• 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.

• 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.

• 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.

• 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.

• 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.

• 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.

• 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.

• Journal of Chest Surgery
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• v.30 no.1
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• pp.108-111
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• 1997

Congenital unilateral agenesis of pulmonary artery is a rare anomaly and it usually occurs in association with other cardiac anomaly such as tetralogy of Fallot. Since most patients affected by this defect without associated congenital cardiac anomaly or pulmonary Infection are asymptomatic, the clinical diagnosis of this anomal is first recognized by a characteristic pattern in chest roentgenogram taken as a routine checking; the findings on chest film consists of cardiac and mediastinal displacement, absence of the pulmonary arterial shadow, smaller hemithorax, and elevationof the hemidiaphragm, all on the affected side. We experienced rlght pulmonary artery agenesis in a 48 year-old male, who complained of massive hemoptysis, and it was diagnosed by digital subtraction pulmonary arteriogram and perfusin scan, and treated by right middle and lower lobe bi-lobectomy, and we report this case with the review of relevant literatures.

• Journal of Chest Surgery
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• v.40 no.1 s.270
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• pp.52-55
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• 2007

Unilateral pulmonary artery agenesis is a rare congenital malformation usually associated with other cardiovascular anomaly such as Tetralogy of Fallot. Isolated pulmonary artery agenesis is very rare, and usually asymptomatic. It is usually highly suspected by routine chest X-ray, and associated symptoms are hemoptysis, blood tinged sputum, repeated pulmonary infection, and dyspnea on exertion. We have recently experienced the right pulmonary artery agenesis in 27 year-old male patient, complaining of minimal hemoptysis and sustained blood tinged sputum. He was successfully treated by right pneumonectomy, so we report this case with the review of associated literature.

• Clinical and Experimental Reproductive Medicine
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• v.48 no.1
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• pp.85-90
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• 2021

We present data from three Caucasian men with Zinner syndrome who attended our center for the treatment of primary couple's infertility. Each patient was scheduled for conventional testicular sperm extraction (cTESE) and cryopreservation. Sperm analysis confirmed absolute azoospermia. Patient 1 had right and left testis volumes of 24 mL and 23 mL, respectively; left seminal vesicle (SV) agenesis, severe right SV hypotrophy with right renal agenesis. Follicle-stimulating hormone (FSH) was 3.2 IU/L. Patient 2 exhibited right and left testis volumes of 18 mL and 16 mL, respectively; a left SV cyst of 32 × 28 mm, ipsilateral kidney absence, and right SV agenesis. FSH was 2.8 IU/L. Patient 3 showed a testicular volume of 10 mL bilaterally, a 65 × 46 mm left SV cyst, right SV enlargement, and left kidney agenesis. FSH was 32.0 IU/L. Sperm retrieval was successful in all patients. Nevertheless, cTESE should be performed on the day of oocyte retrieval.