• Archives of Craniofacial Surgery
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• 제20권3호
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• pp.207-211
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• 2019

Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is a rare subtype of primary cutaneous lymphoma with a favorable prognosis. Primary cutaneous CD30+ lymphoproliferative disorders, which include C-ALCL and lymphomatoid papulosis, are the second most common group of cutaneous T-cell lymphomas. C-ALCL is comprised of large cells with anaplastic, pleomorphic, or immunoblastic cytomorphology, and indeed, more than 75% of the tumor cells express the CD30 antigen. C-ALCL clinically presents with solitary or localized reddish-brown nodules or tumors, and sometimes indurated papules, and they may be with ulceration covering with dark eschar. Multifocal lesions are seen in 20% of the patients. Extracutaneous dissemination, which mainly involves the regional lymph nodes, occurs in 10% of patients. A 69-year-old man noticed a mild elevated cutaneous lesion containing central ulceration covering with brownish black necrotic tissue on the right lower lip, and the lesion was surgically removed. After the first operation, another skin lesion was developed and the histological examination confirmed the diagnosis, C-ALCL. Eight specimens were excised during the 7-month follow-up period. The patient started the treatment with low-dose oral methotrexate (15 mg/wk) and there was no recurrence for 11 months.

• Childhood Kidney Diseases
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• 제7권2호
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• pp.234-238
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• 2003

Fanconi syndrome is a generalized functional disorder of the proximal tubule of the kidney and is characterized by aminoaciduria, glycosuria, hyperphosphaturia, dehydration, rickets, and growth failure. Nephrocalcinosis and hypercalciuria are rare manifestations of Fanconi syndrome. There is no case report of Fanconi syndrome complicated with nephrocalcinosis and hypercalciuria in Korea. A 6-year-old boy presented with genu valgum and waddling gaits for about 3 years. There was no family history of renal disease and his physical examination was normal except for genu valgum and corrected cleft lip and palate. Laboratory investigations showed generalized aminoaciduria, glycosuria, hyperphosphaturia, hypercalciuria, and low-molecular weight proteinuria including ${\beta}$-microglobulin. Serum 25-OH vitamin $D_3$ was within the normal range, and $1,25-(OH)_2$ vitamin $D_3$ was elevated. Bilateral renal medullary hyperechogenicity was demonstrated by ultrasonography. Analysis of the CLCN5 gene revealed no mutation. Here we describe a boy with Fanconi syndrome complicated with nophrocalcinosis and discuss the differential diagnosis.

• Archives of Craniofacial Surgery
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• 제16권1호
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• pp.39-42
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• 2015

Actinomycosis is a subacute or chronic suppurative infection caused by Actinomyces species, which are anaerobic Gram-positive bacteria that normally colonize the human mouth and digestive and urogenital tracts. Cervicofacial actinomycosis is the most frequent clinical form of actinomycosis, and is associated with odontogenic infection. Characterized by an abscess and mandibular involvement with or without fistula, but the cervicofacial form of actinomycosis is often misdiagnosed because the presentation is not specific and because it can mimic numerous infectious and non-infectious diseases, including malignant tumors. We report a rare case of actinomycosis infection with coexisting submandibular sialolithiasis. The patient presented with a $1{\times}1cm$ abscess-like lesion below the lower lip. Punch biopsy of the lesion revealed atypical squamous cell proliferation with infiltrative growth, suggestive of squamous cell carcinoma. The patient underwent wide excision of this lesion, where the lesion was found to be an abscess formation with multiple submandibular sialolithiases. The surgical specimen was found to contain Actinomyces without any evidence of a malignant process. We assumed that associated predisposing factors such as poor oral hygiene may have caused a dehydrated condition of the oral cavity, leading to coexistence of actinomycosis and sialolithiasis.

