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A Retrospective Longitudinal Treatment Review of Multidisciplinary Interventions in Nonsyndromic Robin Sequence With
Pinelopi K Palaska1,2, Gregory S Antonarakis2,3, Sunjay Suri2,4
1Private Practice, Dubai, UAE.
Insights
This study reviews the lifelong treatment of children with nonsyndromic Robin sequence (RS), highlighting multidisciplinary care needs from infancy through adulthood. Comprehensive management is essential for addressing airway, feeding, and developmental challenges in RS patients.
Area of Science:
- Craniofacial Surgery
- Pediatric Otolaryngology
- Developmental Pediatrics
Background:
- Nonsyndromic Robin sequence (RS) presents complex challenges requiring long-term, multidisciplinary management.
- Understanding the comprehensive institutional treatment experience is crucial for optimizing patient outcomes.
Purpose of the Study:
- To document and analyze the longitudinal institutional treatment experience of children with nonsyndromic RS from infancy to early adulthood.
- To provide insights into the management of airway, nutrition, audiology, and surgical interventions throughout development.
Main Methods:
- Retrospective longitudinal review of 117 children with nonsyndromic RS and cleft palate.
- Data collection encompassed airway, nutritional, audiological, orthodontic, and surgical interventions across developmental stages.
- Comparative analysis included literature data from international centers.
Main Results:
- Infancy airway management primarily involved prone positioning (92%); feeding tubes were used in 44%.
- Later interventions included pharyngeal flap surgery (22%), tympanostomy tubes (62%), and 18% diagnosed with obstructive sleep apnea.
- Orthodontic treatment was common for dental issues; orthognathic surgery was infrequent (<18%).
Conclusions:
- The institutional treatment of nonsyndromic RS necessitates a multidisciplinary approach tailored to the child's developmental stage.
- Longitudinal care is vital for addressing the multifaceted needs of individuals with RS throughout their lives.
Objective:
To document and analyze the overall longitudinal institutional treatment experience of children with nonsyndromic Robin sequence (RS) from infancy to early adulthood.
Design:
Retrospective longitudinal treatment review.
Setting:
A tertiary-care, referral, teaching hospital.
Patients:
Children with nonsyndromic RS and cleft palate (N = 117) born between December, 1985, and January, 2012.
Interventions:
Data regarding airway management, nutritional management, audiological interventions, orthodontic treatment, and surgical interventions were documented and analyzed in different growth/developmental stages. Comparative data from other international centers were collected from the literature.
Results:
Airway management during infancy involved prone positioning (92%), nasopharyngeal airway (6%), tracheostomy (2%), and mandibular distraction osteogenesis (1%). Feeding with nasogastric, gastrostomy, and/or gastrojejunostomy tubes was used in 44%, Haberman feeders in 53%, and Mead Johnson feeders in 3%. Gastroesophageal reflux disease was documented in 6% of the sample. During childhood and early adolescent years, pharyngeal flap surgery was carried out in 22% of the children, while 11% had secondary palatal surgery. Audiological management included the use of tympanostomy tubes in 62%, with several children needing multiple tube replacements. At least 18% were diagnosed with obstructive sleep apnea. Adenoidectomy or adenotonsillectomy was undertaken in 4%. Analysis of data pertaining to middle childhood and adolescent years showed that orthodontic treatment was conducted for most children for crowding, tooth agenesis, and skeletal and/or dental dysplasia. Orthognathic surgery frequency (<18%) was low.
Conclusions:
Institutional treatment experience of children with nonsyndromic RS involves multidisciplinary care at different ages and stages of their development.
