• 대한두경부종양학회지
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• 제36권1호
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• pp.27-31
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• 2020

The midline nasal dermoid cysts are rare congenital neoplasms, which are diagnosed frequently in childhood. Masses are often noticeable at birth gaining size over time with recurrent infections and usually arise from the nasal cavity or lower 1/3 of the nasal dorsum. CT scan as the primary investigation is helpful to determine accurately the size and extent of the lesion as well as the integrity of adjacent bony structures. MRI scan is recommended to rule out an intracranial extension or sinus tracts. Treatment of choice is the complete surgical excision preserving the cyst wall. Here in, we present an unusual case of nasofrontal dermoid cyst in a 19-year-old boy without radiographic evidence of transcranial extension. In this case, we surgically removed nasofrontal dermoid cyst via transcolumellar approach. We also corrected saddle nose deformity after mass removal. Therefore, in this case, we experienced a successful case in which the nasofrontal dermoid cyst was totally removed without facial scar and deformity.

• Journal of Genetic Medicine
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• 제16권1호
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• pp.1-9
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• 2019

Noonan syndrome (NS) and NS-related disorders (cardio-facio-cutaneous syndrome, Costello syndrome, NS with multiple lentigines, or LEOPARD [lentigines, ECG conduction abnormalities, ocular hypertelorism, pulmonic stenosis, abnormal genitalia, retardation of growth and sensory neural deafness] syndrome) are collectively named as RASopathies. Clinical presentations are similar, featured with typical facial features, short stature, intellectual disability, ectodermal abnormalities, congenital heart diseases, chest & skeletal deformity and delayed puberty. During past decades, molecular etiologies of RASopathies have been growingly discovered. The functional perturbations of the RAS-mitogen-activated protein kinase pathway are resulted from the mutation of more than 20 genes (PTPN11, SOS1, RAF1, SHOC2, BRAF, KRAS, NRAS, HRAS, MEK1, MEK2, CBL, SOS2, RIT, RRAS, RASA2, SPRY1, LZTR1, MAP3K8, MYST4, A2ML1, RRAS2). The PTPN11 (40-50%), SOS1 (10-20%), RAF1 (3-17%), and RIT1 (5-9%) mutations are common in NS patients. In this review, the constellation of overlapping clinical features of RASopathies will be described based on genotype as well as their differential diagnostic points and management.

• 대한소아치과학회지
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• 제28권1호
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• pp.166-170
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• 2001

Rubinstein-Tabyi syndrome은 드물게 나타나는 기형증후군으로 정신 발달지연, 넓은 손가락과 발가락, 작은 키, 특징적인 안모 등의 증상들을 주 증상으로 하는 여러 계통의 형태이상이 비 특정 양상으로 나타나는 선천적 증후군이다. 1963년 Rubinstein과 Tabyi에 의해 처음 보고된 이 증후군은 많은 연구에도 불구하고 명확한 원인이 알려져 있지 않고 있으며, 1990년 이후 Rubinstein-Tabyi syndrome에 대한 특정소인 연구에서 이 증후군의 임상적 특징을 보이는 소수의 환자에게서 16번 염색체의 16p13.3의 유전학적 단절이 보고되었다. 그러나, 이 증후군의 특정병인으로 보기는 힘들고, 여전히 이 증후군의 진단에는 특징적인 임상증상들로 인해 가능하다. 이 증후군의 치과적 증상으로는 높게 위치한 구개, 소하악증 다발성 우식증 등이 보고되고 있다. 다발성 우식증을 주소로 내원한 6세 5개월된 남아가 넓은 엄지손가락과 발가락, 정신 및 운동발달지연과 안검하수증, 이상형태의 귀 등을 포함하는 안면부 이상 등의 증상들을 보였다 저자들은 환자가 보이는 특징적인 임상 증상들로 인해 Rubinstein-Tabyi syndrome으로 진단을 내리고 환자의 전신 상태를 고려하여 모든 치과 치료를 전신마취 하에 시행하였다.

