Distraction Osteogenesis in Pierre Robin Sequence

Research Article

Austin J Otolaryngol. 2016; 3(2): 1074.

# Distraction Osteogenesis in Pierre Robin Sequence

¹Maxillofacial Surgeon Hospital Del Salvador, Maxillofacial Surgeon Hospital San Borja Arriarán, Member of Chilean Society of Maxillofacial Surgery, Master in Education, Professor of Oral and Maxillofacial Surgery University of Chile, Santiago, Chile

²Oral and Maxillofacial Surgery, Hospital El Carmen de Maipú, Oral surgery instructor, Faculty of Medicine Pontifical Catholic University of Chile. Santiago, Chile

*Corresponding author: Faria R, Department of Maxillofacial Surgery, Universidad de Chile, Santiago, Chile

Received: May 10, 2016; Accepted: July 01, 2016; Published: July 04, 2016

## Abstract

The Pierre Robin sequence is a pathology derived from an alteration on the first and second branquial arch. The major problems of these patients are: the breathing problems due to micrognathia and glossoptosis that causes an upper airway obstruction.

The management has changed in the last years, with new surgical techniques to allow treating etiologic problems.

We did a review of literature about different kinds of treatment and show a 25 patient group treated with Pierre Robin Sequence.

Patients and Methods: We treated 25 new born patients, with Pierre robin sequence and severe breathing problems. 6 of them need mechanical ventilator assistance. We made an average of 18,8mm of mandibular distraction to improve their breathing parameters.

Results: After the first 6mm of mandibular distraction, spontaneous ventilation was observed, in the majority of these patients. At the end of distraction, the breathing function and facial balance was obtained.

Conclusion: Mandibular distraction osteogenesis is the best etiological surgical treatment for the Pierre Robin sequence with respiratory obstruction.

Keywords: Distraction osteogenesis; Pierre Robin sequence; Micrognathia; Upper airway obstruction

## Introduction

The Pierre Robin sequence is one of much pathology caused by developmental alterations of the 1st and 2nd branchial arch. It has been suggested to be an autonomic recessive disorder, characterized by mandibular hypoplasia, resulting in a cleft palate and Glossoptosis [1]. A Cleft palate makes sucking and swallowing difficult, allowing easy access of fluids into the larynx and Glossoptosis contributes to respiratory alteration. The mandibles poor development offers an inadequate space for the tongue to descend, a development which usually takes place during the 7th week of in-uterus life [2]. The vertical position of the tongue is the main factor that obstructs the horizontalization of the palate process, causing a clef palate.

The physiological alterations derived from this sequence are various. The most serious alteration and complex is the compromise of the Upper Airway (U.A.). The U.A. s obstruction can present several degrees of severity, from a simple chronic airway limitation and episodic apneas, to severely obstruction the ventilation causing respiratory acute insufficiencies.

Along with the respiratory problems, swallowing alterations can make oral nutrition difficult, which frequently leads to malnutrition. The gastroesophagic reflux, secondary to the chronic limitation of the U.A. also contributes to the nutritional problems, often making necessary the installation of naso-gastric probe or even a gastrectomy [3].

The aim of initial management of these new born is to correct ventilation, which can be achieved in a decubital - prono position. However a great vital risk remains with these conservative maneuvers.

Duhamel [4] reports a 50% mortality rate, while Bush [5] and Dykes [6] mention a rate between 10% to 30%.

When these maneuvers are insufficient, a therapy of oxygen administration through masks is needed. If the oxygen is saturated with hemoglobin, or the arterial gases parameters are still below normal ranges, the requirement of tracheal intubation is evaluated as an urgent and transitory way to assure the U.A.

The first surgical strategies to deal with these patients were based exclusively on urgent maneuvers with aimed to save the patient’s life through therapeutically tracheotomy.

Tracheotomy procedures made in new born have a high morbidmortality rate. Some authors described 1% to 5% mortality rate [7]. Morbidity is related to tracheal stenosis and language development disorders, reaching a 50% rate of intellectual failure as a consequence [8]. It is important to highlight that a patient who suffers from Pierre Robin sequence must maintain the tracheotomy procedure until a safe airway is achieved, which, in expert’s considerations, is not until the patient is 2 to 4 years old [9].

In 1937 Callister [10] and later in 1949 Longmire [11] described a new technique of skeletal traction with orthopedic devices, with the main purpose of stimulating the mandibular growth and development without the need of osteotomies. Their reports showed good results in the management of the U.A. and weight gain after 2 and 3 months of treatment, however, no mandibular osteotomies were performed, instead, the forces applied were directly transmitted to the temporo mandibular joints, causing joint dislocations and sometimes even ankylosis.

In 1946 Douglas [12] describes a suture technique of the tongue and lip in order to manage the S.A. obstruction by the exclusive manipulation of the tongue’s position. Later, in 1982 Parsons [13] defines the two criteria which are the groundwork to justify the adhesion of the tongue to the lip in these types of patients; an inadequate weight/stature gain during 7 days and / or the maintenance of an endotracheal tube for longer than 3 days. He did not describe how long this junction had to be kept, or if any posterior alteration in feeding or fonoarticulation occurred. However, the main disadvantage of the technique is its lack in treating the etiologic factor of the sequence, keeping the patient with his micrognathia (Figure 1).