Outcomes of children with constipation and autism spectrum disorder treated with antegrade continence enemas.

BACKGROUND: Treatment of functional constipation (FC) in children with autism spectrum disorder (ASD) is challenging due to sensory and behavioral issues. We aimed to understand whether antegrade continence enemas (ACEs) are successful in the treatment of FC in children with ASD. METHODS: A single-institution retrospective review was performed in children diagnosed with ASD and FC who underwent appendicostomy or cecostomy placement from 2007 to 2019. Descriptive statistics regarding soiling and complications were calculated. RESULTS: There were 33 patients included, with a median age of 9.7 years at the time of ACE initiation. The average intelligence quotient was 63.6 (SD = 18.0, n = 12), the average behavioral adaptive score was 59.9 (SD = 11.1, n = 13), and the average total Child Behavioral Checklist score was 72.5 (SD = 7.1, n = 10). Soiling rates were significantly lower following ACE initiation (42.3% vs. 14.8%, p = 0.04). Behavioral issues only prevented 1 patient (3.0%) from proper ACE use. Eleven patients (36.6%) were able to transition to laxatives. There were significant improvements in patient-reported outcomes measures and quality of life. CONCLUSION: Placement of an appendicostomy or cecostomy for management of FC in children with severe ASD was successful in treating constipation and improving quality of life.

An Unusual Case of Extranodal Marginal Zone Lymphoma Mimicking Abdominal Cocoon Syndrome in an Adolescent Patient.

Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is an indolent non-Hodgkin lymphoma rarely seen in pediatric patients. MALT lymphoma most commonly involves the gastrointestinal tract or peri-orbital tissues, potentially as sequela of chronic antigenic stimulation or immune dysregulation. Rare cases of MALT lymphoma arising from the gynecologic tract have been reported in older adult patients. We present the unique case of a 16-year-old postpubescent female with MALT lymphoma localized to the gynecologic tract, who initially presented with abdominal fullness, abnormal uterine bleeding, and obstructive acute kidney injury secondary to urinary outflow obstruction. Intraoperatively, dense fibrosis of the uterus and left fallopian tube was noted which mimicked abdominal cocoon syndrome. She was treated with 6 cycles of bendamustine and rituximab with complete anatomic and metabolic remission. In this report we highlight a very unusual presentation of a rare malignancy in the pediatric population as well as unique treatment considerations given this patient's young age and tumor location.

Potential of siRNA-Bearing Subtilosomes in the Treatment of Diethylnitrosamine-Induced Hepatocellular Carcinoma.

Therapeutics, based on small interfering RNA (siRNA), have demonstrated tremendous potential for treating cancer. However, issues such as non-specific targeting, premature degradation, and the intrinsic toxicity of the siRNA, have to be solved before they are ready for use in translational medicines. To address these challenges, nanotechnology-based tools might help to shield siRNA and ensure its specific delivery to the target site. Besides playing a crucial role in prostaglandin synthesis, the cyclo-oxygenase-2 (COX-2) enzyme has been reported to mediate carcinogenesis in various types of cancer, including hepatocellular carcinoma (HCC). We encapsulated COX-2-specific siRNA in Bacillus subtilis membrane lipid-based liposomes (subtilosomes) and evaluated their potential in the treatment of diethylnitrosamine (DEN)-induced hepatocellular carcinoma. Our findings suggested that the subtilosome-based formulation was stable, releasing COX-2 siRNA in a sustained manner, and has the potential to abruptly release encapsulated material at acidic pH. The fusogenic property of subtilosomes was revealed by FRET, fluorescence dequenching, content-mixing assay, etc. The subtilosome-based siRNA formulation was successful in inhibiting TNF-α expression in the experimental animals. The apoptosis study indicated that the subtilosomized siRNA inhibits DEN-induced carcinogenesis more effectively than free siRNA. The as-developed formulation also suppressed COX-2 expression, which in turn up-regulated the expression of wild-type p53 and Bax on one hand and down-regulated Bcl-2 expression on the other. The survival data established the increased efficacy of subtilosome-encapsulated COX-2 siRNA against hepatocellular carcinoma.

Impact of Neurodevelopmental Disorders on Bowel Management Outcomes in Children with Functional Constipation.

