Anorectal Malformations Patients Research Articles

Attrition rate among patients of Anorectal Malformations on colostomy: A matter of concern

Background: Anorectal malformations (ARM) are correctable congenital malformations with good prognosis. Traditionally it is managed by staged procedure, with diversion colostomy, followed by definitive procedure and stoma reversal in the third stage. It is recommended to complete all stages of repair by 6 months of age for better long-term continence. We had fewer patients coming for definitive surgery and stoma reversal than the patients undergoing colostomy for ARM, so we planned this study to find out the attrition rate among patients undergoing stoma for ARM and explore the reasons for the same. Methods: An observational study was conducted in the Pediatric Surgery department of Chacha Nehru Bal Chikitsalaya, New Delhi. All the case records of patients with ARM who underwent diversion stoma at our center from January 2018 to December 2019 were retrieved. Further follow-up case records were retrieved with the same Unique Health Identification Number (UHID). When the definitive surgery/ stoma closure was not found in the hospital records till December 2022, the parents were contacted telephonically to find out if the children had undergone definitive surgery at some other center, were awaiting surgery, or had died. The attrition was calculated as the difference between the number of patients for whom a stoma was done and the number of patients who underwent definitive surgery and stoma closure till the data acquisition. Results: A total of 105 patients were included in the study with male preponderance. Colostomy was done for 73.33% of patients in the neonatal period (77/105). The cloacal malformation was found in 7 (6.67%), rectal atresia in 6 (5.71%), and 9 had congenital pouch colon (8.57%). The age at stoma creation for ARM ranged from 1 day to 9.3 years (mean 0.67 ±1.96 years). Cardiac and renal anomalies were among the major associations. 28 of the neonates (26.67%) had stomal complications. Stomal issues were reported in 32.4%. Mortality was reported in 37 patients (35.24%) including 35 neonates. The attrition rate in our study was found to be 38.24% (26 out of 68). Factors contributing to attrition range from anemia and protracted waiting lists to the anesthesiologist’s pursuit of optimal patient stabilization, alongside familial or personal matters faced by caregivers. Conclusion: There was a high attrition rate in operated patients of ARM in whom stoma was constructed. The main reasons for the same were anemia and a long surgical waiting list.

Using Odih-Score (Modified Klotz Score) for quality of defecation after anorectal malformation following posterior sagittal anorectoplasty (PSARP): Study of validation

Abstract* Objective The diagnostic scale or score is a clinical assessment tool used to determine diagnosis and evaluate further therapy results. The Klotz Score is a previous score modified to create a new instrument, the Odih-score. This new instrument is specifically designed to evaluate the quality of defecation success after operative anorectal malformation and is expected to be simpler and easier to apply in research hospitals. Its primary use is to evaluate the quality of defecation in post-PSARP anorectal malformation patients. Method The study conducted between 2016 and 2021, a longitudinal prospective study was conducted at RSUD Arifin Achmad Pekanbaru Riau Indonesia. The study aimed to validate the reliability of the Odih-Score (Modified Klotz Score) research instrument for evaluating defecation quality in post-PSARP anorectal malformation patients. Pearson validation test and Cronbach alpha reliability test were used as parameters for the validation, while the sensitivity and specificity of diagnostic tests were evaluated using research instrument (IBM SPSS Statistics version 25) The research was approved by the Ethical Clearance Board of the Faculty of Medicine, University of Riau, under reference number No.031/UN.19.5.1.1.8/UEPKK/2019. Results The analysis of the validation test results indicates that the Odih-Score is statistically significant (Sig.<0.05) and has a greater r-count value (0.573-0.790) than the r-table value (0.202). The research reliability test also shows a high level of reliability (Cronbach alpha 0.814) for all five items of the Odih-Score instrument. Further, the sensitivity and specificity test as a diagnostic test resulted in an AUC value of 0.798, with an Odih-Score cut-off point of 11. Conclusion Odih-Score (Modified Klotz’s score) is considered valid and reliable and can be used as a diagnostic test instrument to evaluate the quality of post-anorectal Malformation defecation with PSARP.

