Surgery For Hirschsprung Disease Research Articles

Histopathology of Ganglion Cells in the Proximal Resected Bowel Correlates With the Clinical Outcome in Hirschsprung Disease: A Pilot Study

IntroductionIntraoperative leveling biopsy by identifying ganglion cells is crucial to determine surgical margin during surgery for Hirschsprung disease (HSCR). The anastomosis should be performed at least 5 cm proximal to the ganglionic segment to prevent transition zone pull-through. However, the length of the transition zone could be much longer than expected and the histological evaluation of the entire circumference of the proximal margin is recommended, which is time-consuming and not applicable for leveling biopsy. We found that the histopathologic features of ganglion cells varied in the examined bowel specimens and demonstrated a pattern similar to immature and degenerated neuron cells. We assumed that the histopathologic features of ganglion cells in the proximal resected bowel were associated with the clinical outcome and might guide the leveling biopsy. In this study, we described a histopathologic grade of ganglion cells based on the degree of maturity and degeneration. We assessed the correlation between the histopathological grade of ganglion cells in the proximal surgical margin and clinical outcome. MethodsThree hundred fifty seven patients with HSCR treated between 2013 and 2020 were included. The ganglion cells were divided into six grades based on the histopathologic features in frozen sections. Medical records and detailed histopathologic results of intraoperative frozen sections were reviewed. Follow-up data were collected to evaluate clinical outcomes. The pediatric incontinence and constipation scoring system was used to predict bowel function. ResultsThe histopathologic results of proximal resected bowel from 357 HSCR patients were presented as follows: Grade I in 52 patients (14.6%), Grade II in 186 patients (52.1%), Grade III in 107 patients (30.0%), and Grade IV in 12 patients (3.4%). The median follow-up time was 46.8 mo (13.0-97.6 mo). The histopathologic grade of ganglion cells from the proximal resected margin was significantly related to postoperative constipation problems and the incidence of Hirschsprung-associated enterocolitis. The results from the pediatric incontinence and constipation scoring system indicated a positive correlation between better postoperative bowel function and lower histopathologic grade of ganglion cells. ConclusionsThis pilot study showed an association between the histopathologic features of ganglion cells in the proximal surgical margin and the clinical outcome. It may provide additional information for intraoperative pathologic consultation in leveling biopsy to prevent insufficient resection of the affected colon. A prospective study is warranted to validate these findings before clinical application.

The potential effects and mechanism of echinacoside powder in the treatment of Hirschsprung's Disease.

Possible complications, such as intestinal obstruction and inflammation of the intestinal tract, can have a detrimental effect on the prognosis after surgery for Hirschsprung disease. The aim of this study was to investigate the potential targets and mechanisms of action of echinacoside to improve the prognosis of Hirschsprung disease. Genes related to the disease were obtained through analysis of the GSE96854 dataset and four databases: OMIM, DisGeNET, Genecard and NCBI. The targets of echinacoside were obtained from three databases: PharmMapper, Drugbank and TargetNet. The intersection of disease genes and drug targets was validated by molecular docking. The valid docked targets were further explored for their expression by using immunohistochemistry. In this study, enrichment analysis was used to explore the mechanistic pathways involved in the genes. Finally, we identified CA1, CA2, CA9, CA12, DNMT1, RIMS2, RPGRIP1L and ZEB2 as the core targets. Except for ZEB2, which is predominantly expressed in brain tissue, the remaining seven genes show tissue specificity and high expression in the gastrointestinal tract. RIMS2 possesses a high mutation phenomenon in pan-cancer, while a validated ceRNA network of eight genes was constructed. The core genes are involved in several signaling pathways, including the one-carbon metabolic process, carbonate dehydratase activity and others. This study may help us to further understand the pharmacological mechanisms of echinacoside and provide new guidance and ideas to guide the treatment of Hirschsprung disease.

