Total Colonic Aganglionosis Research Articles

Influence of Diet on Bowel Function and Abdominal Symptoms in Children and Adolescents with Hirschsprung Disease-A Multinational Patient-Reported Outcome Survey.

Introduction: This study aimed to understand the influence of diet and nutrition items on gastrointestinal symptoms in patients with Hirschsprung Disease (HD). Method: An online questionnaire was created to obtain patient-reported outcomes using the multinational Holistic Care in Hirschsprung Disease Network. This was distributed in Dutch, English, German, Italian, Polish, and Swedish via patient associations. Information on demographics, the extension of disease, current diet, and the influence of food ingredients on bowel function were obtained. Results: In total, 563 questionnaires were answered by parents or patients themselves. The length of the aganglionic segment was short in 33%, long in 45%, total colonic aganglionosis (TCA) in 11%, and involved the small intestine in 10%. Overall, 90% reported following a mixed diet, and 31% reported taking probiotics, with twice as many patients taking probiotics in the TCA group compared to standard HD. Mealtimes and behaviours around eating were affected by 61%, while 77% had established food items that worsened symptoms, and of these, 80% stated that they had worked these items out themselves. A high-fibre diet was followed by 24% and 18% a low-fibre diet. Symptoms were reported, particularly from dairy in 30%, fruits in 39%, pulses in 54%, and sugar in 48%. Conclusions: This first multinational survey on diet and bowel function in HD reports an association between certain dietary items with gastrointestinal symptoms. This study can support an improved understanding of the interaction between food items and bowel function in children with HD. We suggest a multidisciplinary approach to balance dietary exclusions and support adequate growth, preventing nutrition deficiencies and enhancing quality of life.

Surgical Strategies in Total Colonic Aganglionosis: Primary Pullthrough-Pathway of Care.

Background: Total colonic aganglionosis, as a rare variant of Hirschsprung Disease, still poses challenges to surgeons in terms of diagnosis and management. The optimal preparation for pullthrough is crucial for reconstructive surgery. This study aims to explore our surgical pathway for children with total colonic aganglionosis (TCA) and to describe the prehabilitation necessary to prepare for successful reconstructive pullthrough surgery. Methods: A prospective review of children with TCA receiving an abdominal surgical intervention between 1/22 and 4/24. The cohort included children receiving mapping +/- primary ileoanal pullthrough. An analysis of preoperative, perioperative, and postoperative data, and a short-term follow-up were performed. Results: A total of 18 children with TCA and no prior pullthrough received an abdominal intervention during the 29-month study period, and 5/18 (27.8%) were female. The children had a median of 4 (range 2-7) prior external surgeries; all had a stoma; 6 (33%) children received parental nutrition; 12 children underwent a mapping of the ganglia distribution and bowel length at a median age of 11 months (range 3-54), and in 10 of them, we relocated the stoma. There was a mean involvement of 15 (5-93) cm small bowel aganglionosis, with the remaining mean ganglionic small bowel having a length of 178 cm (110-254). A total of 11 children underwent straight primary ileoanal pullthrough of the stoma site at a median age of 16.7 months (10-133). Conclusions: The timely diagnosis of TCA still challenges the care team, and most children have a rough journey involving several surgeries until their diagnosis is established. The ensure bowel function with an adequate working stoma is the key to enabling enteral nutrition and growth, which are the baseline requirements to undertake a successful pullthrough procedure and restore continuity. Careful perioperative bowel management and parents' active involvement supports children with Hirschsprung Disease achieving the best possible quality of life.

Technical evaluation and fecal continence of laparoscopic-assisted procedures for total colonic aganglionosis

Total colonic aganglionosis (TCA), defined as aganglionosis extending from the anus up to the distal ileum, is a rare variant of Hirschsprung's disease. Surgery for TCA aims to remove the aganglionic colon and reconstruct the intestinal tract by bringing the normally innervated ileum down to the anus while preserving normal sphincter function. Laparoscopy with surgeries including laparoscopic-assisted Duhamel-like and Soave-like pull-through procedures have been progressively applied to the treatment of TCA over the past two decades and may become the dominant treatment for TCA in the future. Postoperative fecal continence, involving issues such as normal defecation, Hirschsprung-associated enterocolitis (HAEC), frequent stool and perianal erosion (perineal rash), soiling/fecal incontinence and constipation/obstructive symptoms, is very important to patients. These complications require nutritional support and comprehensive postoperative gastroenterological care and even require permanent ileostomy and redo pull-through. Some patients have previously seen a psychologist for frequent complaints of diarrhea and other physical symptoms. Patients with TCA postoperatively must be evaluated regularly by pediatric surgeons, enterostoma therapists, gastroenterologists, dieticians and psychologists to achieve a good nutritional status and psychological health.

