Chromosomal conditions affect 1 in 150 live births and include karyotypic findings such as aneuploidy (numerical abnormalities) and smaller chromosomal microdeletions or duplications, referred to as copy number variants (structural abnormalities). Common sex-chromosome abnormalities include Turner syndrome, triple X syndrome, and Klinefelter syndrome. These sex-chromosome disorders are compatible with life and have a range of clinical features including intellectual disability, height discrepancies, and a change in reproductive function. This group of disorders is difficult to diagnose prenatally. The most common prenatally diagnosed aneuploidies include trisomy 13, trisomy 18, and trisomy 21. Trisomy 13 and 18 are most known for their ability to be diagnosed on prenatal ultrasound by multiple anomalies and their low survival rate. Trisomy 21, the most commonly discussed aneuploidy, is characterized by a widely variable phenotype with intellectual disability and an average life span of 6 decades. Microdeletion and microduplication syndromes include a host of phenotypes that typically involve some level of intellectual disability and characteristic physical features. DiGeorge syndrome, for example, exhibits a highly variable phenotype, even between first-degree relatives. Other notable microdeletion/microduplication disorders include deletion 1p36, Wolf-Hirschhorn, cri-du-chat, Williams-Beuren, and WAGR (Wilms tumor–aniridia–genitourinary anomalies–retarded development) syndromes. All aneuploidies, including sex chromosome disorders, can be identified through standard karyotype testing. All microdeletions and microduplications require testing through a chromosomal microarray.

PURPOSE:Anhydramnios secondary to anuria before 22 weeks of gestational age and congenital bilateral renal agenesis before 26 weeks of gestational age are collectively referred to as early-pregnancy renal anhydramnios. Early-pregnancy renal anhydramnios occurs in at least 1 in 2000 pregnancies and is considered universally fatal when left untreated because of severe pulmonary hypoplasia precluding ex utero survival The Renal Anhydramnios Fetal Therapy (RAFT) trial is a nonrandomized, nonblinded, multicenter clinical trial designed to assess the efficacy, safety, and feasibility of amnioinfusions for patients with pregnancies complicated by early-pregnancy renal anhydramnios. The primary objective of this study is to determine the proportion of neonates surviving to successful dialysis, defined as use of a dialysis catheter for ≥14 days. METHODS:A consortium of 9 North American Fetal Therapy Network (NAFTNet) centers was formed, and the RAFT protocol was refined in collaboration with the NAFTNet Scientific Committee. Enrollment in the trial began in April 2020. Participants may elect to receive amnioinfusions or to join the nonintervention observational expectant management group. Eligible pregnant women must be at least 18 years of age with a fetal diagnosis of isolated early-pregnancy renal anhydramnios. FINDINGS:In addition to the primary study objective stated above, secondary objectives include (1) to assess maternal safety and feasibility of the serial amnioinfusion intervention (2) to perform an exploratory study of the natural history of untreated early pregnancy renal anhydramnios (3) to examine correlations between prenatal imaging and lung specific factors in amniotic fluid as predictive of the efficacy of serial percutaneous amnioinfusions and (4) to determine short- and long-term outcomes and quality of life in surviving neonates and families enrolled in RAFT IMPLICATIONS: The RAFT trial is the first clinical trial to investigate the efficacy, safety, and feasibility of amnioinfusions to treat the survival-limiting pulmonary hypoplasia associated with anhydramnios. Although the intervention offers an opportunity to treat a condition known to be almost universally fatal in affected neonates, the potential burdens associated with end-stage kidney disease from birth must be acknowledged. CLINICALTRIALS:gov identifier: NCT03101891.

Introduction In the past two decades, Enhanced Recovery After Surgery (ERAS) pathways for adults have improved efficiency of care and decreased length of stay (LOS) without increasing postoperative complications. The effects of enhanced recovery pathways for children are less well known. In this retrospective cohort study, we evaluated the effects of an enhanced recovery protocol (ERP) implementation in children undergoing colorectal surgery. Methods We introduced a colorectal ERP in 2017. Children and young adults (ages 2-22 years) were divided into pre-intervention (2014-2016) and post-intervention groups (2017-2019) for analysis. We abstracted data, including demographics, primary surgery, LOS, postoperative pain scores, and postoperative complications. Results A total of 432 patients were included. Of those,148 (34%) were pre-ERP implementation and 284 (66%) were post-ERP implementation. Post-ERP patients experienced significantly shorter LOS (5.7 vs. 8.3 days, p<0.01); required less intraoperative local anesthetic (9.5% vs. 38.5%, p<0.01) because 55% of patients received an epidural and 18% received an abdominal plane block; and used less postoperative opioid (62.5% vs. 98.7%, p<0.01) than did pre-ERAS patients. After protocol implementation, average pain scores were lower on postoperative day 1 (3.6 vs. 4.5, p<0.05) and across the hospitalization (3.0 vs. 4.0, p<0.01). Conclusion Enhanced recovery pathways decrease LOS, opioid use, and postoperative pain scores for children undergoing colorectal surgery and should be considered for this patient population. (C) 2022 Elsevier Inc. All rights reserved.

