Complex Congenital Defects Research Articles

ACUTE RESPIRATORY DISTRESS REVEALING A COMPLETE ATRIOVENTRICULAR CANAL IN A 22-MONTH INFANT

The atrioventricular canal (AVC) is a complex and rare congenital cardiac anomaly. It is potentially severe due to the complexity of its lesions and associated fixed pulmonary arterial hypertension. We report the case of a 22-month-old infant girl with Trisomy 21 syndrome, admitted for acute respiratory distress. TTE showed a complete AVC with pulmonary arterial hypertension. Prognosis depends on the promptness of diagnosis and therapeutic management.

Content Analysis of Brief Telephonic Conversation with Parents of Children with Cleft Lip and Palate During Sustained Anticipatory Guidance Sessions.

Cleft lip and palate (CLP) represents complex congenital anomalies that necessitate a comprehensive approach to care, involving multiple healthcare disciplines. The primary aim of the study is to explore the content of periodic telephone support calls between parents/caregivers of children with CLP and pediatric dentist during sustained anticipatory guidance. The qualitative analysis was conducted with the available telephonic recordings that was obtained from the STOP database. 40 recordings of 8 children were used for this study. The recorded interviews were translated and transcribed verbatim and analysed using the thematic content analysis method. The four major themes that were addressed by the dentist were the general health of the baby, oral health and development, surgery-related concerns and emotional support. It was also observed that the parents demonstrated a positive shift in oral health related behaviour significantly improving their child's oral hygiene practices. The telephonic support calls offer valuable insights into the concerns and topics the parents of children with CLP are eager to discuss. These calls not only address general concerns, receiving emotional support from the professionals, demonstrate the health related behavior shift that takes place during SAG and reassures parents of their support.

Association of selected gene variants with nonsyndromic orofacial clefts in Kuwait

Introduction and objectivesNon-syndromic orofacial clefts (NSOFCs) are complex congenital abnormalities involving both environmental and genetic factors involved in orofacial development. This study aimed to investigate the genetic association of specific genetic variants at different CYRIA gene loci with the development of NSOFCs in Kuwait. MethodsFour genetic variants (rs7552, rs3758249, rs3821949, and rs3917201) at four selected gene loci (CYRIA, FOXE1, MSX1, and TGFB3) were genotyped in a total of 240 DNA samples (patients (n = 114) and random controls (n = 126)) employing TaqMan® allele discrimination assay. For each variant and its genotype, the frequencies were determined and tested for Hardy-Weinberg Equilibrium. Genotype frequencies was compared between patients and controls using Pearson’s test. Logistic regression analyses were employed to test for the associations of the four selected variants with the occurrence of NSOFCSs. ResultsSignificant differences in the distribution of genotypes between cases and controls, rs7552, rs3821949, and rs3917201 were found to have a positive association with NSOFCs. After adjusting for gender, the GG genotype of the rs7552 variant, the AG genotype of the rs3821949 variant, and the CC genotype of the rs3917201 variant showed nearly a two-fold increased risk of NSOFC (p < 0.05). ConclusionThis study reports significant findings on the contribution and modest effect of CYRIA rs7552, MSX1 rs3821949, and TGFB3 rs3917201 in the development of NSOFCs. Our findings provide further evidence on the molecular mechanism and the role of the selected genes in NSOFCs.

ERN eUROGEN Guidelines on the Management of Anorectal Malformations Part III: Lifelong Follow-up and Transition of Care

Abstract Introduction Anorectal malformations (ARMs) are complex congenital anomalies of the anorectal region, oftentimes also affecting the genitourinary system. Although successful surgical correction can often be achieved in the neonatal period, many children will experience functional problems in the long term. The European Reference Network for rare and complex urogenital conditions (eUROGEN) assembled a panel of experts to address these challenges and develop comprehensive guidelines for the management of ARM. Methods The Dutch Quality Standard for ARM served as the foundation for the development of guidelines applicable on a European level. Literature was searched in Medline, Embase, and Cochrane. The ADAPTE method was utilized to incorporate the newest available evidence. A panel of 15 experts from 7 European countries assessed currency, acceptability, and applicability of recommendations. Recommendations from the Dutch Quality Standard were adapted, adopted, or rejected and recommendations were formed considering the current evidence and/or expert consensus. Results Lifelong follow-up, integration, and transition of care were assessed. A total of eight new studies were identified. The panel adapted 18 recommendations, adopted 6, and developed 6 de novo. Overall, the level of evidence was considered low. Conclusion Successful lifelong follow-up and transition of care require a dedicated team of pediatric and adult specialist and an individually tailored patient-centered approach. This guideline summarizes the best available evidence on follow-up of ARM patients and provides guidance for the development of structured transition programs.

