• 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.

• International Journal of Advanced Culture Technology
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• v.8 no.1
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• pp.1-12
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• 2020

Pierre Robin syndrome is characterized by micrognathia, glossoptosis, and cleft palate. Infants with Pierre Robin syndrome causes feeding difficulty, upper airway obstruction, and other symptoms. This study aims to examine the effects of applying dysphagia treatment to infants with Pierre Robin syndrome. The study participant was an infant who was born four weeks premature and referred for dysphagia treatment approximately 100 days after birth. At the initial assessment, the infant showed oral sensory sensitivity, a high level of facial and masticatory muscle tension, and a low stability of the chin and cheeks with almost no normal "sucking-swallowing-breathing" pattern. We set the baseline period and intervention period using the AB design. During the baseline period, non-nutritive sucking training using a rubber nipple was conducted without implementing an oral stimulation intervention. During the intervention period, non-nutritive sucking training and an oral stimulation intervention were performed. After the intervention period, the infant's daily oral intake and oral intake per time significantly increased compared to that during the baseline period. We observed that the oral intake time of the infant decreased during the intervention period compared to that in the baseline period, which indicated an improvement in control over the chin, tongue, and lip movements, a change in muscular tension, and stabilization of the "sucking-swallowing-breathing" pattern. We provided dysphagia treatment before breastfeeding, it was positive effects such as normal development of the infant, transition from tube feeding to bottle feeding, and enhancement of overall oral motor function.

• Proceedings of the KOR-BRONCHOESO Conference
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• 1983.05a
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• pp.18.1-18
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• 1983

In 1923 Pierre-Robin first described the triad of micrognathia, glossptosis and high-arched or cleft palate. Respiratory distress is not an uncommon occurrence and requires prompt and appropriate treatment. The syndrome occurs once in approximately 50,000 births. Although the etiology of the syndrome is obscure, it is known that the syndrome probably results from a primary deficiency early in fetal life of mandibular development. The symptoms of airway obstruction develop as the base of the tongue falls posteriorly and approximates the posterior pharyngeal wall because of micrognathia, and is aggravated when the infant is in the supine position. The problems of mild airway obstruction can be solved by keeping the infant in the prone position. In case there are feeding difficulties, prompt glossopexy should be preformed as airway is aggravated, but tracheostomy is best avoided. There is some forms of glossopexy including Douglas operation, the insertion of Kirschner wire and Duhamel suture. We identified micrognathia, glossoptosis and high-arched palate in a 2-month-old infant who was brought to our hospital with complaints of dyspnea and feeding difficulties, and we reports this case, since we think this infant whom we observe after tracheostomy because we found Duhamel suture could not relieve the symptoms of airway obstruction as Pierre-Robin syndrome.

• The Journal of the Korean dental association
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• v.15 no.8
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• pp.607-610
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• 1977

When micrognathia (Small jaw) and glossoptosis (Falling backward of the tongue) occur in the newborn, there is a grave danger of upper airway obstruction. These deformities are frequently associated with an incomplete cleft of the palate, and the entity has been referred to as the Pierre Robin Syndrome. This is to report a case of Pierre Robin Syndrome of 40 day old Korean male infant, whose birth weight was about 3.6kg, and gestation period was 39 weeks. From birth, dyspnea and feeding difficulty were developed. The authors treated the patient by surgical procedure (glossopexy). The post operative course was uneventful.

• 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.

• Imaging Science in Dentistry
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• v.49 no.4
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• pp.323-329
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• 2019

Pierre Robin sequence (PRS) is characterized by the triad of micrognathia, glossoptosis, and airway obstruction. PRS does not have a single pathogenesis, but rather is associated with multiple syndromes. This report presents the case of a 35-year-old woman with PRS and scoliosis. Among the syndromes related to PRS, cerebro-costo-mandibular syndrome (CCMS), which is characterized by posterior rib gap defects and vertebral anomalies, was suspected in this patient. However, no posterior rib gap defect was detected on radiological examinations. Although over 80 cases of CCMS have been reported to date, few cases of PRS with scoliosis alone have been reported. Therefore, this report demonstrated the clinical, radiological, and cephalometric characteristics of an adult patient with PRS and scoliosis, but without rib anomalies.

• The Journal of the Korean dental association
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• v.19 no.6 s.145
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• pp.475-475
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• 1981

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.12 no.2
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• pp.87-91
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• 2016

Pierre Robin syndrome (PRS) is characterized by the triad of congenital mandibular hypoplasia, glossoptosis and cleft palate. Infant PRS patients are frequently suffering from upper airway obstruction, gastroesophageal reflux and growth retardation caused by above mentioned problems. We report a dental caries treatment of 3-year old girl with Pierre Robin syndrome with multiple caries. The cause of multiple caries was mainly presumed as patient's eating habit caused by her general condition. She had some feeding problems and had history of gastric tube. She was still using milk bottle and took more than an hour to finish a meal. The treatment was performed under general anesthesia considering patient's condition; mild autism, poor cooperation and respiratory problem due to micrognathia. Severely affected upper incisors were treated with pulp treatment and restored with zirconia crown for esthetic purpose. Lower incisors were treated with pulp treatment and restored with composite resin. Upper right first primary molar was restored with stainless steel crown and other primary molars were treated with composite resin. There were no postoperative complications. According to her parents, the patient's compliance to oral hygiene management was greatly improved after the treatment since she was very pleased with the esthetic result and highly motivated by her looks. The treatment without sedation or general anesthesia would be possible once the airway is improved as the mandible grows.

• Proceedings of The Korean Cleft Lip And Palate Association
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• 2003.06a
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• pp.44-44
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• 2003

• Archives of Plastic Surgery
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• v.43 no.6
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• pp.506-511
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• 2016

Background The indications for surgical airway management in patients with Robin sequence (RS) and severe airway obstruction have not been well defined. While certain patients with RS clearly require surgical airway intervention and other patients just as clearly can be managed with conservative measures alone, a significant proportion of patients with RS present with a more confusing and ambiguous clinical course. The purpose of this study was to describe the clinical features and objective findings of patients with RS whose airways were successfully managed without surgical intervention. Methods The authors retrospectively reviewed the medical charts of infants with RS evaluated for potential surgical airway management between 1994 and 2014. Patients who were successfully managed without surgical intervention were included. Patient demographics, nutritional and respiratory status, laboratory values, and polysomnography (PSG) findings were recorded. Results Thirty-two infants met the inclusion criteria. The average hospital stay was 16.8 days (range, 5-70 days). Oxygen desaturation (<70% by pulse oximetry) occurred in the majority of patients and was managed with temporary oxygen supplementation by nasal cannula (59%) or endotracheal intubation (31%). Seventy-five percent of patients required a temporary nasogastric tube for nutritional support, and a gastrostomy tube placed was placed in 9%. All patients continued to gain weight following the implementation of these conservative measures. PSG data (n=26) demonstrated mild to moderate obstruction, a mean apneahypopnea index (AHI) of $19.2{\pm}5.3events/hour$, and an oxygen saturation level <90% during only 4% of the total sleep time. Conclusions Nonsurgical airway management was successful in patients who demonstrated consistent weight gain and mild to moderate obstruction on PSG, with a mean AHI of <20 events/hour.