A novel evaluation technique for measuring the distance between the anastomosis and intersphincteric groove via three-dimensional endoanal ultrasonography in children with Hirschsprung disease

BackgroundThis study aimed to describe our experience with three-dimensional endoanal ultrasonography (3D-EAUS) in patients who underwent surgery for Hirschsprung disease and to summarize the relationship between postoperative anal function and the distance between the anastomosis and intersphincteric groove (DBAI) measured via 3D-EAUS.ResultsWe retrospectively reviewed patients with a history of undergoing surgery for Hirschsprung disease who visited our outpatient clinic between December 2018 and December 2019. All patients underwent 3D-EAUS for DBAI measurement. We used the Krickenbeck classification to evaluate postoperative anorectal function.Eleven patients (all males aged 3–14 years) were evaluated. Four (36.4%), four (36.4%), and three (27.3%) patients had no soiling, grade 1 soiling, and grade 3 soiling, respectively. Four (36.4%) and seven (63.6%) patients had no constipation and grade 3 constipation, respectively. The median DBAI values were 7.0 mm, 8.4 mm, and 5.6 mm (p = 0.14) in the no soiling, grade 1, and grade 3 soiling groups, respectively.Conclusions3D-EAUS enabled precise visualization of the anal anatomy and evaluation of the anastomosis. The DBAI was relatively short in patients with grade 3 soiling, although not significantly so. Further evaluation is warranted.

A novel Lugol\u2019s iodine staining technique to visualize the upper margin of the surgical anal canal intraoperatively for Hirschsprung disease: a case series

BackgroundIn cases of Hirschsprung disease, complete and reproducible resection of the aganglionic bowel is ideal to achieve good postoperative bowel function. Reliable identification of the upper margin of the surgical anal canal, which is the squamous-columnar junction, is necessary during transanal pull-through. Here, we describe a novel staining technique using Lugol’s iodine stain to visualize the upper margin of the surgical anal canal.MethodsLugol’s iodine staining was performed in five patients with Hirschsprung disease treated using a single-stage laparoscopic transanal pull-through modified Swenson procedure. In two of these patients, endocytoscopic observation with ultra-high magnification was performed using methylene blue and crystal violet to mark the border of the squamous epithelium at 1 week before surgery. The alignment between the incisional line, which was revealed using Lugol’s iodine staining and endocytoscopic marking, was evaluated. Complications, including postoperative bowel dysfunction, were evaluated.ResultsIn all cases, Lugol’s iodine staining produced a well-demarcated line. The endocytoscopic marking of the upper margin of the surgical anal canal was aligned with the line revealed by Lugol’s iodine staining. There were no complications associated with the transanal pull-through procedure, including postoperative bowel dysfunction.ConclusionsLugol’s iodine staining could be a safe and practical method to visualize the upper margin of the surgical anal canal intraoperatively. This finding may be useful for surgeons to make a consistent removal of the aganglionic bowel during surgery for Hirschsprung disease.

Botulinum toxin injections after surgery for Hirschsprung disease: Systematic review and meta-analysis

BACKGROUNDA large proportion of patients with Hirschsprung disease experience persistent obstructive symptoms after corrective surgery. Persistent obstructive symptoms may result in faecal stasis that can develop into Hirschsprung-associated enterocolitis, a potential life-threatening condition. Important treatment to improve faecal passage is internal anal sphincter relaxation using botulinum toxin injections.AIMTo give an overview of all empirical evidence on the effectiveness of botulinum toxin injections in patients with Hirschsprung disease.METHODSA systematic review and meta-analysis was done by searching PubMed, EMBASE and the Cochrane Library, using entry terms related to: (1) Hirschsprung disease; and (2) Botulinum toxin injections. 14 studies representing 278 patients met eligibility criteria. Data that were extracted were proportion of patients with improvement of obstructive symptoms or less enterocolitis after injection, proportion of patients with adverse effects and data on type botulinum toxin, mean dose, average age at first injection and patients with associated syndromes. Random-effects meta-analysis was used to aggregate effects and random-effects meta-regression was used to test for possible confounding factors.RESULTSBotulinum toxin injections are effective in treating obstructive symptoms in on average 66% of patients [event rate (ER) = 0.66, P = 0.004, I2 = 49.5, n = 278 patients]. Type of botulinum toxin, average dose, average age at first injections and proportion of patients with associated syndromes were not predictive for this effect. Mean 7 duration of improvement after one botulinum toxin injections was 6.4 mo and patients needed on average 2.6 procedures. There was a significant higher response rate within one month after botulinum toxin injections compared to more than one month after Botulinum toxin injections (ER = 0.79, vs ER = 0.46, Q = 19.37, P < 0.001). Botulinum toxin injections were not effective in treating enterocolitis (ER 0.58, P = 0.65, I2 = 71.0, n = 52 patients). There were adverse effects in on average 17% of patients (ER = 0.17, P < 0.001, I2 = 52.1, n = 187 patients), varying from temporary incontinence to mild anal pain.CONCLUSIONFindings from this systematic review and meta-analysis indicate that botulinum toxin injections are effective in treating obstructive symptoms and that adverse effects were present, but mild and temporary.

