• Title/Summary/Keyword: Congenital fusion

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A Case of Lung Metastasis of Mesoblastic Nephroma in Adulthood (성인에서 발생한 중배엽성 신종의 폐전이 1예)

  • Moon, Jin Wook;Kim, Kil Dong;Shin, Dong Hwan;Hahn, Chang Hoon;Jung, Jae;Park, Mu Suk;Jung, Sang Youn;Lee, Jae Hyuk;Kim, Young Sam;Kim, Se Kyu;Kim, Sung Kyu;Chang, Joon
    • Tuberculosis and Respiratory Diseases
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    • v.55 no.4
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    • pp.402-407
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    • 2003
  • Mesoblastic nephroma is a neoplasm of the kidney which is characterized by interlacing bundles of spindle mesenchymal cells. It is usually diagnosed during the first six months of life and is mostly benign. Incidence in adults is exceedingly rare. In most cases, only total excision is required without postoperative adjuvant therapy, and the rare cases of local recurrence have usually been related to incomplete removal. However, mesoblastic nephroma may behave aggressively, in contrast to a congenital mesoblastic nephroma. Several cases of metastatic mesoblastic nephroma have been previously described. We report herein a case of a 42-year-old woman with mesoblastic nephroma which recurred as a large metastatic lung mass seven years after the nephrectomy. The patient presented with chest wall discomfort for four days. Seven years previously, total nephrectomy had been performed because of a right renal tumor which had been diagnosed as a mesoblastic nephroma. There had been no evidence of recurrence for five years, after which she discontinued follow-up. On readmission two years later, chest X-ray and CT scan revealed a large lung mass in the left upper lobe. It was completely excised and the pathologic examination was identical with that of the original renal tumor. Synovial sarcoma was excluded because the fusion transcripts of the SYT-SSX fusion gene associated with the t(X;18) translocation were negative. The final diagnosis was a lung metastasis of mesoblastic nephroma and the patient remained free of disease for 7 months postoperatively.

Three Cases of Cervical Branchial Remnants (경부 새성기형 3례)

  • Choi, Hwan-Jun;Kim, Hyun-Sung;Choi, Chang-Yong;Yang, Hyung-Eun;Tark, Min-Seong
    • Archives of Plastic Surgery
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    • v.37 no.3
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    • pp.297-300
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    • 2010
  • Purpose: Failure of proper migration, fusion, or maturation of the branchial apparatus components results in a variety of congenital defects. Of these, cartilaginous rests are infrequent, while branchial cysts and sinuses are more common, relatively. The purpose of this study is to examine the clinical and pathological features of rare cervical branchial remnants in order to provide basis for its correct diagnosis and treatment. Methods: We report three cases of cervical branchial remnants which were treated in our hospital from December 2004 to December 2009. These cases were examined their clinical features, histologic findings and treatments. The patients had been operated with simple excision, excision of the combined components and preoperative antiboitics. Results: A retrospective review produced 2 cases of the cervical branchial remnants and 1 case of the cervical chondrocutaneous branchial remnant. All cases were on the left side of the neck, and anterior to the sternocleidomastoid muscle. Histopathological examination showed that fistula & sinus were lined with stratified squamous epithelium, additionally, they were consisted of a cutaneous envelope containing sebaceous glands, hair follicles, various amounts of adipose tissue, and elastic fibers. And, One case revealed containing hyaline cartilage. No patient developed complications or reccurences. Conclusion: The authors recommend simple surgical excision of the remnants when discharge, infection, or cosmetic problem occur. Finally, these lesions do not have fistulous tracts or connections with important, deeper organs, and so can be safely transected at the level of the superficial musculature.

Pulp Treatment of Triple Tooth in Primary Dentition: Two Case Reports (유치열에 나타난 삼중치에서 치수 치료 : 증례 보고)

  • Jeong, Hankeul;Lee, Nanyoung;Lee, Sangho
    • Journal of the korean academy of Pediatric Dentistry
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    • v.43 no.2
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    • pp.192-199
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    • 2016
  • Triple tooth is rare in primary dentition; it is the abnormal fusion of three teeth. There are a few reports of double teeth, but triple teeth are rare. These multiple teeth create several clinical problems, not only esthetic problems but also a high caries susceptibility, congenital missing permanent tooth germ(s), orthodontic problems, and periodontal problems. They also make it difficult to perform pulp treatment because of the complex tooth structure. A 1-year 7-month-old male underwent a partial pulpotomy with mineral trioxide aggregate (MTA) when pulp exposure caused by deep caries occurred at a maxillary anterior triple tooth in the first case. The second case was a 1-year 9-month-old male presenting with a fracture line at a mandibular anterior triple tooth. After removing the fractured fragment, a pulpectomy was performed at the remaining primary lateral incisors. Specific complications were not observed during 24 and 6 months of follow-up, respectively.

