Hirschsprung's Disease Patients Research Articles

Dynamic Pathology of Enteric Neural Network using Curcumin-assisted Multiphoton Laser Imaging in Hirschsprung Disease.

In living tissue, it has been difficult to make microscopic-level observations without damaging the tissue. We have invented a novel intravital fluorescent observation method (IFOM) for real-time tissue observation, combining multi-photon laser scanning microscopy (MPLSM) with curcumin vital staining (CVS-IFOM). The aim of this study was to use CVS-IFOM to analyze the enteric nervous system (ENS) in mice and human patients with hypoganglionosis and Hirschsprung disease. In an initial viability study, we compared live ENS images from non-fluorescent C57BL6 mice stained with curcumin (n=5) and GFP mice (n=5) using MPLSM. We then explored CVS-IFOM for the live examination of resected colon tissues from one hypoganglionosis and three Hirschsprung disease patients. In the viability study, detailed ENS histological features were only observed in the curcumin-stained mice. In the hypoganglionosis patient, CVS-IFOM provided ENS details that were not visualized under H&E staining or calretinin immunohistochemistry, allowing the analysis of ENS size, neural bundle number, and neural cell number per plexus. In Hirschsprung disease patients, CVS-IFOM showed a gradual hypoplastic change in the ENS from the oral wedge to the anal wedge, detecting disproportionate changes in the ENS within the same intestinal level, supporting a circumferentially uneven distribution of the intestinal ENS. CVS-IFOM may be supportive for intraoperative pathological diagnosis during surgeries in Hirschsprung disease.

Surgical Outcomes for Patients With Trisomy 21 and Hirschsprung's Disease: An NSQIP-Pediatric Study

IntroductionPrevious studies have demonstrated worse outcomes for Hirschsprung's disease (HD) procedures in Trisomy 21 (T21) patients. Using a large national database, we sought to investigate surgical outcomes in HD patients with T21 compared to non-T21 patients. MethodsWe utilized the deidentified National Surgical Quality Improvement Program Pediatric database from 2012 to 2021. Using International Classification of Diseases, Ninth Revision codes, children <18 y old with HD were included and stratified by T21 diagnosis. Demographics, Current Procedural Terminology codes, case characteristics, length of hospital stay, and postoperative complications were analyzed. ResultsOf 3456 HD patients, 12.0% (n = 413) patients had a concurrent diagnosis of T21. Pull-through (PT) procedures accounted for 54.9% of surgeries (n = 1896), of which 10.0% (n = 189) had T21. T21 patients who underwent PT had a younger gestational age (P < 0.0001), cardiac risk factors (P < 0.0001), hematologic disorders (P < 0.0001), higher American Society of Anesthesiologists class (P < 0.0001), and were older at their index operation (P = 0.03). Though operative times were similar, T21 patients had a longer total length of stay (P = 0.0263), postoperative length of stay (P = 0.0033), and more unplanned reoperations (P = 0.0094). Though only significant in unadjusted analyses, T21 patients had more postoperative complications after PT (P = 0.0034), specifically deep surgical site infections (P = 0.009), organ/space surgical site infections (P = 0.004), wound disruption (P < 0.001), and sepsis (P = 0.025). ConclusionsWe confirm significant differences exist between T21 and non-T21 patients undergoing HD procedures, particularly increased total length of stay, postoperative length of stay, and unplanned reoperations. Understanding these differences will lead to more optimal treatment plans for this unique patient population.

