References: Laxative
Eur J Pediatr Surg. 1999 Apr;9(2):83-9.
Chronic intestinal pseudo-obstruction syndrome in pediatric patients.
Goulet O, Jobert-Giraud A, Michel JL, Jaubert F, Lortat-Jacob S, Colomb V, Cuenod-Jabri B, Jan D, Brousse N, Gaillard D, Nihoul-Fekete C, Ricour C.
Departement de Pediatrie, Hopital Necker-Enfants Malades, Paris, France.
The aim of this study was to report the presentation and outcome of 22 consecutive children (13 female) who presented with a syndrome of chronic intestinal pseudo-obstruction with or without urinary tract involvement. We analyse the main clinical and histopathological features and discuss therapeutic management. Ten patients had signs of intestinal obstruction at birth, in which 6 presented antenatally with megacystis on ultrasound. Six children presented with constipation and/or obstruction between 1 and 6 months of age and in 6 other patients diagnosis was made between the ages of 1 and 12 years. There was a family history in 4 patients. Investigations showed diffusely dilated gut on x-ray with slow transit on small bowel follow through. Absent or abnormal motor migrating complex with low amplitude contractions were demonstrated on duodeno-jejunal manometry in 12/13. Megacystis occurred in 15/21 and megaureter in 2/21. Full thickness biopsies (n = 22) revealed involvement of muscle layers in 8, and abnormal myenteric plexus on histochemistry in 13. In 1, the biopsies were inconclusive. Recurrent urinary tract infections occurred in all with structural urinary tract abnormality and most had bacterial overgrowth. Severe recurrent episodes of obstruction which required parenteral nutrition (PN) occurred in all patients. Drugs were unhelpful and decompression ileostomies or colostomies were performed in 20/22. Five children died from sepsis (n = 3) or sudden death. Eleven patients remain partially or totally dependent on PN despite decompression ileostomy in 10/11. Six patients underwent colectomy and ileorectal pull-through, 2 of which remain on long-term PN, while t
Eur J Pediatr Surg. 1999 Apr;9(2):91-5.
Intestinal transit time in children with intestinal neuronal malformations mimicking Hirschsprung's disease.
Ure BM, Holschneider AM, Schulten D, Meier-Ruge W.
Department of Pediatric Surgery, Children's Hospital of Cologne, Germany.
A total of 106 consecutive children with intestinal neuronal malformations were included in a prospective study. The intestinal transit time was assessed using a modification of Hinton's method. The results of transit time studies, the associated specific histochemical findings, therapeutic procedures, and the clinical course on follow-up assessments over a mean period of 2.4 years were analysed. The intestinal transit time was prolonged in all 53 patients with aganglionosis and in 37 (69.8%) out of 53 children with other intestinal malformations. Eight out of 16 children with IND type B had an abnormal transit time, 1 underwent anterior resection, and 2 had a temporary colostomy. In 7 out of 8 children with hypoganglionosis and 9 out of 10 children with a reduced parasympathetic tone the transit time was prolonged. A resection was performed in 7 and 2 of these children respectively. Both patients with heterotopia of the myenteric plexus had a prolonged bowel transit and parts of the large bowel had to be resected. Only 11 out of 17 children with heterotopia of the submucous plexus, dysganglionosis, or immature ganglia had a prolonged transit time, 2 underwent sphincteromyotomy. At follow-up, all patients with malformations other than aganglionosis stated that symptoms had improved and they were willing to tolerate their complaints. However, 25 reported on persistent constipation, 6 on overflow encopresis. All children who required surgery had a prolonged intestinal transit time, but also 21 (56.8%) of 37 children who were successfully treated without surgery. None of the 16 children with normal transit had to be operated. It is concluded that specific histochemical findings do not always correlate with delayed intestinal
Eur J Pediatr Surg. 1999 Apr;9(2):96-100.
Preoperative enzymo-histochemical diagnosis of dysganglionoses associated with anorectal malformations (ARM) with recto-vestibular and recto-perineal fistula.
Martucciello G, Mazzola C, Favre A, Negri F, Bertagnon M, Morando A, Torre M, Gambini C, Jasonni V.
Department of Pediatric Surgery, Giannina Gaslini Children's Hospital, Genova, Italy.
The posterior sagittal anorectoplasty (PSARP) is widely recognized as the best technique available today for the surgical treatment of anorectal malformations (ARM). However, different retrospective studies on the functional results of PSARP in the treatment of ARM have shown different postoperative degrees of constipation. In particular, even in patients with normal sacrum, about 70% of operated ARM with vestibular fistula and about 50% with perineal fistula can be complicated by fecal constipation and pseudoincontinence. In order to identify preoperatively whether ARM patients present abnormal innervation patterns of rectal pouch and fistula (as reported by Holschneider et al [7]), we decided to study suction rectal biopsies performed by introducing SBT-100 rectal suction biopsy tool into the fistula at 6, 4, 3 and 2 cm from the meatus. To date, this approach has been adopted in 22 ARM cases (15 females and 7 males, age range 7 days-4 years), 6 of them with recto-vestibular fistula and 13 with recto-perineal fistula. Biopsies were frozen in isopentane at liquid nitrogen temperature and cryostatic sections were studied by acetylcholinesterase (AChE), succinic-dehydrogenase (SDH) and alpha-naphthyl-esterase (ANE) enzymo-histochemical techniques. The results concerning the innervation-type of fistula and proximal rectal pouch were confirmed by the biopsies obtained during PSARP. Our overall incidence of rectal innervation intrinsic disorders was 81.82%. In particular, all our cases of vestibular fistula presented associated dysganglionoses. The incidence of associated Hirschsprung's disease was high, corresponding
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