The most common age for the diagnosis of Rokitansky Syndrome is between 13 and 18 years old. The most frequent reports are those where the family seeks medical attention because the girl does not menstruate or has pain and difficulty during sexual intercourse. Some may discover it earlier for other reasons, such as accidents, urinary incontinence, or lower abdominal pain. Pelvic exams are not performed at birth or during childhood, so it is rare for the diagnosis to be made before adolescence. Girls with the syndrome go through puberty like other young adults and have normal sexual characteristics, such as pubic hair development, breast and hip growth appropriate for their age. Therefore, it is rare for a doctor to have any suspicion before adolescence. For diagnosis, the pediatrician or gynecologist can perform an adapted vaginal exam, if your daughter has not had sexual initiation, or may request imaging tests to confirm the diagnosis. Pelvic ultrasound is usually the first test to check if the girl's uterus is present and its size. A very small uterus is called "infantile uterus or uterine remnant". This test can also confirm if the patient has normal ovaries and kidneys. Magnetic Resonance Imaging can assist in visualizing the internal female organs with more detail. Blood tests may be requested to confirm if ovarian hormones and female karyotype (46, XX) are normal.

The cause of the syndrome is still unknown. It is acquired during fetal development, so it is common for many mothers to feel responsible, as with other congenital abnormalities. No maternal behavior or cause is associated with this syndrome. Do not blame yourself, nor worry, you were not the cause of the alteration in your daughter.

If your daughter has been diagnosed with an incomplete or absent vagina, she has the following options: • She can wait and do nothing at the moment until she is ready for treatment; • She can create a vagina. The first option is the use of dilators that expand and stretch her existing vaginal tissue with intermittent pressure. This procedure should be taught by a gynecologist and/or a specialist nurse. Surgery is indicated only in case of failure of clinical treatment.

Our daughter has control over her own body. Deciding to create or not create a vagina should be her decision, after discussions with specialist doctors and parents. If your daughter plans to have vaginal intercourse at some point, creating a vagina will be necessary. If your daughter is considering treatment, she should definitely consider using dilators initially. All surgical options have risks, require general anesthesia, and the use of dilators after the procedure. Therefore, surgery is not a quick or easy solution, nor a way to avoid the need for dilators. Specialized medical groups for Rokitansky Syndrome in Brazil and worldwide recommend vaginal dilators as the first treatment option for creating a vagina without surgery. Surgical options should be discussed if there is no success with dilation.

The technique of progressive dilation, known as the Frank method, was described in 1938. The original dilators were made of glass. Nowadays, dilators are made of plastic and come in different progressive sizes. The most recommended kits have 5 or 6 dilator sizes, ranging from 6 to 15 cm in length. The first dilator indicated, around 6 cm, is similar to a thin vaginal tampon. Your daughter should try to practice dilation daily, with sessions lasting between 20 and 30 minutes. As the vagina expands, the immediately larger size dilator is used until completing the treatment with the larger dilators, which can reach lengths between 14 and 15 cm. The time period to create a vagina will depend on how consistently your daughter uses the dilators. The treatment can vary between 6 and 12 months; there are reports of short-term processes, between 3 and 6 months, when the dilation exercise is performed 2 to 3 times a day. There are also reports of longer processes, over 12 months, in which the girl often interrupts dilations for days or weeks.

Yes, it is very successful. However, there is a large variation in the success rate of dilation, mainly related to the teaching and expertise of healthcare professionals who educate their patients on this method. Make sure your daughter is followed by a gynecology program with experience and a high success rate in training their patients to create a functional vagina.

Usually, your daughter will be asked to return for evaluations with monthly or bi-monthly intervals. Monitoring progress during treatment is very important. Your gynecologist will need to check if she is applying pressure in the right place and at the correct angle, so that the success rate of the treatment increases and the dropout rate decreases.

The best time for your daughter to start treatment is when SHE is ready. The most common age for girls to decide to create a vagina is around 16 or 17 years old. Some young women are encouraged to start treatment when they find a sexual partner. As a mother, father, or guardian, you may want your daughter to solve the problem as quickly as possible; however, remember that treatment should only be started when she is ready.

If your daughter is having difficulty or if you are concerned that she is using the dilator incorrectly, she should be encouraged to talk to her doctor. If she is not satisfied with her progress after using dilators for about 6 months, she and her gynecologist should discuss appropriate ways to proceed with treatment. Perhaps the solution is to wait to use the dilators in the future, or to start discussing surgical options.

There may be one or several reasons why your daughter is avoiding using the dilator. She may feel like she doesn't have time, or be worried about pain, or maybe she is uncomfortable not having privacy. It is good to ask your daughter how the process is going and if she is feeling comfortable with her treatment plan. However, it is not advisable to constantly remind her to use her dilator. It is important for your daughter to maintain regular visits to her gynecologist so that she can be evaluated, difficulties she may be facing can be identified, and guidance and support can be provided regarding her progress. If your daughter continues to avoid using the dilator, it may be that she is simply not ready to create her vagina. Discussing delaying treatment until she is ready can alleviate her anxiety and allow her to have control over the situation. This will allow her to focus on other activities that are more important to her at the moment.

