Anorectal malformations in children

Dhanya VJ, Assistant Professor,SUM nursing college

1. ANORECTAL MALFORMATIONS DHANYA VJ ASSISTANT PROFESSOR CHILD HEALTH NURSING SUM NURSING COLLEGE

2. Anorectal malformations are defined as an arrest of rectal descent resulting in absence of an anal opening and occur during the 4 th -16 th week of gestation. DEFINITION

3. worldwide- 1 in 5000 live births genetic predisposition in certain families slight male preponderance common defect in females is recto vestibular fistula M ales- recto urethral fistula association with certain syndromes INCIDENCE

4. unknown associated with several other congenital anomalies of the urinary tract, rectal atresia and intestines. ETIOLOGY

5. The hind gut forms and extends into the tail fold in the 2 nd week of gestation   At about 13 day, a ventral diverticulum, the allentois or primitive bladder forms   The junction of allentois and hindgut becomes the cloaca into which the genital, urinary and intestinal tubes empty   This is covered by a cloacal membrane PATHOPHYSIOLOGY

6. The urorectal septum descends to divide the common channel into by forming the lateral ridges, which grows in and fuse by 7 th week.   Opening of posterior portion of membrane occurs in the 8 th week Failures in any of these steps lead to anorectal malformations. PATHOPHYSIOLOGY

7. Stenosis of the anus Anal membrane atresia Anal agencies Rectal atresia TYPES

8. Narrowing of the anus at levels 1 to 4cm above the anus. STENOSIS OF THE ANUS

9. ANAL MEMBRANE ATRESIA

10. Present with only anal dimple In female Imperforate anus type 3 with associated fistula Low rectovaginal fistula High rectovaginal fistula Recto peritoneal fistula ANAL AGENSIS TYPES

11. Recto vesical Recto urethral Recto peritoneal IN MALES

12. Anus connected with lower part of the vagina Low recto vaginal fistula

13. Anus communicate with upper part of the vagina High recto vaginal fistula

14. Anus connect with peritoneum Recto peritoneal fistula

15. Rectum communicate with bladder The sphincter mechanism is frequently deformed and often absent. The perineum looks flat Recto vesical fistula

16. Rectum communicate with urethra The sphincter mechanism usually is satisfactory; a few patients have poor perineal muscles and a flat- looking perineum Recto urethral fistula

17. Rectum communicate with peritoneum Recto peritoneal fistula

18. Pouch like structure in anus and rectum Rare anorectal malformations The defect is frequently discovered while rectal temperature is being taken. RECTAL ATRESIA

19. There is obstruction about 2cm above the skin level.These patients need protective colostomy. RECTAL ATRESIA

20. The rectum is completely blind and is usually found approximately 2cm above the perineal skin. The sacrum and the sphincteric mechanism are usually well developed . A colostomy is indicated during the newborn period. This defect is frequently associated with down syndrome. IMPERFORATE ANUS WITHOUT FISTULA

21. IMPERFORATE ANUS WITHOUT FISTULA

22. GENITOURINARY Vesicoureteric reflux Renal agenesis Ureteral duplication Cryptorchidism Hypospadiasis Bicornuate uterus Vaginal septums ASSOCIATED ANOMALIES

23. VERTEBRAL Presacral masses Meningocele Lipoma Dermoid

24. CARDIOVASCULAR Tetralogy of Fallot VSD TGA Hypoplastic left heart syndrome

25. Gastrointestinal Tracheoesophagel fistula Duodenal atresia Malrotation Hirschsprung disease

26. Low lesions Newborn examination reveals absence of an anal orifice in the correct position Normal position of anus is halfway between the coccyx and the scrotum or introitus Primary symptom is constipation Cases with anterior ectopic anus have no symptoms CLINICAL MANIFESTATIONS

27. If no anus or fistula present, it is called “covered anus” After 24 hours meconium bulging may be seen creating a blue or black appearance. In male, the perineal fistula may track anteriorly along the median raphe across the scrotum and even down the penile shaft, this is usually a thin track, with a normal rectum often just a few millimeter from the skin

28. In females, a low lesion enters the vestibule or fourchette . In this case, the rectum has descended through the sphincter complex.