Objective We describe surgeon and parent perceptions of similarity toward each other and evaluate differences in the perceptions of similarity by race. Study Design Observational cohort analysis. Setting Three outpatient sites. Methods Following consultations for children undergoing evaluation for 1 of 3 surgical procedures (tonsillectomy, hernia repair, circumcision), surgeons and parents rated their perception of cultural similarity toward each other on a 6-point Likert scale. Surgeon evaluation of 9 parent characteristics was measured with 7-point Likert scales. Regression analyses were performed to identify predictors of greater surgeon-perceived similarity and to assess associations of perceived similarity with evaluation of parent characteristics. Results Most parents were women (n = 38, 84%), whereas surgeons were primarily men (n = 7, 54%). Of 45 parents, 23 (51%) were non-White, whereas only 4 of 13 clinicians (31%) were non-White. Mean perceived similarity score was 21.7 for parents (range, 10-24) and 18.2 for surgeons (range, 10-24). There was no difference in parent-perceived similarity based on race (White vs non-White parents, mean [SD] = 22.3 [3.4] vs 21.1 [3.0]; P = .26). Surgeons perceived greater similarity with White parents (odds ratio = 4.78; 95% CI, 1.02-22.54; P = .04) and parents with higher income (odds ratio = 11.84; 95% CI, 1.32-106.04; P = .03). Greater perceived similarity by the surgeons was associated with more positive assessments of parent personality characteristics. Conclusion Surgeons perceived similarity more commonly with White parents, while parents' perception of similarity to surgeons was uniform regardless of parent race. Elucidating biases of surgeons may help to tailor interventions promoting culturally competent, equitable communication and decision making for elective surgery.

Prenatal ultrasounds often yield indeterminate (incomplete or minor abnormality) findings with limited clinical utility. We evaluate impact of indeterminate findings on maternal anxiety. A single-U.S.-center prospective cohort study administered the Perinatal Anxiety Screening Scale (PASS; control mean = 13.4; > 20 denotes clinically significant anxiety) before and after prenatal ultrasounds in February-May 2017. Ultrasound reports were coded as: normal; indeterminate; or major abnormality. Primary outcome was anxiety after indeterminate vs. normal ultrasounds. Secondary outcomes included anxiety change from pre-to-post-ultrasound and relative to women’s characteristics. Linear regression adjusted for confounders. Of 286 ultrasounds, 51.0% were normal, 40.5% indeterminate (22.0% incomplete; 18.5% minor abnormality), and 8.0% major abnormalities. Indeterminate findings were unrelated to age, race, parity, infertility, or psychiatric history, but associated with gestational age (26.6%/45.0%/52.5% for first/second/third trimesters; p < 0.001), and obesity (48.8 vs. 37.0%; p = 0.031). Pretest anxiety was highest in second/third trimesters (p = 0.029), and in subjects aged age ≤ 24 or younger(p < 0.001), with a history of anxiety (p < 0.001),) or with prior pregnancy loss (p = 0.011). Mean anxiety score decreased pre-to-posttest across all groups. Indeterminate findings were associated with higher PASS scores than normal findings: pretest 20.1 vs. 16.4 (p = 0.026) and posttest 16.9 vs. 12.2 (p = 0.009; adjusted-p = 0.01). Versus normal ultrasounds, incomplete findings were associated with higher post-ultrasound anxiety (p = 0.007; adjusted-p = 0.01) and smaller decreases from pre-to-posttest (adjusted-p = 0.03), whereas minor abnormalities had higher pretest anxiety (p = 0.029) with larger pre-to-posttest decreases (adjusted-p =0.010). Indeterminate ultrasounds, especially incomplete findings, are associated with significantly higher anxiety than normal findings, suggesting need for evidence-based counseling, management and strategies for decreasing number of indeterminate results.

