• Maxillofacial Plastic and Reconstructive Surgery
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• 제36권1호
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• pp.7-12
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• 2014

Nevoid basal cell carcinoma syndrome (NBCCS) is inherited as an autosomal dominant trait with variable conditions, including multiple basal cell carcinoma, numerous keratocystic odontogenic tumors (KOTs) in the jaws, ectopic calcification of the falx cerebri, bifid ribs, macrocephaly, kyphoscoliosis, cleft palate, frontal and temporal bossing, mild ocular hypertelorism, mild mandibular prognathism, vertebral fusion, and so on. A 16-year-old boy visited the Dong-A University Medical Center, requiring diagnosis and treatment of multiple cystic lesions. He presented with many conditions related to NBCCS, including multiple KOTs, bifid rib, cleft lip, frontal bossing, mild ocular hypertelorism, and mild mandibular prognathism. No characteristic cutaneous manifestations (nevoid basal cell carcinoma) were observed in this patient. We report on a case of multiple KOTs associated with NBCCS with a review of the literature.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제30권3호
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• pp.237-245
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• 2004

왜소악, 설하수, 구개열의 세 가지 특징적 증상과 더불어 간헐적인 청색증, 흉곽의 함몰, 기도 폐색에 의한 호흡 곤란과 수유곤란 등의 임상소견을 나타낸 3례의 Pierre Robin sequence 환아에서, 혀의 위치를 교정하기 위한 구강저골막하박리술과 혀를 전방으로 위치시켜 호흡 곤란과 기도 폐색을 예방하기 위한 설구순접합술과 동시에 시행하고 약 1년 경과 후 설구순분리술을 시행한 결과 호흡 곤란과 수유장애의 해소, 혀의 전방 재위치, 체중 증가 및 하악골의 성장 증진 등 만족할 만한 결과를 얻었다. 구강저골막하박리술과 구순접합술 동시 시술은 Pierre Robin sequence 환아에서 기도 폐색의 외과적 치료를 위한 간단하고도 신뢰할만한 방법이라고 사료된다.

• Journal of Dental Anesthesia and Pain Medicine
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• 제21권2호
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• pp.129-137
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• 2021

Background: Inappropriate use of intravenous and inhaled anesthetics may be dangerous in infants undergoing facial cleft surgeries. This study primarily aimed to compare the effect of infraorbital nerve block on sevoflurane requirement in infants undergoing cheiloplasty. Intraoperative opioid consumption, hemodynamics, blood glucose levels, extubation time, and delirium were also compared. Methods: This prospective, randomized, double-blinded study was conducted in 34 infants undergoing cheiloplasty under general anesthesia. After induction, group A received bilateral infraorbital nerve block with 0.5 mL of 0.5% bupivacaine and group B 0.5 mL saline. An increase in heart rate or blood pressure > 20% was managed by increasing sevoflurane by 2-2.5%, followed by fentanyl 0.5 ㎍/kg bolus. The chi-square test and independent-sample t-test were used where applicable. Results: Demographics, duration of surgery, and intravenous fluids used were comparable between the groups. Compared to group A, patients in group B had significantly higher consumption of fentanyl (14.2 ± 4.4 ㎍ vs. 22.1 ± 6.2 ㎍) and sevoflurane (14.2 ± 4.8 mL vs. 26.8 ± 15.6 mL). Intraoperative hemodynamic parameters were significantly lower in group A, the number of times increases in hemodynamic parameters occurred, and fentanyl supplemental bolus was required remained significantly lower in group A than in group B. Intraoperative glycemic levels remained higher in group B, and the extubation time was significantly shorter in group A than in group B (4.40 ± 1.60 min vs. 9.2 ± 2.18 min). Group A had a lesser occurrence of postoperative delirium. Conclusion: Supplemental infraorbital block in infants undergoing cheiloplasty under general anesthesia resulted in significantly decreased anesthetic requirements and optimal hemodynamic and glycemic levels with faster extubation and lesser delirium.

