PIERRE ROBIN SEQUENCE ASSOCIATED WITH ARACHNOID CYST: A RARE NEUROLOGICAL MANIFESTATION

Abstract

Pierre Robin Sequence (PRS) is a rare congenital condition defined by the triad of micrognathia, glossoptosis, and upper airway obstruction, commonly accompanied by cleft palate. While PRS most often occurs as an isolated anomaly, it may also be associated with other systemic malformations, including cardiac, renal, and neurological abnormalities. The coexistence of PRS with intracranial lesions such as arachnoid cysts is exceedingly uncommon and scarcely reported in the literature. We describe a case of a neonate who presented at birth with classical features of PRS, along with significant feeding difficulties and respiratory distress. Neuroimaging with computed tomography revealed a large left temporoparietal arachnoid cyst, highlighting an unusual neurological association. Early recognition of central nervous system involvement in infants with PRS is critical, as such comorbidities can substantially worsen clinical outcomes and increase the risk of morbidity and mortality. This case underscores the importance of maintaining a high index of suspicion for associated neurological anomalies in PRS and advocates for comprehensive evaluation beyond the craniofacial features alone. Prompt diagnosis allows timely intervention and supports optimal management strategies. Furthermore, this report emphasizes the need for a multidisciplinary approach involving neonatology, neurology, radiology, otolaryngology, and pediatric surgery to ensure coordinated care. Awareness of rare associations such as arachnoid cysts can aid clinicians in improving early detection and long-term prognosis in affected infants.