e-ISSN: 2664-1194     print ISSN: 2304-6554
Opitz G/BBB Syndrome
Azerbaijan Journal of Perinatology and Pediatrics

Abstract

Opitz G/BBB syndrome (OS) is a multiple congenital anomalies disorder characterized by
malformations of the midline including hypertelorism, laryngo-tracheo-esophalgeal defects and
hypospadias. There are two clinically indistinguishable genetic subtypes of Opitz G/BBB: X-linked
Opitz G/BBB syndrome (XLOS), and autosomal dominant Opitz G/BBB syndrome (ADOS) (see
these terms).
Due to the complex nature of the syndrome multidisciplinary team of maxillofacial surgeons,
ophthalmologists, pediatricians, urologists, cardiologists, pulmonologist, speech therapist, geneticist
will be involved in the patient,s treatment. Treatment is directed first of all, correction and
restoration surgery.

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