable constipation [8,11,28,116]. This occurs most commonly in patients with a repaired vestibular fistula [2,8,11]. Many of these patients have an adynamic megarec-tum, which cannot be emptied with medical management or regular enemas. Aganglionosis, although a rare occurrence in patients with ARM, should be ruled out by rectal biopsies. In recalcitrant cases, resection of the dilated distal colon has given favorable results. Peña et al. [117, 118] and Cheu and Grosfeld [116] have used anterior resection with good outcome. Rintala et al. and Powell et al. [2,11] combined resec tion of the megarectum with endorectal pull-through, which cured the constipation in all of their patients. In the series of Rintala et al., which comprised 13 patients with surgically treated megarectum, 1 had an associated short-segment aganglionosis. The constipation may not be resolved permanently, however; recurrent constipation has been reported following rectosigmoid resection of a megarectum [22].

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Constipation Prescription

Constipation Prescription

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