Impact on Patient Care of a Multidisciplinary Center Specializing in Colorectal and Pelvic Reconstruction

Aim of the study: Many patients with an anorectal malformation (ARM) or pelvic anomaly have associated urologic or gynecologic problems. We hypothesized that our multidisciplinary center, which integrates pediatric colorectal, urologic, gynecologic and GI motility services, could impact a patient's anesthetic exposures and hospital visits.Methods: We tabulated during 2015 anesthetic/surgical events, endotracheal intubations, and clinic/hospital visits for all patients having a combined procedure.Main results: Eighty two patients underwent 132 combined procedures (Table 1). The median age at intervention was 3 years [0.2-17], and length of follow up was 25 months [7-31]. The number of procedures in patients who underwent combined surgery was lower as compared to if they had been done independently [1(1-5) vs. 3(2-7) (p < 0.001)]. Intubations were also lower [1[1-3] vs. 2[1-6]; p < 0.001]. Hospital length of stay was significantly lower for the combined procedures vs. the theoretical individual procedures [8 days [3-20] vs. 10 days [4-16]] p < 0.05. Post-operative clinic visits were fewer when combined visits were coordinated as compared to the theoretical individual clinic visits (urology, gynecology, and colorectal) [1[1-4] vs. 2[1-6]; p = < 0.001].Conclusions: Patients with anorectal and pelvic malformations are likely to have many medical or surgical interventions during their lifetime. A multidisciplinary approach can reduce surgical interventions, anesthetic procedures, endotracheal intubations, and hospital/outpatient visits

Pilonidal Disease Mimicking Fistula-in-Ano in a 15-Year-Old Female

Pilonidal disease typically presents with an abscess or intermittent pain and drainage in the sacrococcygeal region during the pubertal years. Further examination typically reveals pits in the midline of the sacrococcyx area due to entrapment of hair with recurrent entrapment, infection, and drainage. The following paper describes an unusual presentation of a pilonidal cyst with fissure and perianal drainage

Patient and parental scar assessment after single incision versus standard 3-port laparoscopic appendectomy: Long-term follow-up from a prospective randomized trial

Background: Single site laparoscopy for appendectomy is a technique with several case series suggesting a cosmetic advantage, but without prospective comparative data. We conducted a prospective, randomized trial comparing single site laparoscopic appendectomy to the standard 3-port approach, including scar assessment at early and long-term follow-up. Methods: Enrolled patients over 12 years old and parents of patients less than 12 years old were asked to complete the validated Patient Scar Assessment Questionnaire (PSAQ) at early follow-up around 6 weeks and by phone after 18 months. The PSAQ consists of 4 scored subscales: Appearance, Consciousness, Appearance Satisfaction, and Symptom Satisfaction. Each subscale has a set of questions with a 4-point categorical response (1 = most favorable, 4 = least favorable). The sum of the scores quantifies each subscale. Results: Early questionnaires were obtained from 98 3-port and 100 single-site patients with the single-site approach producing superior overall scar assessment (P = 0.003). By telephone follow-up, questionnaires were completed by 49 3-port and 56 single-site patients at a median of 25 (18-32) months. In this longerterm follow-up, overall scar assessment was not significantly different between groups (P = 0.06). Conclusion: Patients or parents express superior scar assessment with the single site approach at early followup, but this difference disappears in the long-term. © 2014 Elsevier Inc. All rights reserved. Laparoscopic appendectomy has dramatically increased over the past 12 years as the operative method of choice for appendiciti

Redo posterior sagittal anorectoplasty for lateral mislocation in patients with anorectal malformations

