[Delayed puberty with extreme uterine hypotrophy: do not conclude too early to the absence of the uterus].
ABSTRACT To emphasize the difficulties to distinguish between uterine agenesis and extreme uterine hypotrophy in the context of primary amenorrhoea with delayed puberty.
Among adolescents who consulted with our center because of primary amenorrhoea, from 1997 to 2005, three patients were referred for a suspicion of Mayer-Rokitansky-Kuster-Hauser Syndrome, after ultrasonography had failed to visualize the uterus. The 3 patients underwent endocrine and genetic evaluations. Transabdominal ultrasonography and MRI performed pelvic examination. Patients were placed under estrogen treatment.
Endocrine evaluation indicated primary ovarian failure for patient 1, and hypogonadotrophic hypogonadism for patients 2 and 3. Karyotype was 46,XX in all patients. Initial pelvic ultrasonography revealed the absence of uterus. MRI allowed visualizing prepubertal uterus for patient 1, a hypotrophic uterus for patient 3 and concluded to uterine agenesis for patient 2. In all cases estradiol substitutive therapy induced uterine growth and confirmed retrospectively the diagnosis of extreme uterine hypotrophy.
Pelvic ultrasonography can be misleading in the evaluation of primary amenorrhoea. No visualization of uterus on ultrasonography can occur in the context of delayed puberty and should not induce a premature diagnosis of Mayer-Rokitansky-Kuster-Hauser syndrome. Indeed, such a diagnosis has therapeutic, reproductive and psychological consequences.
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ABSTRACT: Congenital absence of vagina is known from ancient times of Greek. According to the literature data, incidence is 1/4 000 to 1/20 000. Treatment of this anomaly includes non-operative and operative procedures. McIndoe procedure uses split skin graft by Thiersch. The aim of this study was to establish anatomic and histological characteristics of vagina reconstructed by McIndoe method in Mayer Küster-Rockitansky Hauser (MKRH) syndrome and compare them with normal vagina. The study included 21 patients of 18 and more years with congenital anomaly known as aplasio vaginae within the Mayer Küster-Rockitansky Hauser syndrome. The patients were operated on by the plastic surgeon using the McIndoe method. The study was a retrospective review of the data from the history of the disease, objective and gynecological examination and cytological analysis of native preparations of vaginal stain (Papanicolau). Comparatively, 21 females of 18 and more years with normal vaginas were also studed. All the subjects were divided into the groups R (reconstructed) and C (control) and the subgroups according to age up to 30 years (1 R, 1C), from 30 to 50 (2R, 2C), and over 50 (3R, 3C). Statistical data processing was performed by using the Student's t-test and Mann-Writney U-test. A value of p < 0.05 was considered statistically significant. The results show that there are differences in the depth and the wideness of reconstructed vagina, but the obtained values are still in the range of normal ones. Cytological differences between a reconstructed and the normal vagina were found. A reconstructed vagina is smaller than the normal one regarding depth and width, but within the range of normal values. A split skin graft used in the reconstruction, keeps its own cytological, i.e. histological and, so, biological characteristics.Vojnosanitetski pregled. Military-medical and pharmaceutical review 02/2009; 66(2):107-12. · 0.27 Impact Factor
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ABSTRACT: Uterine agenesis is one of the differential diagnoses in adolescent girls with delayed menstruation. It may also be suspected earlier in childhood during investigations for other genitourinary conditions. However, accurate confirmation that the uterus is absent can be extremely difficult before puberty because of its small size. We describe ten girls referred to a specialist centre with a presumed diagnosis of an absent uterus which was later found to be incorrect. We conclude that imaging should be undertaken by clinicians with experience in management of this age group and in some girls it may be necessary to delay final diagnosis until after puberty.BJOG An International Journal of Obstetrics & Gynaecology 01/2010; 117(2):212-5. · 3.76 Impact Factor