Single institution experience of cloacal malformation.

INTRODUCTION: The aim of this study is to report on the outcomes of patients born with cloacal malformation, managed at a single institution more than the last 28 years. The focus of this study is the long term renal and colorectal outcomes. METHODS: Patients were identified from the departmental database from 1994 to 2021. The medical records and operative notes were retrospectively reviewed. RESULTS: Twenty-one patients fulfilled the inclusion criteria. Eleven long common channel (LCC) and ten short common channel (SCC) cloacae patients were identified. Median age at the time of primary reconstruction was 11 months in both groups. In the LCC group, seven (63.6%) patients underwent a Total Urogenital Mobilisation (TUM), and 4 (36.4%) required a vaginal replacement. 6/11 (54.5%) of patients required drainage of a hydrocolpos. In the SCC group, four patients required a TUM, two patients underwent mobilisation of the rectum and vagina alone, and three underwent rectal mobilisation alone. Two patients have required renal transplant for congenital renal dysplasia, and two have developed chronic renal failure associated with the sequalae of vesicoureteric reflux. Eleven (52.3%) of the patients manage their bowels with an antegrade continent enema (ACE), and two of the LCC cloaca are defunctioned with a colostomy. Clean intermittent catheterisation is performed by 12 (57%) of the patients, either per urethra or via a Mitrofanoff channel. CONCLUSION: The urinary and faecal continence are the main challenges in the management of cloaca patients. Many require surgical intervention to achieve social continence. LEVEL OF EVIDENCE: Level IV.

Functional outcomes, quality of life, sexual function, and fertility of adult patients undergoing ileo-anal pouch anastomosis in childhood.

BACKGROUND: We conducted a questionnaire-based study of pouch function, quality of life, sexual function, and fertility among patients who had undergone ileal pouch anal anastomosis (IPAA) in childhood and who are now more than 18 years old. METHODS: A consecutive series of patients were asked to complete the following questionnaires: Pouch function score (PFS), short form 36 (SF-36), International index of erectile function (IIEF) (males), and Female sexual function index (FSFI) (females). Fertility in females was also assessed. Data are quoted as a median (SD). RESULTS: Of 144 patients who had IPAA, 101 were eligible, and 70 responded. Mean age at surgery was 14 years, and mean current age is 26 years. Most patients had either ulcerative colitis or familial adenomatous polyposis. The questionnaire was completed by 38/70 (54%). Median PFS score was 6 (5). SF-36 were lower than previous reports. Median FSFI was 30 (7.6), 84% of possible maximum. Median IIEF was 69, 92% of possible maximum. Successful conception was reported in 5/7 women who had tried. However, there were several miscarriages and two surgical emergencies during pregnancies. CONCLUSIONS: IPAA can be performed in childhood with similar pouch function to reported adult series. Quality of life appears poorer, but sexual function is maintained. There may be an association with adverse events in pregnancy. LEVEL OF EVIDENCE: IV.

Parity and Psychosocial Risk Factors Increase the Risk of Depression During Pregnancy Among Recent Immigrant Women in Canada.

Prior investigations have examined risk factors associated to postpartum depression in immigrant women, but depression during pregnancy has received less attention. This study describes the prevalence and early determinants of antenatal depression among recent (≤ 5 years) and long-term immigrants (> 5 years), compared to Canadian-born women. 503 women completed standardized self-report questionnaires measuring sociodemographics and psychosocial factors. Multivariate logistic regressions identified first trimester risk factors for depression in each immigrant group. The prevalence of depressive symptoms was highest for recent immigrant (25.3-30.8%) compared to long-term immigrant (16.9-19.2%) and Canadian-born women (11.7-13.8%). Among recent immigrants, multiparity, higher stress and pregnancy-specific anxiety in early pregnancy increased the risk of antenatal depression. Among long-term immigrants, stress in the first trimester was significantly associated with antenatal depressive symptoms. Knowledge of modifiable risk factors (pregnancy-specific anxiety and stress) may help improve antenatal screening and inform the development of tailored interventions to meet the mental health needs of immigrant women during the perinatal period.

The core trainee 'residential': an opportunity for trainees to feel connected in a world of virtual teaching.