• 대한구순구개열학회지
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• 제16권1호
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• pp.37-49
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• 2013

Cleft lip is a common congenital facial deformity which might cause speech, hearing, appearance, and psychological disorder. For the purpose of appropriate management for the cleft lip patients according to their individual situations, reviews of the historical evolution for the cleft lip treatment were summarized. More than 15 English written articles with 4 related historical books were reviewed, and the chronology of the cleft lip management from ancient to recent twenty first century, via middle ages and Renaissance, were summarized. Multifactorial causes of cleft lip, before the modern understanding of embryological background of it, most management of cleft lip has been explained under the basis of religions and/or superstitions. As the anatomic and embryologic knowledges were known and revealed, various misconceptions were corrected continously, and the simple closure of the lip defect was also evoluted to the applications of plastic concept. Recently, cosmetic outcomes with functional results, such as speech, hearing, psychological status, have been considered importantly, under the multidiciplinary care system. For the better understanding of cleft lip management as a routine esthtetic and funtional reconstructive procedure, the various historical treatment trends were reviewed and summarized as time goes on. This review presentation will discuss the appropriate management for cleft lip patients.

• Archives of Plastic Surgery
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• 제38권3호
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• pp.251-256
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• 2011

Purpose: A nasolabial cyst is a rare non-odontogenic, soft-tissue, developmental cyst arising anywhere on the face inferior to the nasoalar region. It is thought to arise from either epithelial remnants trapped along the lines of fusion during the development of face or the remnants of the developing nasolacrimal duct. This study examines various features of nasolabial cysts with bony involvement to provide a basis for correct diagnosis and treatment. Methods: Eight cases of nasolabial cyst treated in Soonchunhyang Hospital between March 2002 and July 2010 were examined in terms of their clinical features and radiological and histological findings. Seven patients underwent surgical excision of the cyst via an intraoral, sublabial approach. One underwent incision and drainage. Results: Our eight patients were seven women and one man. The most frequent symptoms and signs were facial deformity and swelling of the nasolabial fold. Computed tomography (CT) showed a well-circumscribed cystic mass lateral to the pyriform aperture. Seven cases had erosive lesions on CT, and the intraoperative findings were consistent with a nasolabial cyst with a bony defect. Typical histopathological findings showed that these cysts were most frequently lined with respiratory epithelium with ciliated columnar cells and cuboid cells. No patient developed complications or recurrences. Conclusion: A nasolabial cyst is often unrecognized or confused with other intranasal masses, including fissural and odontogenic cysts, midface infections, or swelling in the nasolabial area. Therefore, a careful clinical and radiological evaluation should be preformed when considering the differential diagnosis. We present eight patients with nasolabial cysts treated via a gingivobuccal approach with excellent functional and cosmetic results.

• 대한구순구개열학회지
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• 제15권2호
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• pp.61-68
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• 2012

Cleft lip and palate is the most common congenital facial malformation and has a significant developmental, physical, and psychological impact on those with the deformity and their families. When treating the patients with unilateral cleft lip, many surgeons adopt the rotation advancement flap method originally developed by Millard, or the triangular flap technique developed by Tennison, Randall or the modifications of these techniques. Among these, Millard's rotation advancement flap method has its advantage in designing the flap using the patient's anatomic landmarks. For performing this rotation advancement technique, skillful operation is needed to obtain esthetically satisfactory results. Vomer flap sometimes is used to repair anterior hard palate in complete cleft lip and palate patients. Vomerine tissue is readily available in the vicinity of the palatal defect and elevation of the vomerine flap is relatively simple procedure. In this article, we will introduce the comprehensive vomer flap technique conjunction with primary lip closure and review the comparative studies of the outcome of simultaneous repair of cleft lip and cleft hard palate with Millard's rotation advancement method and vomer flap.