OBJECTIVES: Patients experiencing functional constipation (FC) can participate in structured bowel management programs (BMPs) to manage constipation or fecal incontinence when standard management fails. We sought to evaluate the efficacy of BMPs for children with FC with and without neurodevelopmental disorders. METHODS: We performed a retrospective review of children with FC who participated in our BMP from 2014 to 2021. Stool/urinary continence, bowel regimen, surgical history, parent-reported outcomes measures (PROMs: Cleveland Clinic Constipation Score, Baylor Continence Scale, Vancouver Symptom Score for Dysfunctional Elimination), and Pediatric Quality of Life Inventory (PedsQL) were assessed pre- and at least 9 months post-BMP. RESULTS: The cohort included 156 patients with a median age of 9 years and follow-up of 627 days (IQR: 389-808 days). Two sub-cohorts included patients with FC only (69%) and FC plus a neurodevelopmental disorder (31%): 59% attention-deficit/hyperactivity disorder, 33% autism spectrum disorder, and 8% obsessive-compulsive disorder. Both groups had significantly improved follow-up bowel movement frequency and continence (39%-90% neurodevelopmental, 44%-82% FC only, P < 0.001) and urinary continence (65%-90% neurodevelopmental, 69%-91% FC only, P < 0.02). There was a significant improvement in most of the PROMs at follow-up. Both groups experienced a clinically meaningful improvement in overall PedsQL scores (pre- and postBMP difference of >4.5). CONCLUSIONS: Patients with FC with and without a neurodevelopmental disorder had significant improvement in stool and urinary continence after undergoing a BMP. Further studies are needed to see if this improvement is durable over a longer period of time in this challenging cohort.

Improvement in bladder function in children with functional constipation after a bowel management program.

PURPOSE: We sought to determine if children with functional constipation (FC) would have an improvement in bladder function with treatment of constipation with a bowel management program (BMP). METHODS: A single-institution review was performed in children aged 3-18 with FC who underwent a BMP from 2014 to 2020. Clinical characteristics, bowel management details, and the Vancouver Symptom Score for Dysfunctional Elimination Syndrome (VSS), Baylor Continence Scale (BCS), and Cleveland Clinic Constipation Score (CCCS) were collected. Data were analyzed using linear mixed effect modeling with random intercept. RESULTS: 241 patients were included with a median age of 9 years. Most were White 81 and 47% were female. Univariate tests showed improvement in VSS (- 3.6, P < 0.0001), BCS (- 11.96, P < 0.0001), and CCCS (- 1.9, P < 0.0001) among patients having undergone one BMP. Improvement was noted in VSS and CCCS among those with more than one BMP (VSS: - 1.66, P = 0.023; CCCS: - 2.69, P < 0.0001). Multivariate tests indicated undergoing a BMP does result in significant improvement in VSS, BCS, and CCCS (P < 0.0001). CONCLUSIONS: There is significant improvement in bladder function in children with FC who undergo a BMP. For patients with bowel and bladder dysfunction and FC, a BMP is a reasonable treatment strategy for lower urinary tract symptoms.

Evaluation and Management of Persistent Problems After Surgery for Hirschsprung Disease in a Child.

PURPOSE OF REVIEW: Ideally, after operative intervention, a child born with Hirschsprung disease (HD) should thrive, achieve fecal continence, and avoid recurrent episodes of abdominal distention and enterocolitis. However, a significant number of patients continue to struggle following their pull-through procedure. The purpose of this review is to present an organized and practical approach to the evaluation and management of the symptomatic patient post pull-through operation for HD. RECENT FINDINGS: Children diagnosed with HD who are not doing well after their initial operation can be categorized in three distinct groups: (1) those that have fecal incontinence, (2) those with obstructive symptoms, and (3) those with recurrent episodes of enterocolitis. It is important to have a systematic diagnostic approach for these patients based on a comprehensive protocol. All three of these patient groups can be treated with a combination of either medical management, reoperation when a specific anatomic or pathologic etiology is identified, or botulinum toxin for non-relaxing sphincters contributing to the obstructive symptoms or recurrent enterocolitis. For patients not doing well after their initial pull-through, a systematic workup should be employed to determine the etiology. Once identified, a multidisciplinary and organized approach to management of the symptomatic patients can alleviate most post pull-through symptoms.

