• Title/Summary/Keyword: RARITY

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A Case of Symptomatic Tracheal Diverticulum and Surgical Resection as a Treatment Modality

  • Lee, Shin-Young;Joo, Seok;Lee, Geun Dong;Ham, Seok Jin;Park, Chul Hwan;Lee, Sungsoo
    • Journal of Chest Surgery
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    • v.49 no.5
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    • pp.405-407
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    • 2016
  • Tracheal diverticulum is often diagnosed incidentally and, due to its rarity, there is no standard treatment. It is a benign entity, but has the potential to cause specific symptoms, such as chronic upper respiratory infection and chronic cough. Symptomatic tracheal diverticulum can be medically treated, but likelihood of recurrence is high. We report a case of surgical resection of symptomatic tracheal diverticulum to prevent recurrence.

The Medical Treatments of Intestinal Behçet's Disease: An Update

  • Lee, Hye Won;Kim, Won Ho;Cheon, Jae Hee
    • Intestinal research
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    • v.11 no.3
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    • pp.155-160
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    • 2013
  • Behçet's disease (BD) is a systemic immunological disorder characterized by recurrent mucosal ulcerative lesions including oral and genital ulcerations in association with skin and ocular involvements. BD also can involve the gastrointestinal tract. Gastrointestinal involvement of BD is one of the major causes of morbidity and mortality for this disease. However, clinical data are quite limited because of the rarity of intestinal BD. Therefore, the management of intestinal BD is heavily dependent on expert opinions and standardized medical treatments of intestinal BD are yet to be established. In this brief review, the authors summarized the currently available medical treatments such as 5-aminosalicylic acids, corticosteroids, immuno-modulators, and anti-TNF agents. Moreover, we sought to suggest a treatment algorithm for intestinal BD based on the recently published and updated data.

A Case of Spontaneous Common Iliac Atery Dissection

  • Noh, Juho;Rhee, Il;Kim, Minsung;Lee, Jonghyun;Kim, Kisu;Park, Byungwhan
    • Kosin Medical Journal
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    • v.33 no.3
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    • pp.431-437
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    • 2018
  • Spontaneous and isolated dissection of the limb arteries without involvement of the aorta is extremely rare, and has been reported previously in pregnant patients in association with collagen vascular disease, and in cases of high-energy trauma or intensive activity in athletes. There is no consensus yet on indications for medical or surgical therapeutic modality. Due to the rarity of spontaneous dissection of external iliac artery, its natural history has been poorly described. A healthy 50-year-old male with normotension was admitted with an acute onset of left flank pain. Left external iliac artery dissection was diagnosed by abdominal computed tomography.

Airway management of a patient incidentally diagnosed with Mounier-Kuhn syndrome during general anesthesia

  • No, Hyun-Joung;Lee, Jung-Man;Won, Dongwook;Kang, Pyoyoon;Choi, Seungeun
    • Journal of Dental Anesthesia and Pain Medicine
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    • v.19 no.5
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    • pp.301-306
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    • 2019
  • Mounier-Kuhn syndrome (MKS) is a disease characterized by dilation of the trachea and mainstem bronchi. Due to the risk of airway leakage, pulmonary aspiration, and tracheal damage, MKS can be fatal in patients undergoing tracheal intubation. Moreover, MKS may not be diagnosed preoperatively due to its rarity. In this case, a patient undergoing neurosurgery was incidentally diagnosed with MKS during general anesthesia. During anesthesia induction, difficulties in airway management led the anesthesiologist to suspect MKS. Airway leakage was resolved in this case using oropharyngeal gauze packing. Anesthesiologists should be aware of the possibility of MKS and appropriate management of the airways.

Primary Malignant Tracheal Tumor : 3 Cases (원발성 악성 기관 종양 3례)

  • 고중화;전영명;신상준;주희재
    • Korean Journal of Bronchoesophagology
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    • v.3 no.1
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    • pp.137-147
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    • 1997
  • The rarity of primary tumor of the trachea, which was recently estimated in a circumscribed population to be 2.7 new cases per million per year, explains the relatively limited experience that has been acquired even by major institutions. Although there may already by a high degree of airway obstruction, tracheal tumors are usually misdiagnosed as bronchial asthma or chronic bronchitis because of its nonspecific symptoms. Surgery is the treatment of choice. Recently, the authors experienced three cases of primary tracheal malignant tumors ; one case of mucoepidermoid carcinoma and two cases of adenoid cystic carcinoma. The authors report on these cases with a review of the literature for give help in differential diagnosis and treatment planing of tracheal tumor.