MRCPsych courses play a key role in helping trainees prepare for Membership examinations and specialist training. Historically, their social aspect, although arguably as important, has perhaps not been sufficiently prioritised. During a pandemic, when teaching is largely delivered virtually, the social benefit of meeting peers is highlighted by its absence. Given the future likelihood of increased virtual teaching, it is of paramount importance to explore ways of enhancing the sociability of teaching courses. In addition to the social needs of trainees, there is a recognised need to increase and integrate the neuroscience component of the curriculum to better meet the needs of modern day mental health research, treatment and practice. This article describes how the Birmingham MRCPsych course successfully addressed both these issues with a 2-day 'residential' in October 2019, and considers whether future residentials could be delivered virtually.

Understanding Psychiatric Disorders in Idiopathic and Inherited (Monogenic) Forms of Isolated and Combined Dystonia: A Systematic Review.

OBJECTIVE: The relationship between idiopathic and inherited (monogenic) forms of isolated and combined dystonia and psychiatric disorders remains unclear. In the present review, the authors aimed to provide increased clarity on this association through a systematic review of all controlled quantitative studies using a structured or semi-structured psychiatric interview to diagnose psychiatric disorders in individuals with these conditions. METHODS: Three databases were searched to identify 20 eligible studies, with a total of 1,275 participants fulfilling inclusion criteria. Eligible articles were quality appraised and divided into four sections (idiopathic forms of dystonia [N=11], early-onset torsion dystonia [N=2], gene mutation positive myoclonus dystonia; DYT-SGCE [N=6], and rapid-onset dystonia-parkinsonism [N=1]). RESULTS: For each study, results were grouped into subcategories (overall psychiatric comorbidity, anxiety disorders, mood disorders, substance misuse, and other [personality disorder and cognitive impairment]). For idiopathic dystonia, higher rates of psychiatric comorbidity, including mood and anxiety disorders, were noted when cases were compared with both healthy control subjects and control groups with a medical comorbidity. However, for major depressive disorder and obsessive-compulsive disorder (OCD) specifically, no differences were seen between groups. Study subjects with DYT-SGCE appeared to be at higher risk of psychiatric comorbidity, major depressive disorder, OCD, and alcohol dependence than control populations. CONCLUSIONS: Overall, the prevalence of psychiatric comorbidity appears to be increased in individuals with idiopathic and inherited (monogenic) forms of isolated and combined dystonia compared with control subjects. This finding is not consistent for all comparisons, and further research is required to understand the nature of these associations and the underlying causative etiologies.

A Novel Surgical Approach for the Management of Cloacal Exstrophy with a Giant Omphalocele.

Cloacal exstrophy is a rare malformation that presents as a lower midline abdominal wall defect which affects the gastrointestinal and genitourinary systems. The components of cloacal exstrophy characteristically include omphalocele, exstrophy of perineal structures, and imperforate anus. Most of these patients also have renal anomalies such as pelvic kidney, fused kidneys, or solitary kidneys. This congenital condition can also be associated with spinal issues, such as spinal dysraphism. When combined with spinal defects, it is referred to as the omphalocele, exstrophy, imperforate anus, and spinal defects (OEIS) complex, and is one of the most challenging surgical conditions to manage. Here, we present a unique case of a low-birth-weight patient with OEIS and a liver containing giant omphalocele and the novel surgical technique used to manage her cloacal exstrophy whereby the cecal plate was not separated from the bladder halves, but rather left for an autoaugment, and the ileum was connected to the hindgut.

Assessing the previously repaired patient with an anorectal malformation who is not doing well.

In this review, the care of children with a previously repaired anorectal malformation is explored. We know that the surgical care of children with anorectal malformations is complex; however, despite an increased understanding of the congenital anomaly and significant technical advances in the operative repair, many of these children continue to have poor functional outcomes. In this article we focus on the common surgical complications, discuss typical presentations, consider appropriate investigations, and review the risks and benefits of revisional surgery in those patients that are 'not doing well' following their primary reconstruction.

Clinical Quiz: Reconstructive Strategies in a 5-Month-Old Infant with a Cloaca.

Herein we present a case of 5-month-old female born with a cloaca. She underwent a colostomy at birth and then underwent endoscopy and cloacagram to plan for the definitive reconstruction. The case is presented with a focus on the reconstructive strategies, and questions for the readers are posed in a quiz format.

Bedside breath tests in children with abdominal pain: a prospective pilot feasibility study.

