• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.30 no.3
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• pp.237-245
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• 2004

The deformities of micrognathia and glossoptosis in the newborn are frequently associated with a cleft palate, which is known as Pierre Robin sequence. Upper airway obstruction is the most serious problem in these patients. Treatment of Pierre Robin sequence includes either positional or surgical intervention. Mild cases are often managed in the prone position. However, when the patient fails to thrive due to chronic upper airway obstruction, or severe respiratory distress ensures despite positional treatment, surgical intervention is mandatory to relieve the obstruction. We experienced three infants with Pierre Robin sequence who showed a symptom triad of micrognathia, glossoptosis, and cleft palate. Intermittent cyanosis, depression of the chest, respiratory difficulty and feeding problems were also observed. To relieve severe upper airway obstruction caused by micrognathia and glossoptosis, we simultaneously performed modified tongue lip adhesion (TLA) and a subperiosteal release of the floor of the mouth (SRFM). Respiratory and feeding difficulties were relieved, the tongue positioned anteriorly, body weight increased, and mandibular growth improved. Simultaneous TLA and SRFM may constitute a simple and reliable method for surgical treatment of airway obstruction in patients with Pierre Robin sequence.

• Archives of Plastic Surgery
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• v.35 no.3
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• pp.349-353
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• 2008

Purpose: There are 3 well-known surgical procedures to treat Pierre Robin sequence: tongue-lip adhesion, distraction osteogenesis of mandible, and tracheostomy. The classical tongue-lip adhesion is an effective way to keep airway. The tongue, however, becomes quite non-mobile and appears dormant until the patient could control upper airway and the adhesion can be maintained for prolonged period. Most of all, this procedure does not provide the correction of the micrognathia. Distraction osteogenesis is a good technique to correct micrognathia and to prevent tracheostomies in patients with Pierre Robin sequence. But airway keeping procedure is needed during the distraction period. The purpose of this study is to determine the usefulness of temporary tongue-lip traction during the initial period of mandibular distraction in Pierre Robin sequence patients with severe airway problems requiring operative procedure. Methods: It was a prospective study of 2 Pierre Robin sequence patients aged between 4 months and 6 months requiring surgical procedure to correct recurrent and severe pulmonary complications. Two patients underwent distraction osteogenesis of mandible. During the operation, deep one tension suture was performed to tract the tongue and lip. When the patient gained control of upper airway at the initial period of distraction and micrognathia was corrected, the traction suture was removed. Results: All patients were followed up. No patients complained severe pulmonary complications and tracheostomy could be avoided. No patients had severe pulmonary complication. The pulmonary condition of patients was good. Conclusion: In severe Pierre Robin sequence case, temporary tongue-lip traction is a good assistant method in distraction osteogenesis because this method can avoid tracheostomy.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.5 no.2
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• pp.92-95
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• 2009

In 1923, Pierre Robin, a French stomatologist, first reported the association of micrognathia with glossoptosis. As more than 80% of all Pierre Robin cases accompanied with other syndrome such as Stickler syndrome, velocardiofacial syndrome, fetal alcohol syndrome, Treacher Collins syndrome, etc, it is called Pierre Robin sequence(PRS). PRS is described in the literature as a triad of anomalies characterized by micrognathia, glossoptosis and cleft palate. Clinically, the triad consists of airway obstruction and feeding difficulty, which are more frequent and severe in the neonatal period. In this case, a 14-month old boy with PRS was referred to the department of pediatric dentistry, Yonsei university dental hospital, who resolved airway obstruction by lateral positioning and feeding problem by percutaneous endocutaneous gastrotomy insertion. PRS is a developmental disorder as well as an anatomic obstructive disorder, therefore it should be dealt with by a multidisciplinary team.

