• Journal of Oral Medicine and Pain
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• 제38권2호
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• pp.175-185
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• 2013

측두하악장애를 주소로 하는 환자 276명에 대해 임상검사와 방사선검사를 시행하였다. 하악과두 저형성으로 진단된 189명의 실험군과 골관절염으로 진단된 87명의 대조군을 임상적으로 비교하여 다음과 같은 결과를 얻었다. 1. 하악 과두저형성은 골관절염과 임상적으로 많은 부분이 유사하였다. 2. 하악 과두저형성은 골관절염에 비하여 방사선적 진단부위와 통증부위의 일치율이 현저하게 낮았다. 3. 하악 과두저형성 양측이환군이 편측이환군에 비해 교합불편감 및 이악물기습관이 많았다. 4. 편측이환군 비교시 하악 과두저형성은 편측저작습관이 높았으며, 개구제한감을 더 많이 느꼈다. 골관절염은 아침의 불편감 및 두통을 많이 호소하였으며 진단부위와 단순관절음 일치율이 높았다. 5. 양측이환군 비교시 하악 과두저형성이 골관절염보다 평상시 두통을 더 많이 호소하였으며, 수평피개량도 컸다.

• Imaging Science in Dentistry
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• 제41권1호
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• pp.39-42
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• 2011

Facial asymmetry is not an uncommon occurrence in day to day dental practice. It can be caused by various etiologic factors ranging from facial trauma to serious hereditary conditions. Here, we report a rare case of non-syndromic facial asymmetry in a young female, who was born with this condition but was not aware of the progression of asymmetry. No relevant family history was recognized. She was also deficient in both deciduous and permanent teeth in the corresponding region of maxilla. Hence, the cause of this asymmetry was believed to be a segmental odontomaxillary hypoplasia of left maxilla accompanied by agenesis of left maxillary premolars and molars and disuse atrophy of corresponding facial musculature. This report briefly discussed the comparative features of segmental odontomaxillary hypoplasia, hemimaxillofacial dysplasia, and segmental odontomaxillary dysplasia and justified the differences between segmental odontomaxillary hypoplasia and the other two conditions.

• Journal of Chest Surgery
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• 제45권5호
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• pp.348-350
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• 2012

We herein report a very rare case of adult right-sided Bochdalek hernia accompanied with hepatic hypoplasia and inguinal hernia. A 29-year-old man was admitted with right-sided pneumothorax. A computed tomography was performed and revealed large right sided Bochdalek hernia with hepatic hypoplasia. Under thoracolaparotomy, the defect was closed with Gore-Tex soft tissue patch. After the operation, left-sided inguinal hernia was found. However, it turned out that it had been present during infancy and spontaneously resolved during adolescence. This is the first report of right-sided Bochdalek hernia with hepatic hypoplasia and inguinal hernia in an adult.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제43권6호
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• pp.407-414
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• 2017

Objectives: The study was designed to evaluate the efficacy of performing a second, repeat anterior maxillary distraction (AMD) to treat residual cleft maxillary hypoplasia. Materials and Methods: Five patients between the ages of 12 to 15 years with a history of AMD and with residual cleft maxillary hypoplasia were included in the study. Inclusion was irrespective of gender, type of cleft lip and palate, and the amount of advancement needed. Repeat AMD was executed in these patients 4 to 5 years after the primary AMD procedure to correct the cleft maxillary hypoplasia that had developed since the initial procedure. Orthopantomogram (OPG) and lateral cephalograms were taken for evaluation preoperatively, immediately after distraction, after consolidation, and one year postoperatively. The data obtained was tabulated and a Mann Whitney U-test was used for statistical comparisons. Results: At the time of presentation, a residual maxillary hypoplasia was observed with a well maintained distraction gap on the OPG which ruled out the occurrence of a relapse. Favorable movement of the segments without any resistance was seen in all patients. Mean maxillary advancement of 10.56 mm was achieved at repeat AMD. Statistically significant increases in midfacial length, SNA angle, and nasion perpendicular to point A distance was achieved (P=0.012, P=0.011, and P=0.012, respectively). Good profile was achieved for all patients. Minimal transient complications, for example anterior open bite and bleeding episodes, were managed. Conclusion: Addressing the problem of cleft maxillary hypoplasia at an early age (12-15 years) is beneficial for the child. Residual hypoplasia may develop in some patients, which may require additional corrective procedures. The results of our study show that AMD can be repeated when residual deformity develops with the previous procedure having no negative impact on the results of the repeat procedure.

