Anorectal malformation

pediatric nursing

1. Anorectal malformation Presented by Mrs. Arifa T N Second year M.Sc Nursing MIMS CON

2. Introduction Anorectal malformations refer to anomalies of the rectum and distal anus, the urinary tract, and the genital tract. They have an incidence of approximately 1 in 5000 live births worldwide These malformations may range from simple imperforate anal to include other associated complex anomalies of genitourinary (GU) and pelvic organs, Which may require extensive treatment for fecal, urinary, and sexual function. The term imperforate anus (absence of the anal opening)

3. Anorectal malformations are frequently associated with other anomalies. Some babies have VACTERL conditions. VACTERL refers to the presence of three or more of the following anomalies: Vertebral anomalies, Anal atresia , Congenital heart disease, Tracheoesophageal fistula, Renal anomalies, and Limb defects Boys with an imperforate anus frequently have a rectourethral fistula and girls generally have a rectovestibular fistula.

4. Anorectal malformation Anorectal malformations are congenital malformation caused by abnormal development of the rectum and anus.

5. Etiology Generally any congenital malformation can cause ARM Family history unusual Autosomal recessive when genetically linked Incidence 1/5000 live births

6. Embryology and Pathophysiology The major part of the anus and rectum develops during 4 th to 6 th weeks of IUL The cloaca becomes the common channel for developing the urinary, genital and rectal system The division of cloaca occurs at 6 th week forms the an anterior urogenital sinus and posterior intestinal channel by the urorectal septum. Failure or any interception in the division of cloaca into the urogenital tract and the rectum results in the high and intermediate type of anomalies .

7. Classification Infant with out normal anus With a visible abnormal opening Anal stenosis Ano perineal fistula Anovestibular fistula with an invisible but manifested opening of the bowel Rectovagianal fistuala Recto urethral fistula Rectovesicular fistula No manifested opening of the bowel Persistant anal membrane Rectal atresia

8. Clinical features No or abnormally formed anal opening Failure to pass meconium within the first 24 hours of birth may be indicative of imperforate anus Fistula Presence of mechonium in urine Progressive abdominal distension Vomiting Rectal tube cannot be inserted

9. Imperforate anus Anal stenosis Anal agenesis Rectal agenesis Recto perineal fistula Rectovaginal fistula Cloacal malformations in girls, in which the urinary tract, vagina, and rectum fuse together, forming a common channel

10. diagnosis Physical examination Cystoscopy An IV pyelogram and a voiding cystourethrogram are performed to evaluate associated anomalies involving the urinary tract. Other diagnostic examinations that may be performed include pelvic MRI, radiography, ultrasonography, and fluoroscopic examination of pelvic anatomic contents and lower spinal anatomy.

11. Management Newborn is stabilized andkept NPO for further evaluation IV fluids are provided to maintain glucose and fluid and electrolyte balance. Current recommendation is that surgery be delayed at least 24 hours to properly evaluate for the presence of a fistula and possibly other anomalies

12. Management Surgical Varies according to the defect Low ARM Rectal cut back anoplasty or Y-V plasty Dialation of fistula High ARM Colostomy Posterior sagittal anorectoplasty (PSARP) or other pull-through with colostomy

13. This surgery generally occur between 3 to 6 months of age, although timing varies among surgeons. When the operative site has healed, approximately 2 weeks after surgery, anal dilations are begun. When the desired size of the anal opening has been achieved, the colostomy is closed

14. Complications UTI Intestinal obstruction Fecal impaction Colostomy related problems Recurrence of fistula Anal stenosis Post operative complications

15. Nursing management Assisting in identification of anorectal malformations A newborn that does not pass stool within 24 hours after birth or has meconium that appears at a location other than the anal opening requires further assessment. Preoperative care includes diagnostic evaluation, GI decompression, Bowel preparation, and IV fluids

16. Nursing diagnosis Acute pain Impaired skin integrity Body image disturbance Interrupted family process Parental anxiety Knowledge deficit Risk for infection Risk fluid volume deficit Risk for electrolyte imbalance Risk for complications Pre-post operative nursing diagnosis

17. General preoperative care Postoperative nursing care after anoplasty is primarily directed toward healing the surgical site without other complications. A program of anal dilations is usually initiated then the child returns for the 2-week checkup Feedings are started soon after surgical repair, and breastfeeding is encouraged because it causes less constipation

18. In neonates with anomalies such as cloaca (female), rectourethral prostatic fistula (males), and vestibular fistula (females), a descending colostomy is performed to allow fecal elimination and avoid fecal contamination of the distal imperforate section and subsequent urinary tract infection in infants with urorectal fistulas. Postoperative nursing care is directed toward Maintaining appropriate skin care at the stoma sites (both distal and proximal), Managing postoperative pain, and Administering iv fluids and antibiotics. Postoperative ng decompression may be required with laparotomy , and Maintenance of appropriate drainage

19. The PSARP is a common surgical procedure for the repair of anorectal malformations in infants approximately 1 to 2 months after the initial colostomy Preoperative PSARP care often involves Irrigation of the distal stoma to prevent fecal contamination of the operative site Parents must be given accurate yet simple information regarding the infant's appearance postoperatively and expectations as to their level of involvement in the child's care Parents are instructed in perineal and wound care or care of the colostomy as needed Anal dilations may be necessary for some infants Parents should observe stooling patterns and observe for signs of anal stricture or complications

20. Information on dietary modifications and administrationof medications is included in counseling Quality of life enhanced for the child and family

21. Family Support, Discharge Planning, and Home Care Long-term follow-up is essential for children with complex malformations Toilet training is delayed, and complete continence is seldom achieved at the usual age of 2 to 3 years Bowel habit training, Bowel management irrigation programs, Diet modification, and Administration of stool softeners or fiber help children improve bowel function and social continence Daily bowel irrigations (if not acheived ) Support and reassurance are important during the slow progression to normal, socially acceptable function.

22. Evaluation The child’s pain is effectively managed Incisions heal without signs of infection. Fluid and electrolyte balance is maintained. Adequate bowel function is demonstrated. The parents demonstrate an understanding of ostomy care and other treatment protocols

23. Thank you