Around 60 percent of girls born with ARMs will experience some type of gynecological condition or abnormality. Many of these conditions are not diagnosed until puberty especially if the patient has not had the benefit of coordinated care involving a pediatric gynecologist. It is critical to have gynecologic follow-up throughout infancy, childhood, adolescence and adulthood to minimize the risks of potential problems and diagnose any conditions early on.
Managing Gynecologic Conditions in Neonatal Care
A pediatric gynecologist will be an essential part of the medical team that will oversee care for girls who are diagnosed with ARM at birth. Early assessment of the gynecologic anatomy with a combination of physical exam and/or ultrasound will determine the type of interventions that are needed. The pediatric gynecologist may evaluate for abnormalities in the child’s uterine structures, vagina, or cervix The exam may reveal abnormalities that may have been missed if not performed by an experienced pediatric gynecologist. This is critical to timely intervention and reduces the risk of delayed diagnosis at puberty or in adulthood and which could increase potential for endometriosis, uterine pain, infertility and other psychosocial concerns such as negative self-perception and low self-esteem.
In addition, pediatric gynecologists offer counseling to families to openly discuss their daughter’s future reproductive and sexual health..A pediatric gynecologist’s role ensures that sensitive issues will be addressed and that all concerns can be discussed freely.
Managing Gynecologic Conditions in Puberty
Puberty marks the time when the Mullerian structures (the uterus, fallopian tubes, cervix and vagina) become fully matured. During this time, girls with ARM should have regular monitoring with pelvic ultrasounds and be evaluated for physical signs of pubertal progression and cyclic abdominal pain.
Girls with ARM are at risk for menstrual obstruction, ovarian cyst formation, and/or difficulty with tampon insertion. They may require medications to suppress menstrual cycles or referral to a pelvic floor physical therapist.
At puberty, the pediatric gynecologist also begins to provide age-appropriate counseling and anticipatory guidance for each patient regarding gynecologic anatomy, menstruation, tampon use (if desired), and future reproductive and sexual health. Doctors should provide education and encourage open and frank discussions with parents to address any questions and concerns they may have.
Red Flag Signs in Puberty for Girls with ARM
Puberty typically occurs between the ages of 8 and 13. Girls who have not begun showing signs of puberty by age 13, or have not begun to menstruate by age 15 should be evaluated for possible “red flags” of potential reproductive problems including amenorrhea, cyclical abdominal pain, and/or painful menses.
Red flag signs include:
- A lack of functioning of endometrial tissue or the presence of an underdeveloped uterus
- The presence of any menstrual obstruction or outflow tract obstruction
- The presence of pain in patients who are menstruating (dysmenorrhea). This can be caused by a bicornuate (heart shaped) uterus or other abnormality of the uterus as a result of ARM.)
Menstrual obstructions detected in young girls after the onset of puberty are likely to require hormonal suppression and possible surgical intervention. Regular monitoring and early intervention is key because late diagnosis of menstrual obstruction is known to increase the risk of endometriosis, pelvic inflammatory disease, and pyocolpus (accumulation of pus in the vagina).