Comparing Consistency and Usability of Common Bowel Function Scoring Systems in Anorectal Malformation Patients

BackgroundAssessment of postoperative bowel function in anorectal malformation (ARM) patients is crucial for benchmarking outcomes. We compared existing bowel function scoring systems in various aspects in patients with ARM. MethodsWith ethical approval, this was a cross-sectional study involving 5 paediatric surgery referral centres in Malaysia, comparing the Kelly, Japanese Study Group of Anorectal Anomalies (JSGA), Holschneider and Krickenbeck bowel function questionnaires. We recruited patients aged 4–17 years, who had completed definitive surgery & stoma closure (where relevant) > 12 months prior to participation. We standardised outcomes of each scoring system into categories (‘good’, ‘fair’, ‘poor’ and ‘very poor’) to facilitate comparison. Parents & patients were surveyed and asked to rate the ease of understanding of each questionnaire. The difference in protocol scores rated between parents and patients were compared. Association of each bowel function scoring protocol with type of anomaly was assessed. Statistical significance was p < 0.05. ResultsThirty-nine parents (21 mothers, 18 fathers) and 23 patients were included in this study. Fair agreement was found between Kelly and Krickenbeck protocols (κ = 0.343; p < 0.001), between JSGA constipation and Holschneider protocols (κ = 0.276; p = 0.002); JSGA constipation and Krickenbeck protocols (κ = 0.256; p = 0.004); and between Holschneider and Krickenbeck protocols (κ = 0.273; p = 0.003). Only the Kelly protocol showed significant correlation between parents and patients' answers (ρ = 0.459, p = 0.028). Krickenbeck demonstrated the best negative correlation of patients’ scores with ARM types (ρ = −0.401, p = 0.001). The Kelly protocol ranked highest when comparing ease of understanding. ConclusionAll the questionnaires appeared comparable in assessing postoperative faecal continence in ARM patients. The Kelly questionnaire performed best in 3 key areas of assessment. Level of evidenceLevel III Cross-Sectional Study.

Functional Outcome of Anorectal Malformations in a Tertiary Care Hospital in Bangladesh

Background: The survival of anorectal malformation (ARM) patients has increased in the last ten years due to advances in newborn care and surgical methods for ARM patients. As a result, the current therapy of ARM patients is centred on functional results following definitive surgery. Aim of the study: The purpose of this study was to determine the type of ARM and measure functional outcomes, such as voluntary bowel movement (VBM), soiling, and constipation, in our patients following final surgery using the Krickenbeck classification. Methods: The current study is a cross-sectional study to retrospectively review medical records of ARM patients who underwent a definitive surgery at TMSS Medical College, Bogura, Bangladesh from 01 January 2022 to 31 December 2022 in which 45 cases were included. All collected data was entered into a Microsoft Excel Work Sheet and analyzed in SPSS 11.5 using descriptive statistics. Results: A total of 40 paediatric patients underwent hypospadias surgery. Of these, 38 (95%) cases were investigated. Most common type of hypospadias was coronal type (26.3%). Most of our patients did not have any associated urogenital problem (63.2%) and the most common associated anomaly was microphallus (7.9%). Most common surgical techniques was tabularized incised plate (65.8%). Most of our patients did not have any postoperative complications (52.6%) and most common complications was urethrocutanous fistula (31.6%). the presence of severe chordee (AOR=3.09; 95% CI 1.21-7.54; p=0.013) was significantly associated with postoperative problems. Conclusion: Functional outcomes of ARM patients in our institution are considered relatively favourable, with more than half of children showing VBM and just a small number of kids suffering from soiling and constipation. The frequency of VBM may be connected to birth weight and gender, but not ARM type, whereas soiling and constipation did not appear to be related to birth weight, gender, or ARM type. .......

Survival and factors associated with mortality among infants with anorectal malformation: a population-based study from a middle-income country.