Are the complications after laparo-assisted endo-rectal pull-through for Hirschsprung disease related to the change of the anal tone?

The main advantage of the laparo-assisted transanal endorectal pull-through technique (LA - TERPT) for Hirschsprung Disease (HD) is the respect to the rectal-anal anatomy. Postoperative complications have been observed recently. The present study aims to determine how often these postoperative complications occur in these patients. From January 2009 to December 2018, a retrospective analysis was conducted on 36 children (25 males) with HD who underwent LA-TERPT. Data were collected on the age of diagnosis and surgery, sex, the presence of other pathologies, and cases of enterocolitis. In all cases, anorectal manometry (ARM) was performed to evaluate the anal tone. The median age at diagnosis was 2 months and the mean age at surgery was 5 months. Nine related pathologies were identified: five cases of Down syndrome, one case of hypertrophic stenosis of the pylorus, atresia of the esophagus, polydactyly, and anorectal malformation. A patient with total colonic aganglionosis was identified through laparoscopic serummuscular biopsies. Enterocolitis was diagnosed in 7 cases before and 6 after surgery. At follow-up, the complications recorded were: 5 cases of constipation (treated with fecal softeners), one case of anal stenosis (patient with anorectal malformation), 16 cases of soiling (treated with enemas) and 1 child with fecal incontinence (treated with a transanal irrigation system). The ARM was performed in all 36 cases and showed normal anal tone, except for one case with anal hypotonia. LA-TERPT is an important surgical technique for HD. According to the literature, soiling is the most main complication after HD surgery, probably due to "pseudo-incontinence" with normal anal sphincter tone.

Outcomes following endorectal pull-through for Hirschsprung disease: a retrospective study

PurposeTo share our experience in the surgical management of patients with Hirschsprung disease (HD) using the trans-anal endorectal pull-through “Soave” technique.MethodsThe study included cases diagnosed as HD who were operated during the period 2008 through 2018. Cases of total colonic aganglionosis were excluded to be discussed in a separate report.ResultsThe study included 67 consecutive cases of HD. All included cases underwent trans-anal endorectal pull-through which was purely trans-anal in 31 (46%), while abdominal-assisted trans-anal endorectal pull-through was applied in the remaining (54%). Early postoperative complications included 2 cases of partial dehiscence at the colo-anal anastomosis; 1 of them deteriorated after re-exploration and unfortunately died. Twenty-six cases were available to assess functional outcomes after corrective surgery. Their age at follow-up ranged between 44 months and 17 years (mean = 78.8 months; median = 72 months). All cases achieved voluntary defecation. However, fecal soiling was a common finding after surgery for HD (69%). Constipation was another common postoperative finding (73%). Lastly, a history of attacks of postoperative enterocolitis was prevalent in 19 cases (73%), which ranged between 1 and 7 attacks usually in the first 2 years after the operation.ConclusionThe trans-anal endorectal pull-through provides an effective surgical treatment for Hirschsprung disease. The high prevalence of fecal soiling after surgery highlights the importance of long-term follow-up to provide the required support for these patients during adolescence and transition into adulthood.

Extended total colonic aganglionosis and total intestinal aganglionosis: Challenging enemies.

Extended 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. A 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). Normally 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 < 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%. Extended 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.