© 2020 Elsevier Inc. Background: Children undergoing repair of an anorectal malformation (ARM) may have persistent soiling and/or constipation postoperatively. An anatomic reason should be sought; one of the causes for these problems that may require reoperation is mislocation of the rectum and anus outside of the muscle complex. Methods: We reviewed our population of children who underwent re-do anorectoplasty surgery between 2014 and 2019. Indications for surgery and outcomes were recorded. Results: Twelve patients had a lateral mislocation and underwent reoperation. There were no immediate complications in this subgroup. 9 of 10 patients are clean, and 4 are now able to have voluntary bowel movements. Conclusion: For patients who are found to have a significant lateral mislocation, we describe a new surgical technique that replaces the rectum and neo-anus directly in the midline through the muscle complex which may improve functional outcome

Development of a Standardized Algorithm for Management of Newly Diagnosed Anorectal Malformations

Neonates with a new diagnosis of anorectal malformation (ARM) present a unique challenge to the clinical team. ARM is strongly associated with additional midline malformations, such as those observed in the VACTERL sequence, including vertebral, cardiac, and renal malformations. Timely assessment is necessary to identify anomalies requiring intervention and to prevent undue stress and delayed treatment. We utilized a multidisciplinary team to develop an algorithm guiding the midline workup of patients newly diagnosed with ARM. Patients were included if born in or transferred to our neonatal intensive care unit (NICU), or if seen in clinic within one month of life. Complete imaging was defined as an echocardiogram, renal ultrasound, and spinal magnetic resonance imaging or ultrasound within the first month of life. We compared three periods: prior to implementation (2010–2014), adoption period (2015), and delayed implementation (2022); p ≤ 0.05 was considered significant. Rates of complete imaging significantly improved from pre-implementation to delayed implementation (65.2% vs. 50.0% vs. 97.0%, p = 0.0003); the most growth was observed in spinal imaging (71.0% vs. 90.0% vs. 100.0%, p = 0.001). While there were no differences in the rates of identified anomalies, there were fewer missed diagnoses with the algorithm (10.0% vs. 47.6%, p = 0.05). We demonstrate that the implementation of a standardized algorithm can significantly increase appropriate screening for anomalies associated with a new diagnosis of ARM and can decrease delayed diagnosis. Further qualitative studies will help to refine and optimize the algorithm moving forward

Assessment of the Heineke–Mikulicz anoplasty for skin level postoperative anal strictures and congenital anal stenosis

© 2018 Elsevier Inc. Introduction: Acquired skin-level strictures following posterior sagittal anorectoplasty (PSARP) and some rare cases of congenital anal stenosis can be managed using a Heineke–Mikulicz like anoplasty (HMA). We hypothesized that this procedure was an effective, safe, and durable outpatient procedure in select patients. Methods: We retrospectively reviewed all patients who underwent HMA for skin level strictures following PSARP or for certain congenital anal stenoses from 2014 to 2017. Results: Twenty-eight patients (19 males, 9 females) with a mean age of 5.8 years (range 0.5–24.4) underwent HMA. Twenty-six had a prior PSARP, of which 18 were redo, and 8 were primary procedures. Two patients had congenital skin level anal stenosis. The mean follow up was 1.0 years (range 0.4–2.9). The average preprocedure anal size was Hegar 8, which after HMA increased 8 Hegar sizes to 16 (95% CI 7–9, p \u3c 0.001). There were no operative complications. One patient restenosed and required a secondary procedure. Conclusion: HMA is a safe procedure for skin-level anal strictures following PSARP (primary and redo) and can also be used in some rare cases of congenital anal stenosis. Long-term follow up to determine the restricture rate is ongoing. A plan to do an HMA if a stricture develops may offer an alternative to routine anal dilations, particularly after a redo PSARP in an older child. Type of study: Case series. Level of evidence: Level IV

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

© 2018, Society for Imaging Informatics in Medicine. 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 \u3c 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

Gynecologic anatomic abnormalities following anorectal malformations repair

© 2017 Elsevier Inc. 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 4.6 years (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

Does clinic visit education within a multidisciplinary center improve health literacy scores in caregivers of children with complex colorectal conditions?

