Herlyn-werner-wunderlich syndrome: MRI findings, radiological guide (two cases and literature review), and differential diagnosis

<p>Abstract</p> <p>Background</p> <p>Herlyn-Werner-Wunderlich (HWW) syndrome is a very rare congenital anomaly of the urogenital tract involving Müllerian ducts and Wolffian structures, and it is characterized by the triad of didelphys uterus, obstructed hemivagina and ipsilateral renal agenesis. It generally occurs at puberty and exhibits non-specific and variable symptoms with acute or pelvic pain shortly following menarche, causing a delay in the diagnosis. Moreover, the diagnosis is complicated by the infrequency of this syndrome, because Müllerian duct anomalies (MDA) are infrequently encountered in a routine clinical setting.</p> <p>Cases presentation</p> <p>two cases of HWW syndrome in adolescents and a differential diagnosis for one case of a different MDA, and the impact of magnetic resonance (MR) imaging technology to achieve the correct diagnosis.</p> <p>Conclusions</p> <p>MR imaging is a very suitable diagnostic tool in order to perform the correct diagnosis of HWW syndrome.</p

Clinical approach for the classification of congenital uterine malformations

A more objective, accurate and non-invasive estimation of uterine morphology is nowadays feasible based on the use of modern imaging techniques. The validity of the current classification systems in effective categorization of the female genital malformations has been already challenged. A new clinical approach for the classification of uterine anomalies is proposed. Deviation from normal uterine anatomy is the basic characteristic used in analogy to the American Fertility Society classification. The embryological origin of the anomalies is used as a secondary parameter. Uterine anomalies are classified into the following classes: 0, normal uterus; I, dysmorphic uterus; II, septate uterus (absorption defect); III, dysfused uterus (fusion defect); IV, unilateral formed uterus (formation defect); V, aplastic or dysplastic uterus (formation defect); VI, for still unclassified cases. A subdivision of these main classes to further anatomical varieties with clinical significance is also presented. The new proposal has been designed taking into account the experience gained from the use of the currently available classification systems and intending to be as simple as possible, clear enough and accurate as well as open for further development. This proposal could be used as a starting point for a working group of experts in the field

Immature ovarian teratoma in an adolescent: a case report and review of the literature

BACKGROUND: Benign cystic teratomas are relatively common tumors in reproductive age women, but can occur at any age. While the incidence of malignant elements in a teratoma is low (approximately 1-2%), the survival of patients with immature teratoma is poor. Definitive diagnosis is mandatory. CASE: We describe a case of a 13-year-old African American female, gravida 0, presenting with a large pelvic mass, determined to be a benign cystic teratoma by intra-operative frozen section. However, due to the size of the tumor and the preponderance of neural elements we performed a full surgical staging procedure (excluding hysterectomy and complete removal of adnexa). The final pathology report revealed foci of immature neural tissue, with a final diagnosis of an immature cystic teratoma Stage Ia. CONCLUSION: Foci of immature neural elements can be readily missed on frozen section, especially with a large tumor. Full surgical staging at the time of initial laparotomy is justified when encountering an apparently mature cystic teratoma with a preponderance of neural elements on frozen section