Congenital diaphragmatic hernias rarely present after 2 months of age and are typically diagnosed in the perinatal period. Moderate to severe diaphragmatic hernias present with respiratory symptoms, while late-onset hernias have a more varied presentation, depending on the age and content of the hernia. Very rarely, such hernias are found on incidental imaging, in which surgical repair is frequently recommended. A young girl with Loeys-Dietz syndrome and prior abdominal surgeries presents with 1-year history of increasingly severe, intermittent, abdominal and left shoulder pain. Prior imaging incidentally revealed a left diaphragmatic hernia with omentum protruding into the thoracic cavity. This was managed expectantly due to her other medical and surgical issues. Serial imaging revealed that the herniated omentum was increasing in size and symptoms began to develop. An uncomplicated primary thoracoscopic repair was performed. We report the first case of a congenital diaphragmatic hernia in a patient with Loeys-Dietz syndrome.

Introduction: Parent experience is a core component of the quality of pediatric care and an increasingly common focus of quality improvement initiatives. However, the parent experience of communication in the pediatric surgical setting remains unexplored. Methods: We conducted semi-structured interviews with 20 parents of children undergoing surgery. Interviews were analyzed using directed qualitative content analysis. Results: Content analysis revealed 3 overarching themes. The theme of “provider–parent communication” included interpersonal behaviors and communication-originating skills of the surgeon. Parents valued surgeons incorporating multimodal information-sharing techniques, recognizing children’s psychological needs, providing reassurance, engaging in teamwork, and including parents. The theme of “parental emotional experiences” included domains of parent worry, intimidation, offense, self-doubt, mistrust, and strength surrounding their child’s surgery. Parents felt simultaneously responsible for their child’s welfare and for understanding medical information. The theme of “process improvement” included preparation for surgery, efficiency, managing delays, anesthesia induction, emergence from anesthesia, privacy, and preparation for recovery. Conclusions: Themes identified through these parental narratives and proposed solutions inform quality improvement efforts related to surgeon communication strategies and facilitate family-centered surgical care for children. Parents often provided solutions after they described concerns, which attests to the utility of parent perspectives.

Early pregnancy renal anhydramnios (EPRA) occurs when congenital anomalies of the kidneys and urogenital tract (CAKUT) result in fetal anuria and absence of amniotic fluid prior to 22 weeks gestational age. The underlying etiologies of these conditions include environmental and multi-genetic factors. In this study, we sought to elucidate the genetic underpinnings of EPRA through prenatal chromosomal microarray (CMA), multi-gene panel testing, and whole-exome sequencing (WES). We hypothesized a low diagnostic yield consistent with the previously reported genetic diagnostic yield for CAKUT. We performed a retrospective cohort study of all patients referred to our tertiary-care medical center for evaluation and treatment under the Renal Anhydramnios Fetal Therapy (RAFT) trial (NCT03101891) from 11/2018 to 9/2020. The medical records of patients undergoing RAFT evaluation were reviewed for prenatal or postnatal diagnostic testing results, and three-generation pedigrees were reviewed to identify additional affected family members. During the study period, 64 patients were referred to the RAFT trial, and 32 (50%) presented for evaluation. Prenatal diagnosis was completed via karyotype (n=5), CMA (n=18), and/or WES (n=5) in 22 patients (69%), and 10 patients declined diagnostic testing. Diagnostic results were identified in 4/22 (18%) cases, including 1 fetus with an abnormal karyotype (47,XXX), 2 with pathogenic CMA findings (a 15q26.1 deletion and a 2q21.1 duplication), and 1 with a pathogenic heterozygous variant in GREB1L identified on postnatal WES. Pedigree review revealed CAKUT presentations in additional family members in 7/32 (22%) cases. Genetic diagnosis was achieved for 4 EPRA-affected pregnancies in the RAFT trial cohort. Future research is needed, including whole-exome sequencing of all presenting cases, to identify novel causative mutations and enable improved reproductive decision-making and better genetic and obstetric care provision for at-risk couples and affected fetuses.

