Chronic constipation: no longer stuck! characterization of colonic dysmotility as a new disorder in children 1

Presented at the 55th Annual Meeting of the Section on Surgery of the American Academy of Pediatrics, New Orleans, Louisiana, October 31-November 2, 2003.
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Clinical features

Recently, we reviewed 180 patients with intractable constipation, 161 of whom were found on NTS to have a functional abnormality in the proximal colon (STC), and 19 of whom had normal proximal motility but holdup in the rectosigmoid colon, consistent with functional fecal retention (FFR).38 Constipation was a major feature in both (about 90%), but soiling was more common with STC (64%) than with FFR (42%). Abdominal distension and bloating was also more common in STC (46%) than FFR (25%).

By

Nuclear transit study

Gastrointestinal transit times have been determined by a number of investigators using radio-opaque markers.34, 39, 40, 41, 42 From these studies, estimates of colonic transit times range from 17 to 38 hours. Use of a radio-isotope to measure transit was first described in Italy,42 and we adapted this method to investigate our patients.

Our standard protocol for NTS was to consider this test only in children old enough to cooperate (>112 to 2 years) and after failure of at least 6 months of

Twenty-four-hour manometry

Recently we have augmented our functional study of patients with STC by using antegrade colonic intubation via an appendix stoma (established for antegrade enemas with ethical approval; Stanton etal, in preparation).43 In a group of volunteers, we inserted a custom-designed SILASTIC® 8-(Dow Corning, Midland, MI) hole catheter with an inter-sidehole distance of 7.5 cm (Dent sleeve, 10F). The catheter was passed via the appendix stoma with 1 mL of bisacodyl enema solution and without anesthesia

Histology

More than 210 children have undergone laparoscopic seromuscular colonic biopsies with or without a rectal mucosal biopsy as part of our systematic effort to identify any histologic anomalies.8, 10, 11, 12, 13, 45 In the early part of the decade (1993 to 1997) selection for biopsy was based on the severity of the symptoms and the presence of the characteristic clinical features described above. However, in the last 5 years, patients were offered biopsy only if the transit study suggested

Treatment options

We have tried a number of treatments for this new clinical problem with variable success. Some early patients underwent hemi- or subtotal colectomy with initial improvement and then recurrence of the symptoms, albeit now more amenable to conservative therapy (as a last resort). About 10% of patients have had a proximal stoma (right colostomy or ileostomy), with good palliation.

Most successful has been antegrade colonic enemas via an appendicostomy (initially done open and recently done

Future questions

There are numerous unresolved issues with STC in children, not the least of which is why the gender ratio is 50:50 when STC in adults is much more common in women. Other intriguing issues include whether there is a defect in water absorption to account for the soft stools. Interestingly, substance P has a major role in water balance. Because 10 of 200 families have a sibling, parent, or first cousin affected, we need to explore the genetic causes. The RET gene is an interesting contender

Acknowledgements

The authors thank Joel Bornstein, James Keck, P. Hengel, V. Robertson, and M. Antello.

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