Standardization of radiograph readings during bowel management week.

INTRODUCTION: During "bowel management week," abdominal radiographs are used to monitor the amount and location of stool. A radiologist familiar with the treatment plan can provide an improved interpretation. The goal of this paper is to standardize the radiological reports during a bowel management week. METHODS: We saw 744 patients during bowel management week from May 2016 until March 2023. Diagnosis included: anorectal malformation (397), idiopathic constipation (180), Hirschsprung disease (89), and spina bifida (78). Laxatives were the treatment for 51% of patients, and 49% received enemas. Characteristic radiographs were selected for each treatment group for a proposed reading standardization. RESULTS: When the stool is visualized, it is crucial to report its location. Having a contrast enema helps with the correct interpretation of the colonic anatomy. It is also essential to always compare the amount of stool with the radiograph from the previous day to determine if there is an increase or decrease in stool. Examples of radiographs are shown to guide the use of the preferred proposed terminology. CONCLUSION: Providing information regarding which treatment modality the patient is receiving and stating that a patient is on a bowel management week treatment is crucial for the radiologist to provide adequate interpretation. The radiologist must be familiar with the treatment goals and purpose of the daily radiograph.

Intestinal organoids in infants and children.

Recent advances in culturing of intestinal stem cells and pluripotent stem cells have led to the development of intestinal organoids. These are self-organizing 3D structures, which recapitulate the characteristics and physiological features of in vivo intestinal epithelium. Intestinal organoids have allowed the development of novel in vitro models to study various gastrointestinal diseases expanding our understanding of the pathophysiology of diseases and leading to the development of innovative therapies. This article aims to summarize the current usage of intestinal organoids as a model of gastrointestinal diseases and the potential applications of intestinal organoids in infants and children. Intestinal organoids allow the study of intestinal epithelium responses to stress factors. Mimicking intestinal injury such as necrotizing enterocolitis, intestinal organoids increases the expression of pro-inflammatory cytokine genes and shows disruption of tight junctions after they are injured by lipopolysaccharide and hypoxia. In cystic fibrosis, intestinal organoids derived from rectal biopsies have provided benefits in genetic studies and development of novel therapeutic gene modulation. Transplantation of intestinal organoids via enema has been shown to rescue damaged colonic epithelium in mice. In addition, tissue-engineered small intestine derived from intestinal organoids have been successfully established providing a potential novel treatment and a new hope for children with short bowel syndrome.

[The primary care of a patient with a history of a gastrointestinal malformation and abdominal wall or diaphragmatic defects]. / La atención primaria del paciente con el antecedente de una malformación digestiva, defectos de pared abdominal o diafragmáticos.

Survival of patients with congenital malformation has improved over the last decades. Primary care paediatricians must be aware of the most common problems that this group of patients suffers. More importantly, paediatricians can offer a holistic view that is often lost in specialised consultation. This article is focused on common congenital malformation, such as oesophageal atresia, abdominal wall defects, anorectal malformation and Hirschsprung disease, and congenital diaphragmatic hernia. The main problems are shown, with special emphasis on long-term complications and all the dimensions of the individual.

Damaged anal canal as a cause of fecal incontinence after surgical repair for Hirschsprung disease - a preventable and under-reported complication.

INTRODUCTION: Fecal incontinence after the surgical repair of Hirschsprung disease is a potentially preventable complication that carries a negative impact on patient's quality of life. METHODS: Patients that were previously operated for Hirschsprung disease and presented to our bowel management clinic with the complaint of fecal incontinence were retrospectively reviewed. All patients underwent a rectal examination under anesthesia looking for anatomic explanations for their incontinence. RESULTS: One hundred three patients were identified. 54 patients had a damaged anal canal. 22 patients also had a patulous anus. The operative reports mentioned the pectinate line in 32 patients, in 12 it was not mentioned, and in 10 patients the operative report was not available. All patients with a damaged anal canal suffered from true fecal incontinence; 45 of them are on daily enemas (41 are clean and 4 are still having "accidents"), 7 are not doing bowel management due to noncompliance and 2 patients have a permanent ileostomy. 49 patients did not have a damaged anal canal, 25 of those responded to changes in diet and medication and are having voluntary bowel movements. CONCLUSION: Fecal incontinence may occur after an operation for Hirschsprung disease. When the anal canal is damaged, incontinence is always present, severe, and probably permanent. The preservation of the anal canal may avoid this complication.

