ANAL RECTAL MALFORMATIONS
(ARMs)
PRESENTER:- SR. MASSENGA
GENERAL SURGERY DEPARTMENT
SUBTOPICS
 LIST OF ABBREVIATION
 DEFINITION
 EPIDEMIOLOGY
 CLASSIFICATION
 CLINICAL PRESENTATION
 ASSOCIATED ANOMALIES
 INVESTIGATION
 TREATMENT
 COMPLICATIONS
 BMC CHALLENGES
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
Definition
• ARMs
are birth defects in which the rectum and anus are
malformed, do not develop properly.
ARMs
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.
ROUTES
Rectum
Colon
Anus
Female
Males
RVF
RVF
RUF
RSF
RVF
Rectal Terminus
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.
QUESTION
DO WE HAVE
PATIENTS WITH
ARMs?
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
8
JUNE
357
122
20
6
JULY
416
92
11
8
AUGUST
397
120
17
4
SEPTEMBER
314
133
18
5
OCTOBER
385
140
14
6
NOVEMBER
379
96
25
5
DECEMBER
334
92
11
9
3872
1283
175
75
TOTAL
BMC DATA 2014
1. 6% ARMs out of surgical pediatric pts
2. 13.6% ARM out of other CMF
3. 40.6% operated out of ARMs
• Why 49.4% were not operated?
BMC DATA 2015
40 Children
9 Children
5 Children 1° surgeries
4 Children= 10% definitive surgeries
WHY THE REST WERE NOT OPERATED?
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
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.
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
ARM
Pelvic
diaphragm
is composed
of 3 muscles
HIGH OR LOW ARM
HIGH OR ARMs
2. CLASSIFICATION OF ARM, INVERTOGRAM
3. CLASSIFICATION OF ARM
FEMALES
MALES
Rectoperineal fistula
Rectoperineal fistula
Rectovestibular fistula
Rectourethral fistula
Bulbar
Prostatic
Persistent cloaca
<3cm common channel
>3cm common channel
Imperforate anus withought
fistula
Rectal atresia
Rectovesical fistular
Imperforate anus withought
fistula
Rectal atresia
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
ARM
ARM
ARM
ARM
ARM
RECTAL ATRESIA
ARM
RVF
ARM Bucket handle deformity
ARMCLOACA
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
ARMBEZARRE
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
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
CLINICAL PRESENTATION
• Imperforate anus is a clinical diagnosis based on
careful inspection of the perineum.
No anal opening…imperforate anus
• Single perineal opening…Cloaca
Low imperforate anus in a male. “Bucket
handle” shown at site
of covered anus with small amount of
meconium extruding (arrow).
LOW ARM
Well-developed median raphe and
anal dimple.
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
HIGH ARM
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
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."
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.
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
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.
• "The most complex the anorectal anomaly,
the more likely the presence of an associated
vertebral and spinal anomaly"
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
ASSOCIATED MALFORMATIONS
• LIMBS
Clubfeet
• Invertogram
INVESTIGATONS
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.
INVESTIGATONS
Colostogram
For imperforated
anus with fistula.
It shows
colourethral
fistula.
This is high
imperforate anus
in male
INVESTIGATONS
• Retrograde barium enema/ vascular contrast
Lateral view
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
TREATMENT
• Prophylactic antibiotics
• Colostomy-emergency vs elective
Definitive surgery-Elective
• Low ARM…PSARP
• High ARM…Pull down
• HD…Pullthrough
BMC EXPERIENCE
TAKE HOME MESSAGE
ARMs are unpreventable.
They are surgically treatable.
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.
6. Peña A. Anorectal malformations. In: Ziegler M, Azizkhan
R, Weber T, eds. Operative Pediatric Surgery. McGraw Hill
Publishers, 2003, Pp 739–761.
7. Peña A. Atlas of Surgical Management of Anorectal
Malformations. Springer-Verlag, 1989.
8. Peña A. Total urogenital mobilization: an easier way to repair
cloacas. J Pediatr Surg 1997; 32:263–268.
9. Peña A, Guardino K, Tovilla JM. Bowel management for fecal
incontinence in patients with anorectal malformations. J Pediatr
Surg 1998; 33:133–137.
10. Malone PS, Ransley PG, Kiely EM. Preliminary report: the
antegrade continence enema. Lancet 1990; 336:1217–1218.
11. 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.
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.