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Pediatric Surgery International Feb 2024Hirschsprung's disease (HD) may result in an impaired quality of life (QoL) due to bowel problems, postoperative complications and other health-related issues. The...
BACKGROUND
Hirschsprung's disease (HD) may result in an impaired quality of life (QoL) due to bowel problems, postoperative complications and other health-related issues. The Hirschsprung and Anorectal Malformation Quality of Life (HAQL) questionnaire is a disease-specific instrument developed in the Netherlands to measure the QoL in patients with HD and anorectal malformations. The aim of this study was to translate, culturally adapt and validate HAQL in a Danish Hirschsprung population.
MATERIAL AND METHODS
Translation and cultural adaptation were performed according to international guidelines. Invitations to participate in the validation were sent to 401 patients operated for HD during the period from 1985 to 2012. A total of 156 patients completed the translated and culturally adapted Danish versions of HAQL and 35 parents of children and adolescents completed the corresponding parent questionnaire. Reliability was evaluated in terms of internal consistency using Cronbach's α and test-retest reliability using Intraclass Correlation Coefficient for the retest step. Known groups comparison was performed with comparison of mild HD (defined as recto-sigmoidal HD) and serious HD (defined as more proximal disease).
RESULTS
The internal consistency of the dimensions was overall satisfactory for adults and adolescents but more problematic for children, where Cronbach's α was less than 0.7 in 60% of the dimensions. For both children and adolescents, the α-value was unsatisfactory for social functioning, emotional functioning, and body image. The test-retest reliability was overall good. The known groups comparison was only able to demonstrate a significant difference between mild and severe HD within one dimension.
CONCLUSIONS
The translated version of the HAQL questionnaires provides an overall reliable instrument for evaluating disease-specific QoL in a Danish HD population, but it is important to acknowledge the limitations of the questionnaire, especially in children and adolescents.
Topics: Adult; Child; Adolescent; Humans; Quality of Life; Anorectal Malformations; Reproducibility of Results; Surveys and Questionnaires; Hirschsprung Disease; Denmark
PubMed: 38334791
DOI: 10.1007/s00383-024-05634-y -
Diagnostic Pathology Feb 2024Differences in the preparation, staining and scanning of digital pathology slides create significant pre-analytic variability. Algorithm-assisted tools must be able to...
BACKGROUND
Differences in the preparation, staining and scanning of digital pathology slides create significant pre-analytic variability. Algorithm-assisted tools must be able to contend with this variability in order to be applicable in clinical practice. In a previous study, a decision support algorithm was developed to assist in the diagnosis of Hirschsprung's disease. In the current study, we tested the robustness of this algorithm while assessing for pre-analytic factors which may affect its performance.
METHODS
The decision support algorithm was used on digital pathology slides obtained from four different medical centers (A-D) and scanned by three different scanner models (by Philips, Hamamatsu and 3DHISTECH). A total of 192 cases and 1782 slides were used in this study. RGB histograms were constructed to compare images from the various medical centers and scanner models and highlight the differences in color and contrast.
RESULTS
The algorithm was able to correctly identify ganglion cells in 99.2% of cases, from all medical centers (All scanned by the Philips slide scanner) as well as 95.5% and 100% of the slides scanned by the 3DHISTECH and Hamamatsu brand slide scanners, respectively. The total error rate for center D was lower than the other medical centers (3.9% vs 7.1%, 10.8% and 6% for centers A-C, respectively), the vast majority of errors being false positives (3.45% vs 0.45% false negatives). The other medical centers showed a higher rate of false negatives in relation to false positives (6.81% vs 0.29%, 9.8% vs 1.2% and 5.37% vs 0.63% for centers A-C, respectively). The total error rates for the Philips, Hamamatsu and 3DHISTECH brand scanners were 3.9%, 3.2% and 9.8%, respectively. RGB histograms demonstrated significant differences in pixel value distribution between the four medical centers, as well as between the 3DHISTECH brand scanner when compared to the Philips and Hamamatsu brand scanners.
CONCLUSIONS
The results reported in this paper suggest that the algorithm-based decision support system has sufficient robustness to be applicable for clinical practice. In addition, the novel method used in its development - Hierarchial-Contexual Analysis (HCA) may be applicable to the development of algorithm-assisted tools in other diseases, for which available datasets are limited. Validation of any given algorithm-assisted support system should nonetheless include data from as many medical centers and scanner models as possible.