Limited data on the survival of anorectal malformation (ARM) patients from lower- and middle-income countries is available. This retrospective population-based study from the State of Johor, Malaysia, determines the incidence, mortality rate, and survival of ARM patients and factors associated with mortality. Kaplan-Meier survival analysis was used to estimate the survival of ARM patients at 1, 5, and 10years. In addition, multivariate Cox regression analysis was used to analyze mortality-related factors. There were 175 ARM patients among 803,850 live births, giving an overall ARM incidence of 2.2 (95% confidence interval [CI], 1.9 to 2.5) per 10,000 live births. The male-to-female ratio was 1.5:1. There were 122 (69%) non-isolated ARM, of which 41 were Trisomy-21 and 34 had VACTERL association. Seventy-three (42%) had congenital heart disease (CHD), with 38 severe and 35 non-severe CHD. Overall, 33 (19%) patients died, with a median age of death of 5.7months (interquartile range (IQR) 25days to 11.2months). The overall estimated 1-, 5-, and 10-year survival rate for ARM patients was 82% (95% CI, 76-89%), 77% (95% CI, 70-84%), and 77% (95% CI, 70-84%), respectively. Univariate analysis shows that non-isolated ARM, VACTERL association, and severe CHD were associated with mortality. However, only severe CHD is the independent factor associated with mortality, with a hazard ratio of 4.0 (95% CI, 1.9-8.4). Conclusion: CHD is common among ARM patients, and one in five ARM patients had a severe cardiac defect, significantly affecting their survival. What is Known: • VACTERL association and congenital heart disease are common in patient with anorectal malformation. • Low birth weight and prematurity are associated with a lower rate of survival. What is New: • Congenital heart disease is common in ARM patients in a middle-income country. • Severe congenital heart disease plays a significant role in the survival of patients with an anorectal malformation in lower- and middle-income countries.

Karakteristik Malformasi Anorektal di Rs. Bhayangkara dan RSIA Sitti Khadijah 1 Makassar Tahun 2017 – 2022

Anorectal malformation (MAR) is considered as one of the most common congenital intestinal anomalies with an incidence of 1 in 4000 - 5000 births. The purpose of this study was to determine the characteristics of anorectal malformations at Bhayangkara Hospital &amp; RSIA Sitti Khadijah 1 Makassar in 2017 – 2022. This research is a retrospective descriptive study with a cross sectional approach. This type of research was chosen because researchers wanted to find out about the characteristics of anorectal malformation cases at Bhayangkara Hospital &amp; RSIA Sitti Khadijah 1 Makassar in 2018 – 2022. This study involved 13 samples. Based on research that has been done, that the majority of anorectal malformation patients at Bhayangkara Hospital and RSIA Sitti Khadijah 1 Makassar are male (69.2%), have a history of maternal ANC frequency of 4 times (46.2%), and do not have history of the mother taking drugs during pregnancy (61.5%). It was also found that the average birth weight was 2,661 ± 787 grams, the gestational age was 38 ± 1.5 weeks, and the mother's age at delivery was 30 ± 6.7 years. The incidence of anorectal malformations in Makassar is still low. From the data obtained from the two studies, it was not too significant between the two hospitals, Rs. Bhayangkara and RSIA Sitti Khadijah 1 Makassar.

Three dimension high definition manometry evaluated postoperative anal canal functions in children with congenital anorectal malformations.

We aimed to evaluate the function of the reconstructed anal canal in postoperative anorectal malformations (ARMs) patients through three dimension (3D) high-definition anorectal manometry. From January 2015 to December 2019, 3D manometry was performed as a postoperative functional assessment of patients with ARMs divided into age subgroups based on the time of manometry. Manometric parameters, such as the length of the anorectal high-pressure zone (HPZ-length), the mean resting and squeeze pressure of HPZ (HPZ-rest and HPZ-sqze), recto-anal inhibitory reflex (RAIR), and strength distribution of the anal canal, were collected and compared with age-matched controls. Their functional outcomes were analyzed with SPSS 23.0 software for statistical analysis. 171 manometric measurements were performed on 142 postoperative patients (3 months∼15 years). The HPZ-rest in all patients was significantly lower than in age-matched controls (p < 0.05). HPZ-sqze was notably decreased in patients older than 4 years, whereas other age groups were comparable to controls (p < 0.05). The proportions of asymmetric strength distribution and negative RAIR were higher in ARMs patients. The type of anorectal malformations and lower HPZ-rest were the impact factors affecting postoperative functional outcomes. The majority of the ARMs patients had acceptable functional outcomes. 3D manometry can objectively assess the reconstructed anal canal function. The patients with fecal incontinence had a high proportion of extremely low HPZ-rest and HPZ-sqze, negative RAIR, and asymmetric strength distribution. The manometric details will help the clinicians explore the causes of defecation complications and guide further management.

