Vaginal dilators may be pressed alongside the vaginal area on a regular basis in order to further open the vaginal canal. Franks procedure is a technique that used a progressive series of vaginal dilators that are inserted into the dimple of the vagina while using pressure. This will widen any space that exists between the bladder and the rectum. Franks procedure can be performed directly by the patient, therefore requiring no surgery or anesthesia. The procedure/technique can take months to complete, with regular compliance necessary. The overall success rate for females who use Franks procedure is 80%. If this procedure does not work, then surgery would be the next method of treatment. Another alternative form of treatment would be surgery, or the creation of a new vagina. Prognosis
The prognosis for vaginal atresia is one that is complicated. There are variations in patients anatomic findings as well as an absence in consistent surgical techniques which makes it difficult to give a prognosis for this condition. Along with other conditions that give rise to an abnormal perineum (i.e. ambiguous genitalia and other various abnormalities that range from cloaca to urogenital sinus), individuals with vaginal atresia often report reconstruction as an outcome of treatment. Due to this, it is difficult to compare outcomes between individuals with vaginal atresia. Rokitansky-Mayer-Küster-Hauser Syndrome
Fertility options for girls and women with Rokitansky-Mayer-Küster-Hauser syndrome has a bit more information.
Sphingolipidoses are a class of lipid storage disorders or degenerative storage disorders caused by deficiency of an enzyme that is required for the catabolism of lipids that contain ceramide, also relating to sphingolipid metabolism. The main members of this group are Niemann–Pick disease, Fabry disease, Krabbe disease, Gaucher disease, Tay–Sachs disease and metachromatic leukodystrophy. They are generally inherited in an autosomal recessive fashion, but notably Fabry disease is X-linked recessive. Taken together, sphingolipidoses have an incidence of approximately 1 in 10,000, but substantially more in certain populations such as Ashkenazi Jews. Enzyme replacement therapy is available to treat mainly Fabry disease and Gaucher disease, and people with these types of sphingolipidoses may live well into adulthood. The other types are generally fatal by age 1 to 5 years for infantile forms, but progression may be mild for juvenile- or adult-onset forms. Accumulated products
Gangliosides: Gangliosidosis
GM1 gangliosidoses
GM2 gangliosidoses
Tay–Sachs disease
Sandhoff disease
GM2-gangliosidosis, AB variant
Glycolipids
Fabrys disease
Krabbe disease
Metachromatic leukodystrophy
Glucocerebrosides
Gauchers disease

Comparison
Metabolic pathways
See also
Lipid storage disorder

References
External links

Sphingolipidoses at the US National Library of Medicine Medical Subject Headings (MeSH)