Treatment Of Hirschsprung's Disease Research Articles

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.

Chinese expert consensus statement on the diagnosis and treatment of Hirschsprung disease.

Chinese expert consensus statement on the diagnosis and treatment of Hirschsprung disease.

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.

Post-surgical Outcomes of Different Surgical Techniques in Hirschsprung's Disease: A Literature Review.

Hirschsprung's disease (HD) is a rare condition that affects newborns and is characterized by the lack of ganglion cells in the colon. Typical symptoms include difficulty passing stool, vomiting, and trouble feeding. Various surgical methods are available to manage the condition. The aim of the study is to investigate and compare the post-surgical outcomes of different surgical techniques used in the treatment of HD. A thorough literature search was conducted using various electronic databases to identify relevant studies to be referred to.Double-blinded screening of the identified articles led to the final selection of 40 out of 440 HD, including transanal endorectal pull-through (TERPT), laparoscopic approaches, and modified techniques.Several studies have investigated surgical procedures for HD, including TERPT, laparoscopic methods, and modified techniques. These have shown positive outcomes, with fewer complications, improved bowel function, and favorable cosmetic results. Individual patient characteristics and surgeon expertise should guide procedure selection.Surgery for HD aims to restore normal bowel function, but post-surgical outcomes can include constipation or fecal incontinence. Complications like enterocolitis, anastomotic stricture, and sphincter damage may occur. Laparoscopic approaches have shorter hospital stays. However long-term follow-up is essential to assess quality of life, psychological well-being, and potential side effects.

Our Experience of the De la Torre-Mondragon Technique in the Surgical Management of Hirschsprung's Disease: A Report on 52 Cases.

The authors report their experience of the transanal endorectal pull through technique as described by De la Torre Mondragon in the treatment of Hirschsprung's disease (HD). It was a retro prospective, observational and descriptive hospital based study involving all proven cases of HD managed within a time frame of 8 years. Fifty two patients with a mean age of 18 months at the time of surgery. The male pre dominance was remarkable. The mean duration of the surgery was 2 h and a half hours. The surgical indication was based on the history, clinical findings and on the contrast enema (transition zone) with a 24 h film (prolonged contrast evacuation) and calculation of the rectosigmoid index (<1). With a mean follow up of 16 months, the morbidity was dominated by soiling, anastomotic strictures and enterocolitis. The mortality in one case was related to a post operative enterocolitis that was not amenable to resuscitation. Soiling, anastomotic strictures and enterocolitis are the main post operative complications of TEPT in the treatment of HD in our practice.

Outcome of Transanal Endorectal Pull-Through in Patients with Hirschsprung's Disease.

Transanal endorectal pull-through (TERPT) has become one of the preferred treatments for Hirschsprung's disease (HD) in our setting. This report aims to evaluate the current outcome of TERPT in the setting. A retrospective review of 71 children who had TERPT for histologically-confirmed HD in 11 years (2006-2017) in Nigeria. There were 48 boys and 23 girls; aged 3 days-12 years at initial presentation (median = 10 months). Three (4.2%) patients had associated anomalies (duodenal atresia; anorectal malformation and sensorineural deafness with hypopigmented skin patches each). Age at TERPT was 2 months to 15 years (median = 3 years), with surgery waiting time of 1 month-14.9 years (median = 18 months). Sixty-six (93.0%) patients had rectosigmoid, four (5.6%) patients had long segment and one (1.4%) had total colonic disease. Five (7.0%) patients with large megacolon and one (1.4%) with the total colonic disease had assisted abdominal resection of the colon at TERPT. Seventeen (23.9%) patients had post-operative complications, including post-operative enterocolitis 8 (11.3%); anastomotic dehiscence 3 (4.2%); retained aganglionic segment 2 (2.8%); anastomotic stenosis 2 (2.8%), resulting in prolonged hospital stay (P = 0.0001; range = 1-30 days; median = 5 days). The mortality rate was 4.2% (3) from malignant hyperthermia in one patient and in 2 patients, the cause of mortality was unclear. Patients were followed up for 3-6 years (median = 3.5 years). Bowel movement stabilised to 2-4 times daily by 6 weeks after surgery. TERPT is a safe treatment for HD in this setting with good short-term outcomes. Longer follow-up is necessary to further evaluate the long-term bowel movement outcomes.!

