1. ANAL RECTAL MALFORMATIONS (ARMs)
PRESENTER:- SR. MASSENGA
GENERAL SURGERY DEPARTMENT

2. SUBTOPICS LIST OF ABBREVIATION DEFINITION EPIDEMIOLOGY CLASSIFICATION
CLINICAL PRESENTATION
ASSOCIATED ANOMALIES
INVESTIGATION
TREATMENT
COMPLICATIONS
BMC CHALLENGES

3. LIST OF ABBREVIATIONS ARM…Anal rectal malformation
PSARP… posterior sagittal anorectoplasty
CMF…Congenital malformation
ASD…Atrial septal defect
PDA…Patent ductus arteriosus
FOF…Tetralogy of Fallot
VSD…Ventricular septal defect
NGT…Nasal gastric tube
Abd uss…Abdominal ultrasound
ECHO…
NMRI…Nuclear magnetic resonance imaging

4. Definition
ARMs
are birth defects in which the rectum and anus are malformed, do not develop properly.
happen as a fetus is developing during pregnancy.
During a bowel movement, stool passes from the large intestine to the rectum and then to the anus.

5. Colon ROUTES Rectum Anus Female Males RVF RVF RVF RUF RSF
Rectal Terminus

6. INCIDENCE
Most authors have written that the average incidence worldwide is 1 in 5,000 live births.
Some authors have shown relationship btn families and ARM. There is genetic predisposition with ARMs being diagnosed in succeeding generations.
Not yet confirmed.
The causes of anorectal malformation are unknown.

7. DO WE HAVE PATIENTS WITH ARMs?
QUESTION
DO WE HAVE PATIENTS WITH ARMs?

8. YEAR 2014
Total No. of surgical pediatric pts
Total No. of pts with CMF
Total No. of patients with ARM
Total no of operated patients with ARM
JAN
252 85 13 3
FEB
297 97 16 5
MARCH
212 86 11 8
APRIL
244
116 10 6
MAY
290
104 9
JUNE
357
122 20
JULY
416 92
AUGUST
397
120 17 4
SEPTEMBER
314
133 18
OCTOBER
385
140 14
NOVEMBER
379 96 25
DECEMBER
334
TOTAL
3872
1283
175 75

9. Why 49.4% were not operated? BMC DATA 2014
1. 6% ARMs out of surgical pediatric pts
% ARM out of other CMF
% operated out of ARMs
Why 49.4% were not operated?

10. BMC DATA 2015
40 Children 9 Children 5 Children 1° surgeries 4 Children= 10% definitive surgeries WHY THE REST WERE NOT OPERATED?

11. BMC CHALLENGES 2015
1.Diagnostic tools i. Fluoroscopy machine was not working Cloaca 4patients(5, 3, 2,1) vascular contrast Other types of ARM, barium enema ii. Fresh frozen section machine Some children had Hirschsprung‘s disease iii. Rectal bleeding. Endoscopy; Low GI examinations/ Pediatric scopy 2. AGE; >1yr

12. PATHOGENESIS Embryology Hindgut.
When various steps in the process of development of organs (organogenesis) fail, anal malformations occur.
The exact etiology of this failure has not yet identified.

13. 1.CLASSIFICATION OF ARMs
Described at the WINGSPREAD CONFERENCE U.S.A in 1994
1. High,
2. Intermediate
3. Low ARM in relationship to the level of the
puboretalis portion of the levator ani Muscles.
For the purpose of simplifying the treatment, lesions were classified only as
High or
Low ARM depending on the position of the
puboretalis portion of the levator ani Muscles

14. ARM
Pelvic diaphragm is composed of 3 muscles

15. HIGH OR LOW ARM

17. HIGH OR ARMs

18. 2. CLASSIFICATION OF ARM, INVERTOGRAM

19. 3. CLASSIFICATION OF ARM FEMALES MALES Rectoperineal fistula
Rectovestibular fistula
Rectourethral fistula
Bulbar
Prostatic
Persistent cloaca
<3cm common channel
>3cm common channel
Rectovesical fistular
Imperforate anus withought fistula
Rectal atresia

20. ARM­DIAGRAMATICALLY Anal atresia Membranous anus Rectoscrotal
Rectovestibular anus
Rectal vesical
Rectourethral fistula
Anterior positioned anus
Posterior positioned anus
Rectovaginal
Rectoperineal
Bucket handle deformity
Anal sternosis
Rectal atresia

21. ARM

22. ARM

23. ARM

24. ARM

25. ARM
RECTAL ATRESIA

26. ARM
RVF

27. ARM Bucket handle deformity

28. ARM­CLOACA

29. Single perineal opening
ARM
Cloaca.
a situation that has urethral, vaginal and rectal openings all sharing a common single external orifice.
Single perineal opening
Cloacas with common channel/chamber, holds fecal matter, urine and reproductive fluid, they are eliminated through a single opening
Common channel less than 3cm
Good outcome >3cm

30. ARM­BEZARRE

31. ARM
The most common defect in females is imperforated anus with rectovestibular fistula
whereas the common defect in males is imperforated anus with rectourethral fistula

32. CLINICAL PRESENTATION
Thorough perineal inspection
1. If meconium is seen on perineum, it is evidence
of a rectoperineal fistula.
2. If meconium is in the urine, the diagnosis of a
rectourinary fistula is obvious.
1+2= low lesion

33. CLINICAL PRESENTATION
Imperforate anus is a clinical diagnosis based on careful inspection of the perineum.
No anal opening…imperforate anus
Single perineal opening…Cloaca

34. Low imperforate anus in a male
Low imperforate anus in a male. “Bucket handle” shown at site of covered anus with small amount of meconium extruding (arrow).

