• Title/Summary/Keyword: malformation

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Vascular Malformation of Flexor Hallucis Longus Muscle Associated with a Flexion Deformities of Toes: A Case Report (장무지 굴곡근의 혈관성 기형에 동반된 발가락의 굴곡 변형: 증례 보고)

  • Ahn, Jong-Chul;Kim, Jung-Rae;Shin, Ji-Hoon;Shin, Duk-Seop
    • The Journal of the Korean bone and joint tumor society
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    • v.17 no.2
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    • pp.100-105
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    • 2011
  • Vascular malformations may typically present with palpable mass that can be either asymptomatic or can present with symptoms including swelling and pain. On rare occasions, vascular malformation of muscle may produce joint deformities caused by contracture of the involved muscle. When vascular malformation involves the flexor muscle of the leg, ankle equinus deformity may occur. However, there are no reports of toe deformities secondary to intermuscular or intramuscular vascular malformations of flexor muscles of toe. Thus, we report a case of vascular malformation of flexor hallucis longus muscle with flexion contracture of toes in a 40-years-old woman who was treated with surgical excision.

A Communicating Bronchopulmonary Foregut Malformation Associated with Absence of the Left Pericardium - A case report - (좌측 완전 심낭결손증을 동반한 Communicating Bronchopulmonary Foregut Malformation - 1예 보고 -)

  • Yoo, Dong-Gon;Park, Chong-Bin;Kang, Pil-Je;Lee, Jong-Hyeog;Kim, Chong-Wook
    • Journal of Chest Surgery
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    • v.40 no.11
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    • pp.793-797
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    • 2007
  • A communicating bronchopulmonary foregut malformation (CBPFM) is a rare congenital anomaly that is characterized by a fistula between isolated respiratory tissue and the esophagus or stomach, The presence of accessory lung tissue arising from the primitive gastrointestinal tube is a common factor in the development of all forms of bronchopulmonary foregut malformations. Recurrent pneumonia associated with cystic radiographic structures is a characteristic of the condition. Further imaging studies using esophagogram, bronchography, computerized tomography, MRI, and arteriography can help in making a diagnostic evaluation. The treatment is a surgical resection of the involved lung tissue, and fistula closure with a good prognosis. We encountered a case of CBPFM, who presented with an extralobar pulmonary sequestration and bronchogenic cyst communicating with a tubular esophageal duplication that was associated with a complete left pericardial defect.

Generalized Pulp Stones of Primary Dentition in a Patient with Molar-Incisor Malformation : A Case Report (Molar-Incisor Malformation 환자의 유치열 내 전반적인 치수석 관찰의 증례 보고)

  • Lee, Dongyun;Shin, Jisun
    • Journal of the korean academy of Pediatric Dentistry
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    • v.47 no.3
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    • pp.337-343
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    • 2020
  • Molar-incisor malformation (MIM) is a new type of root anomaly reported recently. The characteristics of MIM are dysplastic root formations, constriction of pulp chambers and presence of calcified matrices at the level of cementoenamel junction in permanent first molars and primary second molars. In some cases, permanent maxillary incisors are also affected. The permanent first molars of the patient in this case report were affected with MIM. Generalized pulp stones were observed in overall primary dentition. Micro-computed tomography (micro-CT) imaging and scanning electron microscope-energy dispersive X-ray spectrometer analysis were performed on the extracted mandibular first molar and maxillary primary second molar of the patient. Micro-CT images revealed the discontinuity of enamel directly connected to an accessory canal of the root.

Split hand/foot malformation with long-bone deficiency and BHLHA9 duplication: A prenatal diagnosis report

  • Cho, In Ae;Park, Ji Kwon;Baek, Jong Chul;Ha, A Na;Kang, Min Young;Lee, Jae Ik;Park, Ji Eun;Shin, Jeong Kyu;Choi, Won Jun;Lee, Soon Ae;Lee, Jong Hak;Paik, Won Young
    • Journal of Genetic Medicine
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    • v.12 no.2
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    • pp.123-127
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    • 2015
  • Distal limb deformities are congenital malformations with phenotypic variability and high genetic heterogeneity. Split hand/foot malformation, also known as ectrodactyly, is a congenital limb malformation characterized by a defect of the central rays of the hands and/or feet. Split hand/foot malformation with long-bone deficiency (SHFLD) is a rare condition related to a 17p13.3 duplication. Recently, genomic duplications encompassing BHLHA9 have been associated with SHFLD. We report a case of SHFLD presenting with campomelia of the right femur, bilateral agenesis of fibulae, bilateral club feet, and oligosyndactyly of the hands and feet, that was associated with a 17p13.3 duplication, as determined prenatally using array comparative genomic hybridization.

