The prenatal diagnosis of Binder syndrome before 24 weeks of gestation: case report

Abstract

A case of Binder syndrome was diagnosed at 21 weeks of gestation using two-dimensional and three-dimensional ultrasound. The first indication of any abnormality was a flattened fetal nose demonstrated in the mid-sagittal plane. Further ultrasound imaging showed the virtual absence of the naso-frontal angle, giving the impression of a flat forehead and small fetal nose. Suspected mild hypertelorism was also seen using transverse and coronal planes. Differential diagnosis of this condition is discussed.

Maxillo-Nasal Dysplasia (Binder Syndrome): Antenatal Discovery and Implications

“…BS has a characteristic clinical appearance including midface hypoplasia, convex upper lip, broad philtrum, crescentshaped nostrils and a deep fold or fossa between nose and upper lip. The naso-frontal angle is absent and the nose is hypoplastic with flattened alae and nasal tip [3]. The nasal mucosa has been described as atrophic, but the sense of smell is normal.…”

“…MND was first reported by Noyes [3] in 1939, while Binder [4] described a condition called MND, characterized by a short nose with a flat bridge, a short columella, an acute naso-labial angle, perialar flatness, a convex upper lip and a tendency to Angle class II malocclusion. Since this condition was first described in 1962, more than 200 cases have been reported, but mainly by plastic and reconstructive surgeons [5][6][7][8][9][10].…”

The Binder syndrome: Review of the literature and case report

“…The sense of smell is completely normal. Five per cent of affected individuals have been found to have hearing loss and 5% nonspecific congenital heart defects [22]. Maxillo-nasal dysplasia can also be combined with other malformations.…”

“…It is important to examine the prenatal facial features as they may well give an indication of an underlying severe fetal abnormality. However, the finding of a small flattened nose with no other abnormal features, in a fetus with a normal karyotype, is likely to carry a good prognosis with the possibility of satisfactory surgical correction [22].…”

Prenatal sonographic diagnosis of fetal valproate syndrome: a case report

“…VACTERL association (vertebral anomalies, anal atresia, cardiovascular anomalies, tracheoesophageal fistula, esophageal atresia, renal (kidney) and/or radial anomalies, limb defects) is associated with radial ray defects, but it is not usually accompanied by facial anomaly [8]. Although Binder syndrome is characterized by a flat midface and nasal hypoplasia, it is not usually accompanied by radial ray defects [9]. …”