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Soft-tissue osteoma of the temple

  • Roh, Si-Gyun;Kim, Yun-Seob;Kim, Jong-Lim;Shin, Jin-Yong;Lee, Nae-Ho
    • Archives of Craniofacial Surgery
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    • v.22 no.5
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    • pp.276-279
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    • 2021
  • A 65-year-old woman presented with a solid mass on the right temporal area. The mass had grown for over 2 years without any initiating event of trauma or inflammation. Before excision, the patient went through a computed tomography scan, revealing a calcified mass without bony connection. Under general anesthesia, an excisional biopsy was performed. Microscopic examination confirmed a diagnosis of soft tissue osteoma. Soft tissue osteoma is rare, especially in the head and neck region. Osteomas in the temporal region have not been reported yet. Due to its rarity, osteoma might be misdiagnosed as another soft tissue or bone origin tumor. Its treatment of choice is simple excision. In this review, we present an unusual clinical form of soft tissue osteoma.

A frameshift mutation in the TRPS1 gene showing a mild phenotype of trichorhinophalangeal syndrome type 1

  • Park, Jin-Mo;Lee, Yun Jeong;Park, Jin-Sung
    • Journal of Genetic Medicine
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    • v.15 no.2
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    • pp.97-101
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    • 2018
  • Tricho-rhino-phalangeal syndrome (TRPS) is a hereditary disorder characterized by craniofacial and skeletal abnormalities. A mutation of the TRPS1 gene leads to TRPS type I or type III. A 20-year-old male patient visited our neurologic department with chronic fatigue. He presented with short stature, sparse hair, pear-shaped nose, and brachydactyly. Radiologic study showed short metacarpals, metatarsals with cone-shaped epiphyses, hypoplastic femur and hip joint. Panel sequencing for OMIM (Online Mendelian Inheritance in Man) listed genes revealed a de novo heterozygous frameshift mutation of c.1801_1802delGA (p.Arg601Lysfs*3) of exon 4 of the TRPS1 gene. The diagnosis of TRPS can be challenging due to the rarity and variable phenotype of the disease, clinicians should be aware of its characteristic clinical features that will lead a higher rate of diagnosis.

Aortic Reconstruction Using a Main Pulmonary Artery Flap in an Isolated Aortopulmonary Window

  • Shin, Hong Ju;Jung, Younggi;Shin, Jae Seung
    • Journal of Chest Surgery
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    • v.52 no.4
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    • pp.236-238
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    • 2019
  • Aortopulmonary window (APW) is a rare cardiac anomaly that was reported to occur in only 43 cases over 33 years at a large-volume cardiac center. It can present as an isolated anomaly or in combination with another cardiac anomaly. The surgical technique for APW has evolved from simple ligation to separation of the 2 great arteries. However, because of the rarity of APW, there is no standard surgical treatment for this disease entity. Herein, we present successful aortic reconstruction using a main pulmonary artery flap after separation of the 2 great arteries in a neonate with isolated APW.

Distant metastasis of follicular thyroid carcinoma to the mandible: a rare case report

  • Jeon, Young-Tae;Kim, Chul-Hwan;Park, Sung-Min;Kim, Min-Kyoo
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • v.45 no.5
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    • pp.294-298
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    • 2019
  • Treatment of metastatic carcinoma of the oral cavity can be a challenge due to its rarity. The current case report details metastatic carcinoma of the mandible originating from follicular thyroid carcinoma, which is the second most prevalent tumor of the thyroid. As the mandibular lesion developed, the primary thyroid lesion of thyroid. Here, we report a case of metastatic carcinoma of the mandible in a 67-year-old female that was treated with partial mandibulectomy and fibular free flap.

An accurate diagnosis of odontogenic cutaneous sinus tract by different computed tomography unit setting

  • Sodnom-Ish, Buyanbileg;Eo, Mi Young;Kim, Soung Min
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • v.47 no.1
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    • pp.51-56
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    • 2021
  • Due to their rarity and the lack of associated dental symptoms, odontogenic cutaneous sinus tracts (OCSTs) are often misdiagnosed and confused with cutaneous lesions or non-odontogenic infections. It has been estimated that 50% of individuals affected by OCSTs are subjected to inappropriate treatments before the correct diagnosis is established. We describe the diagnosis and treatment of two cases of OCSTs. By using a computed tomography (CT) with soft tissue window setting, the extent of cortical bone destruction and the path of the sinus tract in the soft tissue was easily identified. Thus, we recommend the use of imaging techniques such as CT, which can confirm the odontogenic origin and the exact location of the OCST.