Congenital Cystic Adenomatoid Malformation Associated with Extralobar Pulmonary Sequestration - A case report - (외엽형 폐격리증을 동반한 선천성 낭종성 신종양 기형 - 1례 보고 -)

  • Lee, Jae-Kwang;Kweon, Jong-Bum;Park, Kuhn;Kwack, Moon-Sub;Sim, Sung-Bo
    • Journal of Chest Surgery
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    • v.33 no.7
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    • pp.594-596
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    • 2000
  • Congenital cystic adenomatoid malformation and Extralobar Pulmonary sequestration are very rare congenital anomalies. We experienced a 4 year-old female patient who had Congenital cystic adenomatoid malformation in her lower lobe of left lung. We accidently found extralobar pulmonary sequestration associated with Congenital cystic adenomatoid malformation at operation field. The resection of the left lower lobe and the extralobar pulmonary sequestration were performed. The arterial supply of the extralobar pulmonary sequestration was one anomalous artery arised from the thoracic aorta. The Venous drainage of expralobar pulmonary sequestration was intercostal vein into the azygous vein. The patient was discharged without any problem.

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A case of multicystic dysplastic kidney and cystic adenomatoid malformation of the lung identified as incidental findings (우연히 발견된 편측성 다낭성 신 이형성증과 폐의 선천성 낭성 선종양 기형이 합병된 증례)

  • Lee, Sun-Joo;Lee, Ji-Hun;Kim, Hyun-Hee;Kim, So-Young;Hahn, Seung-Hoon;Hwang, Ja-Young;Lee, Wonbae
    • Clinical and Experimental Pediatrics
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    • v.49 no.7
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    • pp.796-799
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    • 2006
  • Multicystic dysplastic kidney and congenital cystic adenomatoid malformation of the lung are independent disorders, but both result from abnormal morphogenesis during embryogenesis. Congenital cystic adenomatoid malformation of the lung is associated with renal anomalies as well as other extrapulmonary anomalies and almost all cases with these anomalies are stillborn. We report a case of a 21-month-old male who was admitted with the impression of acute infectious gastroenteritis; multicystic dysplastic kidney with congenital cystic adenomatoid malformation of the lung was detected incidentally during evaluation.

Congenital Cystic Adenomatoid Malformation Associated with Extralobar Pulmonary Sequestration -1 case report- (외엽형 폐격리증을 동반한 선천성 낭종성 선종양 기형 -1례 보고-)

  • Jeon, Sang-Hyeop;Kim, Byeong-Jun;Lee, Hyeong-Ryeol
    • Journal of Chest Surgery
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    • v.29 no.2
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    • pp.223-226
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    • 1996
  • Pulmonary sequestration and congenital cystic adenomatoid malformation are two infrequent congenital pulmonary diseases and the combination of these two entities is rare. We had experienced a 3 week old male patient with a pulmonary mass, who had been sufyerring from tachypnea and chest wall retraction after birth. The pulmonary mass was suspected as a congenital cystic adenomat id malformation by chest CT. And therefore, we performed urgent operation via standard thoracotomy incision. Upon a thoracotomy, there was an extrapleural mass with anomalous blood supply near the posterior diaphragm and multiple cystic lesions in right lower lobe. The two anomalous arteries arising from the thoracic aorta and one vein draining into the azygos vein were ligated. and then the extrapleural mass was removed and a right lower lobectomy was performed. Final histologic diagnosis was congenital cystic adenomatoid malformation associated with extralobar pulmonary sequestration. The patient was discharged without any problem.

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A STUDY OF MORPHOGENESIS OF DIGITAL MALFORMATION ON RAT EMBRYO BY X-IRRADIATION (방사선조사시 태내백서의 지지기형성과정에 관한 실험적 연구)

