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1. Anorectal malformation
Introduction
Anorectal malformations (ARMs) are among the more frequent congenital anomalies
encountered in pediatric surgery, with an estimated incidence 1 in 5000 live births. Antenatal
diagnosis of an isolated ARM is rare. Most cases are diagnosed in the early neonatal period.
Anorectal malformations are birth defects or developmental deformities of the lower end of
the alimentary tract that is anorectal canal. With this defect, the anus and rectum don’t
develop properly. They are the lower part of the digestive tract.
Definition
Anorectal malformation is congenital anomalies of the anorectal canal or anal area may range
from simple imperforate anal to include other associated complex anomalies of genitourinary
(GU) and pelvic organs, which may require extensive treatment for faecal, urinary, and
sexual function.
Incidence
Anorectal malformations occur approximately in 1 in every 5000 live births. They are slightly
more common in males (1.2 to 1). The majority of male patients with an anorectal
malformation have some form of connection to the urinary system, or a recto-urethral fistula
(approximately 70% of this patient population). The most common type of anorectal
malformation in female patients is a recto-vestibular fistula.
Source author smith ac,avansino topic anorectal malformation last update date
august12,2020 https://www.ncbi.nlm.nih.gov/books/NBK542275/
Kanti Children's hospitals have served a total of 103 patients in the Surgical ICU of Kanti
Children’s Hospital, from October to December 2018. Anorectal malformation 9(October),
7 (November), 6(December)
Source:- https://realmedicinefoundation.org/wp-
content/uploads/2019/09/RMF_Nepal___Kanti_Children_s_Hospital___Q4_2018.pdf
Causes
a. Unknown
b. Congenital:-it occurs due to arrest in embryonic development of the anus, lower
rectum and uro-genital tract at the 8th
week of embryonic life.
c. It may be related with down syndrome and familial incidence
Classification
Depending on the relations with the levator muscles, ano-rectal malformation is classified
into:-
(i) Low anomalies: The rectum has decended normally through the puborectalis muscle,
the internal and external sphincters are present and well developed with normal
function and there is no connection to the genitourinary tract. In low anomalies, there
is 1.5 cm or less between the blind end of colon and anal dimple.
(ii) Intermediate anomalies:- The rectum is at the or below the level of the puborectalis
muscle; the anal dimple and external sphincter are positioned normally.
(iii)High anomalies:- The rectum ends above the puborectalis muscle and there is absence
of the internal sphincter. This is usually associated with a genitourinary fistula (recto

2. urethral in male or rectovaginal in fistula). In high anal agenesis, there is more than
1.5cm between the blind end of colon and anal dimple.
Pathophysiology
The ano- rectal and genitourinary tract originates from an embryologic structure called
cloaca. By 6 weeks of gestation, cloaca is divided into the anterior urogenital sinus and
posterior ano-rectal canal by a septum. The rectum and urinary tract separate completely by
the 7th
week of gestation. further development of the ano-rectal and uro-genital organs occur
up to 12 weeks of gestation.
Developmental failure during those period results several form of malformation with or
without an obvious anal opening. Anomalies reflect the stage of the development of these
processes. Failure to develop the urorectal septum results in a fistula between the bowel and
urinary tract(in boys) or the vagina (in girls). Many have a fistula from the distal rectum to
the perineum or genitourinary system.
Clinical presentation
a) Absence of anal opening or abnormally formed anal opening at birth (during perineal
examination)
b) Absence of meconium
c) Meconium discharge from other site than anus
d) Progressive distension of abdomen that may associate with vomiting.
e) Fistula formation and passage of stool through the fistula (on examination)
f) Ribbon like stool
g) Male:- fistula formed between rectum and vagina or perineum.
h) Female:- fistula formed between rectum and vagina or perineum.
Feature according to type of defects.
a. Imperforated anus:- Infants fails to pass meconium, and greenish bulging membrane
is seen on examination.
b. Anal stenosis :- Infants passes ribbon like stools with difficulty
c. Anal agenesis: failure to develop anus so presence of only anal dimple. Intestinal
obstruction develops, if there is absence of any fistula. Usually fistulas are found to
the perineum or urethra in male and perineum or vulva in female.
d. Rectal agenesis: it presents with fistula. In male baby, fistula may communicate with
posterior urethra and in female with upper vagina.
e. Rectoperineal fistula: it is manifested as small orifice in the perineum. In male baby it
is found close to the scrotum and in female at the vulva.
f. Rectovaginal fistula: it is the communication between rectum and vagina and stool
passes through the vagina.
Diagnosis Procedure
a. History and clinical presentations that is absence of anal opening at birth
b. Neonatal examination: assess patency of anus and rectum and perineal area of the
newborn for passage of stool at abnormal site.
c. Observation of the newborn regarding passage of meconium.
d. Abdominal USG: evaluate anatomic malformation
e. Urine examination for presence of meconium and epithelial debris in urine
f. An intravenous pyelogram (IVP) and cystourethrography are done for the infant with
suspected anomalies of the urinary tract.
g. Evaluation of the child for presence of other anomalies in the body

