scholarly journals Diagnosis, Symptoms, and Outcomes of Hirschsprung’s Disease from the Perspective of Gender

2017 ◽  
Vol 2017 ◽  
pp. 1-8 ◽  
Author(s):  
Christina Granéli ◽  
Eero Dahlin ◽  
Anna Börjesson ◽  
Einar Arnbjörnsson ◽  
Pernilla Stenström
Keyword(s):  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Bowel Function ◽  
Cross Sectional Study ◽  
Postoperative Treatment ◽  
First Year ◽  
Structured Interviews ◽  
Cross Sectional ◽  
Long Term Follow Up ◽  
Pull Through

Background/Aim. Hirschsprung’s disease (HD) has a skewed gender distribution, with a female to male ratio of 1 : 4. This study aims to examine differences between boys and girls with HD regarding preoperative features and postoperative treatment and outcome. Method. The first part of the study was conducted as a retrospective review of all HD patients who underwent transanal endorectal pull-through (TERPT). Pre-, peri-, immediate post-, and first-year postoperative data were collected. The second part was conducted as an observational cross-sectional study by comparing bowel function scores (BFS) determined by structured interviews of patients 4 years old and older. Results. Included were 39 boys and 12 girls. Of these, 25 boys and 9 girls were older than 4 years and participated in the BFS interview. Boys had a higher frequency of hospitalizations during the first postoperative year compared to girls (n=20 and n=2, p<0.05). At long-term follow-up, more boys reported abnormal frequency of defecation, 16 compared to 2 (p<0.05). There was no difference between genders in terms of preoperative symptoms and overall bowel function later. Conclusion. Boys with HD had more hospitalizations and a higher rate of abnormal frequency of defecation than girls with HD.

scholarly journals Outcomes in Hirschsprung’s disease with coexisting learning disability

2021 ◽  
Author(s):  
Joseph R. Davidson ◽  
Kristiina Kyrklund ◽  
Simon Eaton ◽  
Mikko P. Pakarinen ◽  
David Thompson ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Learning Disability ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Core Outcome Set ◽  
Functional Results ◽  
Cross Sectional Study ◽  
Cross Sectional ◽  
Related Quality

AbstractThis study describes functional and health-related quality of life (HRQoL) outcomes in patients with Hirschsprung’s disease (HSCR) with associated learning disability or neurodevelopmental delay (LD), completing a core outcome set for HSCR. This was a cross-sectional study from a tertiary pediatric surgery center. Patients treated between 1977 and 2013 were prospectively contacted to complete an outcomes survey. Children under 12 and older patients with LD were assisted to complete these by a proxy. Bowel and urologic function were assessed (Rintala’s BFS and modified DanPSS) along with HRQoL (PedsQL/GIQLI/SF-36). Thirty-two patients with LD were compared to 186 patients with normal cognition. Patients with LD had 76% survival over the follow-up period, compared to 99% in the remainder of the cohort. Poor functional outcomes were common in the patients with LD, considerably higher than cognitively normal patients: with weekly issues withholding stool, soiling and fecal accidents in over half of patients surveyed (44–60%), and urinary incontinence in 46%. Use of permanent stoma was significantly higher (22% vs. 4%; p = 0.001). HRQoL was worse in domains of physical functioning in adults and children but not for social or emotional domains in adults. Subgroup analysis of patients with Down syndrome suggested similar functional results but better QoL. Multivariate analysis demonstrated a dramatically higher incidence of poor continence outcomes in patients with LD (adjusted OR 9.6 [4.0–23]).Conclusions: We provide LD-specific outcomes showing inferior function but similar HRQoL to other patients with HSCR, this is much needed in the counselling of families of these children. What is Known:• Hirschsprung’s disease is commonly associated with syndromes or other anomalies with resultant cognitive impairments.• The outcomes for these patients specifically have been poorly described in the literature. What is New:• Objective functional and quality of life surveys demonstrate significant differences from patients without cognitive impairment.• Patients with learning disability Patients with associated LD were almost ten times more likely to have an associated poor functional outcome, with very little impact on proxy-reported quality of life.

