Occipital encephalocele associated with mediastinal enteric duplication cyst

Enteric duplication is a rare developmental anomaly. This anomaly can be seen anywhere along the gastrointestinal tract, and less than 2% of the cases extend to the mediastinum. Developmental neuroectodermal and cutaneous ectodermal abnormalities, especially vertebral midline fusion abnormalities, are frequently associated with enteric duplications. We report a case of a newborn with occipital encephalocele, also having a mediastinal enteric duplication cyst. The clinical course of this case is described in detail to emphasize the occurrence of life-threatening complications of these associated developmental abnormalities during the treatment of the primary pathology in the neurosurgery practice. In the literature, we failed to demonstrate any co-existence of these two developmental abnormalities and review the embryogenesis of the association of these congenital malformations presented here.