• The Journal of Korean Academy of Prosthodontics
• /
• v.54 no.2
• /
• pp.167-171
• /
• 2016

Patients undergone partial maxillectomy experience post-operative masticatory, phonetic, and swallowing difficulties. They also encounter social and psychological challenges due to changes in their facial appearances. Thus, functional and esthetic recovery through maxillofacial prosthesis becomes significant for these patients. The objective of an appropriate obturator is to restore palate and improve phonetic and swallowing ability by separating the oral cavity, nasal cavity, maxillary sinus, and nasopharynx. In this case report, an obturator was fabricated for a patient who had partial resection from the maxillary posterior region to the pharynx due to squamous cell carcinoma. The purpose of this case study is to describe the results because the patient was successfully improved both functionally and esthetically.

• Journal of the korean academy of Pediatric Dentistry
• /
• v.27 no.2
• /
• pp.222-228
• /
• 2000

Ectodermal dysplasia is a hereditary disease characterized by a congenital dysplasia of one or more ectodermal structures and their accessory appendages. At least 120 subtypes of ectodermal dysplasia have been reported. The disease is usually transmitted as an X-linked recessive trait in which the gene is carried by the female and manifested in the male. Hypohidrotic ectodermal dysplasia is manifested as a triad of defects that include hypohidrosis, hypotrichosis and hypodontia. The characteristic facial features consist of asteatosis, onchodysplasia, sparse and fine blond hair, prominent forehead, a depressed nasal bridge, thick everted lips. The patient may suffer from dry skin, hyperthermia and unexplained high fever as a result of the deficiency of sweat glands. This case report presents detailed procedures of rehabilitating functional and esthetic defect of a 6-year-old boy with hypohidrotic ectodermal dysplasia along with the review of relevant literatures.

• Maxillofacial Plastic and Reconstructive Surgery
• /
• v.21 no.4
• /
• pp.401-406
• /
• 1999

Secondary deformities of the lip and nose in individuals with repaired unilateral and bilateral clefts may vary in severity, depending on the state of the original defect, the care taken in the initial surgical procedure, the pattern of the patient's facial growth, and the effectiveness of interceptive orthodontic technique. Because each patient has a unique combination of deformities, their surgical reconstruction usually requires the modification and combination of several surgical techniques. Residual lip deformities after primary repair may be esthetic or functional and include scars, skin shortage or excess(vertical and transverse), orbicularis oris muscle malposition or diastasis. The key to accurate repair of secondary cleft lip deformities is a precise diagnosis. This requires observation of the patient in animation and repose. The quality of the scar is not the only factor determining the overall appearance of the lip. Observing the patient in the animated position is critical to assess muscular function. Factors that require precise analysis include lip length, the appearance of the Cupid's bow and philtrum, and nasal symmetry. Only after this detailed analysis can a decision be made as to wether a major or minor deformity exists. We report successful cases using various techniques for the secondary lip deformities.

• Proceedings of the KOR-BRONCHOESO Conference
• /
• 1983.05a
• /
• pp.14.3-14
• /
• 1983

In recent years there has been considerable interest in reconstructive surgery of the trachea for cervical tracheal stenosis developed by complication of endotracheal intubation or tracheotomy, or trauma of the neck. The methods used to reconstruct the tracheal defects can be repaired with end-to - end anastomosis, cervical flaps, and autogenous graft materials. Since Grillo had undertaken tracheal reconstruction after circumferential resection in dogs, resection and end - to - end anastomosis was used in cases of circumferential stenosis. And, costal, nasal septal and auricular cartilage have been used for the autogenous graft materials. Since Caputo and Consiglio had undergone tracheoplasty with auricular cartilage, Morgenstein reported successful repair of a tracheal defect with a composite postauricular cartilage graft. The advantages of the auricular cartilage graft are its easy accessibility, availability and familiarity to the otolaryngologist. In past 2 years, We performed the tracheoplasty with auricular cartilage graft and end- to end an astomosis after segmental resection in 5 patients who had suffered from tracheal stenosis. And we obtained good results. So, we reported the cases with review of the literatures.