Botulinum toxin use in paediatric colorectal surgery.

PURPOSE: To evaluate outcomes of intrasphincteric botulinum toxin injection (ISBTI) in children with intractable constipation. METHODS: Retrospective case-note review of patients ≤ 16 years of age undergoing ISBTI between January 2010 and February 2014. Data collected included patient demographics, diagnosis, complications, follow-up duration and functional outcomes. Successful outcome was defined as resolution/improvement in symptoms and failed when there was no change in symptoms. Statistical analyses were performed using PRISM (GraphPad, CA, USA). p values <0.05 were considered as significant. RESULTS: 43 patients [male 29, median age 5 years 9 months (range 13 months-13 years 5 months)] underwent 86 ISBTIs. Underlying diagnoses were idiopathic constipation (67 %), Hirschsprung disease (26 %), anorectal malformation (5 %), gastrointestinal dysmotility (2 %). 72 % (31/43) reported improvement in symptoms after the first ISBTI. 39 % of patients had recurrence of symptoms at 12-month median follow-up. 10 patients non-responsive to ISBTI required an antegrade continence enema or stoma. There was no correlation between age (p = 0.3), gender (p = 0.7), diagnosis (p = 0.84), or number of ISBTIs (p = 0.17) with successful outcome. CONCLUSION: Successful outcomes occurred in 72 % patients after the first ISBTI. 25 % required further surgical management of their symptoms. Further work is required to help predict which patients will benefit from ISBTI.

Experience of the MACE procedure at a regional pediatric surgical unit: a 15-year retrospective review.

INTRODUCTION: The Malone antegrade continence enema (MACE) procedure is an established treatment option for children with constipation or fecal incontinence. This study retrospectively analyses the management and outcomes of children who underwent MACE procedures at a regional pediatric surgery unit. PATIENTS AND METHODS: Children who underwent a MACE procedure in our unit between 1998 and 2012 were identified. Demographic and clinical data were obtained from contemporaneous records. Using the continence scale described by Malone, overall outcomes were categorized as full, partial, or failure (full: totally clean or minor rectal leakage on night of washout; partial: clean but significant stoma or rectal leakage, occasional major leak and/or still wearing protection but perceived by child or parent to be an improvement; failure: regular soiling or constipation persisted, no perceived improvement, procedure was abandoned). Data entry and statistical analysis were performed using Excel and SPSS (IBM Corp., Armonk, New York, United States). RESULTS: A total of 40 children (29 male) were identified for inclusion. Underlying diagnoses were idiopathic constipation (16), anorectal anomalies (14) and Hirschprung's disease (10). The MACE procedure was performed laparoscopically in 26 cases and using an open technique in 14 cases. Mean age at the time of surgery was 8.9 years (range, 3-19 years) and mean follow-up time was 6.5 years (range, 1-10 years). Outcomes categorized as full were 62.5% for chronic idiopathic constipation (CIC), 71.4% for anorectal malformation (ARM), and 70% for Hirschsprung disease (HD). Overall success rates (full and partial outcomes combined) were 87.5% for CIC, 92.8% for ARM, 100% for HD, and 92.5% for all diagnoses taken together. Eleven MACE procedures (27.5%) were reversed, in seven (17.5%) due to the return of spontaneous and regular bowel movements and in four (10%) due to stomal problems. CONCLUSION: This study identified a high success rate (combining full and partial outcomes) of 92.5% for MACE procedures within our unit. An encouraging finding is that the procedure was reversed in a significant proportion of patients following the return of normal bowel habits.

Management of extremely low birth weight neonates with bowel obstruction within 2 weeks after birth.

