• Archives of Plastic Surgery
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• 제38권4호
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• pp.547-551
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• 2011

Purpose: Pierre Robin sequence is a congenital malformation in which micrognathia causes glossoptosis and airway obstruction. If conservative treatment fails, surgical procedures such as tongue-lip adhesion can be performed. However, this procedure remains a subject of debate, with favorable results being countered by reports of complications. To overcome the above limitations, we revised the traditional method of tongue-lip adhesion using an alveolar protector. Methods: Between 1992 and 2011, a total of eight patients were identified with Pierre Robin sequence and were treated with tongue-lip adhesion. Two of these eight tongue-lip adhesion procedures were performed with an alveolar protector. The operative technique for tongue-lip adhesion was similar to that described in other published reports. The alveolar protector was inserted between the ventral surface of the tip of the tongue and the lower labial sulcus. Results: Tongue-lip adhesion failed in two patients because of wound dehiscence. The primary surgical success rate was 66.7%. In the two tongue-lip adhesion procedures performed with the alveolar protector, we observed no postoperative complications. Conclusion: Resistance to traction of the tongue can be encountered with nonunionized symphysis menti, causing loosening of the traction suture through the symphysis menti. This can lead to backward positioning of tongue, resulting in dehiscence of tongue lip adhesion. The alveolar protector is a good adjunct to tongue-lip adhesion because this method avoids postoperative loosening of the traction suture and wound dehiscence. It is a simple and effective auxiliary method that yields functional improvement.

• Archives of Plastic Surgery
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• 제44권5호
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• pp.434-438
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• 2017

This is a brief clinical report describing an 18-month-old female with Robin sequence found to have an incidental mandibular cystic lesion on a head computed tomography scan in the preoperative workup before performing mandibular distraction. She underwent enucleation of the tumor, which was found to be a dentigerous cyst. One year following cyst enucleation, mandibular distraction was performed in order to alleviate her tongue-based obstruction. This case demonstrates the ability of the mandibular bone to successfully regenerate after undergoing cyst enucleation.

• 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.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제32권3호
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• pp.242-245
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• 2010

Pierre Robin sequence (PRS) describes the clinical triad of micro- and/or retrognathia, glossoptosis and cleft soft palate. Glossopexy has been demonstrated to be an effective treatment in selected cases of obstruction caused by glossoptosis (generally 6 to 10 months of glossopexy period). If radiofrequency therapy (RF) can reduce tongue volume in PRS, it will be helpful in early releasing of the glossopexy. Two-dayold patient showed a PRS triad. Intermittent cyanosis, respiratory difficulty and feeding problems were also observed. The respiration was not improved and prolonged intubation increased the possibility of respiratory complications like pneumonia. The surgical intervention- glossopexy and RF was done 20 days after birth. We applied RF combined with conventional glossopexy and could get successful results while reducing the overall treatment time to 6 weeks. The follow-up until 12 months after birth was uneventful. Considering that early recovery is highly beneficial to PRS patients by reducing risks associated with glossopexy and low energy RF application is very simple and low risk to patient, our combination therapy should be considered for the treatment of airway problem related to PRS.

• 대한치과교정학회지
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• 제51권5호
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• pp.337-345
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• 2021

Objective: To investigate the phenotypes and predominant skeletodental pattern in pre-adolescent patients with Pierre-Robin sequence (PRS). Methods: The samples consisted of 26 Korean pre-adolescent PRS patients (11 boys and 15 girls; mean age at the investigation, 9.20 years) treated at the Department of Orthodontics, Seoul National University Dental Hospital between 1998 and 2019. Dental phenotypes, oral manifestation, cephalometric variables, and associated anomalies were investigated and statistically analyzed. Results: Congenitally missing teeth (CMT) were found in 34.6% of the patients (n = 9/26, 20 teeth, 2.22 teeth per patient) with 55.5% (n = 5/9) exhibiting bilaterally symmetric missing pattern. The mandibular incisors were the most common CMT (n = 11/20). Predominant skeletodental patterns included Class II relationship (57.7%), posteriorly positioned maxilla (76.9%) and mandible (92.3%), hyper-divergent pattern (92.3%), high gonial angle (65.4%), small mandibular body length to anterior cranial base ratio (65.4%), linguoversion of the maxillary incisors (76.9%), and linguoversion of the mandibular incisors (80.8%). Incomplete cleft palate (CP) of hard palate with complete CP of soft palate (61.5%) was the most frequently observed, followed by complete CP of hard and soft palate (19.2%) and CP of soft palate (19.2%) (p < 0.05). However, CP severity did not show a significant correlation with any cephalometric variables except incisor mandibular plane angle (p < 0.05). Five craniofacial and 15 extra-craniofacial anomalies were observed (53.8% patients); this implicated the need of routine screening. Conclusions: The results might provide primary data for individualized diagnosis and treatment planning for pre-adolescent PRS patients despite a single institution-based data.

• 대한장애인치과학회지
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• 제15권1호
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• pp.55-59
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• 2019

PRS는 소하악증, 설하수증, 구개열의 세가지 임상적 특징을 갖는 질환으로 상기도 폐쇄로 인한 호흡곤란, 섭식장애를 나타낸다. PRS 환아는 치과 치료 시 호흡 관리에 대한 고려가 필요하고 또한 다양한 증후군이 동반될 수 있어 치과 치료시 전신적인 상태에 대한 고려가 필요하다. 본 증례에서는 다발성 치아우식증을 주소로 내원한 PRS 환자에 대한 보고로 기도 확보 및 유지의 어려움이 예상되었으나 치과적 치료를 전신마취 하 성공적으로 시행되었다.

• 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.

• Journal of Interdisciplinary Genomics
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• 제3권2호
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• pp.30-34
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• 2021

Campomelic dysplasia (CD) is a rare genetic disorder characterized by multiple skeletal anomalies and the abnormal development of male reproductive organs. To date, the SOX9 gene is the only known causal gene for CD, and approximately 90 causative mutations in SOX9 have been identified worldwide. CD is diagnosed based on clinical characteristics of skeletal dysplasia (e.g., short bowed long bones, kyphoscoliosis, bell-shaped thoracic cage with 11 pairs of ribs, and hypoplastic scapulars), typical facial features of Pierre Robin sequence with cleft palate, and gonadal dysgenesis in 46,XY individuals. Most patients with CD exhibit life-threatening respiratory failure owing to laryngotracheomalacia and hypoplastic thorax during the neonatal period. Although fatal complications decrease after infancy, several medical conditions continue to require proper management. A better understanding of this rare but lethal condition may lead to more appropriate treatments for patients.