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

BACKGROUND: 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. OBJECTIVE: The objective of the study was to determine the correlation and agreement in sacral ratio calculations. MATERIALS AND METHODS: 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. RESULTS: 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 0.07 units 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. CONCLUSION: Even though the AP and lateral sacral ratios had moderate positive correlation, the mean sacral ratio determined by images in the lateral plane was 0.07 units 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.

Does Hirschsprung-Associated Enterocolitis Differ in Children With and Without Down Syndrome?

BACKGROUND: Children with Hirschsprung disease (HD) and Down syndrome (DS) are reported to experience a higher incidence of Hirschsprung-associated enterocolitis (HAEC) than those without DS, although whether their individual episodes are more severe is unknown. METHODS: A single-institution retrospective cohort study was performed on all patients with HD admitted with HAEC between 2009 and 2017. Patients were divided into two groups based on an associated diagnosis of DS, and demographics, clinical characteristics at presentation, and clinical outcomes were compared. RESULTS: Of 86 admissions for HAEC over the study period, 12 (14%) were for patients with DS. Patients admitted with and without DS were similar in terms of age (3.0 versus 2.6 y, P = 0.72), male gender (58% versus 77%, P = 0.17), and proportion with disease proximal to the left colon (9% versus 12%, P = 0.70). Patients admitted with DS were more likely to be tachycardic (75% versus 19%, P ≤ 0.001) and hypotensive (33% versus 7%, P = 0.01) at presentation and presented for treatment after a longer duration of symptoms than those without DS (84 versus 24 h, P = 0.001). Patients admitted with DS were more likely to require intensive care unit admission (58% versus 12%, P = 0.006) and received a longer course of antibiotics (13 d versus 7 d, P = 0.02). The length of stay was similar (6 versus 4 d, P = 0.34). CONCLUSIONS: The results of our study suggest that patients with DS experience more severe episodes of HAEC. Ongoing study is needed to identify preventive strategies to decrease the morbidity of HAEC in children with HD and DS.

The relationship between dispositional empathy and prefrontal cortical functioning in patients with frontal lobe epilepsy.

BACKGROUND & OBJECTIVE: Rehabilitation focuses brain-behavior relationship which highlights interaction between psychological and neurobiological factors for better patient care. There is a missing link in the literature about socio-cognitive aspects of frontal lobe epilepsy. Our objective was to examine prefrontal cortical functioning (PCF) and empathic abilities in patients with frontal lobe epilepsy (FLE). Further, we analyzed whether any relationship between components of dispositional empathy and PCF exists in patients with FLE. METHODS: The study was designed in an experimental paradigm. Sixty patients with FLE were recruited from Sheikh Zayed and Jinnah hospital, Pakistan. Sixty healthy individuals in response to an advertisement took part in the study as control subjects. Participants completed interpersonal reactivity index. Following they performed clock drawing test and word-color identification task switching experiment. RESULT: Patients with FLE demonstrated weaker PCF (i.e., cognitive flexibility and executive function) as compared to healthy control subjects. Patients with FLE scored lesser on cognitive empathy as compared to healthy control subjects. On contrary, there was no significant difference between patient and control group on affective empathy. Cognitive not affective empathy was potential predictor of PCF. CONCLUSION: Cognitive empathy is a significant marker of prefrontal cortical functioning (PCF) in FLE. Higher cognitive empathy would lead to efficient PCF.

Frontal-subcortical defects correlate with task switching deficits in Parkinson`s disease.

OBJECTIVE: To examine correlation between frontal-subcortical/ parietal-cortical functioning and task switching. METHODS: An experimental study was designed to examine objectives with 80 participants (40 patients with idiopathic Parkinson`s disease (PD) and 40 healthy controls). Patients were recruited from February until September 2016 at Recep Tayyip Erdogan Hospital Pakistan, Muzaffargarh, Pakistan and healthy controls participated from community. Participants were administered Parkinson`s disease cognitive rating scale and Word-Digit categorization task switching experiment. RESULTS: In contrast to healthy controls, (i) PD patients showed impaired cognitive performance on frontal-subcortical and posterior-cortical functions as assessed through PD cognitive rating scale (ii) PD patients showed task switching deficits. Frontal-subcortical defects correlated with task switching deficits. Lesser the scores on frontal-subcortical functions, larger switch costs will appear. Frontal-subcortical defects significantly predicted task switch costs. CONCLUSION: Frontal-subcortical not the posterior-cortical dysfunctions are significant marker of task switching deficits.