Ernica Clinical Consensus Statements on Total Colonic and Intestinal Aganglionosis

BackgroundHirschsprung disease is a congenital intestinal motility disorder characterized by an absence of enteric ganglion cells. Total colonic aganglionosis and near total or total intestinal aganglionosis, defined as absence of ganglion cells in the entire colon and with variable length of small bowel involved, are life-threatening conditions which affect less than 10 % of all patients with Hirschsprung disease. The aim of this project was to develop clinical consensus statements within ERNICA, the European Reference Network for rare congenital digestive diseases, on four major topics: Surgical treatment of total colonic aganglionosis, surgical treatment of total intestinal aganglionosis, management of poor bowel function in total colonic and/or intestinal aganglionosis and long-term management in total colonic and or intestinal aganglionosis. MethodsA multidisciplinary panel of representatives from ERNICA centers was invited to participate. Literature was searched, using specified search terms, in Medline (ALL), Embase and Google Scholar. Abstracts were screened and full text publications were selected. The panel was divided in four groups that extracted data from the full text publications and suggested draft statements for each of the major topics. A modified Delphi process was used to refine and agree on the statements. ResultsThe consensus statement was conducted by a multidisciplinary panel of 24 participants from 10 European countries, 45 statements reached consensus after 3 Delphi-rounds. The availability of high-quality clinical evidence was limited, and most statements were based on expert opinion. Another 25 statements did not reach consensus. ConclusionsTotal colonic and total intestinal aganglionosis are rare variants of Hirschsprung disease, with very limited availability of high-quality clinical evidence. This consensus statement provides statements on the surgical treatment, management of poor bowel function and long-term management for these rare patients. The expert panel agreed that patients benefit from multidisciplinary and personalized care, preferably in an expert center. Type of StudyClinical consensus statement. Level of Evidence3a.

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.

Клінічна симптоматика агангліонозу кишечника та його ускладнень у дітей віком від 3 до 6 років

Clinical symptoms of colonic aganglionosis (CA) in children 3 to 6 years of age are difficult due to its clinical presentation which represents as the complications of this pathology, because of it the classic signs of Hirschsprung’s disease (HD) are partially eliminated or hidden, which makes it difficult for the effective diagnosis of this pathology in these children. Purpose - to study the clinical symptoms of CA and its complications in children 3 to 6 years of age and to determine the significance of clinical symptoms for the verification of HD. Materials and methods. We have done retrospective analysis of the clinical manifestations in 240 children 3 to 6 years of age with HD, considering the extent of CA, the presence of associated malformations and complications. There were 95 (39.58%) patients with the rectal form of CA, 143 (59.59%) with the rectosigmoid form, 2 (0.83%) with the subtotal form. There were no patients with total CA in this age group. There were 192 boys (80.0%), and 48 girls (20.0%). At the age of 3-4 years 71 (29.58%) patients were diagnosed with HD, at the age of 4-5 years - 79 (32.91%), and at the age of 5-6 years - 90 (37.51%) patients. Results. The diagnosis of HD can be of course considered as late for patients from 3 to 6 years of age. We found associated malformations in 45 (18.75%) patients. Associated intestinal malformations were found in 26 (10.83%) patients, of which 19 (7.92%) had malformations that significantly increased the clinical symptoms of CA and its complications: malrotation (n=17 (7.08%)) and internal abdominal hernia (2 (0.83%)). Enterocolitis (EC) was found in 98 (40.83%) patients. The 1st degree of EC was established in 9 (3.75%) children, 2nd degree - in 34 (14.16%) and 3rd degree - in 55 (22.91%) patients. Among these 55 with 3rd degree of EC the critical moment in the progression of the course of EC was toxic megacolon (TM), which occurred in 14 (25.46%). Hypotrophy was established in 18 (7.49%) patients. Anemia of various degrees was diagnosed in 35 (14.58%) children. The acute stage of the course of HD was diagnosed in 98 (40.83%) patients, subacute - in 77 (32.08%) and chronic - in 65 (27.08%) children. The clinical course of HD was correlated with the extent of the colon affected with CA and is closely related to the presence of its complications: EC, anemia and hypotrophy. Conclusions. Clinical symptoms of CA in children aged 3 to 6 years remains typical and informative upon careful evaluation of complaints, medical history, and objective and laboratory results. The severity of the course, associated developmental malformations and late diagnosis are the main reasons for the appearance of severe complications of HD in children 3 to 6 years of age - EC (40.83%), TM (25.46%), hypotrophy (7.49%) and anemia (14.58%). Variants of complications in CA are characteristic and predictable. Comparison of the clinical signs of CA and its complications contribute to the correct assessment of the patient's condition and timely establishment of the correct diagnosis. The research was carried out in accordance with the principles of the Declaration of Helsinki. The research protocol was approved by the Local Ethics Committee of all institutions mentioned in the work. Informed consent of the children’s parents was obtained for the research. No conflict of interests was declared by the authors.