• 대한두개안면성형외과학회지
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• 제12권1호
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• pp.71-74
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• 2011

Purpose: Sarcoidosis is a systemic noncaseating granulomatous disease of an unknown origin, and can involve any organ including the skin. The infiltration of sarcoid granuloma in an old cutaneous scar is an uncommon cutaneous manifestation of sarcoidosis. This paper reports a 35-year old female who presented with cutanesous nodules in previous facial scars. Methods: A 35-year-old female presented with cutaneous nodules for 2 months in previous scars of the forehead and lower lip that she had acquired in the childhood. An excisional biopsy of the lower lip mass, serologic examinations and radiologic studies were performed. Results: The excisional biopsy revealed noncaseating granulomas consistent with sarcoidosis. Chest CT revealed both hilar and paratracheal lymphadenopathy with nodular densities in both lung fields. Routine laboratory tests, serologic tests, serum angiotensin converting enzyme level, sputum for acid-fast bacilli, ophthalmoscopic examination, TB-PCR and NTM-PCR showed normal findings. Therefore, the patient was diagnosed with sarcoidosis. The patient refused fiberoptic bronchoscopy and medication with oral steroid. Currently, the clinical manifestation and progress are being monitored closely, and treatment is expected to start with oral steroid according to the progress. Conclusion: For the proper management of cutaneous sarcoidosis, particularly scar sarcoidosis, plastic surgeons should be aware of the many clinical and histopathological features of sarcoidosis and recommend a systemic evaluation for early diagnosis and proper treatment.

• 대한치과교정학회지
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• 제34권3호
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• pp.261-267
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• 2004

비증후군성 구순구개열은 가장 빈도가 높은 선천성 기형 중의 하나로 특히 한국이나 일본과 같은 극동 지방에서 높은 발생율을 보이고 동유럽에서는 드물게 보고되고 있다. 이러한 인종에 따른 차이는 이 질환에 유전적인 배경이 있음을 의미한다. 본 연구의 목적은 한국인의 비증후군성 구개열과 연관이 있다고 알려진 SfaN1 단일 염기 다형성증의 발현빈도가 한극인 구개열 가족과 루마니아 구순구개열 가족 사이에 통계적으로 유의할만한 차이가 있는지를 알아보기 위하여 시행하였다. 한국인 26가족과 루마니아 18가족을 대상으로 하였다. 전체 인원수는 한국인의 경우 78명이었고 루마니아의 경우 41명이었다. 유전자 서열분석에 사용된 샘플은 각 참여자의 혈액이나 타액을 채취하여 분석하였다. SfaN1 단일 염기 다형성 증은 $TGF-{\beta}3$ 유전자의 5번 인트론에서 관찰된다 (A18141G) 결과를 보면 한국인과 루마니아인의 비증후군성 구개열 가족사이에는 통계적으로 유의할만한 차이 가 인지되었다. 전체 샘플 중에서 AA allele는 한국인에서는 18명(23.1%)이었으나 루마니아는 27명(65.9%)이었다. AS allele는 한국인에서는 27명 (34.6%)이었으나 루마니아에서는 13명 (31.7%)이었다. GG allele는 한국인에서는 33명 (42.3%)이었으나 루마니아에서는 1명 (2.4%)이었다. 두 집단 사이의 차이는 통계적으로 유의하였다 (p<0.001). 결론적으로 한국인과 루마니아인의 비증후군성 구개열 가족 사이에 SfaN1 단일 염기 다형성증의 발현빈도는 한국인에서 통계적으로 유의할만하게 높게 나타났으며, 이는 한국에서 루마니아보다 비증후군성 구개열의 빈도가 높게 나타나는 현상을 부분적으로 설명하여 주는 것으로 사료된다.

• 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.