Thought suppression predicts task switching deficits in patients with frontal lobe epilepsy.

OBJECTIVE: To examine the relationship between task switching and thought suppression in connection with frontal lobe epilepsy (FLE). METHODS: This experimental study included 30 patients with FLE admitted to the Services and Jinnah Hospital, Lahore, Pakistan between February and November 2013, and 30 healthy individuals from the local community. Participants performed a task switching experiment where they switched between emotion and age categorizations among faces. In addition, they completed a thought suppression questionnaire. RESULTS: There were 3 important results: (i) Patients with FLE showed weaker task switching abilities than healthy individuals. This result is attributed toward executive dysfunctions in patients with FLE. (ii) Contrary to the control group, patients with FLE showed larger switch cost for the age than the emotion categorization. This result can be seen in the context of social cognition deficits and poor inhibitory control in patients with FLE. In addition, larger switch costs reflected a binding effect with facial emotion as compared to age. The integration might represent emotion as an intrusive facial dimension that interrupted task switching performance. (iii) Patients with FLE had more recurrent suppression of thoughts than controls. Thought suppression was a significant predictor for switch costs. High scores on thought suppression were correlated with task switching deficits. CONCLUSION: The results suggest that thought suppression causes significant cognitive decline.

Cognitive deficits and emotion regulation strategies in patients with psychogenic nonepileptic seizures: a task-switching study.

This study examined the task-switching ability and emotion regulation strategies in 72 patients with psychogenic nonepileptic seizures (PNES) and 72 healthy individuals, where participants categorized emotion and age dimensions among faces. Results demonstrated cognitive impairment in terms of the interrupted ability to switch between emotion and nonemotion face categorizations in patients with PNES. In contrast, healthy individuals exhibited efficient switching between these face categorizations. In patients with PNES, there was an asymmetric relationship between emotion and age tasks, while this asymmetry was absent in the healthy group. The results demonstrated that patients with PNES used expressive suppression to regulate their emotions more frequently than the control group. On the other hand, patients with PNES less frequently reappraised their cognitions than healthy individuals. Switching deficits in patients with PNES were positively correlated with expressive suppression but were negatively correlated with cognitive reappraisal. This is the first study demonstrating the presence of switching deficits in terms of inferior cognitive control of emotion in patients with PNES as compared to healthy individuals. The switching deficits are associated with emotion regulation strategies. These findings suggest that emotion regulation strategies are significant markers of switching deficits in patients with PNES.

Displaced aggression predicts switching deficits in people with temporal lobe epilepsy.

This study examined the relationship between task-switching abilities and displaced aggression in people with temporal lobe epilepsy (PWE). Participants (35 PWE and 35 healthy controls) performed emotion and gender classification switching tasks. People with temporal lobe epilepsy showed larger switch costs than controls. This result reflected task-switching deficits in PWE. People with temporal lobe epilepsy reported higher anger rumination, revenge planning, and behavioral displaced aggression compared with controls. Displaced aggression was a significant predictor of the task switch costs. It is suggested that displaced aggression is a significant marker of task-switching deficits.

Examining Results of Post-Thelarche Screening Pelvic Ultrasound in Females with Anorectal Malformations.

STUDY OBJECTIVE: Mullerian duct anomalies are common in females with anorectal malformations (ARMs), although there are no universally recommended screening protocols for identification. Historically, at our institution, we have recommended a screening pelvic ultrasound (PUS) 6 months after thelarche and menarche. We aimed to evaluate outcomes associated with our post-thelarche screening PUS in females with ARMs. METHODS: An institutional review board-approved retrospective chart review was performed for all female patients 8 years old or older with ARMs and documented thelarche. Data were collected on demographic characteristics and clinical course. The primary outcome was adherence to the recommended PUS. Secondary outcomes included imaging correlation with suspected Mullerian anatomy and need for intervention on the basis of imaging findings. RESULTS: A total of 112 patients met the inclusion criteria. Of them, 87 (77.7%) completed a recommended post-thelarche screening PUS. There were no differences in completion on the basis of age, race, establishment with a primary care provider, insurance status, or type of ARM. Nine patients (10.3%) had findings on their PUS that did not correlate with their suspected Mullerian anatomy; five (5.7%) required intervention, with two requiring menstrual suppression, two requiring surgical intervention, and one requiring further imaging. CONCLUSION: Most patients completed the recommended post-thelarche screening PUS. In a small subset of patients, PUS did not correlate with suspected Mullerian anatomy and generated a need for intervention. Post-thelarche PUS can be a useful adjunct in patients with ARMs to identify gynecologic abnormalities.