Short-Term Complications Between Duhamel and Soave Procedures in Hirschsprung Patients: A Systematic Review

Hirschsprung's disease, or congenital megacolon, is a birth defect where ganglions are absent in the gut wall, leading to constipation and intestinal obstruction. It affects 1 in 500 births worldwide and, if untreated or treated late, significantly impacts children's growth and quality of life. Understanding the short-term complications of the Soave and Duhamel methods in Hirschsprung disease patients. A systematic review was conducted based on the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. In our initial PubMed search and hand-searching, we identified 36 articles. After screening titles and abstracts, four were relevant for discussion. We included three articles in our systematic review after reading the full texts. Enterocolitis was more frequent with the Duhamel procedure (28%) compared to the Soave procedure (10%). The risk of Hirschsprung-associated enterocolitis (HAEC) increased in patients with a history of enterocolitis in both treatment groups. Two studies indicate that short-term complications such as constipation and bloating occur more frequently after the Soave procedure than the Duhamel procedure. Based on the three studies we discussed, HAEC was more frequent in the Duhamel procedure than in the Soave procedure, with significant differences. Constipation was more common in the Soave procedure. Both procedures may cause complications such as voluntary bowel movement, soiling, and fecal incontinence, with no statistically significant differences. Minor complications in the Soave procedure include poor weight gain and strong-smelling flatulence. In the Duhamel procedure, minor complications include hardened stools and poor appetite.

The Role of Patient Organisations-Patients' and Parents' Views and Experience of Hirschsprung's Disease.

In many countries, patient organisations offer advice and the exchange of experiences to Hirschsprung's disease patients and their families. Professional treatment by experienced health care providers and the availability of life-long multidisciplinary follow-up care are essential. However, outside the clinic, patients and their families have to manage life on a day-to-day basis at home, which often brings up uncertainties and questions: Parents go through different stages during the diagnosis and treatment of their child, the affected children themselves go through many different stages of development, and even through the course of adulthood, new questions regarding the chronic disease may arise. Patient organisations can support the patients and their families at all stages of life by listening, offering information in an understandable way, connecting people, and sharing others' experiences. This enables families and patients to develop a better understanding of the rare disease and promotes their management strategies and confidence. The holistic approach of patient organisations aims to complement the medical treatment. Therefore, the referral of all patients and their families to patient organisations should be part of the medical advice in the treatment of Hirschsprung's disease.

Treatment of Persistent Soiling in Hirschsprung Disease With Antegrade Continence Enemas

IntroductionPatients with Hirschsprung disease (HD) can experience soiling after their pull-through. We evaluated the use of antegrade continence enema (ACE) for this patient population and investigated the timing and factors associated with getting the patient clean. MethodsWe performed a single-institution retrospective review (from January 2020 to January 2023) of patients with HD and prior pull-through who had persistent soiling, failed laxative treatment or rectal enemas, and were treated with antegrade enemas. The primary outcome was time to become “clean of stool” which was defined as at least one bowel movement per day, no more than one stool accident per week, and no significant stool burden radiographically. Kaplan–Meier survival analysis was performed, and univariate cox proportional hazard regression was used to assess factors associated with time to continence. ResultsThirty patients who met the criteria underwent ACE creation at a median age of 6.5 y (interquartile range 5.3-9.8) with a median follow-up time of 11.5 mo (interquartile range 5.6-16.5). Most patients became clean by 4 mo (13 of 20, 65%) with similar results at 1-y follow-up (16 of 21, 76%). The median time to becoming clean was 4.3 mo (95% confidence interval: 1.7-15.0. Patients with hypermotility were more likely to continue to soil at 1 y (80% versus 13%, P = 0.01). There were no additional factors significantly associated with time to cleanliness. ConclusionsACE is a useful modality for HD patients with soiling. Most became clean of stool in 4 mo. The presence of hypermotility was associated with a higher incidence of persistent soiling at 1 y.

Diagnosis of Hirschsprung disease by analyzing acetylcholinesterase staining using artificial intelligence.