Risk factors for end stage renal disease in children with anorectal malformation and outcome comparison to children with isolated urological anomalies

End stage renal disease (ESRD) requiring kidney transplant (KT) remains an important cause of morbidity in anorectal malformations (ARM) patients. Current literature is scarce on defining the risk factors for ESRD and the outcomes of KT in ARM patients. This study aimed to identify predisposing factors to ESRD in ARM patients and verify if the long term outcome of KT in these patients differs from pure urological anomalies (UA). Databases of ARM and KT patients treated at our center between 2000 and 2016 were used for comparing characteristics of ARM cases which developed ESRD and those who did not, and the outcome features of MAR-KT vs UA-KT. Out of 117 ARM patients, 9 developed ESRD. All of them had a complex ARM. Association with UA were significantly higher in ARM-KT compared to other ARM patients (100% vs. 52%, p=0.001). The most common UA associated to KT in ARM patients was renal dysplasia. During the same period 23 patients underwent KT as a sequelae of pure urological anomalies (UA-KT group). The most represented UA were primary vesico-ureteral reflux (65.2%) and posterior urethral valves (14%) in UA-KT (table 2). ARM-KT patients required more often hemodialysis before KT (50% vs. 8.7%, p=0.05) and an aorto-caval anastomosis at the transplant (75% vs. 30%, p=0.04) compared to UA-KT. Moreover ARM-KT patients experienced more often graft failure and and the need for a second KT (50% vs. 8.6%, p=0.02). To our knowledge, this is the first study describing the differences in terms of risk factors and outcomes of KT in ARM patients. We observed a need for KT in 7% of ARM, which is at the higher end of the range reported in the literature. Bilateral dysplasia and cloaca malformation seem the leading cause to ESRD for ARM patients compared to vesico-ureteral reflux and posterior urethral valve in UA-KT. Our experience showed that KT has worst outcome in ARM vs pure UA patients. Patients with complex ARM are more frequently associated to renal dysplasia which lead to KT. Graft in ARM patients seems to be the more vulnerable and prone to failure.

Epidemiological Comparison of Anorectal Malformation With Other Gastrointestinal Abnormalities in Patients in the Pediatric Ward.

BackgroundAnorectal malformations are congenital defects wherein there is defective development of the anus and rectum. For babies born with congenital anorectal malformations, prompt treatment is crucial which requires detection of the anomalies at the earliest. This study aimed to determine the epidemiology ofanorectal malformations in the Pediatric Unit of a tertiary care hospital in Pakistan over a period of 19 months.MethodologyAn analytical cross-sectional study was conducted retrospectively from January 2020 to September 2021 using a non-randomized consecutive sampling technique. Patients aged less than eight years were included, whereas burnt, torn, and incomplete records from the Hospital Management Information System (HMIS) were excluded. SPSS version 26 (IBM Corp., Armonk, NY, USA) was used for data entry and analysis. Binomial and multinomial logistic regression were applied for analyzing the association between explanatory and dependent variables.ResultsOf the 1,108 patients, 72 (6.5%) patients had anorectal malformations. Gastrointestinal diseases made up about 64.3% of all diseases. Among gastrointestinal causes, the prevalence of anorectal malformation was up to 10.1%. The mortality of anorectal malformation patients was low (2.85%) compared to mortalities due to other gastrointestinal abnormalities (8.25%). Anorectal malformation had significantly lower odds of mortality (adjusted odds ratio = 0.19, p < 0.05) compared to other gastrointestinal abnormalities.ConclusionsThis study has provided data about the prevalence of anorectal malformation and its mortality which were calculated as 6.5% and 2.58%, respectively. Female gender, neonates, and delayed presentation were seen to have higher mortality, highlighting the need to screen all neonates pre- and post-natally to avoid any misdiagnosis.