• Archives of Craniofacial Surgery
• /
• v.22 no.6
• /
• pp.296-302
• /
• 2021

Background: The reconstruction of large full-thickness alar defects requires complex surgical procedures that are usually performed in two stages, with concomitant disadvantages in terms of patient trauma, surgical risk, and cost. This study presents a functional folded nasolabial island flap (FNIF) that can be used to repair large-sized full-thickness alar defects in a straightforward manner. Methods: This retrospective study included seven patients who received a FNIF for a full-thickness alar defect between January 2007 and December 2020. The FNIF is different from the conventional nasolabial flap in that it is folded and twisted to achieve nostril reconstruction with a satisfactory three-dimensional mucosal lining in a single stage. The cosmetic and functional results of FNIF were evaluated by both patients and physicians. Results: The age ranged from 51 to 82 years (mean, 65.6 years). The causes of the defects were squamous cell carcinoma, basal cell carcinoma, and trigeminal trophic syndrome. The nostril lining did not collapse, there was no hypertrophic scarring, and air movement through the nostrils on the flap side was normal. Overall, FNIF produced excellent aesthetic and functional outcomes, with minimal patient discomfort. There were no postoperative complications. Conclusion: Compared with existing reconstruction methods for large full-thickness alar defects, FNIF can easily achieve aesthetic and functional success in a single-stage procedure. It provides satisfactory results for both the patient and the surgeon.

• Journal of Dental Rehabilitation and Applied Science
• /
• v.39 no.4
• /
• pp.229-236
• /
• 2023

Maxillary bone defects may follow surgical treatment of benign and malignant tumors, trauma, and infection. Palatal defects often lead to problems with swallowing and pronunciation from the leakage of air into the nasal cavity and sinus. Obturators have been commonly used to solve these problems, but long-term use of the device may cause irritation of the oral mucosa or damage to the abutment teeth. Utilizing implants in the edentulous area for the fabrication of the obturators has gained attention. This case report describes a patient, who had undergone partial resection of the maxilla due to adenocarcinoma, in need of a new obturator after losing abutment teeth after long-term use of the previous obturator. Implants were placed in strategic locations, and an implant-retained maxillary obturator was fabricated, showing satisfactory results in the rehabilitation of multiple aspects, including palatal defect, masticatory function, swallowing, pronunciation, and aesthetics.

• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.17 no.3
• /
• pp.103-108
• /
• 2017

Kabuki syndrome is a rare congenital disorder that causes multiple birth defects and mental retardation. Mutation of the lysine methyltransferase 2D (KMT2D) gene is the primary cause of Kabuki syndrome. We report a 4-year-old Korean girl diagnosed with Kabuki syndrome based on distinctive facial features (eversion of the lower lateral eyelid, arched eyebrows, depressed nasal tip, prominent ears), skeletal anomalies, short stature, and molecular analysis, which revealed a novel frameshift mutation in the KMT2D gene. A 4-year-old patient had a past history of congenital cardiac malformations (coarctation of the aorta, ventricular septal defect, atrial septal defect, patent ductus arteriosus), subclinical hypothyroidism and dysmorphic features at birth including webbed neck, short fingers, high arched palate, micrognathia and horseshoe kidney. She showed unique facial features such as a long palpebral fissure, long eyelashes, arched eyebrows with sparseness of the lateral third, broad nasal root, anteverted ears, and small mouth. Her facial features suggested Kabuki syndrome, and genetic analysis discovered a novel heterozygous frameshift mutation (c.4379dup, p.Leu1461Thrfs*30) in exon 15 of the KMT2D gene. The diagnosis of our 4-year-old patient was made through thorough physical examination and history taking, and genetic testing. It is challenging to diagnose patients with Kabuki syndrome at birth, since the characteristic facial features are expressed gradually during growth. Clinical suspicion aroused by regular follow-ups may lead to earlier diagnosis and interventions.