Classical Hirschsprung disease (HD) is defined by the absence of ganglion cells in the rectosigmoid colon. The diagnosis is made from rectal biopsy, which reveals the aganglionosis and the presence of cholinergic hyperinnervation. However, depending on the method of rectal biopsy, the quality of the specimens and the related diagnostic accuracy varies substantially. To facilitate and objectify the diagnosis of HD, we investigated whether software-based identification of cholinergic hyperinnervation in digitalized histopathology slides is suitable for distinguishing healthy individuals from affected individuals. N = 190 samples of 112 patients who underwent open surgical rectal biopsy at our pediatric surgery center between 2009 and 2019 were included in this study. Acetylcholinesterase (AChE) stained slides of these samples were collected and digitalized via slide scanning and analyzed using two digital imaging software programs (HALO, QuPath). The AChE-positive staining area in the mucosal layers of the intestinal wall was determined. In the next step machine learning was employed to identify patterns of cholinergic hyperinnervation. The area of AChE-positive staining was greater in HD patients compared to healthy individuals (p < 0.0001). Artificial intelligence-based assessment of parasympathetic hyperinnervation identified Hirschsprung disease with a high precision (area under the curve [AUC] 0.96). The accuracy of the prediction model increased when nonrectal samples were excluded (AUC 0.993). Software-assisted machine-learning analysis of AChE staining is suitable to improve the diagnostic accuracy of Hirschsprung disease.

Intradyadic Correlation Between Parent-reported and Child-reported Quality of Life in Patients With Anorectal Malformation and Hirschsprung's Disease in Comparison to Healthy Controls

BackgroundQuality of life (QOL) is an important clinical endpoint in paediatric chronic conditions. How parent-proxy reports differ from child self-reported QOL in patients with anorectal malformation (ARM) and Hirschsprung's disease (HD) has not been well examined to date. This study evaluates agreement between parent-proxy and child-reported QOL scores in ARM and HD patients compared to healthy controls. MethodsWe recruited ARM and HD patients aged 5–17 years and their parents at four tertiary referral centres between December 2020 to February 2023 who had corrective surgery done >12 months prior. Healthy controls were age-matched and gender-matched. They completed the Pediatric Quality of Life Inventory™ (PedsQL™) Generic Core Scales and General Well-Being (GWB) Scale. The questionnaires were administered in parallel parent-proxy-report and child self-report formats. Appropriate statistical analysis was performed with p < 0.05 significance. Data are reported as median (interquartile range). Ethical approval was obtained. ResultsThere were 65 ARM, 54 HD and 83 controls. There were no significant differences between parent-reported scores and child-reported scores overall in the Total, Psychosocial Health and Physical Health components of Core Scales for ARM, HD and controls. However, parent-reported scores were significantly higher than child-reported scores overall in ARM, HD and controls in the GWB Scale. ConclusionOur findings show that parent-rated and child-rated overall QOL was similar in Core Scales for ARM, HD, and controls. However, parents overestimated child's overall GWB for ARM, HD and controls. This highlights the importance of capturing the perspectives of both parents and children to inform strategies to improve patient care. Level of EvidenceLevel III.

A multi-center cross-sectional comparison of parent-reported quality of life and bowel function between anorectal malformation and Hirschsprung's disease patients with versus those without Down syndrome.

Down syndrome (DS) is a common abnormality associated with anorectal malformation (ARM) and Hirschsprung's disease (HD). However, quality of life (QOL) in ARM and HD patients with DS is under-researched. This study compares parent-reported QOL and bowel function in ARM and HD patients with DS to those without. Between December 2020 to February 2023, parents of ARM and HD patients with and without DS aged 3-17 years who had undergone surgery > 12 months prior at four tertiary referral centers were recruited. We used the Pediatric Quality of Life Inventory™ (PedsQL™) Generic Core Scales, General Well-Being (GWB) Scale and Family Impact (FI) Module questionnaires, and the Rintala bowel function score (BFS). There were 101 ARM, 9 (8.9%) of whom had DS; and 87 HD, of whom 6 (6.9%) had DS. Parent-reported Core scores in ARM and HD with DS were comparable to those without DS. However, ARM and HD with DS had worse scores in the FI Module and bowel function than those without DS. Although parent-reported QOL in ARM and HD with DS is similar to those without DS, family impact and BFS are worse. Our findings are limited by small sample size in proportion of DS patients.

