• The korean journal of orthodontics
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• v.52 no.6
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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.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.22 no.4
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• pp.383-396
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• 2000

Before the advent of early plastic or maxillofacial reconstructive surgery, facial features could only be replaced and mimicked by prosthetic, artificial means. Facial deformity or dysfunction, whether congenital or acquired by trauma or mutilating disease, has long been an area of constant research, development and innovation in many cultures of the world. One of the greatest contributors to the need for maxillofacial prosthetics has been physical conflict and warfare. The use of maxillofacial prostheses is not merely the replacement of a missing or disfigured aspect of the face, but the rehabilitation of that individual back into the society from which they originate. Rehabilitation includes the restoration of the person's self worth and confidence, not just physically but psychologically. In sixteenth century, Ambroise Pare, French military surgeon, first have tried many maxillofacial prosthetics for injured war soldiers with papier-mache, silver, gold and copper. According to patient's demand who lost their maxillofacial figures, maxillofacial personnel have increased and prosthetic's skill have been advanced all of the world. Over the last decade, there has been a very rapid development in technical possibilities to provide patients with facial prostheses retained by skin penetrating implants. This article will present overall history of maxillofacial prostheses and some background information on the materials used from the old world. And to overcome still many limitation of prosthetic, new minds and new ideas for technique and materials should be developed.

• Archives of Plastic Surgery
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• v.38 no.2
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• pp.166-172
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• 2011

Purpose: Facial contouring surgery for improving congenital, acquired deformity and senile change were attempt in past. Recently contouring surgery became more interested subject for improving the flat forehead and temple area. Many synthetic materials were used such as Collagen, silicon, polyacrylamide gel as liquid form and Gore-tex, silicon implant, endotine as solid form. But, these synthetic implants associate complications as foreign body reaction, infection, displacement, granuloma formation and absorption. Auto-fat injection are used for disfigurement of many part of body. We did auto-fat injection for facial contouring of forehead and temple region. Auto-fat injection is suitable without foreign body reaction, displacement, and toxic reaction. Also auto-fat is relatively simple to obtain from patient and less expensive and able to repeat surgeries. Methods: From 2006 to 2009, 150 patients were treated with Auto-fat injection for facial contouring. For follow up, we sent questionnaire to all patients but 110 patients returned answer sheets. The patients consisted of 20 male patients and 90 female patients with an age ranged from 26 to 60, and the mean 43. Fat tissue were injected 6-8 cc in forehead, 7-12 cc in temple area and fat were harvested from thigh and abdomen. Results: In follow up, all patients, showed absorption of injected fat varied degree and except two patients all patients underwent secondary fat injection. Complications were minimal and neuropraxia of facial nerve were recovered. Most of the patients were satisfied with result of procedure, and answered that they recommend same procedure to their friends and will do surgery again. Conclusion: Auto-fat injections were implemented for facial contouring in 150 patients and obtained satisfactory result. Auto-fat injection is relatively easy procedure and applicable widely. Even though, by passing time, some of the injected fats are absorbed, auto-fat injection could be choice of treatment for contouring forehead and temple. With accumulations of cases and development of surgical technique, better result could be expected.

• Journal of Chest Surgery
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• v.22 no.4
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• pp.680-686
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• 1989

Supravalvular aortic stenosis may be defined as an obstructive congenital deformity of the ascending aorta which originates just distal to the level of the origin of the coronary arteries. It may occur in several different anatomical form. Three type have been described; the hourglass, the hypoplastic and the membranous type, each term identifying the gross characteristic of the lesion causing by the aortic obstruction. Non cardiovascular condition commonly associated with supravalvular aortic stenosis are mental retardation, facial anomalies, hypercalcemia, etc. The diagnosis can be established preoperatively by left heart catheterization and selective angiography. Recently, we experienced a case of multiple localized supravalvular aortic stenosis involving, just above the sinus Valsalva and just proximal of the innominate artery. The surgical correction which was performed by a vertical incision across the each narrowing of aorta with replacement of diamond shaped double velour Woven Dacron patch under the CPB. He was discharged without any event.

• Archives of Craniofacial Surgery
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• v.19 no.2
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• pp.131-134
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• 2018

Treacher Collins syndrome is a congenital disorder that is characterized with a wide range of cranio-facial deformities. Zygomatic hypoplasia or aplasia is one of the key features, and surgical reconstruction of the consequent depression on the zygomatic area is deemed necessary by many patients. Various surgical options are available-injectables, alloplastic materials, autologous grafting, and autogenous tissue transfer. It depends on each patient which technique to use. Here, we present a clinical case, in which bilateral free groin flaps were adopted in attempt to resolve the remnant aesthetic deformity associated with zygomatic depression, despite a series of previous surgical efforts, in a 25-year-old Treacher Collins syndrome male patient.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.33 no.4
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• pp.297-306
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• 2007

Distraction osteogenesis was pioneered by Ilizarov in the treatment of injured extremities. Its subsequent application to Oral and Maxillofacial Surgery has opened a new chapter in the treatment of facial deformity. Careful monitoring of the period of distraction and consolidation of the callus is important and has been well described. Complications, such as infection, haematoma and premature ossification, are difficult to diagnose and can compromise the outcome after the surgery. Too slow rate of distraction results in premature fusion of cortices and too rapid information of bone 'cyst' within the callus distraction. I experienced 2 patients of congenital unilateral mandibular hypoplasia, so called Hemifacial Microsomia. After distraction surgery, I evaluated callus formation of mandibular distraction with Ultrasound Sonography during distraction and consolidation period. Plain radiography, although it enables accurate measurement of the distraction gap, did not give sufficient detail to allow assessment of early stages of bone formation, But, ultrasound monitoring could enable continuous monitoring of the distraction gap without exposure to ionizing radiation and allow detection of fine detail, which may influence manipulation of the callus.