Long-Term Outcomes and Quality of Life in Patients after Soave Pull-Through Operation for Hirschsprung's Disease: An Observational Retrospective Study

2017 ◽  
Vol 28 (05) ◽  
pp. 445-454 ◽  
Author(s):  
Tania Mahler ◽  
Martine Dassonville ◽  
Dinh Truong ◽  
Annie Robert ◽  
Philippe Goyens ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Fecal Incontinence ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Bowel Function ◽  
Long Term Outcomes ◽  
Neurologic Impairment ◽  
Pull Through

Introduction Patients after pull-through operation for Hirschsprung's disease (HD) are at high risk of defecation disorders. This study aimed at investigating their long-term outcomes and quality of life (QoL) in comparison with controls. Patients and Methods Patients older than 5 years operated on for HD were interviewed to complete detailed questionnaires on bowel function. Patients without neurologic impairment were enrolled in a QoL survey to compare with controls matched for sex and age and selected randomly from the general population using sampling set in a ratio of four controls to one case of HD. Results In total, 53 operated patients were enrolled. Mean age of the patients was 16 ± 8 years, with 68% boys. Rectosigmoid aganglionosis was the most seen form of HD in 38 (72%) cases. Open Soave was performed in 40 (75.5%) cases, and minimally invasive surgery Soave (MIS Soave) in 13 (24.5%) cases. At investigation, prevalence of fecal incontinence and constipation were 22.6 and 13.2%, respectively. Regarding QoL survey, 45 patients and 180 controls were enrolled, excluding 8 patients with neurologic impairment. Thirty-seven (82.2%) patients were classified as having a good QoL (score ≥ 9 points); whereas six had a fair QoL (5–8 points) and two had a poor QoL (< 5 points). QoL score in the cases and the controls were 10.2 ± 2.5 and 11.9 ± 0.4 points, respectively. Long aganglionosis form of HD was significantly associated with a low QoL (score < 8 points), adjusted odds ratio = 9, 95% confidence interval [1.3; 64.1] (p < 0.05). In subscales analyses, the prevalence of each dimension including fecal continence, school absenteeism, unhappiness or anxiety, food restriction, and peer rejection was significantly higher in operated patients than in controls (p <0.001). Conclusion Although the QoL of patients operated on for HD in general was with good outcomes, fecal incontinence and constipation still are problematic issues and challenges in a high percentage of patients. Therefore, a long-term and multidisciplinary follow-up is essentially required for these patients.

Dr. Orvar Swenson and the Pull-Through

2019 ◽  
Vol 85 (12) ◽  
pp. 1311-1313
Author(s):  
John J. Newland ◽  
Katerina Dukleska ◽  
Scott Cowan ◽  
Charles J. Yeo ◽  
Renee Tholey
Keyword(s):  
Pediatric Surgery ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Toxic Megacolon ◽  
Bowel Function ◽  
Standard Textbook ◽  
Surgery Research ◽  
Peter Bent Brigham Hospital ◽  
Pull Through ◽  
Rectal Biopsies

Dr. Orvar Swenson is best remembered for developing the Swenson pull-through, a technique he developed to treat Hirschsprung's disease. After graduating from Harvard Medical School and beginning his residency at Peter Bent Brigham Hospital, Dr. Swenson observed that patients with Hirschsprung's disease and toxic megacolon resumed normal bowel function after placement of transverse colostomies. His observation led to studying the patency of his patients’ colons using barium enema contrast studies. At the collapsed portion of the colon, he performed rectal biopsies leading to the discovery that the cause of Hirschsprung's disease is that the collapsed portion of the colon lacks the Auerbach plexus. The Swenson pull-through removes this aganglionic portion of the colon and cures the patient. His career grew from there as he traveled to academic institutions teaching his technique. He is remembered fondly for his contributions to pediatric surgery through the restructuring of pediatric surgery departments, pediatric surgery research, and writing and editing multiple volumes of Pediatric Surgery, the standard textbook for pediatric surgeons. He died peacefully in 2012 at the age of 103 years.

scholarly journals RISK FACTORS INFLUENCING ENTEROCOLITIS DEVELOPMENT IN PEDIATRIC PATIENTS WITH HIRSCHSPRUNG’S DISEASE

2020 ◽  
Vol 8 (3) ◽  
pp. 218
Author(s):  
Andro Pramana Witarto ◽  
Alpha Fardah Athiyyah ◽  
I.G.B. Adria Hariastawa ◽  
I Gusti Made Reza Gunadi Ranuh
Keyword(s):  
Risk Factors ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Secondary Data ◽  
Prevalence Ratio ◽  
Cross Sectional Study ◽  
Cross Sectional ◽  
Significance Level ◽  
Potential Risk Factors ◽  
History Of

