To the 13 years old, the model Kesia Nascimento, who is now 19, she already had a more developed body than girls her age. But, despite that, she still hadn’t had her first period. “My mom had it a little later and I thought it was normal. We decided to wait a little longer, “says the young woman.
With 15 she still did not have her period. He went to the doctor’s office and nothing was out of the ordinary, he had not shown any other symptoms.
At 15 the gynecologist requested to do some tests in which no no change was noted. But, due to the pandemic, it was not possible to take her to an evaluation with a professional.
A year later, when I was 17 years old, Nascimento underwent blood counts, transvaginal ultrasounds and other gynecological tests. “The doctor made a face at that moment, because the images showed that I did not have a uterus or vaginal canal“, she remembers.
At that moment, she says, she joked with the professional and told her that she wanted to be a mother.
The doctor prescribed a new examination for Nascimento and told him that he could have a kind of rudimentary uterus. After taking an X-ray, the medical report suggested that he suffered from a rare condition.
Discovery of the syndrome
After the exams, the consultation with the gynecologist changed Nascimento’s life forever.
The doctor explained that the young woman suffered from Rokitansky syndrome, which causes women to be born without a uterus and with a short vagina.
“When she told me that , I was shocked. You never expect something like this. I was listening with my mom, and when the doctor stopped talking, I went to the bathroom to cry. I wiped my tears and came back”, she recalls.
Upon returning home, Nascimento did not find many answers about her condition and felt even more frustrated. “It was all very scientific. There was nothing that had an accessible language, for teenagers”, she recalls.
She says that she had anxiety during that period and, although she did not dream of motherhood, she did see the possibility of being a mother as stagnant.
What is Rokitansky syndrome?
It is a congenital malformation that causes the absence of the uterus or an incomplete development of a portion of the vagina.
This condition arises during embryogenesis, that is, shortly after the formation of the embryo, in the sixth week of gestation.
“The syndrome is like an anatomical alteration. It modifies the shape of the organ, but the woman has ovaries and her sexual characteristics develop. Girls usually go to the doctor because they don’t menstruate“, explains Natália Piovani, gynecologist and professor at the Universidade Positivo, in Curitiba (Brazil).
Some imaging tests help diagnose the syndrome. (Photo: GETTY IMAGES)The absence of menstruation occurs because the endometrium, the tissue that covers the uterus, does not come off precisely due to the absence of the organ.
Generally, women have menarche, their first menstruation, between 9 and the years.
The syndrome can be divided into three types. The typical syndrome, type 1, is the most common and can affect approximately 70 % of patients. In it, there is a reduced change in the reproductive system.
Type 2, known as the atypical and less prevalent form of the syndrome, is associated with ovarian disease and congenital kidney, bone and ear abnormalities . Type 3, more severe and rare, occurs with multiple malformations.
Although it is not so widespread, doctors do not consider Rokitansky syndrome a rare disease, since it affects one in five thousand women.
The condition is usually discovered more frequently due to the absence of menstruation or, if the adolescent has already started her sexual life and has not yet had menarche, if she feels a lot of pain and discomfort when having sex.
As the external genitalia are normal, it is more difficult to diagnose the syndrome. Doctors usually notice something “abnormal” during the consultation and order gynecological tests.
“We can request a pelvic exam, an ultrasound and also a genetic test to verify if the patient has XX chromosomes“, explains Claudia Takano, coordinator of the genital malformations clinic at the Federal University of São Paulo (Unifesp), the only one that treats the syndrome through the Unified Health System, public and free in Brazil.
Is there treatment?
It is possible to treat Rokitansky syndrome with therapies and exercises recommended by doctors. The most traditional is to use dilators, which stretch the vagina to the ideal size.
“The patient has two thirds of the vagina. Because of this, she often has difficulty in sexual relations. The treatment consists of starting with small dilators and then larger ones”, says Priscila Medina, a gynecologist with a residency in gynecology and obstetrics at Hospital Beneficência Portuguesa.
Follow-up is multidisciplinary, so consultations are necessary with gynaecologists, physiotherapists and, mainly, psychologists. With this last professional, issues related to female sexuality and motherhood are addressed.
As a last resort, when treatment with dilators does not work, surgery is indicated. There are several techniques, but the most common is the creation of a new vaginal canal.
The patient is hospitalized for four to five days and cannot have sexual intercourse for up to four months.
The therapeutic line also includes support groups, conversations and meetings with other women who have the syndrome.
Lack of empathy and medical reception
Like Nascimento, the student Débora Moraes (19 years) was diagnosed with Rokitansky syndrome. As she had not yet menstruated at 16 years old, she went to the doctors to find out if she was sick.
Initially, her gynecologist said she had a punctured hymen, but He did not give many details of what the young woman could actually have. With the tests in hand, she went to another health professional and discovered that she did not have a uterus, but neither did she receive much more information there, nor did they tell her about the syndrome.
“I didn’t understand what was wrong . The doctors didn’t know how to explain it correctly“, she says.
Moraes says that the whole process was very frustrating and although she needed the doctors to support her and guide her better about the condition, that didn’t happen.