Extended total colonic aganglionosis and total intestinal aganglionosis: Challenging enemies

AbstractAimExtended total colonic aganglionosis (ETCA) represents uncommon forms of Hirschsprung disease (HD), with aganglionosis extending into the proximal small bowel. ETCA management is challenging and associated with poor outcomes and high mortality. This study compares management and outcomes of ETCA to more common HD forms.MethodsA retrospective cohort of HD patients (2012–2023) from two institutions. Three HD forms were compared: short‐segment HD (SSHD, n = 19), long‐segment HD or total colonic aganglionosis (LS/TCA, n = 9) and ETCA (n = 7).ResultsNormally innervated segments in ETCA patients ranged 0–70 cm. Median times to first surgery were; ETCA = 3 days versus TCA = 21 days (p = 0.017) and SSHD = 95 days (p &lt; 0.001), respectively. Median number of surgeries were; ETCA = 4, versus TCA = 2 (p = 0.17) and SSHD = 1 (p = 0.002), respectively. All the patients underwent a definitive pull‐through procedure, except four ETCA patients with a permanent jejunostomy and residual aganglionic segment of 57–130 cm. ETCA patients had 92% lower odds of enterocolitis (14%) compared to TCA patients (67%, p = 0.054), and comparable odds to SSHD patients (16%, p = 0.92). ETCA mortality was 14%.ConclusionExtended total colonic aganglionosis patients require earlier and multiple interventions. Leaving an aganglionic segment may be advantageous, without increasing risk for enterocolitis. Tailored surgical treatment and rehabilitation programmes may prevent mortality and need for transplantation.

Neutrophil Lymphocytes Ratio (NLR) of One-Step and Multi-Step Procedure for Hirschsprung Disease Patients At Arifin Achmad General Hospital

Hirschsprung disease (HD) is a congenital anomaly due to intestinal aganglionosis starting from the internal anal sphincter and spreading to the proximal area with varies extension of the segment. Definitive treatment for this abnormality is to perform surgery to remove the aganglionic bowel and can be carried out in one-step and multi-step procedure. In the process of wound healing after surgery, it involves inflammatory mediators as a marker of whether there is a stress response from the endocrine system such as neutrophil-lymphocyte ratio (NLR). This study aims to compare NLR as an inflammatory biomarker in one-step and multi-step procedure of hirschsprung disease patients at Arifin Achmad Hospital. The design of this study was cross-sectional using medical records and using total sampling for number of samples. Age, gender, and method of surgery were evaluated by univariate and bivariate to determine whether there were statistical differences. Seven HD patient taken from Mei-October at Arifin Achmad Hospital taken for evaluated. The results showed that the most samples were in &amp;lt; 1-month-old (42.9%) and male (71,4%) for gender. The average number of NLR was in 7.33 after one-step procedure and 2.77 for multi-step procedure. This number of NLR both not statistically different neither for the post-operative examination nor the comparison between procedure.

Long-term assessment of functional outcome in operated cases of Hirschsprung disease

Background: Hirschsprung disease (HD) is a rare congenital anomaly (1 in 5000 live births) characterized by varying levels of aganglionosis. The evolution of surgical options and outcomes for the successful management of HD has been driven by the aspiration of pediatric surgeons. Hirschsprung's disease postoperative results of pull-through procedure could be influenced by surgical and pathological proficiency and the quality of perioperative care. Controversies persist as to which technique offers the best outcome. The absence of incontinence and constipation remains the most important marker of a good outcome. Methods: Medical records of HD children who underwent temporary colostomy procedures were analyzed retrospectively from July 2017 to June 2021. Risk factors were evaluated to determine the outcome of HD procedures. Results: Ninety-two HD patients were included. Based on the univariate analysis, most patients in the study (58.7%) were between the ages of 2 and 12 months. The study population predominantly consisted of males (68.5%) who had low vegetable intake (65.2%) and occasionally exhibited stool stains (66.3%), but their stool consistency was mainly normal (47.8%). Further examination through bivariate analysis revealed a significant negative correlation between stool stains and SOAVE (name of the condition). Similar findings were observed for stool consistency, indicating a significant correlation. Consequently, both variables were subjected to multivariate analysis. The multivariate analysis demonstrated a significant association between stool consistency and SOAVE among patients aged 2-12 months (p=0.000, OR =2.044E-7) and those aged 1-5 years (p=0.000, OR =2.044E-7). An odds ratio (OR) value of less than 1 implies that individuals with normal stool consistency have a lower likelihood of experiencing SOAVE at a young age. Conclusions: This study showed that children with HD correlated with low daily vegetable intake. Normal feces were associated with higher SOAVE ages and a lower possibility of experiencing SOAVE at a young age.

