• Korean Journal of Head & Neck Oncology
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• v.36 no.1
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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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• v.16 no.1
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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.

• Journal of the korean academy of Pediatric Dentistry
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• v.28 no.1
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• pp.166-170
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• 2001

Rubinstein-Tabyi syndrome(RTS) is a congenital multisystem dysmorphic syndrome with many non-specific features, making diagnosis occasionally difficult. The major features of this syndrome include mental retardation, characteristic facial appearance, short stature, microcephaly, and broad thumbs and halluces. This syndrome was first described by Rubinstein and Tabyi in 1963, and many studies have been continued about this syndrome, but specific pathogenesis of the Rubinstein-Tabyi syndrome phenotype is still not clear. High arched palate, micrognathia and multiple caries etc have been reported in Rubinstein-Tabyi syndrome. In this report, a 6-year and 5-month-old boy visited at our department due to multiple dental caries, who showed broad thumbs, mental and physical development retardation, and characteristic facial appearance including both ptosis and ear deformity. This patient was diagnosed as a Rubinstein-Tabyi syndrome, and treated the multiple dental caries under general anesthesia. This study was aimed to observe the relationship between medical and dental characteristics.

• Korean Journal of Cleft Lip And Palate
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• v.16 no.1
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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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• v.38 no.3
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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.

• Korean Journal of Cleft Lip And Palate
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• v.15 no.2
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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.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.7 no.2
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• pp.123-126
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• 2011

Noonan syndrome (NS) is a developmental disorder characterized by dysmorphic facial features in association with short stature, mental retardation and congenital heart disease. NS may be sporadic or inherited as an autosomal dominant or recessive trait. The children with NS usually have ocular hypertelorism, downslanting palpebral fissures, low-set ears and a webbed neck, chest deformity. In addition, oral features include micrognathia, high arched palate, dental malocclusion, dental anomalies and rarely, cleft palate. The phenotype of NS bears similarities to that of Turner syndrome. However, NS occurs in both males and females with a normal sex chromosome 46, XX and 46, XY constitution. This case presents the intermittent treatment of an 8-year-old girl who was referred from a local clinic for the extraction of supernumerary teeth and treatment of dental caries. The focus of this case report is the oral aspects on NS and particularities of the dental treatment in subjects affected by this genetic disease.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.19 no.3
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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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• v.27 no.2
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• pp.183-187
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• 2005

The coronal approach has been used for over a century by neurosurgeons to access to the anterior cranium. Indications for the coronal approach expanded from use in the correction of congenital skeletal anomalies to applications in acute maxillofacial trauma and secondary deformity correction, oncologic surgery and reconstruction, and esthetic surgery. Complications were such as injury to frontal branch of the facial nerve, motor nerve paralysis, hematoma under flap, trismus, ptosis, epiphora, infection and anterior temporal depression. $Medpor^{(R)}$ is made up of dense polyethylene connected in porous structures. It is easily shapable without collapsing the pores due to it's hardness and tissue growth takes place at the porosities. Based on these advantages, $Medpor^{(R)}$ has been used in augmentation and restoration in craniofacial defect. A temporal depression after the coronal approach for treatment of Le Fort III fracture was successfully reconstruction with $Medpor^{(R)}$ and we report this case with review of literature.

• Korean Journal of Cleft Lip And Palate
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• v.2 no.1_2
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• pp.29-41
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• 1999

Cleft lip and palate is the most frequent congenital facial deformity of the orofacial area. Successful management of patients with cleft lip / palate requires a multidiciplinary approach from birth to adult stage. Coordinated treatment by the cleft palate team is an essential requirement to obtain optimum treatment results. One of the negative effect of the early surgical interventions of lip and palate is a significant incidence of maxillary growth restriction that produces secondary deformities of the jaws and malocclusion that includes congenital missing of lateral incisor, malformed teeth, rotation or ectopic position of upper anterior teeth, and it has been thought due to the resistance of palatal scar tissue. In Orthodontic treatment for cleft lip / palate patients, expansion of upper dental arch or palatal suture is often needed to correct posterior and/or anterior cross bite and align upper teeth. Various appliances such as hyrax, quad-helix, fan-type expansion screw and jointed-fan type expander can be used for palatal expansion. In the orthodontic treatment of the cleft lip / palate patient, we must consider patient age and severity of palatal constriction for proper appliance selection, and must pay special attention to maintain the treatment results.