• 대한장애인치과학회지
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• 제5권2호
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• pp.92-95
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• 2009

1. 이 증례는 Pierre Robin Sequence로 진단받은 14개월 남아로 PRS의 3요소 중 하악왜소증, 설하수증의 소견을 보이고 있었으며 기도확보는 측와위를 통하여, 섭식은 PEG 도관삽입술을 시행하여 유지하고 있었다. 2. PRS는 해부학적 폐쇄 질환이기도 하지만 성장 장애이기도 하므로 여러 분야의 다원적 접근 및 치료계획이 필요하며, 다른 증후군들과 연관되어 심장질환, 신경질환, 안과질환, 청력문제 등이 동반될 수 있으므로 치과 진료실 에서 PRS 환아의 진료시 이 점을 유의해야할 것이다.

• International Journal of Advanced Culture Technology
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• 제8권1호
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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.

• 대한기관식도과학회:학술대회논문집
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• 대한기관식도과학회 1983년도 제17차 학술대회연제순서 및 초록
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• pp.18.1-18
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• 1983

1923년 Pierre-Robin이 소하악증, 설하수증 및 고궁구개(high-arched palate)혹은 구개 파열의 3주징을 처음으로 기술하였다. 기도장애가 자주 발생하고, 신속하며 적절한 치료를 필요로 한다. 이 증후군은 출생아 5만명 중 1명 정도로 드물게 발생한다. 이 증후군의 원인은 불명확하지만 하악골발달의 일차적 부전 때문으로 알려져 있다. 소하악증으로 인해 혀의 기저부가 후방으로 이동하여 인두후벽에 접근하여 기도폐쇄의 증상이 발생하며 이는 영아가 앙와위를 취할 때 악화된다. 환자를 복와위로 유지시킴으로서 경한 기도폐쇄의 문제들은 해결되지만, 섭식장애가 있을 경우에는 기도폐쇄가 악화되므로 빨리 설고정술을 실시하는 것이 좋으나 기관절개술은 최대한 피하는 것이 좋다. 설고정술에는 Douglas 수술법, Kirschner wire 삽입법 및 Duhamel 봉합법 등이 있다고 알려져 있다. 저자들은 최근 호흡곤란 및 섭식장애를 주소로 내원한 출생 2개월 된 남아에서 소하악증, 설하수증 및 고궁구개 (high-arched palate)를 확인하였으며 Duhamel 봉합법으로 기도폐쇄의 증상이 호전되지 않아 기관절개술을 시행한 후 관찰 중인 이 환자를 Pierre-Robin Syndrome으로 생각하기에 보고하는 바이다.

• 대한치과의사협회지
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• 제15권8호
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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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• 제42권1호
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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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• 제49권4호
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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.

• 대한치과의사협회지
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• 제19권6호통권145호
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• pp.475-475
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• 1981

• 대한장애인치과학회지
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• 제12권2호
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• pp.87-91
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• 2016

저자는 소하악증으로 인해 기도유지가 어려우며 섭식 장애로 인한 다발성 우식을 보이는 피에르 로빈 증후군 환아의 증례를 보고하는 바이다. 환자는 소하악증 및 구개열, 심방중격 결손, 새끼 손가락의 측만지증(clinodactyly), 외사시(exotropia) 등의 증상을 동반하고 있었으며, 신체 발육이 매우 저하된 상태로 섭식 장애로 인한 구강내 침식 및 다발성 우식의 소견을 보이고 있었다. 환아는 가벼운 자폐 증상을 가지고 있으며 어린 나이로 협조를 구하기가 어려웠고 광범위한 치료가 필요한 상황이었으며 의식하 진정법시 하악의 발육 저하로 인한 기도 확보의 어려움이 예상되었기에 전신마취하 치과치료를 시행하였다. 피에르 로빈 증후군은 기도유지의 어려움과 섭식 장애로 구강위생 관리에 불리한 조건을 가지고 있지만 나이가 듦에 따라 점차 정상적인 하악의 성장이 이루어지므로 행동조절에 의한 일상적인 치과 처치도 가능할 것이다.

• 대한구순구개열학회:학술대회논문집
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• 대한구순구개열학회 2003년도 종합학술대회
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• pp.44-44
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• 2003

• Archives of Plastic Surgery
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• 제43권6호
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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.