• 대한장애인치과학회지
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• 제7권2호
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• pp.123-126
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• 2011

본 증례는 누난 증후군으로 진단 받은 환아에 대한 내용으로, 성장 지연, 작은 키, 좁고 높은 악궁 등의 특징적인 소견을 보였다. 체중은 8.8kg이었으며 신장은 100cm으로 2세 아이들의 성장 양상과 비슷한 수준을 나타내고 있었다. 이소맹출 되고 있는 매복 상악 측절치에 의해 상악 중절치의 맹출이 방해 받고 있었으며, 다수의 치아우식증이 관찰되었다. 이에 상악궁의 심한 총생이 예상되며, 측절치 치배로 인해 영구 상악 중절치 발육에 영향을 줄 것을 고려하여 전신마취 하에 측절치의 발거 및 치아우식증의 치료를 시행하였다. 주기적인 관찰기간 동안 영구 중절치의 발육에 큰 문제가 나타나지 않았으며, 정상적인 성장이 이루어지는 것을 관찰할 수 있었다.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제19권3호
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• pp.233-242
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• 1997

Recently the goal of orthognathic surgery has been focused on esthetic improvement of the patients. Also early corrective surgery was favorable selected by most of the oral and maxillofacial surgeons. We should consider the etiologic factor of the patient's dentofacial deformities when treatment is planned, because this is the major factor in estimating the predictability or stability of result. The more researches were carried on the etiologic factors of the dentofacical deformities, The more possibility of the early surgical correction will be increased. The authors analyzed about etiologic factors and epidemiologic studies of the forth patients who had received the orthognathic surgery. The analyzed results were as follows: 1. The predilection ration between male and female was 17:23, and 32 patients (80%) of 40 patients were aged twenties. 2. 26patients(65%) complained estetic problems as well as functional problems. 10 patients(25%) complained only esthetic problems, and 4 of 40 patients complained only functional problems. 3. Mandibular prognathism was found to be done most frequently(25, 39%). Facial asymmetry (13, 20%) and angle hypertrophy were found to be next in sequence. 4. Sagittal split ramus ostetomy was done most frequently(27, 35%). Lefort I osteotomy(13, 17%), angle reduction (12,16%), and genioplasty(11, 15%) were done also. 5. The number of the cases due to nonspecific etiologic factor was 22(55%), that of cases due to inhertied tendency was 12(30%), that of cases due to congenital anomaly was 3(7.5%), and that of cases due to trauma was 3(7.5%). 6. The number of patients who got only maxilliary surgery was 2(5%), that of patients who got only mandibular surgery was 23(57.5%), and that of patients who got simultaneous two jaw surgery was 15(37.5%).

• Maxillofacial Plastic and Reconstructive Surgery
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• 제27권2호
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• pp.183-187
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• 2005

관상접근법 후 발생한 측두부 함몰의 기전은 아직 명확하지는 않으나 중측두동맥의 손상에 의한 측두지방대의 소실과 변위, 측두근의 부피감소, 봉합시의 잘못된 위치에 의해 발생된다. 이에 저자등은 관상접근법 후 발생한 측두부 함몰을 빠른 혈관화와 골조직의 성장, 낮은 합병증을 보이는 $Medpor^{(R)}$를 이용하여 심미적인 개선을 얻을 수 있었고 이후 현재까지 양호한 경과를 보이기에 문헌고찰과 함께 보고하는 바 이다.

• 대한구순구개열학회지
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• 제2권1_2호
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• pp.29-41
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• 1999

구순구개열은 악안면의 선천기형중 발생빈도가 가장 높은 것으로 알려져 있으며,출생시부터 성장이 완료되는 시기까지 일관된 치료계획에 의한 종합적인 진료체계가 필수적이다. 구순구개열이 존재할 경우 구개부의 조기 봉합후의 반흔조직 형성으로 인한 치열궁의 협착 또는 중안모의 함몰 경향과 함께 상악 측절치의 선천적 결손 또는 기형치, 전치의 회전과 경사 등이 수반되므로 교정치료에 의한 기능적, 심미적인 치열의 회복이 필요하게 된다. 구순구개열 환자의 교정치료에 있어서 상악치열의 정상적인 배열과 구치부 반대교합의 치료를 위해 상악골 확대가 종종 필요하며, 이를 위하여 Hyrax appliance, Quad-helix 및 Fan-type expansion screw, Jointed fan type expander 등 다양한 장치가 활용될 수 있다. 심하지 않은 구순구개열 환자의 교정치료에서 상악골 확대를 위한 장치의 선택은 환자의 치열궁 형태, 연령, 구치부와 전치부의 치열궁 폭경 등 다양한 요인이 고려되어야 하며, 치료 후 악궁형태의 보정에 특별한 주의를 기울여야 할 것이다.