Effect of Laparoscopic Soave Combined with Deloyers Turnover on the Efficacy and Prognosis of Children with Congenital Hirschsprung's Disease.

Objective: To analyze the effect of laparoscopic Soave combined with Deloyers turnover on the efficacy and prognosis of children with congenital Hirschsprung's disease, and to explore an effective and safe operation, so as to provide a reference for clinical development of treatment plan and promote the faster recovery of children. Methods: A total of 80 children with Hirschsprung's disease admitted to our hospital from July 2021 to June 2022 were selected and included in the traditional group and minimally invasive group according to different surgical procedures, with 40 cases in each group. The traditional group was treated with open Soave, and the minimally invasive group was treated with laparoscopic Soave combined with Deloyers reversal. Compared two groups in terms of operation indicators (operation time, intraoperative blood loss, fasting time, intestinal function recovery time, and hospital stay), the stress response (serum cortisol, heart sodium, plasma epinephrine, and norepinephrine), intestinal flora (Bifidobacterium, Lactobacillus, Escherichia coli, and Enterococcus faecalis), anal function, recent complications (urinary retention, hematochezia, anus week dermatitis, incision infection, and abdominal bleeding), long-term complications (constipation, anastomotic stenosis, enterocolitis, and dirty feces). Results: The operation time, intraoperative blood loss, fasting time, intestinal function recovery time, and hospital stay in the minimally invasive group were significantly shorter than those in the traditional group (P < .05). The levels of serum cortisol, atrial natriuretic peptide, plasma epinephrine, and norepinephrine in the minimally invasive group were lower than those in the traditional group (P < .05). The levels of Bifidobacterium and Enterococcus faecalis in the minimally invasive group were higher than those in the traditional group (P < .05). The excellent and good rate of anal function in the minimally invasive group was higher than that in the traditional group (P < .05). The incidence of short-term and long-term complications in the minimally invasive group was lower than that in the traditional group (P < .05). Conclusion: Joint Deloyers flip Soave under laparoscopic surgery for children with congenital Hirschsprung disease has a better curative effect, with shorter operation time, less blood loss compared to traditional open surgery.

Adult Hirschsprung's disease presenting as chronic constipation: a case report.

BACKGROUND: Hirschsprung's disease is a congenital disorder identified by the absence of ganglion cells at the Meissner's plexus of the submucosa and Auerbach's plexus of the muscularis. This disease can be found in approximately 1 in 5000 live births. It is a congenital disorder that is rarely diagnosed in adults, where 95% of cases are diagnosed in infants aged under 1 year old. Here we present a rare case of adult Hirschsprung's disease to enrich the body of knowledge  in diagnosing adult patients with chronic refractory constipation symptoms. CASE REPORT: An 18-year-old Indonesian woman came to the general surgery department of Unggul Karsa Medika Teaching Hospital with a defecating problem (constipation) since childhood. There was no history of her passage of meconium. A contrast enema study showed that the sigmoid colon was dilated and the rectum was narrowed, with rectosigmoid index < 1. With these findings, it was suspected that the patient may have ultra-short segment Hirschsprung's disease. The patient was then referred to the digestive surgery department of referral hospital for surgical treatment. CONCLUSION: In adult patients presenting with history of constipation since childhood, it is necessary to consider the possibility of Hirschsprung's disease that was not diagnosed in early childhood. Hirschsprung's disease in adults is usually a short or ultra-short aganglionic segment because it shows relatively mild symptoms. Surgical removal of the aganglionic segment of the gut is the definitive treatment for Hirschsprung's disease.

Non-standard approach to infants and children with megacolon: laparotomy and endorectal pull-through for diagnosis and treatment in difficult countries with low resources in a non-profit setting: return to the past Soave's ERPT.