Comparison of MRI with Distal Colostogram in Patients with Anorectal Malformations- A Cohort Study

Introduction: Anorectal Malformations (ARMs) are the second most common paediatric surgical emergencies with management strategy is restoration of near normal anatomy and physiology which require accurate and proper imaging diagnosis. Aim: To evaluate Magnetic Resonance Imaging (MRI) findings and its comparison with Distal Colostogram (DC) against surgical findings in anorectal malformation patients. Materials and Methods: A cohort study was done at two centers Jawaharlal Nehru Medical College, Aligarh (JNMC, Aligarh) and Vardhman Mahavir Medical College (VMMC) and Safdarjung Hospital, New Delhi involving 60 patients of ARMs. MRI was done in all patients and distal colostogram was done in 46 (74.4%) patients. All patients were evaluated for associated anomalies. Both the imaging modalities were compared for the type of ARM and fistula against the surgical findings and Chi-square and Fisher’s test were used to calculate statistical significance. Results: Out of total 60 patients included in this study, there were 37 (61.7%) males and 23 females (38.3%). High ARM was seen in 34 (56.7%) patients and low ARM was seen in 26 (43.3%). There was no significant difference between distal colostogram and MRI in differentiating the type of ARM (p-value 0.13). Spinal anomalies (52.5%) were the most common associated anomalies, followed by genitourinary (47.5%) and musculoskeletal (32.5%). Conclusion: From the results of present study no significant difference was found between DC and MRI findings regarding the type of ARM.

Reoperative surgery in anorectal malformation patients.

Currently accepted primary repair of congenital anorectal malformations (ARMs) includes a posterior sagittal incision, which allows for optimal visualization and identification of important pelvic structures and anatomical features. Reconstructive surgery involves meticulous dissection and separation of pelvic structures, and careful reconstruction can result in good functional outcomes for many patients, who live without ongoing sequelae from their malformation. However, some patients may require reoperative procedures for anatomic or functional reasons. Males and females present with slightly different symptoms and should be approached differently. Males are most likely to require reoperations for anorectal or urethral pathologies, but the urinary system is often spared in females-they instead must contend with Mullerian duct anomalies, of which there are many varieties. Depending on the original malformation and severity of symptoms, redo surgery may be needed to optimize function and quality of life. Surgical management with reoperative surgery in ARMs ranges from straightforward to complex, depending on the issue. One must weigh the risks of reoperative surgery and potentially creating more scarring against the need for a better anatomical and functional outcome. Current management trends and practice patterns with regards to reoperative surgery in ARM patients are not widely studied or standardized but we provide an overview of the more common pathologies, preoperative evaluation and workup required to identify the issues, and options for reoperative repair in these patients.

Correlation between the lateral and anteroposterior sacral ratios in anorectal malformations.

The sacral ratio has been used as a tool for evaluating sacral development in patients with anorectal malformations. Sacral ratios can be calculated by obtaining sacral radiographs in the anteroposterior (AP) and lateral planes. The objective of the study was to determine the correlation and agreement in sacral ratio calculations. In this single institution retrospective cohort study, we reviewed medical charts of all pediatric anorectal malformation patients treated between March 2014 and September 2018 who had both AP and lateral images of their sacrum. All sacral ratios were measured by three radiologists. Pearson's correlation coefficients and corresponding 95% confidence intervals (CIs) were used to assess the correlation between the AP and lateral radiographs. A weighted Kappa statistic was used to measure the agreement between how the AP and lateral sacral ratios categorized observations into risk groups. Our initial cohort consisted of 646 observations from patients with anorectal malformations who had radiographs obtained in both AP and lateral planes. We excluded all observations (n=76) where the radiographs were deemed to be inadequate or not appropriately centered to measure sacral ratio. For a given pair of measurements, the mean lateral sacral ratio was 0.07units greater than the AP plane (95% CI 0.06-0.09, paired t-test P-value <0.0001). AP and lateral images had a moderate positive correlation (Pearson's r=0.76, 95% CI 0.73-0.79, P<0.0001) and moderate agreement in risk categorization (unweighted kappa = 0.60, P<0.0001). AP and lateral readings conducted by all three radiologists had excellent inter-rater reliability with intraclass correlations for AP and lateral sacral ratios of 0.88 and 0.84, respectively. Even though the AP and lateral sacral ratios had moderate positive correlation, the mean sacral ratio determined by images in the lateral plane was 0.07units greater than the AP plane. AP and lateral sacral ratios concluded different risk categories relatively often. Future studies are needed to determine whether AP or lateral sacral ratios correlate better with continence in patients with anorectal malformations.