Examination of piperonyl butoxide developmental toxicity as a Sonic hedgehog pathway inhibitor targeting limb and palate morphogenesis

Piperonyl butoxide (PBO) is a pesticide synergist with widespread use and human exposure that was discovered to inhibit Sonic hedgehog (Shh) signaling, a pathway required for numerous developmental processes. Previous examinations of PBO’s potential for developmental toxicity have generated seemingly conflicting results. We investigated the impact of acute PBO exposure targeting Shh pathway activity during palate and limb morphogenesis. Timed-pregnant C57BL/6 J mice were exposed to a single PBO dose (67–1800 mg/kg) at gestational day (GD) 9.75, and litters were collected at GD10.25 and GD10.75 to examine Shh pathway activity or GD17 for phenotypic assessment. PBO exposure induced dose-dependent limb malformations and cleft palate in the highest dose group. Following PBO exposure, reduced expression of the Shh pathway activity markers Gli1 and Ptch1 was observed in the embryonic limb buds and craniofacial processes. These findings provide additional evidence that prenatal PBO exposure targeting Shh pathway activity can result in malformations in mice that parallel common etiologically complex human birth defects.

Genetic association of GJA8 with long-segment Hirschsprung's disease in southern Chinese children.

Hirschsprung's disease (HSCR) is a complex congenital neurodevelopmental disorder affecting colons caused by both genetic and environmental factors. Although several genes have been identified as contributing factors in HSCR, the pathogenesis is still largely unclear, especially for the low prevalent long-segment HSCR (L-HSCR). Gap junction protein alpha 8 (GJA8) is involved in several physiological processes and has been implicated in several diseases. However, the relationship between GJA8 single nucleotide polymorphism (SNP) rs17160783 and HSCR in the southern Chinese population remains unknown. The study aimed to explore the association of genetic variants in GJA8 and HSCR susceptibility in southern Chinese. SNP rs17160783 A>G in GJA8 was genotyped by TaqMan SNP Genotyping Assay in all samples, which included 1,329 HSCR children (cases) and 1,473 healthy children (controls). Odds ratio (OR) and 95% confidence interval (CI) were used to evaluate the association of GJA8 polymorphisms with HSCR susceptibility. The GTEx database and transcription factor binding site (TFBS) prediction were used to analyze the potential regulatory function of rs17160783. Genetic association analysis illustrated that rs17160783 could increase the risk of L-HSCR (Padj=0.04, ORadj =1.48, 95% CI: 1.02-2.14). We also found that GJA8 expression was increased in HSCR and neurodevelopmentally impaired animal models. External epigenetic data revealed that GJA8 rs17160783 may have the potential to regulate the expression of the GJA8, possibly by altering the binding of transcription factors for GJA8, and consequently impacting the PI3K-Akt signaling pathway during the enteric nervous system (ENS) development. Our results suggested that rs17160783 might play a regulatory role in GJA8 expression and increase the susceptibility of L-HSCR in children from southern China.

Percutaneous Closure of Type IV Aortopulmonary Window. An Illustrative Case

The aortic-pulmonary window is a rare congenital heart defect characterized by a direct communication between the aorta and pulmonary artery, bypassing the normal route through the heart. This defect can lead to significant hemodynamic changes, including increased pulmonary blood flow and volume overload. Symptoms often manifest in infancy or early childhood and can range from mild to severe, depending on the size of the defect and associated cardiac anomalies. Percutaneous treatment of the aortic-pulmonary window represents a promising advancement in the management of this complex congenital defect. While surgical repair remains a viable option for some cases, percutaneous methods provide a less invasive alternative with favorable outcomes. Continued research and technological improvements are essential for optimizing treatment strategies and improving patient quality of life.