BACKGROUND: Hirschsprung's disease is an important cause of pediatric constipation with high risk of bacterial enterocolitis. Its diagnosis is histological and the suction biopsy is the gold standard. In resource-limited countries, the main diagnostic exam is the contrast enema and mini-invasive surgery lacks. We present the management of a cohort of patients with megacolon in Haiti, a low-resource country. METHODS: Children with megacolon and fecal impaction admitted at St Damien Children Hospital in Port-Au-Prince in June, August and December 2017 were included. We considered only patients with an evident transition zone on contrast enema who underwent endorectal pull-through (ERPT). Short term complications were recorded. RESULTS: Twenty children with clinical megacolon were admitted, eleven were included in the study. No suction rectal biopsy and intraoperative histological evaluation were performed. In ten children a Soave ERPT with anastomosis at 5POD was performed, in the other case a Boley primary anastomosis was preferred. One patient complicated with a peritonitis. No major complications were recorded. Colostomy was not considered a good option. CONCLUSIONS: In developing countries, Soave ERPT with definitive anastomosis after few days could be considered a valid option. Colostomy is suggested only in case of scant general conditions or bad colon appearance.

Functional constipation refractory to medical management: The colon is the problem.

BACKGROUND: Management of refractory constipation in children has not been standardized. We propose a protocolized approach which includes a contrast enema, anorectal manometry and exclusion of Hirschsprung disease (HD). For those without HD or with normal sphincters, an assessment of the colonic motility may be needed. The subgroups of dysmotility include (1) slow motility with contractions throughout, (2) segmental dysmotility (usually the sigmoid), or (3) a diffusely inert colon. We offered a Malone appendicostomy in all groups with the hope that this would avoid colonic resection in most cases. METHODS: Patients with medically refractory constipation were reviewed at a single institution (2020 to 2021). For patients without HD or an anal sphincter problem, assessment of colonic motility using colonic manometry was performed followed by a Malone appendicostomy for antegrade flushes. RESULTS: Of 196 patients evaluated for constipation refractory to medical management, 22 were felt to have a colonic motility cause. These patients underwent colonic manometry and Malone appendicostomy. 13 patients (59%) had a slow colon but with HAPCs throughout, 5 (23%) had segmental dysmotility, and 4 (18%) had a diffuse colonic dysmotility. 19 (86%) responded well to antegrade flushes with 17 reporting no soiling and 2 having occasional accidents. 3 patients (14%) failed flushes and underwent a colon resection within 6-month following Malone procedure. CONCLUSION: We propose a protocol for medically refractory constipation which provides a collaborative framework to standardize evaluation and management of these patients with antegrade flushes, which aids in avoidance of colonic resection in most cases. LEVEL OF EVIDENCE: Level IV.

Postoperative hyperthermia-induced multiple organ failure in a child with Down syndrome: a case report.

BACKGROUND: Psychological stress has been reported to cause hyperthermia. Persistent excessive hyperthermia can, in turn, cause hypercytokinemia and organ damage. We report a case of postoperative severe hyperthermia leading to a systemic inflammatory response and multiple organ failure in a child with Down syndrome. CASE PRESENTATION: A 10-month-old native Japanese boy with Down syndrome and Hirschsprung's disease is described. Newborn screening showed congenital hypothyroidism and a ventricular septal defect, but these conditions were stable upon administration of levothyroxine and furosemide. His development was equivalent to that of a child with Down syndrome. He developed a noninfectious high fever twice after preoperative preparations at age 8 months and again at 9 months. He was readmitted to hospital at age 10 months to undergo the Soave procedure to correct Hirschsprung's disease. However, he contracted a fever immediately after the surgical procedure. Hyperthermia (42 °C) was refractory to acetaminophen treatment and deteriorated to multiple organ failure due to hypercytokinemia, with increased serum levels of interleukin-6 (44.6 pg/mL) and interleukin-10 (1010 pg/mL). He died on postoperative day 2 with hypoxemia, respiratory/metabolic acidosis, increased serum levels of transaminases, reduced coagulation, and pancytopenia. Various infectious and noninfectious causes of hyperthermia could not be identified clearly by culture or blood tests. CONCLUSIONS: We speculated that the proximate cause of the fever was psychological stress, because he suffered repeated episodes of hyperthermia after the invasive procedure. Hyperthermia, together with the immune-system disorders associated with Down syndrome, may have induced hypercytokinemia and multiple organ failure. This rare case of noninfectious postoperative hyperthermia leading to multiple organ failure may help to shed further light on the currently unclear pathogenic mechanism of hyperthermia and associated multiple organ failure during the perioperative period in children.

Hirschsprung's disease presenting as intractable anemia: a report of two cases and review of the literature.

