scholarly journals Development of Clinical Referral Score Model for Early Diagnosis of Hirschsprung’s Disease in Suspected Pediatric Patients

Healthcare ◽  
2021 ◽  
Vol 9 (6) ◽  
pp. 678
Author(s):  
Jiraporn Khorana ◽  
Phawinee Phiromkanchanasak ◽  
Jitthiwimon Kumsattra ◽  
Suparada Klinoun ◽  
Suthasinee Aksorn ◽  
...  
Keyword(s):  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Clinical Score ◽  
Contrast Enema ◽  
Term Infant ◽  
Clinical Parameters ◽  
Rectal Suction Biopsy ◽  
Clinical Scoring ◽  
History Of ◽  
Score Model

The diagnosis of Hirschsprung’s disease (HSCR) relies on history, physical examination, and investigations. Some of investigation modalities could not be done in primary hospital. This study was aimed to develop the clinical score model for diagnosing and early referrals of HSCR, especially in areas where investigations were not available. Overall 483 consecutive suspected HSCR patients who were under 15 years old from January 2006 to December 2020 were included in this study, with 207 (42.86%) patients diagnosed with HSCR and 276 (51.14%) patients in the non-HSCR group. Five clinical parameters were included in the prediction model. The AuROC of clinical parameters, which included having an age younger than one month, male gender, the term infant, history of delayed meconium passage, and history of enterocolitis, was 72%. The prediction score ranged from 0–7, with a score 0–3 meaning a low risk to be HSCR (LHR+ = 0.37). We concluded that patients with suspected HSCR who had clinical score 4–7 had a high probability to be HSCR and, thus, it was suggested that these patients have an early referral for further investigations, which were contrast enema and rectal suction biopsy. In the case of a low probability of HSCR, clinical observation is still warranted. This clinical scoring system can be used as a screening tool to prevent delay diagnosis and complications.

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 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 A Case Report on 2 years Child: Hirschprung’s Disease

2021 ◽  
pp. 558-564
Author(s):  
Anushri Kale ◽  
Aditi Badwaik ◽  
Pallavi Dhulse ◽  
Archana Maurya ◽  
Bibin Kurian
Keyword(s):  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Ganglion Cells ◽  
Past History ◽  
Rectal Biopsy ◽  
Case Presentation ◽  
Surgical History ◽  
History Of ◽  
Pull Through ◽  
Hirschprung's Disease

Introduction: Hirschsprung's disease is the most common cause of large intestinal obstructing in neonates. Hirschsprung's disease is a congenital anomaly caused by migratory failure of neural crest cells leading to abnormal innervations of the bowel .The defect begins in the internal and sphincter and extends proximally for a variable length of gut. Hirschprung's disease is a disorder of the gut caused due to congenital absence of ganglion cells in the sub-mucosal and myenteric plexus of intestine. This disease is also known as megacolon or congenital Aganglionic megacolon. Case presentation: A 2 years old male child, known case of Hirschprung’s disease, was brought to AVBRH for further management. As narrated by the mother, the child was not passing stool since birth. There was no history of abdominal distention, vomiting or fever. The child pass the stool with the help of enema which was given to the child in the morning and evening. Mast. Devansh has past history of NICU stay. In View of not passing stools, USG of abdomen and pelvis done. Endorectal pull through surgery was done on 4/02/2021 under general anesthesia. No any past surgical history. There is a past history of rectal biopsy done in 1 1/2 month of age. Conclusion:The patient was admitted in AVBRH for further management. Then the report mainly focused on surgical management and quality nursing care due to which patient was discharged without any further complication and satisfaction.

Calretinin versus Hematoxylin and Eosin Stain for Diagnosis of Hirschsprung’s Disease; Comparison in Ganglionic, Transitional, and Aganglionic Zones

2020 ◽  
Vol 103 (6) ◽  
pp. 559-565
Keyword(s):  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Ganglion Cells ◽  
Transitional Zone ◽  
Diagnostic Study ◽  
Double Blind ◽  
Muscular Layer ◽  
Rectal Suction Biopsy ◽  
Suction Biopsy ◽  
Hematoxylin And Eosin