• Journal of Dental Rehabilitation and Applied Science
• /
• v.25 no.4
• /
• pp.329-336
• /
• 2009

In the case of congenital malformation, acquired trauma, or maxillectomy by neoplasm, the defect in maxilla is combined with nasal cavity, maxillary sinus and oral cavity. This results in abnormal function in pronunciation, mastication, and swallowing. In this situation, the purposes of prosthodontics are also applied. The purposes of obturator for acquired maxillary deficiency patients are restoring pronunciation, mastication, and swallowing, and make patients comfortable and esthetic satisfaction. Reducing weight of the obturator makes edentulous ridge heathy and functional. It also improves patients' comfort. Hollow obturator also reduces unnecessary stress because improves physiologic function, as it weights less than 6.55-33.06% compare to usual one. This case presents the double-processing method for maxillary obturator in the patient treated hemi-maxillectomy due to malignancy neoplasm. We report this case because the patient acquired functional and esthetic satisfaction from double-processing obturator.

• Clinical and Experimental Pediatrics
• /
• v.50 no.2
• /
• pp.213-217
• /
• 2007

Pompe disease is a genetic disorder caused by a deficiency of acid ${\alpha}$-glucosidase (GAA). Infantile onset Pompe disease is uniformly lethal. Affected infants generally present in the first few months of life with hypotonia, generalized muscle weakness, and a hypertrophic cardiomyopathy, which is rapidly followed by death, usually by the age of one. The late-onset form is characterized less severe symptoms and prognosis. Therapy for Pompe disease is intended to directly address the underlying metabolic defect via intravenous infusions of recombinant human GAA to replace the missing enzyme. We report a case of atypical infantile-onset Pompe disease that presented symptoms in infancy but had less severe clinical manifestations and improved after GAA enzyme replacement ($Myozyme^{(R)}$, Genzyme Co., MA, USA) therapy. It is very important that pediatricians become aware of signs and symptoms of Pompe disease, such as a nasal voice or a waddling gait at an early stage so that these patients can benefit from appropriate GAA replacement therapy as soon as possible.

• Journal of The Korean Ophthalmological Society
• /
• v.54 no.9
• /
• pp.1445-1451
• /
• 2013

Purpose: To report long-term changes in the average retinal nerve fiber layer (RNFL) thickness in 2 patients who had intravitreal bevacizumab (IVB) injection for diabetic papillopathy. Case summary: A 36-year-old patient with diabetes complained of decreased visual acuity (20/200) in the right eye. The fundus examination showed optic disc swelling in both eyes. The average RNFL thickness based on optical coherence tomography (OCT) increased to $278{\mu}m$ and Goldmann perimetry showed nasal visual field defect in the right eye. The IVB was injected into the right eye. Three weeks after the IVB injection, RNFL thickness decreased to $135{\mu}m$ and visual acuity improved to 20/25 in the right eye. However, RNFL thickness increased from 126 to $207{\mu}m$ and visual acuity decreased to 20/32 in the left eye. Thus, IVB was injected into the left eye. In week 3, RNFL thickness decreased to $147{\mu}m$ and visual acuity improved to 20/20 in the left eye. At 12 months after IVB injection, RNFL thickness was $87{\mu}m$ in the right eye and $109{\mu}m$ in the left eye. A 57-year-old patient with diabetes complained of decreased visual acuity (20/200) and showed optic disc swelling in the right eye. The average RNFL thickness increased to $252{\mu}m$ and Goldmann perimetry showed an enlarged blind spot in the right eye. IVB was injected into the right eye. After 3 weeks, RNFL thickness decreased to $136{\mu}m$ and visual acuity improved to 20/70 in the right eye. Six months after IVB injection, RNFL thickness was $83{\mu}m$ in the right eye. Conclusions: Visual acuity progressively improved within 3 weeks and RNFL thickness measured by spectral domain OCT showed progressive thickness reduction in 2 cases of diabetic papillopathy patients who had IVB injections.