Clinical and manometric evaluation of postoperative anorectal function in patients after trans-anal pull-through for Hirschsprung disease

BackgroundTransanal endorectal pull-through (TEPT) surgery is a new trend in the treatment of Hirschsprung disease, and evaluating its functional outcome is difficult. The purpose of this study is to evaluate the defecation pattern after TEPT surgery in pediatrics.Material and methodsIn this cross-sectional survey, 40 patients with Hirschsprung disease were studied. They underwent a one-stage transanal pull-through procedure from May 2007 till April 2015 in Namazi hospital by the same surgeon. All the patients had the aganglionic segment in the rectosigmoid, confirmed by pre-operation barium enema and post-operation histopathology. The patients were 40 children with mean operation age of 36.6 months old. The success rate of surgery was evaluated by following the questionnaire form. Clinical evaluation with bowel function score and anorectomanometry were carried out.ResultOne hundred percent of them had a daily stool. Eighty-five percent of patients never experienced pain during defecation, 10% experienced occasional pain, and the rest felt pain with every defecation. Just one case (5%) reported using a laxative. Regarding improvements after the operation, 75% were completely okay, 20% felt much better, and 5% reported some improvements. The average National Health Service score was 8.375, which seems satisfactory. The mean average anal sphincter in the group with soiling was 39.67 and in the group without soiling was 34.83, which is in the normal range in both groups, and there was no significant difference between these groups (P > 0.05).ConclusionThe defecation pattern after TEPT surgery were satisfactory in almost all cases, even in infancy. Most patients had satisfactory manometry and clinical result.

Management of Extreme Prematurity (Manuscript for Seminars in Pediatric Surgery)

Harald Hirschsprung presented the first comprehensive and detailed description of clinical outcomes in patients with congenital megacolon. In the century following his discovery, we have seen realization of the etiology, diagnosis, and treatment of Hirschsprung disease. In this article we describe the surgical management of Hirschsprung disease starting with Orvar Swenson, who pioneered the field with the first full thickness transrectal dissection followed by several additional innovative surgeons who have contributed to the modifications that have brought us to the modern surgical management of Hirschsprung Disease.

Surgical history of Hirschsprung disease

Harald Hirschsprung presented the first comprehensive and detailed description of clinical outcomes in patients with congenital megacolon. In the century following his discovery, we have seen realization of the etiology, diagnosis, and treatment of Hirschsprung disease. In this article we describe the surgical management of Hirschsprung disease starting with Orvar Swenson, who pioneered the field with the first full thickness transrectal dissection followed by several additional innovative surgeons who have contributed to the modifications that have brought us to the modern surgical management of Hirschsprung Disease.

Clinical Evaluation of Laparoscopic Surgery for Hirschsprung Disease Combined with Colorectal Anastomosis with a Stapling Technique in Infants.

