MULTIDISCIPLINARY COLORECTAL CARE FOR PATIENTS WITH ANORECTAL ANOMALIES, HIRSCHPRUNG'S, AND IDIOPATHIC CONSTIPATION Jessica Roybal, MD, MPH Pediatric Surgery Erika Smith, MD Pediatric Gastroenterology Ochsner for Children New Orleans, Louisiana PATRICIA 7 yr old F followed by GI for several years for constipation Born at term, unsure of when she passed meconium Her mom reports that she developed constipation around age 1, with introduction of whole milk Took away milk, started using enemas at age 2. Started miralaxand exlax chews at age 3. Constipation worsened when she started school, her mom thought she was withholding stool Seen initially by GI at age 4. Contrast enema done, followed by a cleanout. Recs: high fiber diet, increased miralax, stool calendar, toilet sitting. Soon referred to physical therapy. Seen 4 times with little progress, then discharged Referred to Ochsner multidisciplinary Colorectal Clinic. PATRICIA Having stool accidents all the time. Does not stool in the toilet Her mom says she has no sense of when she needs to stool No urinary accidents Recs: Cleanout again, add senna Little improvement, repeat impaction Thyroid, celiac labs normal Tried high volume enemas at home little success To be continued 1
OBJECTIVES To describe the goals of the multidisciplinary Colorectal Clinic at Ochsner To define bowel continence To discuss true incontinence vs pseudoincontinence To review Hirschsprung s disease and anorectal malformations, their work-up, and the longterm issues in patients affected To outline management of refractory constipation and encopresis and to introduce a bowel management program We have no disclosures PEDIATRIC COLORECTAL CLINIC Patients: Hirschsprung s disease Anorectal malformations Functional constipation/encopresis refractory to primary GI intervention When and where? Second Thursday afternoon of each month, Pediatric Building, 2 nd floor Team: Jessica Roybal, M.D. pediatric surgeon Erika Smith, M.D. pediatric gastroenterologist Lisette Webre. P.T. physical therapist Jill West, Ph.D. psychologist (starting soon) Goal: Continence! True or articifial NORMAL DEFECATION Rao. Clinical Gastro and Hepatol, 2010; 8:910-9. 2
BOWEL CONTINENCE 3 necessary components for bowel control Sphincter control Anal sensation Recto-sigmoid motility (reservoir) Without any one of these, can develop partial or total incontinence Bischoff A et al. J Ped Surg, 2009. INCONTINENCE INCONTINENCE True incontinence Pseudo-incontinence Congenital Acquired Functional constipation Anorectal malformation Hirschsprung s (Duhamel) Meningomyelocele Pre-sacral tumor Absent/poor sacrum Anorectal malformation Post-op complication Hirschsprung s Anorectal Malformation Kirk Thame presentation Constipation and Encopresis HIRSCHSPRUNG S DISEASE 3
HIRSCHSPRUNG S PATIENT 2 yo M diagnosed with HD at birth Underwent a Swenson pullthrough Did well for the first year 12 mos admitted for gastroenteritis vs enterocolitis. Brief course of rectal irrigations and flagyl and got better. Admitted again within 1 month for bloody stools which resolved quickly. KUB showed a dilated rectum. Follow up EGD showed gastritis and colonoscopy showed patchy erythema of the colon. Placed on miralax took it for a while, then parents stopped it bc he was stooling well 7 mos later, having some intermittent constipation. Restarted miralax HIRSCHSPRUNG S PATIENT 2 yrs of age, more constipated. Started potty training and seemed to be withholding. Seen in CRC. KUB showed massively dilated colon Contrast enema?kink Diagnostic laparoscopy no obstruction Rectal biopsy - normal ganglion cells Botox to internal anal sphincter with some improvement but short-lasting Repeated Botox at a higher concentration much improved. Continuing senna, toilet sitting, rewards for not withholding. HIRSCHSPRUNG DISEASE Most common distal intestinal obstruction in newborns Incidience: 1/4,000 10% have Down s syndrome Failure to pass meconium in first 24hrs May also present as severe constipation in an older child May present with enterocolitis 75-80% - recto-sigmoid 12% - long colonic segment 8% - total colonic 4
