The Turkish Journal of Pediatrics 2008 , Vol 50 , Num 4
Prenatally diagnosed lethal type Larsen-like syndrome associated with bifid tongue
1 Divisions of Pediatric Pathology, Hacettepe University Faculty of Medicine, Ankara, Turkey
2 Divisions of Clinical Genetics, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey
3 Department of Obstetrics and Gynecology, Hacettepe University Faculty of Medicine, Ankara, Turkey
Orhan D, Balcı S, Deren Ö, Ütine EG, Başaran A, Kale G. Prenatally diagnosed lethal type Larsen-like syndrome associated with bifid tongue. Turk J Pediatr 2008; 50: 395-399.

Larsen syndrome is characterized by multiple joint dislocations, associated with a typical facial appearance and frequently other abnormalities. Both dominant and recessive patterns of inheritance have been reported. A lethal form of Larsen syndrome (Larsen-like syndrome) has been described as a combination of the Larsen phenotype and pulmonary hypoplasia. In this report, we present a 24-week-old female fetus with a possible prenatal diagnosis of thanatophoric dysplasia in whom postmortem examination revealed lethal type Larsen-like syndrome associated with bifid tongue, severe micrognathia and non-immune hydrops fetalis. These findings have not been reported previously in the lethal type Larsen syndrome. Keywords : Larsen-like syndrome (lethal type), bifid tongue, multiple dislocations, severe micrognathia, prenatally diagnosed

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