• 대한치과의사협회지
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• 제55권12호
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• pp.870-882
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• 2017

Maxillary growth is hindered by the restricting pressure from the scar tissue formed after lip closure and palate closure surgeries of the cleft. Therefore, the anteroposterior skeletal relationship of both jaws exacerbates as patient grows. Conventional facemask treatment is valuable for dentoalveolar compensatory treatment and for very mild maxillary hypoplasia. To achieve further maxillary protraction, bone-anchored facemask or bone-anchored maxillary protraction can be attempted. For moderate maxillary hypoplasia, surgical orthodontic treatment after growth completion can be an efficient treatment reducing uncontrollable problems. For moderate to severe maxillary hypoplasia, distraction osteogenesis (DO) can be used alone or with later surgical orthodontic treatment. To compensate the severe relapse after DO, overcorrection and bone plate placement after DO are recommended. In case of hypernasality, maxillary anterior segmental distraction osteogenesis can be chosen to prevent exacerbation of the hypernasality.

• Journal of Chest Surgery
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• 제29권5호
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• pp.554-558
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• 1996

대동맥 협부 발육부전, 동맥관 개존, 대동맥기시 우폐동맥을 동반한 원위 대동맥폐동맥 중격결손을 가진 생후 50일된 여아에서 동맥관 결찰,대동맥 협부 발육부전 부위의 절제와 대동맥의 단단문합,우폐동맥과 주폐동맥의 직접 연결과자가심낭편을이용한대동맥폐동맥 중격결손의 봉합을 포함한 일차 완전 교정술을 인공 체외 순환과 완전 순환 정지하에 시행하여 좋은 수술 결과를 얻었다.

• Journal of Genetic Medicine
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• 제14권1호
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• pp.27-30
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• 2017

Adrenal hypoplasia congenita (AHC) is a rare cause of adrenal insufficiency during neonatal period. Mutations in the gene coding for DAX1 cause X-linked adrenal hypoplasia. Most affected patients are shown to have salt wasting and hyperpigmentation on the skin during the neonatal period and require intensive medical care. In addition, it is usually associated with hypogonadotropic hypogonadism in adolescence. The DAX1 gene is expressed in the adrenal cortex, pituitary gland, hypothalamus, testis, and ovary. We report on a patient with genetically confirmed AHC whose initial clinical presentations were consistent with congenital adrenal hyperplasia. A point mutation in the DAX1 gene identified in this report resulted in a truncated DAX1 protein. Our patient was diagnosed with AHC.

• Journal of Medicine and Life Science
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• 제19권3호
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• pp.121-124
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• 2022

This case report describes a case of isolated (not associated with other anomalies) lower vaginal hypoplasia complicated by megalourethra (urethral dilatation) due to frequent urethral sexual intercourse in a regularly menstruating (hypomenorrheic) woman. The patient was a 24-year-old woman who underwent transperineal ultrasonography as well as magnetic resonance imaging followed by sequential cystoscopic and vaginoscopic examinations. Finally, a pull-through procedure was used to create a new vagina. The main outcome measure was patent vagina formation. The procedure successfully corrected isolated lower vaginal hypoplasia. The postoperative course was smooth, with the restoration of regular intercourse in the correct location. It was concluded that all gynecologists should have increased awareness of rare female genital anomalies to avoid serious sequelae, particularly in newly married patients.

• 대한구순구개열학회지
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• 제12권2호
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• pp.85-94
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• 2009

Cleft lip and palate patients show midface hypoplasia, maxillary hypoplasia due to scar of previous surgery, and manifest as a class III malocclusion, retruded midface and shallow palate. These deformities have been treated with traditional orthognathic surgery. Although conventional Le Fort I osteotomy was performed on most cleft patinets with midface hypoplasia, it showed limited amount of maxillary advancement and high relapse tendency. Recently, when great amount of advancement are required in severe maxillary hypoplasia, distraction osteogenesis using RED system is widely used. But, several months of consolidation period is needed after distraction osteogenesis, occlusal relationship is not stable until mandibular setback surgery has done in mandibular hyperplasia cases and during these period, patients may feel discomfort. We present clinical cases of immediate rigid internal fixation after completion of maxillary distraction using RED system and simultaneous mandibular setback procedure in adult cleft and lip patients who show both maxillary hypoplasia and mandibular prognathism.

• Journal of Chest Surgery
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• 제36권9호
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• pp.683-686
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• 2003

우심실 형성부전이 있으면서 단신방, 그리고 스펀지 좌심실이 있는 매우 드문 질환인 독립된 우심실 형성부전 환아를 경험하였다. 수술 전 우심실의 크기는 좌심실의 반이었으며 삼첨판막의 크기는 z-value로 -4이었다. 수술 시 6세였으며 수술은 양심실성 교정을 시도하여 심방을 2개로 나누어주었으며 3mm 구멍을 남겨 두었다. 수술 후 경과는 좋았으며 외래 추적에서 우심실 기능 부전소견은 없었다. 술 후 27개월에 심도자가 시행되었으며 삼참판막은 잘 자랐으며(z-value=-0.4), 심방중격의 구멍은 저절로 막혔음을 확인하였다. 독립된 우심실 형성부전을 경험하여 성공적으로 양심실성 교정을 시행하였기에 보고하는 바이다.