Neurocognitive development in isolated Robin sequence treated with the Tuebingen palatal plate

Abstract

Objectives We aimed to determine the neurocognitive development of cleft palate patients with and without Robin sequence (RS). Materials and methods Children with isolated RS with cleft palate and children with cleft palate only (CPO) were contacted at the age of 5–6 years. All RS children had undergone initial polygraphic sleep study (PG) with a mixed-obstructive apnea index (MOAI) of ≥ 3/h and were consequently treated with the Tuebingen palatal plate. A standardized clinical examination as well as a neuropediatric and neuropsychological examination included the Wechsler Pre-school and Primary Scale of Intelligence (WPPSI-III), Kaufman Assessment Battery for Children (K-ABC), and an assessment of developmental milestones. Results In total, 44 children (22RS, 22CPO) were included. RS children were younger at study (70.5 ± 7.3 and 75.2 ± 7.5 months; P = .035). Both groups achieved the evaluated milestones within the normed time frame. WPPSI-III and K-ABC results showed no group differences. Mean values for Verbal IQ (101.8 ± 11.1 vs. 97.1 ± 15.7), Performance IQ (102.9 ± 12.1 vs. 99.6 ± 14.5), Processing Speed Quotient (98.9 ± 15.6 vs. 94.5 ± 15.7), Full-Scale IQ (103.2 ± 12.1 vs. 98.4 ± 15.3), and Sequential Processing Scale (102.1 ± 13.1 vs. 94.2 ± 17.3) were within the reference range (IQ 85–115) for RS and CPO children, respectively, indicating average performance of both groups. Conclusion No neurocognitive, physical, or mental impairments were detected suggesting that RS children having upper airway obstruction (UAO) treated early and effectively may use their potential for an age-appropriate neurocognitive development. Clinical relevance Tuebingen palatal plate treatment successfully releases UAO. Thus, isolated RS does not necessarily result in developmental delay or an impaired neurocognitive outcome. Trial registration Deutsches Register Klinischer Studien, DRKS00006831, https://www.drks.de/drks_web/

Developmental outcome of children with Robin sequence treated with the current Paris protocol

“…More recently, the German team from Tübingen evaluated the development of 22 children with RS who were 5.8 ± 0.6 years old, all receiving a pre‐epiglottic baton plate. The authors showed with the Wechsler Preschool and Primary Scale of Intelligence, First Edition, and the Kaufman Assessment Battery for Children that the children's development was within the normal range 16 . They recently confirmed their good results with the Wechsler Intelligence Scale for Children, Fifth Edition, in 21 children aged 9.7 years old 22 .…”

“…Tracheostomy remains the ultimate solution for all teams 9–12 . According to the studies that analysed the long‐term developmental outcome, the main point is that children with isolated RS have the potential for development within the normal range or very close to it 13–16 …”

“…[9][10][11][12] According to the studies that analysed the long-term developmental outcome, the main point is that children with isolated RS have the potential for development within the normal range or very close to it. [13][14][15][16] Until 2013 in Paris, tracheotomy was the second-line treatment for children with RS and severe UAO after prone positioning and nasopharyngeal intubation attempts. A longitudinal study conducted from 1999 to 2011 showed the efficiency of this procedure for the development of children with isolated RS.…”