Long-Term Functional Outcomes and Multidisciplinary Management after Ileorectal Duhamel Pull-Through for Total Colonic Aganglionosis-20-Year Experience in a Tertiary Surgical Center.

Management of patients with total colonic aganglionosis (TCA) is challenging for pediatric surgeons. The purpose of this study was to review our institution's 20-year experience regarding long-term outcomes and multidisciplinary team management of these patients after an ileorectal Duhamel pull-through procedure. Retrospective review was conducted for all patients diagnosed with TCA in our tertiary institution. Data were collected on demographics, clinical presentation, complications, need for additional surgery, and long-term effects on bowel function. Of a total of 202 patients with Hirschsprung's disease, 13 were diagnosed with TCA (6.4%). Clinical presentation was variable. Eleven presented in neonatal period with distal bowel obstruction and two presented with constipation in early infancy. Ileorectal Duhamel pull-through was performed in all patients. Median follow-up was 13 years. Eleven are toilet trained, of whom five are fully continent. Six continue to have problems with bowel continence or constipation. One developed recurrent episode of Hirschsprung's associated enterocolitis. Two patients had stoma re-established. Patients experiencing difficulties in bowel function are jointly managed by a multidisciplinary team consisting of surgeons, gastroenterologists, pediatric psychologists, and clinical nurse specialists. TCA can be associated with significant long-term morbidity. Nearly half of the patients in this series have ongoing problems with bowel continence requiring a permanent stoma in some. Diligent follow-up coupled with inputs of a multidisciplinary team has greatly helped manage these complex patients in our institution.

Postoperative Hirschsprung’s associated enterocolitis (HAEC): transition zone as putative histopathological predictive factor

AimsHirschsprung’s-associated enterocolitis (HAEC) is the most severe complication of Hirschsprung disease (HD), and its pathogenesis is still unknown. Length of transition zone (TZ) interposed between aganglionic and normal bowel has...

Preoperative management comprising tube irrigation using a trans-anal indwelling tube for infants with hirschsprung disease can allow single-stage radical surgery

BackgroundPreoperative management of Hirschsprung’s disease (HD) is currently being conducted with the goal of performing single-stage radical surgery without ileostomy.MethodsWe retrospectively reviewed HD cases between 2013 and 2022, as well as their outcomes related to preoperative management.ResultsThirty-nine patients with HD were included in this study, including short-segment HD (30 cases), long-segment HD (4 cases), and total colonic aganglionosis (5 cases). Among these 39 patients, 95% (37 of 39 patients) underwent single-stage radical surgery after management with glycerin enema use (n = 13), irrigation with tube insertion each time irrigation was performed (n = 13), and irrigation using a tube placed in the bowel (n = 11).ConclusionsPreoperative management of patients with HD allowed for single-stage surgery of long-segment HD and total colonic aganglionosis. Cases that could be managed without performing an emergency enterostomy during the neonatal period were managed with irrigation until radical surgery was performed.

A unicentric cross-sectional observational study on chronic intestinal inflammation in total colonic aganglionosis: beware of an underestimated condition

BackgroundInflammatory Bowel Diseases (IBD) are known to occur in association with Hirschsprung disease (HSCR). Most of cases are represented by Crohn Disease (CD) occurring in patients with Total Colonic Aganglionosis (TCSA) with an estimated prevalence of around 2%. Based on these considerations and on a number of provisional data belonging to our Center for Digestive Diseases, we developed a unicentric cross-sectional observational study aimed at describing phenotype, genotype, pathology and metagenomics of all patients with TCSA and Crohn-like lesions.ResultsOut of a series of 62 eligible TCSA patients, 48 fulfilled inclusion criteria and were enrolled in the study. Ten patients did not complete the study due to non-compliance or withdrawal of consent and were subsequently dropped out. A total of 38 patients completed the study. All patients were tested for chronic intestinal inflammation by a combination of fecal calprotectine (FC) or occult fecal blood (OFB) and underwent fecal metagenomics. Nineteen (50%) tested positive for FC, OFB, or both and subsequently underwent retrograde ileoscopy. Fourteen patients (36.8%) presented Crohn-like lesions, occurring after a median of 11.5 years after surgery (range 8 months − 21.5 years). No statistically significant differences regarding demographic, phenotype and genotype were observed comparing patients with and without lesions, except for need for blood transfusion that was more frequent in those with lesions. Faecal microbiome of patients with lesions (not that of caregivers) was less biodiverse and characterized by a reduction of Bacteroidetes, and an overabundance of Proteobacteria. FC tested negative in 3/14 patients with lesions (21%).ConclusionsOur study demonstrated an impressive 10-folds higher incidence of chronic inflammation in TCSA. Up to 50% of patients may develop IBD-like lesions postoperatively. Nonetheless, we failed in identifying specific risk factors to be used to implement prevention strategies. Based on the results of our study, we suggest screening all TCSA patients with retrograde ileoscopy regardless of FC/OFB values. The frequency of endoscopic assessments and the role of FC/OFB screening in prompting endoscopy is yet to be determined.

