Early history of the therapy of Hirschsprung's disease: Facts and personal observations over 50 years

1996 ◽  
Vol 31 (8) ◽  
pp. 1003-1008 ◽  
Author(s):  
Orvar Swenson
Keyword(s):  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Early History ◽  
History Of

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.

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%).

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 Disparities in the gut metabolome of post-operative Hirschsprung's disease patients

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Vera Plekhova ◽  
Ellen De Paepe ◽  
Katrien Van Renterghem ◽  
Myriam Van Winckel ◽  
Lieselot Y. Hemeryck ◽  
...  
Keyword(s):  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Heparan Sulphate ◽  
Amino Sugar ◽  
Mucosal Barrier ◽  
Structural Abnormality ◽  
Targeted Analysis ◽  
Tyrosine Metabolism ◽  
Life Threatening ◽  
History Of

AbstractHirschsprung's disease (HD) is a congenital structural abnormality of the colon seen in approximately 1 to 5000 live births. Despite surgical correction shortly after presentation, up to 60% of patients will express long-term gastrointestinal complaints, including potentially life-threatening Hirschsprung-associated enterocolitis (HAEC). In this study fecal samples from postoperative HD patients (n = 38) and their healthy siblings (n = 21) were analysed using high-resolution liquid chromatography—mass spectrometry aiming to further unravel the nature of the chronic gastrointestinal disturbances. Furthermore, within the patient group, we compared the faecal metabolome between patients with and without a history of HAEC as well as those diagnosed with short or long aganglionic segment. Targeted analysis identified several individual metabolites characteristic for all HD patients as well as those with a history of HAEC and long segment HD. Moreover, multivariate models based on untargeted data established statistically significant (p < 0.05) differences in comprehensive faecal metabolome in the patients’ cohort as a whole and in patients with a history of HAEC. Pathway analysis revealed the most impact on amino sugar, lysine, sialic acid, hyaluronan and heparan sulphate metabolism in HD, as well as impaired tyrosine metabolism in HAEC group. Those changes imply disruption of intestinal mucosal barrier due to glycosaminoglycan breakdown and dysbiosis as major metabolic changes in patients’ group and should be further explored for potential diagnostic or treatment targets.

scholarly journals Hirschsprung’s disease presenting as intractable anemia: a report of two cases and review of the literature

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Xiaoang Sun ◽  
Jun Chu ◽  
Chenchen Li ◽  
Zhaohui Deng
Keyword(s):  
Blood Transfusion ◽  
Iron Supplementation ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Clinical Presentation ◽  
Poor Response ◽  
Hemoglobin Level ◽  
Oral Iron ◽  
History Of ◽  
Medical Histories

Abstract Background This report summarizes the clinical characteristics of intractable anemia as part of the clinical presentation of Hirschsprung’s disease (HD) and aims to strengthen clinicians’ ability to recognize early signs of HD. Case presentation An 11-year-old boy with a 6-year history of intractable anemia, low hemoglobin level (55 g/L), poor response to oral iron supplementation and blood transfusion, and difficulty with defecation was diagnosed with HD. A 19-month-old boy with a 3-month history of intractable anemia, low hemoglobin level (64 g/L), poor response to oral iron supplementation and blood transfusion, delayed meconium passage, and history of intestinal obstruction was also diagnosed with HD. Both patients underwent surgery, after which anemia was corrected effectively in both cases. Two more cases of intractable anemia as the chief complaint and diagnoses of HD over different durations since the onset of anemia (ranging from 1.7 years to 21 years) were identified in a literature search. Both patients underwent surgery, after which anemia was corrected. Conclusions Intractable anemia as part of the clinical presentation of HD is extremely rare. Detailed inquiries of medical histories and physical examinations are key to early diagnoses and preventing misdiagnoses. Anemia in HD patients may primarily be caused by impaired iron absorption due to HD.

scholarly journals Hirschsprung’s Disease: A Rare Adult Diagnosis

2020 ◽  
Vol 4 (3) ◽  
pp. 480-481
Author(s):  
Kaitlyn Schmutz ◽  
Gaea McGaig ◽  
B. Jason Theiling
Keyword(s):  
Computed Tomography ◽  
Emergency Department ◽  
Young Adult ◽  
Chronic Constipation ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Failure To Thrive ◽  
Case Presentation ◽  
Gut Colonization ◽  
History Of

Case Presentation: Approximately 94% of patients with Hirschsprung’s disease (HD) are diagnosed before the age of five. In our case, a young adult with years of constipation presented to the emergency department with significant abdominal distention. He was ultimately diagnosed with HD, which was identified using computed tomography (CT). Discussion: In HD, we find defects in gastric motility due to improper gut colonization. Without childhood recognition, HD often leads to chronic constipation and failure to thrive in adulthood. CT is a key step in identifying this rare adult diagnosis that should be considered in all patients with a history of chronic constipation.

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