Role of urodynamics in male patients of high-anorectal malformations: a prospective study.

Association of spinal or vertebral anomalies and the iatrogenic denervation during surgical correction of anorectal malformation patients especially in boys can lead to neurogenic bladder inthese subset of patients. The paucity of literature with regard to urodynamic studies focusing exclusively in male children with high-anorectal malformations (HARM) lead us to analyze the urodynamic changes. The objective was to study urodynamic profile in male patients who have undergone surgery for anorectal malformation. Male high-anorectal malformation patients who had completed all the stages of repair were prospective studied. Following the basic work up, all patients based on the urodynamics were categorized into 2 groups as safe or unsafe bladders. Unsafe bladder was defined as detrusor pressure > 40cm (high detrusor pressure) or pressure variability of 15cm of water (detrusor overactivity) or significant post-void residue. MRI was limited to patients with only abnormal urodynamics to rule out spinal causes of neurogenic bladder and due to financial constraints, it could not be offered to all patients. 41 HARM meet the exclusion criteria. All patients were asymptomatic with none having history of urinary tract infections. Ultrasound showed bladder wall thickening in 31.7% patients. UDS revealed reduction in bladder capacity and compliance was noted in 31.7% and 30% patients, respectively. Elevated detrusor pressures (> 40cm of water) were noted in 10% (4/41), detrusor overactivity with concomitant elevated detrusor pressures in 19.5% (8/41) and normal UDS in 70% (29/41). 13 patients (31.7%) had abnormal cystometric parameters with 12(30%) having unsafe bladders. MRI confirmed sacral agenesis in 1 patient with unsafe bladder. Urodynamics can demonstrate occult neurovesical dysfunction in patients with HARM. This would help in early renal protective therapy and prevent the burden of long-term sequelae of neurovesical dysfunction in HARM patients.

Accuracy Of Transperineal Sonography And Distal Loopogram In Determining Distal Rectal Pouch-Perineal Distance And Fistula On Anorectal Malformation Post Colostomy Patients At Dr. Mohammad Hoesin Palembang

Abstract&#x0D; Background : Imperforated anus is a congenital abnormality which is better known as anorectal malformation. The incidence of anorectal malformations is 1 in 4000 to 5000 live births. The incidence of anorectal malformations in Europe varies from 1.14 to 5.96 per 10,000 people and can change each year.1-3 The surgical approach in the management of anorectal malformations depends on the classification of anorectal malformations based on low, intermediate and high location. The distal loopogram or distal colostrography / colostrogram examination is an examination that is very important to determine the location of the distal rectum before definitive repair, but this examination has the disadvantage of using contrast and x-ray radiation. One alternative radiological examination that can be used for faster initial management is sonographic.7&#x0D; Method : This study was a diagnostic test study to assess the diagnostic accuracy of the distal loopogram / colostrography examination and sonographic examination with the transperineal approachon post-colostomy Anorectal Malformations patients at Mohammad Hoesin Hospital, Palembang in August 2019-October 20120 or until the number of research samples is met.&#x0D; Result : There were 21 samples in this study, 11 (52.4%) samples were dominated by clinical appearance without fistula. On the results of ultrasound examination, the majority of samples of rectouretral fistulas were 7 (33.3%). Loopogram examination found the majority of samples without fistulas as many as 10 (47.6%) samples. On intraoperative examination, 7 (33.3%) samples were obtained for rectouretral fistulas. The result of pouch-perineum measurement on ultrasound examination was 1.15 ± 0.6 cm, loopogram was 0.7 ± 1.5 cm, intra-operasionem was 1.8 ± 1.6 cm. In this study, USG has a very high accuracy value with a sensitivity of 100% and a specificity of 93.8% while the Loopogram has a sensitivity of 100% and a specificity of 68.8%.&#x0D; Conclusion : Transperineal ultrasound has a very high accuracy value with a sensitivity of 100% and a specificity of 93.8% while the Loopogram has a sensitivity of 100% and a specificity of 68.8%. Although there is a significant difference between the distal rectum -perineal distance calculation with a difference of -0.38 ± 0.70 cm, these two methods are equally accurate in assessing the distal rectal pouch -perineal distance and the location of the fistula.

