Victoria A. Lane

Surgical decision-making in the management of children with intractable functional constipation: What are we doing and are we doing it right?

BACKGROUND Children with intractable functional constipation (FC) may eventually require surgery, often guided by motility testing. However, there are no evidence-based guidelines for the surgical management of intractable FC in children. AIM To assess the diagnostic and surgical approach of pediatric surgeons and pediatric gastroenterologists towards children with intractable FC. METHODS A survey was administered to physicians attending an international conference held simultaneously in Columbus (Ohio, USA) and Nijmegen (the Netherlands). The survey included 4 questions based on cases with anorectal and colonic manometry results. RESULTS 74 physicians completed the questionnaire. Anorectal manometry was used by 70%; 52% of them would consider anal sphincter botulinum toxin injections for anal achalasia and 21% would use this to treat dyssynergia. Colonic manometry was used by 38%; 57% of them reported to use this to guide surgical decision-making. The surgical approach varied considerably among responders answering the case questions based on motility test results; the most commonly chosen treatments were antegrade continence enemas and anal botulinum injections. CONCLUSION Surgical decision-making for children with intractable FC differs among physicians. There is a need for clinical guidelines regarding the role of anorectal and colonic manometry in surgical decision-making in children with intractable FC.

Determinants of quality of life in children with colorectal diseases

BACKGROUND Health related Quality of Life (HRQoL) is an important outcome in medical care. The aim of our study was to identify characteristics associated with lower HRQoL scores in children with anorectal malformation (ARM) and Hirschsprung disease (HD). METHODS Patients younger than 18years, with HD or ARM, who were evaluated at our center from April 2014 to August 2015, were identified. The results of comprehensive questionnaires regarding diagnosis, symptoms, comorbidities and previous medical/surgical history, and validated tools to assess urinary status, stooling status and HRQoL were evaluated. RESULTS In children aged 0-4years, vomiting and abdominal distension were found to be associated with a significant reduction in total HRQoL scores. In children >4years of age, vomiting, nausea, abdominal distension, and abdominal pain were also associated with a significantly lower HRQoL. The strongest predictor of lower HRQoL scores on regression tree analysis, in all age groups, was the presence of a psychological, behavioral or developmental comorbidity. CONCLUSION Patients with either HD or an ARM who have a psychological, behavioral or developmental problem experience significantly lower HRQoL than children without such problems, suggesting that provision of behavioral/developmental support as part of the multidisciplinary care of these children may have a substantial impact on their HRQoL.

Rectal atresia and anal stenosis: the difference in the operative technique for these two distinct congenital anorectal malformations

Rectal atresia and anal stenosis are rare forms of anorectal malformations. The aim of the definitive surgical repair in such cases is to preserve the anal canal, the dentate line, and the sphincter complex. We present a case of rectal atresia and anal stenosis to demonstrate the differences in the operative repair. The techniques described leave the anterior wall of the very distal anal canal untouched in both rectal stenosis and anal atresia; however, the dissection of the rectum differs. The atretic rectum in rectal atresia is mobilized and sutured to the anal canal circumferentially. In anal stenosis, the posterior rectum is mobilized in the form of rectal advancement, and the posterior 180° is anastomosed directly to the skin (as in a standard PSARP) with preservation of the anal canal as the anterior 180° of the final anoplasty. These patients have an excellent prognosis for bowel control and fecal continence, and therefore, complete mobilization and resection of the anal canal must be avoided.

Surgical Management of Functional Constipation: Preliminary Report of a New Approach Using a Laparoscopic Sigmoid Resection Combined with a Malone Appendicostomy