Continent stoma with Modified Mansoura Technique (Indiana W pouch with nipple valve fixation) in adult bladder exstrophy: A rare case report on step-by-step surgery technique and outcome

Introduction: Bladder exstrophy (BE), a complex and severe congenital defect of urogenital sinus and the musculoskeletal system, in adulthood is very rare. The surgical management of adulthood bladder exstrophy is complex and post operative complications are possible. This case report show surgical management step-by-step and post operative outcomes of neglected bladder exstrophy with contracted bladder, right renal agenesis, bilateral unpalpable undescended testis (UDT), and megarecti with scibala. Case Presentation: A 23 years old male with neglected bladder exstrophy with contracted bladder, right renal agenesis, bilateral unpalpable undescended testis (UDT), and megarecti with scibala underwent Modified Mansoura Technique (Indiana W pouch + Nipple valve fixation) for the bladder exstrophy, orchidopexy with Shehata technique. Conclusion: Modified Mansoura Technique (Indiana W pouch + Nipple valve fixation) approach yielded favorable outcomes without post operative complications, at least during the monitoring period in hospital. However, continued follow-up is necessary to ensure long-term success.

Craniofacial morphological variability in orthodontic patients with non-syndromic orofacial clefts: an approach using geometric morphometrics

ObjectivesOrofacial clefts are complex congenital anomalies that call for comprehensive treatment based on a thorough assessment of the anatomy. This study aims to examine the effect of cleft type on craniofacial morphology using geometric morphometrics.Materials and methodsWe evaluated lateral cephalograms of 75 patients with bilateral cleft lip and palate, 63 patients with unilateral cleft lip and palate, and 76 patients with isolated cleft palate. Generalized Procrustes analysis was performed on 16 hard tissue landmark coordinates. Shape variability was studied with principal component analysis. In a risk model approach, the first nine principal components (PC) were used to examine the effect of cleft type.ResultsWe found statistically significant differences in the mean shape between cleft types. The difference is greatest between bilateral cleft lip and palate and isolated cleft palate (distance of means 0.026, P = 0.0011). Differences between cleft types are most pronounced for PC4 and PC5 (P = 0.0001), which together account for 10% of the total shape variation. PC4 and PC5 show shape differences in the ratio of the upper to the lower face, the posterior mandibular height, and the mandibular angle.ConclusionsCleft type has a statistically significant but weak effect on craniofacial morphological variability in patients with non-syndromic orofacial clefts, mainly in the vertical dimension.Clinical relevanceUnderstanding the effects of clefts on craniofacial morphology is essential to providing patients with treatment tailored to their specific needs. This study contributes to the literature particularly due to our risk model approach in lieu of a prediction model.

Double outlet right ventricle accompanied by complex congenital anomalies: A rare association

Double outlet right ventricle (DORV) comprises a heterogeneous series of associated cardiac anomalies that involve the RV outflow tract in which both of the great arteries arise entirely or predominantly from the RV. The anatomic dysmorphology is variable, and there may be associated cardiac anomalies. We report here a case of a complex cyanotic heart disease in a 14 year old adolescent comprising of DORV accompanied by multiple congenital cardiac anomalies: single atrium, complete AV canal defect, severe AV valve regurgitation, D-malposition of great arteries, subaortic obstruction and pulmonary valvular stenosis.

Predictive factors reducing the effectiveness of the rehabilitation process in patients with congenital orofacial clefts combined with malformations in other organs and systems

Relevance. The successful completion of the rehabilitation process for patients with complex congenital defects, specifically those with a combination of orofacial clefts (OFC) and malformations in other organs and systems, presents a significant challenge. This challenge is pertinent not only to medical practice but also to the socio-psychological development of the child.Objective. To identify predictive factors that diminish the success of the rehabilitation process in patients with congenital OFC combined with malformations in other organs and systems by systematising and addressing these factors at various stages of specialised care.Materials and methods. An analysis of medical records was conducted for 2,475 patients with congenital orofacial clefts (OFC) over a 24-year period (1998-2022) who were under dispensary observation at the "Bonum" Center in Yekaterinburg. Among these patients, 823 (33.25%) had OFC combined with malformations in other organs and systems. A comprehensive study was conducted among these 823 individuals within the framework of dynamic observation and treatment, focusing on patients who had reached 18 years of age and were subsequently removed from dispensary observation. The study included an examination of staged treatment and discharge summaries, alongside the clinical characteristics of the restored midface area following surgical correction of OFC amidst correction for concomitant congenital malformations.Results. Among the 823 individuals surveyed, a retrospective study registered 591 (71.81%) who had completed the main stages of comprehensive specialised care amidst concomitant malformations, while the remaining 232 (28.19%) continue to receive combined rehabilitation. Within the 591 patients, a subgroup of 123 (20.81%) individuals who had reached 18 years of age, completed the full cycle of medical-social rehabilitation at the "Bonum" Center, and were removed from dispensary observation due to age, was selected and studied in detail. Questionnaires, parent interviews, and analysis of the results obtained were conducted. The study identified predictive factors reducing the effectiveness of the rehabilitation process in patients with congenital orofacial clefts (OFC) combined with malformations in other organs and systems.Conclusion. The multifaceted study conducted has facilitated the identification and systematisation of predictive factors that reduce the success of the rehabilitation process. Consequently, this enables the enhancement of comprehensive specialised treatment outcomes for this category of patients.

