Alessandra C. Gasior

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.

Surgical management of functional constipation: An intermediate report of a new approach using a laparoscopic sigmoid resection combined with malone appendicostomy

INTRODUCTIONnWe previously reported our surgical technique for functional constipation for patients who have failed medical management using a novel collaborative approach with gastroenterology input, pre-operative contrast enema, colonic manometry, and laxative protocol combined with a laparoscopic colonic resection with Malone appendicostomy. Now we report our intermediate outcomes.nnnMETHODSnPatients who failed bowel management program for functional constipation were reviewed from 3/2014-2/2017. Patients with Hirschsprung disease, anorectal malformation, tethered cord, spina bifida, Trisomy 21, cerebral palsy, mitochondrial disease, or prior colon resection were excluded.nnnRESULTSnOf 31 patients (14 females; median age 12years, follow-up 10.3months) with functional constipation and failed medical management, 26 (84%) had preoperative colonic manometry which, in addition to the contrast enema, guided laparoscopic colon resection. Ten patients (32.3%) are clean with no flushes (1 takes no laxatives, 8 are on low dose laxatives only, and 1 patient was clean on laxatives but chose to switch back to flushes). Of the 21 patients that remain on antegrade flushes, 20 (95.2%) are clean, and one patient (4.8%) continues to soil. We define clean as no soiling and no abnormal stool burden on x-ray. Laxative trials are planned for all patients on an antegrade flush regimen.nnnCONCLUSIONnOur intermediate results show that laparoscopic colon resection with Malone appendicostomy allows the majority of patients to be clean on antegrade flushes, and some to be on no or minimal laxatives.nnnTYPE OF STUDYnRetrospective review.nnnLEVEL OF EVIDENCEn3.

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

BACKGROUND AND AIMnSenna 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 Centers experience with Sennas secondary effects.nnnMETHODSnWe 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.nnnRESULTSnEight 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 60mg/day; 60 [12-100] vs. 17.5 [1.7-150] (p<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 15mg/day [4.4-150] vs. 17.5mg/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.nnnCONCLUSIONnThere 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.nnnLEVEL OF EVIDENCEnIV.

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

INTRODUCTIONnHealth 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.nnnMETHODSnWe 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.nnnRESULTSnWe included 233 caregivers. The average number of clinic visits was 3.5 over 1.2years. 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).nnnCONCLUSIONnEmphasis 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.nnnTYPE OF STUDYnCase Control/Retrospective Comparative Study.nnnLEVEL OF EVIDENCEnLevel III.

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

INTRODUCTIONnWe 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.nnnMETHODSnThis 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.nnnRESULTSnFive 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.nnnCONCLUSIONSnThe 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 descriptive model for a multidisciplinary unit for colorectal and pelvic malformations

INTRODUCTIONnPatients with anorectal malformations (ARM), Hirschsprung disease (HD), and colonic motility disorders often require care from specialists across a variety of fields, including colorectal surgery, urology, gynecology, and GI motility. We sought to describe the process of creating a collaborative process for the care of these complex patients.nnnMETHODSnWe developed a model of a devoted center for these conditions that includes physicians, psychologists, social workers, nurses, and advanced practice nurses. Our weekly planning strategy includes a meeting with representatives of all specialties to review all patients prior to evaluation in our multidisciplinary clinic, followed by combined exams under anesthesia or surgical intervention as needed.nnnRESULTSnThere are 31 people working directly in the Center at present. From the Centers start in 2014 until 2017, 1258 patients were cared for from all 50 United States and 62 countries. 360 patients had an ARM (110 had a cloacal malformation, 11 had cloacal exstrophy), 223 presented with HD, 71 had a spinal malformation or injury causing neurogenic bowel, 321 had severe functional constipation or colonic dysmotility, and 162 had other diagnoses including familial polyposis, Crohns disease, or ulcerative colitis. We have had 170 multidisciplinary meetings, 170 multispecialty outpatient, and 52 nurse practitioner clinics. In our bowel management program we have seen a total of 514 patients in 36 sessions.nnnCONCLUSIONnThis is the first report describing the design of a multidisciplinary team approach for patients with colorectal and complex pelvic malformations. We found that approaching these patients in a collaborative way allows for combined medical and surgical decisions with many providers simultaneously, facilitates therapy, and can potentially improve patient outcomes. We hope that this model will help establish new-devoted centers in other locations to encourage centralized care for these rare malformations.nnnLEVEL OF EVIDENCEnIV.