Experiences of youth growing up with anorectal malformation or Hirschsprung's disease: a multicenter qualitative in-depth interview study.

Patients with anorectal malformation (ARM) and Hirschsprung's disease (HD) live with long-term impact of these diseases even into adulthood. We aimed to explore the physical, social and emotional impact of these diseases in adolescents and young adults to develop best practices for transition care. We conducted one-on-one in-depth interviews with ARM and HD patients aged ≥ 11years who had undergone surgery at four tertiary referral centers. All interviews were audio-recorded and transcribed verbatim. We analyzed transcripts for recurring themes, and data were collected until data saturation was reached. Three researchers independently coded the transcripts for major themes using thematic analysis approach. We interviewed 16 participants (11 males) between October 2022 and April 2023. Ages ranged from 11 to 26years. Five major themes emerged: (1) personal impact (subthemes: physical, emotional and mental health, social, school), (2) impact on family, (3) perceptions of their future (subthemes: relationships, career, state of health), (4) sources of support (subthemes: family, peers, partner), and (5) transition care (subthemes: concerns, expectations).Only females expressed concerns regarding future fertility. This study highlights the evolving problems faced by adolescents and young adults with ARM and HD, especially gender-specific concerns. Our findings can inform efforts to provide individualized care.

Utility of colonic manometry in children with Hirschsprung disease.

Abnormal motility of the residual colon has been reported in post-pull-through Hirschsprung disease (PT-HSCR) patients with persistent defecation problems. We reviewed the role of colonic manometry (CM) in the management of defecation disorders in these patients. We retrospectively reviewed the medical record of PT-HSCR children who underwent CM for persistent symptoms of abnormal defecation. We reviewed their clinical course and its relation to CM findings. Thirty PT-HSCR patients underwent CM, of which five were diagnosed with transition zone pull-through and were excluded. Of the remaining 25 patients, 16 had colonic dysmotility, 8 had normal CM, and one had colonic hypermotility. In patients with dysmotility, five responded to ongoing medical management, three required surgical intervention (ileostomy), three remained symptomatic with medical management but not yet received surgical intervention, and five were lost to follow-up. In patients with normal CM, four responded to ongoing medical therapy, two required additional surgery (antegrade enema procedure), and two were lost to follow-up. The patient with hypermotility improved with adding loperamide. Colonic dysmotility can occur in PT-HSCR patients with persistent defecation problems. CM was helpful in delineating the degree of colonic neuromuscular dysfunction. CM results were used in conjunction with other clinical data to determine optimal management. Our findings support that medical management should first be optimized before consideration of colonic manometry and surgical interventions.

Impact of Colorectal Nurse Specialist supervised parental administration of rectal washouts on Hirschsprung's disease outcomes: a retrospective review.

