Skip to Main Content


Very rare syndrome that results from a twinning abnormality with endocrine dysfunction.


Hypopituitarism Microphthalmia Syndrome.


Very rare syndrome that occurs in monozygotic twins by loss of an X chromosome early in embryogenesis with complete separation of 45,X and 46,XX cell lineages at the time of the twinning event.


After intrauterine growth retardation, twins present with different physical appearance. Clinical features of the syndrome combine short stature with microphthalmos and endocrine dysfunction (hypothyroid, hypoglycemia, and hypothalamo-hypophysial axis anomalies). Micropenis and ectopic testes can be associated. Mosaicism is observed in peripheral blood, but not in skin fibroblast cultures (probably by anastomoses between the placentae of the twins).


No literature available. Careful intraoperative monitoring is needed. Evaluate endocrine function (clinical, laboratory, CT/MRI). Fasting should include a concomitant glucose infusion and frequent blood glucose measurements. Perioperative anesthetic management may need adaptation to endocrine function, particularly concerning adrenal function that could necessitate steroid stress dose and fluid regimen adaptation.

Kaplowitz PB, Bodurtha J, Brown J, et al: Monozygotic twins discordant for Ullrich-Turner syndrome. Am J Med Genet 41:78, 1991.  [PubMed: 1951467]

Want remote access to your institution's subscription?

Sign in to your MyAccess profile while you are actively authenticated on this site via your institution (you will be able to verify this by looking at the top right corner of the screen - if you see your institution's name, you are authenticated). Once logged in to your MyAccess profile, you will be able to access your institution's subscription for 90 days from any location. You must be logged in while authenticated at least once every 90 days to maintain this remote access.


About MyAccess

If your institution subscribes to this resource, and you don't have a MyAccess profile, please contact your library's reference desk for information on how to gain access to this resource from off-campus.

Subscription Options

AccessAnesthesiology Full Site: One-Year Subscription

Connect to the full suite of AccessAnesthesiology content and resources including procedural videos, interactive self-assessment, real-life cases, 20+ textbooks, and more

$995 USD
Buy Now

Pay Per View: Timed Access to all of AccessAnesthesiology

24 Hour Subscription $34.95

Buy Now

48 Hour Subscription $54.95

Buy Now

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.