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Streptococcus gallolyticus (SG) is an uncommon cause of neonatal infections. We describe the first case of fulminant lethal neonatal sepsis due to SG reported in literature. Our patient was an extremely low birth weight premature infant that was born to a mother with prolonged rupture of amniotic membranes and chorioamnionitis. We also review the cases of neonatal SG infections reported in literature. Fifty-eight percent neonatal SG infections presented in the first week of life. Importantly, SG meningitis is more commonly reported with early-onset infections compared to group B streptococcal meningitis which is more common with late-onset infections. SG must be included in differential for neonatal sepsis, particularly, in presence of meningitis in first week of life. Most cases are sensitive to penicillin, however, cases of reduced sensitivity to penicillin have also been reported.

We present a neonate with early onset apnea and bradycardia in the absence of primary cardio-respiratory and central nervous system disorders that eventually required chronic ventilator support starting at six hours of life. Molecular testing of paired-like homeobox 2b (PHOX2B) gene mutation confirmed the diagnosis of congenital central hypoventilation syndrome (CCHS). CCHS is a rare genetic disorder characterized by impaired central respiratory control with or without broad spectrum of autonomic nervous system (ANS) dysregulations. Ocular ANS dysregulation is a rare finding in CCHS individuals, and it is usually discovered later in life. However, the ophthalmic evaluation of this neonate on first day of life revealed persistent mild dilated oval pupils with limited light reactivity.