Accuracy of perinatal diagnosis of 45,X/46,XY mosaicism and electronic consultation of affected parents

J Pediatr Urol. 2009 Aug;5(4):274-8. doi: 10.1016/j.jpurol.2008.12.002. Epub 2009 Mar 28.

Abstract

Objective: To assess parental satisfaction with e-mail consultations and fetal outcomes after consultation for offspring with 45,X/46,XY mosaicism.

Patients and methods: A chart review was performed of prenatal and postnatal e-mail consultations concerning 45,X/46,XY mosaicism at our institution. Prenatally, mosaicism was detected by amniocentesis; postnatally by phenotype and subsequent genotyping. Parents e-mailed us for a second opinion after internet searches. Follow-up parental satisfaction was evaluated by e-mail.

Results: E-mail consultation occurred in six prenatal and eight postnatal cases. One of the eight postnatal cases had a prenatal diagnosis but without our second opinion. In 7/8 postnatal cases, abnormal neonatal phenotype was evaluated by genotyping. Termination of pregnancy was a consideration in all six prenatal consultations, although not discussed by us. One fetus was stillborn. Postnatal karyotype was mosaic in 11/13 surviving neonates. Phenotypes in surviving fetuses were normal male (2/13), hypospadias and an undescended testis or streak gonad (9/13), female with mild clitoral hypertrophy (1/13), and normal female (1/13). The stillborn fetus was noted only to have a penis.

Conclusion: All parents reported in follow up that e-mail consultations provided them with enhanced clinical information, allowing them to make better informed clinical decisions.

MeSH terms

  • Abnormalities, Multiple / diagnosis
  • Abnormalities, Multiple / genetics
  • Adult
  • Amniocentesis
  • Electronic Mail*
  • Female
  • Follow-Up Studies
  • Genetic Counseling / standards*
  • Genitalia, Female / abnormalities
  • Genitalia, Male / abnormalities
  • Humans
  • Infant, Newborn
  • Male
  • Medical Records
  • Mosaicism*
  • Parents / psychology
  • Phenotype
  • Pregnancy
  • Remote Consultation / standards*
  • Sex Chromosome Aberrations*
  • Stillbirth