Background: Hirschsprung-associated enterocolitis (HAEC) is one of the worst and most common complications of Hirschsprung’s disease (HD). The mortality rate of HD patients with enterocolitis is still considered to be higher compared to that of those without enterocolitis. Purpose: This study aimed to identify and evaluate potential risk factors for HAEC development. Methods: A cross-sectional study was conducted using secondary data from the medical records of HD patients treated from January 2015 to September 2018 at Regional Public Hospital (RSUD) Dr. Soetomo, Surabaya. The inclusion criteria were HD patients who had or had not experienced enterocolitis. The analysis was done by comparing the presence of risk factors between groups of HD patients with and without preoperative and/or postoperative HAEC. The results were presented as the median value and frequency. To evaluate further, a prevalence ratio (PR) with a 95% confidence interval was performed. The Mann–Whitney U test was also performed with a significance level of p < 0.05 for one factor: length of aganglionic intestinal segments. Results: This study showed that 12 of the 40 HD patients studied (30%) had experienced enterocolitis. The risk of developing HAEC was associated with patients who had a history of previous enterocolitis (PR 6.60 [2.94 < PR < 14.80]). Regarding surgical details, patients who had had surgery only once (31.30% compared to 14.30%), surgery with one surgical method (29.40% compared to 20.00%), and a primary procedure had a higher incidence of HAEC (29.40% compared to 27.30%). Conclusion: HD patients with a history of previous enterocolitis were found to have a higher risk of developing HAEC.

scholarly journals Children with Hirschsprung's Disease and Syndromes with Cognitive Dysfunction: Manifestations, Treatment, and Outcomes

2019 ◽  
Vol 05 (03) ◽  
pp. e103-e109
Author(s):  
Josefine Hedbys ◽  
Johan Hasserius ◽  
Christina Granéli ◽  
Einar Arnbjörnsson ◽  
Kristine Hagelsteen ◽  
...  
Keyword(s):  
Cognitive Dysfunction ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Retrospective Chart Review ◽  
Bowel Function ◽  
Cross Sectional ◽  
Pediatric Surgeon ◽  
Initial Symptoms ◽  
Patient Reported

Abstract Introduction To assess differences in initial symptoms, treatments, and bowel function between children with Hirschsprung's disease (HD) with or without a cognitive dysfunction (CD). Materials and Methods The study included children with HD who underwent transanal endorectal pull-through. A retrospective chart review was performed to collect data on patient characteristics, diagnosis, and treatment. Data on bowel symptoms in children older than 4 years without a colostomy were compiled during a cross-sectional, patient-reported follow-up. Results Fifty-three children with HD were included; of these, 12 (23%) had CD. The median birth weight was lower, frequency of vomiting as the presenting symptom was lower, and time until the first contact with a pediatric surgeon was higher in children with CD than in those without (3,295 vs. 3,623 g, p = 0.013; 28 vs. 66%, p = 0.02; and 4 days vs. 1 day, p = 0.048, respectively). At follow-up, 5 (15%) of 33 children aged over 4 years had CD. More children without CD had some ability to hold back defecation and sense the urge to defecate than those with CD (p = 0.002 and p = 0.001, respectively). Conclusion HD children who have CD present with different initial symptoms, have a delay in the first consultation with a pediatric surgeon, and experience poorer bowel function outcomes than HD children without CD. Therefore, HD children with CD should receive special attention in both clinical practice and research.

Perioperative Complications of Transanal Pull-through Surgery for Hirschsprung's Disease

2018 ◽  
Vol 28 (02) ◽  
pp. 152-155 ◽  
Author(s):  
Mikko Pakarinen
Keyword(s):  
Surgical Complications ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Perioperative Complications ◽  
Surgical Techniques ◽  
Bowel Function ◽  
Optimal Operation ◽  
Surgical Approaches ◽  
Repeated Surgery ◽  
Pull Through

AbstractTransanal pull-through is currently one of the most commonly used operation to treat Hirschsprung's disease (HD) worldwide. It has undergone some technical modifications during the last decades and still contains variable surgical practices. As high quality comparative studies between different surgical approaches are missing, debate over the optimal operation for HD still continues. Transanal pull-through is associated with multiple potential complications, which may result in permanently impaired functional outcome, life-long morbidity, and significant social restrictions. Although some of the surgical complications can be successfully managed by repeated surgery, the best changes for optimal bowel function and fecal continence are lost. Surgical complications are best treated by efficient prevention with careful adherence to established surgical techniques and principles during the primary operation.

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