Plasticity of the enteric nervous system in patients with intestinal neuronal dysplasia associated with Hirschsprung's disease: a report of three patients

Intestinal neuronal dysplasia is a controversial form of dysganglionosis that has been described both as an isolated disorder and in association with Hirschsprung's disease. It has been blamed for the bad outcome of bowel function in patients operated on for Hirschsprung's disease. According to various authors, intestinal neuronal dysplasia could be a primary disorder or secondary to other diseases of the bowel. The aim of this study was to assess the plasticity of the enteric nervous system in patients with Hirschsprung's disease-associated intestinal neuronal dysplasia and its ability to evolve spontaneously to normal innervation patterns. Since we prospectively introduced the assessment of the enteric nervous system of the ganglionated bowel in patients operated on for Hirschsprung's disease, 31 patients have been operated on for Hirschsprung's disease in our institution between 1995 and 2002. Among these patients, nine suffered postoperatively from severe constipation and five from bouts of entocolitis. IND was found in eight of them. We studied the evolution of the innervation in three of these patients by repeated serial full-thickness biopsies of the colon. All three patients underwent a colostomy before or after the pull-through procedure. Histopathological assessment of the enteric nervous system was made with conventional acetylcholinesterase histochemistry, rapid acetylcholinesterase histochemistry and immunohistochemistry for the Protein Gene Product 9.5 and the antigen CD56. This evolution was compared with the clinical outcome of bowel function. In our three patients with Hirschsprung's disease-associated intestinal neuronal dysplasia, this form of dysganglionosis evolved to normal innervation patterns within a period ranging from 9 to 18 months. This evolution was accompanied by an improvement of bowel function in all. We conclude that Hirschsprung's disease-associated intestinal neuronal dysplasia can evolve to a normal innervation, at least under certain circumstances such as a colostomy. Specific histopathological techniques are required to assess accurately the enteric nervous system

Management of cryptorchidism in children: guidelines

QUESTION: To develop clinical guidelines for the management of cryptorchidism in pre-pubertal boys, from early diagnosis through therapy to long-term follow-up and prognosis. METHOD: Systematic review of articles from the medical literature, referenced since 1966, using validated search strategies through the following databases: Medline, Cochrane Database of Systematic Reviews, Cochrane Register of Controlled Trials, EMBASE, DARE, ACP Journal Club, National Guidelines Clearinghouse, Guidelines International Network. Relevant articles published after 1988 were taken as the basis for the statements. Each statement was graded on the basis of the study design and on its methodological quality (GRADE approach). A multidisciplinary panel of local experts discussed and evaluated each statement on the strength of this evidence. RESULTS: 28 statements based on the best available evidence were drafted. The experts agreed with all but two statements, which were rated uncertain. CONCLUSIONS: Cryptorchidism is best diagnosed clinically, and treated by surgical orchiopexy at age 6-12 months, without a routine biopsy. If no testis is palpable, or if other signs of hypovirilisation such as hypospadias are present, the chromosomal sex and hormonal status must be assessed. Laparoscopy is the best way of diagnosing and managing intra-abdominal testes

Simulating glacier lake outburst floods (GLOFs) with a two-phase/layer debris flow model considering fluid-solid flow transitions

Glacier lake outburst floods (GLOFs) initiate with the rapid outburst of a glacier lake, endangering downstream populations, land, and infrastructure. The flow initiates as a mud flow; however, with the entrainment of additional solid material, the flood will often transform into a debris flow. As the run-out slope flattens, the coarse solid material deposits and the flow de-waters. The flow transforms back into a muddy, hyperconcentrated flow of fine sediments in suspension. These flow transitions change the flow composition dramatically and influence both the overall mass balance and flow rheology of the event. In this paper, we apply a two-phase/layer model to simulate flow transitions, solid–fluid phase separations, entrainment, and run-out distances of glacier lake outburst floods. A key feature of the model is the calculation of dilatant actions in the solid–fluid mixture which control flow transitions and phase separations. Given their high initial amount of fluid within the flow, GLOFs are sensitive to slope changes inducing flow transitions, which also implies changes in the flow rheology. The changes in the rheology are computed as a function of the flow composition and do not need any adaptation by ad-hoc selection of friction coefficients. This procedure allows the application of constant rheological input parameters from initiation to run-out. Our goal is to increase the prediction reliability of debris flow modeling. We highlight the problems associated with initial and boundary (entrainment) conditions. We test the new model against the well-known Lake 513 (Peru, 2010), Lake Palcacocha (Peru, 1941), and Lake Uchitel in the Aksay Valley (Kyrgyzstan) GLOF events. We show that flow transition modeling is essential when studying areas that have significant variations in slope

Interstitial cells of Cajal are normally distributed in both ganglionated and aganglionic bowel in Hirschsprung's disease

Surgery for Hirschsprung's disease is often complicated by post-operative bowel motility disorders. The impact of intestinal neural histology on the surgical outcome has been previously studied, but no information is available concerning the influence of the distribution of interstitial cells of Cajal (ICC) on these complications. These cells are considered to be pacemakers in the gastrointestinal tract. The aim of this study was to assess the distribution of ICC in the proximal segment of resected bowel in Hirschsprung's disease and confront these results with the clinical outcome. Using immunohistochemistry for light microscopy, we compared the pattern of distribution of ICC in the proximal segment of resected bowel in Hirschsprung's disease with that in normal colon. We correlated these results with the corresponding neural intestinal histology determined by CD56 and the protein gene product 9.5 immunohistochemistry. The distribution of ICC in the proximal segment of resected bowel is identical to that of normal colon, regardless of normal or abnormal colon innervation. ICC distribution does not seem to contribute to post-operative bowel motility disorders in patients operated for Hirschsprung's diseas

Attitude therapeutique chirurgicale face a l'ulcere de stress complique de l'enfant. [Surgical therapeutic attitude towards complicated stress ulcers in children]

Perforation or uncontrollable hemorrhage following a stress ulcer rarely occurs in high risk children if strict prophylactic measures are adopted. However, we have observed and operated 3 cases over a period of 2 years. Despite the diverse etiologies and contrary to some authors, we have always chosen the simplest and least mutilating operation in order to preserve the physiologic function of the digestive tract. The aim of this article is to compare our therapeutic attitude with that of the literature

Ureterosigmoidostomy: a long-term follow-up of 15 patients with urinary diversion

We reviewed 15 patients who underwent 16 ureterosigmoidostomies from 1975 to 1989. The underlying disease was bladder exstrophy in 13 patients. Urinary diversions have been performed according to Mathisen in 15 patients, one reoperation has been performed according to Coffey. Standard post-operative controls consist of physical examinations, capillary blood gas samples, intravenous pyelograms, inulin clearance measurements, 123I-hippuran scintigraphies and colonoscopies. Results concerning continence are judged very satisfactory in 80% of patients. 34% of patients presented with one or more episodes of unilateral or bilateral pyelonephritis. Intravenous pyelograms showed improved or unchanged urinary tract dilatation in 45% of kidneys and moderate and severe dilatation in 55%. Inulin clearance remained in normal range in 4 out of 6 patients followed. Renal function assessed by means of renal scintigraphy remained unchanged in 61% of kidneys, slightly decreased in 22% and severely in 17%. All patients are kept on a sodium-citrate therapy. Only one patient shows growth disturbances. Out of 9 patients followed with colonoscopy, none showed signs of malignancy