Topics: Humans; Hirschsprung Disease; Image Processing, Computer-Assisted; Algorithms; Microscopy
PubMed: 38321431
DOI: 10.1186/s13000-024-01452-x -
Pediatric Surgery International Feb 2024The purpose of surgeries performed for congenital anomalies in children is to increase the survival rates and provide a developmental comparison to that of their peers.
BACKGROUND
The purpose of surgeries performed for congenital anomalies in children is to increase the survival rates and provide a developmental comparison to that of their peers.
AIM
The objective of this study was to investigate the development of children following surgery for congenital anomalies and the risk factors affecting their development.
METHODS
Our study included 33 children who underwent surgery for gastrointestinal anomalies in our clinic between 2011 and 2016, and did not have any syndrome, chromosomal abnormality, or additional abnormality. Developmental levels were evaluated using the Ages and Stages Questionnaire (ASQ) and the ASQ: Social-Emotional (ASQ: SE) scales adapted for the use on Turkish children. Data on patient history were obtained retrospectively from patient files.
RESULTS
The study included 33 patients, including 11 with esophageal atresia, 6 with intestinal atresia, 11 with anorectal malformation, and 5 with Hirschsprung's disease. Developmental delay was found in the ASQ of 72.7% of the patients and the ASQ: SE tool was 27% of the patients. The rate of patients with scores below the threshold from each parameter of ASQ was higher than that of the normal population (p < 0.05). Development delay was detected using the ASQ scale in 100% of those with microcephaly at birth, in 91% of premature infants born between 1500 and 2500 g, and in 83.3% of those with low birth weight to gestational age.
CONCLUSIONS
In children who underwent surgery due to congenital anomalies, an evaluation through developmental tests, a post-surgical follow-up process, and a referral to the relevant disciplines when necessary may increase the success of surgery as well as increase the life quality of the patient.
Topics: Infant, Newborn; Child; Infant; Humans; Retrospective Studies; Esophageal Atresia; Hirschsprung Disease; Anorectal Malformations; Ambulatory Care Facilities
PubMed: 38300305
DOI: 10.1007/s00383-023-05625-5 -
Cureus Jan 2024Patients with inflammatory bowel disease can present with numerous infectious complications, including intra-abdominal abscess, perforations of the intestine, fistula...
Patients with inflammatory bowel disease can present with numerous infectious complications, including intra-abdominal abscess, perforations of the intestine, fistula formation, and the occurrence of septicemia. Toxic megacolon (TM) is a potentially fatal complication of inflammatory bowel disease (IBD) and is associated with high morbidity and mortality. In this case report, we report a 49-year-old male patient who was admitted to the intensive care unit for the management of severe sepsis that was secondary to an inaugural toxic megacolon complicating a silent inflammatory bowel disease, with a Lichtiger score of 11. Nonresponse to anti-bacterial therapy, noradrenaline, and intravenous corticosteroid therapy required an emergency total colectomy. After surgery, the patient died because of his unresolved septic shock. Correct management of this condition requires an accurate assessment of the patient's history, a correct physical examination, abdominal radiographs, and sigmoid coloscopy, and frequently requires surgery. The indications for surgery in cases of toxic megacolon, massive hemorrhage, perforation, peritonitis, or non-response to medical therapy are the most important ones. Patients with a history of inflammatory bowel disease are particularly prone to infectious complications since therapy for these inflammatory diseases is based on the use of immunosuppressive drugs and frequent abdominal surgeries.
PubMed: 38298320
DOI: 10.7759/cureus.51459 -
Chinese Medical Journal Mar 2024
Topics: Humans; Hirschsprung Disease; Consensus; China
PubMed: 38297440
DOI: 10.1097/CM9.0000000000003010 -
European Journal of Pediatric Surgery :... Apr 2024Patient-reported outcome measures (PROMs) can be employed in both research and clinical care to enhance our understanding of outcomes that matter to patients. This... (Review)
Review
A Narrative Review of Patient-Reported Outcome Measures and Their Application in Recent Pediatric Surgical Research: Advancing Knowledge and Offering New Perspectives to the Field.