Late Diagnosis of Hirschsprung Disease: Clinical Presentation and Long-Term Functional Outcomes

PurposeHirschsprung Disease (HD) is typically diagnosed in the neonatal period. A small subset of patients have a prolonged course of abdominal distention and constipation prior to diagnosis. Late HD is defined as having been diagnosed at greater than or equal to one year of age. The literature is limited and offers conflicting data on the implications of a late diagnosis. We aim to investigate the presentation, operative approach, and functional outcomes of a large cohort of patients with a late HD diagnosis. MethodsAll patients with a late diagnosis of HD (after 1 year of age) at our institution between 1997 and 2021 were included. ResultsTwenty-eight patients were diagnosed with HD at a median age of 3.4 years. Chronic constipation, failure to thrive, and enterocolitis occurred in 100 %, 31 %, and 14 %, respectively. All patients underwent contrast enema and biopsies during their workup, identifying primarily rectosigmoid disease (n = 27) and total colonic aganglionosis (n = 1). Surgical intervention was performed in 27 patients, with 4 patients (15 %) needing a stoma (3 with plan for staged pull-through, 1 long-term stoma) and 23 patients (85 %) undergoing a single-stage pull-through. Postoperative complications included Hirschsprung-associated enterocolitis (n = 5), ostomy prolapse and revision (n = 2), abdominal distention requiring ileostomy creation (n = 2), redo pull-through (n = 2), retroperitoneal hematoma (n = 1), and cecostomy tube placement (n = 1). At a median follow-up of 5.4 years, 83 % of eligible patients achieved fecal continence with 43 % needing laxatives for persistent constipation. ConclusionRecognizing a late presentation of HD requires a high index of suspicion. Patients with a late diagnosis did not experience an increased rate of permanent stoma, complications, or redo surgery compared to rates reported for the larger HD population. Similar long-term functional outcomes were achieved compared to the larger HD population. Level of EvidenceIV.

Duhamel Versus Swenson Pull-Through for Total Colonic Aganglionosis: A Multi-Institutional Study

BackgroundTotal colonic aganglionosis (TCA) is a rare variant of Hirschsprung disease (HD) where the colon and portion of distal ileum lack ganglion cells. Most pediatric use either a straight ileoanal (Swenson or Yancey-Soave) or a short Duhamel pull-through for TCA. There are no large studies comparing these techniques. We aimed to compare short-and medium-term outcomes between these approaches. MethodA retrospective review was performed among children with TCA from 2001 to 2019 undergoing a primary Duhamel or Swenson pull-through across three large children's hospitals. Patients undergoing redo and patients with greater than 30 % small bowel aganglionosis were excluded. We gathered data on demographics, operative approach, and outcomes at one, two, and three years. Continuous variables were analyzed with t-tests and categorical variables with Chi square or Fisher's tests. ResultsThere were 54 patients, with 26 (48 %) undergoing Duhamel and 28 (52 %) undergoing Swenson pull-through. There were no differences in sex, age, medical comorbidities, or operative details, including age at pull-through, laparoscopic vs open, length of involved small bowel, and operative time. Length of stay and post-operative complications were not different. Three years after pull-through, patients undergoing Duhamel had fewer stools per day (1-3 stools 69.6 % vs 14.3 %, p = 0.003) and were less likely to be prescribed fiber supplementation (4.2 % vs 43.8 %, p = 0.003). There were no differences in irrigations, botulinum toxin administration, loperamide, or HD admissions. ConclusionBoth Duhamel and straight pull-throughs are safe for treatment of TCA, with acceptable short- and medium-term outcomes. Further studies on patient-reported outcomes are necessary to examine long-term differences. Level of EvidenceIII.

