Improving the accuracy of contrast enema in diagnosis of Hirschsprung disease

QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Amir M Eskander ◽  
Hesham M Mansour ◽  
Khaled A Ali Shehata
Keyword(s):  
Sensitivity And Specificity ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Contrast Enema ◽  
University Hospitals ◽  
Cross Sectional ◽  
Gold Standard Method ◽  
Abnormal Finding ◽  
Specificity And Sensitivity ◽  
Wide Range

Abstract Background Hirschsprung’s disease (HD) is one of the most important causes of chronic constipation in children due to its possibly fatal Amir Maurice Eskander complications if left untreated. Contrast enema (CE) is the first investigation done to diagnose Hirschsprung’s disease as it’s the safest, least invasive, most widely available and least expensive as compared to anal manometry and rectal biopsy however it had shown a wide range of sensitivity and specificity in previous studies. Aim of the work to improve the accuracy of contrast enema by studying each possible abnormal finding related to Hirschsprung’s disease and calculating its sensitivity and specificity in comparison with biopsy, as a gold standard method to determine the most reliable signs to use for diagnosis. Patients and Methods This cross sectional study was carried at pediatric radiology unit Ain Shams university hospitals, starting from July 2018 to June 2019. 43 patients met all inclusion and exclusion criteria and were included in the study. Contrast enemas results and findings were compared to the patient’s final diagnosis. PPV, NPV, specificity, and sensitivity was calculated for each finding. Results Absence of rectal gas in the plain film, transitional zone, abnormal recto-sigmoid index, enlarged colonic caliber and delayed evacuation showed significant association with HD. Abnormal recto-sigmoid index was the most reliable sign in our study to diagnose HD having the highest sensitivity and specificity. Conclusion CE is an excellent initial investigation in HD and combining it with detailed history taking and clinical examination can lead to decrease in unnecessary rectal biopsies.

scholarly journals DIAGNOSTIC ACCURACY OF BARIUM ENEMA FINDINGS IN HIRSCHSPRUNG'S DISEASE

2016 ◽  
Vol 29 (3) ◽  
pp. 155-158 ◽  
Author(s):  
Mehran PEYVASTEH ◽  
Shahnam ASKARPOUR ◽  
Nasrollah OSTADIAN ◽  
Mohammad-Reza MOGHIMI ◽  
Hazhir JAVAHERIZADEH
Keyword(s):  
Barium Enema ◽  
Predictive Value ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Transitional Zone ◽  
Dentate Line ◽  
Contrast Enema ◽  
Cross Sectional ◽  
Specificity And Sensitivity ◽  
Sensitivity Specificity

ABSTRACT Background: Hirschsprung's disease is the most common cause of pediatric intestinal obstruction. Contrast enema is used for evaluation of the patients with its diagnosis. Aim: To evaluate sensitivity, specificity, positive predictive value, and negative predictive value of radiologic findings for diagnosis of Hirschsprung in patients underwent barium enema. Methods: This cross sectional study was carried out in Imam Khomeini Hospital for one year starting from 2012, April. Sixty patients were enrolled. Inclusion criteria were: neonates with failure to pass meconium, abdominal distention, and refractory constipation who failed to respond with medical treatment. Transitional zone, delay in barium evacuation after 24 h, rectosigmoid index (maximum with of the rectum divided by maximum with of the sigmoid; abnormal if <1), and irregularity of mucosa (jejunization) were evaluated in barium enema. Biopsy was obtained at three locations apart above dentate line. PPV, NPV, specificity , and sensitivity was calculated for each finding. Results: Mean age of the cases with Hirschsprung's disease and without was 17.90±18.29 months and 17.8±18.34 months respectively (p=0.983). It was confirmed in 30 (M=20, F=10) of cases. Failure to pass meconium was found in 21(70%) cases. Sensitivity, specificity, PPV, and NPV were 90%, 80%, 81.8% and 88.8% respectively for transitional zone in barium enema. Sensitivity, specificity, PPV, and NPV were 76.7%, 83.3%, 78.1% and 82.1% respectively for rectosigmoid index .Sensitivity, specificity, PPV, and NPV were 46.7%, 100%, 100% and 65.2% respectively for irregular contraction detected in barium enema. Sensitivity, specificity, PPV, and NPV were 23.3%, 100%, 100% and 56.6% respectively for mucosal irregularity in barium enema. Conclusion: The most sensitive finding was transitional zone. The most specific findings were irregular contraction, mucosal irregularity, and followed by cobblestone appearance.

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.