Aim: To evaluate the effect of laparoscopic-assisted resection and colorectal anastomosis with a stapling technique in the treatment of Hirschsprung disease (HSCR) in infants. Methods: From June 2018 to January 2019, 25 cases of HSCR diagnosed by clinical examination and pathology were selected at the Children's Hospital, School of Medicine, Zhejiang University, China. All children were treated with standard laparoscopic-assisted transanal endorectal pull-through surgery (the modified Swenson technique). The short segment type and the typical type with a descending colon-rectum anastomosis were both included. The long segment type had an ascending colon-rectal anastomosis after ascending colon turnover. The colorectal anastomosis was divided into traditional manual anastomosis and straight intraluminal stapler (SIS) anastomosis. According to the different methods of anastomosis, the patients were divided into a traditional group and a SIS group. Age, gender, body weight, operation time, blood loss, postoperative anal exhaust and defecation time, postoperative hospitalization time, and postoperative short-term complications were analyzed retrospectively. Results: A total of 25 children were diagnosed with HSCR. There were 17 boys and 8 girls, and their average age was 10.20 months (interquartile range, 8.60-11.30). Their average body weight was 7.90 kg (interquartile range, 7.50-8.40). There were 17 cases of the typical type, 5 cases of the short segment type, and 3 cases of the long segment type. The different colorectal anastomosis methods were divided into 10 cases in the traditional group and 15 cases in the SIS group. There were no intraoperative complications, wound infections, or anastomotic fistula. Compared with the SIS group, children in the traditional group had an increased operative time (129.5 versus 103.00 minutes; P < .0001), increased intraoperative blood loss (20.00 versus 7.00 mL; P < .0001), increased postoperative hospitalization time (12.00 versus 9.00 days; P = .0003), and increased postoperative defecation time (18.40 versus 13.20 hours; P < .0001). After 6-12 months of follow-up, there was no anastomotic stenosis or enterocolitis in the SIS group. In the traditional group, 1 child had anastomotic stenosis, which improved 6 months after anal dilatation. One case of enterocolitis occurred 4 months after the operation and was cured after enema and infusion. Conclusion: Laparoscopic-assisted resection combined with colorectal anastomosis with the stapling technique in the treatment of HSCR in infants is feasible. It had a short operation time, less bleeding, less trauma, and a rapid recovery of postoperative intestinal function. The anastomosis was smooth, wide, and reliable, and anastomotic fistula and stenosis did not occur.

Clinical Efficacy of an Indwelling Transanal Tube for the Prevention of Anastomotic Leakage After Hirschsprung's Disease: A Single Center Experience with Chinese Patients.

Background: The aim of this study was to investigate the clinical effectiveness of an indwelling transanal tube for the prevention of anastomotic leakage (AL) after a radical operation for Hirschsprung's disease (HD). Methods: We retrospectively analyzed the clinical data from 158 patients who had undergone laparoscopic-assisted Soave procedures for HD at our hospital from May 2015 to May 2019. Patients were divided into two groups depending upon whether the anal drainage tube was retained or not retained: an indwelling group (group A, n = 86) and a no-indwelling group (group B, n = 72). Results: All 158 children had a successful operation by a laparoscopic technique. There was no significant difference in the duration of the operation, the length of the incision, the amount of bleeding, or the postoperative hospitalization time between the two groups. Compared with the no-indwelling group, maintaining the transanal tube had significant advantages for preventing incidences of AL (P < .05). The 4-year follow-up showed that the incidence of postoperative enterocolitis with the indwelling transanal tube was significantly lower than in the group without the drainage tube (P < .05). Conclusions: The laparoscopic-assisted Soave procedure with an indwelling transanal tube is a safe and feasible method for the treatment of HD in children. This method can not only drain intestinal contents but also reduce the occurrence of AL.

Endoscopic Decompression with a Transanal Drainage Tube for the Treatment of Hirschsprung's Disease in Newborns

Background: To evaluate the effectiveness and safety of endoscopic decompression with a transanal drainage tube (ED-TDT) in the treatment of neonatal Hirschsprung's disease (HD). Materials and Methods: Six newborns (4 boys and 2 girls; age at operation 9-29 days, median 24 days) were admitted to our hospital due to the delayed passage of meconium after birth, abdominal distension, and vomiting. HD was diagnosed by the retrograde enema radiography. All patients underwent ED-TDT for decompression of the dilated colon. During the operation, an endoscope was inserted through the anus to the ascending colon or ileocecal region, which was then used to insert a guidewire to the ascending colon. The endoscope was then removed, and a colorectal drainage tube was introduced over the guidewire. This drainage was placed until a pull-through procedure was performed. Results: The ED-TDT was successfully performed in all newborns. The intraoperative rectal biopsy confirmed the diagnosis of HD. The duration of the operation was 25-70 minutes (median 52.5 minutes). There were no perforations, bleeding, or other complications after surgery. After surgery, the abdominal distention was significantly relieved, and the babies could be fed with milk after 6 hours. These babies were discharged after 2-11 days (median 2.5 days) and underwent a pull-through procedure after 1-3 months (median 3 months). Conclusions: ED-TDT is a safe and effective treatment for neonatal HD before a pull-through procedure. It could be used as an alternative therapy to colostomy.