BACKGROUND: This report summarizes the clinical characteristics of intractable anemia as part of the clinical presentation of Hirschsprung's disease (HD) and aims to strengthen clinicians' ability to recognize early signs of HD. CASE PRESENTATION: An 11-year-old boy with a 6-year history of intractable anemia, low hemoglobin level (55 g/L), poor response to oral iron supplementation and blood transfusion, and difficulty with defecation was diagnosed with HD. A 19-month-old boy with a 3-month history of intractable anemia, low hemoglobin level (64 g/L), poor response to oral iron supplementation and blood transfusion, delayed meconium passage, and history of intestinal obstruction was also diagnosed with HD. Both patients underwent surgery, after which anemia was corrected effectively in both cases. Two more cases of intractable anemia as the chief complaint and diagnoses of HD over different durations since the onset of anemia (ranging from 1.7 years to 21 years) were identified in a literature search. Both patients underwent surgery, after which anemia was corrected. CONCLUSIONS: Intractable anemia as part of the clinical presentation of HD is extremely rare. Detailed inquiries of medical histories and physical examinations are key to early diagnoses and preventing misdiagnoses. Anemia in HD patients may primarily be caused by impaired iron absorption due to HD.

Predictability of 48-h delayed retention of contrast in barium enema in cases of chronic constipation.

INTRODUCTION: Diagnosis of Hirschsprung's disease depends on rectal biopsy. This study was designed to find an alternate diagnostic modality to exclude Hirschsprung's disease. AIM: The aim of this study was to find the predictive value of delayed retention of contrast in excluding Hirschsprung's disease. MATERIALS AND METHODS: All cases of chronic constipation presenting during the study duration from June 2014 to June 2016 were included. Those without any obvious history of conservative management were excluded. Parameters considered in barium enema were initial film, routine films, and delayed retention of contrast at 24, 48, and 72 h. They were then subjected to rectal biopsy. The results of rectal biopsy and barium enema were analyzed. RESULTS: One hundred and thirty-eight patients presented during the study duration. One hundred and twenty-eight formed the study group. The average age of presentation was 48 months (range, 1-144). The average duration of prior medical management was 8 months (range, 6-48 months). Forty-two cases were diagnosed as Hirschsprung's disease on rectal biopsy. The symptoms resolved in 31 cases after rectal biopsy and 42 cases after definitive surgery. In the remaining 55 cases, dietary modification along with laxatives was instituted, and they were kept under follow-up. The average follow-up was 12 months (range, 6-48 months). Of the various parameters in barium enema, delayed retention of contrast at 48 h had the highest negative predictive value of 99.67%. CONCLUSION: Delayed retention of contrast at 48 h has the highest negative predictive value in excluding Hirschsprung's disease. This can safely be used to exclude Hirschsprung's disease in cases of chronic constipation.

Evaluating the Alimentary and Respiratory Tracts in Health and disease (EARTH) research programme: a protocol for prospective, longitudinal, controlled, observational studies in children with chronic disease at an Australian tertiary paediatric hospital.

INTRODUCTION: Chronic gastrointestinal and respiratory conditions of childhood can have long-lasting physical, psychosocial and economic effects on children and their families. Alterations in diet and intestinal and respiratory microbiomes may have important implications for physical and psychosocial health. Diet influences the intestinal microbiome and should be considered when exploring disease-specific alterations. The concepts of gut-brain and gut-lung axes provide novel perspectives for examining chronic childhood disease(s). We established the 'Evaluating the Alimentary and Respiratory Tracts in Health and disease' (EARTH) research programme to provide a structured, holistic evaluation of children with chronic gastrointestinal and/or respiratory conditions. METHODS AND ANALYSIS: The EARTH programme provides a framework for a series of prospective, longitudinal, controlled, observational studies (comprised of individual substudies), conducted at an Australian tertiary paediatric hospital (the methodology is applicable to other settings). Children with a chronic gastrointestinal and/or respiratory condition will be compared with age and gender matched healthy controls (HC) across a 12-month period. The following will be collected at baseline, 6 and 12 months: (i) stool, (ii) oropharyngeal swab/sputum, (iii) semi-quantitative food frequency questionnaire, (iv) details of disease symptomatology, (v) health-related quality of life and (vi) psychosocial factors. Data on the intestinal and respiratory microbiomes and diet will be compared between children with a condition and HC. Correlations between dietary intake (energy, macro-nutrients and micro-nutrients), intestinal and respiratory microbiomes within each group will be explored. Data on disease symptomatology, quality of life and psychosocial factors will be compared between condition and HC cohorts.Results will be hypothesis-generating and direct future focussed studies. There is future potential for direct translation into clinical care, as diet is a highly modifiable factor. ETHICS AND DISSEMINATION: Ethics approval: Sydney Children's Hospitals Network Human Research Ethics Committee (HREC/18/SCHN/26). Results will be presented at international conferences and published in peer-reviewed journals. TRIAL REGISTRATION NUMBER: NCT04071314.

