• Clinical and Experimental Pediatrics
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• 제59권1호
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• pp.16-23
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• 2016

Purpose: The 1p36 deletion syndrome is a microdeletion syndrome characterized by developmental delays/intellectual disability, craniofacial dysmorphism, and other congenital anomalies. To date, many cases of this syndrome have been reported worldwide. However, cases with this syndrome have not been reported in Korean populations anywhere. This study was performed to report the clinical and molecular characteristics of five Korean patients with the 1p36 deletion syndrome. Methods: The clinical characteristics of the 5 patients were reviewed. Karyotyping and multiplex ligation-dependent probe amplification (MLPA) analyses were performed for genetic diagnoses. Results: All 5 patients had typical dysmorphic features including frontal bossing, flat right parietal bone, low-set ears, straight eyebrows, down-slanting palpebral fissure, hypotelorism, flat nasal roots, midface hypoplasia, pointed chins, small lips, and variable degrees of developmental delay. Each patient had multiple and variable anomalies such as a congenital heart defect including ventricular septal defect, atrial septal defect, and patent duct arteriosus, ventriculomegaly, cryptorchism, or hearing loss. Karyotyping revealed the 1p36 deletion in only 1 patient, although it was confirmed in all 5 patients by MLPA analyses. Conclusion: All the patients had the typical features of 1p36 deletion. These hallmarks can be used to identify other patients with this condition in their early years in order to provide more appropriate care.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제41권
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• pp.3.1-3.9
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• 2019

Background: The infratemporal fossa (ITF) is an anatomical lateral skull base space composed by the zygoma, temporal, and the greater wing of the sphenoid bone. Due to its difficult approach, surgical intervention at the ITF has remained a heavy burden to surgeons. The aim of this article is to review basic skull base approaches and ITF structures and to avoid severe complications based on the accurate surgical knowledge. Methods: A search of the recent literature using MEDLINE (PubMed), Embase, Cochrane Library, and other online tools was executed using the following keyword combinations: infratemporal fossa, subtemporal fossa, transzygomatic approach, orbitozygomatic approach, transmaxillary approach, facial translocation approach, midface degloving, zygomatico-transmandibular approach, and lateral skull base. Aside from our Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) trial, there have been very few randomized controlled trials. The search data for this review are summarized based on the authors' diverse clinical experiences. Results: We divided our results based on representative skull base approaches and the anatomy of the ITF. Basic approaches to the ITF include endoscopic endonasal, transzygomatic, orbitozygomatic, zygomatico-transmandibular, transmaxillary, facial translocation, and the midfacial degloving approach. The borders and inner structures of the ITF (with basic lateral skull base dissection schemes) are summarized, and the modified zygomatico-transmandibular approach (ZTMA) is described in detail. Conclusions: An anatomical basic knowledge would be required for the appropriate management of the ITF pathology for diverse specialized doctors, including maxillofacial, plastic, and vascular surgeons. The ITF approach, in conjunction with the application of microsurgical techniques and improved perioperative care, has permitted significant advances and successful curative outcomes for patients having malignancy in ITF.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제34권5호
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• pp.367-375
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• 2012

Midfacial reconstruction following resection of extensive malignant oral cavity tumors constitutes a challenging problems for reconstructive surgeons. Rectus abdominis muscle free flap (RAMFF) can be considered as the optimal reconstructive option in this case, because this flap has some advantages including consistent deep inferior epigastric artery anatomy, easy to dissect with well defined skin boundaries, acceptable donor site morbidity and the ability to perform simultaneous flap harvest with oral cancer ablation surgery. The rectus abdominis muscle forms an important part of the anterior abdominal wall and flexes the vertebral column, which is a long strap-like muscle divided transversely by three tendinous intersections, fibrous bands which are adherent to the anterior rectus sheath, which is thickly enclosed by the rectus sheath, except for the posterior part below the arcuate line that is usually located midway between the umbilicus and symphysis pubis. Below the arcuate line, this muscle lies in direct contact with the transversalis fascia and parietal peritoneum. For the better understanding of RAMFF as a routine reconstructive procedure in oral and maxillofacial surgery, the constant anatomical findings muse be learned and memorized by the young doctors in the course of the special curriculum periods for the Korean national board of oral and maxillofacial surgery. This review article will discuss the anatomical basis of RAMFF with Korean language.