PURPOSE: The majority of bowel obstructions in extremely low birth weight (ELBW) neonates are meconium-related ileus (MRI). ELBW neonates with bowel obstruction may recover by conservative treatment, but some do not. Considering the high surgical morbidity rates, unnecessary surgery should be avoided. We sought to identify a reasonable treatment strategy under these conditions. METHODS: ELBW neonates who started to have bowel obstruction with an unclear cause within 14 days of age were enrolled. The study period was from January 2009 to August 2011. The enrolled patients had daily Gastrografin(®) enemas until 14 days of age or until the obstruction resolved. If the obstruction lasted beyond around 14 days of age, the patient underwent surgical intervention. The clinical data of the patients were collected and analyzed. RESULTS: Fourteen patients were enrolled. Twelve patients had MRI, which resolved within 14 days without surgery. Two patients with persistent obstruction underwent surgery, and they were found to have Hirschsprung's disease and ileal volvulus, respectively. CONCLUSION: For ELBW neonates with bowel obstruction of unclear etiology, the early and frequent administration of a Gastrografin(®) enema is reasonable. Surgery should be considered if the obstruction lasts beyond approximately 14 days after birth.

Desafios diagnósticos na doença de Hirschsprung: aplicabilidade de novos métodos imunohistoquímicos e endoscópicos / Challenges in the diagnosis in Hirschsprun's disease: applecability of a new immunohistochemical and endoscopic methods

A identificação pré-operatória da zona de transição na Doença de Hirschsprung (DH) tornou-se um passo fundamental para o planejamento cirúrgico, especialmente para a técnica de abaixamento endorretal transanal. O presente estudo tem como objetivo investigar prospectivamente o valor da determinação colonoscópica da zona de transição na avaliação pré-operatória dos pacientes com DH. A colonoscopia foi realizada em doze pacientes com diagnóstico de DH previamente confirmado pela manometria anorretal, enema opaco e biópsia de sucção do reto. Endoscopicamente, o primeiro local com ausência de peristaltismo foi identificado como o início da zona agangliônica. Pouco acima deste ponto, a zona de transição foi marcada através de uma tatuagem com tinta nanquim. Durante o abaixamento endorretal transanal, uma biópsia de congelação envolvendo a espessura total da parede foi sempre realizada. Os resultados da determinação colonoscópica da zona de transição foram comparados com os obtidos pelo enema opaco. A colonoscopia permitiu a identificação da zona de transição em todos os 12 casos (100%). O enema opaco revelou a presença da zona de transição em apenas 7 pacientes (58,3%). A análise das amostras de congelação, obtidas pouco acima das áreas endoscopicamente marcadas, revelou a presença de células ganglionares em todos os casos. A análise histopatológica das peças cirúrgicas confirmou o diagnóstico de DH em todos os casos, assim como a localização da zona de transição no mesmo local previamente tatuado endoscopicamente...

Preoperative identification of the transition zone in Hirschsprung’s disease (HD) has become an essential issue for surgical planning, especially for Transanal Endorectal Pull- Through (TEPT) procedure. The present study aimed to investigate prospectively, the value of endoscopic marking of the transition zone between normal and aganglionic bowel, as a landmark of the location of pull-through procedure for treatment of HD. Colonoscopy was performed on twelve patients with HD diagnosis, previously confirmed by anorectal manometry, contrast enema and rectal suction biopsies. Endoscopically, the first site with absence of motility was identified as the beginning of the aganglionic area. Just above this point, the transition zone was marked with an Indian Ink tattooing. During the TEPT, a full-thickness biopsy for frozen section analysis was performed just above this mark. The results of colonoscopic making were compared with contrast enema. Colonoscopy allowed the identification and tattooing of the junction between normal bowel with peristalsis and aganglionic bowel without peristalsis in all 12 cases (100%). Barium enema revealed the transition zone in 7 patients (58.3%). Frozen samples, obtained just above the marked areas revealed the presence of ganglion cells in all cases and the histopathological analysis of surgical specimens confirmed the diagnosis of HD in all cases and checked the location of the transition zone at the same site previously identified by colonoscopy...

Failure of resolution of portal fibrosis during omega-3 fatty acid lipid emulsion therapy in two patients with irreversible intestinal failure.