BACKGROUND: There is no definitive method of accurately diagnosing appendicitis before surgery. We evaluated the feasibility of collecting breath samples in children with abdominal pain and gathered preliminary data on the accuracy of breath tests. METHODS: We conducted a prospective pilot study at a large tertiary referral paediatric hospital in the UK. We recruited 50 participants with suspected appendicitis, aged between 5 and 15 years. Five had primary diagnosis of appendicitis. The primary outcome was the number of breath samples collected. We also measured the number of samples processed within 2 h and had CO2 ≥ 3.5%. Usability was assessed by patient-reported pain pre- and post-sampling and user-reported sampling difficulty. Logistic regression analysis was used to predict appendicitis and evaluated using the area under the receiver operator characteristic curve (AUROC). RESULTS: Samples were collected from all participants. Of the 45 samples, 36 were processed within 2 h. Of the 49 samples, 19 had %CO2 ≥ 3.5%. No difference in patient-reported pain was observed (p = 0.24). Sampling difficulty was associated with patient age (p = 0.004). The logistic regression model had AUROC = 0.86. CONCLUSIONS: Breath tests are feasible and acceptable to patients presenting with abdominal pain in clinical settings. We demonstrated adequate data collection with no evidence of harm to patients. The AUROC was better than a random classifier; more specific sensors are likely to improve diagnostic performance. TRIAL REGISTRATION: ClinicalTrials.gov, NCT03248102. Registered 14 Aug 2017.

The use of rotational fluoroscopy and 3-D reconstruction in the diagnosis and surgical planning for complex cloacal malformations.

INTRODUCTION: Cloacal malformations, a confluence of the urinary tract, vagina and rectum into a single common channel, has a broad and complex anatomic spectrum requiring an imaging tool for visualization, measurement, and surgical planning for the reconstruction of these structures. We evaluated the role of 3-D fluoroscopy for this purpose, as it offers a combination of spatial correlation with precise anatomic measurements. METHODS: We examined our imaging protocol for patients with a cloacal malformation and report our experience with rotational fluoroscopy and 3-D reconstruction in 16 consecutive patients referred for cloacal reconstruction. The length of the common channel (CC), the length of the urethra from the bladder neck to the common channel, and the height (and existence or absence) of a vagina or vaginas were determinants of the surgical procedures used for the repair. RESULTS: We performed 16 consecutive 3-D cloacagrams (age range 4 months to 9 years) using a new protocol (Figure 1) that provided the following data which helped with surgical planning: Gynecologic: 3 cases with a single vagina, 5 cases with a duplicated Mullerian system (3 of which were asymmetric) and 2 cases with high vaginas requiring vaginal replacement. Colorectal: Four had a high rectum requiring an abdominal approach, and 6 had a rectum reachable via a posterior sagittal approach. Urologic: Two ectopic ureters requiring reimplantation, 3 patients had vesicoureteral reflux (1 bilateral, 2 unilateral), 1 patient had no bladder, and 7 had a normal sized bladder. Common channel length and urethral length were demonstrated in all cases and used to decide between a total urogenital mobilization or a separation of vagina(s) from the common channel, urogenital separation. CONCLUSION: The 3-D cloacagram can help predict the surgical plan for urologic, gynecologic, and colorectal components of the cloacal repair. It can predict the CC length as well as the length of the urethra. It helps with predicting the need for vaginal replacement and whether an abdominal approach is needed for the rectum. Its effectiveness is based on the ability to adequately distend structures and see their distal most extent, an advantage over other modalities such as MRI. Added benefits (particularly from the 3D view) include a better spatial understanding of the defect and the diagnosis of concomitant urological abnormalities such as vesicoureteral reflux and ectopic ureters. Disadvantages to this procedure include the need for general anesthesia and a higher exposure to radiation. LEVEL OF EVIDENCE: 3.

Development of a Patient-reported Experience and Outcome Measures in Pediatric Patients Undergoing Bowel Management for Constipation and Fecal Incontinence.