• Archives of Plastic Surgery
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• 제34권4호
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• pp.501-503
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• 2007

Purpose: The authors report unusual one case of a patient presenting with maxillary sinus mucocele who had underwent Lefort I procedure 7 years ago. Methods: Case report and literature review Results: A 25 year old man came to us with fullness, pain and nasal obstruction on his left cheek area. He had a history of multiple operations due to cleft lip and palate since birth. Two jaw surgery was performed for correcting class III malocclusion 7 years ago. Computed tomography showed haziness, and fluid filled cystic mass on left maxillary sinus. Nasoendoscopy revealed the bulging of inferior turbinate and mucosa coincided in medial wall of maxillary sinus. Antrostomy with Caldwell-Luc approach was performed. Mucin contaning brownish exudate was leaked out. Severe inflammation of maxillary inner wall and exposure of 2 screws fixed previously were noticed. The curettage and marsupialization were accomplished. The symptoms of patient were improved after that procedure. Conclusion: Maxillary sinus mucocele is related with Lefort I procedure and it may occur even long after that procedure.

• Archives of Craniofacial Surgery
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• 제12권2호
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• pp.97-101
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• 2011

Purpose: Nasal bone fracture is the most common type of facial bone fracture. The standard 6-view photography was not adequate to support the evaluation of nasal deformity and the results of closed reduction. The authors have standardized a bird's eye view photography to more effectively evaluate this nasal deformity. Methods: We reviewed the medical records and radiologic studies of 63 nasal bone fracture patients. We had taken clinical photography including bird's eye view that was standardized as nasal tip was aligned to Cupid's bow of upper lip and light was focused on the nasion of all 63 patients. Results: Nasal deviations and reductions were more noticeable on the newly standardized bird's eye view. This clinical photography was very useful to explain the results of reduction. Conclusion: It was concluded that this photography can be more reliable for evaluation of severity of nasal deformity and the result of closed reduction.

• Archives of Craniofacial Surgery
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• 제21권4호
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• pp.244-248
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• 2020

The infraorbital nerve is a branch of the trigeminal nerve. Injury to the infraorbital nerve can be caused by trauma, including various facial fractures. Due to this nerve injury, patients complain of numbness and pain in the entire cheek, the ala of nose, and upper lip. In general, spontaneous sensory recovery is expected after decompressive surgery. If nerve transection is confirmed, however, neurorrhaphy is typically performed. Here, we present a case in which microsurgery was not performed in a patient with Sunderland grade V avulsion injury of the infraorbital nerve due to a facial bone fracture. Gradual nerve function recovery was confirmed to be possible with conservative treatment and rehabilitation alone. These findings suggest that the nerve function recovery can be expected with conservative treatment, even for severe nerve injury for which microsurgery cannot be considered.

• Korean Journal of Cleft Lip And Palate
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• 제8권1호
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• pp.1-9
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• 2005

1. Introduction : 개방형 비성형술은 직접적인 비소주에 대한 접근으로 진단과 기술상의 장점으로 인하여 과거 수년간 의심의 여지 없이 사용되었으며 또한 비익 연골을 쉽게 사용할 수 있어서 대부분의 경우에 사용되어져 왔다. 그러나 비개방형에 비해 개방형 비성형술의 경우 콧구멍과 비첨부의 비대칭 및 비익부의 길어짐 등의 불만을 호소하는 경우가 많았다. 그러나 개방형 비성형술의 경우 아직도 접근의 용이성 및 대칭성으로 많이 사용되고 있다. 2. Material & Methods : 이에 서울대학교 구강악안면외과에서 1999년부터 2001년까지 개방형 성형술을 시행 받은 환자를 대상으로 술후에 비익과 비첨부의 대칭 및 비공의 크기 정도를 평가해보고 개방형 비성형술의 좋은 결과에 대해 논해보고자 하였다. 3. Resulo : 술 후 환자의 만족도는 높은 편이었으나 양측의 대칭 정도에서는 조금씩 차이를 보여 비첨은 대개 이환측으로 변위되어 있었으며 비공의 크기에서도조금씩 차이를 나타내었다. 4. Conclusion : 지금까지는 주로 비순부의 평균치나 성장 방향을 연구하는데 주로 계측치들이 이용되었던 반면, 수술 후 일어날 수 있는 비부의 변화앙상을 나타내는 데에는 부족한 점이 많았고 특히 구순 구개열 환자에서 연령, 성별에 따른 표준자료의 부족으로 형태학적인 비교 연구 및 표준자료가 부족하였다. 따라서 이번 연구에서는 구순 구개열 환자의 술 전 및 술 후 의 변화 양상을 파악하는데 도움이 될 만한 차트를 만들었고 변화양상을 연구하는데 도움이 될만한 자료를 제시하는 바이다.

