• Title/Summary/Keyword: Agenesis

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A Case of Isolated Right Pulmonary Artery Agenesis (고립성 우측 폐동맥 형성부전증 1례)

  • Kim, Do Youn;Lee, Jae Sung;Kim, Young;Chang, Yoon Soo;Kim, Hyung Jung;Kim, Tae Hoon;Ahn, Chul Min
    • Tuberculosis and Respiratory Diseases
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    • v.57 no.5
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    • pp.489-493
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    • 2004
  • Unilateral pulmonary artery agenesis is a rare congenital anomaly usually associated with other cardiovascular anomalies such as tetralogy of Fallot or septal defect. Unilateral pulmonary artery agenesis without other coexisting cardiovascular abnormality (isolated unilateral pulmonary artery) is extremely rare and often asymptomatic until adulthood. In these patients, diagnostic clue is found in a plain chest roentgenogram, showing a hyperlucent contracted hemithorax. We have recently experienced a case of isolated right pulmonary artery agenesis, which was diagnosed by chest dynamic CT, perfusion scan, echocardiogram and 3-dimensional reconstruction cardiac CT angiography in a 50-year old female who had suffered from mild dyspnea on exertion and improved with conservative treatment. We report this case with a brief review of the relevant literature.

Thin Glomerular Basement Membrane Disease with Herlyn-Werner-Wunderlich Syndrome : Uterus Didelphys, Blind Hemivagina and Ipsilateral Renal Agenesis (비박형 사구체 기저막 질환이 동반된 중복 자궁 일측성 폐쇄질 및 동측 신장 무형성 증후군 ( Herlyn - Werner- Wunderlich syndrome ) 1례)

  • Kim, Myoung-Soo;Park, Yong-Jun;Park, Young-Jun;Park, Noh-Hyuck;Song, Ji-Sun;Kim, Pyung-Kil
    • Childhood Kidney Diseases
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    • v.11 no.2
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    • pp.299-305
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    • 2007
  • Herlyn-Werner-Wunderlich syndrome(HWWs) is a rare variant of Mullerian ductal anomalies characterized by the presence of a hemivaginal septum, a didelphic uterus, and ipsilateral renal agenesis. It usually presents after menarche with progressive pelvic pain, and palpable mass due to hemihematocolpos. If a cystic mass is detected behind the urinary bladder in children, in association with the absence of a kidney, the diagnosis of uterus didelphys with imperforate vagina and hydrocolpos should be considered. When renal agenesis is found in asymptomatic children, the small size and the tubular shape of the uterus makes it almost impossible to evaluate uterine anomalies, so follow-up should be performed until the end of puberty. Appropriate preoperative diagnosis and treatment will prevent unnecessary procedures and offer relief of symptoms. We report one case of didelphic uterus with blind hemivagina and ipsilateral renal agenesis with biopsy- proven thin glomerular basement membrane disease which is not related to the above syndrome.

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Prevalence, Severity, and Correlation with Agenesis of Permanent Successors of Infraoccluded Primary Molars at Chonnam National University Hospital's Department of Pediatric Dentistry

  • Taegyoung Kim;Namki Choi;Seonmi Kim
    • Journal of the korean academy of Pediatric Dentistry
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    • v.51 no.1
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    • pp.11-21
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    • 2024
  • This study aimed to analyze the prevalence and severity of infraoccluded primary molars (IPM) and their correlation with the agenesis of permanent successors (APS). A total of 4,011 panoramic radiographs from children aged 4 to 11 years who had all 8 primary molars were examined. The prevalence of APS was analyzed based on the severity and tooth type of IPM. The prevalence of IPM was 13.0%, and there was no difference between genders. The majority of the children had two IPMs each. In all tooth types, mild infraocclusion was the most prevalent, whereas severe infraocclusion was the least frequent. Among the types of primary molars, the lower primary first molar (62.3%) was the most affected, followed by the lower primary second molar (27.7%), the upper primary second molar (5.8%), and the upper primary first molar (4.2%). Infraocclusion was 11.2 times more prevalent in the mandible than in the maxilla, with no significant difference between the left and right sides. The correlation between IPM and APS was also investigated. Among the subjects, 177 had one or more agenesis of premolars, of whom 54 (30.5%) had IPM. Additionally, among the 521 individuals with IPM, 54 individuals exhibited APS (10.4%). This study identified a noteworthy prevalence of infraocclusion, with notable variations among molar types. The LPFM was particularly affected. The majority of cases were classified as mild in severity. Furthermore, a compelling association between IPM and APS was established. Understanding this connection may enhance treatment strategies for infraoccluded teeth and tooth agenesis.

A Case of Mayer-Rokitansky-K$\ddot{u}$ster-Hauser(MRKH) Syndrome with Imperforate Anus and Unilateral Renal Agenesis (편측 신장 무형성 및 쇄항을 동반한 Mayer-Rokitansky-Küster-Hauser(MRKH) 증후군 1례)

  • Kim, Tae-Hyung;Kim, Jin-Hee;Kim, Soo-Yung
    • Childhood Kidney Diseases
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    • v.10 no.2
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    • pp.233-237
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    • 2006
  • The clinical features of the Mayer-Rokitansky-K$\ddot{u}$ster-Hauser(MRKH) syndrome include normal female secondary sex characteristics, normal genitalia, congenital absence of vagina, rudimentary or bipartite uterus, normal ovarian function and normal ovulation, 46, XX, karyotype, frequent association of renal, skeletal and other congenital anomalies. We experienced a case of a premature infant with MRKH syndrome who had imperforate anus and unilateral renal agenesis.