INTRODUCTION
Patient-reported outcome measures (PROMs) can be employed in both research and clinical care to enhance our understanding of outcomes that matter to patients. This narrative review aims to describe PROM use in recent pediatric surgical research, identify and describe psychometrically robust PROMs, providing an overview of those derived from pediatric patient input, and make recommendations for future research.
MATERIALS AND METHODS
A search was conducted to identify articles published from 2021 to August 2023 describing the availability and/or use of at least one valid or reliable PROM in children with conditions including anorectal malformations, biliary atresia, congenital diaphragmatic hernia, duodenal atresia, esophageal atresia, abdominal wall defects, Hirschsprung's disease, sacrococcygeal teratoma, and short bowel syndrome. Articles were categorized based on their objectives in applying PROMs. Psychometrically robust PROMs were identified and described.
RESULTS
Out of the 345 articles identified, 49 met the inclusion criteria. Seventeen focused on esophageal atresia and 14 on Hirschsprung's disease. Twenty-nine PROMs were identified, with 12 deemed psychometrically robust. Seven psychometrically robust PROMs were developed using patient input in the primary item generation. Most PROMs were applied to advance understanding of conditions and/or treatment and fewer were developed or psychometrically evaluated. No PROMs were assessed for their impact or incorporated into an implementation study.
CONCLUSIONS
This review reveals gaps in the application of PROMs in recent pediatric surgical research. Emphasis should be placed on the development and utilization of psychometrically robust PROMs, broadening the scope of covered diseases, conducting impact assessments, and evaluating implementation strategies.
Topics: Humans; Child; Quality of Life; Hirschsprung Disease; Patient Reported Outcome Measures; Short Bowel Syndrome
PubMed: 38272041
DOI: 10.1055/s-0043-1778108 -
African Journal of Paediatric Surgery :... Jan 2024The authors report their experience of the transanal endorectal pull through technique as described by De la Torre Mondragon in the treatment of Hirschsprung's disease... (Observational Study)
Observational Study
BACKGROUND
The authors report their experience of the transanal endorectal pull through technique as described by De la Torre Mondragon in the treatment of Hirschsprung's disease (HD).
MATERIALS AND METHODS
It was a retro prospective, observational and descriptive hospital based study involving all proven cases of HD managed within a time frame of 8 years.
RESULTS
Fifty two patients with a mean age of 18 months at the time of surgery. The male pre dominance was remarkable. The mean duration of the surgery was 2 h and a half hours. The surgical indication was based on the history, clinical findings and on the contrast enema (transition zone) with a 24 h film (prolonged contrast evacuation) and calculation of the rectosigmoid index (<1). With a mean follow up of 16 months, the morbidity was dominated by soiling, anastomotic strictures and enterocolitis. The mortality in one case was related to a post operative enterocolitis that was not amenable to resuscitation.
CONCLUSION
Soiling, anastomotic strictures and enterocolitis are the main post operative complications of TEPT in the treatment of HD in our practice.
Topics: Humans; Infant; Male; Constriction, Pathologic; Enterocolitis; Hirschsprung Disease; Prospective Studies; Female
PubMed: 38259017
DOI: 10.4103/ajps.ajps_44_22 -
African Journal of Paediatric Surgery :... Jan 2024Transanal endorectal pull-through (TERPT) has become one of the preferred treatments for Hirschsprung's disease (HD) in our setting. This report aims to evaluate the...
BACKGROUND
Transanal endorectal pull-through (TERPT) has become one of the preferred treatments for Hirschsprung's disease (HD) in our setting. This report aims to evaluate the current outcome of TERPT in the setting.
MATERIALS AND METHODS
A retrospective review of 71 children who had TERPT for histologically-confirmed HD in 11 years (2006-2017) in Nigeria.