• 대한치과교정학회지
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• 제50권5호
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• pp.346-355
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• 2020

The treatment of skeletal Class III malocclusion in adolescents is challenging. Maxillary protraction, particularly that using bone anchorage, has been proven to be an effective method for the stimulation of maxillary growth. However, the conventional procedure, which involves the surgical implantation of mini-plates, is traumatic and associated with a high risk. Three-dimensional (3D) digital technology offers the possibility of individualized treatment. Customized mini-plates can be designed according to the shape of the maxillary surface and the positions of the roots on cone-beam computed tomography scans; this reduces both the surgical risk and patient trauma. Here we report a case involving a 12-year-old adolescent girl with skeletal Class III malocclusion and midface deficiency that was treated in two phases. In phase 1, rapid maxillary expansion and protraction were performed using 3D-printed mini-plates for anchorage. The mini-plates exhibited better adaptation to the bone contour, and titanium screw implantation was safer because of the customized design. The orthopedic force applied to each mini-plate was approximately 400-500 g, and the plates remained stable during the maxillary protraction process, which exhibited efficacious orthopedic effects and significantly improved the facial profile and esthetics. In phase 2, fixed appliances were used for alignment and leveling of the maxillary and mandibular dentitions. The complete two-phase treatment lasted for 24 months. After 48 months of retention, the treatment outcomes remained stable.

• 대한두개안면성형외과학회지
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• 제19권2호
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• pp.102-107
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• 2018

Background: Nasal fracture and orbital blowout fracture often occur concurrently in cases of midface blunt trauma. Generally, these multiple fractures treatment is surgery, and typically, the nasal bone and orbit are operated on separately. However, we have found that utilizing a transconjunctival approach in patients with concurrent nasal bone fracture and orbital blowout fracture is a useful method. Methods: The participants in the present study included 33 patients who visited the Plastic Surgery outpatient department between March 2014 and March 2017 and underwent surgery for nasal fracture and orbital blowout fracture. We assessed patients' and doctors' satisfaction with surgical outcomes after indirect open reduction via a transconjunctival approach for the treatment of nasal bone fracture with associated orbital blowout fracture. Results: According to the satisfaction scores, both patients and doctors were satisfied with transconjunctival approach. Conclusion: We presented here that our method enables simultaneous operation of nasal fracture accompanied by orbital blowout fracture, rather than treating the two fractures separately, and it allows precise reduction of the nasal fracture by direct visualization of the fracture site without any additional incisions or difficult surgical techniques. Also, by preventing the use of excessive force during reduction, this method can minimize damage to the nasal mucosa, thereby reducing the incidence of nasal bleeding.

• Archives of Plastic Surgery
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• 제33권3호
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• pp.335-340
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• 2006

Lower palpebral bags and tear trough grooves are the common signs of aging. Weakness of the orbital septum, atrophy of the orbicularis oculi muscle, slackness and laxity of the lower lid skin are the main causes of these aging changes. Tear trough groove with skeletonization of the lower orbital rim can not be corrected easily by the conventional lower blepharoplasty, but the augmentation of the volume, can be an effective method. Lower lids provide a well vascularized recipient site, which is vital for proper grafting of fat to occur. We treated tear trough groove with free fat graft of surgically removed orbital fat. Surgical excision of the fat pad and septal reinforcement with free fat graft below the levator labii superioris alaeque nasi muscle were performed to correct tear trough groove. This technique was performed in 29 consecutive individuals from October, 2001 to January, 2005. Palpebral bags and tear trough groove were corrected concomitantly and obtained a youthful attractive midface with no secondary hollowness of lower lid and irregularity. The overall results obtained were satisfactory without any complications. Recurrence of tear trough deformity due to grafted fat absorption was not noted during the 7 months of the follow-up period. The authors conclude that the procedure is safe and effective in selected patients.