Reconstruction Techniques for Tissue Defects Formed after Preauricular Sinus Excision

  • Lee, Myung Joon;Yang, Ho Jik;Kim, Jong Hwan
    • Archives of Plastic Surgery
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    • v.41 no.1
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    • pp.45-49
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    • 2014
  • Background Preauricular sinuses are congenital abnormalities caused by a failure of fusion of the primitive tubercles from which the pinna is formed. When persistent or recurring inflammation occurs, surgical excision of the infected tissue should be considered. Preauricular defects inevitably occur as a result of excisions and are often difficult to resolve with a simple suture; a more effective reconstruction technique is required for treating these defects. Methods After total excision of a preauricular sinus, the defect was closed by a plastic surgeon. Based on the depth of the defect and the degree of tension when apposing the wound margins, the surgeon determined whether to use primary closure or a posterior auricular flap. Results A total of 28 cases were examined. In 5 cases, including 2 reoperations for dehiscence after primary repair, reconstruction was performed using posterior auricular transposition flaps. In 16 cases of primary closure, the defects were closed using simple sutures, and in 7 cases, closure was performed after wide undermining. Conclusions If a preauricular defect is limited to the subcutaneous layer and the margins can be easily approximated, primary closure by only simple suturing may be used to perform the repair. If the defect is deep enough to expose the perichondrium or if there is tension when apposing the wound margins, wide undermining should be performed before primary closure. If the extent of the excision exposes cartilage, the procedure follows dehiscence of the primary repair, or the tissue is not sufficiently healthy, the surgeon should use a posterior auricular flap.

Morphology and Reproduction of Symphyocladia latiuscula (Harvey) Yamada (Rhodophyta, Rhodomelaceae) in Korea (한국산 홍조 참보라색우무(Symphyocladia latiuscula (Harvey) Yamada)의 형태와 생식)

  • 최도성
    • Journal of Plant Biology
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    • v.34 no.1
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    • pp.59-66
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    • 1991
  • The morphotaxonomic characters and life history of Symphyocladia latiuscula (Harvey) Yamada were investigated with field and laboratory culture materials. The species is well defined by a 5-15 cm hight, entirely corticated thallus and congenital fusion of 5-7 segments between main axis and laterals. Although all the reproductive structures are basically similar to those of other species of the genus, it is characteristic that the pinnulae are transformed into the flabellated tetrasporangial stichidia. Vegetative trichoblasts are known to be absent in the Pterosiphonieae, but their presence in S latiuscula does not accept its taxonomic position in that tribe. As a result, the occurrence of vegetative trichoblast is not useful for a taxonomic character to distinguish the tribes. The species shows a Polysiphonia-type life history involving a dimorphic alternation of gametophytes and sporophytes in culture and field. It grows below the middle intertidal zone in all the coasts of Korea throughout the year, although fertile plants are normally found during the summer months.months.

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Long-Term Result of the Epiphyseal Transplantation in Distal Forearm (전완원위부 변형에 대한 혈관부착 성장판 이식술)

  • Chung, Duke-Whan;Han, Chung-Soo;Yoo, Myung-Chul;Han, Soo-Hong
    • Archives of Reconstructive Microsurgery
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    • v.4 no.1
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    • pp.16-22
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    • 1995
  • We analyzed 11 children who underwent epiphyseal transplantation to the forearm for manage growing deformity ranged from 2 years 6 months to years(average 5 years 10 months) follow-up period. Etiologies of the functional impairment of the eleven were five traumatic, three congenital and three tumorous conditions. Lesions of epiphysis were distal radius in eight patients and distal ulna in three patients. Operation was performed with removal of non-functioning or deformed epiphysis followed by transplantation of free vascularized proximal fibular epiphysis with microvascular anastomesis. Evaluation was performed radiologically and functionally. The 9(81.8%) patients showed growth of transplanted epiphysis by radiological examination during follow up. At the last follow up, average growth rate was 0.86cm per year excepts 2 cases of no growth. Active wrist motion near normal to contralateral joint was achieved in 7 patients. In other 2 patients, active joint motion was improved but weaker than contralateral joint. Complications on donor site were two transient peroneal nerve palsy which have been resolved after 2 and 5 months post operation and one valgus ankle deformity. The ankle deformity was corrected with $Langenski\"{o}ld$ operation of the dital tibiofibular fusion. At recipient site, there was one superficial infection and it was easily controlled by systemic antibiotics. Many subsequent reports have described successful nonvascularized epiphyseal transplante, but overall results have been inconsistent and unsatisfactory. Other experimental and clinical studies in the transfer of vascularized epiphyses has encourage its clinical application. We also could gel successful growth in several cases with free vascularized epiphyseal transplantation.

