Ovotesticular Disorder of Sexual Development Follow-up

Updated: Jan 12, 2017
  • Author: Molina B Dayal, MD, MPH; Chief Editor: Richard Scott Lucidi, MD, FACOG  more...
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Follow-up

Complications

Traditionally, complications from the surgical treatment of patients with disorders of sexual development were related to stenosis after vaginal reconstruction procedures and urinary tract problems associated with correction of severe hypospadias.

Today, patients are diagnosed and operated on earlier and tend to have fewer complications.

Gender assignment should be made prior to age 18 months, when children develop gender identity, so as to minimize psychosocial trauma.

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Prognosis

Aside from the physical and emotional consequences associated with genital ambiguity, patients with ovotesticular disorder of sexual development usually do not possess other developmental malformations.

These individuals generally are of average intelligence and have a normal life expectancy.

Fertility potential does exist in people with ovotesticular disorder of sexual development who are given a female sexual assignment. Ovulation can occur, and several pregnancies have been reported in this group. To date, all documented offspring have been male. One report describes a 46,XX/46,XY infertile chimeric male who fathered a child with sperm obtained from his testicular tissue through intracytoplasmic sperm injection. [10, 11, 12]

People with ovotesticular disorder of sexual development who are given a male sex assignment rarely reproduce. Spermatogenesis has been reported in only 12% of these cases, and tubular atrophy with hypoplastic testicular tissue is the norm. There are only 3 reported cases of males with OT-DSD fathering children.

Patients should be counseled that infertility is common despite excision of discordant tissue and genital reconstruction.

Many patients with ovotesticular disorder of sexual development are sexually active with a small portion being sexually dissatisfied. Those raised as males may complain of an inability to have and/or maintain erections, while females may complain of vaginal stenosis, recurrent cystitis, and hot flushes. [13]

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Patient Education

Once the diagnosis of genital ambiguity is made, ongoing psychological support for the patient, parents, and other family members is critical. [14]

Psychological counseling is perhaps even more important for patients with a diagnosis delayed until puberty or adult life.

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