Особенности диагностики и тактики хирургического лечения болезни Гиршпрунга у грудных детей

The aim is to present optimal ways of early diagnosis and tactics of Hirschsprung's disease (HD) surgical correction in newborns and young children based on literature data and our own experience. Materials and methods. Clinical observations on early diagnosis and optimization of the tactics of surgical treatment of HD in 58 infants over the past 9 years have been carried out. All patients underwent a complex of general physical examinations, including the collection of anamnesis and follow-up of patients, clinical examination with rectal examination, laboratory, bacteriological, radiological (survey, polypositional irrigography) and morphological (intraoperatively remote area of the colon agangliosis zone) studies. The features of the clinical course and tactics of surgical treatment of HD in infants, the choice of the surgical method were analyzed, and the risk of complications during the operation was studied. The patients were divided into two groups. The first group included 32 (55.2%) infants with a typical clinical course of HD, radiographically confirmed agangliosis in the rectosigmoid zone, and chronic constipation. The second group included 26 (44.8%) infants with an atypical clinical course of HD with diarrheal syndrome against the background of dysbiosis, enterocolitis, among them there were 2 children with symptoms of Hirschsprung-associated enterocolitis. Research methods: General clinical and biochemical analysis of blood and urine. Scatological examination of feces, sowing on the pathogenic flora of feces and urine. EKG. Ultrasound examination of the abdominal cavity and echocardiography. X-ray examination: an overview roentgenogram of the abdominal and thoracic cavity vertically, irrigoraphy, excretory urography. Results and conclusions. In newborns with sub- and decompensated typical course of chronic constipation in the absence of the effect of conservative therapy, after the diagnosis of HD is established, an early radical operation or colostomy application above the agangliosis zone is recommended. In infants with a favorable typical clinical course and a left-sided form of HD, transanal endorectal reduction of the large intestine (TENTC) can be performed according to indications, starting from one month of age. In this case, the operation of choice is TENTK without colostomy. In subtotal and rectosigmoidal forms of HD with a long aganglionic zone, laparoscopic assistance is recommended, which makes it possible to release the splenic flexure of the colon from ligaments and constrictions, take a full-thickness biopsy of the transitional zone of aganglionicosis, and this makes it possible to conduct express diagnostics, establish resection levels and freely lower the proximal area colon during TENTC. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of all participating institution. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: Hirschsprung’s disease, clinic, infants, trans anal colon reduction.

Diagnosing Hirschsprung disease by detecting intestinal ganglion cells using label-free hyperspectral microscopy

Hirschsprung disease (HD) is a congenital disorder in the distal colon that is characterized by the absence of nerve ganglion cells in the diseased tissue. The primary treatment for HD is surgical intervention with resection of the aganglionic bowel. The accurate identification of the aganglionic segment depends on the histologic evaluation of multiple biopsies to determine the absence of ganglion cells in the tissue, which can be a time-consuming procedure. We investigate the feasibility of using a combination of label-free optical modalities, second harmonic generation (SHG); two-photon excitation autofluorescence (2PAF); and Raman spectroscopy (RS), to accurately locate and identify ganglion cells in murine intestinal tissue without the use of exogenous labels or dyes. We show that the image contrast provided by SHG and 2PAF signals allows for the visualization of the overall tissue morphology and localization of regions that may contain ganglion cells, while RS provides detailed multiplexed molecular information that can be used to accurately identify specific ganglion cells. Support vector machine, principal component analysis and linear discriminant analysis classification models were applied to the hyperspectral Raman data and showed that ganglion cells can be identified with a classification accuracy higher than 95%. Our findings suggest that a near real-time intraoperative histology method can be developed using these three optical modalities together that can aid pathologists and surgeons in rapid, accurate identification of ganglion cells to guide surgical decisions with minimal human intervention.