• Journal of Dental Anesthesia and Pain Medicine
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• 제16권4호
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• pp.309-312
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• 2016

Patients with cleft lip and palate (CLP) must undergo corrective surgeries during infancy and early childhood. Many patients with CLP undergo orthognathic surgery during their childhood for correction of skeletal asymmetries or pharyngoplasty with a pharyngeal flap to improve the quality of speech and velopharyngeal function. During orthognathic surgeries, nasotracheal intubation is performed under general anesthesia. In our case report, the patient had undergone palatoplasty and pharygoplasty previously. During the orthognathic surgery, a flexible fiberoptic bronchoscope-guided nasotracheal tube was inserted through the pharyngeal flap ostium; however, active bleeding occurred in the nasopharynx. Bleeding occurred because the flap was torn. After achieving hemostasis, the surgery was completed successfully. Thus, if a patient may show the potential for velopharyngeal port obstruction, nasotracheal intubation should be performed with utmost care.

• Archives of Plastic Surgery
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• 제32권4호
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• pp.516-520
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• 2005

Multilayered cartilage graft has been clinically used at tip plasty, total ear reconstruction, and cleft lip nasal deformities. Perichondrial flap or a free perichondrium has been also commonly used for induction of neocartilage. However, the influence of perichondrium on multilayered cartilage graft to strength and growth rate is still unknown. The purpose of this study was to compare the strength and growth rate of different multilayered cartilage graft and to present the most ideal model of multilayered cartilage graft. Twenty New-zealand white rabbit were used. Triple layers of cartilage grafts with various perichondrium were inserted into six separate pockets in paravertebral region of rabbits. The grafts were grossly and microscopically observed on 8 and 16 weeks after grafting. On gross observation, the grafts showed marked enlargement of three layers of cartilage grafts with perichondrium compare with three layers of cartilage grafts without perichondrium In histologic examination, the number of PCNA-labelled cells were significantly high on three layers of cartilage grafts with perichondrium. In conclusion, this study suggested that triple-layer graft of cartilage grafts with perichondrium on the outside of triple-layer graft has strength and an effect on chondrogenetic induction by the perichondrium.

• 대한두개안면성형외과학회지
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• 제21권4호
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• pp.244-248
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• 2020

The infraorbital nerve is a branch of the trigeminal nerve. Injury to the infraorbital nerve can be caused by trauma, including various facial fractures. Due to this nerve injury, patients complain of numbness and pain in the entire cheek, the ala of nose, and upper lip. In general, spontaneous sensory recovery is expected after decompressive surgery. If nerve transection is confirmed, however, neurorrhaphy is typically performed. Here, we present a case in which microsurgery was not performed in a patient with Sunderland grade V avulsion injury of the infraorbital nerve due to a facial bone fracture. Gradual nerve function recovery was confirmed to be possible with conservative treatment and rehabilitation alone. These findings suggest that the nerve function recovery can be expected with conservative treatment, even for severe nerve injury for which microsurgery cannot be considered.

• Advances in pediatric surgery
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• 제9권2호
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• pp.81-88
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• 2003

Congenital dilatation of the common bile duct (choledochal cyst) is an uncommon disease. Although the etiology is unknown yet, various theories such as distal obstruction of the common bile duct, congenital weakness of the duct and anomalous union of the pancreaticobiliary duct have been offered to explain the occurrence of choledochal cyst. Thirty - six cases of choledochal cyst over 22 years were analyzed clinically and classified according to Todani's classification and Kimura's anomalous union of pancreaticobiliary duct type. Todani type 1 consisted with 22 cases which were subdivided into 19 cases of type Ia, 1 case of type Ib and 2 of type Ic. Type IVa consisted with 14 cases including one case of Caroli's disease. There were 25 type BP cases and 10 type PB cases and 1 normal pancreatobiliary junction. Serum alkaline phosphatase was increased significantly in almost all cases. Seven patients (19.4%) had associated congenital anomalies such as double gallbladder, left - sided gallbladder, common bile duct web, biliary atresia, accessory hepatic duct, heterotopic pancreas, cleft lip and 2 cases of intestinal malrotation. All patients underwent cyst excision and Roux - en - Y hepaticojejunostomy and cholecystectomy. There was one death due to methicillin resistant Staphylococcus aureus sepsis.