Agenesia della vagina
Table of Contents
Che cos’è l’agenesia della vagina?
L’agenesia della vagina è una malformazione poco frequente che può presentarsi raramente isolata, con organi interni normoconformati, ma che di solito rientra nel quadro di sindromi malformative urogenitali più complesse. E’ caratterizzata dall’assenza della vagina o dalla presenza di una fossetta vaginale di lunghezza inferiore a 5 cm. La forma più tipica e frequente è l’ agenesia vaginale della sindrome di Mayer-Rokitansky-Küster-Hauser che presenta un’incidenza di un caso ogni 4000-5000 nate femmine.
Eziopatogenesi
Per comprendere la patogenesi di questa malformazione è necessario rifarsi all’embriogenesi del canale vaginale; peraltro questa a tutt’oggi non è ancora perfettamente conosciuta.
Quali sono le manifestazioni cliniche dell’agenesia della vagina?
La sindrome di Mayer Rokitansky Kuster Hauser è caratterizzata da:
- Agenesia vaginale, uterina e tubarica, mentre le ovaie sono in genere normalmente presenti;
- In un terzo delle pazienti si riscontrano inoltre anomalie renali (agenesia, ectopia ecc)
- Ureterali (duplicazioni, stenosi ecc..)
- Meno frequenti le malformazioni scheletriche come spina bifida, deformità costale e degli arti.
Agenesia vaginale: diagnosi
La diagnosi di agenesia vaginale, se non è fatta ispettivamente in età infantile, viene posta in età puberale per la mancata comparsa del menarca. È importante distinguere l’agenesia vaginale della sindrome di Mayer Rokitansky Kuster Hauser dall’ agenesia isolata, dall’ agenesia parziale e dall’imenene imperforato, in quanto l’amenorrea in questi casi è accompagnata da un normale svolgimento del ciclo mestruale a livello uterino. La raccolta di sangue mestruale nella vagina, nell’utero e nelle tube, rispettivamente ematocolpo, ematometrio ed ematosalpinge, determina di solito, imponente sintomatologia dolorosa.
Elementi di diagnosi oltre dati soggettivi della crisi dolorosa, sono forniti dalla palpazione per via rettale della vagina e dell’utero e dall’ecografia. La diagnosi di certezza può essere fatta con TAC, risonanza o esplorazione diretta degli organi pelvici per via laparoscopica o laparotomica. È necessario inoltre procedere ad una urografia per diagnosticare le possibili malformazioni associate nefro-ureterali.
Agenesia vaginale: terapia
Il trattamento dell’ agenesia vaginale è strettamente chirurgico e viene effettuato allo scopo di creare un canale vaginale sufficiente all’espletamento di normali rapporti sessuali. La neovagina viene creata in età post-puberale e a sviluppo corporeo ultimato, con l’apertura chirurgica di una cavità a partire dal vestibolo, nel contesto del tessuto cellulo-adiposo del piccolo bacino. Tale cavità viene quindi rivestita da epitelio per mezzo di lembi o innesti di cute.
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