Comparative assessment of fully laparoscopic Duhamel-Z with minimal rectorectal dissection vs. laparoscopy-assisted Duhamel-Z with blunt manual rectorectal dissection for total colonic aganglionosis.

Early postoperative outcome (EPO) was compared between fully laparoscopic Duhamel-Z (F-Dz) and laparoscopy-assisted Duhamel-Z (A-Dz) anastomoses performed for total colonic aganglionosis (TCA). EPO was assessed quarterly for the first year after F-Dz/A-Dz using a continence evaluation score (CES) based on stool frequency (motions/day) and stool consistency (0 = liquid, 1 = soft, 2 = formed), presence of anal erosion (0 = severe, 1 = moderate, 2 = mild), and incidence of enterocolitis.Surgical technique involved taking the ileostomy down, dissecting the colon laparoscopically, and preparing the pull-through ileum through the stoma wound. In F-Dz (n = 3), a working port (SILS trocar) was inserted, and laparoscopic retrorectal dissection with forceps used to create a retrorectal tunnel from the peritoneal reflection extending downward as narrow as possible along the posterior wall of the rectum to prevent lateral nerve injury and preserve vascularity. After completing the tunnel, the ileum was pulled-through from an incision on the anorectal line and a Z-shaped ileorectal side-to-side anastomosis performed without a blind pouch. In A-Dz (n = 11), the retrorectal pull-through route was created through a Pfannenstiel incision using blunt manual (finger) dissection along the anterior surface of the sacrum. Subject backgrounds were similar. Mean quarterly data were: frequency (F-Dz: 4.67, 4.67, 4.67, 3.33) vs. (A-Dz: 7.27, 7.09, 6.18, 5.36) p < .05; consistency (F-Dz: 0.33, 0.67, 0.67, 0.67) vs. (A-Dz: 0.27, 0.45, 0.70, 0.73) p = ns; anal erosion (F-Dz: 0.33, 0.33, 0.33, 0.67) vs. (A-Dz: 0.18, 0.36, 0.45, 0.64) p = ns; and enterocolitis (F-Dz: 1 episode in 1/3 cases or 33.3%) vs. (A-Dz: 7 episodes in 6/11 cases or 54.5%) p = ns. Overall, EPO after F-Dz was better than after A-Dz.

Rare presentation and retrograde diagnosis of total colonic aganglionosis in a female infant: a case report

BackgroundTotal colonic aganglionosis is an extremely rare variant of Hirschsprung’s disease, which is predominant in males and can be seen in 1:50,000 live births. The presented case not only depicts a rare case, but also unusual clinical, laboratory, and instrumental data.Case presentationA 2-day-old Caucasian female newborn was transferred to our hospital from maternity. The initial presentation was reverse peristalsis, abdominal distention, and inability to pass stool. Fever had started before the patient was transferred. Hirschsprung’s disease was suspected, and tests such as contrast enema and rectal suction biopsy were done. Before enterostomy, the management of the disease included fluid resuscitation, colonic irrigation, antibiotic administration, enteral feeding, and supportive therapy. During ileostomy operation, no transition zone was visualized and full-thickness biopsy samples were retrieved from the rectum and descending colon. After surgical intervention, status significantly improved—defervescence and weight gain most importantly improved.ConclusionIt is well known that diagnosis of total colonic aganglionosis may be delayed for months or even years since the transition zone may not be visible and rectal suction biopsy, unlike full-thickness biopsy, is not always reliable. It might be more prudent not to be derailed because of negative radiography and rectal suction biopsy. Also, doctors should be more suspicious of the disease if signs and symptoms are starting to be consistent with Hirschsprung-associated enterocolitis, despite biopsy and radiology results.

