Cleft palate in Pierre Robin sequence

Houda Oubejja,Fouad Fouad Ettayebi,Souha Qurouach,Chadi Elhasnaoui,Imane Menbahi,Ismail Benomar,S Bourekkadi,A Soulaymani,K Slimani,A Mokhtari,H Hami

doi:10.1051/e3sconf/202131902013

Abstract

Pierre Robin’s sequence PRS is a congenital malformative sequence with a retrognathism, a glossoptosis and an upper respiratory obstruction with an associated cleft palate. This malformative association can be disabling for both the child and his parents, because of its aesthetic, functional, psychological and social impacts. Patients and methods: We have investigated 14 patients with PRS presenting cleft palates, collected over a period of 5 years, from March 2014 to November 2019 in the pediatric surgical emergencies department (PSE) in Rabat Children’s Hospital (HER). Results: Nine of the 14 patients had a complete cleft palate. The average age of intervention was 23 months ranging from 13 months to 4 years. The study showed a slight female predominance (n = 8, 57%). Regarding the treatment, 3 patients were treated with the Bardach’s technique, 3 had the Von Langenbeck’s technique, 3 had the Push-back technique and one patient who suffered from a post-operative persisting velar division was treated by veloplasty 3rd generation. The hospital stay was around 24 hours and the post-operative follow-up was uncomplicated for all our patients. Conclusion: Although we had a relatively high success with our approach, the complexity and lack of consensus regarding the management of Pierre Robin’s sequence still requires the involvement of a multidisciplinary team. The main aim is to give the best aesthetical and functional results in order to reduce the suffering of the PRS children and families.

Highlights

Pierre Robin sequence (PRS) is a congenital malformative sequence of the face associating a retrognathism, a glossoptosis, and an obstruction of the upper airway
The subsequent mandibular hypoplasia would lead to a small mouth volume, an abnormal position of the tongue and an impaired closure of the palate
The aim of this study is to describe our experience in the management of PRS children, the place of the palatoplasty

Summary

Introduction

Pierre Robin sequence (PRS) is a congenital malformative sequence of the face associating a retrognathism, a glossoptosis, and an obstruction of the upper airway (secondary to an abnormal position of the tongue). The retrognathism forces the tongue to fall back (glossoptosis) causing the obstruction of the upper airway. This glossoptosis, in addition to the obstruction of the upper airway is probably the cause behind the cleft palate. The most common ones are Stickler, Treacher Collins and the velocardiofacial syndrome (65%). To this day, the exact cause of PRS is still unknown. The diagnosis is mainly clinical and consists on a retrognathism and a glossoptosis with more or less a cleft palate. A multidisciplinary team should be involved in the management of children with PRS

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