ANORECTAL MALFORMATIONS

Dr T.T. Sholadoye
Division of paediatric Surgery
ABUTH
 Objectives
• To discuss the
 embryology
 classification
 investigation
 treatment and
 Outcomes of treatment
of children with anorectal malformations
 Introduction
• Wide spectrum of congenital anomalies involving
the anus & rectum as well as the urinary and
genital tract

• Source of distress to the parents and a challenge

to the pediatric surgeon
• Faecal continence after surgery is the goal of
treatment
 Introduction(History)
• Recognized since antiquity

“An infant with an anal obstruction is not worth

rearing”
Soranus 200 AD (Circa)
 Historical perspective

• Paulus Aeginata:1st survivor of surgery ARM

• Ammusat (1835): 1st anoplasty

• Stephen (1953): preserving puborectalis

• Alberto Pena (1980): PSARP

 Introduction(Epidemiology)
• Incidence: 1: 4,000- 5,000 births

• Male : female almost equal( M>F in zaria)

• 60% have low anomalies, high lesion are
commoner in boys
• The most common type in males is ARM+ recto-urethral
fistula while in females its recto-vestibular fistula
 EMBRYOGENESIS
• Urorectal septum begin
to descend by 4th week
caudally towards cloacal
membrane.(8th week)
• Abnormality in these
folds and seperation
results in ARM
 ASSOCIATED
ANOMALY
• Most cases have associated
anomalies that affect other
systems. The higher ARM are
associated with more associated
anomalies. Some of these
anomalies e.g. CVS defects, can be
life-threatening.
• V.A.C.T.E.R.L-H
vertebral defects, anal atresia, cardiac defects,
tracheo-esophageal fistula, renal anomalies, and
limb abnormalities.
H : hydrocephalus
 ETIOLOGY
• Etiology unknown
• Genetic:
• 8% have Genetic causes
• The risk of a family having a second child with ARM 1%
• Environment: exposure to ethinylthiourea, retinoic
acid derivatives and Adriamycin
• Infections- cytomegalovirus, toxoplasmosis
• Father’s occupation- car manufacture plant
WINGSPREAD, 1984
PENA’S CLASSIFICATION - 1995
PENA’S CLASSIFICATION

Perineal fistula Vestibular fistula

Perineal fistula Bulbar & Prostatic fistula

 KRICKENBECK CLASSIFICATION – 2005

A consensus classification that group into major clinical groups

and rare/Regional Variant
 DIAGNOSIS
• Prenatal diagnosis ( usually non specific
features seen on radiological studies)

• Post natal diagnosis : mostly clinical (95%)

 CLINICAL FEATURES
• History: One of 3 main presentations

1. Absent anal orifice

2. Failure to pass meconium

3. Passage of meconium from an abnormal orifice.

 CLINICAL FEATURES
• Cutanous fistula may be situated anywhere from the normal anal
site to the tip of the penis
• Single perineal opening in female is persistent cloaca
• Flat buttocks is a clue to the underlying under development of
the muscle complex and bone in gluteal region

• Additionally features
– intestinal obstruction :
• Constipation
• Abdominal distension
• Colics
• Vomiting
– Hydrocolpus in persistent cloaca
• Associated anomalies
 INVESTIGATION OF ARM
• A – TO DETERMINED NEED FOR COLOSTOMY
• Decision on clinical ground (95%)
• CROSS TABLE LATERAL X-RAY: A gap > 1cm between the
gas shadow and skin usually represents a high anomaly.

• INVERTOGRAM: not frequently done in recent time.

• PERINEAL USS: performed after 24hrs may provide useful

diagnostic information, but this is highly operator dependent.
 INVESTIGATION OF ARM
• TO DETERMINED ASSOCIATED ANOMALIES
• ECHOCARDIOGRAPHY: Cardiac anomalies

• ABDOMINAL USS: Renal abnormalities

• SPINAL CORD IMAGING(X-ray, MRI,USS)

– Missing sacral vertebra
– Hemisacrum
– Hemi vertebra (thoraco lumber)
– Asymetric/short/straight protruding sacrum

• Orogastric tube to rule out esophageal atresia size 10 tube arrest at

10cm.
• TO DETERMINED FITNESS FOR SURGERY
• FULL BLOOD COUNT AND DIFFERENTIAL
• ELECTROLYTE, UREA & CREATININE, GXM ETC
 Initial Resuscitation
• A : clear the airway
• B : Breathing
• NG Tube for decompression
• N.P.O
• C : Circulation
• Intravenous fluids
• Urethral catheterization
• D : Antibiotics, colostomy
 COLOSTOMY

• Divided descending
colostomy
– Permits decompression
– Protects definitive repair
– Allows for distal
colostogram
 Surgical treatments

• Perineal operation: Anal Stenosis, Anoplasty,

• Posterior saggital anorectoplasty (PSARP )

• PSARVUP

• Laparoscopic-assisted pull-through
 POST – OP COMPLICATIONS
• EARLY:
– Wound infection
– Wound dehiscence
– Neurogenic bladder
– Urethral injury
– Necrosis of mobilized rectum
– Mortality- 10 -20% now 3%
– Complications of colostomy
 POST – OP COMPLICATIONS
• LATE :
– Anastomotic stricture
– Narrow introitus
– Urethro vaginal fistula
– Persistent recto-urethral fistula
– Prolapsed bowel
– Incontinence
– Constipation
 Prognostic Factors
• The level/type of anomaly

• Age at anorectal repair

• Associated congenital anomaly

• Presence of severe sacral anomaly

• Presence of internal sphincter

• Abnormal colonic motility

• Method of anorectal reconstruction

• Primary or secondary procedure

 Conclusion
• Anorectal malformation is the most common
neonatal cause of intestinal obstruction in
ABUTH, prompt evaluation and determination
of extent of disease with appropriate treatment
improves outcome
• Most patient achieves faecal continence and
near normal life
 References
• Ameh EA, Chirdan LB (2000) Neonatal intestinal obstruction in Zaria, Nigeria.
East Afr Med J 77:510–513

• Ameh EA, et al (2001) Emergency neonatal surgery in a developing country.

Pediatr Surg Int 17:448–451
• Paediatric surgery for Africa
 ABSENT ANAL OPENING
•
 MECONIUM SOMEWHERE
•
PERINEAL FISTULA
 FAILURE TO PASS MECONIUM

•
 Abdominal distension
•
bucket-handle malformation
“BUCKET HANDLE” DEFORMITY

03/27/2022 34
A flat perineum
A flat perineum-GIRL
TETHERED CORD
In anorectal malformation (ARM) colostomy may
be required in the following:
A. Perineal fistula
B. Rectovestibular fistula
C. Persistent cloaca
D. Rectal atresia in females
E. Rectal atresia in males
• Treatment of ARM:
A. Surgical repaire at birth in all cases
B. Definitive surgery at 6-9month in high type
C. Transverse colostomy is needed in all types
D. Anal dilatation is dangerous
E. Cysto-urethrogram should be done in all lesions
• Any Questions