Ano-Rectal Malformation

Subject: Child Health Nursing

Overview

Anorectal abnormalities are a group of illnesses that afflict both boys and girls that affect the distal anus and rectum, as well as the urinary and vaginal tracts. Defects range from the extremely small and readily treated to those that are complex, difficult to manage, frequently coupled with additional defects, and have a poor functional prognosis. It is a developmental deformity of the ano-rectal canal at the lower end of the alimentary tract. The main cause is unknown however, a Congenital cause can occur due to an arrest in embryonic development of the anus, lower rectum and urogenital tract at the 8th week of embryonic life.An absence of anus or presence of thin transparent membrane, an absence of meconium stool and green tinged urine are the symptoms of anorectal malformation.Therapeutic management for the anal membrane, it is incised or perforated with blunt instrument, for anal stenosis, only simple dilatation is performed by the doctor using dilator and the size of the dilator is increased progressively,and if there is a large distance between the anal opening and that central portion of the external anal sphincter, the anomalous anal opening is moved to the correct position, and the perineal body is reconstructed.

Anorectal abnormalities are a group of illnesses that afflict both boys and girls that affect the distal anus and rectum, as well as the urinary and vaginal tracts. Defects range from the extremely small and readily treated to those that are complex, difficult to manage, frequently coupled with additional defects, and have a poor functional prognosis. It is a developmental deformity of the lower end of the alimentary tract i.e. the ano- rectal canal.

Causes

  • Unknown
  • Congenital: it occurs due to arrest in embryonic development of the anus, lower rectum and urogenital tract at the 8th week of embryonic life.

Pathophysiology

During the sixth week of gestation, the rectum and anus begin to form from an enlarged part of the caudal hindgut. From the caudal hindgut to the cloaca to the uro-rectal septum. The rectum and urogenital sinus are now separated by the uro-rectal septum. A recto-anal membrane will grow from the rectum around the eighth week of gestation, and this membrane will break down and establish a continuous patent passage between the outside and the remainder of the gut. If the cloaca partitions abnormally in the uro-rectal septum, it results in anal deformity. Similarly, an external opening will emerge from the urogenital sinus to the lower half of the urogenital tract.

Clinical features

  • Absence of anus or presence of thin transparent membrane
  • Absence of meconium stool
  • Green-tinged urine
  • Meconium discharge from another site than an anus.
  • Poorly developed and dimple
  • Progressive abdominal distension
  • Ribbon-like stool

Types

  1. Imperforate anus: Infant fails to pass meconium and a greenish bulging membrane is seen on examination.
  2. Anal stenosis: Infant passes ribbon like stools with difficulty.
  3. Anal agenesis: Failure to develop anus so the presence of only anal dimple. Intestinal obstruction develops if there is an absence of any fistula. Usually, fistula is found to the perineum or vulva in a female.
  4. Rectal agenesis: It presents with fistula. In a male baby, a fistula may communicate with the posterior urethra and in a female with an upper vagina.
  5. Rectovaginal fistula: It is the communication between rectum and vagina and stool passes through a vagina.
  6. Rectoperineal fistula: It is manifested as a small orifice in the perineum.

Diagnosis

  • Physical examination
  • History and clinical presentation i.e. absence of anal opening at birth
  • Ultrasonography to locate the rectal pouch
  • X-ray: held the baby upside down (an inverter gram) in doubtful cases.
  • Urine examination for the presence of meconium and epithelial debris.
  • Micturating cystourethrogram (MCU) and intravenous pyelogram to rule out urinary abnormalities.
  • Electrocardiogram to rule out cardiac problems.

Therapeutic management

  • A blunt instrument is used to incise or perforate the anal membrane.
  • For anal stenosis, the doctor just performs simple dilatation using a dilator, gradually increasing the size of the dilator.
  • A cutback angioplasty is performed if the anal opening is anterior to the external sphincter and there is a limited space between the aperture and the center of the external sphincter.
  • If there is a significant gap between the anal opening and the central region of the external anal sphincter, the anomalous anal opening is shifted to the correct position, and the perineal body is rebuilt.

Nursing Management

  • Identification of undetected cases, such as those who have not passed meconium within 48 hours, and referral to the appropriate center.
  • Assist parents in accepting the condition and adhering to the treatment regimen.

Basic preoperative care includes care during diagnostic investigation and collection of a report:

  • Withholding feeding
  • GI decompression with NG tube drainage
  • IV fluids
  • Care of the colostomy if the infant/child has a colostomy.
  • Provide hygiene and general care to the child.
  • Ensure adequate fluid, electrolyte, and nutrition to the infant/child to improve the general health condition of the child.

