Does presence of a VACTERL anomaly predict an associated gynecologic anomaly in females with anorectal malformations?: A Pediatric Colorectal and Pelvic Learning Consortium Study.

BACKGROUND: VACTERL association is defined by the presence of 3 or more anomalies in any of the following systems: vertebral, anorectal, cardiac, trachea-esophageal, renal, or limb. This study hypothesized that the presence of VACTERL association would correlate with an increased risk of gynecologic anomalies in patients with anorectal malformation (ARM). METHODS: This study is a cross-sectional, retrospective analysis from the prospectively collected, multicenter registry of the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC). The 834 female patients with ARM who were enrolled in the registry by January 1, 2020 were included in this study. The relationship of VACTERL association with presence of a gynecologic anomaly was evaluated with Fisher's exact test. The relationship of each VACTERL system with presence of a gynecologic anomaly was assessed in patients with cloaca, rectovestibular fistulas and rectoperineal fistulas. P-values reported were based on a 2-sided alternative and considered significant when less than 0.05. RESULTS: 834 patients with ARM underwent VACTERL screening and gynecologic evaluation with the three most common subtypes being cloaca (n = 215, 25.8%), rectovestibular fistula (n = 191, 22.9%) and rectoperineal fistula (n = 194, 23.3%). A total of 223 (26.7%) patients with ARM had gynecologic anomalies. VACTERL association was seen in 380 (45.6%) of patients with ARM. Gynecologic anomalies were present in 149 (39.1%) vs. 74 (16.3%) of subjects with vs. without VACTERL association (p < 0.001). VACTERL association did not significantly increase the risk of gynecologic anomaly in patients with cloaca and VACTERL (n = 88, 61.5%) vs. cloaca without VACTERL (n = 39, 54.2% p = 0.308). VACTERL association increased the risk of gynecologic anomalies in patients with rectoperineal fistulas (n = 7, 14.9% vs n = 9, 6.1% p = 0.014) and rectovestibular fistulas (n = 19, 31.1% vs. n = 13, 10.0% p<0.001). In patients with ARM who had a VACTERL association, when one of the associated anomalies was renal, there was an even higher risk of having an associated gynecologic anomaly (n = 138, 44.2% vs. n = 85, 16.3% p<0.001). CONCLUSIONS: VACTERL association in patients with rectoperineal and rectovestibular fistulas correlates with an increased risk of gynecologic anomalies. The presence of VACTERL associated findings, especially renal, should prompt a thorough evaluation of the gynecologic system. LEVEL OF EVIDENCE: III. Retrospective comparative study.

Digital photography in the evaluation and management of female patients with congenital adrenal hyperplasia: A standardized protocol for quality improvement.

INTRODUCTION: Digital photography can be securely stored in the medical record and enhance documentation of physical exam findings and monitor wound healing. A standardized protocol that respects the dignity of the patient and maintains the fidelity of objective documentation is needed for patients with differences in sexual development (DSD) and congenital adrenal hyperplasia (CAH). OBJECTIVE: The purpose of this study was to evaluate the feasibility, acceptability, and applications of a HIPAA-compliant digital photography protocol in the care of female patients with CAH. STUDY DESIGN: A protocol for standardized digital imaging including consent, permission, data capture, and storage in the electronic medical record (EMR) was implemented. Patients undergoing physical examination during multidisciplinary CAH clinic visits, preoperative evaluation, and postoperative follow-up from October 2020 through May 2021 were included. Male patients with CAH, patients with clitoromegaly or urogenital sinus not from CAH, and patients seen through telehealth were excluded. Consent was obtained from caregivers and permission from patients. Images of the exam were taken during clinic visits or at the time of surgery with no identifying features included. Images were directly uploaded into the patient's chart in the HIPAA-protected EMR separate from other clinical documentation and not stored on personal devices. RESULTS: There were 17 patients with CAH seen with median age 6 years (range 2 weeks-18 years). There was a median of 3 photos per patient during the study period with cooperation from both the patient and their caregiver. Amongst the patients seen, 6 patients underwent reconstruction with a median of 10 photos per patient. Images were available and used for preoperative planning and counseling. Patients with previous images did not require repeat examinations and were subjected to fewer genital examinations. Fewer providers were present during exams. Images taken by providers and caregivers during the postoperative period were used to monitor wound healing and surgical outcomes. DISCUSSION: Protocol implementation improved patient care by reducing the number of exams and number of providers present, enhancing clinical documentation, and providing a means of tracking the physical exam over time. This was in concordance with guidelines for limiting exams for patients with DSD and CAH. Implementation of best practices for medical photography was important in respecting patient dignity and confidentiality. CONCLUSION: Implementation of standardized digital photography was feasible and acceptable to patients and caregivers. Digital images reduced the need for repeat physical examination and provided a visual means of enhancing clinical documentation.

Learning professionalism: perspectives of preclinical medical students.

PURPOSE: To identify and examine how students respond to and engage with formal professionalism teaching strategies, and what factors outside the formal curriculum may influence professional development. METHOD: Individual semistructured interviews were conducted with 56 students completing the preclinical curriculum at the University of Washington School of Medicine in 2004 and 2005. Interviews were recorded, transcribed, and analyzed using qualitative methods. RESULTS: Students identified role modeling as an important modality for learning professionalism, even during their preclinical years. Role models included classroom faculty and peers, in addition to physicians in clinical settings. Small-group discussions and lectures helped some students identify and analyze the professional behaviors they observed, but they elicited negative responses from others. Students believed their professionalism derived from values, upbringing, and experiences prior to medical school. Some students reflected on their evolving professionalism while working directly with patients. CONCLUSIONS: Medical schools should ensure that students are exposed to excellent role models-ideally, faculty who can articulate the ideals of professionalism and work with students longitudinally in clinical settings. Lectures about professionalism may alienate rather than inspire students. Students' premedical experiences and values influencing professionalism should be acknowledged and appreciated. Bedside teaching and reflection on students' inner experience as they begin to work directly with patients deserve further exploration as opportunities to teach professionalism.