Prenatal diagnosis of Binder's syndrome: report of two cases.

OBJECTIVE

Binder syndrome is a rare congenital malformation presenting an abnormal maxilla's development associated to other characteristical facial features, like absence or decreased nasal bridge, short nasal columella, convex upper lip with associated dental Angle Class III malocclusion, atrophy of the nasal mucosa, and absence of the frontal sinus. Mental retardation and other clinical signs may also be present. Two cases of Binder syndrome were diagnosed at 22 weeks of gestation during the second trimester ultrasound (2D).

CASES REPORT: The first feature detected, in both cases, was a flattened fetal nose in the mid-sagittal plane. Further controls objectivated absence of the naso-frontal angle and a mild hypertelorism. In both cases the parents were informed of the findings and the impossibility of excluding other associated features diagnosed after birth. Once the differential diagnosis was performed, one of the couples decided to terminate the pregnancy. The findings postmortem confirmed the diagnosis. In the second case, the newborn presented the phenotype previously detected, however, a normal psychomotor development was eventually evidenced.

CONCLUSION

The Binder syndrome is an uncommon clinical entity with a recognizable congenital condition characterized by a retruded midface and an extremely flat nose. The exact birth prevalence remains unknown. It is important to understand that Binder's syndrome has a variable prognosis, depending on the other associated features it presents. When diagnosed, an accurate differential diagnosis has to be performed.