Abstract

Pierre Robin sequence is a condition where baby is born with a small lower jaw, is typically described as micrognathia, glossoptosis, and cleft palate. Pierre Robin sequence can be an isolated finding, or can be associated with a genetic syndrome in approximately 50% of cases. 1.1. Objective: Report a Pierre Robin sequence in a neonate at Caracas Universitary Hospital. 1.2. Clinical case: Full-term male neonate shows signs of gastroesophageal reflux during breastfeeding and alteration in the palate, which is why they request an evaluation by the Plastic and Reconstructive surgery service. At the physical examination, adequate mouth opening is evident: a palatal cleft is visualized that involves the hard and soft palate of approximately 25 x 30 mm, which allows visualization of the posterior surface of the pharynx. Absence of choanae with hypoplasic tongue. Suction reflex preserved with decreased pressure. Findings are complete palatal cleft, microglossia and micrognathia. 1.3. Discusion and conclusion: Clinicians mainly focus on the management of the morbidities caused due to PRS. Managing respiratory obstruction is prioritized over addressing feeding dysfunction, however; our patient does not have this problem. Supplemental feeding using a nasogastric/orogastric tube (mild cases) and gastronomy tube (chronic and persistent cases) helps resolving feeding difficulties. Despite the relative low frequency, the Pierre Robin sequence is a complex scenario for any neonate and his family. Requires a multidisciplinary team in order to obtain the best results and outcomes avoiding complications.

Keywords

pierre robin sequence, clef palate, micrognathia, neonate