Objective: To compare the results of calretinin stain with hematoxylin and eosin (H&E) stain for diagnosis of Hirschsprung’s disease. Materials and Methods: A prospective double-blind diagnostic study was done. Hirschsprung’s patients who underwent transanal endorectal pull through (TERPT) surgery between February 2015 and February 2017 were included. The pulled-through specimens were sampled at the ganglionic, transitional, and aganglionic zones. Each specimen was stained with calretinin and H&E. Agreement and kappa analysis were done. Results: Fifty-one Hirschsprung’s patients (153 specimens) were included for analysis. There were 37 males (72.5%) and 14 females (27.5%) with a median age at surgery of four months. Thirty-one specimens showed a negative stain for calretinin (aganglionic bowel) and 33 specimens showed no ganglion cells in the H&E stain. One hundred twenty-two specimens in calretinin stain were consistently positive with ganglionic bowel and 120 specimens in the H&E stain showed ganglion cells. Agreement and Cohen’s kappa coefficient were 97.4% and 0.921 (95% confidence interval 0.845 to 0.997), respectively. Disconcordance was found in four specimens. Three out of four were in the transitional zone. One was in the aganglionic zone, which has no muscular layer attached. (Thirty-six specimens had no muscular layer.) Conclusion: Calretinin stain was found to be comparable with the H&E stain and could be used for diagnosis of Hirschsprung’s disease. In rectal suction biopsy specimen in which the muscular layer was not included, both calretinin and H&E can be used. Keywords: Megacolon, Hematoxylin and eosin (H&E), Calretinin, Rectal suction biopsy (RSB), Full thickness biopsy

Rectal Biopsy as an Aid in the Diagnosis of Hirschsprung's Disease

PEDIATRICS ◽  
1956 ◽  
Vol 18 (2) ◽  
pp. 176-176
Keyword(s):  
Chronic Constipation ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Rectal Wall ◽  
Rectal Biopsy ◽  
Distal Segment ◽  
Large Bowel Obstruction ◽  
Atypical Symptoms ◽  
Typical History ◽  
History Of

The child with a typical history of chronic constipation, and with physical signs of a distended abdomen and an empty rectal ampulla, who is found to have a widely dilated colon with a narrow distal segment by the radiologist, presents no diagnostic problem. However, the diagnosis of Hirschsprung's disease may be very difficult to establish if part of the classical diagnostic criteria are absent or inconclusive. In such cases, the authors have found rectal biopsy to be a useful diagnostic procedure. They cite as indications for biopsy: (1) suspected Hirschsprung's disease in the newborn infant, because the colon does not become dilated and hypertrophied until the intestinal tract has been functioning for several months; (2) symptoms of upper large bowel obstruction developing soon after birth, because barium enema studies of infants whose entire colons are aganglionic reveal no obstruction of the lumen of the large intestine; (3) radiologic evidence of dilatation of the entire distal segment of the colon, because, in patients with a short aganglionic lesion, the obstruction may be so low that the radiologist is unable to demonstrate a narrow segment extending through the rectosigmoid, and (4) atypical symptoms of megacolon, including diarrhea, episodes of intestinal obstruction, or other symptoms of colonic dysfunction. This procedure has been performed by Swenson and his colleagues on 40 patients. Biopsy specimens obtained from 19 patients, subsequently proved to have Hirschsprung's disease by study of the resected rectum and sigmoid, contained no ganglionic cells. In the other 21 patients, biopsy was performed because Hirschsprung's disease was suspected. Ganglionic cells were present in all of these specimens, and the success of subsequent conservative management of these patients confirmed the diagnosis of chronic constipation. Control material was obtained from 10 cadavers with normal colons, and ganglionic cells were found in all of these specimens. The authors emphasize that this technique is only as good as the specimen obtained. An adequate biopsy of rectal wall must be removed. Multiple histologic sections must be carefully examined before the diagnosis of Hirschsprung's disease is confirmed.

TREATMENT OF CONGENITAL MEGACOLON IN 50 INFANTS

PEDIATRICS ◽  
1966 ◽  
Vol 38 (2) ◽  
pp. 185-193
Author(s):  
Walton K. T. Shim ◽  
Orvar Swenson
Keyword(s):  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Average Weight ◽  
Older Children ◽  
The Past ◽  
History Of ◽  
Pull Through ◽  
Definitive Surgery ◽  
Criteria For Selection ◽  
Selection Of