29. In a boy with high imperforate anus, the perineum appears flat There may be air or meconium passed via the penis (urethra) when the fistula is high In rectourethral fistula, the sphincter mechanism is satisfactory, the sacrum may be bifid and the anal dimple is near the scrotum High lesions

30. In retrovestibular fistulas, the sphincter mechanism is poorly developed and sacrum is hypoplastic or absent. In boys with trisomy 21, all the features may be present, but there will be no fistula, the sacrum and sphincter mechanisms are usually well developed and prognosis is good.

31. In girls with high imperforate anus, there may be the appearance of a rectovaginal fistula. A true rectovaginal fistula is rare. Most fistulas to the vestibule outside the hymen orifice. These have a good prognosis with a normal sacrum and anal dimple and intact sphincter function Cont…

32. C linical evaluation and a urinalysis – male P erineal inspection - females DIAGNOSIS

33. I nvertogram

34. Mainly required before redo surgery. Clearly shows the anatomy of sphincter muscles, levator ani , muscle complex. Delineates the rectal pouch and fistula. Clearly shows the relationship between intestine and surrounding muscles . CT SCAN

35. Provides exquisite soft tissue imaging in all three plane. Better than CT in soft tissue imaging and no radiation hazard. Scan is expanded to include pelvis , kidneys and spinal cord. Clearly shows whether the pulled through intestine is within levator ani sling or not MRI SCAN

36. Early decision-making IV line - fluids and antibiotics Insertion of nasogastric tube is inserted Meconium is usually not seen at the perineum in a baby with a recto- perineal fistula until at least 16–24 hours. MANAGEMENT

37. Clinical inspection of the buttocks -flat "bottom" or flat perineum Operation can be performed during newborn period, and after 1year corrective surgical repair is performed colostomy or an anoplasty MANAGEMENT

38. Clinical evaluation and urine analysis X-ray, cross-table lateral film with the baby in prone position colostomy Decision-making for male newborns

39. SURGERY

40. Surgically created opening between the colon and the abdominal wall to allow fecal elimination. It may be temporary or permanent diversion TPYES Transverse colostomy Descending colostomy COLOSTOMY

41. DESCENDING COLOSTOMY made from the descending portion of the colon located in the lower-left quadrant of the abdomen, with separated stomas . The proximal stoma is connected to the upper gastrointestinal tract and drains stool. The distal stoma is connected to the rectum and will drain small amounts of mucus material.

42. Colostomy

43. P erformed at 1–2 months of age 90% of male patients done anorectal repair, while 10% require laparotomy Rectobladder neck fistula operation involves both a posterior sagittal incision and an abdominal component, which can be done with laparoscopy or laparotomy . Anorectal repair

44. Rectovestibular Fistulas Rectovestibular fistula is the most common defect in girls and has an excellent functional prognosis. The diagnosis is based on clinical examination. A meticulous inspection of the neonatal genitalia allows the clinician to observe a normal urethral meatus and a normal vagina, with a third hole in the vestibule, which is the rectovestibular fistula .

45. Rectovestibular Fistulas

46. The laparoscopically -assisted anorectal approach consists in mobilizing and bringing the rectum through the pelvic floor sphincter muscles through a minimal posterior incision. Perianal dissection towards the laparoscopic light source favours accurate placement of a trocar to pull the rectum through the external sphincter muscle complex Laparoscopically assisted anorectal approach

47. Thorough physical examination by the attending nurse to identify anorectal malformation kept nil per orally Colostomy care Start feeding after the child has recovered from anaesthesia Nursing management

48. After the diagnosis of occlusion had been made, gastric suction may be used. IV feeding and nasogastric suction help prevent abdominal distension immediate post operatively Keep the anal site clean and dry Diaper should not be used. The dressing must be changed frequently Nursing management

49. Wound infection and retraction Incontinence Strictures (rectal or vaginal) Acquired atresias Recurrent fistulas Severe pelvic fibrosis Rectal mucosal prolapsed Constipation COMPLICATIONS