SC335 - Kidney Transplant (KT) in Anorectal Malformation (ARM) Patients: risk factors and outcome

SC335 - Kidney Transplant (KT) in Anorectal Malformation (ARM) Patients: risk factors and outcome

Multi-institutional review of bowel management strategies in children with anorectal malformations

Background/rationaleTo describe current bowel management program (BMP) strategies in anorectal malformation (ARM) patients based on patient-level predictors using data from a multi-institutional consortium. Materials/MethodsPatient bowel function and BMP were reviewed from Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) data. The PCPLC is comprised of multidisciplinary specialists researching colorectal and pelvic disorders. Seven US institutions submitted de-identified clinical data on ARM patients into a centralized patient registry. ResultsThe primary ARM of 624 patients was categorized into Mild (45.2%), Moderate (40.4%) or Complex (14.2%) anomaly classifications. Patient-specific BMP were examined based on age and on the presence of spinal cord/sacral anomalies.418 (67%) enrolled patients were prescribed BMP (<5 yo 56.4%; ≥5–<12 yo 86.7%; ≥12 81.5%). Constipation was the primary chief complaint (80.2%). Forty percent of patients on a BMP were toilet trained and approximately one-half (48.5%) reported daytime stool accidents.Secondary surgical interventions for antegrade continence enemas (ACE) were examined; 14.5% of patients employed ACE strategies and utilization increased with age and varied based on anatomic anomalies. ConclusionsThis is the first report on BMP strategies for patients with ARM from the Pediatric Colorectal and Pelvic Learning Consortium. Individual patient characteristics are explored for their impact on bowel management strategy utilization. Level of EvidenceIV

Should the search for ganglia in the distal rectal fistula in patients with anorectal malformation be abandoned?

PurposeOccurrence of Hirschsprung’s disease in anorectal malformation (ARM) patients is rare, but many surgeons still ask to pathologists to search for ganglia in the terminal rectum/fistula; the histological procedure is time and money consuming and the results confounding. A consecutive series of ARM patients, in which the presence of ganglia in terminal rectum was revised, is herein presented. Materials and methodsRectal specimens of ARM patients who underwent corrective surgery in the last 6 years were retrieved. The histological protocol included H&E staining and calretinin immunohistochemistry. Each specimen is processed until all material is examined if no ganglia are retrieved after the first twelve sections. ResultsForty cases were examined. Eight patients were younger than 1 month of age at operation. The mean length of the specimen was 1.5cm (range: 1–3 cm). Upon clinical request, ganglia were searched in 15/40 cases (37.5%) and resulted absent in 10/15 (66.5%). All patients have been followed and none developed signs or symptoms suggestive for Hirschsprung. ConclusionsThe practice to search for ganglia in the terminal rectum/fistula in ARM patients should be abandoned, as incidence of associated colorectal diseases is rare. Moreover, the procedure is expensive both in terms of laboratory’s reagents and working time of expert pathologists and technicians. Level of EvidenceLevel IV (Case Series with no Comparison Group)

Accuracy of Lateral Invertogram in diagnosing and classifying anorectal malformations