Objective Congenital diaphragmatic hernia (CDH) is a congenital defect associated with significant mortality and morbidity. We sought to assess the health-related quality of life (HRQoL) for caregivers of infants/children with CDH and determine risk factors for poorer HRQoL. Methods Families were recruited from a CDH-specific outpatient clinic and HRQoL was assessed by a validated HRQoL instrument (PedsQLTM Family Impact Module) at several time points. Mixed models were used to identify demographic and clinical factors associated with worse HRQoL for caregivers. Results A total of 29 subjects were recruited at a mean age of 2.4 +/- 2.3 years. In terms of defect size, 6.9% had a Type A, 37.9% a Type B, 31.0% a Type C, and 24.1% a Type D. The mean HRQoL score at the first encounter was 67.6 +/- 18.3; scores are reported from 0 to 100 with higher scores representing the higher reported quality of life. Lower median household incomes (p = .021) and use of extracorporeal membrane oxygenation (p = .013) were associated with poorer HRQoL scores. The presence of respiratory symptoms decreased HRQoL for caregivers, including daytime symptoms (p < .001) and nighttime symptoms (p < .001). While emergency department visits were not associated with a decrease in HRQoL, hospital admissions (p = .002), and reoperations for CDH (p < .001) were. Conclusion Our study found a reduced quality of life associated with socioeconomic factors and severity of ongoing disease. Further study is needed to confirm these findings and identify strategies for aiding families cope with the chronicity of this congenital disease.

Blunt impact-induced traumatic abdominal wall hernia (TAWH) is an uncommon pediatric surgical problem classically associated with handlebar injury but increasingly seen with seatbelt use in motor vehicle collisions (MVC). Herein we describe the largest case series of pediatric TAWH to date and review the literature to establish the unique syndromic characteristics of MVC-associated TAWH. In this single-institution series, we discuss four pediatric patients, all with seatbelt-associated TAWH after high-speed MVC characterized by full-thickness disruption of the lateral abdominal wall. We then performed a review of the literature to identify additional pediatric MVC-associated TAWH and define the characteristics of patients who sustained this unique injury. In addition to the four patients in our case series, five additional pediatric patients presenting with TAWH after restrained MVC were identified in the literature. Of these nine patients, eight (89%) presented with an obvious seatbelt sign (bruising/laceration to the abdominal wall). Six (67%) had associated injuries typical of the seatbelt syndrome, including four spinal flexion injuries (44%) and five bowel injuries requiring repair or resection (56%). Overall, 56% of seatbelt-associated TAWH occurred in children with a BMI percentile > 95%. In this case series and literature review, we note a high rate of seatbelt syndrome injuries in pediatric patients presenting with TAWH after restrained MVC. Suspicion for TAWH should be high in children presenting with a seatbelt sign and should trigger a low threshold for pursuing additional axial imaging. Level IV; case series.

Introduction: Prenatal closure of open spina bifida via open fetal surgery improves neurologic outcomes for infants in selected pregnancies. Fetoscopic techniques that are minimally invasive to the uterus aim to provide equivalent fetal benefits while minimizing maternal morbidities, but the optimal technique is undetermined. We describe the development, evolution, and feasibility of the laparotomy-assisted 2-port fetoscopic technique for prenatal closure of fetal spina bifida in a newly established program. Methods: We conducted a retrospective cohort study of women consented for laparotomy-assisted fetoscopic closure of isolated fetal spina bifida. Inclusion and exclusion criteria followed the Management of Myelomeningocele Study (MOMS). Team preparation involved observation at the originating center, protocol development, ancillary staff training, and surgical rehearsal using patient-matched models through simulation prior to program implementation. The primary outcome was the ability to complete the repair fetoscopically. Secondary maternal and fetal outcomes to assess performance of the technique were collected prospectively. Results: Of 57 women screened, 19 (33%) consented for laparotomy-assisted 2-port fetoscopy between February 2017 and December 2019. Fetoscopic closure was completed in 84% (16/19) cases. Over time, the technique was modified from a single- to a multilayer closure. In utero hindbrain herniation improved in 86% (12/14) of undelivered patients at 6 weeks postoperatively. Spontaneous rupture of membranes occurred in 31% (5/16) of fetoscopic cases. For completed cases, median gestational age at birth was 37 (range 27-39.6) weeks and 50% (8/16) of women delivered at term. Vaginal birth was achieved in 56% (9/16) of patients. One newborn had a cerebrospinal fluid leak that required postnatal surgical repair. Conclusion: Implementation of a laparotomy-assisted 2-port fetoscopic spina bifida closure program through rigorous preparation and multispecialty team training may accelerate the learning curve and demonstrates favorable obstetric and perinatal outcomes.