Vesicovaginal Fistula Secondary to Left Ectopic Ureter in a 3-Year-Old Female with Left Ureter-Duplication in Tamale Teaching Hospital, Ghana: A Case Report

Purpose: Vesicovaginal fistula (VVF) is a pathological connection between the bladder and vagina, primarily caused by gynaecological surgery, obstructed labour, and cesarean section. VVF significantly impairs quality of life and is prevalent in developing countries, with Africa seeing up to 130,000 new cases annually. Surgical repair, especially transvaginal approaches, remains the primary treatment. Paediatric cases of VVF, often linked to congenital anomalies like ectopic ureter, are rare and complex to manage. Methodology: We report a case of a 3-year-old female with continuous urinary leakage since birth, initially flagged for renal anomalies via prenatal imaging. Clinical examination was normal with no distress or developmental issues. Diagnostic assessments, including abdominal ultrasound and micturating cystourethrogram (MCUG), revealed left hydronephrosis, hydroureter, and a definitive vesicovaginal fistula. A CT Urogram confirmed the diagnosis, showing contrast leakage into the vagina. Findings: Diagnostic cystoscopy revealed a normally positioned right ureteric orifice, while the left was aberrantly positioned near the bladder neck. Culposcopy identified a ureterocele in the posterior fornix of the vagina. During laparotomy, a dilated left distal ureter was observed entering the vagina, indicating true ureter duplication. The surgical intervention involved left ureteric reimplantation using the antireflux method, successfully resolving the anomaly. Post-operative recovery was smooth, with significant improvement in urinary symptoms and no recurrence or complications noted during follow-up. Unique contribution to theory, policy and practice: VVF secondary to ectopic ureter in paediatric patients is rare but manageable with early diagnosis and tailored surgical intervention. Our case underscores the importance of a multidisciplinary approach and the efficacy of ureteral reimplantation in treating complex congenital anomalies, leading to favourable outcomes and symptom resolution.

Laparoscopic-assisted vaginoplasty in complex congenital urogenital anomaly: A case report

Laparoscopic-assisted vaginoplasty in complex congenital urogenital anomaly: A case report

Art or Artifact: Evaluating the Accuracy, Appeal, and Educational Value of AI-Generated Imagery in DALL·E 3 for Illustrating Congenital Heart Diseases.

Artificial Intelligence (AI), particularly AI-Generated Imagery, has the potential to impact medical and patient education. This research explores the use of AI-generated imagery, from text-to-images, in medical education, focusing on congenital heart diseases (CHD). Utilizing ChatGPT's DALL·E 3, the research aims to assess the accuracy and educational value of AI-created images for 20 common CHDs. In this study, we utilized DALL·E 3 to generate a comprehensive set of 110 images, comprising ten images depicting the normal human heart and five images for each of the 20 common CHDs. The generated images were evaluated by a diverse group of 33 healthcare professionals. This cohort included cardiology experts, pediatricians, non-pediatric faculty members, trainees (medical students, interns, pediatric residents), and pediatric nurses. Utilizing a structured framework, these professionals assessed each image for anatomical accuracy, the usefulness of in-picture text, its appeal to medical professionals, and the image's potential applicability in medical presentations. Each item was assessed on a Likert scale of three. The assessments produced a total of 3630 images' assessments. Most AI-generated cardiac images were rated poorly as follows: 80.8% of images were rated as anatomically incorrect or fabricated, 85.2% rated to have incorrect text labels, 78.1% rated as not usable for medical education. The nurses and medical interns were found to have a more positive perception about the AI-generated cardiac images compared to the faculty members, pediatricians, and cardiology experts. Complex congenital anomalies were found to be significantly more predicted to anatomical fabrication compared to simple cardiac anomalies. There were significant challenges identified in image generation. Based on our findings, we recommend a vigilant approach towards the use of AI-generated imagery in medical education at present, underscoring the imperative for thorough validation and the importance of collaboration across disciplines. While we advise against its immediate integration until further validations are conducted, the study advocates for future AI-models to be fine-tuned with accurate medical data, enhancing their reliability and educational utility.