AIMS AND OBJECTIVES: The aim of this review is develop a reliable and valid questionnaire that assesses patient-reported experience measures (PREMs) and patient-reported outcome measures (PROMs) of caregivers, families, and patients with severe constipation and fecal incontinence who failed conservative treatments and require a formal bowel management program (BMP). METHODS: A 5-step iterative process was utilized to ensure the reliability and validity of the final instrument. Parents or guardians of patients undergoing our week-long BMP were first asked 20 open-ended questions aimed at understanding the challenges in managing their child's condition. Responses were transformed into an initial 41-item survey constituted of 2 constructs relating to PREMs (24 items) and PROMs (17 items). Survey items were then administered to 359 parents and guardians undergoing BMP. Cognitive interviews were performed with 20 families to assess face validity and to further refine the survey. Instrument readability and reliability was assessed by Flesch-Kincaid and Crohnbach α analyses. Items that weakly correlated were deleted to yield a final instrument that was both valid and reliable. RESULTS: A 33-statement survey was developed that encompasses important physical and emotional health concerns, quality of life, treatment success, financial considerations, schooling, parental employment, and social concerns. The final instrument, the Patient-Reported Experience and Outcome Measure in a Bowel Management Program (PREOM-BMP), is divided into 2 separate constructs that assess important patient-reported experience (22 items) and outcome (11 items) measures. Reliability analyses on the final instrument yielded a Crohnbach α of 0.925. CONCLUSIONS: The PREOM-BMP offers physicians and nursing professionals a brief and valid tool to measure the impact of a bowel management program on both the child and the family unit.

Diagnosis and management of a remnant of the original fistula (ROOF) in males following surgery for anorectal malformations.

PURPOSE: A complication of the surgical management of anorectal malformations (ARMs) is a retained remnant of the original fistula (ROOF) formerly called a posterior urethral diverticulum (PUD). A ROOF may have multiple presentations or may be incidentally discovered during the work-up of ARM after previous surgery. We sought to define the entity and the surgical indications for excision of a ROOF when found. METHODS: We performed a retrospective cohort study of all male patients who presented to our center following previous repair for ARM at another institution, who came for evaluation of problems with urinary and/or fecal continence, from 2014 to 2017. Charts were reviewed for symptoms, original type of malformation, preoperative imaging, treatment, and postoperative follow-up. RESULTS: Of 180 referred male patients, 16 had a ROOF. 14 underwent surgical repair to address this and for other redo indications, and 2 did not require intervention. 13 patients had an additional reason for a redo such as anal mislocation or rectal prolapse. Indications for ROOF excision were urinary symptoms (e.g. UTI, dribbling, passage of mucous via urethra, stone formation), to make a smoother posterior urethra for intermittent catheterization, or for prophylactic reasons. Patients were repaired at an average age of 4.2 years, using a PSARP only approach with excision of the ROOF for all except one patient who needed a laparotomy due to abdominal extension of the ROOF. No patient needed a colostomy. The original ARM repairs of the patients were PSARP (9), laparoscopic assisted (4) and abdominoperineal pullthrough (3). Preoperative evaluation included pelvic MRI, VCUG, and cystoscopy. The ROOF was visualized on 14 of 16 MRIs, 10 of 14 VCUGs, and 14 of 15 cystoscopies. Urinary symptoms associated with a ROOF and ease of catheterization were improved in all repaired cases. CONCLUSION: Patients not doing well from a urinary or bowel standpoint post ARM pull-through need a complete evaluation which should include a check for a ROOF. Both modalities MRI and cystoscopy are needed as a ROOF can be missed on either alone. A VCUG was not reliable in identifying a ROOF. Excision is needed in patients to improve urinary symptoms associated with these lesions and to minimize the small but theoretical oncologic risk present in a ROOF. LEVEL OF EVIDENCE: Level III.

Primary or Redo Posterior Sagittal Anorectoplasty without a Stoma: To Feed or Not to Feed?