Parenteral omega-3 fatty acid lipid emulsions have been evaluated for their potential role in reversing intestinal failure-associated liver disease. We report our experience using Omegaven in 2 patients with irreversible intestinal failure and intestinal failure-associated liver disease. Despite biochemical and histologic improvement in cholestasis, both patients had persisting, significant portal fibrosis on liver biopsy.

Antegrade enemas for defecation disorders: do they improve the colonic motility?

PURPOSE: The aim of the study was to describe the changes in colonic motility occurring after chronic antegrade enema use in children and young adults. METHODS: Colonic manometry tracings of patients who had used antegrade enemas for at least 6 months and were being evaluated for possible discontinuation of this treatment were retrospective reviewed. RESULTS: Seven patients (median age of 12 years, range 3-15 years) met our inclusion criteria. Four patients had idiopathic constipation, 2 had tethered cord, and 1 had Hirschsprung disease. Colonic manometry before the use of antegrade enemas showed dysmotility in 6 (86%) children, mostly in the distal colon. None of the patients underwent colonic resection between the 2 studies. All the patients had colonic manometry repeated between 14 and 46 months after the creation of the cecostomy. All patients with abnormal colonic manometry improved with the use of antegrade enema with a complete normalization of colonic motility in 5 (83%) patients. CONCLUSION: Use of antegrade enema alone, without diversion or resection, may improve colonic motility.

Management principles of organic causes of childhood constipation.

Constipation is extremely common in the pediatric population; however, it is a symptom and not a disease. Organic causes of constipation include Hirschprung's disease, anorectal malformation, and spina bifida. This article also discusses constipation and urinary tract pathology and dysfunction. The function of the gastrointestinal and urinary tracts are intertwined, and this concept will assist the practitioner in managing patients accordingly. Children and adolescents who experience bowel dysfunction require patience and sensitive support from their health care providers so that they can live productive and emotionally healthy lives.

[Diagnostics and treatment of chronic constipation in children--the experience of the department of paediatric surgery]. / Postepowanie diagnostyczno-terapeutyczne u dzieci z przewleklymi zaparciami--doswiadczenia oddzialu chirurgii dzieciecej.

The chronic constipation in children can pose a big problem both in pediatric and surgical practice. The purpose of this work is to present diagnostic and treatment procedures in children with chronic constipation. For over three years 70 children with defecation problems have been referred to the Department of Pediatric Surgery in Katowice (Poland). As a result of diagnostic procedures (anorectal manometry, barium enema and rectal biopsy) Hirschsprung disease was diagnosed in 21 cases and chronic constipation in 49. Children with Hirschsprung disease were treated surgically. Children with diagnosed chronic constipation were treated conservatively (cleansing enemas, oral laxatives, diet) and good results were obtained in 30 patients. 19 children underwent anal divulsion and 16 improved. In 2 cases sphincter myotomy was performed and good result was obtained in both of them. The diagnostics and treatment of chronic constipation in pediatric surgical ward is advantageous, because: Hirschsprung disease can be excluded, cleansing of colon sometimes requires general anesthesia and in selected cases surgical procedure is necessary.

Diagnosis and therapy of ultrashort Hirschsprung's disease.