• Archives of Craniofacial Surgery
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• 제12권1호
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• pp.71-74
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• 2011

Purpose: Sarcoidosis is a systemic noncaseating granulomatous disease of an unknown origin, and can involve any organ including the skin. The infiltration of sarcoid granuloma in an old cutaneous scar is an uncommon cutaneous manifestation of sarcoidosis. This paper reports a 35-year old female who presented with cutanesous nodules in previous facial scars. Methods: A 35-year-old female presented with cutaneous nodules for 2 months in previous scars of the forehead and lower lip that she had acquired in the childhood. An excisional biopsy of the lower lip mass, serologic examinations and radiologic studies were performed. Results: The excisional biopsy revealed noncaseating granulomas consistent with sarcoidosis. Chest CT revealed both hilar and paratracheal lymphadenopathy with nodular densities in both lung fields. Routine laboratory tests, serologic tests, serum angiotensin converting enzyme level, sputum for acid-fast bacilli, ophthalmoscopic examination, TB-PCR and NTM-PCR showed normal findings. Therefore, the patient was diagnosed with sarcoidosis. The patient refused fiberoptic bronchoscopy and medication with oral steroid. Currently, the clinical manifestation and progress are being monitored closely, and treatment is expected to start with oral steroid according to the progress. Conclusion: For the proper management of cutaneous sarcoidosis, particularly scar sarcoidosis, plastic surgeons should be aware of the many clinical and histopathological features of sarcoidosis and recommend a systemic evaluation for early diagnosis and proper treatment.

• Archives of Plastic Surgery
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• 제48권5호
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• pp.518-523
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• 2021

Epignathus is a rare congenital orofacial teratoma that arises from the sphenoid region of the palate or the pharynx. It occurs in approximately 1:35,000 to 1:200,000 live births representing 2% to 9% of all teratomas. We present the case of a newborn of 39.4 weeks of gestation with a tumor that occupied the entire oral cavity. The patient was delivered by cesarean section. Oral resection was managed by pediatric surgery. Plastic surgery used virtual 3-dimensional models to establish the extension, and depth of the tumor. Bloc resection and reconstruction of the epignathus were performed. The mass was diagnosed as a mature teratoma associated with cleft lip and palate, nasoethmoidal meningocele that conditions hypertelorism, and a pseudomacrostoma. Tridimensional technology was applied to plan the surgical intervention. It contributed to a better understanding of the relationships between the tumor and the adjacent structures. This optimized the surgical approach and outcome.

• Journal of the korean academy of Pediatric Dentistry
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• 제43권3호
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• pp.313-319
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• 2016

Wolf-Hirschhorn syndrome (WHS), associated with the deletion of the short arm of chromosome 4, causes multiple congenital malformations. Patients suffer from various deformities, including mental and growth disorders, epilepsy, hypotonia, congenital heart defects, and atypical craniofacial features. The "Greek warrior helmet appearance" is the most characteristic feature, with a prominent glabella, high arched eyebrow, broad nasal bridge, and hypertelorism. Cleft lip with or without cleft palate is observed in 30% of patients. Dental structure anomalies also exist including multiple tooth agenesis and over-retained primary molars caused by MSX1 gene impairment, and cone-shaped and taurodontic teeth. This case, a 9-year-old girl with WHS, showed intellectual disability, delayed growth development, previous occurrence of seizures, otitis media, and the typical facial features of WHS. Dental findings included multiple congenital missing teeth, over-retained primary teeth, and severe caries on the primary molars. Dental treatments were performed under general anesthesia. This report documents the characteristics of WHS, including general and oral features, and discusses the importance of oral hygiene and preventive dental management.