  • Khim Jhai Dhuck
    • Journal of Korean Academy of Oral and Maxillofacial Radiology
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    • v.11 no.1
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    • pp.33-40
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    • 1981
  • The author studied on the effects of x-irradiation to the development of digital malformation in gestation rats. The time-matings occured between 6 p.m. and 8 a.m. and females with copulation. plugs at 8 a.m. were isolated and properly marked for evidence of copulation. The lower abdomen of mothers were exposed to x-irradiation on the 11½th day of gestation, the critical period developing digital malformation, respectively 100, 150, 200, 250, 300 and 350 rads. At 18½th day of post-conception total 50 pregnant females were dissected and the incidence of digital malformations were obtained. Rat embryos on the 12, 13, 14, 15, 16th day of gestation irradiated by 250 rads were examined for morphogenesis of digital malformation. Digital radiating lines were examined in water and histologically by H-E stain. Supra vital stain samples by Nile-blue sulfate in 37℃ normal saline were prepared for the observation of cell necrosis regions and morphogenesis of digits. The results obtained were as follows; 1. By x-irradiation on 11th day of gestation, digital malformations of Ectrodactylia, Syndactylia Polydactylia and Hematodactylia were developed. Ectrodactylia showed the effective relationship to the amount of irradiation, however Syndactylia ans Polydactylia did not. 2. By x-irradiation, cell necrosis of digital germ was appeared markedly, but in 48 hours after irradiation was depressed to the periphery of digital germ and in 72hours after irradiation was disappeared. Digital radiating line showed marked stage of malformation in 48hours after irradiation and continued to show the same amount of physiological cell necrosis as the compared control group in 72hours. after irradiaion. But in the Syndactylia, physiological cell necrosis was not able to be recognized. 3. Ectrodactylia induced by x-irradiation was considered as the direct resoult of cell necrosis of digital origin, however, Polydactylia and Syndactylia were considered as the resoult of some effect in repair process of x-irradiation damages.

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Improvement in Diagnostic Accuracy of Hemangioma and Vascular Malformation through Multidisciplinary Clinic (협력진료를 통한 혈관종 및 혈관기형 진단 정확도 향상)

  • Kang, Kyung-Dong;Bae, Yong-Chan;Nam, Su-Bong;Choi, Soo-Jong;Jung, Do-Sang;Kim, Chang-Won
    • Archives of Plastic Surgery
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    • v.37 no.6
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    • pp.758-762
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    • 2010
  • Purpose: It is difficult to provide accurate diagnosis and proper treatment for vascular anomalies, because vascular anomalies have a wide spectrum of lesion with various symptoms. Furthermore, vascular anomalies often require the services of a wide range of specialists. So, many patients have been discouraged by the lack of expertise and treatment options available to them. In this study, we aimed to describe the scope of the Vascular Malformation clinic (VAM clinic), a multidisciplinary clinic for hemangioma and vascular malformation patients and investigate of diagnostic accuracy of VAM clinic. Methods: 68 patients have included in a retrospective, medical record review study in the VAM clinic from may 2002 to august 2009. Data included the types of diagnoses of patients seen in the clinic, locations of lesions, comparison of accuracy of diagnoses at other clinic and initial diagnoses at VAM clinic and types of recommended treatments. Results: Initial diagnosis at VAM clinic showed 94.2% accuracy, while diagnosis at other clinic only showed 12.5% accuracy. Head and neck is most frequently occurred lesions in patients of VAM clinic. And VAM clinic is able to provide various and proper treatments to patients. Conclusion: Hemangiomas and vascular malformations can present with various medical problems. VAM clinic, the multidisciplinary clinic provided accurate diagnoses and proper treatments to patients.

Clinical Features of Molar Root-Incisor Malformation: A Retrospective Study

  • Sejin Chun;Hyuntae Kim;Ji-Soo Song;Teo Jeon Shin;Hong-Keun Hyun;Jung-Wook Kim;Ki-Taeg Jang;Young-Jae Kim
    • Journal of the korean academy of Pediatric Dentistry
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    • v.51 no.3
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    • pp.279-289
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    • 2024
  • This study aimed to identify the clinical characteristics of molar root-incisor malformation (MRIM) and provide clinical considerations for treatments. Panoramic radiographs and electronic medical records of 64 patients with MRIM were retrospectively reviewed. Age, gender, follow-up period, medical history, complications, distribution of MRIM teeth, treatment, and prognosis were analyzed. Females were affected 1.56 times more than males, and the average age was 8.2 years. Dental complications, including periapical lesions, abscesses, and alveolar bone loss, were observed in 71.9%, and eruption disturbance of adjacent teeth was noticed in 37.5%. Most patients had medical histories in the first year of life. The most prevalent history was prematurity or low birthweight, followed by neurological conditions, surgeries, medications, and infections. All patients had MRIM on permanent first molars. The primary second molars were the second most frequently involved, followed by maxillary permanent central incisors and primary first molars. The prevalence was low on permanent lateral incisors and canines. Extraction was the most prevalent treatment done on MRIM-affected teeth, and most extraction sockets were managed by the eruption of permanent second molars. Few cases received endodontic treatment but with low success rates. Early detection of MRIM and prompt, appropriate treatment are important to avoid unnecessary discomfort from complications.