BACKGROUND: Although ultrashort Hirschsprung's disease (UHD) was enzyme-histochemically characterised about 35 years ago, its existence is still often ignored. The aim of this study is to summarise the clinical diagnostic, incidence, gender ratio, morphological characteristics, and therapy over 15 years. METHODOLOGY: The reliable diagnosis of suspected UHD requires a minimal enema of contrast medium to exclude Hirschsprung's disease (HD). In UHD during pressing or crying no reflux of contrast medium is observed. Final proof of UHD is an enzyme-histochemical biopsy examination of distal rectal mucosa. The biopsies must demonstrate submucosa and be taken from the dentate line and 1 cm, 2 cm, 4 cm and 6 cm above the dentate line. The cryostat sections must be cut 15 microm thick; this thickness is reduced to 4.5 microm by the thawing, spreading and drying of the sections on microscope slides. A reliable diagnosis of UHD needs an enzyme-histochemical acetylcholinesterase reaction of native sections of rectal mucosa. RESULTS: UHD develops with first symptoms of chronic constipation in the second half of the first year of life. The chronic constipation proves to be therapy resistant. In HD constipation occurs in the first weeks of life or after weaning. In contrast to HD, no nerve fibres with increased AChE activity are observed in the lamina propria mucosa. Nets of nerve fibres with increased AChE activity can be found only in the muscularis mucosa and the musculus corrugator cutis ani (MCCA). The therapy of choice has proven to be a partial myectomy of the distal internal sphincter if dilatation of the internal sphincter was ineffective. UHD is either limited to the anal ring, or extends 3 - 4 cm into the distal rectum. Over the past 15 years, UHD had in our series an incidence of 13.4 % of all aganglionoses. The gender ratio of girls to boys was 1 : 2. CONCLUSION: UHD is reliably diagnosed by an AChE reaction in native biopsy sections from the anocutaneous transitional zone and, potentially, from 3 - 4 cm above the pectinate line. As UHD is always accompanied by aganglionosis of the distal internal sphincter, an increase in AChE activity is observed in the nerve fibres of the MCCA. The therapy of choice is a partial myectomy of the distal internal sphincter.

[Hirschsprung in children over 6 years of age]. / Hirschsprung en niños mayores de seis años.

INTRODUCTION: Hirschsprung's disease or colonic agangliosis is a rare condition in schoolchildren and adolescents. It is common in infants and chronic constipation is the usual symptom. MATERIALS, METHODS, AND RESULTS: In a period of 5 years, 11-years-old patients were diagnosed and treated for Hirschsprung's disease. We studied six male patients; mean age was 8.6 years. All patients had classic disease and nine, severe chronic constipation, and two were admitted with encopresis. One had 10 kg of feces in 40 cm of colon. Diagnosis was established with barium enema and tissue biopsy. All patients had colostomy. In eight patients, Duhamel procedure was performed with good results. CONCLUSIONS: This disease is a rare condition in older patients and Duhamel procedure is a good surgical option.

Functional ileus in neonates: Hirschsprung's disease-allied disorders versus meconium-related ileus.

Sixty-eight neonates with functional ileus were reviewed. Twelve required laparotomy; in seven, histological studies revealed decreased ganglia and ganglion cells of the myenteric plexus (MP) (Group A), and in five, MP was normal (Group B). In the remaining 56 cases, obstructive symptoms were relieved following conservative therapy (Group C). All Group A cases except one had normal birth weight, while Group B and C cases showed significantly lower birth weights. A marked caliber change of the small intestine and/or small-caliber distal intestine with meconium stagnation in the proximal intestine was commonly demonstrated at operation in Group A and B, or on contrast enema in Group C. Four Group A cases died of enteritis, and three survivors suffered from prolonged obstructive symptoms. The grade of histological abnormality of MP correlated with the clinical outcome. In Group B, three died of sepsis shortly after surgery, but two survivors have been free from symptoms. Group A can be categorized as Hirschsprung's disease-allied disorders (HAD). Group B and C can be categorized as meconium-related ileus (MRI). The similarity of the macroscopic findings of HAD and MRI, and the occurrence of MRI exclusively in low birth weight neonates, strongly suggest that functional immaturity of MP plays a role in the etiology of MRI.

Clinical significance and application of anorectal physiology.

During the last decade, interest in anorectal disorders has increased continuously. This review presents the results of Hungarian authors' contributions to a better understanding of anorectal physiology over the last 10 years. It has been demonstrated that the surgical technique of pelvic floor reconstruction can be refined and surgical complication rates reduced through the analysis of anal basal pressure components and exploration of internal anal sphincter function. Objective assessment of anal continence (distinguishing continent from incontinent patients) is a consistent problem in clinical practice. The balloon-retaining test, a special application of manometry, facilitates evaluation of anorectal function in incontinent patients. Constipation is an extremely common symptom. Surgical treatment of these ailments should not be regarded as a first-choice therapy. Disordered defecation due to anismus can be identified by defecometry, and can eventually be treated by biofeedback training. The data presented here demonstrate the enormous impact of sophisticated diagnostic techniques on the therapeutic options in treatment of anorectal diseases.