• Proceedings of The Korean Cleft Lip And Palate Association
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• 2002.05a
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• pp.43-44
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• 2002

• Clinical and Experimental Pediatrics
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• v.52 no.5
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• pp.603-606
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• 2009

The Pierre Robin sequence (PRS) is the nonrandom association of micrognathia, cleft palate, and glossoptosis, leading to respiratory and feeding difficulties that appear neurogenic rather than mechanical in causation. Genetic determinants are thought to underlie this functional and morphological entity, based on the existence of Mendelian syndromes with PRS. Here, we demonstrate the association of PRS with trisomy 8p due to duplication of a segment as the karyotype 46,XX,dup(8)(p21.3p23.1) and confirm the additional materials as chromosome 8 via whole chromosome paint probes. Our observation supports the hypothesis regarding a genetic basis for nonsyndromic PRS, strengthens the possible genetic association with isolated cleft palate, and provides a candidate PRS locus in chromosomal region 8(p21.3p23.1).

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.34 no.4
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• pp.460-467
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• 2008

Pierre Robin sequence as a symptom triad of micrognathia, glossoptosis, and cleft palate results in upper airway obstruction and feeding problems. If mild, it is often managed in the prone position. When positional treatment fails, however, surgical intervention such as tongue-lip adhesion, tracheostomy, and mandibular distraction osteogenesis is mandatory to relieve airway obstruction. There has been growing interest in the application of distraction osteogenesis for the management of craniofacial abnormalities. The mandibular distraction osteogenesis to newborns may prevent the airway obstruction, decrease the potential tracheostomy, and reduce the likehood of orthognathic surgery after growth. We experienced an infant with Pierre Robin sequence who showed mandibular hypoplasia, glossoptosis, incomplete cleft palate, intermittent cyanos is, depression of the chest, and respiratory difficulty associated with airway obstruction. We treated the airway obstruction by tongue-lip adhesion at 2 weeks of age, and treated the mandibular retrognathism and depression of the chest byusing internal mandibular distraction osteogenesis at 7 month of age. The mandible moved forwardly, the upper airway space was enlarged, and the antero-posterior distance of the mandible was elongated after the mandibular distraction. Mandibular distraction osteogenesis may be a promising technique to avoid the need of tracheostomy and orthognathic surgery, and to correct airway obstruction in infants with congenital craniofacial malformation.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.42 no.1
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• pp.47-50
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• 2016

Patients with Pierre Robin sequence exhibit varying degrees of airway obstruction and feeding difficulty. In some patients, airway obstruction may be profound, warranting surgical intervention to maintain a patent airway. The purpose of this article is to highlight the advantages of the tongue-lip adhesion procedure for the management of airway obstruction in such patients compared to the currently available options.

• Proceedings of The Korean Cleft Lip And Palate Association
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• 2003.06a
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• pp.40-40
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• 2003

• Proceedings of The Korean Cleft Lip And Palate Association
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• 2002.05a
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• pp.41-41
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• 2002

• Maxillofacial Plastic and Reconstructive Surgery
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• v.33 no.3
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• pp.270-275
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• 2011

Pierre Robin Sequence (PRS) is known as an anomaly consisting of respiratory obstruction with glossoptosis, micrognathia and cleft palate in a newborn. The etiology of PRS is not known, but several factors may be involved simultaneously. Mortality rate of PRS is about 5~30% and the treatment method is divided into both conventional treatments and surgical interventions. If the respiratory obstruction is not resolved by the conventional method, surgical treatment, such as subperiosteal release of the floor of the mouth, tongue-lip adhesion, tracheostomy, distraction osteogenesis may be needed. This study reports a case of PRS in a newborn male at 20 days, with dyspnea and feeding difficulties. Clinical examination showed micrognathia with glossoptosis and cleft palate as the typical PRS triad. We tried surgical intervention with subperiosteal release of the floor of the mouth and tongue-lip adhesion and surgery was successful. At $19^{th}$ months, we also repaired the incomplete cleft palate successfully using 2-Flap palatoplasty.