Laparoscopic-Assisted Colonic Derotation in Patients With Hirschsprung Disease.

BACKGROUND: Children with Hirschsprung disease (HSCR) proximal to the splenic flexure or those needing a redo pull-through (PT) are at risk for tension and ischemia of the PT which could result in leak, stricture, or loss of ganglionated bowel. Colonic derotation is a technique used to minimize tension and avoid duodenal obstruction. The aim of this study was to describe this technique and outcomes in a series of patients requiring this intervention. METHODS: All patients underwent initial diversion and colonic mapping. The derotation procedure involves mobilization of the remaining colon, counterclockwise rotation via the stoma closure site, placement of the pull through (the right colon) lying on the right of the pelvis, and ligation of the middle colic artery with preservation of the marginal branch running from the ileocolic artery. This maneuver prevents compression of the duodenum by the mesenteric vessels and allows for an isoperistaltic, tension-free anastomosis. Intraoperative indocyanine green fluorescence angiography (ICG-FA) was utilized in many of the cases to map the blood supply of the pull-through colon. We reviewed outcomes for all children with HSCR who underwent colonic derotation from 2014 to 2023. Descriptive statistics were performed. RESULTS: There were 37 children included. Most were male (67.5%) with the original transition zone proximal to the rectosigmoid (81.1%). The median age at PT was 9.3 months [6.1-39.7]. Median operative time was 6.6 h [4.9-7.4] and 19 cases (51.4%) used ICG-FA. Most children had no 30-day postoperative complications (67.6%); in those who did develop complications, readmissions for electrolyte imbalance was most common (50.0%). There were zero cases of anastomotic leak at PT anastomosis. At long-term follow up, median 4.4 years [2.3-7.0], three children (8.1%) developed an anastomotic stricture, all were amenable to anal dilation, and five experienced episodes of enterocolitis (14.7%). Most children had between 1 and 4 stools per day (58.8%). CONCLUSION: Colonic derotation is a useful strategy to ensure well-perfused colonic length, protect the marginal artery blood supply, avoid duodenal compression, and ensure a tension-free anastomosis with minimal complications. TYPE OF STUDY: Original research, retrospective cohort. LEVEL OF EVIDENCE: III.

Antegrade Continence Enema Alone for the Management of Functional Constipation and Segmental Colonic Dysmotility (ACE-FC): A Pediatric Colorectal and Pelvic Learning Consortium Study.

PURPOSE: The purpose of the study was to determine if antegrade continence enema (ACE) alone is an effective treatment for patients with severe functional constipation and segmental colonic dysmotility. METHODS: A retrospective study of patients with functional constipation and segmental colonic dysmotility who underwent ACE as their initial means of management. Data was collected from six participating sites in the Pediatric Colorectal and Pelvic Learning Consortium. Patients who had a colonic resection at the same time as an ACE or previously were excluded from analysis. Only patients who were 21 years old or younger and had at least 1-year follow-up after ACE were included. All patients had segmental colonic dysmotility documented by colonic manometry. Patient characteristics including preoperative colonic and anorectal manometry were summarized, and associations with colonic resection following ACE were evaluated using Fisher's exact test and Wilcoxon rank-sum test. p-Values of less than 0.05 were considered significant. Statistical analyses and summaries were performed using SAS version 9.4 (SAS Institute Inc., Cary, North Carolina, United States). RESULTS: A total of 104 patients from 6 institutions were included in the study with an even gender distribution (males n = 50, 48.1%) and a median age of 9.6 years (interquartile range 7.4, 12.8). At 1-year follow-up, 96 patients (92%) were successfully managed with ACE alone and 8 patients (7%) underwent subsequent colonic resection for persistent symptoms. Behavioral disorder, type of bowel management, and the need for botulinum toxin administered to the anal sphincters was not associated with the need for subsequent colonic resection. On anorectal manometry, lack of pelvic floor dyssynergia was significantly associated with the need for subsequent colonic resection; 3/8, 37.5% without pelvic dyssynergia versus 1/8, 12.5% (p = 0.023) with pelvic dyssynergia underwent subsequent colonic resection. CONCLUSION: In patients with severe functional constipation and documented segmental colonic dysmotility, ACE alone is an effective treatment modality at 1-year follow-up. Patients without pelvic floor dyssynergia on anorectal manometry are more likely to receive colonic resection after ACE. The vast majority of such patients can avoid a colonic resection.

