1. IN THE NAME OF GOD EVALUATION AND TREATMENT OF FECAL INCOTINENCY IN CHIDREN Ahmad Khaleghnejad Tabari MD Pediatric Surgery Research Center, Mofid Chidren’s Hospital Shaheed Beheshti University of Medeical Sciences Tehran, Iran Second annual meeting of Iranian Continence Society, June 2011

2. Etiology Three major cause: 1-Congenital Anomalies 2-Mental Retardation 3-Childhood Encopresis

3. Congenital Anomalies 1- Myelomeningocele 2- Tethered cord 3- Lipomeningocele 4- High anorectal atresia (deficiencies of pelvic musculature and innervation ) 5- Three to five sacral missing

4. Acquired causes 1- Encopresis ( chronic constipation ) 2- Trauma to the sacrum and spinal cord 3- Anal sphincter destruction by systemic disease (Crohn’s disease, severe proctitis, extensive anorectal infection) 4- Inappropriate anorectal recostruction ( Imperforate anus, Hirschsprung’s disease, Ulcerative colitis ) 5 – Neurologically handicapped children

5. Evaluation of children with fecal incontinence 1- History 2- Physical examination 3- X-ray 4- Ultrasound 5- MRI 6- Anorectal manometry 7- Electrophysiologic study 7- Defecography

6. History 1- Normal bowel movement > intermittent incontinency >pschycologic 2- Congenital anomalies 3- Perirectal disease and operation 4- Neurologic impairment 5- Trauma

7. Physical examination 1- Abdominal palpation (mass, feces) 2- Stroking of the perianal skin-the external sphincter reflex-anal wink ( periphery sensory and motor nerves, reflex arc ) 3- Rectal examination ( fecal mass, strength of the anal sphincter, puborectalis muscle palpation ) 4- Lax anus, decrease perianal sensation, absence of the anal wink ( congenital or acquired neural deficiency )

8. Imaging studies 1- Lombo-sacral spine film ( Vertebral anomalies, sacral vertebra missing ) 2- Ultrasound (tethered cord, anal sphincters) 3- MRI ( tethered cord, levator and sphincteric complex, position of the anus )

9. 9 Hemisacrum with presacral mass. Currarino’s triad: 1.Anorectal anomaly 2.Sacral bone abnormality 3.Presacral mass

10. 10 Absent lumbosacral vertebrae, a severe vertebral anomaly.

11. Normal anatomy as viewed by anal endosonography (a)

12. Lateral internal anal sphincteratomy within the 6 to 10 o’clock position as viewed by anal endosonography (b)

13. Obstetric traum of the I & E sphincters within the 9 to 1 o’clock position as viewed by anal endosonography (c)

14. 14 Tethered cord

15. 15 Axial T1-weighted image shows the ectopic anterior location of the anal canal (arrow), ventral of the superficial transverse perineal muscle (arrowheads), and outside the normally developed external anal sphincter (curved arrow) Normal anatomy

16. 16 Unsuccessful repair PR-sling incomplete

17. 17 Axial SE T1-weighted image in a boy, after reconstructive surgery for a high anorectal malformation. The neorectum (black arrow) is positioned outside and to the right of a normally developed external anal sphincter (white arrow)

18. Functional studies 1- Anorectal manometry ( anorectal sphincter reflexes, sensation and coordination) 2- Three balloon probe 3- Rectal sensation in 10 mL 4- Rectal compliance 5- internal anal sphincter relaxation (rectoanal inhibitory reflex ) in 20 mL External sphincter contraction (rectoanal contraction )

19. Fecal incontinence associated with spinal cord injury

20. The reflex responsiveness of the anal region 1- rectoanal inhibitory reflex 2-rectoanal cotraction

21. Functional studies 1- Electrophysiologic assessment (pudental nerve terminal motor latency) 2- Defecography ( rectoanal angle, completeness of emptying and descent of the pelvic floor one cm below the pubococcygeal line)

22. Fecal incontinence associated with pudendal neuropathy (a)

23. Fecal incontinence associated with pudendal neuropathy (b)

24. Fecal incontinence associated with pudendal neuropathy (c)

25. Fecal incontinence associated with pudendal neuropathy (d)

26. TREATMENT Three approaches to treatment of incontinence : 1- Control of stool consistency 2- Conditioning or Biofeedback therapy 3- Operation to strengthen the sphincter muscles

27. TREATMENT BOWEL MANAGEMENT PROGRAMM Treatment in neurologic deficiency: ( myelominingocele, spinal malformations and variant of high imperforate anus ) 1- Dietary and pharmacologic manipulation to thicken the stool 2- Regular emptying of the rectum each morning with glycerin suppositories, saline enema or Bisacodyl suppositories within 30 minutes of a meal 3- Malone appendicocecostomy or sigmoidostomy tube for antegrade enema

28. TREATMENT Biofeedback Biofeedback therapy play a role in patients with decreased sphincter function 1- A rectal balloon manometry device is placed into the rectum 2- The rectal and sphincter pressures are shown to the patient 3- The rectal balloon is inflated and the patient is encouraged to contract the external sphincter in response 4- The sensation of rectal distention and external sphincter contraction is learned which may enhance continence

29. TREATMENT Encopresis In patients with encopresis associated with chronic constipation, incontinence is relieved when constipation is alleviated 1- Initial evacuation of stool by aggressive enema program, disimpaction in operating room, rectal water soluble contrast administration under fluoroscopy guidance 2- Administration of stool softeners, mineral oil, polyethylene glycol 3- Biofeedback therapy in pshycogenic incontinence 4- Malone appendicocecostomy or sigmoidostomy tube for antegrade enema 5- Resection of megasigmoid

30. Malone Appendicostomy

31. Megarectosigmoid in contrast enema

32. Resection of megarectosigmoid

33. TREATMENT OF INCONTINENCE AFTER ANORECTAL MAIFORMATION OPERATIONS Incontinence after repair with normal sacrum and appropriately positioned and functioning sphincter muscle, dietary manipulation and regular evacuation of rectum ( saline enema, Malone ) The rectum positioned inappropiately outside the levator of external sphincter muscles on PE, MRI, endosono and electromyographic localization, remedial operative correction via PSA is indicated

34. 34 Axial SE T1-weighted image in a boy, after reconstructive surgery for a high anorectal malformation. The neorectum (black arrow) is positioned outside and to the right of a normally developed external anal sphincter (white arrow)

35. TREATMENT OF INCONTINENCE AFTER ANORECTAL MAIFORMATION OPERATIONS Surgical transplantation of one or two gracilis muscles arround the external sphincter, stimulation with special devices prianal autologous fat injection may enhance continence Artificial anal sphincter devices Stem cell implantation for muscle formation

36. Thank you for your attention