To highlight the utility of Colorectal Nurse Specialist (CNS) supervised parental administration of rectal washouts in the management of Hirschsprung's disease (HD). Retrospective case note review of HD patients treated at a tertiary children's hospital in United Kingdom from January 2011 to December 2022. Data collected included demographics, complications, enterocolitis, obstructive symptoms and stomas. Primary pull-through (PT) is done 8-12weeks after birth. Parental expertise in performing rectal washouts at home is ensured by our CNS team before and after PT. PT was completed in 69 of 74 HD patients. Rectal washouts were attempted on 63 patients before PT. Failure of rectal washout efficacy necessitated a stoma in four patients (6.4%). Of the 65 patients who had PT and stoma closed, three (4.5%) required a further stoma over a mean follow-up period of 57 months (Range 7-144 months). Two of these had intractable diarrhoea due to Total Colonic Aganglionosis (TCA). One patient (1.5%) had unmanageable obstructive symptoms requiring re-diversion. Hirschsprung-associated enterocolitis (HAEC) requiring hospital admission occurred in 14 patients (21%). Our stoma rates are lower compared to recent UK data. This could potentially be due to emphasis on parental ability to perform effective rectal washouts at home under CNS supervision.

Clinical Relevance of Pathological Diagnosis of Hirschsprung's Disease with Acetylcholine-Esterase Histochemistry or Calretinin Immunohistochemistry.

Hirschsprung disease (HD) manifests as a developmental anomaly affecting the enteric nervous system, where there is an absence of ganglion cells in the lower part of the intestine. This deficiency leads to functional blockages within the intestines. HD is usually confirmed or ruled out through rectal biopsy. The identification of any ganglion cells through hematoxylin and eosin (H&E) staining rules out HD. If ganglion cells are absent, further staining with acetylcholine-esterase (AChE) histochemistry or calretinin immunohistochemistry (IHC) forms part of the standard procedure for determining a diagnosis of HD. In 2017, our Institute of Pathology at University Hospital of Heidelberg changed our HD diagnostic procedure from AChE histochemistry to calretinin IHC. In this paper, we report the impact of the diagnostic procedure change on surgical HD therapy procedures and on the clinical outcome of HD patients. We conducted a retrospective review of the diagnostic procedures, clinical data, and postoperative progress of 29 patients who underwent surgical treatment for HD in the Department of Pediatric Surgery, University of Heidelberg, between 2012 and 2021. The patient sample was divided into two groups, each covering a treatment period of 5 years. In 2012-2016, HD diagnosis was performed exclusively using AChE histochemistry (AChE group, n = 17). In 2017-2021, HD diagnosis was performed exclusively using calretinin IHC (CR group, n = 12). There were no significant differences between the groups in sex distribution, weeks of gestation, birth weight, length of the aganglionic segment, or associated congenital anomalies. Almost half of the children in the AChE group, twice as many as in the CR group, required an enterostomy before transanal endorectal pull-through procedure (TERPT). In the AChE group, 4 patients (23.5%) required repeat bowel sampling to confirm the diagnosis. Compared to the AChE group, more children in the CR group suffered from constipation post TERPT. Elevated AChE expression is linked to hypertrophied extrinsic cholinergic nerve fibers in the aganglionic segment in the majority of patients with HD. The manifestation of increased AChE expression develops over time. Therefore, in neonatal patients with HD, especially those in the first 3 weeks of life, an increase in AChE reaction is not detected. Calretinin IHC reliably identifies the presence or absence of ganglion cells and offers multiple benefits over AChE histochemistry. These include the ability to perform the test on paraffin-embedded tissue sections, a straightforward staining pattern, a clear binary interpretation (negative or positive), cost-effectiveness, and utility regardless of patient age. The ability of calretinin IHC to diagnose HD early and time-independently prevented repeated intestinal biopsies in our patient population and allowed us to perform a one-stage TERPT in the first months of life, reducing the number of enterostomies and restoring colonic continuity early. Patients undergoing transanal pull-through under the age of 3 months require a close follow-up to detect cases with bowel movement problems.

