• Clinical and Experimental Pediatrics
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• 제46권6호
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• pp.606-609
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• 2003

저자들은 특징적인 진찰 소견과 피부 조직 생검에서 제 I형 교원질 유전자 발현의 감소를 보인 Goltz 증후군 1례를 경험하였기에 보고하는 바이다.

• Journal of Chest Surgery
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• 제20권2호
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• pp.404-410
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• 1987

An 18-day-old male neonate with hypoplastic left heart syndrome underwent surgical intervention by modification of the Norwood procedure on September 23, 1986. Hypoplastic left heart syndrome is a serious congenital cardiac anomaly that has a fatal outlook if left untreated. Included in this anomaly are [1] aortic valve atresia, and hypoplasia of the ascending aorta and aortic arch, [1] mitral valve atresia or hypoplasia, and [3] diminutive or absent left ventricle. Patent ductus arteriosus is essential for any survival, and there is usually a patent foramen ovale. Coarctation of the aorta is frequently associated with the lesion.z With a limited period of cardiopulmonary bypass, deep hypothermia, and circulatory arrest, the ductus arteriosus was excised. The main pulmonary artery was divided immediately below its branches, and the distal stump of the divided pulmonary artery was closed with a pericardial patch. The aortic arch was incised, and a 1 5mm tubular Dacron prosthesis was inserted between the main pulmonary artery and the aortic arch. A 4mm shunt of polytetrafluoroethylene graft was established between the new ascending aorta and the right pulmonary artery to provide controlled pulmonary blood flow. Following rewarming, the heart started to beat regularly, but the patient could not be weaned from cardiopulmonary bypass. At autopsy, the patient was found to have hypoplasia of the aortic tract complex with mitral atresia and aortic atresia. A secundum atrial septal defect was noted. Right atrial and ventricular hypertrophy was present, and the left ventricle was entirely absent. Although unsuccessful in this case report, continuing experience with hypoplastic left heart syndrome will lead to an improvement in result.

• 대한치과마취과학회지
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• 제10권2호
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• pp.190-196
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• 2010

Difficult airway management including difficult intubation, difficult ventilation and difficult mask ventilation is a life threatening issue during anesthesia care. A 23-year-old woman with Treacher Collins syndrome was scheduled for distraction osteogenesis. She had hypoplasia of mandible and malar bone, bilateral deformities of auricles with partial deafness and antimongoloid slant of the palpebral fissures. A 56-year-old woman with mandibular hypoplasia due to childhood trauma was scheduled for distraction osteogenesis. She had a history of difficult intubation. We anticipated a difficult intubation and ventilation. Fiberoptic bronchoscopic guided awake intubation was selected for anesthesia induction. After intravenous injection of midazolam and remifentanil, 10% lidocaine pump spray on the pharyngolarynx with a direct laryngoscope and on the nasal canal. However fiberoptic bronchoscopic guided awake intubation was failed due to severe gag reflex. After intravenous injection of propofol and remifentanil using the target controlled infusion (TCI), mask ventilation was easily performed and, after intravenous injection of vecuronium, fiberoptic bronchoscopic guided intubation was easily performed using a wire reinforced endotracheal tube. The operation was completed successfully without any adverse events.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제34권2호
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• pp.127-132
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• 2012

Maxillary sinus hypoplasia (MSH) is an uncommon clinical disease that represents a persistent decrease in sinus volume, which results from centripetal reaction of the maxillary sinus walls. We present a unilateral MSH case of a 46-year-old male patient with a history of nasal obstruction and headache for 3 years. He had a history of Caldwell Luc operation (CLOP) 10 years ago, and no enophthalmos, hypoglobus or facial asymmetry. After confirming the right diagnosis of MSH, filled with bone in the computed tomography scan, hyperplastic bone was removed by the CLOP approach. The uncinate process and infundibular passage were found to be degenerated and ostium was also examined to be obstructed under endoscopic confirmation. MSH can be mistaken for chronic maxillary sinusitis because of the plain x-ray appearance, so the aggravated state of MSH can be the result of surgeon's misjudgment. With additional literature reviews, this rare experience is first introduced in our Korean oral and maxillofacial surgery field.

• Journal of Korean Neurosurgical Society
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• 제29권3호
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• pp.402-406
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• 2000

The term 'craniovertebral junction' refers to the occipital bone that surrounds the foramen magnum and the atlas and the axis vertebrae. A wide variety of congenital, developmental, and acquired anomalies exist at the craniovertebral junction. The authors present a rare case of the congenital anomaly of the craniovertebral junction consisting of atlantal hypoplasia, atlantal assimilation, and basilar invagination in a 58-year-old male. An occiput-C2 arthrodesis was performed. The clinical and imaging features are described, and the embryology and etiology of this anomaly are discussed.

