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Recovering Breathing and Feeding of a Newborn with Pierre Robin Sequence
Francisnele Maria de Aquino Fraporti Tomáz1, Alvaro Henrique Borges1, Alexandre Meireles Borba1
1Department of Dentistry, University of Cuiabá, Cuiabá, MT, Brazil.
Insights
Pierre Robin sequence (PRS) treatment using mandibular distraction osteogenesis (MDO) significantly improved a child's airway and feeding. This PRS intervention led to better respiratory function and quality of life.
Area of Science:
- Craniofacial Surgery
- Pediatric Otolaryngology
- Congenital Disorders
Background:
- Pierre Robin sequence (PRS) presents with micrognathia, glossoptosis, and airway obstruction, often with cleft palate.
- Early diagnosis and intervention are crucial for managing PRS complications.
Observation:
- A neonate diagnosed with PRS experienced airway instability requiring intubation, tracheostomy, and glossopexy.
- Mandibular distraction osteogenesis (MDO) was performed due to persistent respiratory and feeding difficulties.
Findings:
- Following MDO, the patient exhibited improved maxillofacial development by age 6, with a patent airway and no neurological deficits.
- The MDO procedure facilitated respiratory and feeding improvements, enabling decannulation of the tracheostomy and timely palatoplasty.
Implications:
- Mandibular distraction osteogenesis offers a viable treatment option for severe Pierre Robin sequence, enhancing patient quality of life.
- While MDO improved outcomes, the long-term need for orthognathic surgery remains uncertain.
Abstract:
Pierre Robin sequence (PRS) is characterized by the triad micrognathia, glossoptosis, and airway obstruction, commonly associated with cleft palate. This study reports the case of a patient with characteristics consistent with the diagnosis of PRS in the 1st week of life. To stabilize the airway, oro-tracheal intubation was performed without success, followed by tracheostomy and glossopexy and after a small improvement in symptoms, it was decided to perform the mandibular distraction osteogenesis (MDO). At the age of 6, her maxillofacial development was within expectations, with patent airway, the presence of deciduous teeth, and without neurological impairment. The MDO described in this case allowed patient's respiratory and feeding improvement, thus avoiding episodes of cyanosis, allowing timely removal of the tracheostomy and glossopexy, and execution of palatoplasty at the right time. While it is uncertain whether MDO will replace the possible need for future orthognathic surgery, treatment greatly improved the quality of life of the patient.
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