Fifty infants less than 1 year old with Hirschsprung's disease were treated at Children's Memorial Hospital in the past 6 years. Thirty-two of the 35 barium enema studies yielded "diagnostic" roentgenographic studies, and 3 were "negative" for Hirschsprung's disease. Fourteen of the 50 infants had a history of diarrhea. Enterocolitis in infants may be extremely rapid in onset and progression. It should be vigorously treated with rectal irrigations and intravenous fluids. Nineteen patients (38%) had aganglionic segments extending above the rectosigmoid colon, and eight (16%) were aganglionic proximal to the splenic flexure. This includes three patients with aganglionosis of the entire colon and one that was aganglionic from the jejunum to anus. Emphasis is placed on an aggressive combined attack by pediatrician, radiologist, pathologist, and surgeon to diagnose and prepare the sick infant as early as possible for colostomy or definitive abdominal-perineal pull-through operation. Twelve infants with an average weight of 12.5 lb were operated on primarily with no deaths or complications. It is our belief that definitive surgery can be done with greater ease in an infant than in older children, but careful selection and preparation of the patient and meticulous operative technique must be exercised. The important criteria for selection of infants for immediate resection are the absence of emaciation and enterocolitis, although the infant may be small. Twenty-six infants had colostomy and resection with no deaths or complications. Our raw mortality figures show 10 deaths in 50 patients (20%). There were 7 deaths in 47 surgical patients (15%).

Modified Duhamel Retrorectal Pull-Through for Hirschsprung’s Disease

2021 ◽  
Vol 15 (10) ◽  
pp. 2886-2889
Author(s):  
Shafiq ur Rehman ◽  
Muhammad Anwar ◽  
Zarlish Fazal
Keyword(s):  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Operation Time ◽  
Clinical Score ◽  
Toilet Training ◽  
Paediatric Surgery ◽  
Short Term ◽  
Female Ratio ◽  
Bowel Habits ◽  
Pull Through

Objectives: To evaluate the post-operative complications and short-term outcomes of modified Duhamel retrorectal pull-through procedure for Hirschsprung’s disease. Design: Prospective descriptive study Place and Duration of Study: Department of Paediatric Surgery Sahiwal Teaching Hospital Sahiwal from 1stJanuary 2018 to 31st December 2020. Methodology: Thirty seven histologically confirmed cases of Hirschsprung’sdisease having previous decompressing colostomy/stoma age between 1 to 12 years of agewere included. Children under one year of age, with sever comorbidities like Down syndrome and cardiac abnormalities, and those who require re-do pull through procedure were excluded. Modified Duhamel retrorectal pull-through procedure was performed in all cases. The demographic information included age, sex, proximal level of aganglinosis, complications of definite procedure, length of hospitalization andmortality. Other information recorded included long term complications like obstructive symptoms, enterocolitis, incontinence and soiling at follow up. Krickenbeck classification was used to evaluate faecal incontinence and constipation. Kelly’s clinical score was used to assess the anal sphincter. Bowel habits were assessed only in children above three years of age. Results: Twenty six (70.27%) were males and 11 (29.72%) females with male to female ratio 2.3:1 and mean age at operation was 2.89±1-9 years. Twenty nine (78.37%) children were ≤3 years of age and 8 (21.62%) were >3 years. Mean weight was 12.91 kgs, operation time was 126.81 time, fasting time was 6.67 days and hospital stay was 11.91 days. Length of aganglionic segment was short segment 27 (72.97%), long segment 9 (24.32%) and total colonic 1 (2.7%). Hirschsprung associated enterocolitis 7 (18.91%) and wound infection 6(16.21%) were most common reported complications. Constipation in 5(13.51) and soiling with retentive constipation was present in 3 (8.10%) patients. Out of total 31 patients who reached toilet training age, 28 (90.32%) developed satisfactory voluntary bowel habits. Conclusion: Modified Duhamel pull-through procedure was found to be safe, applicable and with lower associated complications and satisfactory short term functional outcomes in our settings. Key words: Hirschsprung’s disease, Modified Duhamel pull-through procedure, Complications, Outcomes

The Usefulness of Calretinin Immunohistochemistry for Rectal Suction Biopsy to Diagnose Hirschsprung’s Disease

2016 ◽  
Vol 22 (2) ◽  
pp. 23
Author(s):  
Jaeyeop Jeong ◽  
Sang Pyo Kim ◽  
Eunyoung Jung ◽  
Soon-Ok Choi
Keyword(s):  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Rectal Suction Biopsy ◽  
Suction Biopsy
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