• Imaging Science in Dentistry
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• 제54권1호
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• pp.13-24
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• 2024

Purpose: Pycnodysostosis (PYCD), an autosomal recessive syndrome, is characterized by an imbalance in bone remodeling that produces various clinical and radiographic craniofacial manifestations. This review represents a systematic examination of these manifestations, as well as oral features associated with PYCD. Materials and Methods: A systematic review was conducted across 8 databases from February to March 2023. The search strategy focused on studies reporting cases of PYCD that examined the clinical and radiographic craniofacial and oral characteristics associated with this syndrome. Results: The review included 84 studies, encompassing a total of 179 cases of PYCD. More than half of the patients were female (55.3%), and the mean age was 14.7 years. Parental consanguinity was reported in 51.4% of the cases. The most common craniofacial clinical manifestation was a prominent nose, observed in 57.5% of cases. Radiographically, the most frequently reported craniofacial characteristics included the presence of an obtuse mandibular angle (84.3%) and frontal cranial bosses(82.1%). Clinical and radiographic examinations revealed oral alterations, with micrognathia present in 62.6% of patients and malocclusion in 59.2%. Among dental anomalies, tooth agenesis was the most commonly reported, affecting 15.6% of patients. Conclusion: Understanding the clinical and radiographic craniofacial features of PYCD is crucial for dental professionals. This knowledge enables these clinicians to devise effective treatment plans and improve patient quality of life.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제22권4호
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• pp.442-448
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• 2000

저자들은 2례의 제2새열낭종을 경험하였다. 증례 1은 26세의 여자에서 3년 전부터 서서히 크기가 증가하다가 1년 전 임신과 함께 현저히 크기가 증가한 병력이 있으며, 낭종은 우측 악하부와 흉쇄유돌근 전내측에 위치한 무통성의 파동을 보이는 $7.5{\times}5{\times}4cm$ 크기의 연성 종물로서 내외측으로 누공 형성은 없었다. 증례 2는 56세 남자에서 2년전 악하간극 농양으로 진단하고 절개 및 배농을 시행한 병력이 있으며, 낭종은 좌측 악하부와 흉쇄유돌근 전내측에 위치한 무통성의 파동을 보이는 $5.5{\times}4{\times}3cm$ 크기의 연성 종물로서 역시 내외측으로 누공 형성은 보이지 않았다. 조직학적으로 중층편평상피로 이장되어 있었고 상피 하방에서는 림프양 조직이 관찰되었으며, 완전절제술을 시행한 다음 술후 $3{\sim}4$년이 경과된 현재까지 재발이나 다른 합병증 없이 양호한 경과를 보이고 있다.

• 대한장애인치과학회지
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• 제8권2호
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• pp.113-117
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• 2012

저자는 ARS를 가진 15세 여자 환아에 대한 임상적, 방사선학적 관찰을 통해 다음과 같은 지견을 얻었다. 1. 환아는 Axenfeld-Rieger Syndrome의 안과적 증상인 전안부 형성부전을 나타낸다. 2. 환아의 구강 및 구개악안면 소견으로 다수의 영구치 결손 및 왜소치, 상악 형성 부전, 구치부의 반대교합이 관찰되었다. 3. 환아의 성장에 따른 주기적인 치과적 검진과 추가적인 치료가 필요할 것으로 생각된다.

• 대한장애인치과학회지
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• 제12권2호
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• pp.92-95
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• 2016

Beckwith-Wiedemann syndrome은 다양한 임상적 특징을 보이는 질환이다. 거대설은 BWS 환아에서 가장 흔히 관찰되는 특징으로 그로 인한 섭식 문제와 호흡곤란이 야기될 수 있어 치과 치료 시, 주의를 요하게 된다. 또한 거대설로 야기될 수 있는 골격적인 문제에 대한 장기적인 평가가 필요하다.

• 대한소아치과학회지
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• 제21권2호
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• pp.605-610
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• 1994

Resricted opening of the mouth in children can be derived from a variety af extra and intra articular causes. A 5-year-old female patient with an elongated right coronoid process and with congenital missing teeth came to clinic for wearing of esthetic denture. A proper medical consultation and laboratory test had done for assessment a systemic syndrome related to her dental anomalies. The result was that her systemic findings dindn't accord specific signs and symptoms of Hallermann-Streiff syndrome. Unilateral coronoidectomy was fulfilled to improve mouth opening and subsequently endodontic and restorative procedure. Maxillary partial denture was delivered for esthetic problem. The periodic recall medical/dental check-up are recommended.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제44권2호
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• pp.86-90
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• 2018

About one-third of patients with transsphenoidal basal encephaloceles have associated congenital anomalies, including cleft palate. Moreover, they are often plagued by symptomatic exacerbations in the form of upper respiratory obstructions, cerebrospinal fluid leaks, meningitis, etc., with few patients being asymptomatic. We herein present a rare asymptomatic case of transsphenoidal basal encephalocele in an 18-month-old child with cleft palate and highlight a modified version of two-flap palatoplasty.