3. h. Electrocardiogram to rule out cardiac problem
Management
The reconstructive operation is needed to correct or repair the malformations. It depends
upon the sex of the baby and type of anomalies presence. General measures include:
a. Admit the child in tertiary level hospital.
b. With hold oral feeding.
c. Start intravenous fluid therapy.
d. Reassure the parents and explain the nature of anomalies as well as treatment process.
e. Administer vitamin 'k' injection and antibiotics as per prescription.
f. Maintain warmth to prevent baby from hypothermia.
g. Nutrition- feedings is started soon after surgical repairment and breast feeding is
encouraged to minimize the risk of constipation.
h. Monitoring vitals and record accurately.
i. Maintain strict intake output record.
Surgery
Surgery is indicated as a definitive treatment: Surgery for low ano-rectal malformations
includes rectal cutback anoplasty or y- v plasty for male and dilation of fistula with definitive
repairment and perineal anoplasty for female child. Dilation initiated when the child returns
for the second week's follow up visit.
In case of high ano-rectal malformations: Initial colostomy is done during neonatal period
and definitive reconstructive surgery as posterior sagittal ano-rectoplasty at the age of 10-
12months with infant is having weight7-9 kg. Thereafter colostomy closure is done 10-
12weeks of successful definitive surgery.
Note: anoplasty - moving the fistula opening to the center of the sphincter and enlarging the
rectal opening.
Type specific management
Anal stenosis: Manual dilation.
Imperforated anal membrane: Excision followed by daily dilatation.
Prophylactic antibiotics therapy.
Nursing Management
Assessment: Nursing assessment should focus on pass of meconium within 48 hours of birth,
and stool come from any abnormal opening.
Nursing Diagnoses
 Fluid volume deficit related to excessive loss through vomiting.
 Impaired skin integrity related to the colostomy.
 Risk for infection related to surgical procedures
Nursing Interventions
Avoid infection.
 Teach the caregivers to keep the area around the colostomy clean with soap and
water
 To diaper the baby in the usual way;
 Monitor white blood cell (WBC) count;

4.  wash hands and teach patient and
 Wash hands before contact with patients and between procedures with the patient.
Protect skin integrity.
 A protective ointment is useful to protect the skin around the colostomy;
 Monitor site of impaired tissue integrity at least once daily for colour changes,
redness, swelling, warmth, pain, or other signs of infection; and
 Keep a sterile dressing technique during wound care.
Restore balanced fluid volume.
 Administer parenteral fluids as prescribed;
 Immediate administered infusion of fluids for patients with abnormal vital signs
 Monitor both intake and output.
 Teach family members how to monitor output in the home;
Pre operative care
a. Inform parents about baby's condition
b. Maintenance of warmth.
c. Fluid and electrolytes therapy and NG decompression.
d. Written Consent.
e. Parental measurement instruction on surgical procedure,
f. Measurement of abdominal surgical girth.
g. Vitals monitoring and proper recording.
h. Physical preparation, pre-medications, etc.
i. Diagnostic care and collection of report.
j. Keep baby nil per orally
Post operative care
a. Maintain airway status.
b. Pain management
c. Care of soakage from suture site and other immediate post surgery care.
d. Measure to prevent from infection
e. Positioning of baby on side lying position with the hip elevated or a supine position
with the legs suspended at 90º to the trunk to prevent pressure over suture area.
f. Postoperative nasogastric decompression and drainage care.
g. Colostomy care and check for return of peristalsis.
h. Provide post-operative medication
i. Emotional support for family
j. Parents teaching on colostomy care at home, breastfeeding and other feeding, skin
care, bowel and bladder care, prevention of fecal impaction, regular bowel habit
training and follow up visit and keep perineal area dry and clean.
Complication
 Urinary infection
 Intestinal obstruction
 Faecal impaction
 Colostomy infection
 Fistula and anal stenosis
REFERENCE OF ANORECTAL MALFORMATION
 Uprety K, Child Health Nursing, fourth Edition (2071 Bhadra), Tara Books and
Stationery, Chhetrapati, Kathmandu, pg 138- 140

5.  Shrestha T. Essential Child Health Nursing. first Edition 2015,August. Medhavi
Publication; Jamal, Kathmandu Page no.286-290
 WilsonD, Rodgers CC, Hockenberry M, Wongs Essential of pediatric Nursing 10 th
edition, ELSEVIER, page no 1422-1425
 Adhikari T, Essential of Pediatric Nursing, first 2014 edition, vidyarthi pustak
bhadar, bhotahity, Kathmandu, page no177-182
 https://www.urmc.rochester.edu/encyclopedia/content.aspx?contenttypeid=90&conte
ntid=P01980#:~:text=Anorectal%20malformations%20are%20birth%20defects,end%
20of%20the%20large%20intestine
 https://realmedicinefoundation.org/wp-
content/uploads/2019/09/RMF_Nepal___Kanti_Children_s_Hospital___Q4_2018.pdf
 https://www.ncbi.nlm.nih.gov/books/NBK542275/