When a woman has not been successful with dilator maneuvers, various surgical techniques are described and indicated in the minority of cases. Vaginoplasty or neovaginoplasty are the names of surgeries to create or enlarge a vagina. We often create fantasies, believing that surgery will easily form a vagina without problems. This is not true. Every surgical procedure has risks and possible complications during surgery and daily in a lifetime. At the appropriate time, the medical team may be able to talk to the patient and family about surgical possibilities and the team's experience with each technique. Surgeries that used parts of the intestine to create the vagina were the first to be performed in the late 19th century. Due to mucus secretion in the area, bleeding, pain, and other side effects, this technique became less utilized. Naturally, technology has evolved greatly in the last two centuries. Davydove's technique surgery uses part of the peritoneum for vaginal construction. However, cases of discomfort and pain from traction are reported. Another technique, initially described by McIndoe, used a skin graft on a rubber mold. The procedure, now called McIndoe-Banister, has evolved in relation to molds and grafts. In the postoperative period, daily use of dilators is necessary for approximately 3 months. Williams' technique uses the skin of the labia majora to form a "vaginal pouch." Women who undergo this technique mention discomfort during sexual activity due to the axis of the new vagina and also the occurrence of hair growth. Vecchietti's surgery fixes threads by laparoscopy, which are attached to a traction system in the abdomen. Time, costs, care with equipment, and a second surgery to remove the device are the complaints of patients during treatment. Make sure your daughter is being accompanied by an experienced medical team, sensitive to the feelings, concerns, and doubts that everyone may have.

The majority of sexual pleasure and orgasm comes from clitoral stimulation. Normally, women do not feel pain during sexual intercourse and develop comfortable and pleasurable movements and postures after treatment. At the appropriate time, talk to your daughter about sexual activity. A good suggestion is to recommend the use of lubricants. Before attempting vaginal intercourse, your daughter should talk to her gynecologist.

If your daughter is born with an incomplete vagina but has a normal-sized uterus, she is likely able to become pregnant and have a baby. If she is born without a uterus or with a small uterus, she cannot have a "natural" pregnancy. Since her ovaries should produce eggs normally, she can have a baby carried by another person, called a surrogate mother. Adoption can also be an option for her.

A family member, friend, or another person can "lend" their womb for 9 months. Beautiful stories of surrogate mothers are narrated and available for those who would like to know them. Your daughter would use her own egg, her partner's sperm, or a donor's, and would be the biological mother. Uterine transplantation is a complex process, with many steps, and is performed in large medical centers. The first step is the in vitro fertilization procedure, in which eggs and sperm are harvested and embryos are created and frozen for later use (up to here, the same process as surrogate mother). Next, it is necessary to find a compatible uterine donor, and then the transplant surgery is performed. It is necessary to wait for a period of months or even a year using immunosuppressive medications so that, afterwards, the embryo is implanted in the transplanted uterus. Immunosuppressive drugs must be taken to avoid rejection of the transplanted uterus. Some complications are reported, but if all goes well, a cesarean section is performed at the time of birth. After the baby is born, there is the option to keep the received uterus for a second pregnancy or to remove it immediately after delivery. Currently, the procedure is performed on an experimental basis and is only indicated for women who have not undergone previous surgical neovaginoplasty. There are already some babies born from transplanted uteruses in the world, but there are also cases of failure with complications during the process. Science is constantly seeking options for fertility. When she is ready to have children, other options should be available.

Adolescents usually sense when their parents are stressed. It's okay to tell your daughter that you're feeling uncomfortable or having difficulty talking. At the same time, say that you want to talk to her and offer support. Most likely, it is uncomfortable for her too. Although both may be feeling shy to talk, she will know that you are there to help her deal with the whole situation.

Your daughter may feel uncomfortable about discussing her diagnosis because it often raises questions about her sexuality. The diagnosis of vaginal alteration can prematurely force a discussion about her as a sexual being, a conversation that can leave daughters and parents in an uncomfortable or embarrassing situation. Trying to have a conversation when she is not willing to talk will be frustrating for both of you, so it is best to wait until your daughter is ready. You can suggest that she read about Rokitansky Syndrome, participate in online support groups with other girls who have the same diagnosis, or even talk to a trusted friend or sibling about her feelings. You should also let her know that you are available to listen if she wants to talk. Remember, it is very important to start communicating by asking your daughter what is the best way to help her! Your daughter should be as involved as possible in planning her care and ultimately in the decision about her treatment.

If your daughter is under 18 years old, your consent for medical treatment will be required. It is essential that all options are discussed in clear language with her, so that she can participate in these important decisions about her body. Ultimately, the medical team will respect her wishes regarding treatment.

Sharing your daughter's diagnosis with others is a personal decision. It is always best to ask for her permission first. Typically, parents are selective about who they share this personal information with, as they find comfort in talking to a close relative or friend. Additionally, participating in online support groups for parents of girls with Rokitansky can be very enriching.

It is up to you to decide if you want to share information and with whom. However, the more information you know about your daughter's diagnosis, the easier it will be to answer specific questions. Being able to share information will make these discussions easier. However, do not hesitate to say "I don't know". It is good for parents to set boundaries when people ask inappropriate questions. Remember, your daughter's privacy should be respected.

It is not uncommon for young women with a diagnosis of Rokitansky to go through temporary changes in their emotions and behaviors, as they adjust to a new understanding of how their body has developed. These concerns should decrease over time, as your daughter will understand her diagnosis, be able to verbalize her concerns, and engage in her medical care. Typical signs that your daughter is upset may include changes in sleep or eating patterns, comments about her body, concerns about treatment and medical appointments, and changes in behavior, such as becoming quiet, withdrawn, or irritable. If you, your daughter, or the medical team have concerns, do not wait too long. You should talk to the gynecologist about referring her to a therapist or social worker. It would be helpful if the chosen professional is familiar with the syndrome. You can provide the Guide for Healthcare Professionals or share information with them prior to your daughter's first meeting. As parents, you may also have difficulty controlling your emotions regarding your daughter's diagnosis. If you are experiencing significant mood changes or your feelings are affecting your behavior at work or home, you should seek professional help. By doing so, you will be showing your daughter that it is okay to seek help.