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Eur J Pediatr Surg. 2000 Feb;10(1):17-22.
Clinical long-term follow-up results in intestinal neuronal dysplasia (IND).

Schmittenbecher PP, Gluck M, Wiebecke B, Meier-Ruge W.

Department of Pediatric Surgery, Dr von Hauner's Children's Hospital, University of Munich, Germany.

Commonly available information on intestinal neuronal dysplasia (IND) is sparse. Especially well documented long-term courses are lacking. The aim of this study was to correlate defecation as a clinical parameter of the long-term course in malformations of the enteric nervous system with the morphological diagnosis. 57 children with intestinal neuronal dysplasia (IND) or aganglionosis with cranial intestinal dysganglionosis (agIND), diagnosed between 1983 and 1992, were analysed including histomorphological classification, collection of clinical data and evaluation of the defecation mode by questionnaire as a parameter of the long-term course. Of 29 dysganglionic (IND) patients, 9/29 cases (31 %) had been treated conservatively, 18/29 cases surgically (62.1%), in two children (6.9%) no therapy had been necessary. All 28 patients with Hirschsprung's disease and cranial IND (agIND) underwent resection. 46 of the children could be followed up 3.64 years after the end of the main therapeutic period and with a mean age of 6.7 years at the time of follow-up; 43.5% of the analysed children still showed severe constipation. 23.9% only were really cured; 15.2% had normal defecation still using conservative treatment and 17.4% had diarrhea. No significant difference was found between both groups, IND and agIND, and the results were independent of treatment modality. The results were much worse than in idiopathic constipation as reported in the literature and even worse in comparison to unselected Hirschsprung collectives. It has to be concluded that in IND with chronic constipation intensive long-term care is necessary and it is crucial that treatment algorithms should be outlined urgently together by pediatric gastroenterologists a



Digestion. 2000;61(3):207-14.
Prospective assessment of the clinical value of anorectal investigations.

Vaizey CJ, Kamm MA.

St. Mark's Hospital, London, UK.

BACKGROUND/AIM: Anorectal physiological testing and imaging have become part of routine colorectal and gastro-enterological practice, but their clinical value is controversial. We prospectively evaluated the new diagnostic information, impact on management and prognostic information provided by anorectal testing. METHODS: One hundred consecutive patients referred for testing were studied. The referring doctor's diagnosis, reason for referral, planned management and expectation of the value of investigations were recorded. Incontinent patients underwent anorectal physiological testing and endo-anal ultrasound. Patients with constipation underwent anorectal physiological testing and a study of whole-gut transit time to distinguish between slow and normal transit. Constipated patients over age 45, or those younger patients who digitated to assist defaecation, also underwent evacuation proctography to identify large rectoceles. RESULTS: Fifty-one patients had faecal incontinence. Of 12 patients with suspected anterior external anal sphincter obstetric damage, all of whom were planned for surgical repair, 3 were unsuitable for repair, 3 had a normal sphincter, 2 had a weak but structurally intact sphincter, and 1 had internal anal sphincter damage only. Of 6 patients with failed anterior repair, 3 had a defect suitable for repeat repair, 2 had intact repairs and good function, and 1 had extensive damage requiring reconstructive surgery. The tests also influenced management for incontinent patients after haemorrhoidectomy surgery (n = 5), after fistula surgery (n = 5), with congenital abnormalities (n = 3), after cerebrovascular accident (n = 1) and those with no presumptive diagnosis (n = 15). Of the 20 patients referred with constipation, demonstration of a recto-anal reflex in 1 patient with a megarectum excluded the need for ful



Radiol Med (Torino). 1999 Nov;98(5):368-72.
[Defecography by spiral computed tomography]

[Article in Italian]

Ferrando R, Fiorini G, Beghello A, Tornago S, Cicio G, Derchi LE, Consigliere M, Resasco M.

Istituto di Radiologia, Cattedra R, Universita, Genova, GE.

PURPOSE: We investigated the possible role of helical CT defecography in pelvic floor disorders by comparing our results with those of conventional defecography. MATERIAL AND METHODS: Our series consisted of 90 patients, namely 62 women and 28 men, ranging in age 24-82 years. They were all submitted to conventional defecography, and 18 questionable cases were also studied with helical CT defecography. The conventional examination was performed during the 4 standard phases of resting, squeezing, Valsalva and straining; we used a remote-control unit. The parameters for helical CT defecography were: 5 mm beam collimation, pitch 2, 120 KV, 250 mAs and 18-20 degrees gantry inclination to acquire coronal images of the pelvic floor. The rectal ampulla was distended with a bolus of 300 mL nonionic iodinated contrast agent (dilution: 3 g/cc). The patient wore a napkin and was seated on the table, except for those who could not hold the position and were thus examined supine. Twenty-second helical scans were performed at rest and during evacuation; multiplanar reconstructions were obtained especially on the sagittal plane for comparison with conventional defecographic images. RESULTS: An unquestionable diagnosis could be made in all the 18 patients submitted to helical CT defecography. The diagnosis was in agreement with proctology results and added new information in all cases. Sixteen patients had constipation and 2 fecal incontinence--one from rectal prolapse and the other from a rectovaginal fistula. In this latter case helical CT defecography permitted to confirm the fistula and suggest its course. One patient had a previously undetected ovarian cancer metastatic to the anterior rectal wall. DISCUSSION AND CONCLUSIONS: Coronal helical



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