Should we look for Hirschsprung disease in all children with meconium plug syndrome?

BACKGROUND: Meconium plug syndrome (MPS) is associated with Hirschsprung disease (HD) in 13-38% of cases. This study sought to assess institutional variation in utilization of rectal biopsy in children with MPS and the likelihood of diagnosing HD in this population. METHODS: Patients with MPS on contrast enema in the first 30 days of life from the Pediatric Health Information System database in 2016-2017 were included. Institutional rates of rectal biopsies performed during the initial admission were calculated and then used to predict institutional rates of early HD diagnoses using Poisson regression. RESULTS: Of 373 newborns with MPS, 106 (28.4%) underwent early rectal biopsy, of whom 43 (40.5%) had HD. Fifty-seven (15.3%) were ultimately diagnosed with HD. Eight (14%) of these patients had a delayed diagnosis. HD rates between institutions did not differ significantly (range 0-50%, p=0.52), but usage of early rectal biopsy did (range 0-80%, p=0.03). Each additional early biopsy increased the early HD diagnosis rate by 35% (ß=0.30, 95% CI 0.15-0.45, p<0.0001). CONCLUSION: The incidence of HD is increased in children with MPS. There is significant hospital variability in the utilization of early rectal biopsy, and opportunity exists to standardize practice. TYPE OF STUDY: Study of Diagnostic test Level of Evidence: Level III.

Controlled outcome of Hirschsprung's disease beyond adolescence: a single center experience.

PURPOSE: The aim of this study was to assess the function and quality of life of Hirschsprung's Disease (HD) beyond adolescence and relate it to matched controls. METHODS: All 203 patients diagnosed with HD at our department from 1961 to 1995 were identified. 21 had died, 43 had unclear diagnosis and 16 could not be traced. The remaining 123 patients were sent bowel function and SF-36 quality of life questionnaires. 69 patients (mean age 37.8, range 22-58, 13 female) responded and were matched with 138 age and sex-matched controls. RESULTS: Function: HD-patients had significantly higher number of bowel movements per week, higher incidence of soiling, urgency, permanent stomas, use of laxatives, enemas and loperamide. HD-patients also scored significantly lower in their satisfaction with their bowel function. There was, however, no significant difference in Miller Incontinence score. QOL: HD-patients reported a significantly higher incidence of negative impact by their bowel function on daily life, social interaction and ability to go on vacation. There were no significant differences in SF-36-scores. CONCLUSIONS: Bowel function has a lifelong negative impact on the lives of patients with HD. This strongly indicates a need for structured follow-up beyond adolescence.

How to establish a successful bowel management programme in children: a tertiary paediatric centre experience.

BACKGROUND: Bowel management is a term used to describe a medical approach to the management of faecal incontinence. AIMS: To present the outcomes of an individualised bowel management programme developed by a tertiary paediatric centre and evaluate contributory factors for successful bowel management in children. METHODS: A retrospective review of children attending a bowel management clinic in a tertiary centre in Dublin, Ireland, over 5 years (2010-2015). The main outcome measure was the ability to achieve a regular bowel pattern and remain socially clean. Multiple linear regression analyses were used to determine the factors contributing to successful bowel management. RESULTS: One hundred ninety-two children attended the clinic over 5 years. The median age at commencement of washouts was 7 years. Underlying diagnosis was spina bifida in 50%, imperforate anus in 17.7%, Hirschsprung's disease in 14.6%, idiopathic constipation in 7.8%, and other conditions in 9.9% of patients. Children with spina bifida and Hirschsprung's disease preferred Peristeen washouts, while those with imperforate anus and idiopathic constipation preferred Willis washouts (p < 0.001). Our programme was successful in 93.7% of cases. Regression analysis showed that the underlying condition (p < 0.001), washout medication (p = 0.016), and individuals administering washouts (p < 0.001) contributed to a successful bowel management programme. CONCLUSION: Treatment protocols should be individualised based on the underlying condition and outcomes. Decision-making must be cognisant of the physical, social, psychological, and developmental needs of the child and family. A partnership approach is advocated, which includes child and parent/carer preferences allowing them to make an informed decision.