SURGERY FOR HIRSCHSPRUNG S DISEASE SHORT AND LONG-TERM ISSUES IN HIRSCHSPRUNG PTS 1. Obstructive symptoms/constipation *most common* 2. Enterocolitis 3. Soiling May have a combination of these These usually resolve by age 5 Only a small percentage of patients (~3%) need a second surgery PERSISTENT OBSTRUCTIVE SYMPTOMS AFTER PULL-THROUGH FOR HD Mechanical obstruction i.e. stricture, obstructing muscle cuff after a Soave, retained spur after a Duhamel, twist in the pulled through bowel Recurrent or acquired aganglionosis needs a biopsy of the neorectum Disordered motility in the proximal colon or small bowel if severe, may benefit from antegrade enemas Internal sphincter achalasia can treat with botox Functional megacolon caused by stool-holding behavior need bowel management 5
SOILING AFTER PULL-THROUGH FOR HD Abnormal sphincter function due to sphincter injury during pull-through or previous myectomy or sphincterotomy -> assess with manometry Abnormal sensation lack of sensation of a full rectum, inability to detect the difference between gas and stool (if anastomosis is done too low in anal canal) needs bowel mgt Pseudo-incontinence massively distended rectum -> overflow incontinence -> bowel mgt or hyper-peristalsis of the pulled-through bowel -> need to constipate and slow down with meds HIRSCHSPRUNG S ASSOCIATED ENTEROCOLITIS May be present before and/or after surgical correction of HD Can be severe or life threatening most common cause of death in children with Hirschsprung disease More common in: Children diagnosed at a younger age Pts with longer segment disease Clinical features: fever, abdominal distention, diarrhea (with explosive or foulsmelling stool), lethargy, elevated WBC, evidence of intestinal distension on AXR HIRSCHSPRUNG S ASSOCIATED ENTEROCOLITIS Treatment: intravenous fluids broad-spectrum antibiotics decompression of the rectum and colon with frequent rectal irrigations with normal saline ***Extremely important to educate the family about the risk of this complication and urge early return to the hospital if the child should develop any concerning symptoms*** 6
ANORECTAL MALFORMATIONS ANORECTAL MALFORMATIONS (ARM) Failure for the rectum to pass through the sphincter muscular complex Rectovestibular fistula Rectourethral fistula ANORECTAL MALFORMATIONS (ARM) ARM occur in 1/4000-5000 births Spina bifida occurs in 1-2/1000 births Can be associated with other defects Part of the VACTERL association 40% will have associated genitourinary abnormalities Likelihood increases as the fistula level gets higher 25% of patients with ARM suffer from fecal incontinence despite appropriate surgical intervention 7
ANORECTAL MALFORMATIONS Rectoperineal fistula -> Perineal anoplasty in the newborn period Female Male Rectovestibular fistula: Most common type of ARM in females ANORECTAL MALFORMATION TYPES Rectourethral and Recto-bladderneck fistulas (male only): ANORECTAL MALFORMATIONS TREATMENT 8
