Dataset Information

Surgical treatment of children with total colonic aganglionosis: functional and metabolic long-term outcome.

ABSTRACT: Total colonic aganglionosis (TCA) is a rare variant of Hirschsprung's disease occurring in 3-10% of the cases. Only few studies reported the long-term clinical and metabolic outcomes of patients with TCA. The aim of this study was to evaluate the functional and metabolic long-term outcomes of children undergoing surgical treatment for TCA.A 15-year retrospective study was performed. Blood chemistry tests and stool analysis performed at the last follow-up visit were recorded. Height and weight development were assessed using the corresponding percentiles for age. Faecal continence and quality of life were evaluated using a detailed questionnaire.Eleven patients were included in the study. The median age at surgery was 6 months (range: 3-72 months). After histological confirmation, all patients underwent a total colectomy. Ileoanal anastomosis (n?=?6), ileorectal anastomosis (n?=?1), J-pouch (n?=?1) and Duhamel procedure (n?=?3) were performed. Temporary ileostomy was closed after a median of 8 weeks in 10/11 patients. After a median follow-up of 78 months (range: 27-199 months), all evaluated patients were continent. Height and weight were appropriate for age in only 5 patients. Vitamin B12 and folic acid serum levels were normal in all examined patients. Ten patients had normal hemoglobin serum levels. Seven patients had low transferrin saturation in serum. Hemoccult tests were negative in all examined patients. Despite complex postoperative courses in some cases, patients and parents showed good overall satisfaction in terms of quality of life.The majority of patients reported a good quality of life. This can result from the adaptation of the patients to certain disease states. The failure to thrive seems to be related with the extent of aganglionosis. The inclusion of these patients in interdisciplinary long-term follow-up care, in which pediatric surgeons, gastroenterologists, and dieticians are involved, is essential.

SUBMITTER: Urla C

PROVIDER: S-EPMC6094876 | biostudies-other | 2018 Aug

REPOSITORIES: biostudies-other

ACCESS DATA

Publications

Surgical treatment of children with total colonic aganglionosis: functional and metabolic long-term outcome.

Urla Cristian C Lieber Justus J Obermayr Florian F Busch Andreas A Schweizer Roland R Warmann Steven W SW Kirschner Hans-Joachim HJ Fuchs Jörg J

BMC surgery 20180815 1

<h4>Background</h4>Total colonic aganglionosis (TCA) is a rare variant of Hirschsprung's disease occurring in 3-10% of the cases. Only few studies reported the long-term clinical and metabolic outcomes of patients with TCA. The aim of this study was to evaluate the functional and metabolic long-term outcomes of children undergoing surgical treatment for TCA.<h4>Methods</h4>A 15-year retrospective study was performed. Blood chemistry tests and stool analysis performed at the last follow-up visit ...[more]

PMID: 30111320

Similar Datasets

Project description:Objective: This study aims to investigate the clinical features, long-term outcomes, and prognostic predictors of a multicenter cohort of children with moyamoya disease. Methods: A series of 303 consecutive pediatric moyamoya disease (MMD) patients were screened in the present study. The clinical characteristics were retrospectively collected, and long-term outcomes was evaluated. Furthermore, logistic regression analyses were performed to determine the prognostic predictors for the clinical outcome. Results: The mean onset age at diagnosis was 9.4 years old. The gender ratio (girl-to-boy ratio) was 1.1:1.0. Among these 303 patients, 13 patients underwent different surgical modalities in bilateral hemispheres, while eight patients failed to follow-up, and were excluded. Therefore, a total of 282 patients were analyzed. Among these patients, 17 patients underwent combined bypass (CB), 47 patients underwent direct bypass (DB), 150 patients underwent indirect bypass (IB), and 68 patients underwent conservative treatment. Furthermore, recurrent stroke events were observed in 35 patients (12.4%). The Kaplan-Meier analysis demonstrated that there was no significant difference in either ischemia or hemorrhage-free time among the different surgical modalities (P = 0.67 and 0.79, respectively). Furthermore, longer ischemia-free time was observed in the surgical group, when compared to the conservative group (P < 0.01). In addition, 82.7% (177/214) of patients who underwent surgical treatment obtained good outcomes (mRS 0-1), which were significantly higher than the rate of patients who underwent conservative treatment (52.9%, 36/68; P < 0.01). The rate of patients with improved symptoms was also significantly different (93.0 vs. 16.2%, P < 0.01). However, no significant difference was observed in the rate of good outcomes, disability, and improved symptoms among the different surgical modalities. The logistic regression analyses revealed that postoperative ischemic events were the only risk factor associated with unfavorable clinical outcome (OR:3.463; 95% CI:1.436-8.351; P < 0.01). Conclusion: CB, DB, and IB might have similar effects on long-term clinical outcome in pediatric MMD. However, surgical revascularization is superior, when compared to conservative treatment. Furthermore, postoperative ischemic events were confirmed as potential prognostic factors associated with unfavorable clinical outcome.

Project description:BackgroundBurn patients often have functional problems due to joint scar contracture. Patients suffering from such contracture often experience considerable limitations in daily life. Therefore, surgical treatment is often necessary. Skin grafts, especially full-thickness skin grafts and flaps remain the most commonly used surgical methods in clinical practice. However, there are no clear guidelines stating which technique is the most effective treatment. Herein, we conducted a retrospective cohort study over 10 years of experience at a single center to investigate whether flaps or FTSGs exhibit a better long-term effect.MethodsWe performed a retrospective chart review of patients with joint burn scar contracture and collected data related to patient demographic profiles, and detailed descriptions of the scars, surgical procedures, and follow-up were collected. We performed follow-up evaluation of three aspects: adverse events (recontracture, ache, and pruritus), satisfaction scores for function and aesthetics, and scar quality (Vancouver Scar Scale score).ResultsFollow-up results 1 year after surgery from 88 patients were analyzed. In total, 4 (10%) patients in the flap group and 13 (27.1%) patients in the FTSG group had recontracture; the incidence of recontracture was lower in the flap group than in the FTSG group (P=0.043). The functional satisfaction score of the flap group was higher than that of the FTSG group (P=0.027). Moreover, follow-up results 5 year after surgery for 47 patients were analyzed. In total, 1 (4.8%) patient in the flap group and 7 (26.9%) patients in the FTSG group had recontracture; the incidence of recontracture was significantly lower in the flap group than in the FTSG group (P=0.044). The functional satisfaction score in the flap group was higher than that of the FTSG group (P=0.041). In this study, no significant differences in scar quality were observed between the two groups.ConclusionsIf conditions permit, the application of different types of flaps may represent a better choice than FTSGs in terms of reducing the recontracture rate and improving joint function.

Dataset Information

Surgical treatment of children with total colonic aganglionosis: functional and metabolic long-term outcome.

Publications

Surgical treatment of children with total colonic aganglionosis: functional and metabolic long-term outcome.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets