Protrusion of brain stem into oropharynx in newborn: Unusual severe case of Chiari 1 malformation and Clival hypoplasia

Chiari 1 Malformation in children is defined as 6 mm or more caudal extension of cerebellar tonsils below the foramen magnum. It is one of the deformities in the spectrum of craniovertebral junction anomalies. We report an unusual case of newborn girl with hypoplasia of bassiocciput of the clivus and extension of brainstem into the oropharynx, in addition to extension of cerebellar tonsils below the foramen magnum. Right anterior arch and right posterior arch of C1 vertebra are absent. Dens of C2 vertebra is asymmetric. Laryngoscope showed a membrane covering the brainstem in the oropharynx. Endotracheal and orogastric tubes were placed. A ventriculo-peritoneal shunt was placed for hydrocephalus. The patient was discharged home at 4.5 months of age with home Hospice care. To the best of our knowledge, this has not been described in the literature. The case illustrates a rare defect in the clivus and unusual protrusion of brainstem into oropharynx.