“Once I left the clinic and I cried a lot. The first doctor had no empathy and even advised me not to tell anyone“, says the young woman who came to think that her case was hopeless.
Moraes’s situation worsened and he It took a while to find specialists who would advise her on possible treatments at such a delicate moment.
Motherhood is possible
When receiving the diagnosis, one of the first thoughts and frustrations that arise is the fact of not being able to get pregnant in a traditional way.
Nascimento says that not imagining herself as a mother was very painful. And cries out a pain that happens to almost everyone: “We feel the pain of a child who was not born,” says the model.
Moraes really wanted to be a mother, but after diagnosis, sleep was interrupted. “It is the dream of most of us. There are women who don’t accept it”.
After a lot of research, both Nascimento and Moraes looked for the Roki Institute, a reference center for this syndrome, and began a psychological follow-up to evaluate possibilities in relation to the maternity.
They knew that, although it is difficult, there are alternatives.
It was explained to them that it is possible to be a mother through a surrogate uterus or the adoption. “A third alternative, which is still being studied, is a uterus transplant,” Takano says.
Moraes says that she is currently doing well and coping better with the matter. Keisa, who saw “the syndrome as a problem that left me infertile and unprepared for sexual life” now also sees it differently.
A center to help to girls and women
This disease is not widespread in Brazil and, as happened to Moraes, it is not easy to find a doctor who knows how to treat the condition .
Due to lack of information and late diagnosis, many adolescents and women suffer from the effects of Rokitansky syndrome over the years.
For this reason, Dr. Claudia Melotti decided to create the Roki Institute, which is one of the main references in receiving patients with the syndrome. She also suffered from the problem and discovered the absence of the uterus at 13 years.
However, she only had the full diagnosis six years later. “I found out when I had 13 years. When trying to have sex with my boyfriend, I had a lot of pain and difficulty in penetration”, she says.
As it is still a subject with little information among the medical profession itself, points out that many women spend their entire lives without receiving any kind of guidance and even receive incorrect treatment. According to her, it is also very difficult to care for and offer psychological help to women.
And, she says, both surgery and dilation treatment are usually successful.
The place offers, free of charge, follow-up with doctors, physiotherapists, psychologists and a whole support network for those who wish to seek help. “In addition to promoting continuing medical education, we also provide dilators,” says Melotti.
In the case of Moraes, she had remote support and they helped her with pelvic dilation . Thanks to this, she had changes and improvements. “They welcomed me and it helped me a lot, because the syndrome affected my mental health a lot. Now I’m happier“, he highlights.
Nascimento also affirms that, after meeting women who were married and living with the syndrome, he had more hope for the future.
“I felt represented after joining the support group. Someone understands what I went through“, he says.
When receiving the diagnosis, one of the first thoughts and frustrations that arise is the fact of not being able to get pregnant in a traditional way.
Nascimento says that not imagining herself as a mother was very painful. And cries out a pain that happens to almost everyone: “We feel the pain of a child who was not born,” says the model.
Moraes really wanted to be a mother, but after diagnosis, sleep was interrupted. “It is the dream of most of us. There are women who don’t accept it”.
After a lot of research, both Nascimento and Moraes looked for the Roki Institute, a reference center for this syndrome, and began a psychological follow-up to evaluate possibilities in relation to the maternity.
They knew that, although it is difficult, there are alternatives.
It was explained to them that it is possible to be a mother through a surrogate uterus or the adoption. “A third alternative, which is still being studied, is a uterus transplant,” Takano says.
Moraes says that she is currently doing well and coping better with the matter. Keisa, who saw “the syndrome as a problem that left me infertile and unprepared for sexual life” now also sees it differently.
A center to help to girls and women
This disease is not widespread in Brazil and, as happened to Moraes, it is not easy to find a doctor who knows how to treat the condition .
Due to lack of information and late diagnosis, many adolescents and women suffer from the effects of Rokitansky syndrome over the years.
For this reason, Dr. Claudia Melotti decided to create the Roki Institute, which is one of the main references in receiving patients with the syndrome. She also suffered from the problem and discovered the absence of the uterus at 13 years.
However, she only had the full diagnosis six years later. “I found out when I had 13 years. When trying to have sex with my boyfriend, I had a lot of pain and difficulty in penetration”, she says.
As it is still a subject with little information among the medical profession itself, points out that many women spend their entire lives without receiving any kind of guidance and even receive incorrect treatment. According to her, it is also very difficult to care for and offer psychological help to women.
And, she says, both surgery and dilation treatment are usually successful.
The place offers, free of charge, follow-up with doctors, physiotherapists, psychologists and a whole support network for those who wish to seek help. “In addition to promoting continuing medical education, we also provide dilators,” says Melotti.
In the case of Moraes, she had remote support and they helped her with pelvic dilation . Thanks to this, she had changes and improvements. “They welcomed me and it helped me a lot, because the syndrome affected my mental health a lot. Now I’m happier“, he highlights.
Nascimento also affirms that, after meeting women who were married and living with the syndrome, he had more hope for the future.
“I felt represented after joining the support group. Someone understands what I went through“, he says.
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