BACKGROUND:Congenital pulmonary airway malformation (CPAM) is the most common prenatally-diagnosed lung malformation. This lesion, classified as macrocystic or microcystic, can lead to significant fetal compromise. Management options include observation, maternal antenatal steroid administration, and fetal surgical intervention. Current evidence suggests that microcystic (but not macrocystic) lesions and those with a cyst volume ratio (CVR) >1.6 are responsive to steroid therapy. The objective of this study was to identify patterns of prenatal steroid administration for the management of CPAMs and to identify characteristics of CPAMs prompting steroid administration.METHODS:An 18-question survey was distributed to obstetricians from the Pregnancy-Related Care Research Network (PRCRN) and the North American Fetal Therapy Network (NAFTNet), from January to April 2019, to capture antenatal steroid prescribing patterns.RESULTS:Response rates were 28.3% (138/487) for PRCRN and 63.3% (19/30) for NAFTNet. Among PRCRN members, 16.8% administered prenatal steroids, with most (77.2%) doing so for both microcystic and macrocystic CPAMs; corresponding percentages for NAFTNet members were 90.9% and 52.6%. Two thirds (65.6%) of obstetricians who administer steroids do so for a CVR > 1.6, without evidence of mediastinal shift or hydrops fetalis.CONCLUSIONS:There is a lack of consensus among obstetricians as to the CPAM characteristics that should prompt administration of prenatal steroids. Many surveyed obstetricians do not use cyst type or CVR to guide decision-making regarding steroid therapy.

Background Early pregnancy renal anhydramanios (EPRA) occurs when the fetus is anuric before 22 weeks gestational age (GA) and is considered universally lethal. Serial amnioinfusions have successfully ameliorated the lethal pulmonary hypoplasia associated with EPRA and have resulted in cases of neonatal survival, peritoneal dialysis, and renal transplant. Objective We sought to evaluate the lung pathology of untreated fetuses and neonates that had EPRA. Study design This is a retrospective case series of all fetuses and neonates diagnosed with isolated EPRA that underwent autopsy at a single tertiary care center between 1987 and 2018. Autopsy data were correlated with ultrasound findings and GA at delivery. Fetal weights, lung weights, and lung developmental stage were recorded. Results Nineteen cases met criteria for analysis and ranged from 16 to 38 weeks GA at termination or birth. The observed-to-expected (O/E) lung-to-body-weight ratio was significantly associated with GA ( r = −0.51, p = 0.03), such that as GA increased the O/E ratio decreased. When limited to patients >22 weeks, this relationship strengthened ( r = −0.75, p = 0.01). Importantly, overall O/E body weight had no relationship with GA. Conclusion This study shows that the degree of pulmonary hypoplasia in EPRA increases with the length of anhydramnios. This suggests that amnioinfusions are likely to be of most benefit the soonest they can feasibly be initiated.

Necrotizing enterocolitis (NEC) is an important cause of morbidity and mortality in the premature population. This chapter focuses on important antenatal and intrapartum risk factors for NEC development. These factors include mode of delivery, growth restriction and small-for-gestational-age infants, use of antenatal corticosteroids, mode of delivery, chorioamnionitis, and delayed cord clamping. By understanding these maternal-fetal–related risk factors, along with those factors present in the postnatal period, we hope to provide a more complete understanding of the factors that lead to the development of this devastating disease.

Abstract Objectives We sought to characterize patterns of in utero dilation in isolated severe fetal ventriculomegaly (ISVM) and investigate their value in predicting obstetrical and postnatal outcomes. Methods This is a retrospective cohort study. ISVM was defined as a sonographic cerebral ventricle atrial with width ≥15 mm in the absence of additional cerebral or other anatomic anomalies. The aim of this study was to characterize two ISVM groups using a receiver operator curve to evaluate the rate of ventricular progression versus need for ventriculoperitoneal (VP) shunt postnatally. Outcomes were compared between the groups using Pearson’s chi-squared test, Student t-test, and descriptive statistics. Results Based on the ROC analysis, ventricular growth of ≥3 mm/week versus <3 mm/week distinguished fetuses likely to require a postnatal VP shunt. Fetuses were characterized as accelerators if ventricle growth was ≥3 mm/week at any point and plateaus if <3 mm/week. Accelerators showed a greater average rate of ventricle progression than plateaus (4.1 vs. 1.0 mm/week, respectively, p = .031) and were more likely to be delivered at earlier gestational ages (34.7 vs. 37.1 weeks respectively, p = .02). Ninety percent of accelerators demonstrated a need for shunt placement compared with 18.8% of plateaus (p < .001). Significantly more plateaus (87.5%) underwent a trial of labor while accelerators were more likely to have planned cesareans (70%, p = .009). Conclusions This study characterizes ISVM into two distinct populations based upon the rate of ventricle expansion, differentiated by the need for postnatal shunting. Once a ventricular growth pattern is determined, these distinctions should prove useful in prenatal management and delivery planning.