• Journal of Genetic Medicine
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• 제10권1호
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• pp.57-61
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• 2013

Acromegaloid Facial Appearance syndrome is a very rare syndrome combining acromegaloid-like facial appearance, thickened lips and oral mucosa and acral enlargement. Progressive facial dysmorphism is characterized by a coarse facies, a long bulbous nose, high-arched eyebrows, and thickening of the lips, oral mucosa leading to exaggerated rugae and frenula, furrowed tongue and narrow palpebral fissures. We report a case of acromegaloid facial appearance syndrome in a 19-year-old male patient who presented with all the characteristic features of the syndrome along with previously unreported anomalies like dystrophic nails, postaxial polydactyly and incisal notching of teeth.

• Journal of Dental Anesthesia and Pain Medicine
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• 제19권5호
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• pp.307-312
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• 2019

Klippel-Feil syndrome is characterized by congenital fusion of two or more cervical vertebrae, a low hair line at the back of the head, restricted neck mobility, and other congenital anomalies. We report a 16-year-old young man with Klippel-Feil syndrome, Sprengel deformity of the right scapula, thoracic kyphoscoliosis, and mandibular prognathism with an anterior open bite. He was treated with orthodontic treatment and maxillofacial surgery. An anticipated difficult airway due to a short neck with restricted neck movements and extrinsic restrictive lung disease due to severe thoracic kyphoscoliosis increased his anesthesia risk. Due to his deviated nasal septum and contralateral inferior turbinate hypertrophy, we chose awake fiber optic orotracheal intubation followed by submental intubation. Considering the cervical vertebral fusion, he was carefully positioned during surgery to avoid potential spinal injury. He recovered well and his postoperative course was uneventful.

• 대한소아치과학회지
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• 제28권2호
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• pp.316-322
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• 2001

감각신경아세포종으로 생후 1년 6개월부터 약 1년간 6주기의 화학요법과 생후 1년 6개월에 29회에 걸친 방사선 조사(AP 4540 R+LAT 1080 R : total 5620 R)를 받은 후, 후유증으로 상악치아 및 상악골의 발육장애를 보이는 10세 남아의 임상적, 방사선학적 관찰 및 치료 후 다음과 같은 지견을 얻었기에 보고하는 바이다. 1. 성장중인 어린이에서 악성종양의 치료를 위한 방사선 조사는 연조직 및 경조직(골, 연골, 치아)등의 성장장애를 유발할 수 있으므로 시술 전 충분한 고려가 필요하다. 2. 치배의 손상은 치관 및 치근의 형성장애를 유발하고 이에 따라 치조골의 성장장애가 나타나므로 치근이 없는 치아일지라도 치조골의 흡수를 억제하기 위하여 hawley type의 부분의치 등을 이용하여 잔존시켜야 한다. 3. 향후 성장이 완료된 이후(만 18세 이상)에 무치근 치아 및 무치악 부위에 틀니(denture)나 임플란트 등의 보철수복이 필요할 것으로 여겨진다.

• Journal of Dental Anesthesia and Pain Medicine
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• 제16권2호
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• pp.103-109
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• 2016

Background: One nostril must be selected for nasotracheal intubation. In some cases, structural anomalies within the nasal cavity hinder the insertion of the tube or complications, such as epistaxis, develop. This study examined the possibility of using radiography to select the nostril that would induce fewer complications. Methods: Four hundred and five patients who underwent nasotracheal intubation under general anesthesia were studied. A 7.0-mm internal diameter nasal right angle endotracheal (RAE) tube and 6.5-mm internal diameter nasal RAE tube were inserted into men and women, respectively. Complications were considered to have developed in cases in which insertion of the tube into the nasal cavity failed or epistaxis occurred. The tube was inserted into the other nostril for insertion failures and hemostasis was performed in cases of epistaxis. The degree of nasal septal deviation was determined from posteroanterior skull radiographs or panoramic radiographs; the incidence of complications was compared depending on the direction of the septal deviation and the intubated nostril. Results: The radiographs of 390 patients were readable; 94 had nasal septum deviation. The incidence of complications for cases without nasal septum deviation was 16.9%, that for cases in which the tube was inserted into the nostril on the opposite side of the deviation was 18.5%, and that for cases in which the tube was inserted into the nostril with the deviation was 35.0%, showing a high incidence of complications when intubation is performed through the nostril with septum deviation (chi-square test, P < 0.05 ). Conclusions: Although there were no differences in the incidence rates of complications between intubation through the left nostril and that through the right nostril, radiological findings indicated that incidence of complications significantly increased when the tube was inserted into the nostril with the septum deviation.