A recalcitrant case of Jacquet erosive diaper dermatitis after surgery for Hirschsprung disease in a boy with Waardenburg-Shah syndrome

We herein present a 4 year-old boy with Waardenburg-Shah syndrome who developed Jacquet erosive diaper dermatitis following a total colectomy and ileoanal anastomosis procedure for Hirschsprung disease. The diagnosis was made according to history and typical clinical findings. Complete resolution of the recalcitrant lesions after an ileostomy procedure supported the diagnosis. This case highlights the importance of being familiar with the predisposing factors and clinical presentation of this rare and severe form of chronic irritant dermatitis, since it may easily be misdiagnosed as other diseases in children and may lead to unnecessary diagnostic procedures, treatments, and anxiety due to suspicion of child abuse.

Prevalence, Risk Factors, and Prognosis of Postoperative Complications after Surgery for Hirschsprung Disease

BackgroundAlthough most of patients do well after surgery for Hirschsprung disease (HSCR), there are complications in some instances that impact social aspects and quality of life. The aim of this study was to explore the prevalence, risk factors, and prognosis of these complications, providing guidance for surgeons and healthcare personnel. MethodsA cohort of patients (N = 229) was retrospectively reviewed in the aftermath of surgery for HSCR. All medical data and operative notes were assessed. Early and late postoperative complications were solicited by questionnaire, using logistic regression and the Cox proportional hazards regression model for analysis. ResultsA total of 181 patients qualified for the study. Enterocolitis and soiling/incontinence constituted the most frequent complications, whether early or late in the postoperative period. Risk factors for developing enterocolitis included low weight, low-level IgA, preoperative enterocolitis, and lengthy aganglionic segment in the early term; whereas preoperative enterocolitis and diet control impacted complications emerging later. Risk factors in early soiling/incontinence were low weight, operative age of < 2 months, low IgA level, and lengthy aganglionic segment. Lengthy aganglionic segment, operative age of < 2 months, and toilet training were factors long-term. Prognostic factors included diet control and toilet training. ConclusionEnterocolitis and soiling/incontinence remain the most frequent complications after surgery for HSCR. Risk factors in early and late postoperative periods differed, with diet control and toilet training contributing favorably to enterocolitis and soiling/incontinence, respectively.

Anal endosonography and bowel function in patients undergoing different types of endorectal pull-through procedures for Hirschsprung disease

BackgroundThe reasons for fecal incontinence after surgery for Hirschsprung disease (HD) remain unclear. The aim of this study was to examine the anal sphincters by anal endosonography and manometry after transanal endorectal pull-through, with or without laparotomy or laparoscopy, in HD patients. Furthermore, we aimed to correlate these findings to bowel function. Patients and methodsFifty-two HD patients were followed after endorectal pull-through. Anal endosonography and manometry were performed without sedation at the age of 3 to 16years. ResultsEndosonographic internal anal sphincter (IAS) defects were found in 24/50 patients, more frequently after transanal than transabdominal procedures (69 vs. 19%, p=0.001). In a multiple variable logistic regression model, operative approach was the only significant predictor for IAS defects. Anal resting pressure (median 40mm Hg, range 15–120) was not correlated to presence of IAS defects. Daily fecal incontinence occurred more often in patients with IAS defects (54 vs. 25%, p=0.03). ConclusionsPostoperative IAS defects were frequently detected and were associated with daily fecal incontinence. IAS defects occurred more often after solely transanal procedures. We propose that these surgical approaches are compared in a randomized controlled trial before solely transanal endorectal pull-through is performed as a routine procedure.