© 2017 Introduction Health literacy is low in an estimated one-third of the US population. Little is known about the health literacy of caregivers of children with colorectal conditions. The objective of this study was to investigate whether a timed health literacy intervention could improve health literacy in this population. Methods We used the BRIEF Health Literacy screening (BHLS) tool on caregivers of children who came to our colorectal clinic. Health literacy was categorized as inadequate, marginal, or adequate. The number of caregivers with adequate health literacy was compared to the number of clinic visits and socioeconomic status. Results We included 233 caregivers. The average number of clinic visits was 3.5 over 1.2 years. At the first clinic visit, 70% (n = 98) of caregivers had “adequate” health literacy. Scores improved to 88% (p = 0.024) after the fourth visit. Socioeconomic factors were not associated with health literacy. Patients of caregivers with “adequate” health literacy visited our clinic 3.8 times, compared to 2.7 times for those with lower literacy (p = 0.006). Conclusion Emphasis on providing an education-based approach at each visit increased health literacy significantly. As expected, health literacy was lowest during the first visit, which we believe is the optimal time to implement educational interventions. Type of study Case Control/Retrospective Comparative Study. Level of evidence Level III

Are Senna based laxatives safe when used as long term treatment for constipation in children?

© 2018 Elsevier Inc. Background and aim: Senna is a stimulant laxative commonly used by pediatricians, pediatric gastroenterologists, and pediatric surgeons. Many clinicians avoid Senna for reasons such as tolerance or side effects but this has little scientific justification. We recently found several patients we were caring for developed perineal blistering during the course of Senna treatment. Because of this we chose to review the literature to identify side effects in children taking this medication as well as to analyze our Center\u27s experience with Senna\u27s secondary effects. Methods: We performed a literature review (MEDLINE, PUBMED) using the keywords of Senna, sen, sennosides and children, and pediatric and functional (idiopathic) constipation. We looked for articles with information regarding perineal blisters related to Senna as well as other secondary effects of Senna laxatives in children when used on a long-term basis. We also reviewed the charts of our patients who had previously taken Senna or are currently taking Senna, looking for adverse reactions. Results: Eight articles in the literature reported perineal blisters after administration of Senna laxatives in 28 patients. Of those occurrences, 18 patients (64%) had accidental administration of Senna and 10 (36%) had Senna prescribed as a long term treatment. All of the blistering episodes were related to high dose, night-time accidents, or intense diarrhea with a long period of stool to skin contact. At our institution, from 2014 to 2017, we prescribed Senna and have recorded data to 640 patients. During the study period, 17 patients (2.2%) developed blisters during their treatment. Patients who developed blisters had higher doses 60 mg/day; 60 [12–100] vs. 17.5 [1.7–150] (p \u3c 0.001). All of the blistering episodes were related to night-time accidents, with a long period of stool to skin contact. 83 (13%) patients presented minor side effects such as abdominal cramping, vomiting or diarrhea which resolved once the type of laxatives were changed or enemas were started. The doses of Senna was not significantly different in these patients 15 mg/day [4.4–150] vs. 17.5 mg/day [1.5–150]. There were no other long-term side effects from Senna found in the pediatric literature for long-term treatment besides abdominal cramping or diarrhea during the first weeks of administration. We found no evidence of tolerance to Senna in our review. Conclusion: There is a paucity of information in the literature regarding side effects of sennosides as a long-term therapy, and to our knowledge, this is the first review of Senna side effects in children. Senna induced dermatitis is rare, but may occur when patients need a higher dose. All of the cases described had a long period of exposure of the skin to stool. Besides the perineal rash with blisters, we could find no other described major side effect with Senna administration in the pediatric population or evidence of the frequently mentioned concern of the development of tolerance to Senna. Pediatric caregivers should advise families of the rare side effect of skin blistering and educate them to change the diaper frequently in children who are not toilet- trained to reduce stool to skin exposure. We can conclude from this review that Senna is a safe treatment option for constipation in children. Level of evidence: IV