Background: The initial management of a new born baby with anorectal malformation could be made only after an accurate determination of the level of the anomaly. &#x0D; Objective: To determine the accuracy of lateral invertogram in diagnosing and classifying anorectal anomalies.&#x0D; Methods: This was a retrospective study carried out between January 2003 and December 2013. The accuracy, sensitivity, and specificity of lateral invertogram in diagnosing and classifying anorectal malformations in the cases studied were determined.&#x0D; Results: A total of 62 children with anorectal malformations patients were seen during the study period. The age range was 4 hours to 6 years. A total of 50 (80.6%) had lateral invertogram while only 39 films of lateral invertogram were available for analysis. A total of 22 patients had high anomalies while 12 patients had low anomalies. The accuracy of lateral invertogram in identifying anorectal anomaly was 100% and its ability to differentiate between high and low anomalies was 76.5%. The Sensitivity in identifying high anomalies was 59% while the specificity was 31.8%. The sensitivity in identifying low lying lesions was 66.5% while the specificity was 50%.&#x0D; Conclusion: The accuracy, sensitivity, and specificity of lateral invertogramin diagnosing and classifying anorectal malformations were found to be satisfactory in the present study.

Accuracy of Lateral Invertogram in diagnosing and classifying anorectal malformations

Background: The initial management of a new born baby with anorectal malformation could be made only after an accurate determination of the level of the anomaly. Objective: To determine the accuracy of lateral invertogram in diagnosing and classifying anorectal anomalies. Methods: This was a retrospective study carried out between January 2003 and December 2013. The accuracy, sensitivity, and specificity of lateral invertogram in diagnosing and classifying anorectal malformations in the cases studied were determined. Results: A total of 62 children with anorectal malformations patients were seen during the study period. The age range was 4 hours to 6 years. A total of 50 (80.6%) had lateral invertogram while only 39 films of lateral invertogram were available for analysis. A total of 22 patients had high anomalies while 12 patients had low anomalies. The accuracy of lateral invertogram in identifying anorectal anomaly was 100% and its ability to differentiate between high and low anomalies was 76.5%. The Sensitivity in identifying high anomalies was 59% while the specificity was 31.8%. The sensitivity in identifying low lying lesions was 66.5% while the specificity was 50%. Conclusion: The accuracy, sensitivity, and specificity of lateral invertogramin diagnosing and classifying anorectal malformations were found to be satisfactory in the present study.

Anorectal malformation patients’ outcomes after definitive surgery using Krickenbeck classification: A cross-sectional study

BackgroundThe survival of anorectal malformation (ARM) patients has been improved in the last 10 years because of the improvement in management of neonatal care and surgical approaches for ARM patients. Thus, the current management of ARM patients are focusing on the functional outcomes after definitive surgery. Here, we defined the type of ARM and assessed the functional outcomes, including voluntary bowel movement (VBM), soiling, and constipation, in our patients following definitive surgery using Krickenbeck classification. MethodsWe conducted a cross-sectional study to retrospectively review medical records of ARM patients who underwent a definitive surgery at Dr. Sardjito Hospital, Indonesia, from 2011 to 2016. ResultsForty-three ARM patients were ascertained in this study, of whom 30 males and 13 females. Most patients (83.7%) were normal birth weight. There were ARM without fistula (41.9%), followed by rectourethral fistula (25.5%), perineal fistula (18.6%), vestibular fistula (9.3%), and rectovesical fistula (4.7%). The VBM was achived in 53.5% patients, while the soiling and constipation rates were 11.6% and 9.3%, respectively. Interestingly, patients with normal birth weight showed higher frequency of VBM than those with low birth weight (OR = 9.4; 95% CI = 1.0–86.9; p = 0.04), while male patients also had better VBM than females (OR = 3.9; 95% CI = 1.0–15.6) which almost reached a significant level (p = 0.09). However, VBM was not affected by ARM type (p = 0.26). Furthermore, there were no significant associations between gender, birth weight, and ARM type with soiling and constipation, with p-values of 1.0, 1.0, and 0.87; and 0.57, 1.0, and 0.94, respectively. ConclusionsFunctional outcomes of ARM patients in our hospital are considered relatively good with more than half of children showing VBM and only relatively few patients suffering from soiling and constipation. The frequency of VBM might be associated with birth weight and gender, but not ARM type, while the soiling and constipation did not appear to be correlated with birth weight, gender, nor ARM type. Further multicenter study is necessary to compare our findings with other centers.