Robin sequence Investigations into its epidemiology, nomenclature, and treatment

Robin sequence (RS) is defined as the triad of micro- or retrognathia, glossoptosis and obstructive respiratory distress in the newborn with or without a cleft palate. RS can occur both in isolation or within a spectrum of further congenital anomalies, including syndromes. Given the history of this condition’s heterogeneity in nomenclature and diagnosis, this thesis described the ongoing debate in the specialist literature and took stock of the current situation. In its epidemiological section, this thesis was the first to derive an estimate for the birth prevalence of RS in the Netherlands of 1 in 5,600 live births, which was moderately higher than in other European countries. Furthermore, this thesis demonstrated an increased infant mortality rate for patients with RS of 3,62% relative to 2.45% in patients with isolated cleft palate and 0.45% for the general population, mostly caused by concomitant congenital heart defects. Moreover, we found that 47.8% of RS patients suffered from a partial agenesis of the permanent dentition compared with 29.8% of patients with isolated cleft palate and rates of 3.2-7.6% in the general population. Regarding the treatment of RS, this dissertation detailed the experiences of single cleft palate and presents a treatment algorithm. In addition, a mixed model analysis showed that, for RS patients that underwent surgery to safeguard the airway, both Tongue Lip Adhesion and Mandibular Distraction Osteogenesis restored postoperative weight gain, but did not establish a superiority of either technique in this respect.