The effect of extended care on behavior mental state of patients with Hirschsprung disease

Objective To explore the effect of extended care on behavior mental state of patients with Hirschsprung disease.Methods 40 cases of patients with Hirschsprung disease underwent surgery in our hospital from January 2009 to June 2010 were set as the control group.42 cases of patients with Hirschsprung disease underwent surgery in our hospital from July 2011 to June 2012 were set as the experimental group.The patients in the control group received conventional guidance after Hirschsprung disease surgery before discharge,which included dietary guidance,anus functional exercise,regular anal expansion,follow-up upon discomfort and regular re-visit.The patients in the experimental group not only received conventional guidance after Hirschsprung disease surgery before discharge,but also received systematic extended care plan.The nursing effect was compared between two groups.Results Conduct problems and hyperactivity index of patients in the experimental group were compared with those in the control group,the differences were significant.The scores of learning problems,psychosomatic disorders,impulsivity-hyperactivity and anxiety were lower,and compared with those in the control group,the differences were not significant.Conclusions Implementation of extended care for patients with Hirschsprung disease surgery can improve behavior mental state of them. Key words: Extended care; Health education; Hirschsprung disease; Surgery; Behavior mental state

Inflammatory Bowel Disease Manifesting After Surgical Treatment for Hirschsprung Disease

Eight children developed chronic inflammatory bowel disease (IBD) 4 to 21 years after surgery for Hirschsprung disease. Three had trisomy 21 and 6 experienced chronic or recurrent enterocolitis. Four had a family history of IBD. Clinical presentation included chronic diarrhea, hematochezia, abscess, and fistula formation. Three required surgery for fistula, stricture, and small bowel obstruction and the other 5 were managed medically. Recognition of this condition may be important in the long-term follow-up of children with Hirschsprung disease, and patients who have carried a diagnosis of chronic enterocolitis may warrant further investigation looking for evidence of IBD.

Is daily dilatation by parents necessary after surgery for Hirschsprung disease and anorectal malformations?

Background/Purpose Most surgeons recommend daily dilatation after surgery for Hirschsprung disease and anorectal malformations. Our goal was to critically evaluate the potential risks and benefits of this practice. Methods A retrospective chart review was carried out of all children undergoing repair of Hirschsprung disease or anorectal malformation over 5 years. Patients with long segment Hirschsprung disease or anal stenosis were excluded. Results There were 95 patients, of which 34 had Hirschsprung disease and 61 had an anorectal malformation. Postoperatively, 65 underwent routine dilatation by parents; and 30 underwent weekly calibration by the surgeon, with daily dilatation by the parents only if the anastomosis was felt to be narrow. Of the 30 children undergoing weekly calibration, only 5 (17%) developed late narrowing that required conversion to the daily parental dilatation approach. There were no significant differences between the 2 approaches with respect to stricture development, anastomotic disruption, perineal excoriation, or enterocolitis. Conclusion Weekly calibration by the surgeon is associated with similar outcomes to daily dilatation by the parents. Because this approach is kinder to the parents and the child, it should be seriously considered for the postoperative management of children with Hirschsprung disease or anorectal malformations.