Endothelial-to-Mesenchymal Transition in Human and Murine Models of Congenital Diaphragmatic Hernia

Introduction: Congenital diaphragmatic hernia (CDH) is a complex congenital disorder, characterized by pulmonary hypertension (PH) and hypoplasia. PH secondary to CDH (CDH-PH) features devastating morbidity and mortality (25–30%) among neonates. An unmet need is determining mechanisms triggering CDH-PH to save infants. Prior data suggest abnormal remodeling of the pulmonary vascular extracellular matrix (ECM), presumed to be driven by endothelial-to-mesenchymal transition (EndoMT), hinders postnatal vasodilation and limits anti-PH therapy in CDH. There are limited data on the role of EndoMT in CDH-PH. Methods: The purpose of the study was to investigate how EndoMT contributes to CDH-PH by identifying cells undergoing EndoMT noted by alpha smooth muscle actin (α-SMA) expression in human umbilical vein endothelial cells (HUVECs) and lung tissue obtained from murine pups using the nitrofen model. N = 8 CDH, N = 8 control HUVECs were stained for α-SMA and CD31 after being exposed for 24 h to TGFB, a known EndoMT promoter. N = 8 nitrofen, N = 8 control murine pup lungs were also stained for α-SMA and CD31. α-SMA and CD31 expression was quantified in HUVECs and murine tissue using Fiji imaging software and normalized to the total number of cells per slide noted by DAPI staining. Results: CDH HUVECs demonstrated a 1.1-fold increase in α-SMA expression (p = 0.02). The murine model did not show statistical significance between nitrofen and control pup lungs; however, there was a 0.4-fold increase in α-SMA expression with a 0.8-fold decrease in CD31 expression in the nitrofen pup lungs when compared to controls. Conclusion: These results suggest that EndoMT could potentially play a role in the ECM remodeling seen in CDH-PH.

A multidisciplinary approach to the treatment of children and adolescents with Beckwith-Wiedemann syndrome

IntroductionBeckwith-Wiedemann syndrome (BWS) is a rare and complex congenital disorder characterized by a spectrum of symptoms and somatic findings. The prevalence of classic BWS is 1:26,000 births in Europe, and is equal in both sexes. The causes of the disorder are complex and are related to alterations in the expression of one or more genes in the region of chromosome 11. The heterogeneity of the clinical picture results in a spectrum of clinical features, the most common of which include: excessive growth of one side or certain parts of the body, macroglossia, abdominal wall deficits such as umbilical hernia, hypoglycemia, enlarged abdominal organs and an increased risk of developing certain types of tumors in adulthood.ObjectivesThe aim of this article is to highlight the importance of an early multidisciplinary approach in the management of children and adolescents with BWS.MethodsUsing clinical practice and a review of the existing limited literature, we examined the complexity of the disease and the importance of psychiatric, psychotherapeutic, and psychological interventions in the treatment of children and adolescents with rare diseases such as BWS.ResultsAccording to our clinical practice, a number of uncertain physical symptoms and possible complications may in some children with BWS lead to psychomotor retardation and lack of self-confidence due to the often impaired physical appearance. Affected children and adolescents are more likely to be exposed to abuse at school, show more behavioral and learning difficulties, difficulties in social adjustment, and resultant emotional difficulties. After initial genetic and pediatric treatment and subsequent regular monitoring, it is necessary to pay additional attention to the development of psychological sequelae in order to involve them and their families in psychotherapeutic treatment, and intervene in a timely manner so that they can achieve or maintain psychological stability and functionality. Many adolescent patients with BWS do not have significant somatic difficulties that would require pediatric intervention, but often present with symptoms of mental illness.ConclusionsPsychological stress in children and adolescents suffering from rare somatic diseases represents a negative experience of an emotional and social nature, which affects the course of the disease and interferes with the treatment. Due to a number of possible physical manifestations and outcomes of such diseases, extensive psychological support and care by child and adolescent psychiatrists and the entire medical team is required. A multidisciplinary approach is crucial in the treatment of these patients and results in improved functionality and quality of life.Disclosure of InterestNone Declared

A rare case of severe congenital diaphragmatic hernia

Background. Diaphragmatic hernia is a complex congenital anomaly, often associated with other structural defects with lethal potential. Congenital diaphragmatic hernia (CHD) often causes a chain of multiple complications since intrauterine development, the most frequent of which is pulmonary hypoplasia complicated, in turn, by the installation of persistent pulmonary hypertension in the newborn after birth. Although the medical and surgical management of CDH has improved considerably, morbidity and mortality rates remain high. Case report. We presented the case of a newborn diagnosed with a massive Bochdalek-type left diaphragmatic hernia, with the ascension into the thoracic cavity of the intestine, spleen, stomach, left lobe of the liver, the antenatal diagnosis was established one month before birth, with fatal evolution within 48 hours of at birth despite the intensive support applied. Conclusions. The presented case is a very rare case of severe CHD with a fatal outcome mainly due to the wide diaphragmatic defect that allowed the thoracic ascension of the spleen, stomach, intestinal loops, and the right liver lobe associated with hypo/aplasia of the left lung, severe displacement and compression of the heart and severe pulmonary hypertension.