Introduction Functional constipation is a common problem in children. It usually can be managed with laxatives but a small subset of patients develop intolerable cramps and need to be temporarily treated with enemas. The senior author has previously reported: 1) open sigmoid resection as a surgical option, but this did not sufficiently reduce the laxative need, then 2) a transanal approach (with resection of rectosigmoid), but this led to a high rate of soiling due to extensive stretching of the anal canal and loss of the rectal reservoir. The understanding of these procedures’ results has led us to use a laparoscopic sigmoid ± left colonic resection with a Malone appendicostomy for these patients, to decrease the laxative requirements, temporarily treat with antegrade flushes, and to reduce postoperative soiling. Methods A single‐institution retrospective review (3/2014‐9/2015) included patients who failed our laxative protocol, and therefore were considered surgical candidates. Patients with anorectal malformation (ARM), Hirschsprung disease, spina bifida, tethered cord, trisomy 21, cerebral palsy, mitochondrial disease, prior colon resection at other facilities, or those that did not participate in our laxative program were excluded. Demographics, duration of symptoms, prior treatments, postoperative complications, and postoperative bowel regimens were evaluated. Results A total of 6 patients (3 males; median age of 12.5 years) presented with soiling related to constipation and intolerance to laxatives. Four patients failed preoperative cecostomy (done prior to referral to us). An average of 4.7 medication treatments were previously tried. In all, 4 patients had required in‐patient disimpactions. Duration of symptoms was 7.5 years (median). The median senna dose was 30 mg (range, 15‐150 mg), and all patients had intolerable symptoms or failed to empty their colon, which we considered a failed laxative trial. All had contrast enemas that demonstrated a dilated and/or redundant sigmoid colon, and colonic manometry was abnormal in 4. All patients underwent laparoscopic sigmoid and left colon resection, or only sigmoid resection (a low anterior resection). Two patients had postoperative colitis treated with oral antibiotics. The median follow‐up was 52 days (range, 8‐304 days). Five patients are on antegrade enemas with plans to convert to laxatives at 6 months, 1 is taking laxatives alone at a 33% lower dosage. Five of six are completely clean, 1 soils occasionally and their daily flush is being adjusted. Conclusion Only a minority of patients with functional constipation are medically unmanageable. This preliminary report shows that laparoscopic colon resection combined with antegrade flushes is an effective surgical technique to treat that group. A laparoscopic approach, guided by contrast enema and colonic manometry, allows for a defined resection of the abnormal segment of colon with the advantages of minimally invasive surgery including allowing for an extensive rectal resection (an improvement over open sigmoid resection) and avoidance of overstretching of the anal canal and removal of the rectal reservoir (an improvement over the transanal approach). Having antegrade access is useful to manage soiling and avoiding cramping from laxatives in the early postoperative period. Although our series is small, we believe that long‐term most patients can avoid antegrade flushes and be on no, or a dramatically reduced, laxative dose.

The Appendix and Aganglionosis. A Note of Caution—How the Histology Can Mislead the Surgeon in Total Colonic Hirschsprung Disease

We present the case of a child with presumed total colonic Hirschsprung disease (HD) to highlight the problems the surgeon is likely to encounter if he/she relies on the appendix alone for histopathologic diagnosis. A newborn male infant, who was presumed to have total colonic aganglionosis when the appendix was found to be aganglionic at the time of initial exploratory laparoscopy, was managed with an ileostomy in the newborn period; however, at the time of his planned pull-through procedure, the rectal biopsy revealed normal ganglion cells. The child was subsequently managed with ileostomy closure and observed for normal feeding and stooling prior to discharge home. We discuss the histopathologic findings of the appendix in separate cases of confirmed total colonic HD seen in our center, and review the normal histopathologic findings of the appendix.

Use of a Heineke-Mikulicz like stricturoplasty for intractable skin level anal strictures following anoplasty in children with anorectal malformations

INTRODUCTION We introduced a modification of the Heineke-Mikulicz technique to treat intractable skin level anal strictures post posterior sagittal anorectoplasty (PSARP). The aim of this article is to describe the technique and outcome in a series of patients. METHODS This was a retrospective evaluation of patients who had Heineke-Mikulicz like stricturoplasty performed for a post PSARP skin level stricture over a one-year period. RESULTS Five patients who were operated using the technique were reviewed. All had severe anal strictures that could admit Hegar dilator sizes 6 to 9 at 16months to 5years after PSARP. All underwent routine dilatations, which became increasingly painful. As an alternative to continued dilatations, an operative procedure was offered. The surgery was done as a day case and lasted 10 to 30min. The anus at the end of the procedure could comfortably accept a Hegar dilator size 14 to 17. None of the patients had a colostomy after the procedure and there were no complications. CONCLUSIONS The Heineke-Mikulicz like stricturoplasty is a simple surgical procedure that can be done in an ambulatory setting to treat children with intractable skin level anal stricture if this develops following definitive surgery for anorectal malformations.

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

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

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.

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

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.

Gynecologic anatomic abnormalities following anorectal malformations repair

INTRODUCTION  Previous research in children with Hirschsprungs disease (HD) and Downs syndrome (DS) has focused on colorectal outcomes. We set out to review urinary outcomes in this patient group. 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.