Robin Sequence:Clinical implications and functional outcomes following (non-) surgical management

Robin Sequence (RS) is a congenital craniofacial abnormality characterized by mandibular hypoplasia, glossoptosis, and varying degrees of upper airway obstruction. The interaction between the various etiological causes, the great variability in clinical expression, and the lack of a uniform definition continuously pose challenges to clinicians. The aim of this thesis is to gain another step towards a better understanding of this complex and challenging condition by focusing on the clinical characteristics and functional outcomes regarding airway, feeding, swallowing, and growth in patients with RS. In this way, we attempt to add valuable information to the current knowledge of RS and therefore contribute to an improved quality of care for these patients

Non-Surgical Respiratory Management in Relation to Feeding and Growth in Patients with Robin Sequence; a Prospective Longitudinal Study

Objective: To reflect upon our non-surgical respiratory management by evaluating clinical outcomes regarding airway, feeding, and growth during the first year of life in patients with Robin Sequence. Design: Prospective study. Setting: Sophia Children's Hospital, Rotterdam, the Netherlands. Patients/ Participants: 36 patients with Robin Sequence who were treated between 2011 and 2021. Interventions: Positional therapy and respiratory support. Main Outcome Measure(s): Data on respiratory outcomes included polysomnography characteristics and capillary blood gas values. Feeding outcomes were based on the requirement of additional tube feeding. Outcomes on growth were expressed as standard-deviation-scores (SDS) for weight-for-age (WFA) and height-for-age (HFA). Results: Twenty patients were treated with positional therapy (PT), whilst the other 16 patients required respiratory support. Twenty-two patients presented with non-isolated Robin Sequence (RS). During the first year of life, obstructive apnea hypopnea index decreased, oxygen levels enhanced, and capillary blood gas values improved. Eighty-six percent (31/36) experienced feeding difficulties, which completely resolved in 71% (22/31) during their first year of life. From start treatment, to stop treatment, to the age of 1 year, the SDS WFA worsened from −0.40 to −0.33 to −1.03, respectively. Conclusions: Non-surgical respiratory treatment resulted in an improvement of respiratory outcomes to near normal during the first year of life in patients with RS. These patients often experience feeding difficulties and endure impaired weight gain up to 1 year of age, despite near normalization of breathing. The high prevalence of feeding difficulties and impaired weight for age indicate the urgency for early recognition and adequate treatment to support optimal growth.</p

Robin Sequence: Clinical implications and functional outcomes following (non-) surgical management

Robin Sequence (RS) is a congenital craniofacial abnormality characterized by mandibular hypoplasia, glossoptosis, and varying degrees of upper airway obstruction. The interaction between the various etiological causes, the great variability in clinical expression, and the lack of a uniform definition continuously pose challenges to clinicians. The aim of this thesis is to gain another step towards a better understanding of this complex and challenging condition by focusing on the clinical characteristics and functional outcomes regarding airway, feeding, swallowing, and growth in patients with RS. In this way, we attempt to add valuable information to the current knowledge of RS and therefore contribute to an improved quality of care for these patients