Outcomes and Trends of Surgical Ventricular Septal Defect Repair in a Tertiary Care Hospital in Pakistan: a Retrospective Study

Background: Ventricular septal defect is the most common congenital heart malformation in newborns. About 77% of newborns diagnosed with congenital heart disease had ventricular septal defect in Pakistan. VSD closure is one of the commonest procedure performed by pediatric cardiac surgeons. With advancement in preoperative and postoperative management, VSD closure has a very low incidence of postoperative complications. Methodology: Patients who underwent VSD closure at our center from 2017 to 2021 were reviewed in the database. Patients with a simple VSD were included and those with a concomitant complex congenital cardiac anomaly were excluded. Data was analyzed in SPSS 23. Results: Among the patients, 62.8% were males and 37.2% were females. Mean age was 10.36 + 9.69 years, with 39.7% of the subjects below the age of 5. Increased ICU stay was defined as stay in the ICU for more than 48 hours and increased hospital stay was described as postoperative stay of more than 7 days. Nine patients had a prolonged hospital stay and four patients had a prolonged ICU stay. One patient went under reopening due to bleeding tamponade and one had a deep sternum infection. In hospital mortality rate was 2.4%. Conclusion: VSD has a very low mortality rate in our center. Further studies are required to identify risk factors for morbidity and mortality. Long term follow-up studies should be conducted to identify rate of long term complications.

Fetal In-Utero Microneurosurgical Repair of Myelomeningocele and Myeloschisis: Neursurgical Outcome Updated

Conference presented at the Brazilian Academy of Neurosurgery Pediatric Symposium on December 9th, 2024. Myelomeningocele (MMC) is recognized as the most prevalent neural tube defect (NTD) and the most complex congenital anomaly that is still consistent with life. In the era of folic acid fortification, its incidence has been observed at 3.4 per 10,000 live births. The observed decrease in incidence is attributed to a synergy of enhanced folate supplementation, along with advancements in prenatal diagnostics, and, in certain cases, the option of selective termination. Recently, innovative technology and growing expertise have sparked considerable excitement within the scientific community regarding fetal MMC repair. In this lecture, Dr. Elbabaa shares his surgical outcomes and provides an updated overview of the current state of the art in this surgical field.

Treatment of Anorectal Malformations in German Hospitals: Analysis of National Hospital Discharge Data from 2016 to 2021.

Anorectal malformations (ARMs) are complex congenital anomalies. The corrective operation is demanding and schedulable. Based on complete national data, patterns of care have not been analyzed in Germany yet. All cases with ARM were analyzed (1) at the time of birth and (2) during the hospital stay for the corrective operation, based on the national hospital discharge data (DRG statistics). Patient's comorbidities, treatment characteristics, hospital structures, and the outcome of corrective operations were analyzed with respect to the hospitals' caseload. From 2016 to 2021, 1,726 newborns with ARM were treated at the time of birth in 388 hospitals. Of these hospitals, 19% had neither a pediatric nor a pediatric surgical department. At least one additional congenital anomaly was present in 49% of cases and 7% of the newborns had a birthweight below 1,500 g.In all, 2,060 corrective operations for ARM were performed in 113 hospitals in the same time period. In 24.5% of cases, at least one major complication was documented. One-third of the operations were performed in 56 hospitals, one-third in 20 hospitals, and one-third in 10 hospitals with median annual case numbers of 2, 5, and 10, respectively.Hospitals with the highest caseload operated cloacal defects more often than hospitals with the lowest caseload (7 vs. 2%) and had more early complications than hospitals with the lowest caseload (30 vs. 21%). This difference was not statistically significant after risk adjustment. Children with ARM are multimorbid. Early complications after corrective surgery are common. Considering the large number of hospitals with a very low caseload, centralization of care for the complex and elective corrective surgery for ARM remains a key issue for quality of care.