Pediatric colorectal robotic surgery.

Robotic colorectal surgery allows adult and pediatric surgeons to overcome the technical limitations of laparoscopic surgery. It also provides improved ergonomics in the field of surgery. Robotic surgery has several advantages in colorectal operations that require complex minimally invasive skills including anorectal malformations, Hirschsprung disease, and inflammatory bowel disease. In this section, we discuss the key aspects of colorectal surgery where robotic instrumentation seems ideal.

Amphotericin B Nano-Assemblies Circumvent Intrinsic Toxicity and Ensure Superior Protection in Experimental Visceral Leishmaniasis with Feeble Toxic Manifestation.

In spite of its high effectiveness in the treatment of both leishmaniasis as well as a range of fungal infections, the free form of the polyene antibiotic amphotericin B (AmB) does not entertain the status of the most preferred drug of choice in clinical settings. The high intrinsic toxicity of the principal drug could be considered the main impedance in the frequent medicinal use of this otherwise very effective antimicrobial agent. Taking into consideration this fact, the pharma industry has introduced many novel dosage forms of AmB to alleviate its toxicity issues. However, the limited production, high cost, requirement for a strict cold chain, and need for parenteral administration are some of the limitations that explicitly compel professionals to look for the development of an alternate dosage form of this important drug. Considering the fact that the nano-size dimensions of drug formulation play an important role in increasing the efficacy of the core drug, we employed a green method for the development of nano-assemblies of AmB (AmB-NA). The as-synthesized AmB-NA manifests desirable pharmacokinetics in the treated animals. The possible mechanistic insight suggested that as-synthesized AmB-NA induces necrosis-mediated cell death and severe mitochondrial dysfunction in L. donovani promastigotes by triggering depolarization of mitochondrial membrane potential. In vivo studies demonstrate a noticeable decline in parasite burden in the spleen, liver, and bone marrow of the experimental BALB/c mice host. In addition to successfully suppressing the Leishmania donovani, the as-formed AmB-NA formulation also modulates the host immune system with predominant Th1 polarization, a key immune defender that facilitates the killing of the intracellular parasite.

Sexual and Reproductive Health Outcomes in Females With Cloacal Malformations and Other Anorectal Malformations.

STUDY OBJECTIVE: To improve our understanding of reproductive health and sexual function in women with cloacal malformations and other anorectal malformations (ARMs) METHODS: An observational cross-sectional survey was administered to individuals assigned female at birth aged 12 to 55 with ARMs and cloacal malformations cared for at our institution. Data included age of thelarche/menarche and questions on body image, gynecologic anatomy, sexual function, and pregnancy. RESULTS: Twenty-one patients responded in the ARM group and 30 in the cloacal malformation group. There were no differences in median age of thelarche/menarche in patients with ARMs (11/12.5 years) compared with patients with cloacal malformation (11/12 years). Patients with ARMs were more likely to have native vaginal tissue than those with cloacal malformations (n = 18, 82% vs n = 12, 40%; P = .03). There were no differences between groups regarding concerns about dyspareunia and functionality of their vagina (P > .05). Forty-two percent of patients with cloacal malformations and 30% of patients with ARMs reported having been sexually active. Two patients with cloacal malformations and 2 with ARMs reported having been pregnant. Patients with cloacal malformations reported a lower quality of life score (80.4) compared with those with ARMs (87.0) (difference > 4.5). CONCLUSIONS: Patients with a cloacal malformation were less likely to have native vaginal tissue and reported a lower quality of life than those with ARMs. Despite this, patients with a cloacal malformation had similar reproductive health and sexual function compared with patients with ARMs. Our results reinforce the need for comprehensive sexual and reproductive health care for all women with ARMs.