Probable Action Dimensions of Madhutailika Yapana Vasti in Hirschsprung's Disease

Hirschsprung’s disease (HD) is a congenital disorder defined by the absence of ganglion cells in terminal rectum which is responsible for non specific symptomatology including chronic constipation which is usually not relieved with oral laxatives. In modern science, the treatment option is surgical removal of affected area followed by anastomosis but more post operative complications and lower quality of life are the major concerns while adopting surgical procedures. Hence the scope of Ayurvedic modalities in HD is to be explored. When we consider the pathology as Udavartha due to deranged Pakwasayagatha Vatha, the prime importance of Vasti can be analyzed. It is the Prakupitha Apana Vayu which leads to Udavartha. Children suffer from chronic accumulation of Mala due to Pratiloma Gati of Vayu which over time becomes hard and dried and may further result in infection. Hence the line of disease management requires Sodhana for the elimination of chronic stasis of Mala with simultaneous Brimhana and Rasayana action for gut brain nourishment and regeneration. Madhutailika Yapana Vasti which is mentioned in Ashtangahrudaya Kalpasidhi Sthana serves these purposes simultaneously. The present work aims at exploring the current knowledge on probable mode of action of Madutailika Yapana Vasti in Hirschsprung’s disease and to highlight the research gaps that we must overcome to further elucidate the vast action dimensions of Yapana Vasti in the same disease.

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Explaining Change Over Time in Quality of Life of Adult Patients With Anorectal Malformations or Hirschsprung's Disease

Diseases of the Colon & Rectum ◽

10.1007/s10350-005-0216-x ◽

2005 ◽

Cited By ~ 1

Author(s):

Esther E. Hartman ◽

Frans J. Oort ◽

Mechteld R. Visser ◽

Mirjam A. Sprangers ◽

Marianne J. G. Hanneman ◽

...

Keyword(s):

Quality Of Life ◽

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Anorectal Malformations ◽

Adult Patients ◽

Change Over Time ◽

Over Time

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Dysmorphic Neurofilament-Positive Ganglion Cells in the Myenteric Plexus at the Proximal Resection Margin Indicate Worse Postoperative Prognosis in Hirschsprung’s Disease

Pediatric and Developmental Pathology ◽

10.1177/1093526619878083 ◽

2019 ◽

Vol 23 (3) ◽

pp. 222-229

Author(s):

Jin Zhu ◽

Ying Zhang ◽

Yi Wang ◽

Shanshan Yu ◽

Yanling Chen ◽

...

Keyword(s):

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Resection Margin ◽

Ganglion Cells ◽

Distal Colon ◽

Postoperative Recovery ◽

Surgical Removal ◽

Colon Tissue ◽

Eosinophilic Cytoplasm ◽

Proximal Resection Margin

Background Hirschsprung’s disease (HD) is a congenital disorder affecting neonates that presents with distal intestinal obstruction. It is the most common type of anorectal malformation. Treatment of HD consists of surgical removal of the distal colon including the most distal aganglionic segment, the transitional zone, and a prudent length of proximal colon that is determined during the surgical procedure to be normally ganglionated by intraoperative demonstration of normal ganglion cells up to and including the surgical resection margin. Methods In a retrospective study of formalin-fixed paraffin-embedded colon tissue from the proximal resection margin (PRM) of 209 HD patients, we made morphometric measures and detected immature ganglion cells defined as dysmorphic by immunohistochemical demonstration of cytoplasmic neurofilament (NF). Results The majority of NF-positive ganglion cells in HD patients appeared immature, with less cytoplasm. Occasional positive ganglion cells in the same patients appeared mature with abundant eosinophilic cytoplasm, Nissl bodies, prominent nucleoli, and adjacent glial cells. Patients with NF-positive ganglion cells in the myenteric plexuses at the PRM may have poor postoperative recovery. Conclusion We propose that NF expression in dysmorphic ganglion cells at the PRM may predict poor outcome after pull-through surgery for HD.

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Explaining Change Over Time in Quality of Life of Adult Patients With Anorectal Malformations or Hirschsprung's Disease

Diseases of the Colon & Rectum ◽

10.1007/s10350-005-0216-4 ◽

2006 ◽

Vol 49 (1) ◽

pp. 96-103 ◽

Cited By ~ 26

Author(s):

Esther E. Hartman ◽

Frans J. Oort ◽

Mechteld R. Visser ◽

Mirjam A. Sprangers ◽

Marianne J. G. Hanneman ◽

...

Keyword(s):

Quality Of Life ◽

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Anorectal Malformations ◽

Adult Patients ◽

Change Over Time ◽

Over Time

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Calretinin Expression in Hirschsprung’s Disease – A Potential Marker of Ganglion Cells

Annals of Punjab Medical College ◽

10.29054/apmc/17.362 ◽

2017 ◽

Vol 11 (3) ◽

pp. 181-186

Author(s):

Mishal Sikandar ◽

Abdul Hannan Nagi ◽

Komal Sikandar ◽

Nadia Naseem ◽

Ihtisham Qureshi

Keyword(s):

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Ganglion Cells ◽

Potential Marker

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Outcomes in Hirschsprung’s disease with coexisting learning disability

European Journal of Pediatrics ◽

10.1007/s00431-021-04129-5 ◽

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.

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

European Journal of Pediatric Surgery ◽

10.1055/s-0037-1604115 ◽

2017 ◽

Vol 28 (05) ◽

pp. 445-454 ◽

Cited By ~ 7

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.

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Long-term outcome and quality of life after the Swenson procedure for Hirschsprung's disease

Journal of Pediatric Surgery ◽

10.1053/jpsu.2002.31625 ◽

2002 ◽

Vol 37 (4) ◽

pp. 639-642 ◽

Cited By ~ 83

Author(s):

YuZuo Bai ◽

Hui Chen ◽

Jing Hao ◽

Ying Huang ◽

WeiLin Wang

Keyword(s):

Quality Of Life ◽

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Term Outcome ◽

Long Term Outcome

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Hirschsprung's Disease (Anganglionic megacolon)

PEDIATRICS ◽

10.1542/peds.24.1.39 ◽

1959 ◽

Vol 24 (1) ◽

pp. 39-39

Keyword(s):

Surgical Technique ◽

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Ganglion Cells ◽

Rectal Mucosa ◽

Diagnostic Value ◽

Radiologic Diagnosis ◽

Comprehensive Review ◽

Diagnosis And Management ◽

Original Technique

This is a concise and comprehensive review of the essentials in the diagnosis and management of megacolon. The author pioneered in the development of a surgical technique for the treatment of megacolon. The experience with this technique has now become extensive and it has been remarkably successful. Comment on modifications of the original technique which have been proposed indicates that none of the modifications are superior and some are inferior. Particular attention is given to the diagnostic value of biopsy of the rectal mucosa and search for the characteristic abnormality, namely, failure of development of ganglion cells. Pitfalls in the radiologic diagnosis and pecularities of the disease in the newborn are discussed. This article contains many practical points regarding the diagnosis and management of megacolon.

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