Air test as a simple method of screening for Hirschsprung's disease.

AIM: To present the technique and the diagnostic accuracy of the air test to diagnose Hirschsprung's disease (HD). MATERIALS AND METHODS: Children who attended hospital for chronic constipation (CC) between January 2012 and December 2016 for whom the air test was performed were enrolled. The test was conducted during contrast enema under fluoroscopic observation using 20-50 ml injections of air into the rectum through a 10 F Nelaton catheter. The demographics, results of the air test, and additional examinations, as well as the outcomes of subsequent treatments were analysed retrospectively. RESULTS: The air test was conducted in 179 patients (median: 3 years, range: 0-14 years), and was positive in 150 and negative in 29 cases. Of the 29 patients with negative results, four were diagnosed with HD by rectal suction biopsy (RSB). Of the remaining 25 patients, RSB was conducted in seven and HD was excluded in all cases. In all 150 patients with positive air test results, CC was adequately controlled with conservative treatment. The sensitivity and specificity of the air test were 100% (4/4) and 85.7% (150/175), respectively. CONCLUSIONS: The air test can be used as a new non-invasive screening method for HD, performed simultaneously with contrast enema.

Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung disease.

Although most children with Hirschsprung disease ultimately do well, many experience a variety of ongoing problems after pull-through surgery. The most common include obstructive symptoms, soiling, enterocolitis and failure to thrive. The purpose of this guideline is to present a rational approach to the management of postoperative obstructive symptoms in children with Hirschsprung disease. The American Pediatric Surgical Association Board of Governors established a Hirschsprung Disease Interest Group. Group discussions, literature review and expert consensus were then used to summarize the current state of knowledge regarding causes, methods of diagnosis, and treatment approaches to children with obstructive symptoms following pull-through for Hirschsprung disease. Causes of obstructive symptoms post-pull-through include mechanical obstruction; persistent or acquired aganglionosis, hypoganglionosis, or transition zone pull-through; internal sphincter achalasia; disordered motility in the proximal intestine that contains ganglion cells; or functional megacolon caused by stool-holding behavior. An algorithm for the diagnosis and management of obstructive symptoms after a pull-through for Hirschsprung disease is presented. A stepwise, logical approach to the diagnosis and management of patients experiencing obstructive symptoms following pull-through for Hirschsprung disease can facilitate treatment. Level of evidence V.

A comparative study of transcutaneous interferential electrical stimulation plus behavioral therapy and behavioral therapy alone on constipation in postoperative Hirschsprung disease children.

PURPOSE: We assessed the effectiveness of transcutaneous interferential (IF) electrical stimulation on constipation in postoperative Hirschsprung's disease (HD) patients. METHODS: Thirty HD children (18 boys and 12 girls) with constipation who had no surgical complication were enrolled and then randomly divided into two treatment groups. The control group underwent only behavioral therapy comprising high fiber diet, hydration, toilet training and pelvic floor muscles exercises while; the IF group underwent behavioral therapy plus IF electrical stimulation. Patients underwent anorectal manometry before and 6months after the treatment. In addition, a complete bowel diary with data on the frequency of defecation per week, stool form and the number of fecal soiling episodes, a constipation score and a visual pain score were obtained from all patients before, after treatment and 6months later. RESULTS: Constipation symptoms were improved in 10 (66%) and 4 (26.6%) patients in IF and control groups, respectively at 6months of follow up (P<0.03). Frequency of defecation per week significantly increased after the treatment in the IF group compared with control group at the 6months of follow up (5.4±2.1 vs. 3.3±1.8 per week, respectively; P<0.009). In addition, mean pain score was significantly decreased in IF group compared with controls after treatment (P<0.05). CONCLUSION: IF electrical stimulation is an effective adjunct to behavioral therapy to overcome symptoms of constipation in postoperative HD patients.

