A prospective observational study of associated anomalies in Hirschsprung’s disease

Background: Hirschsprung's disease may lead to gastrointestinal obstruction if not appropriately treated. The initial-surgery or colostomy is intended to decompress the large bowel, prevent enterocolitis, and the stooling-patterns can be measured after definitive surgery. Objective: To determine the correlation between initial-surgery age with stooling-patterns. Materials and Methods: We performed an analytic observational study in Ulin Hospital in Banjarmasin, South Kalimantan, Indonesia, from November to December 2015 with a total sample of 31 patients. The initial-surgery age was divided into before and after 30 days, while the stooling-patterns were divided into satisfied and not satisfied. Results: There was no significant correlation between initial-surgery age with stooling patterns. Most of the patients, 18/31 (58.1%), underwent initial-surgery after 30 days, and 23/31 (74.2%) had satisfied stooling patterns after surgery. Conclusion: No significant correlation between initial-surgery age and stooling patterns among Hirschsprung's disease patients.

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Hirschsprung's disease in the North of England: Prevalence, associated anomalies, and survival

Birth Defects Research Part A Clinical and Molecular Teratology ◽

10.1002/bdra.23016 ◽

2012 ◽

Vol 94 (6) ◽

pp. 477-480 ◽

Cited By ~ 15

Author(s):

Kate E. Best ◽

Svetlana V. Glinianaia ◽

Mary Bythell ◽

Judith Rankin

Keyword(s):

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Associated Anomalies ◽

The North

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Profile of Hirschsprung’s disease cases diagnosed in tertiary care teaching hospital: a retrospective observational study

International Journal of Contemporary Pediatrics ◽

10.18203/2349-3291.ijcp20200678 ◽

2020 ◽

Vol 7 (3) ◽

pp. 551

Author(s):

S. Prabakaran ◽

K. Kasthuri Thilagam

Keyword(s):

Observational Study ◽

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Tertiary Care ◽

Disease Diagnosis ◽

Retrospective Observational Study ◽

Histopathological Study ◽

Full Thickness ◽

Colostomy Site ◽

Surgery Department

Background: To date, there are very few studies of Hirschsprung’s disease. Hence, the study was conducted to profile Hirschsprung’s disease in a tertiary care setting.Methods: This is a retrospective observational study that evaluated the admitted patients with Hirschsprung’s disease in the pediatric surgery department. Biopsy was taken from the patient in the form of the full thickness of intestine, seromuscular biopsy, resection from the colostomy site when doing closure and or appendix was taken and subjected to histopathological study with routine eosin and haematoxylin stain.Results: Among the study population, 21(28.8%) children were aged less than one month, and only 10(13.7%) were aged 11 years and above. There were 45(61.6%) participants were male and 28(38.4%) female. Most common biopsy site was appendix in 33(46.2%), followed by Ileum full-thickness biopsy in 6(8.2%) , Ileum Seromuscular biopsy in 5(6.8%%) ,Colon full-thickness biopsy in 4(5.6%) subjects. Among the 73 participants, 46(63%) were clinically suspected cases, and the remaining 27(36.9%) were clinically established cases. Among clinically suspected, the majority (50.7%) had ganglionic cells only in proximal segments.Conclusions: the Hirschsprung's disease diagnosis was established mostly in younger males.

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A prospective observational study of associated anomalies in hirschsprung’s disease

International Journal of Contemporary Pediatrics ◽

10.18203/2349-3291.ijcp20195737 ◽

2019 ◽

Vol 7 (1) ◽

pp. 112

Author(s):

Monali Madhukar Patole ◽

Mandar Madhukar Patole ◽

Subhasish Paul

Keyword(s):

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Ganglion Cells ◽

Heart Diseases ◽

Genetic Disorders ◽

Tertiary Care ◽

Standards Of Care ◽

Associated Anomalies ◽

Variable Expression ◽

Medical College

Background: Hirschsprung’s Disease (HSCR) is the commonest congenital gut motility disorder and is characterized by the absence of ganglion cells in a variable length of the distal gut. According to literature , HRSCD may be associated with a chromosomal abnormality or other congenital anomalies in approximately 20% of cases HSCR appeared to be a multifactorial malformation with low, sex dependent penetrance and variable expression according to the length of the a ganglionic segment, suggesting the involvement of one or more gene (s) with low penetrance. So far, eight genes have been found to be involved in HSCR. This frequent congenital malformation now stands as a model for genetic disorders with complex patterns of inheritance. The objective of this study was to collect and study personal and family history and any information regarding known associated anomalies in patients diagnosed as Hirschsprung's disease.Methods: Here author present a series of 89 consecutive HSCR patients who were admitted or attended the Pediatric Surgery OPD of in a tertiary care Government medical college, who were evaluated for associated malformations with the help of available non-invasive investigation.Results: Congenital heart diseases and Down’s syndrome were the two most common associated anomalies. Genitourinary anomalies, Limb anomalies and central nervous system anomalies were other associated anomalies. Conclusions: This study confirmed the underestimation of certain associated anomalies in Hirschsprung patients, such as CNS anomalies. Based on the results of this study, in addition to meticulous general examination author suggest performing cardiologic assessment in patients diagnosed with Hirschsprung’s disease based on clinical features and according to the standards of care adopted for the general population.

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Hirschsprung's Disease in the Preterm Infant: Implications for Diagnosis and Outcome

The American Surgeon ◽

10.1177/000313481307900720 ◽

2013 ◽

Vol 79 (7) ◽

pp. 734-738 ◽

Cited By ~ 7

Author(s):

Katherine J. Baxter ◽

Amina M. Bhatia

Keyword(s):

Preterm Infant ◽

Preterm Infants ◽

Surgical Outcomes ◽

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Clinical Presentation ◽

Ganglion Cells ◽

Delayed Diagnosis ◽

Associated Anomalies ◽

Pull Through

Hirschsprung's disease (HD), congenital absence of ganglion cells, is considered uncommon in preterm infants. The aim was to describe the frequency, presentation, and surgical outcomes of preterm infants with HD. A retrospective cohort study was conducted of all patients diagnosed with HD from 2002 to 2012 at a single children's hospital. Clinical presentation and surgical outcomes were obtained for term (37 weeks of gestation or greater) and preterm infants. One hundred twenty-nine subjects with HD were identified, 24 (19%) preterm and 105 (81%) term. Preterm infants were more likely to be diagnosed after 30 days of life (66.7 vs 37.1%, P < 0.01; median age 2.9 vs 0.3 months, P < 0.05) and to have associated major congenital anomalies (45.8 vs 20.0%, P < 0.01). Fewer preterm infants had primary pull-through operations (45.8 vs 76.2%, P < 0.005). Preterm infants were more likely to have an episode of Hirschsprung's-associated enterocolitis (45.8 vs 24.0%, P < 0.05) but were not more likely to die from any cause (8.3 vs 5.8%, P = 0.64). HD may be more common in preterm infants than previously recognized, and increased comorbidities in these patients may lead to delayed diagnosis and increased morbidity. HD should be considered in the preterm infant presenting with a bowel obstruction, especially when accompanied by associated anomalies.

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