Intestinal obstruction caused by mesodiverticular band in children: a cohort study

To explore clinical characteristics, pathogenesis, diagnosis and treatment of intestinal obstruction due to mesodiverticular band (MDB) in children in a single center in China. The clinical data of 20 children with acute intestinal obstruction due to MDB between 1998 and 2020 were retrospectively analyzed. The male-to-female ratio was 14:6 in 20 cases. Except one case of 7-month pregnant stillbirth, the cases were aged from 7days to 14years, at the median age of 4.31years. The common symptoms were vomiting, abdominal pain and/or abdominal distension. About 40% (8/20) of patients had both MDB and Meckel's Diverticulum (MD), while 60% (12/20) of patients had MDB only. Only one case died because of total colonic aganglionosis, while other children recovered after surgery treatment. MDB led to the strangulation of necrotic bowel in six cases, intestinal perforation in one case, and intestinal rupture in one case. Pathologic examination showed thick-walled arteries and or thick venous vascular structures in the cord. All cases had no complications during 1-year follow-up. MDB results from the remnant of vitelline vessel, and often causes acute intestinal obstruction without special clinical symptoms. Unexplained abdominal pain and distension without surgery history should be paid attention, especially for strangulated intestinal obstruction. Timely surgical exploration is beneficial to avoid intestinal necrosis or even sudden death, and the pathological examination is important for the diagnosis.

Prognostic factors of postoperative Hirschsprung-associated enterocolitis: a cohort study

To identify prognostic factors of postoperative Hirschsprung-associated enterocolitis (HAEC). A retrospective cohort study of Hirschsprung patients between 2006 and 2021 was conducted. Patients with anorectal malformation and non-definitive surgery were excluded. Associated factors for postoperative HAEC were reported with hazard ratio (HR) and 95%confidence interval (CI). Forty-nine patients were excluded due to concurrent anorectal malformation and incomplete data. Of 274 patients, 75 patients (27.4%) had at least one episode of postoperative HAEC. There were 28 patients (37.3%) who had multi-episodes of HAEC. The total episodes of post-operative HAEC in this study were 121 episodes (36.8%). In multivariable survival analysis, significant factors associated with postoperative HAEC were the aganglionic level above sigmoid colon (HR = 3.47, p = 0.023, 95%CI 1.19-10.09), and total colonic aganglionosis (HR = 14.83, p = 0.004, 95%CI 2.33-94.40). The patients who experienced clinical enterocolitis before 2weeks after surgery significantly developed more postoperative HAEC (HR = 5.32, p = 0.038, %CI 1.09-25.92). The incidence of postoperative HAEC was increase in patients with postoperative obstructive symptoms (48.0%). One patient died due to severe sepsis from postoperative HAEC, while three others required intensive care. The long involvement of aganglionic segment and early postoperative HAEC was significantly associated with postoperative HAEC. Frequent follow-up, parental education, and early treatment are recommended in these individuals, particularly in the first year after surgery.

Laparoscopic restorative proctocolectomy with ileal-J-pouch anal canal anastomosis without diverting ileostomy for total colonic and extensive aganglionosis is safe and feasible with combined Lugol's iodine staining technique and indocyanine green fluorescence angiography.

We present the surgical technique and outcomes of reduced-port laparoscopic restorative proctocolectomy with ileal-J-pouch anal canal anastomosis (IPACA) without diverting ileostomy for total colonic and extensive aganglionosis (TCA+). We retrospectively reviewed TCA+ cases between 2014 and 2022. Preoperative ileostomy was performed when transanal bowel irrigation was ineffective. Radical surgery for TCA+ was performed at approximately 6 kg. The surgery was performed using laparoscopy through a multi-channel trocar with or without an additional 3-mm trocar and IPACA reconstruction with indocyanine green fluorescence angiography (ICG) to assess anastomotic perfusion and Lugol's iodine staining to visualize the surgical anal canal. Ten patients with TCA+ were included. Ileostomy was performed in seven cases. The median operation time and blood loss were 274.5 min and 20 ml, respectively. No significant postoperative complications were found. All patients experienced frequent liquid stools and perianal excoriation in the early postoperative period, requiring anti-flatulence or codeine. The median follow-up period was 3.5 years. Three patients required irrigation management 1 year postoperatively, and the others defecated a median of 3.5 times per day. The median Kelly's clinical score was 5 in 5 patients aged >4 years. Reduced-port surgery, combined with Lugol's iodine staining and ICG, was safe, feasible, and had cosmetically and clinically acceptable mid-term outcomes.