Enterocolitis and bowel function in children with Hirschsprung disease and trisomy 21.

BACKGROUND/PURPOSE: The purpose of this study was to study the effect of trisomy 21 (T21) on enterocolitis rates and bowel function among children with Hirschsprung disease (HD). METHODS: A retrospective cohort study of patients with HD treated at our tertiary children's hospital (2000-2015) and a cohort of patients with HD treated in our pediatric colorectal center (CRC) (2011-2015) were performed. RESULTS: 26/207 (13%) patients with HD had T21. 70 (41%) with HD alone were diagnosed with enterocolitis episodes compared to 9 (38%) with HD+T21 (p=0.71). 55/207 patients were managed in the CRC. 11/55 patients (20%) had HD+T21. 25 (58%) with HD had one or more enterocolitis episodes compared to 4 (36%) with HD+T21 (p=0.20). Number of hospitalizations for enterocolitis was similar between all groups. Toilet training was assessed in 32 CRC patients (25 HD, 7 HD+T21). One child with HD+T21 was toilet trained by age 4years versus 12 with HD (p=0.20). Laxative or enema therapy was required for constipation management in 57% HD versus 64% HD+T21. CONCLUSION: Enterocolitis rates in children with HD+T21 did not differ from rates in children with HD alone. The majority of patients with CRC follow-up had constipation requiring laxative or enema therapy, which demonstrates the need for consistent postoperative follow-up. LEVEL OF EVIDENCE: Retrospective Study - Level II.

Neonatal sigmoid volvulus.

BACKGROUND: Neonatal sigmoid volvulus is a rare entity. It is associated with Hirschsprung's disease. Presentation is acute abdominal distention, vomiting and obstipation. Abdominal radiograph will show the "coffee bean" sign, but this is frequently missed and the diagnosis requires a high index of suspicion. Treatment options include contrast enema, colonoscopy or laparotomy, depending on the condition of the baby and local availability. POPULATION AND RESULTS: During the last 6years, 6 infants with sigmoid volvulus were treated in our department. Four presented during the first 48h since birth, and 2 presented at the age of 2 and 7weeks of age. One child was operated and 5 had primary contrast enema with radiologic de-volvulus. Rectal biopsy was performed in all cases; three children had Hirschsprung's disease. Those with normal biopsies responded well to rectal washouts. Two patients had early one stage transanal pullthrough and one had 2 further occasions of sigmoid volvulus prior to definitive surgery. All three recovered with an uneventful course. CONCLUSIONS: Neonatal sigmoid volvulus requires a high level of suspicion. Contrast enema is efficient for primary de-volvulus. Rectal biopsy should be performed and if positive for Hirschsprung's disease, surgery should be performed sooner rather than later.

5 years after an ACE: what happens then?

PURPOSE: Antegrade continence enema (ACE) revolutionised the lives of children with chronic constipation and soiling. Parents often ask how long the ACE will be required. We looked at our patients 5 years after ACE formation to answer the question. METHODS: We reviewed clinical notes of all patients undergoing ACE procedure during January 1990 to December 2010. Only patients with >5 years follow-up were included. Data are given as median (range). RESULTS: 133 patients were included with >5 years of follow-up. Primary pathology was anorectal anomaly (ARA) 64 (48%); spinal dysraphism (SD) 40 (30%); functional constipation (FC) 14 (10%); Hirschsprung's Disease (HD) 10 (8%) and others 5 (4%). Median follow-up was 7 years (5-17 years). Overall 74% still use their ACE; whilst 26% no longer access their stoma, of whom 47% recovered normal colonic function. 50% of HD patient recover colonic function. FC has the highest failure rate at 21%. CONCLUSIONS: Overall 86% achieved excellent clinical outcome with 74% of patient still using their ACE at 5 years. HD has the highest recovery rate of 50%. FC has a more unreliable clinical outcome with 21% recovered colonic function and 21% failed. Outcome varied dependent on the background diagnosis.

[Phosphate Intoxication after Application of Enema--a Life-threatening Iatrogenic Complication]. / Phosphatintoxikation nach Klysmagabe--eine lebensgefährliche iatrogene Komplikation.