ANORECTAL MALFORMATIONS: LONG-TERM PROBLEMS Low lesions (rectoperineal and rectovestibular fistulas) High lesions (rectourethral and rectobladderneck fistulas) Constipation and fecal overflow Incontinence with soiling **Needs close follow up until potty-trained** PCP Pediatric Surgery GI +/- Physical therapist CONSTIPATION AFTER ARM REPAIR Constipation is the most common sequela after surgical repair of ARMS lower the malformation -> constipation more likely Vicious cycle: severe constipation -> megarectosigmoid -> more constipation -> overflow pseudoincontinence **Need to prevent constipation in these patients from the beginning!!!** if their constipation is managed appropriately, they become continent ARM OUTCOMES: CONTINENCE AND CONSTIPATION RATES ARM (n) Totally continent pts ARM (n) Constipation rate Rectoperineal fistula (52) Rectovestibular fistula (135) Rectobulbar urethral fistula (101) Rectoprostatic urethral fistula (105) Rectobladder neck fistula (46) 83% 64% 46% 18% 7% Rectoperineal fistula (72) Rectovestibular fistula (77) Rectobulbar urethral fistula (65) Rectoprostatic urethral fistula (47) Rectobladder neck fistula (7) 58% 55% 59% 42% 14% Totally continent: voluntary BMs with no soiling **Drs Pena and Levitt 9
ARM OUTCOMES: VOLUNTARY BMS AND SOILING RATES ARM (n) Voluntary BM rate ARM (n) Soiling rate Rectoperineal fistula (58) Rectovestibular fistula (146) Rectobulbar urethral fistula (112) Rectoprostatic urethral fistula (109) Rectobladder neck fistula (49) 97% 90% 79% 65% 20% Increasing incontinence Rectoperineal fistula (57) Rectovestibular fistula (135) Rectobulbar urethral fistula (105) Rectoprostatic urethral fistula (110) Rectobladder neck fistula (48) 16% 36% 49% 78% 90% **Drs Pena and Levitt INCONTINENCE IN ARMS Those with high lesions and those with associated spinal or sacral problems don t do as well With good bowel management, most patients can remain clean for 24 hours should be started before starting school Malone appendicostomy or Chait tube for antegrade enema administration (if older) REFRACTORY CONSTIPATION/INCONTINENCE 10 you male with history of encopresis. Stools once every other day, along with daily fecal soiling No urine accidents, hides underwear No pain, no vomiting, some distension Potty trained at 18mos Diet: water, sprite, fruit, no vegetables PMHx: ex 30WGA, stooled regularly until 6 mo of life; T&A Regimen: miralax 1 cap daily; recently had a cleanout, just had another accident today 10
REFRACTORY CONSTIPATION/INCONTINENCE Constipation early in life (50% < 1yo onset) Passage of large stools, obstruct the toilet Retentive posturing, anorexia Urinary incontinence, urinary tract infection Hiding dirty underwear Nonchalant attitude towards the fecal incontinence Lack of awareness of an episode PATHOPHYSIOLOGY Thame, K. Oral Presentation Constipation and Encopresis, 2013. REFRACTORY CONSTIPATION/INCONTINENCE History Past medical/surgical history: anorectal malformations, Hirschsprung s disease, myelominogocele, intestinal resections Stool history Withholding maneuvers Age of onset Urinary symptoms Physical Exam Weight, BMI Abdominal wall musculature, abdominal mass Lower extremity strength, tone, reflexes Abnormalities in the lumbosacral region Rectal exam 11
ASSESSMENT Labs: celiac, thyroid Gastrograffin enema Anorectal manometry ANORECTAL MANOMETRY ANORECTAL MANOMETRY Resting pressure Squeeze pressure Anorectal inhibitory reflex (RAIR) Measurement 70-85% internal anal sphincter Sympathetic motor (L1-L3) 70-85% external anal sphincter Pudendal motor (S2-S4) Sphincter relaxation reflex with balloon distension Myenteric plexus James Croffie Indiana Univ Powerpoint NASPGHAN, 2014. 12
APPROPRIATE SQUEEZE BEAR DOWN DYSSYNERGIA 13
RAIR Hirschsprung s disease TREATMENT 1. Evacuate all hard stool 2. Keep stool coming 3. Sit after meals Thame, K. Oral Presentation. Constipation and Encopresis, 2013. 14
