Current management options of Pierre-Robin sequence

Czech version

Authors: Vojtěch Novotný ¹; Magdalena Koťová ^2,3; Tereza Doušová ¹; Barbora Obermannová ¹
Authors‘ workplace: Pediatrická klinika, 2. lékařská, fakulta, Univerzita Karlova, a Fakultní nemocnice v Motole, Praha ¹; Stomatologická klinika, 2. lékařská fakulta, Univerzita, Karlova a Fakultní nemocnice, Královské Vinohrady, Praha ²; Rozštěpové centrum Praha, Fakultní nemocnice Královské, Vinohrady, Praha ³
Published in: Čes-slov Pediat 2025; 80 (3): 148-153.
Category:
doi: https://doi.org/10.55095/CSPediatrie2025/019

Overview

Novotný V, Koťová M, Doušová T, Obermannová B. Current management options of Pierre-Robin sequence

Pierre Robin Sequence (PRS) is a congenital condition characterized by micrognathia, glossoptosis, and, in some patients, cleft palate of varying extent. This leads to upper airway obstruction and feeding difficulties. PRS requires a comprehensive approach that combines multidisciplinary care with both surgical and non-invasive methods.

In recent years, the Tübingen Palatal Plate (TPP) has emerged as one of the primary non-invasive treatment options. TPP stabilizes the tongue position, helping to eliminate airway obstruction. Additionally, by relieving and advancing the tongue, it stimulates mandibular growth and significantly improves feeding in newborns, reducing the need for surgical interventions such as tracheostomy.

This method is now available at specialized centers in the Czech Republic, including the Pediatric Department of the 2nd Faculty of Medicine, Charles University and University Hospital Motol, in collaboration with the Dental Department of the 3rd Faculty of Medicine, Charles University and FN KV Prague, and with Prague Cleft Center at FN KV, and the Facial Cleft Treatment Center in Brno.

A multidisciplinary team plays a vital role in the care of PRS patients, comprising pediatricians, ENT specialists, plastic and maxillofacial surgeons, orthodontists, and speech therapists. This team ensures treatment optimization and prevents complications.

This article focuses on the available treatment options for PRS in the Czech Republic, highlighting the benefits of TPP and new technologies, such as 3D printing, which allow for precisely tailored devices to enhance treatment efficacy.

Keywords:

cleft palate – multidisciplinary approach – airway obstruction – Pierre-Robin sequence – Tübingen Palatal Plate – micrognathia – glossoptosis

Sources

1. Tan TY, Kilpatrick N, Farlie PG. Developmental and genetic perspectives on Pierre Robin sequence. Am J Med Genet C Semin Med Genet 2013; 163C(4): 295–305.

2. Cole A, Lynch P, Slator R. A new grading of Pierre Robin sequence. Cleft Palate Craniofac J 2008; 45(6): 603–6.

3. Rathé M, Rayyan M, Schoenaers J, et al. Pierre Robin sequence: Management of respiratory and feeding complications during the first year of life in a tertiary referral centre. Int J Pediatr Otorhinolaryngol. 2015; 79(8): 1206–12.

4. Van den Elzen AP, Semmekrot BA, Bongers EM, et al. Diagnosis and management of the Pierre Robin sequence: Results of a retrospective clinical study and review of the literature. Eur J Pediatr 2001; 160(1): 47–53.

5. von Bodman A, Buchenau W, Bacher M, et al. The Tübingen palatal plate – an innovative therapeutic concept in Pierre Robin sequence. Wien Klin Wochenschr 2003; 115(24): 871–873.

6. Bacher M, Sautermeister J, Urschitz MS, et al. An oral appliance with velar extension for treatment of obstructive sleep apnea in infants with Pierre Robin sequence. Cleft Palate Craniofac J 2010; 48(3): 331–336.

7. Poets CF, Koos B, Reinert S, Wiechers C. The Tübingen palatal plate approach to Robin sequence: Summary of current evidence. J Craniomaxillofac Surg 2019; 47(11): 1699–1705.

8. Slator R. Pierre Robin Sequence. Rickham’s Neonatal Surgery. Springer, 2018: 445–456.

9. Universitätsklinikum Tübingen. Robin sequence. Dostupné na: https://www.medizin.uni-tuebingen.de/en-de/das-klinikum/einrichtungen/zentren/zentrum-fuer-lippen-kiefer-gaumenspalten-und-kraniofaziale-fehlbildungen/pierre-robin-sequenz.

10. Sedinová M. Význam fyzioterapie v prognóze pacientů s Pierre Robinovou sekvencí. Dostupné na: https://dspace.cuni.cz/bitstream/handle/20.500.11956/101535/120313317.pdf.

11. Tolarova MM. Pierre Robin Sequence: Background, Pathophysiology, Etiology. Medscape 2022.