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

BACKGROUNDnPublished health-care costs related to constipation in children in the USA are estimated at

Can sacral development as a marker for caudal regression help identify associated urologic anomalies in patients with anorectal malformation

3.9 billion/year. We sought to assess the effect of a bowel management program (BMP) on health-care utilization and costs.nnnMETHODSnAt 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) 12u202fmonths before and 12u202fmonths after completion of the BMP were recorded.nnnRESULTSnOne hundred eighty-four patients were included (center 1u202f=u202f96, center 2u202f=u202f88). 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% (pu202f<u202f0.0005). Unplanned hospital admissions decreased from 65 to 28, i.e., a 56.9% reduction (pu202f<u202f0.0005).nnnCONCLUSIONnIn children with severe functional constipation, a structured BMP decreases unplanned visits to the ED, hospital admissions, and costs for constipation-related health care.nnnLEVEL OF EVIDENCEn3.

Simultaneous Robotic-Assisted Laparoscopy for Bladder and Bowel Reconstruction

BACKGROUND AND AIMnThe sacral ratio (SR) is a well-established tool to quantify sacral development in patients with anorectal malformations (ARM) and can be used as a predictor of fecal continence. We hypothesized that a lower SR correlated with the presence of urologic and renal malformations.nnnMETHODSnWe retrospectively reviewed the medical records of patients with ARM treated at our center from 2014 to 2016. We measured the lateral SR as a marker for sacral development and assessed the spine for the presence of tethered cord (TC). Urological and renal anomalies, including single kidney, hydronephrosis, hypospadias, vesicoureteral reflux (VUR), ectopic ureter, and penoscrotal transposition were assessed. Analysis of variance (ANOVA), t-tests, and multivariable linear regression were used to test for differences in SR with consideration of associated urologic malformations and tethered cord.nnnRESULTSn283 patients with ARM were included for analysis (156 females). The median age was 39months (10-90). Among these, 178 (55.6%) had 1 or more urologic malformations, and 81 (25.3%) had a TC. Hydronephrosis, high-grade VUR (3-5), solitary kidney, and tethered cord were significantly associated with lower SR (p<0.01). In multivariable regression models, the presence of urologic abnormalities remained significantly associated with lower a SR despite the presence or absence of TC (p<0.001).nnnCONCLUSIONnSR is a potentially useful indicator of certain urologic anomalies including hydronephrosis, high grade VUR, and solitary kidney in patients with ARM. This association is independent of the presence of TC. A sacral ratio as a part of the VACTERL screening can help the surgeon identify which patients need closer urologic follow up.nnnLEVEL OF EVIDENCEnIV.

Gynecologic anatomic abnormalities following anorectal malformations repair

INTRODUCTIONnPatients with neurogenic bladder frequently also have bowel dysfunction and a simultaneous urologic and colorectal reconstruction is possible. We present our experience with combined reconstructive procedures using robot-assisted laparoscopy, and demonstrate the utility of a minimally invasive approach that considers both the bowel and bladder management of these patients.nnnMETHODSnWe retrospectively reviewed all patients who underwent combined bowel and urologic reconstruction at our institution since the start of our multidisciplinary robotic program.nnnRESULTSnSeven patients were identified in our cohort with a mean age of 6.4 years (3.8-10.1 years). Six patients had myelomeningocele and 1 had caudal regression. Malone appendicostomies were placed in all 7 patients. A split appendix technique was used as a conduit in 5 patients, in situ appendix in 1, and neoappendicostomy with cecal flap in 1. Six patients had a Mitrofanoff appendiceal conduit created, while 1 patient had a sigmoid colovesicostomy for urinary diversion. Five patients required bladder neck repair. One patient had stenosis of the Mitrofanoff and one patient had an anastomotic leak of the sigmoid anastomosis. The average operating time was 526 minutes (313-724 minutes). The median length of stay (LOS) was 5 days (4-7 days), excluding one outlier who suffered an anastomotic leak and had an extended LOS (50 days). All patients who underwent continent bladder reconstruction are dry on their current catheterizing regimen, 6/7 are clean with antegrade flushes.nnnCONCLUSIONnPatients with neurogenic bladder often have coexisting bowel dysfunction, which provides an opportunity to reconstruct both organ systems simultaneously and achieve social urinary and bowel continence. Before committing to any intervention, the surgeon should consider both the urologic and gastrointestinal needs of the patient, and perform the needed procedures simultaneously. We describe a number of combined operations aimed at bowel and bladder management that can be performed safely using robot-assisted laparoscopy.