TREATMENT 4. Diet 5. Physical therapy 6. Psychology Outpatient: Polyethylene Glycol Benefits: Relatively safe Pitfalls TREATMENT OF FECAL IMPACTION Significant volume which can lead to poor adherence, incontinence Enemas Benefits: can achieve goal faster Pitfalls Inpatient: Invasive, traumatizing, electrolyte disturbance NG Polyethylene Glycol Pitfalls Inpatient Significant, albeit small, risk TREATMENT OF FECAL IMPACTION Randomized control trial 90 participated (male, n = 60; mean age: 7.5 +/- 2.8 years) 46 patients enemas 44 patients PEG Successful disimpaction was achieved with enemas (80%) and PEG (68%; P =.28) No significant difference PEG more fecal incontinence, watery stools (P <.01) No difference in defecation frequency (P =.64), abdominal pain (P =.33), and behavior scores 15
Poor Quality of evidence available No placebo controlled studies evaluating disimpaction therapies Current recommendations for impaction: PEG 1-1.5g/kg/day for 3-6 days is first line therapy for fecal impaction Enema once per day for 3-6 days is recommended if PEG is not available Tubbers et al. J Ped Gastroenterol Nutr, 2014; 58(2): 258-74. MAINTENANCE Osmotic laxatives Polyethylene Glycol Lactulose Milk of magnesia Stimulant laxatives Bisacodyl Senna Behavioral modification Scheduled toilet sitting Avoiding snacks and high sugar products Physical therapy Therapy 18 studies (1643 patients) reviewed 9 agents to either placebo or each other. Limited evidence No RCT to evaluate dosing Conclusion: Polyethylene glycol > placebo, lactulose or milk of magnesia. Polyethylene glycol was generally safe & had lower rates of minor side effects compared to other agents. Gordon et al. Cochrane Review: Evid Based Child Health;,2013 Jan;8(1):57-109. 16
MAINTENANCE NASPGHAN RECOMMENDATIONS PEG first line Followed by lactulose if PEG not available Do not use intermittent enemas for chronic constipation Milk of magnesia, mineral oil and stimulant laxatives may be additional or second line therapy Quality of evidence was low Tubbers et al. J Ped Gastroenterol Nutr, 2014; 58(2): 258-74. IS POLYETHYLENE GLYCOL SAFE? In 2011, the F.D.A placed PEG on the AERS list. Reports of psychiatric/neurological symptoms in children taking PEG 3350. In 2015, the FDA awarded $325,000 to CHOP to investigate further Saint Louis, C. Scrutiny for Laxatives as a Childhood Remedy; Science. 2015. REFRACTORY CONSTIPATION/INCONTINENCE 10 you male with fecal soiling Labs normal (TSH, TTG IgA, IgA) Cleanout, followed by 2 tabs senna nightly, miralax 1 cap BID Restricted sugar PT, scheduled toilet sitting Now stooling 1-2 times per day in the toilet, accidents have resolved 17
BOWEL MANAGEMENT Purpose: Clean the bowel at one time, so the patient remains dry for the next 24 hours (artificial continence) Who is a candidate Ingredients Normal saline 200-1000mL Irritant Glycerin 10-40mL Castille soap 9-36mL Bisacodyl (10mg/30ml) 2.5mg intervals BARD FOLEY CATHETER 24Fr 30 cc balloon PATRICIA 7yo female with refractory constipation and encopresis who had normal labs, imaging up until this point Anorectal manometry done 18
PATRICIA 7yo female with refractory constipation and encopresis who had normal labs, imaging up until this point Anorectal manometry done Rectal biopsy consistent with Hirschsprung s disease Underwent a laparoscopic-assisted transanal Swenson pull through Removed 17cm of rectum and sigmoid colon because of very dilated segment Path: 2 cm of aganglionosis, another 2-3 cm of transition zone Initially did well post-op, back on senna Developed perianal skin irritation, constipation. No anastomotic issues Cleanout, increased senna, IEP for school QUESTIONS? THANK YOU! 19