(Obstet Gynecol. 2020;135:511–521) Congenital diaphragmatic hernia, caused by the absence of the diaphragm or part of the diaphragm, leads to impaired fetal lung development as the abdominal contents move into the chest cavity. This is commonly managed with high-frequency oscillatory ventilation, pulmonary vasodilators, and extracorporeal membrane oxygenation, but the survival rate is <20% in cases with an observed lung/head ratio of <25% and intrathoracic liver herniation. In temporary fetoscopic tracheal balloon occlusion (FETO), the lungs are stretched and increase growth in airways and lung vasculature takes place. FETO has demonstrated increased survival rates, but is also associated with adverse outcomes such as preterm labor, rupture of membranes, and preterm birth. This study aimed to determine feasibility of FETO in cases of severe congenital diaphragmatic hernia.

Congenital abnormalities of the kidney and urinary tract (CAKUT) represent 20% of prenatally diagnosed congenital abnormalities. Although the majority of these abnormalities do not require intervention either pre or postnatally, there is a subset of patients whose disease is so severe that it may warrant intervention prior to delivery to prevent morbidity and mortality. These cases consist of patients with moderate lower urinary tract obstruction (LUTO) in which vesicocentesis, shunting or cystoscopy are options and patients with early pregnancy renal anhydramnios (EPRA) in whom amnioinfusion therapy may be an option. The main causes of EPRA are congenital bilateral renal agenesis (CoBRA), cystic kidney disease (CKD) and severe LUTO. Untreated, EPRA is universally fatal secondary to anhydramnios induced pulmonary hypoplasia. The evidence regarding therapy for LUTO is limited and the stopped early PLUTO (Percutaneous Shunting in Lower Urinary Tract Obstruction) trial was unable to provide definitive answers about patient selection. Evidence for EPRA therapy is also scant. Serial amnioinfusions have shown promise in cases of EPRA due to COBRA or renal failure and this treatment modality forms the basis of the ongoing NIH funded RAFT (Renal Anhydramnios Fetal Therapy) trial. At present, there is consensus that treatment for EPRA should only occur in the setting of a clinical trial.

BackgroundAnorectal malformations (ARMs) have a wide spectrum of presentation ranging from mild defects with perineal fistulas to more severe defects requiring complex management. A primary repair of ARMs with perineal or rectovestibular fistulas has been shown to have good outcomes. However, the timing of the reconstruction is still debated. The aim of this study is to investigate the safety of early versus delayed repair.MethodsThis study was performed using data from the National Surgical Quality Improvement Program-Pediatric (NSQIP-P) from 2012 to 2017. Patients who underwent repair of anorectal malformation with perineal or vestibular fistula were included in the study. Patients with associated diagnosis for Hirschsprung disease, cloaca, rectal prolapse or stenosis, bladder exstrophy, and tracheoesophageal fistula were excluded. 30-day postoperative outcomes included wound and nonwound complications, readmissions, and reoperations. Outcomes were compared by early (≤7 days of age) versus delayed repair (6 weeks to 8 months).ResultsA total of 291 patients were included, with 66 in the early and 231 in the delayed group. Patients in the early group were more likely to be male (68.2% vs 31.8%; p < 0.01) and have cardiac risk factors (71.2% vs 49.4%, p < 0.01). The mean operative time was significantly shorter in the early group (90.1 vs 129.6 min; p < 0.01). 30-day complications were not statistically significant between the two groups (p = 0.76). After multivariate analysis, timing of repair did not affect 30-day complications (p = 0.15).ConclusionOur study shows that early repair of low anorectal malformations with a perineal or vestibular fistula appears to be associated with no increase in risk of postoperative complications as compared to delayed repair. At present, the decision remains dependent on the surgeon's experience and judgment.Level of evidenceLevel III. Retrospective comparative study.