35. LOW ARM

36. Well-developed median raphe and anal dimple.

37. HIGH ARM Perineum is not stained with meconeum
High imperforate anus in a male. Median raphe is flat (not well formed)and without any signs of meconium extrusion.
No anal dimple

38. HIGH ARM

39. ASSOCIATED MALFORMATIONS
Approximately 60% of patients have an associated malformation.
VACTERL
V…Vertebral and spinal cord malformations
A…ARM
C…Cardiac
TE…TracheoEsophageal anomalies
R…Renal and other urinary tract malformations
L…Limb malformations

40. ASSOCIATED MALFORMATIONS
Many associated malformations are incidental findings, but other malformations like cardiac malformations may be life threatening
"If any one of these anomalies is discovered, it is
essential to search for the others."

41. ASSOCIATED MALFORMATIONS
Cardiac malformations CM
1/3 of the pts with ARM, have cardiac malformations. The most common lesions are ASD, PDA followed by the TOF and VSD defect.
The two most common cardiac anomalies are TOF and VSD defects.
Other malformations like transposition of the great arteries is rare.

42. ASSOCIATED MALFORMATIONS
TracheoEsophageal anomalies
Esophageal atresia
TracheoEsophageal fistula,
Gastrointestinal anomalies
Infantile hypertrophied pyloric sternosis
Duodenal atresia,
Duodenal web
Malrotations
Hirschsprung's disease
Abdominal wall anomalies
Omphalocele
Gastroschisis

43. ASSOCIATED MALFORMATIONS
Vertebral and spinal anomalies
Lumbosacral anomalies like butterfly vertebrae, hermivetebrae, hermisacrum and scoliosis are common.
The most frequent spinal anomalies is tethered cord, but spinal lipomas and myelomeningocele are also common.

44. "The most complex the anorectal anomaly, the more likely the presence of an associated vertebral and spinal anomaly"

45. ASSOCIATED MALFORMATIONS
GENITOURINARY ANOMALIES
The most common is vesicoureteric reflux the retrograde flow of urine from the bladder into the ureter, or kidneys G1-v, followed by renal agenesis, renal dysplasia internal structures of one or both of the baby's kidneys do not develop normally.
Others are cryptorchidism absence of one or both testes from the scrotum. It is the most common birth defect of the male genitalia and hypospadias urethral opening is ectopically located on the ventrum of the penis proximal to the tip of the glans penis

46. ASSOCIATED MALFORMATIONS
LIMBS
Clubfeet

47. INVESTIGATONS Invertogram for imperforated anus withought fistula.
a radiopaque marker on the perineum at the anal dimple.
Air acts as the contrast agent, it shows the location of the rectal terminus.

48. INVESTIGATONS
Colostogram For imperforated anus with fistula. It shows colourethral fistula. This is high imperforate anus in male

49. INVESTIGATONS
Retrograde barium enema/ vascular contrast
Lateral view

50. OTHER INVESTIGATIONs NGT…Esophageal atresia, ECHO…Cardiac malformation
Abdominal uss… Renal agenesis
Plain X-ray films… vertebral anomalies
Spine uss and lumbar NMRI for tethered cord

51. TREATMENT Prophylactic antibiotics Colostomy-emergency vs elective
Definitive surgery-Elective
Low ARM…PSARP
High ARM…Pull down
HD…Pullthrough

52. BMC EXPERIENCE

53. They are surgically treatable.
TAKE HOME MESSAGE
ARMs are unpreventable.
They are surgically treatable.

54. REFERENCES 1. Moore SW, Alexander A, Sidler D, Alves J, Hadley GP,
Numanoglu A, Banieghbal B, Chitnis M, Birabwa-Male D,
Mbuwayesango B, Hesse A, Lakhoo K. The spectrum of anorectal
in Africa. Pediatr Surg Int 2008; 24:677–683.
2. Uba AF, Chirdan LB, Ardill W, Edino ST. Anorectal anomaly: a
review of 82 cases seen at JUTH, Nigeria. Niger Postgrad Med J
2006;13(1):61–65
3. Adejuyigbe O, Abubakar AM, Sowande OA, Olayinka OS, Uba
AF. Experience with anorectal malformations in Ile-Ife, Nigeria.
Pediatr Surg Int 2004; 20:855–858.
4. Archibong AE, Idika IM. Results of treatment in children with
anorectal malformations in Calabar, Nigeria. S Afr J Surg 2004;
42(3):88–90.
5. Johnson O, Ghidey Y, Habte D. Congenital malformation of anus
and rectum in Ethiopian children. Ethiop Med J 1981; 19(1):9–15.

55. 6. Peña A. Anorectal malformations
6. Peña A. Anorectal malformations. In: Ziegler M, Azizkhan R, Weber T, eds. Operative Pediatric Surgery. McGraw Hill Publishers, 2003, Pp 739– Peña A. Atlas of Surgical Management of Anorectal Malformations. Springer-Verlag, Peña A. Total urogenital mobilization: an easier way to repair cloacas. J Pediatr Surg 1997; 32:263– Peña A, Guardino K, Tovilla JM. Bowel management for fecal incontinence in patients with anorectal malformations. J Pediatr Surg 1998; 33:133– Malone PS, Ransley PG, Kiely EM. Preliminary report: the antegrade continence enema. Lancet 1990; 336:1217– Meier DE, Foster E, Guzzetta PC, Coln D. Antegrade continent enema management of chronic fecal incontinence in children. J Pediatr Surg 1998; 33(7):1149–1152.

56. TREATMENT
A posterior sagittal anorectoplasty can then be performed between 2 and 12months of age, followed by colostomy reversal.
At two weeks postoperatively the anus is sized with a Hagar dilator (digital anal dilatation) and the caregiver is instructed on frequent home dilatations.
The colostomy may then be closed in six weeks or any convenient time after that.