• Archives of Plastic Surgery
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• 제38권3호
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• pp.273-278
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• 2011

Purpose: It has always been an aspiration for Asians to look more balanced and feminine, considering their facial features regarding relatively flat midface with marked prominences of the zygoma. Many studies have been dealt in this subject. However, the authors would like to emphasize the concept and introduce the technique of repositioning of the malar complex to a cosmetically beneficial point and stationing it on proper position by fixation on zygoma body and arch. Methods: From January 1998 to December 2007, this method was performed in 50 patients of mild to moderate prominence and malposition of the malar complex. A simplified technique of lateral orbital osteotomy and oblique osteotomy on zygomatic arch through intraoral and preauricular incision was developed. Then, liberal malar complex can be moved to a supero-posterior direction and repositioned to a more cosmetically beneficial point. To maintain the stationed position and to protect from vector affected by the attached masticating muscle to zygomatc bone, fixation was done on both zygoma body and arch. Results: We have obtained satisfactory results using this procedure without any observable complications. The advantages of this procedure are proper exposure, inconspicuous scar, safe, more natural contour, improved stability, and shorter healing time. Conclusion: The authors suggest that reduction malarplasty should be approached with underlying concept of repositioning and fixation. In mild moderate malar prominent cases, our technique will provide with maintenance of aesthetic concept, equal to the malar reduction performed under coronal approach and provide with more natural facial contour with stability even with less invasive surgical approach.

• Archives of Plastic Surgery
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• 제38권3호
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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.

• Clinical and Experimental Pediatrics
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• 제59권sup1호
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• pp.14-18
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• 2016

Pediatric epilepsy can be caused by various conditions, including specific syndromes. 1p36 deletion syndrome is reported in 1 in 5,000-10,000 newborns, and its characteristic clinical features include developmental delay, mental retardation, hypotonia, congenital heart defects, seizure, and facial dysmorphism. However, detection of the terminal deletion in chromosome 1p by conventional G-banded karyotyping is difficult. Here we present a case of epilepsy with profound developmental delay and characteristic phenotypes. A 7-year-and 6-month-old boy experienced afebrile generalized seizure at the age of 5 years and 3 months. He had recurrent febrile seizures since 12 months of age and showed severe global developmental delay, remarkable hypotonia, short stature, and dysmorphic features such as microcephaly; small, low-set ears; dark, straight eyebrows; deep-set eyes; flat nasal bridge; midface hypoplasia; and a small, pointed chin. Previous diagnostic work-up, including conventional chromosomal analysis, revealed no definite causes. However, array-comparative genomic hybridization analysis revealed 1p36 deletion syndrome with a 9.15-Mb copy loss of the 1p36.33-1p36.22 region, and fluorescence in situ hybridization analysis (FISH) confirmed this diagnosis. This case highlights the need to consider detailed chromosomal study for patients with delayed development and epilepsy. Furthermore, 1p36 deletion syndrome should be considered for patients presenting seizure and moderate-to-severe developmental delay, particularly if the patient exhibits dysmorphic features, short stature, and hypotonia.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제43권6호
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• pp.407-414
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• 2017

Objectives: The study was designed to evaluate the efficacy of performing a second, repeat anterior maxillary distraction (AMD) to treat residual cleft maxillary hypoplasia. Materials and Methods: Five patients between the ages of 12 to 15 years with a history of AMD and with residual cleft maxillary hypoplasia were included in the study. Inclusion was irrespective of gender, type of cleft lip and palate, and the amount of advancement needed. Repeat AMD was executed in these patients 4 to 5 years after the primary AMD procedure to correct the cleft maxillary hypoplasia that had developed since the initial procedure. Orthopantomogram (OPG) and lateral cephalograms were taken for evaluation preoperatively, immediately after distraction, after consolidation, and one year postoperatively. The data obtained was tabulated and a Mann Whitney U-test was used for statistical comparisons. Results: At the time of presentation, a residual maxillary hypoplasia was observed with a well maintained distraction gap on the OPG which ruled out the occurrence of a relapse. Favorable movement of the segments without any resistance was seen in all patients. Mean maxillary advancement of 10.56 mm was achieved at repeat AMD. Statistically significant increases in midfacial length, SNA angle, and nasion perpendicular to point A distance was achieved (P=0.012, P=0.011, and P=0.012, respectively). Good profile was achieved for all patients. Minimal transient complications, for example anterior open bite and bleeding episodes, were managed. Conclusion: Addressing the problem of cleft maxillary hypoplasia at an early age (12-15 years) is beneficial for the child. Residual hypoplasia may develop in some patients, which may require additional corrective procedures. The results of our study show that AMD can be repeated when residual deformity develops with the previous procedure having no negative impact on the results of the repeat procedure.