INTRODUCTION: Enemas are used in pediatric patients with constipation. Retention of phosphate containing enemas with prolonged resorption or reduced renal elimination of phosphate can result in life-threatening hyperphosphatemia with subsequent lethal hypocalcemia and acidosis. CASE PRESENTATION: We report the case of a 6-month-old child who received phosphate-containing enema to treat acute aggravation of constipation. The used enema here was not licensed for this age group. Phosphate intoxication resulted (phosphate 19.87 mmol/l) and presented like a sepsis. Hyperphosphatemia was treated by hemodialysis. A non-diagnosed Hirschsprung disease had led to prolonged resorption of phosphate containing enema and to an ileus and toxic megacolon that had to be operated. CONCLUSION: Insufficient elimination of phosphate containing enema can result in lethal or life threatening hyperphosphatemia, hypocalcemia and metabolic acidosis. These can be treated efficaciously by hemodialysis. Because of the high risk of intoxication in using enemas containing phosphate in infants or in patients with gastrointestinal or renal comorbidities, physicians treating constipation should choose enemas without phosphate but with ingredients with lower risk like glycerol or sorbitol in this age group.

Enfermedad de Hirschsprung asociada al síndrome de Mowat-Wilson: a propósito de un caso / Hirschsprung disease associated with mowat-wilson syndrome: report of a case

Introducción: La enfermedad de Hirschsprung (EH) o megacolon agangliónico es un trastorno congénito que se caracteriza por la ausencia de células ganglionares intramurales de los plexos mioentéricos submucosos (de Auerbach y Meissner, respectivamente) en tramos distales del intestino, debido al fracaso de la migración de los precursores de estas células desde la cresta neural durante el desarrollo embrionario y asociada a otras anomalías en el 18% restante de los casos, en ocasiones formando parte de síndromes polimalformativos específicos. Objetivo: Este artículo tiene como objetivo presentar la evolución clínica y nutricional de un paciente pediátrico de 14 meses diagnosticado de EH al nacer asociado a MWS, así como evaluar los resultados clínicos de este paciente. Métodos: A través de la revisión de la historia clínica, se estudió la evolución de los datos antropométricos (peso y talla) así como los parámetros analíticos para su valoración. Adicionalmente, se evaluaron las complicaciones asociadas al soporte nutricional y la estrategia terapéutica en el contexto multidisciplinar. Resultados: Paciente de 14 meses, sexo masculino, hijo de padres sanos no consanguíneos inmigrantes de Colombia acude al servicio de urgencias de nuestro hospital por presentar distensión abdominal y vómitos con ausencia de deposiciones espontáneas. Descripción de la composición de la nutrición parenteral recibida durante los 28 días que duró su ingreso hospitalario. Conclusión: La asociación de la enfermedad de Hirschsprung y el síndrome de Mowat-Wilson puede conducir a la necesidad de nutrición parenteral de manera prolongada y presentar con mayor frecuencia desvío del estoma produciéndose un mayor número de complicaciones postoperatorias en estos pacientes, tal y como es el caso de nuestro paciente (AU)

Introduction: Hirschsprung’s disease (HD) or aganglionic megacolon is a congenital disorder characterized by the absence of ganglion intramural cells of the submucosal myenteric plexus (namely Auerbach and Meissner, respectively) in distal sections of the intestine. This is due to a failure in the migration of the precursors of these cells from the neural chalk during the embryonic development and also due to other abnormalities associated (18 % of cases), in some cases involving specific polymalformation syndromes. Objectives: The aim of the work is to present the clinical and nutritional evolution of a 14 months aged pediatric patient who was diagnosed with HD since was born associated with Mowat- Wilson syndrome (MWS). In addition, it is also targeted to evaluate the clinical results from this patient. Methods: Reviewing the medical history of the patient, the evolution of the anthropometric data (weight and height) as well as the analytical parameters for further studies were carried out. In addition, the upcoming issues associated with nutritional support and therapeutic strategies in the multidisciplinary context were evaluated. Results: A male, 14 months aged patient, son of not consanguineous healthy immigrants parents from Colombia went to the emergency department of our hospital suffering abdominal distension and vomiting with no spontaneous bowels. A detailed description of the composition of parenteral nutrition administered within the period of 28 days of hospital admission. Conclusion: As observed in this patient, the association of HD and MWS can lead to the need of prolonged parenteral nutrition and frequently present diverting stoma leading to a greater number of postoperative complications in this population (AU)