Anorectal Malformation (ARM)

Overview

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Anorectal malformation (ARM) is a congenital defect in which the anus and rectum do not develop properly. This condition can vary significantly in severity, from minor anomalies to complex forms where the rectum may not connect to the colon at all. ARM is typically diagnosed in infancy and often requires surgical intervention.

This condition can vary significantly in severity, from minor anomalies to complex forms where the rectum may not connect to the colon at all. ARM is typically diagnosed in infancy and often requires surgical intervention.

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Symptoms
  • Absence of Anus: In newborns, the most obvious sign is the absence of a visible anus.
  • Abdominal Distension: Swelling or bloating of the abdomen may occur.
  • Failure to Pass Meconium: Newborns may not pass their first stool within the first 24 hours of life.
  • Fecal Impaction: Difficulty in bowel movements, leading to constipation or obstruction.
  • Signs of Infection: Fever, irritability, or signs of distress in the newborn.
Complications
  • Infection: Risk of infection in the urinary tract or abdominal cavity.
  • Bowel Obstruction: Difficulty in bowel movements can lead to severe complications.
  • Fecal Incontinence: Long-term challenges with bowel control.
  • Psychosocial Issues: Body image and self-esteem concerns as the child grows.
Causes

The exact cause of ARM is not fully understood, but it is believed to result from a combination of genetic and environmental factors during fetal development. It may be associated with other congenital anomalies in some cases.

Prevention

Currently, there are no known prevention methods for ARM, as it occurs during fetal development without specific risk factors that can be controlled.

Risk Factors
  • Family History: A history of congenital anomalies in the family may increase the risk.
  • Genetic Syndromes: Certain genetic syndromes, such as VACTERL association (which includes vertebral, anal, cardiac, tracheal, esophageal, renal, and limb anomalies), are associated with ARM.
  • Maternal Health: Some maternal health factors, including diabetes and certain medications, may contribute to the risk.
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How is it treated?

  • Surgical Intervention: The primary treatment for ARM is surgery to create a functional anus and connect the rectum to the colon. The specific surgical approach depends on the type and severity of the malformation.
  • Post-operative Care: Ongoing monitoring and care to ensure proper bowel function and address any complications.
  • Long-term Management: This may include additional surgeries, dietary management, and support for bowel control as the child grows.
  • Early diagnosis and treatment are crucial for optimizing outcomes for children with anorectal malformation. Regular follow-ups with pediatric gastroenterologists and surgeons are essential for managing long-term effects.
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How is it Diagnosed?

Anorectal Malformation (ARM) refers to a spectrum of congenital defects involving the anus and rectum. Diagnosis typically begins at birth with a physical examination. Newborns with ARM may present with absent or abnormally located anal openings, or signs of bowel obstruction such as abdominal distension, vomiting, or failure to pass meconium within the first 24 hours.

Initial evaluation involves inspection of the perineum to check for the presence, location, and patency of the anal opening. In male infants, a fistula may open into the urethra, while in females, it may be into the vestibule or vagina.

To determine the level and type of malformation, imaging studies are essential. An invertogram (cross-table lateral X-ray) is commonly performed 12 to 24 hours after birth to assess the distance between the rectal pouch and the perineal skin. Ultrasound of the abdomen and pelvis helps detect associated anomalies, particularly of the urinary tract and spine. MRI or distal colostogram may be needed for detailed anatomy before surgical planning.

ARM is often associated with other congenital anomalies, especially those falling under the VACTERL association (Vertebral, Anorectal, Cardiac, Tracheoesophageal, Renal, and Limb anomalies). Therefore, a thorough systemic examination and echocardiography, renal ultrasound, and spinal imaging are standard.

Timely diagnosis enables planning for surgical intervention, which may involve primary repair or staged surgery. Multidisciplinary evaluation is crucial for optimal outcomes.

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