INTRODUCTION: Repair of anorectal malformations (ARMs), primarily or with a reoperation, may be performed in certain circumstances without a diverting stoma. Postoperatively, the passage of bulky stool can cause wound dehiscence and anastomotic disruption. To avoid this, some surgeons keep patients NPO (nothing by mouth) for a prolonged period. Here, we report the results of a change to our routine from NPO for 7 days to clear fluids or breast milk. MATERIALS AND METHODS: After primary or redo ARM surgery, patients given clear liquids were compared to those who were kept strictly NPO. Age, indication for surgery, incision type, use of a peripherally inserted central catheter (PICC) line, and wound complications were recorded. RESULTS: There were 52 patients, including 15 primary and 37 redo cases. Group 1 comprised 11 female and 15 male patients. The mean age at surgery was 4.9 years (standard deviation [SD]: 2.3). There were 8 primary cases and 18 redo cases. Twelve (46.6%) received a PICC line. The average start of clear liquids was on day 5.3 (SD: 2.2) after examination of the wound, and the diet advanced as tolerated. The first stool passage was recorded on average on day 2.3 (SD: 1.3). Four minor wound complications and no major wound complications occurred.Group 2 comprised 14 females and 12 male patients. The mean age at surgery was 3.5 (SD: 2.4) years. There were 7 primary and 19 redo cases. One (3.8%) patient required a PICC line. A clear liquid diet was started within 24 hours after surgery. A regular diet was started on average on day 5.8 (SD: 1.3). The first stool passage was recorded on an average of day 1.6 (SD: 0.9). Three minor wound complications occurred; however, there was no significant difference between the two groups (SD: 0.71). One major wound complication occurred. However, there was no significant difference in major wound complications between the groups (SD: 0.33). CONCLUSION: No increase in wound problems was noted in children receiving clear liquids or breast milk compared with the strict NPO group, and PICC line use was reduced. We believe this change in practice simplifies postoperative care without increasing the risk of wound complications.

Urinary Outcomes in Patients with Down's Syndrome and Hirschsprung's Disease.

INTRODUCTION: Previous research in children with Hirschsprung's disease (HD) and Down's syndrome (DS) has focused on colorectal outcomes. We set out to review urinary outcomes in this patient group. MATERIALS AND METHODS: The medical records of all patients aged five years and older with HD were reviewed, and patients and caregivers filled out the Vancouver Symptom Score at intake, which is designed and validated to diagnose dysfunctional elimination syndrome. RESULTS: A total of 104 patients with HD were included in this study. Of these, 16 (15%) patients had DS. There were no significant differences in the prevalence of enterocolitis or colorectal symptoms between patients with or without DS. Five of 88 (6%) patients without DS and 7 of 16 (44%) (p = 0.00001) with DS reported having urinary accidents. Patients with HD and DS scored higher on the Vancouver score (9 vs. 17.5; p = 0.007), indicating more severe urinary symptoms. Patients who also reported fecal accidents scored significantly higher on the Vancouver (12 vs. 9; n = 61; p = 0.016), indicating more problems. CONCLUSION: Patients with DS appear to be a unique subset of HD patients who have a higher prevalence of urinary symptoms after surgery. In the postoperative care of patients with HD and DS, a strong focus should be placed on postoperative urinary care in addition to their bowel care. This could significantly ease care and contribute to the quality of life of the parents and the patient.

Transcending Dimensions: a Comparative Analysis of Cloaca Imaging in Advancing the Surgeon's Understanding of Complex Anatomy.

Surgeons have a steep learning capacity to understand 2-D images provided by conventional cloacagrams. Imaging advances now allow for 3-D reconstruction and 3-D models; but no evaluation of the value of these techniques exists in the literature. Therefore, we sought to determine if advances in 3-D imaging would benefit surgeons, lead to accelerated learning, and improve understanding for operative planning of a cloaca reconstruction. Questionnaires were used to assess the understanding of 2-D and 3-D images by pediatric surgical faculty and trainees. For the same case of a cloacal malformation, a 2D contrast study cloacagram, a 3D model rotatable CT scan reconstruction, a software enhanced 3D video animation (which allowed the observer to manipulate the structure in any orientation), and a printed physical 3D cloaca model that could be held in the observer's hand were employed. Logistic mixed effect models assessed whether the proportion of questions about the case that were answered correctly differed by imaging modality, and whether the proportion answered correctly differed between trainee and attending surgeons for any particular modality. Twenty-nine pediatric surgery trainees (27 pediatric general surgery and 2 pediatric urology surgery trainees) and 30 pediatric surgery and urology faculty participated. For trainees, the percentage of questions answered correctly was: 2-D 10.5%, 3-D PACS 46.7%, 3-D Enhanced 67.1%, and 3-D Printed 73.8%. For faculty, the total percentage of questions answered correctly was: 2-D 22.2%, 3-D PACS 54.8%, 3D Enhanced 66.2%, and 3-D printed 74.0%. The differences in rates of correctness across all four modalities were significant in both fellows and attendings (p < 0.001), with performance being lowest for the 2-D modality, and with increasing percentage of correct answers with each subsequent modality. The difference between trainees and attendings in correctness rate was significant only for the 2-D modality, with attendings answering correctly more often. The 2-D cloacagram, as the least complex model, was the most difficult to interpret. The more complex the modality, the more correct were the responses obtained from both groups. Trainees and attendings had similar levels of correct answers and understanding of the cloacagram for the more advanced modalities. Mental visualization skills of anatomy and complex 3-D spatial arrangements traditionally have taken years of experience to master. Now with novel surgical education resources of a 3-D cloacagram, a more quickly advancing skill is possible.

