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Purpose: To report outcomes of children with constipation refractory to medical management and manometrically proven distal colonic dysmotility, managed with rectosigmoidectomy followed by Duhamel operation (Duhamel).Methods: Children who underwent a Duhamel from 2009 onwards for intractable constipation and left colonic dysmotility were retrospectively reviewed. The primary end point was resolution of constipation, and secondary end point was postoperative complications. Continuous data were median (range).Results: 11 patients (4 males) had Duhamel at 11 years (5-16) with constipation started from 2 years (1-8). Hirschsprung's disease was excluded. All Duhamels were performed with a covering ileostomy: 9 following a Hartmann procedure, one following a previously failed reversal of Hartmann, and one Duhamel performed with a pre-existing ileostomy. All ileostomies were subsequently closed. Median resection length was 22 cm (11-31). Length of stay was 8 days (5-23). Follow-up was 5 years (0.5-7). Age at final review was 15 years (10-18). Resolution of constipation occurred in nine patients (4 required antegrade continence enemas (ACE), 5 with laxative); two had persistent constipation and faecal incontinence despite ACE, ultimately requiring an ileostomy. Two postoperative small bowel obstructions required laparotomy.Conclusion: Duhamel performed in children with manometrically proven distal colonic dysmotility yielded 82% resolution of refractory constipation; half of them subsequently needed ACE. [ABSTRACT FROM AUTHOR]

Achalasia is a rare esophageal motility disorder: its optimal treatment in children is still a matter of debate. Records of children treated for achalasia, over an 18-year period, were reviewed.Forty-eight children (median age at diagnosis 10 years; range 3-17 years) were identified. Twenty-eight patients were initially treated with Heller's myotomy (HM) and 20 with balloon dilatation (BD). At last follow-up (median 3 years; range 1-5.5 years), 43.8% (21/48) of children were symptom free. The number of asymptomatic children was significantly higher among those treated initially with HM compared to BD (HM 15/28, 53.6% BD 6/20, 30%, p < 0.05). All children who underwent BD required HM due to symptom recurrence. The median (range) total number of procedures was significantly higher in the BD group (BD 3 (1-7); HM 1 (1-5); p < 0.05) with a shorter time to the second intervention (BD 14 months, 95%CI 4-24; HM 58 months, 95%CI 38-79; p < 0.05). Of 108 procedures, esophageal perforation occurred in two children after HM (two out of 48 HM procedures in total, 4%) and one child after BD (1/60, 1.7%).Conclusion: Less than half of children with achalasia are symptom free after initial treatment with either BD or HM. HM, however, when performed as first procedure, provided longer symptom-free period and reduced need for subsequent intervention. What is Known: • Balloon dilatation (BD) and Heller's myotomy (HM) are safe and effective treatment options for achalasia. • Controversy, however, exists regarding the most effective initial therapeutic approach. What is New: • HM with or without fundoplication may represent the initial therapeutic approach of choice. • Initial BD may negatively affect the outcome of a subsequent HM. [ABSTRACT FROM AUTHOR]

Paediatric GI emergencies constitute a wide range of gut pathologies ranging from those that are common, easily diagnosed and treated to conditions that are rarer, often more severe and challenging to manage. Among a myriad of ordinary clinical symptoms and signs physicians have to identify the child with a serious, life-threatening pathology and initiate the appropriate diagnostic and therapeutic pathway. The aim of the review is to present and discuss a selection of key paediatric GI emergencies that provide challenges in diagnosis and treatment. These conditions are classified by their presentation or pathogenesis and include inflammatory conditions, those presenting with GI obstruction or haemorrhage and the ingestion of foreign bodies or caustic substances. The most recent advances regarding the management of these entities are discussed along with key areas of clinical practice and future research. [ABSTRACT FROM AUTHOR]

OBJECTIVES: Although muscular dystrophy (MD) affects primarily striated muscles, smooth muscle cells of the gastrointestinal tract may also be involved. We recorded gastric electrical activity and gastric emptying time (GET) in children with MD at initial presentation and at 3-yr follow-up in order to detect gastric motor abnormalities and study their evolution along the clinical course.METHODS: Twenty children with MD (median age: 4.6 yr; range age: 3–7 yr) were investigated by means of ultrasonography, for measuring GET, and by electrogastrography (EGG); 70 children served as controls.RESULTS: Ten patients had Duchenne muscular dystrophy (DMD) and 10 Becker muscular dystrophy (BMD). GET was significantly more delayed in MD patients (DMD, median: 195 min; range 150–260 min; BMD, median: 197 min; range: 150–250 min) than in controls (median: 150 min; 110–180 min;p<0.05); it markedly worsened at the follow-up in DMD (median: 270 min; range 170–310 min;p<0.001vscontrols) but not in BMD patients (median: 205 min; 155–275 min;p<0.05vsDMD). Baseline EGG showed a significantly lower prevalence of normal rhythm and significantly higher prevalence of dysrhythmias in both groups of patients as compared to controls (% of normal rhythm: DMD 66.7± 8.2, BMB 67.2± 11.5, controls 85.3± 7.2,p<0.001; % of tachygastria: DMD 28.4± 8.0, BMB 29.8± 12.3, controls 10.6± 5.1,p<0.001; % of dominant frequency instability coefficient: DMD 36.1± 6.0, BMB 33.2± 2.9, controls 17.9± 7.1,p<0.001); furthermore, no difference in fed-to-fasting ratio of the dominant EGG power was found between the two groups and controls (DMD 2.84± 1.27, BMB 2.82± 0.98, controls 3.04± 0.85, ns). However, at the follow-up no significant change in the prevalence of normal rhythm and dysrhythmias occurred in both groups (nsvsbaseline values), whereas only DMD patients showed a marked reduction in fed-to-fasting power ratio (0.78± 0.59;p<0.001vscontrols and BMD;p<0.05vsbaseline), which correlated with the progressive neuromuscular weakness occurring in DMD subjects (r, 0.75;p<0.001).CONCLUSIONS: In children with MD, there is an early abnormality in gastric motility that is due to deranged regulatory mechanisms, whereas contractile activity of smooth muscle cells seems to be preserved. At the follow-up, DMD patients exhibited a progressive failure in neuromuscular function, which was accompanied by a gastric motility derangement with worsening in GET and in EGG features suggesting an altered function of gastric smooth muscle cells.(Am J Gastroenterol 2005;100:695–702) [ABSTRACT FROM AUTHOR]