• 대한소아치과학회지
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• 제36권1호
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• pp.133-138
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• 2009

Crouzon 증후군은 1912년 프랑스의 신경학자 Crouzon에 의해 처음 알려진 질환으로 10q25-10q26 염색체에 위치한 FGFR2 유전자의 돌연변이에 의해 발생되며 발생률은 25,000명당 1명 꼴로 상염색체 우성 유전 경향을 보이는 유전적 질환이다. 본 증례는 서울대학교 치과병원 소아치과에 내원한 9세의 Crouzon 증후군 환아에 대한 것으로 특징적으로 두개봉합이 조기에 폐쇄되는 두개골유합증(craniosynostosis)으로 인한 두개안면골 이상, 안구돌출증, 상악골 저성장 및 상대적인 하악전 돌증을 나타내었다. 이에 Crouzon 증후군의 의과 및 치과적 소견을 살펴보고, 이에 대해 과거의 문헌을 고찰하여 다소의 지견을 얻었기에 보고하는 바이다.

• 대한소아치과학회지
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• 제11권1호
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• pp.249-254
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• 1984

This is a case report of Crouzon's disease as a kind of craniofacial dysostosis by premature closure of unilateral coronal suture, showed plagiocephalic skull. 5-year-old boy was visited for the treatment of dental caries and oral examination. Physical examination showed hypertelorism, internal strabismus, and saddle nose. Intraoral radiographs showed congenital missing of upper right and left deciduous and permanent lateral incisors. Cephalometric analysis showed shortening the posterior cranial base length, clockwise growth pattern and class III and open bite tendency. Posterior-anterior and submentovertex view showed multiple radiolucencies-digital impression on inner surface of cranial vault. Maxillo-facial and neuro-surgical treatment was required to improvement of facial esthetics and optic complications. Continuous examination was needed to the growth and development.

• Archives of Plastic Surgery
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• 제34권1호
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• pp.123-127
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• 2007

Purpose: Rigid external distraction(RED) is a highly effective technique for correction of maxillary hypoplasia in patients with cleft or syndromic craniosynostosis. Despite many advantages of RED, it also has the problem of relapse as the conventional advancement surgery. Bicoronal approach, that is the common approach to gain access to the craniofacial skeleton, had some morbidity, such as hair loss, sensory loss, wide scar and temporal hollowing. We present our clinical experience of RED distraction with minimal invasive approach and early rigid fixation to overcome these disadvantages. Methods: A 27-year-old female patient with Crouzon's disease underwent Le Fort III osteotomy and RED device application through the minimal invasive direct skin incisions. After the latent period of 5 days, distraction was undertaken until proper convexity and advancement were obtained. During the rigid retention period, inflammation occurred on the right cheek, and proper conservative managements were done including continuous irrigation. To maintain the stability of distraction, early rigid fixation was undertaken on the osteotomy sites through another skin incisions. Preoperative and postoperative orthodontic treatments were performed. Serial photographs and cephalometric radiographs were obtained preoperatively, after distraction and 6 months after distraction. Results: The cephalometric analysis demonstrated postoperatively significant advancement of the maxilla and improvement of facial convexity. After 6-month follow-up period, the maxilla was stable in the sagittal plane and no relapse was found. Facial scars were not noticeable and other deformity and morbidity did not occur. Conclusion: This effective and stable technique will be a good alternative for the patients who need large amount of distraction and for adult patients with severe maxillary hypoplasia or syndromic craniosynostosis.

• Archives of Plastic Surgery
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• 제38권4호
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• pp.516-518
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• 2011

Purpose: The prevalence of antibiotic-resistant Streptococcus pneumoniae meningitis has increased worldwide. There are some reports about postoperative antibiotic-resistant Streptococcus pneumoniae infection after craniofacial surgery, but, there is no report in Korea. We present a report on the treatment of postoperative multidrug-resistant Streptococcus pneumoniae (MRSP) meningitis and sepsis after craniofacial surgery based on our experience. Methods: The patient was a 7-year-old boy with Crouzon's disease who was treated by fronto-orbital bar advancement. Intraoperatively, frontal sinus opening was seen during osteotomy which was covered with forehead galeopericranial flap. MRSP meningitis was diagnosed after the surgery, he was treated with intravenous vancomycin, meropenem, and levofloxacin. Results: The patient was treated successfully after 3 weeks of intravenous antibiotics treatment. During the 8 month follow-up period, there was no neurologic sequelae. Conclusion: Postoperative infection after craniofacial surgery is an important phenomenon that needs immediate recognition. Prevention, early diagnosis, and treatment immediate after onset are important as countermeasures against postoperative drug-resistant bacterial infection. To prevent adverse outcome and reoperation, proper antibiotics treatment should be performed.