• 대한치과교정학회지
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• 제52권6호
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• pp.451-460
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• 2022

Moebius syndrome (MBS) is a congenital neurologic disorder that causes cranio-facial abnormalities. It involves paralysis of the VI and VII cranial nerves and causes bilateral or unilateral facial paralysis, eye movement disorder, and deformation of the upper and lower limbs. The orofacial dysfunctions include microstomia, micrognathia, hypotonic mimetic and lip muscles, dental enamel hypoplasia, tongue deformity, open bite or deep overbite, maxillary hypoplasia, high arched palate, mandibular hyperplasia or features indicating mandibular hypoplasia. This case report presents a 7-year-old male patient who was diagnosed with MBS at the age 2 years. The patient displayed typical clinical symptoms and was diagnosed with Class II malocclusion with a large overjet/overbite, tongue deformity and motion limitation, and lip closure incompetency. Treatment was initiated using a removable appliance for left scissor bite correction. After permanent tooth eruption, fixed appliance treatment was performed for correction of the arch width discrepancy and deep overbite. A self-ligation system and wide-width arch form wire were used during the treatment to expand the arch width. After 30 months of phase II treatment, the alignment of the dental arch and stable molar occlusion was achieved. Function and occlusion remained stable with a Class I canine and molar relationship, and a normal overjet/overbite was maintained after 9.4 years of retainer use. In MBS patients, it is important to achieve an accurate early diagnosis, and implement a multidisciplinary treatment approach and long-term retention and follow-up.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제28권2호
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• pp.161-164
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• 2002

Le Fort III 골절단술 후 강성 외장형 신장기를 이용한 중안면부 골신장술은 적은 합병증으로 골편의 많은 전방 이동이 가능하고, 술후에도 신장 축방향을 조절할 수 있는 장점을 가지고 있으며, 견인 철선을 비외측부 안면 피부를 관통하여 견인하는 방법은 견인력이 회전 중심 가까이에 전달되며, 견인장치가 저작시에도 방해가 되지 않는 장점을 가지고 있는 유용한 방법이다. 본과에서는 두개안면부 저성장을 가진 환자에서 Le Fort III 골절단술 후 피부를 관통하여 견인하는 강성 외장형 신장기를 이용하여 적은 합병증으로 매우 심미적인 결과를 얻을 수 있었다. 중안면부의 골신장술을 이용한 치료법은 아직 장기적인 추적 조사가 필요하지만 많은 합병증을 유발하고 골편의 이동량이 제한되는 기존의 골절단술과 소형 금속판을 이용한 골접합술을 대체할 수 있는 유용한 방법으로 생각된다.

• 대한소아치과학회지
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• 제32권4호
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• pp.709-716
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• 2005

구순구개열 환자에서는 이른 시기에 시행된 수술로 인한 구순이나 구개부의 반흔 형성으로 섭식장애나 발음장애를 동반한 상악의 열성장이 나타나게 된다. 때로는 집중적인 교정치료 후에도 심한 상악골 저형성증을 보이며 이러한 경우성장이 완료 된 후에 골이식을 동반한 악교정 수술로 상악골을 전방이동시켜 안모의 개선을 도모하기도 한다. 그러나 이러한 상악골의 전방이동은 연조직의 과도한 신장으로 인한 술후회귀현상, 추가적인 골이식이 필요하다는 한계를 가지고 있다. 골신장술은 이러한 한계점을 극복하는 최신 치료방법으로 구순구개열 환자, 두개골 융합증을 나타내는 환자 등에서 상악골을 포함한 두개 안면골의 개선에 많이 이용되고 있다. 특히 구외장치를 이용하는 골신장술은 골신장기간 중에 견인 방향의 조절이 보다 쉬우며, 충분한 양의 골신장이 가능하여 보다 좋은 결과를 얻을 수 있다. 본 증례는 상악 열성장을 보이는 구순구개열 6세 7개월의 여자 환아로, 변형된 구내 르포씨 1형 골절단술(modified Le Fort I osteotomy) 후 두개골을 고정원으로 이용하는 견고 구외 골신장술을 통해 상악골을 전진시켜 양호한 결과를 얻었기에 이를 보고하는 바이다.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제40권
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• pp.40.1-40.8
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• 2018

Background: A 9-year-old male showed severe defects in midface structures, which resulted in maxillary hypoplasia, ocular hypertelorism, relative mandibular prognathism, and syndactyly. He had been diagnosed as having Apert syndrome and received a surgery of frontal calvaria distraction osteotomy to treat the steep forehead at 6 months old, and a surgery of digital separation to treat severe syndactyly of both hands at 6 years old. Nevertheless, he still showed a turribrachycephalic cranial profile with proptosis, a horizontal groove above supraorbital ridge, and a short nose with bulbous tip. Methods: Fundamental aberrant growth may be associated with the cranial base structure in radiological observation. Results: The Apert syndrome patient had a shorter and thinner nasal septum in panthomogram, PA view, and Waters' view; shorter zygomatico-maxillary width (83.5 mm) in Waters' view; shorter length between the sella and nasion (63.7 mm) on cephalogram; and bigger zygomatic axis angle of the cranial base (118.2°) in basal cranial view than a normal 9-year-old male (94.8 mm, 72.5 mm, 98.1°, respectively). On the other hand, the Apert syndrome patient showed interdigitating calcification of coronal suture similar to that of a normal 30-year-old male in a skull PA view. Conclusion: Taken together, the Apert syndrome patient, 9 years old, showed retarded growth of the anterior cranial base affecting severe midface hypoplasia, which resulted in a hypoplastic nasal septum axis, retruded zygomatic axes, and retarded growth of the maxilla and palate even after frontal calvaria distraction osteotomy 8 years ago. Therefore, it was suggested that the severe midface hypoplasia and dysostotic facial profile of the present Apert syndrome case are closely relevant to the aberrant growth of the anterior cranial base supporting the whole oro-facial and forebrain development.

• 대한치과의사협회지
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• 제24권2호통권201호
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• pp.102-102
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• 1986