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Agenesis of the vulva in a Poodle dog

  • Lee, Jae-il;Lee, Ok-Keun;Kim, Yong-suk;Kim, Myung-Jin;Hong, Sung-Hyeok
    • Korean Journal of Veterinary Research
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    • v.47 no.2
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    • pp.229-231
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    • 2007
  • Vulvar agenesis in an eight-month-old toy poodle dog is described. Urinary incontinence and dysuria were the main clinical signs. The morphology of the urogenital system was assessed with contrast radiography. The constructers of lower urinary tract and reproductive system except for the vulva were normal features. Through episiostomy, a perineal stoma, resembling a vulva, was created, resulting in complete resolution of the clinical signs.

Interhemispheric Osteolipoma with Agenesis of the Corpus Callosum

  • Park, Yong-Sook;Kwon, Jeong-Taik;Park, Un-Sub
    • Journal of Korean Neurosurgical Society
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    • v.47 no.2
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    • pp.148-150
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    • 2010
  • Osteolipoma is an ossified lipoma with distinct components of fat and bone. We present a case of interhemispheric osteolipoma associated with total agenesis of the corpus callosum. A 20-year-old man complained of severe headache, nausea and vomiting. Brain computed tomography showed a low-density mass in an interhemispheric fissure, with high T1 and T2 magnetic resonance signals compatible with fat. The mass measured $4.9\;{\times}\;2.9\;cm$ in size and showed peripheral calcifications. There was another small piece of same signal mass within the lateral ventricular choroid plexus. The interhemispheric lesion was removed by an interhemispheric approach. Osteolipoma is rare in interhemispheric region, however, it should be a differential diagnosis of lesions with fat intensity mass and calcifications.

Diaphragm Translocation as Surgical Treatment for Agenesis of the Right Lung and Secondary Tracheal Compression

  • Kim, Dong Hee;Choi, Se Hoon
    • Journal of Chest Surgery
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    • v.49 no.1
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    • pp.59-62
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    • 2016
  • A 12-month-old boy was diagnosed with agenesis of the right lung. Mediastinal deviation progressed to the diseased side as the patient matured; therefore, tracheal distortion developed. As a result, tracheal compression developed between the vertebral body and aorta. The patient was repeatedly admitted to the hospital because of recurrent pulmonary infection and combined severe respiratory distress. Diaphragm translocation was performed to treat the patient. The postoperative course was favorable, and computed tomography scan findings and symptoms had improved at 1 year after surgery.

Persisting carotid duct and proximal external carotid artery agenesis in an adult

  • Mugurel Constantin Rusu;Adelina Maria Jianu;Alexandra Diana Vrapciu;Mihaela Daniela Manta
    • Anatomy and Cell Biology
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    • v.57 no.2
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    • pp.328-331
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    • 2024
  • The carotid duct (CD) is a transient embryological structure connecting the 3rd and 4th aortic arches. We found a persisting CD in an adult female case, by studying the computed tomography angiogram. On the left side, the proximal external carotid artery (ECA) agenesis was noted. The CD was inserted into the left subclavian artery and continued upwards to reach the level of the atlas, and then it descended to connect to a normally configured segment of that ECA. It could be speculated that the CD-to-ECA connection was possible via unregressed 1st and/or 2nd aortic arches. The segmental ECA agenesis is extremely rare, while its supply via a persisting patent CD was not reported previously to the authors' knowledge. The variants are extremely important during neck surgery because damaging the CD could determine hemorrhage, as well as ischemia in the ECA territory.

Internal carotid artery agenesis presenting with ruptured Acom aneurysm: Rare case report

  • Bhanu Pratap Singh Chauhan;Harkaran Singh Sahni;Jyoti Gupta;Rajan Pandya;Jayant Patidar;Anita Jagetia
    • Journal of Cerebrovascular and Endovascular Neurosurgery
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    • v.25 no.4
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    • pp.447-451
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    • 2023
  • Developmental anomalies of internal carotid artery (ICA), being rare entities, are mostly asymptomatic by themselves because of good collateral supply. However, when present with other associated intracranial anomalies requiring treatment, there can be catastrophic consequences, if special attention is not paid to this condition. We present a case of 36 years old male, who reported to our emergency department with complaints of headache and loss of consciousness. He was diagnosed as a case of ruptured anterior communicating aneurysm with subarachnoid hemorrhage and agenesis of left ICA with trans-cavernous anastomosis. He underwent clipping of aneurysm and was discharged uneventfully. This report highlights the importance of skillful microsurgical clipping in extremely high-risk conditions, in contemporary era of hybrid neurosurgeons.

Surgical Repair of Agenesis of Prepuce in a Dog (개의 포피무형성의 수술적 교정 1례)

  • Kim, Kyungmok;Lee, Joo-Myoung;Park, Hyunjung;Kim, Junghun;Won, Sangcheol;Cheong, Jongtae
    • Journal of Veterinary Clinics
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    • v.31 no.1
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    • pp.51-53
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    • 2014
  • An 10-day-old, male Jindo puppy was presented with anuria. This puppy was diagnosed as agenesis of prepuce and preputial orifice. To treat anuria exploratory operation was performed and the penis was found in situ under the skin. The penis was protruded outside of the skin. Because of strangulation and dryness of the penis by growing up, the artificial prepuce was made by making the subcutaneous tunnel. After that this puppy showed normal urination and had no complication. In case of the agenesis of the prepuce with the penis in situ, artificial prepuce by subcutaneous tunnel would be an alternative choice for saving the puppy.