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 Comparison of BOLD and CBV using 3D EPI and 3D GRASE for cortical layer fMRI at 7T

2019 ◽  
Author(s):  
Alexander JS Beckett ◽  
Tetiana Dadakova ◽  
Jennifer Townsend ◽  
Laurentius Huber ◽  
Suhyung Park ◽  
...  
Keyword(s):  
Sensitivity And Specificity ◽  
Primary Motor Cortex ◽  
Cerebral Blood Volume ◽  
Spin Echo ◽  
Cortical Layer ◽  
Imaging Method ◽  
Bold Imaging ◽  
Specificity And Sensitivity ◽  
Wide Range ◽  
Resolution Imaging

AbstractPurposeFunctional MRI (fMRI) at the mesoscale of cortical layers and columns requires both sensitivity and specificity, which can be compromised if the imaging method is affected by vascular artifacts, particularly cortical draining veins at the pial surface. Recent studies have shown that cerebral blood volume (CBV) imaging is more specific to the actual laminar locus of neural activity than BOLD imaging when using standard gradient-echo (GE) EPI sequences. Gradient and Spin Echo (GRASE) BOLD imaging has also shown greater specificity when compared with GE-BOLD.MethodsHere we directly compare CBV and BOLD contrasts in high-resolution imaging of the primary motor cortex for laminar fMRI in four combinations of signal labeling, VASO (CBV) and BOLD with 3D GE-EPI and zoomed 3D GRASE image readouts.ResultsWe find that both CBV imaging using EPI-VASO and BOLD imaging using GRASE-BOLD, show similar specificity and sensitivity and are thus useful tools for mesoscopic fMRI in the human cortex.ConclusionThese techniques demonstrate sufficient sensitivity and specificity to allow layer-fMRI to be used by neuroscientists in a wide range of investigations of depth-dependent neural circuitry in the human brain.

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.

scholarly journals Sexual function, quality of life, and fertility in women who had surgery for neonatal Hirschsprung’s disease

2021 ◽  
Author(s):  
J R Davidson ◽  
K Kyrklund ◽  
S Eaton ◽  
M P Pakarinen ◽  
D S Thompson ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Cohort Study ◽  
Sexual Function ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Cross Sectional ◽  
Female Patients

The authors explore multi-domain outcomes in a cross-sectional cohort study of patients with Hirschsprung's Disease. They identify hitherto unrecognised issues affecting sexual function and fertility specifically in female patients.

scholarly journals Usefulness of Delayed Films of Contrast Enema for Detecting Hirschsprung’s Disease

Cureus ◽  
2019 ◽  
Author(s):  
Nida Sajjad ◽  
Kiran Hilal ◽  
Kumail Khandwala ◽  
Muhammad Arshad ◽  
Nasir Uddin
Keyword(s):  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Contrast Enema

scholarly journals Sensitivity and specificity of the Brazilian version of the Montreal Cognitive Assessment – Basic (MoCA-B) in chronic kidney disease

2019 ◽  
Vol 41 (4) ◽  
pp. 327-333
Author(s):  
Thaís Malucelli Amatneeks ◽  
Amer Cavalheiro Hamdan
Keyword(s):  
Chronic Kidney Disease ◽  
Cognitive Impairment ◽  
Kidney Disease ◽  
Sensitivity And Specificity ◽  
Cognitive Assessment ◽  
Montreal Cognitive Assessment ◽  
Cognitive Screening ◽  
Cross Sectional ◽  
Specificity And Sensitivity ◽  
Starting Point

Abstract Introduction Cognitive impairment in chronic kidney disease (CKD) is commonly associated with neuropsychiatric disorders. As a complex pathology, at all stages of CKD patients need to have a good understanding of the need for drug and nutritional adherence. Cognitive screening is the starting point for detection of cognitive impairments. Objective To determine the specificity and sensitivity of the Brazilian Portuguese version of the Montreal Cognitive Assessment – Basic (MoCA-B) for identification of cognitive impairment in the CKD population. Methods This was a cross-sectional study with 163 CKD patients undergoing hemodialysis treatment. The Mini-Mental State Examination (MMSE) and MoCA-B were administered. Results The MoCA-B has reliable internal consistency (Cronbach’s alpha = 0.74). A cutoff point of ≤ 21 points provides the best sensitivity and specificity for detection of cognitive impairment. The education variable had less impact on the total MoCA-B score than on the total MMSE score. Conclusions The MoCA-B is a suitable screening instrument for evaluating the global cognition of hemodialysis patients. The results can help health professionals to conduct evaluations and plan clinical management.

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