Postoperative care:

  • Maintain proper hydration, electrolyte, and nutrient balance.
  • Frequent evaluation of the child's vital signs, intake, output, electrolytes, and so on.
  • Apply measures to reduce stress on the repaired area, such as not straining to defecate, avoiding sobbing and restlessness, and so on.
  • Encourage breastfeeding after surgery.
  • Liquid diet and low residue food for many days after surgery to reduce stress on the healed area. Later, stool softeners and laxatives are used.
  • Enema should be avoided since it can cause stress to the healed area.
  • To avoid perineum tension, instruct patients to avoid sitting positions.
  • With dressing, keep the perineum clean and dry. If there is any soakage, replace the perineal pad.

Prognosis

Depends on upon the type of ano- rectal malformations, associated malformations, fecal control a child's general health status

Complication

  • Intestinal obstruction
  • Urinary infection
  • Fecal impaction
  • Colostomy infection
  • Fistula
  • Anal stenosis

 
Things to remember
  • Anorectal abnormalities are a group of illnesses that afflict both boys and girls that affect the distal anus and rectum, as well as the urinary and vaginal tracts.
  • Defects range from the extremely small and readily treated to those that are complex, difficult to manage, frequently coupled with additional defects, and have a poor functional prognosis.
  • It is a developmental deformity of the anorectal canal, which is located at the lower end of the alimentary tract.
  • Anorectal malformation is characterized by the absence of anus or the presence of a thin transparent membrane, the absence of meconium stool, and the presence of green-tinged urine.
Videos for Ano-Rectal Malformation
Ano- rectal malformation
Questions and Answers

Therapeutic management:

  • A blunt instrument is used to incise or puncture the anal membrane.
  • The doctor uses a dilator to accomplish only simple dilatation for anal stenosis, gradually increasing the size of the dilator.
  • A cutback angioplasty is carried out if the anal opening is anterior to the external sphincter and there is little space between the aperture and the external sphincter's center.
  • The abnormal anal opening is shifted to the proper position, and the perineal body is rebuilt, if there is a significant gap between the anal opening and that area of the external anal sphincter.

Nursing management:

  • Identification of cases that went undetected, such as those where meconium was not passed within 48 hours, and referral to the proper center.
  • Assist parents in accepting the condition and adhering to treatment requirements.

Basic preoperative care includes care during diagnostic investigation and collection of report:

  • Preventing feeding.
  • NG tube drainage combined with GI decompression.
  • IV liquids.
  • If the infant or youngster has a colostomy, take care of it.
  • Give the child personal care and general attention.
  • To improve the infant's or child's general health state, give the infant or youngster enough liquids, electrolytes, and nutrition.

Postoperative care:

  • Ensure proper nutritional, electrolyte, and fluid balance.
  • Regular evaluation of the child's vital signs, diet, excretion, electrolytes, etc.
  • Apply precautions to prevent stress on the repaired area, such as not straining to urinate or weeping or becoming restless.
  • Promote breastfeeding after surgery.
  • After surgery, a low-residue diet and a liquid diet should be followed for several days to delay stool passage and prevent stress on the repaired area. Later, laxatives and stool softeners are also administered.
  • Avoid using an enema, which could harm the healed region.
  • To avoid putting strain on the perineum, advise against sitting down.
  • With a dressing, keep the perineum clean and dry. If there is soakage, replace the perineal pad.

The distal anus and rectum, as well as the urinary and genital tracts, are all affected by an extensive range of disorders known as anorectal malformations, which can affect both boys and girls. The functional prognosis of defects can range from the extremely trivial and readily remedied to those that are complex, challenging to handle, frequently accompanied by multiple anomalies, and have a poor functional prognosis.

  • Neither anus nor a thin, translucent membrane were present.
  • There is no meconium stool.
  • Urine with a green tint.
  • Meconium leakage from a different location than an anus.
  • Poorly formed and dimpling.
  • Progressively constricted abdomen.
  • Ribbon-shaped stool.

Types:

  • Imperforate anus:
    • Infant is unable to pass meconium, and an inspection reveals a bulging greenish membrane.
  • Anal stenosis: 
    • Infant struggles to pass ribbon-like stools.
  • Anal agenesis:
    • The existence of only anal dimples due to anus development failure. If there isn't a fistula, intestinal blockage occurs. Fistula typically affects the perineum or vulva in females.
  • Rectal agenesis:
    • It displays a fistula. A fistula may connect to the posterior urethra in a baby boy and the upper vagina in a baby girl.
  • Rectovaginal fistula:
    • Stool travels through a vagina as the rectum and vagina communicate.
  • Rectoperineal fistula:
    • In the perineum, it appears as a little opening..

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