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Experience of Single Stage Treatment of Caniosynostosis, Hypertelorism, Exophthalmos Patient (두개조기유합증, 안와격리증, 안구돌출증 환자의 동시 교정 치험례)

  • Chung, Chul Hoon;Eun, Seok Chan;Seo, Dong Kuk;Jo, Woo Sung;Park, Se Hyuck
    • Archives of Plastic Surgery
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    • v.33 no.2
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    • pp.237-240
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    • 2006
  • The simultaneous correction of the hypertelorism and exophthalmos combined with craniosynostosis is very rarely performed operative procedures in the world. The craniosynostosis is the congenital anomaly that designates premature fusion of one or more sutures in either cranial vault or cranial base. Hypertelorism is not a distinct clinical syndrome in itself, but is a physical finding secondary to facial and cranial maldevelopment and it is defined as a increase in the distance between the medial orbital walls. Exophthalmos can occur following the decrease in the size of the orbit in patients with developmental skeletal disorders such as craniofacial synostosis. The authors experienced 9-year-old male patient, who has complex cranio-facial abnormality. The craniosynostosis was oxycephaly type and primary fronto-orbital advancement surgery had been performed in other hospital. The abnormal cranial vault combined with hypertelorism and exophthalmos due to maldeveloped both orbital walls. Surgical correction was obtained by various cranio-fronto-orbital remodeling technique such as calvarial bone craniotomy, fronto-orbital advancement, paramedian resection, medial canthopexy, Tessier-Wolfe three wall orbital expansions. We achieved a quite satisfactory result both functionally and aesthetically in a complex cranio-facial deformity patient by combination and modification of previously developed various cranio-facial plasty technique and hereby report the case with brief discussion and review of literature.

Clinical Experience with Nasolabial Cysts Using the Sublabial Approach (구강내 접근법을 이용한 비순낭종의 치료 경험)

  • Kwon, Joon-Sung;Choi, Hwan-Jun;Choi, Chang-Yong;Park, Jae-Hong;Park, Nae-Kyeong;Kim, Sook
    • 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.

A Case of Crossed Fused Renal Ectopia with Multicystic Dysplasia (다낭 형성 이상을 보이는 융합된 교차성 신전위 1례)

  • Seo, Eun-Min;Shim, Eun-Jung;Lee, Kwan-Seob
    • Childhood Kidney Diseases
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    • v.12 no.2
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    • pp.262-266
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    • 2008
  • Crossed renal ectopia is a congenital malformation in which both kidneys lie on the same side of the spine, usually side by side longitudinally. More often on the right side. Fusion of the two renal units is eight times more common than nonfusion. Although crossed renal ectopia is uncommon, this unusual entity must be considered in an infant when cystic mass in the abdomen or pelvis paticularly if no kidney can be found on the opposite side. In many cases of crossed fused ectopia with multicystic dysplastic kidney(MCDK), the diagnosis can be strongly suspected from the sonogram, and no other studies may be necessary. However, both intravenous urography and isotope renography is useful to assess the function of the crossed kidney. Crossed renal ectopia and MCDKs are associated with a greater incidence of ureteropelvic junction obstruction and reflux. So, screening voiding cystourethrography should be performed. Very few studies of MCDK in the setting of crossed fused ectopia have been reported. We have experienced a 3-year-old boy with crossed fused renal ectopia with multicystic dysplasia.

Acquired Dermoid Cysts within Subcutaneous Scar Tissue (피하 흉터 조직에서 발견된 후천성 유피낭종)

  • Kim, Hyoung Jin;Burm, Jin Sik;Pyon, Jai Kyong;Kim, Yang Woo
    • Archives of Plastic Surgery
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    • v.34 no.4
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    • pp.508-511
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    • 2007
  • Purpose: Dermoid cysts are almost always caused by congenital events. The most widely accepted pathogenesis is that the cysts are dysembryogenetic lesions that arise from ectodermic elements entrapped during the midline fusion. We report a rare case of dermoid cyst, which occurred not congenitally but newly in the subcutaneous scar tissue secondary to trauma. Methods: A 26-year-old man had a deep submental laceration caused by a car accident and got a primary wound closure 16 months ago. There were 18 cm-long submental hypertrophic scar and newly developed palpable masses inside the subcutaneous layer at the center of the scar. Initial impression was an epidermal cyst or a thyroglossal duct cyst. Ultrasonographic finding showed two cystic masses inside the scars at the submental area, but impressed dermoid cysts. The cystic masses were completely removed with W-plasty and histological examination were followed. Results: The histological diagnosis was dermoid cysts which were mainly composed of keratotic squamous epithelium in their inner surface linings and numerous skin appendages such as sebaceous glands, sweat glands, and hair follicles in their cystic lumens histopathologically. During the follow-up period of 25 months, there was no recurrence of any subcutaneous mass in the site of scar. Conclusion: We report a very unusual case of dermoid cysts developed by an acquired cause, considering that the accidental inclusion of deep skin elements caused by a trauma can be a critical origin of dermoid cysts.