Long-term Follow-up of Patients with Hirschsprung Disease: Unusual Late Complications After Pull-through

Background: A large number of patients who underwent pull-through surgery due to Hirschsprung's disease (HD) were not followed up properly and they suffered from many complications such as fecal soiling, constipation, and etc. Although some of these complications may relief over time, it is rational to consider an evaluative protocol to identify anatomical or pathological complications in these patients. Objective: The aim of the present study is to evaluate and introduce these catastrophic complications. Method: In this historical cohort study, 193 patients with HD who had undergone pull- through surgery between 2006 to 2013 were considered. All files and questionnaires were used to collect patients' information and patients were physically examined individually. The mean duration of the follow-up was 96.4±17.2 months ranging from 60 to 144 months The follow-up performed for all patients via the outpatient clinics or by telephone. All statistical information was analyzed by SPSS software version 17. Results: Results: At first presentation, intestinal obstruction was more frequent than constipation. The most common involved segment was in rectosigmoid. The most common early postoperative complication was stricture in anal canal and the most common late complications were constipation and focal soiling. Conclusion: Although surgical advancements, a large number of patients underwent surgical procedures due to HD experience long term complications. Surgical treatment of HD generally present with high-quality outcome and the majority of children survive in satisfactory situation for long time but occasionally pediatric surgeons meet head-on catastrophic complication in these patients that require extended team work to be resolved

Pitfalls in diagnosis and treatment of Hirschsprung's disease

Abstract Background and purpose Hirschsprung's disease (HD) presents itself by delayed passage of meconium, neonatal bowel obstruction and/or chronic constipation. In 80% of cases the disease is diagnosed in the first year of life. Difficulties or misdiagnosing HD are infrequently met. The aim of this case series is to highlight the pitfalls in diagnosis and treatment of HD. Case presentation: case 1&2 A 4 and 6 months old ♂ were diagnosed as rectal perforation in the neonatal period and colostomy was performed. At the age of 4 and 6 months respectively they developed fecal fistula after colostomy closure. Revision of stoma was done and biopsy proved that both had aganglionic rectal biopsy. Abdominal PT was performed for both after 3 and 4 months. Case 3 A 5 years ♀ came with recurrent attacks of intestinal obstruction since the age of 1 year. Both plain X ray and contrast studies were inconclusive. Finally was diagnosed after a second exploration and histopathological biopsies with total colonic aganglionosis. Case 4 A 1.5 years old presented with neonatal intestinal obstruction. His preoperative gastrografen enema were inconclusive. He underwent surgical exploration at the age of 2 weeks ended with right transverse colostomy and multiple colonic and rectal biopsies proved to be aganglionic. Case 5 A 3 months old ♂ came with caecostomy construction because of caecal perforation during neonatal period. Although there was no histopathological report earlier about the colon but his current rectal biopsy was devoid of ganglion cells. Conclusions:High degree of suspicion with physician care plan and routine biopsy are essential in all non NEC neonatal perforation during exploration to avoid missing of HD.

Transumbilical enterostomy for Hirschsprung's disease with a two-stage laparoscopy-assisted pull-through procedure.