The Impact of Manuscript Learning vs. Video Learning on a Surgeon's Confidence in Performing a Difficult Procedure.

Introduction: Operative surgical videos are a popular educational resource, not commonly a part of a peer-reviewed article. We wanted to evaluate the impact of either reading a peer-reviewed manuscript or watching an operative video on a surgeon's confidence in performing a complex case. Methods: Pediatric surgeons and fellows were asked to complete an initial questionnaire to assess their confidence (formulated as a score) in the diagnosis and operative repair of anal stenosis and rectal atresia. Results: Of 101 pediatric surgeons and fellows, 52 (51%) were randomized into a "manuscript" group and 49 (49%) into a "video" group. The mean confidence before the intervention was the same in the two groups (6.4 vs. 6.6). Attending surgeons started with more confidence than trainees (7.1 vs. 5.3, p < 0.001). In the manuscript group, the average confidence increased to 7.7 (p = 0.005), and in the video group the average confidence increased to 7.9 (p = 0.001) globally. Trainees in the video group significantly improved their confidence to a score of 6.6 (p = 0.035), as did attending surgeons to 8.5 (p = 0.01). In the manuscript group, only attendings significantly improved their confidence by 1.5-8.3 (p < 0.001), whereas trainees did not with a difference of 1.3 (p = 0.194). When considering experience level, physicians who reported never having performed this surgery improved only by reading the manuscript (3.9-6.2) (p = 0.004), not by watching the video (5.4-6.6) (p = 0.106). Surgeons with experience doing this operation (>5 times) did not improve their confidence by reading the manuscript (p = 0.10), nor by watching the video (p = 0.112). Conclusion: Reviewing either a detailed manuscript or operative video on the surgical management of rectal atresia and anal stenosis demonstrated a significant increase in self-reported confidence. Trainees benefitted the most from operative videos, whereas experienced surgeons did not improve their confidence by reading the manuscript nor watching the video.

A structured bowel management program for patients with severe functional constipation can help decrease emergency department visits, hospital admissions, and healthcare costs.

BACKGROUND: Published health-care costs related to constipation in children in the USA are estimated at $3.9 billion/year. We sought to assess the effect of a bowel management program (BMP) on health-care utilization and costs. METHODS: At two collaborating centers, BMP involves an outpatient week during which a treatment plan is implemented and objective assessment of stool burden is performed with daily radiography. We reviewed all patients with severe functional constipation who participated in the program from March 2011 to June 2015 in center 1 and from April 2014 to April 2016 in center 2. ED visits, hospital admissions, and constipation-related morbidities (abdominal pain, fecal impaction, urinary retention, urinary tract infections) 12 months before and 12 months after completion of the BMP were recorded. RESULTS: One hundred eighty-four patients were included (center 1 = 96, center 2 = 88). Sixty-three (34.2%) patients had at least one unplanned visit to the ED before treatment. ED visits decreased to 23 (12.5%) or by 64% (p < 0.0005). Unplanned hospital admissions decreased from 65 to 28, i.e., a 56.9% reduction (p < 0.0005). CONCLUSION: In children with severe functional constipation, a structured BMP decreases unplanned visits to the ED, hospital admissions, and costs for constipation-related health care. LEVEL OF EVIDENCE: 3.

Gynecologic anatomic abnormalities following anorectal malformations repair.