Comparison of Long-term Outcomes between Hirschsprung's Disease Patients after Soave Procedure and Duhamel Procedure Based on the Paediatric Incontinence and Constipation Scoring System: A Retrospective Cohort Study

Introduction: Hirschprung's disease is a congenital disease that causes intestinal motility disorders. Hirschprung's disease is still the most common condition that causes intestinal obstruction in children. The incidence of Hirschprung's disease is 1:5000 live births. The incidence ratio of this disease is around 4:1 between men and women, with a higher percentage of men 1,3,4. Approximately 75% of cases of Hirschprung's disease occur in the rectosigmoid colon area, 17% in the splenic flexure or transverse colon and 8% along the colon and terminal ileum. The Duhamel technique still remains a popular procedure most surgeons especially for long segment treatment and redo procedures.28, 30 The advantage of the Duhamel Procedure is that it is easy to perform, reduces the risk of damage to surrounding structures such as genitourinary innervation, but has the possibility of complications of anastomotic leakage which causes fistulas and abscesses.&#x0D; Materials and Methods: Total subjects of this study was 441 patients with Hirschprung's disease who were treated with the Soave procedure and the Duhamel procedure at Dr. Moewardi General Hospital in Surakarta, Central Java, Indonesia for the period January 2017 - January 2022. This type of research is descriptive analytics with a retrospective cohort.&#x0D; Results: The results of the study can be concluded that patients with HAEC increases the risk of constipation. Congenital abnormalities predispose to fecal incontinence. Differences in surgical techniques do not significantly influence or increase the risk of constipation and fecal incontinence based on statistical tests. However, the increased risk of constipation and fecal incontinence is higher in patients undergoing the Duhamel surgical procedure.&#x0D; Conclusion: Conclusions that can be drawn from research regarding the comparison of long-term outcomes of Hirschsprung disease patients after Soave and Duhamel operations based on the Pediatric Incontinence and Constipation Scoring System at Dr. Moewardi General Hospital in Surakarta, Indonesia for period January 2017 – January 2022, it can be concluded that there is no significant difference in the outcome of patients who underwent the Soave or Duhamel procedures. The Duhamel procedure has a higher prevalence of complications of constipation and fecal incontinence compared to the Soave procedure. The presence of HAEC before the surgical procedure increases the risk of constipation, significantly in the Soave and Duhamel procedures.

Safety and Efficacy of Hook Diathermy in the Dissection of the Mesocolorectum During Laparoscopic-Assisted Pull-Through for Hirschsprung Disease in Low-Resource Settings.

Background: Surgical correction is the main line of treatment for the congenital disorder Hirschsprung's disease (HD). Laparoscopic techniques proved their safety and efficacy in previous studies. LigaSure™ is the gold standard for colorectal dissection. However, other sealing methods could be used during the unavailability of LigaSure. Purpose: This study aimed to assess the safety profile of the hook diathermy technique compared to LigaSure in colorectal dissection during laparoscopic-assisted pull-through for HD. Materials and Methods: This case-control study was held in the pediatric surgery department at Assiut University Hospitals between September 2017 and January 2023. The study included 57 HD patients who were surgically operated on during a laparoscopic-assisted pull-through. They were divided into 2 groups according to the sealing methods: the ligasure group included 25 patients, and the hook diathermy group included 32 patients. Results: Both groups had no statistically significant differences regarding age, sex, or weight. The transition zone was present in all patients, and most transition zones were rectosigmoid. No cases reported intraoperative blood transfusion or conversion to an open or transanal approach. Minimum intraoperative blood loss was reported in both groups, with no significant differences. As regards postoperative complications, including bleeding, leakage, perianal excoriation, and enterocolitis, no significant differences between both groups were found. Conclusion: The LigaSure and hook diathermy techniques are safe and effective sealing methods for colorectal dissection during laparoscopic-assisted pull-through for HD.

Hirschsprung disease: a cost analysis study of the direct, indirect costs and financial coping strategies for the surgical management in Western Uganda.