BACKGROUNDA one-stage laparoscopic operation has recently been considered a favorable option for the management of patients with Hirschsprung's disease (HD) due to its superior cosmetic results. One-stage transanal endorectal pull-through for the treatment of rectosigmoid HD has been widely used in newborns without complications. However, enterostomy is required in some HD cases for enterocolitis and dilated colon. Our transumbilical enterostomy (TUE) and two-stage laparoscopy-assisted anorectoplasty were effective and achieved a similar cosmetic effect to one-stage laparoscopy on the abdominal wall in patients with anorectal malformation, but the effect in patients with HD is unclear.AIMTo evaluate the safety, efficacy and cosmetic results of TUE in two-stage laparoscopy-assisted pull-through for HD.METHODSFrom June 2013 to June 2018, 53 patients (40 boys, 13 girls; mean age at enterostomy: 5.5 ± 2.2 mo) who underwent enterostomy and two-stage laparoscopy-assisted pull-through for HD with stoma closure were reviewed at our institution. Two enterostomy approaches were used: TUE in 24 patients, and conventional abdominal enterostomy (CAE) in 29 patients. Eleven patients with rectosigmoid HD had severe preoperative enterocolitis or a dilated colon. 26 patients had long-segment HD, and 16 patients had total colonic aganglionosis (TCA). The patients with left-sided HD underwent the two-stage laparoscopic Soave procedure, and the patients with right-sided HD and TCA underwent the laparoscopic Duhamel procedure. Demographics, enterostomy operative time, complications and cosmetic results were respectively evaluated.RESULTSThere were no differences between the groups with respect to gender, age at enterostomy, weight and clinical type (P > 0.05). No conversion to open technique was required. Two patients experienced episodes of stomal mucosal prolapse in the TUE group and 1 patient in the CAE group (8.33% vs 3.45%, P > 0.05). No parastomal hernia was observed in either of the two groups. Wound infection at the stoma was seen in 1 case in the TUE group, and 2 cases in the CAE group (4.17% vs 6.90%, P > 0.05). No obstruction was noted in any of the patients in the TUE group, whereas obstruction was found in 1 patient in the CAE group. Enterocolitis was observed in 3 and 5 patients in the TUE and CAE group, respectively (12.50% vs 17.24%, P > 0.05). There was no significant difference between the TUE group and CAE group in terms of the incidence of soiling and constipation (P > 0.05). The cosmetic result using the scar score in the TUE group was better than that in the CAE group (6.83 ± 0.96 vs 13.32 ± 1.57, P < 0.05).CONCLUSIONTUE is a safe and feasible method for the treatment of HD, and the staged enterostomy and two-stage laparoscopy-assisted pull-through achieved a similar cosmetic effect to the one-stage laparoscopic procedure.

Diagnosis and surgical approach of adult Hirschsprung's disease: About two observations and review of the literature. Case series.

IntroductionHirschsprung's disease (HD) is uncommon in adulthood. In this study, we describe the management of two cases of adult Hirschsprung's disease treated with transanal colonic pull-through procedure followed by a delayed coloanal anastomosis.Patients and methodsThis was a retrospective (December 2016 to Jun 2019) study included two cases of adult HD with confirmed Hirschsprung disease who underwent surgery at Zinder National hospital, Niger. The registration number is researchregistry 5174.ResultsThese were two patients aged 21 years (male) and 22 years (female) admitted to the emergency department with an acute bowel obstruction. The history finds a delayed passage of meconium at birth with a history of long-standing recurrent constipation since early childhood for the 2 patients. A lateral colostomy was performed urgently in both patients and the barium enema revealed a disparity of the sigmoid colon with corn shaped transition zone. Histologic examination of the biopsy specimen confirmed the diagnosis of HD. Surgery was done according to transanal endorectal pull-through procedure followed by delayed coloanal anastomosis. Patients were regularly followed over a period of 16 months. Constipation was gone, no continence problem was reported and quality of life was rated satisfactory.ConclusionThe discovery of Hirschsprung's disease is rare in adulthood. Transanal endorectal pull-through procedure followed by delayed coloanal anastomosis with conventional surgery is a suitable option for the treatment of HD and gives a good result.

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

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. 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). 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. We propose that NF expression in dysmorphic ganglion cells at the PRM may predict poor outcome after pull-through surgery for HD.

CHANGES IN MICROBIOTA AND PECULIARITIES OF THE USE OF PROBIOTICS FOR THE PREVENTION OF COMPLICATIONS OF THE SURGICAL TREATMENT OF HIRSCHSPRUNG DISEASE IN CHILDREN

In a review there are presented current data both on the formation of Hirschsprung disease (HD) in children and complications of its surgical treatment. The importance of interrelations of intestinal microbiota, agangliosis of the large intestine and the mechanisms of formation of HD-associated enterocolitis as a leading complication in the postoperative period are described. The value of the intestinal microbiota in its interaction with the immune system as a factor of pathogenesis and prevention of postoperative complications in HD patients is determined. Changes in the intestinal microbiota were shown to play a significant pathogenetic role in the appearance and progression of HD-associated enterocolitis in children. It is indicated that early manipulations with microbial intestinal communities through the use of probiotics can become the basis of a new strategy for the prevention of postoperative complications in HD. Modulation of the intestinal microbiota with antibiotics and probiotics can be a promising method of the management and prevention of complications of surgical treatment of HD in children.