RESULTS
There were 48 boys and 23 girls; aged 3 days-12 years at initial presentation (median = 10 months). Three (4.2%) patients had associated anomalies (duodenal atresia; anorectal malformation and sensorineural deafness with hypopigmented skin patches each). Age at TERPT was 2 months to 15 years (median = 3 years), with surgery waiting time of 1 month-14.9 years (median = 18 months). Sixty-six (93.0%) patients had rectosigmoid, four (5.6%) patients had long segment and one (1.4%) had total colonic disease. Five (7.0%) patients with large megacolon and one (1.4%) with the total colonic disease had assisted abdominal resection of the colon at TERPT. Seventeen (23.9%) patients had post-operative complications, including post-operative enterocolitis 8 (11.3%); anastomotic dehiscence 3 (4.2%); retained aganglionic segment 2 (2.8%); anastomotic stenosis 2 (2.8%), resulting in prolonged hospital stay (P = 0.0001; range = 1-30 days; median = 5 days). The mortality rate was 4.2% (3) from malignant hyperthermia in one patient and in 2 patients, the cause of mortality was unclear. Patients were followed up for 3-6 years (median = 3.5 years). Bowel movement stabilised to 2-4 times daily by 6 weeks after surgery.
CONCLUSION
TERPT is a safe treatment for HD in this setting with good short-term outcomes. Longer follow-up is necessary to further evaluate the long-term bowel movement outcomes.!
Topics: Male; Child; Female; Humans; Hirschsprung Disease; Colonic Diseases; Anastomosis, Surgical; Anorectal Malformations
PubMed: 38259012
DOI: 10.4103/ajps.ajps_93_22 -
BMC Urology Jan 2024Duplication of the bladder with duplication of the posterior urethra is a relatively rare congenital malformation. Cases of sagittal septum duplication of the bladder... (Review)
Review
BACKGROUND
Duplication of the bladder with duplication of the posterior urethra is a relatively rare congenital malformation. Cases of sagittal septum duplication of the bladder with duplication of the posterior urethra have rarely been reported. Furthermore, the combination thereof with congenital megacolon is rare.
CASE PRESENTATION
A 21-year-old male was admitted to our hospital because of frequent urination for two months. He presented to another hospital first with frequent urination and underwent computed tomography (CT) and testicular biopsy. Anti-inflammatory therapy was administered by the doctor to the patient. For further diagnosis and treatment, the patient went to the outpatient department in our hospital on June 6, 2022. After admission, the patient underwent ultrasound, CT, MRI, cystoscopy, and other related examinations and tests. The examination results suggested that the patient had duplication of the bladder with duplication of the posterior urethra. In addition, the patient's mother reported that he had suffered from long-term constipation with abdominal distension before the age of 5 years. At the time, he was admitted to the local hospital and was diagnosed with congenital megacolon based on the relevant examinations. After the patient was diagnosed with duplication of bladder and urethra, the doctor recommended surgical treatment to the patient. However, he considered that he only had frequent urination symptoms, and chose conservative treatment rather than to undergo surgical treatment. Thus, the doctor prescribed anti-inflammatory treatment. Four months later, the patient reported that frequent urination symptoms persisted, and was also considering fertility-related problems. The outpatient follow-up will be continued.
CONCLUSIONS
In this article, we summarize the imaging findings of duplication of the bladder with duplication of the posterior urethra and propose the advantages and disadvantages of each type of imaging examination. We also review the relevant literature on cases of bladders with duplication of the posterior urethra. The related differential diagnosis is summarized, and the significance of guiding clinical treatment and diagnosis is discussed.
Topics: Male; Humans; Child, Preschool; Young Adult; Adult; Urinary Bladder; Urethra; Hirschsprung Disease; Intestines; Anti-Inflammatory Agents
PubMed: 38172803
DOI: 10.1186/s12894-023-01395-3 -
Biomedical Reports Feb 2024infection (CDI) is the most common cause of healthcare-associated diarrhea and among adults, the worldwide incidence rate of the infection is increasing. There is a... (Review)
Review
infection (CDI) is the most common cause of healthcare-associated diarrhea and among adults, the worldwide incidence rate of the infection is increasing. There is a small amount of data in the literature for pediatric patients, but most indicate an increasing trend. is a constituent of the normal microbiota; however, under specific conditions that cause a disruption of the normal bacterial flora, colonization of and the released toxins that cause inflammation and mucosal damage occurs. Risk factors for CDI at any age include hospitalization, exposure to antibiotics, administration of proton pump inhibitors, invasive mechanical ventilation, immunosuppression and presence of associated comorbidities. Clinical manifestations range from asymptomatic colonization to fulminant disease characterized by toxic megacolon, intestinal perforation and, rarely, death. The aim of the present review was to outline the features of CDI in pediatric patients.
PubMed: 38169799
DOI: 10.3892/br.2023.1706