Surgical management of Hirschsprung disease (HD) in low- and middle-income countries is typically a staged procedure, necessitating multiple hospitalizations and clinic visits increasing family financial burden. Currently, there is limited information on the costs borne by caretakers of children with Hirschsprung disease seeking surgical intervention. This study seeks to measure the costs and economic burden of surgical treatment for Hirschsprung disease in western Uganda. A cross-sectional study using cost analysis was conducted among caretakers of patients who completed surgical treatment of HD between January 2017 and December 2021 at two hospitals in western Uganda. The average direct and indirect costs incurred by caretakers presenting at a public and private hospital were computed. A total of 69 patients (M: F = 7:1) were enrolled in the study. The median age at diagnosis was 60.5 (IQR 3-151.25) days for children and two-staged pull-through procedure was the common surgery performed. The mean overall cost for treatment was US $960 (SD = $720), with the majority of costs coming from direct medical costs. Nearly half (48%) of participants resorted to distress financing to finance their child's surgical care. The overwhelming majority of patients (n = 64, 93%) incurred catastrophic expenditure from the total costs of surgery for HD, and 97% of participants fell below the international poverty line at the time treatment was completed. Despite the availability of 'free care' from government hospital and non-profit services, this study found that surgical management of Hirschsprung disease imposed substantial cost burden on families with Hirschsprung disease patients.

Investigation of The Histopathological and Histometric Changes in Rectum Tissue Biopsies of Hirschsprung and Non-Hirschsprung Disease in Neonate and Infant

Hirschsprung disease is one of the most common and problematic infancy and childhood maladies. The most reliable method for diagnosis is the histopathological analysis of colorectal biopsies and the typical finding of Hirschsprung disease is the absence of ganglion cells. The study involved 36 cases of suspected Hirschsprung disease, in which 27 cases were males and 9 cases were females with an age range between (1 day - 1 year). All patients undergo complete history taking, physical examination, radiological investigations, and rectal biopsy. The tissue specimens were obtained from the rectum of neonate and infant patients, maintained in the fixative solution (formalin 10%) for histopathological analysis, and patients were divided into two groups (Hirschsprung disease and non-Hirschsprung disease group) according to histological findings. Statistical analysis was performed on the tabulated data by chi-square, and the automated computer-adopted image analysis program Image J® was utilized for the histometrical examination of rectum tissue. The Hirschsprung disease is more common in males than females. The delayed passage of meconium is the most common symptom of these diseases. We notice that there is a significant difference between the Hirschsprung disease group and the non-Hirschsprung disease group in clinical signs, empty rectum, tight anal sphincter on per rectal examination. Based on histopathology analysis of the rectal biopsy, out of the 36 cases, 27(75%) cases were diagnosed as Hirschsprung disease, and 9 (25%) cases as negative for Hirschsprung disease. In Histometric study of rectal biopsies tissues showed a significant difference in the mucosa, Submucosa, and muscularis externa between Hirschsprung disease and non-Hirschsprung disease patients.

Histological transitional zone pull-through in Hirschsprung disease. Postoperative functional results and current recommendations.

Surgeons create a neorectum to repair patients with Hirschsprung's disease (HD), which should be formed from a normoganglionic bowel. However, the neorectum is occasionally created with a transition zone (TZ) bowel. A neorectum created with a TZ has been postulated as a cause of postoperative enterocolitis or constipation. This study compares the incidence of enterocolitis and constipation in patients with TZ neorectum and normoganglionic bowel. We conducted a retrospective review of patients with rectosigmoid HD who underwent primary pull-through. Patients were divided into normoganglionic neorectum (NNR) and TZ neorectum. The diagnosis was based on the final histopathologic report of the proximal margin. The incidence of enterocolitis and constipation was compared between these two groups. A total of 98 HD patients were analyzed. Seventy-one patients fulfilled the inclusion criteria. 65 (92%) had a NNR, and six patients (8%) had a TZ neorectum. From these patients, 42 (59%) presented with enterocolitis or constipation. However, there was no significant difference between both groups. The present study showed no difference in the incidence of enterocolitis or postoperative constipation in HD patients with normoganglionic or TZ neorectum. These results suggest that TZ neorectum does not cause postoperative obstructive symptoms.