Vulvar Pseudoverrucous Papules and Nodules Secondary to a Urethral–Vaginal Fistula

Irritant contact dermatitis is a common cause of genital eruptions representing at least one-fifth of presenting anogenital symptoms. A spectrum of inflammatory reactions have been identified, some of them with features severely mimicking more serious dermatoses, which may lead unnecessary workup and treatments. We report a case of a 10-year-old girl who presented at birth with cloacal atresia involving the rectum and the urethra. A diverting colostomy and a urethral-vaginal fistula were created to correct the deformity. Physical examination reveals numerous shiny, white-gray, pseudoverrucous papules and nodules coalescing into plaques over the vulva and its surrounding skin. Histological examination showed psoriasiform epidermal hyperplasia with a marked reactive acanthosis and altered cornification with parakeratosis, hypogranulosis, and pale keratinocytes in the upper reaches of the epidermis. The lesions regress when the irritating factor was removed. This case represents a peculiar form of presentation of perianal pseudoverrucous papules and nodules, usually secondary to urinary incontinence or encopresis (inability to control the elimination of stool) after surgery for Hirschsprung disease. Because similar findings have not been previously observed in patients with urethral-vaginal fistula, we attempt to extend the spectrum of presentations for a better knowledge of this condition.

Quality of Life in Adults Operated On for Hirschsprung Disease in Childhood

The aim of this study is to explore the long-term quality of life (QoL) in adults after surgery for Hirschsprung disease in childhood. Altogether 51 patients were operated for HD at our center during the period 1969 to 1989. In 2007, the validated instruments for assessing QoL, the SF-36 health survey and the Gastrointestinal Quality of Life Index (GIQLI), were sent to the 47 patients still alive at the time of the study. A retrospective review of the patient records was also done. Forty-two patients returned both questionnaires (89% response rate) at the median age of 28.5 (range 18-45) years. The median clinical follow-up time was 5.7 years (range 5 months-23 years). At the last clinical control, 4 (9%) patients had a terminal enterostomy, 12 (29%) had soiling, 5 (12%) had constipation, and 2 (5%) experienced recurrent enterocolitis. In contrast to males, the subscores for females were lower for general health and mental health than for an age- and sex-matched general population (P < 0.05). Patients having aganglionosis to the right colon had lower GIQLI scores than those with aganglionosis to the left colon (P < 0.05). In multivariate linear regression analysis, female sex was the only factor associated with lower GIQLI score (P < 0.05). The long-term QoL of adults operated for Hirschsprung disease in their youth is satisfactory. Female scores were lower for general and mental health, compared with the matched control group. The study indicates that the longer the aganglionic segment, the greater its impact on QoL in later life.

Topical Nitric Oxide Treatment after Pull Through Operations for Hirschsprung Disease

The diagnosis and treatment of Hirschsprung disease are well standardized. Symptoms of obstruction after surgery for Hirschsprung disease may result from residual spasticity of the internal anal sphincter. Nitric oxide (NO) is the chemical messenger mediating relaxation of the internal anal sphincter and its exogenous application results in a relaxation response in smooth muscle. The purpose of this study was to investigate topical NO application for obstructive symptoms after surgery for Hirschsprung disease and to correlate the symptoms with manometric findings with a view to directing further management. The authors reviewed application of topical NO on six symptomatic children who were operated for Hirschsprung disease. Eighteen patients older than 3 years of age were evaluated for anorectal manometric, functional and clinical outcome. The symptoms included enterocolitis in three patients and constipation in three patients. NO ointment was applied twice daily for 6 weeks in symptomatic patients and manometry was repeated. Anorectal manometric evaluation of six patients showed high internal anal sphincter tone without reflex relaxation on applying distending pressure to the rectum. Marked improvement of symptoms was noted after 6-week application of topical NO and maximal internal anal resting pressure decreased significantly (35% reduction). After ceasing application of topical ointment, increased maximal anal resting pressure was seen again. Although anal sphincter hypertonicity is not thought to be the only cause of post-operative obstructive symptoms, relaxation of the internal anal sphincter may improve the symptoms. Topical NO can be used for treatment of obstructive symptoms in Hirschsprung disease. We managed our symptomatic patients successfully with local NO application with a reversible chemical sphincterotomy. NO could be used as a therapeutic modality.