• Archives of Plastic Surgery
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• v.33 no.2
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• pp.237-240
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• 2006

The simultaneous correction of the hypertelorism and exophthalmos combined with craniosynostosis is very rarely performed operative procedures in the world. The craniosynostosis is the congenital anomaly that designates premature fusion of one or more sutures in either cranial vault or cranial base. Hypertelorism is not a distinct clinical syndrome in itself, but is a physical finding secondary to facial and cranial maldevelopment and it is defined as a increase in the distance between the medial orbital walls. Exophthalmos can occur following the decrease in the size of the orbit in patients with developmental skeletal disorders such as craniofacial synostosis. The authors experienced 9-year-old male patient, who has complex cranio-facial abnormality. The craniosynostosis was oxycephaly type and primary fronto-orbital advancement surgery had been performed in other hospital. The abnormal cranial vault combined with hypertelorism and exophthalmos due to maldeveloped both orbital walls. Surgical correction was obtained by various cranio-fronto-orbital remodeling technique such as calvarial bone craniotomy, fronto-orbital advancement, paramedian resection, medial canthopexy, Tessier-Wolfe three wall orbital expansions. We achieved a quite satisfactory result both functionally and aesthetically in a complex cranio-facial deformity patient by combination and modification of previously developed various cranio-facial plasty technique and hereby report the case with brief discussion and review of literature.

• Korean Journal of Cleft Lip And Palate
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• v.4 no.2
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• pp.9-18
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• 2001

Cleft lip and palate is the most frequent congenital facial deformity of the orofacial area. Successful management of patients with cleft lip and palate requires a multidisciplinary approach from birth to adult stage. The early surgical intervention of lip and palate induces a significant incidence of maxillary growth restriction that produces secondary deformities of the jaws, and the severity of the skeletal discrepancies tends to increase with growth. The early growth modification treatment to utilize the patient's growth potential is necessary in the cleft lip and palate patients, and we must consider not only the existing skeletal discrepancies but the residual growth amount and the direction. However, once we have obtained good results with orthopedic treatment in mixed dentition stage, we must pay special attention to maintain the treatment results because of high relapse tendencies and the alterations of jaw relationships due to residual growth.

• Journal of Genetic Medicine
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• v.13 no.1
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• pp.31-35
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• 2016

Antley-Bixler syndrome (ABS) is a rare form of syndromic craniosynostosis with additional systemic synostosis, including radiohumeral or radioulnar synostosis. Another characteristic feature of ABS is mid-facial hypoplasia that leads to airway narrowing after birth. ABS is associated with mutations in the FGFR2 and POR genes. Patients with POR mutations present with either skeletal manifestations or congenital adrenal hyperplasia with ambiguous genitalia. We report here two cases of ABS caused by mutations in FGFR2 and POR. Although the patients had craniosynostosis and radiohumeral synostosis in common and cranioplasty was performed in both cases, the male with POR mutations showed an elevated level of $17{\alpha}$-hydroxyprogesterone during newborn screening and was diagnosed with congenital adrenal hyperplasia by adrenocorticotropic hormone stimulation. This patient has been treated with hydrocortisone and fludrocortisone. He had no ambiguous genitalia but had bilateral cryptorchidism. On the other hand, the female with the FGFR2 mutation showed severe clinical manifestations: upper airway narrowing leading to tracheostomy, kyphosis of the cervical spine, and coccyx deformity. ABS shows locus heterogeneity, and mutations in two different genes can cause similar craniofacial and skeletal phenotypes. Because the long-term outcomes and inheritance patterns of the disease differ markedly, depending on the causative mutation, early molecular genetic testing is helpful.

• The korean journal of orthodontics
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• v.46 no.6
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• pp.395-408
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• 2016

We report a case involving a young female patient with severe mandibular retrognathism accompanied by mandibular condylar deformity that was effectively treated with Le Fort I osteotomy and two genioplasty procedures. At 9 years and 9 months of age, she was diagnosed with Angle Class III malocclusion, a skeletal Class II jaw relationship, an anterior crossbite, congenital absence of some teeth, and a left-sided cleft lip and palate. Although the anterior crossbite and narrow maxillary arch were corrected by interceptive orthodontic treatment, severe mandibular hypogrowth resulted in unexpectedly severe mandibular retrognathism after growth completion. Moreover, bilateral condylar deformities were observed, and we suspected progressive condylar resorption (PCR). There was a high risk of further condylar resorption with mandibular advancement surgery; therefore, Le Fort I osteotomy with two genioplasty procedures was performed to achieve counterclockwise rotation of the mandible and avoid ingravescence of the condylar deformities. The total duration of active treatment was 42 months. The maxilla was impacted by 7.0 mm and 5.0 mm in the incisor and molar regions, respectively, while the pogonion was advanced by 18.0 mm. This significantly resolved both skeletal disharmony and malocclusion. Furthermore, the hyoid bone was advanced, the pharyngeal airway space was increased, and the morphology of the mandibular condyle was maintained. At the 30-month follow-up examination, the patient exhibited a satisfactory facial profile. The findings from our case suggest that severe mandibular retrognathism with condylar deformities can be effectively treated without surgical mandibular advancement, thus decreasing the risk of PCR.