Biliary lithiasis in early pregnancy and abnormal development of facial and distal limb bones (Binder syndrome): A possible role for vitamin K deficiency
✍ Scribed by Jessica Jaillet; Elisabeth Robert-Gnansia; Marianne Till; Christine Vinciguerra; Patrick Edery
- Publisher
- John Wiley and Sons
- Year
- 2005
- Tongue
- English
- Weight
- 332 KB
- Volume
- 73
- Category
- Article
- ISSN
- 1542-0752
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✦ Synopsis
BACKGROUND: Binder syndrome is a maxillonasal dysostosis characterized by midface and nasal hypoplasia, sometimes associated with short terminal phalanges of fingers and toes and transient radiological features of chondrodysplasia punctata. Warfarin-or phenytoin-induced vitamin K deficiency during early pregnancy is a well-established etiology for this syndrome, which occurs nevertheless sporadically in most cases. CASE(S): We describe here the first case, to our knowledge, of Binder syndrome in a child whose mother presented with biliary lithiasis in early pregnancy. The mother proved to have a decrease in clotting factors II, VII, and X, and in prothrombin time, at 11 weeks of gestation, which was highly suggestive of vitamin K deficiency. CONCLUSIONS: The biliary lithiasis-induced vitamin K deficiency in early pregnancy is likely to have resulted in Binder syndrome. This observation should prompt physicians to carefully check for vitamin K deficiency in pregnant women presenting with biliary lithiasis, in order to prevent Binder syndrome in the fetus by providing intravenous vitamin K supplementation as soon as possible. Finally, reassuring genetic counseling regarding the genetic risk for future pregnancies is to be provided to the parents.