BACKGROUND/AIM: Patients may present with gynecologic concerns after previous posterior sagittal anorectoplasty (PSARP) for repair of an anorectal malformation (ARM). Common findings include an inadequate or shortened perineal body, as well as introital stenosis, retained vaginal septum, and remnant rectovestibular fistula. An inadequate or shortened perineal body may impact fecal continence, sexual function and recommendations regarding obstetrical mode of delivery. We describe our experience with female patients referred to our center for evaluation of their previously repaired ARM, with a specific focus on perineal body anatomy and concomitant gynecologic abnormalities. We outline our collaborative evaluation process and findings as well as subsequent repair and outcomes. MATERIAL/METHODS: A single site retrospective chart review from May 2014 to May 2016 was performed. Female patients with a history of prior ARM repair who required subsequent reoperative surgical repair with perineoplasty were included. The decision for reoperation was made collaboratively after a multidisciplinary evaluation by colorectal surgery, urology, and gynecology which included examination under anesthesia (EUA) with cystoscopy, vaginoscopy, rectal examination, and electrical stimulation of anal sphincters. The type of original malformation, indication for reoperative perineoplasty, findings leading to additional procedures performed at time of perineoplasty, postoperative complications, and the length of follow up were recorded. RESULTS: During the study period 28 patients were referred for evaluation after primary ARM repair elsewhere and 15 patients (60%) met inclusion criteria. Thirteen patients (86.6%) originally had a rectovestibular fistula with prior PSARP and 2 patients (13.4%) originally had a cloacal malformation with prior posterior sagittal anorectovaginourethroplasty. The mean age at the time of the subsequent perineoplasty was 4.6years (0.5-12). Patients had an inadequate perineal body requiring reoperative perineoplasty due to: anterior mislocation of the anus (n=11, 73.3%), prior perineal wound dehiscence with perineal body breakdown (n=2, 13.4%), acquired rectovaginal fistula (n=1, 6.6%), and posterior mislocated introitus with invasion of the perineal body (n=1, 6.6%). During the preoperative evaluation, additional gynecologic abnormalities were identified that required concomitant surgical intervention including: introital stenosis (n=4, 26.6%), retained vaginal septum (n=3, 20%) and remnant recto vestibular fistula (n=2, 13.3%). CONCLUSIONS: Patients with a previously repaired ARM may present with gynecologic concerns that require subsequent surgical intervention. The most common finding was an inadequate perineal body, but other findings included introital stenosis, retained vaginal septum and remnant recto vestibular fistula. Multidisciplinary evaluation to assess and identify abnormalities and coordinate timing and surgical approach is crucial to assure optimal patient outcomes. TYPE OF STUDY: Case series with no comparison group. LEVEL OF EVIDENCE: IV.

Multiple deprivation, vision loss, and ophthalmic disease in adults: global perspectives.

The association between socioeconomic position and morbidity and mortality has long been recognized. We evaluate the evidence for an association between multiple aspects of deprivation and ocular health in a global context. This is a systematic review of studies that evaluated deprivation in the adult population in the context of the major acquired causes of visual loss such as cataract, diabetic eye disease, glaucoma, age-related macular degeneration, and ocular trauma. The search strategy identified relevant studies reported between 1946 and August 2016, with randomized control trials, case-control, cohort, and cross-sectional study designs being selected for inclusion. The studies identified in this review from across the world demonstrate the extent to which the common themes such as low educational attainment and low income may be associated with increased incidence of various sight-threatening conditions and may adversely affect access to specialist assessment and delivery of treatment. Health inequality may always persist, but an increased recognition of the importance of the various impacts of deprivation may empower policy makers to target limited resources to the most vulnerable groups in order to deliver the greatest benefit.

Drift-dependent changes in iceberg size-frequency distributions.

Although the size-frequency distributions of icebergs can provide insight into how they disintegrate, our understanding of this process is incomplete. Fundamentally, there is a discrepancy between iceberg power-law size-frequency distributions observed at glacial calving fronts and lognormal size-frequency distributions observed globally within open waters that remains unexplained. Here we use passive seismic monitoring to examine mechanisms of iceberg disintegration as a function of drift. Our results indicate that the shift in the size-frequency distribution of iceberg sizes observed is a product of fracture-driven iceberg disintegration and dimensional reductions through melting. We suggest that changes in the characteristic size-frequency scaling of icebergs can be explained by the emergence of a dominant set of driving processes of iceberg degradation towards the open ocean. Consequently, the size-frequency distribution required to model iceberg distributions accurately must vary according to distance from the calving front.