• Clinical and Experimental Pediatrics
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• v.54 no.5
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• pp.219-223
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• 2011

McKusick-Kaufman syndrome (MKS) is an autosomal recessive multiple malformation syndrome characterized by hydrometrocolpos (HMC) and postaxial polydactyly (PAP). We report a case of a female child with MKS who was transferred to the neonatal intensive care unit of Seoul National University Children's Hospital on her 15th day of life for further evaluation and management of an abdominal cystic mass. She underwent abdominal sonography, magnetic resonance imaging, genitography and cystoscopy which confirmed HMC with a transverse vaginal septum. X-rays of the hand and foot showed bony fusion of the left third and fourth metacarpal bones, right fourth dysplastic metacarpal bone and phalanx, right PAP and hypoplastic left foot with left fourth and fifth dysplastic metatarsal bones. In addition, she had soft palate cleft, mild hydronephroses of both kidneys, hypoplastic right kidney with ectopic location and mild rotation, uterine didelphys with transverse vaginal septum and low-type imperforated anus. She was temporarily treated with ultrasound-guided transurethral aspiration of the HMC. Our patient with HMC and PAP was diagnosed with MKS because she has two typical abnormality of MKS and she has no definite complications of retinal disease, learning disability, obesity and renal failure that develop in Bardet-Biedl syndrome, but not in MKS until 33 months of age. Here, we describe a case of a Korean patient with MKS.

• Archives of Plastic Surgery
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• v.38 no.1
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• pp.15-18
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• 2011

Purpose: The anterior iliac crest is a common source for autologous cancellous bone graft. For patients who have previously received cancellous bone grafts from bilateral anterior iliac crests, there may be concerns of whether a sufficient quantity of autologous cancellous bone remains for additional grafts without harvesting it from other sites, such as the posterior iliac crest. Methods: We experienced 3 cases of reharvesting in 2 patients. The diagnosis of the first patient was bilateral facial cleft number 3. This patient received bilateral side cleft alveoloplasty with corticocancellous bone graft from the both anterior iliac crest respectively by a previous surgeon. This patient then needed reharvesting of the anterior iliac crest cancellous bone to correct an ongoing skeletal problem for the bilateral cleft. The other patient had bilateral incomplete cleft of the primary palate. This patient received left side cleft alveoloplasty with cancellous bone graft from the right anterior iliac crest. Before the patient could receive the alveoloplasty on the other side, a radial head osteotomy and cancellous bone graft was performed by orthopedic surgeons who then used the remaining left iliac crest in order to treat a pulled elbow. For the completion of the right side cleft alveoplasty, the anterior iliac crest cancellous bone needed to be reharvested. Prior to the reharvesting, a preoperative computed tomography scan of the pelvis was obtained to assess the maturity of the donor site regeneration. The grafts were then taken from site where a greater amount of regeneration was evident. Results: Long term follow ups showed that the grafts were successfully taken. This sufficient volume was obtainable 14 months after the first harvest. Conclusion: Satisfactory results were achieved after the reharvesting of iliac cancellous bone. Thus, it appears that the reharvesting of the iliac bone is a possible alternative to multiple site grafting, use of allograft or bone substitute materials.

• Archives of Plastic Surgery
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• v.38 no.1
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• pp.77-80
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• 2011

Purpose: We report a patient with DiGeorge syndrome who was later diagnosed as mild metopic synostosis and received anterior 2/3 calvarial remodeling. Methods: A 16-month-old boy, who underwent palatoplasty for cleft palate at Chungnam National University Hospital when he was 12 months old of age, visited St. Mary's Hospital for known DiGeorge syndrome with craniosynostosis. He had growth retardation and was also diagnosed with hydronephrosis and thymic agenesis. His chromosomal study showed microdeletion of 22q11.2. On physical examination, there were parieto-occipital protrusion and bifrontotemporal narrowing. The facial bone computed tomography showed premature closure of metopic suture, orbital harlequin sign and decreased anterior cranial volume. The interorbital distance was decreased (17 mm) and the cephalic index was 93%. Results: After the correction of metopic synostosis by anterior 2/3 calvarial remodeling, the anterior cranial volume expanded with increased interorbital distance and decreased cephalic index. Fever and pancytopenia were noted at 1 month after the operation, and he was diagnosed as hemophagocytic lymphohistiocytosis by bone marrow study. He however, recovered after pediatric treatment. There was no other complication during the 12 month follow up period. Conclusion: This case presents with a rare combination of DiGeorge syndrome and metopic synostosis. When a child is diagnosed with DiGeorge syndrome soon after the birth, clinicians should keep in mind the possibility of an accompanying craniosynostosis. Other possible comorbidities should also be evaluated before the correction of craniosynostosis in patients as DiGeorge syndrome. In addition, postoperative management requires a thorough follow up by a multidisciplinary team of plastic surgeons, neurosurgeons, ophthalmologists and pediatricians.

• Asian Pacific Journal of Cancer Prevention
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• v.17 no.3
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• pp.1251-1254
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• 2016

Background: Oral squamous cell carcinoma (OSCC), the most common malignancy of the oral cavity, shows geographical variation with respect to the age, sex, site and habits of the population. The histolopathologic grade of the tumor is closely related to its tissue of origin. This study was conducted to establish the prevalence of OSCC in relation to patient sex, age, habits and sites of lesions. Materials and Methods: A total of 130 cases of histopathologically diagnosed OSCC were selected for the study, out of which 66, 38 and 26 were well (WD), moderately (MD)and poorly differentiated (PD), respectively. Sections were stained with haematoxylin and eosin and graded according to a modified Borders's system. Then statistically analyzed different grades of OSCC for correlations with other variables. Results: In our study the majority cases of OSCC were found in the 5th to 7th decades of life, males acconting for 53%. The most common site was the buccal mucosa and most cases had habit of tobacco use either in the form of chewing or smoking or both. When the different grades of OSCC were compared with different sites a statistically significant value was observed (P=0.029). Conclusions: The incidence of high grade PD is very much less in female patients but in males such lesions were common. In our location population the buccal mucosa is the most common site due to the tobbaco habits of the patients and majority cases of the buccal mucosa are WD whereas in tongue, floor of the mouth and palate PD are common.

• Asian Pacific Journal of Cancer Prevention
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• v.15 no.4
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• pp.1633-1637
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• 2014

Background: Lymphoma is a malignant neoplasm of lymphoid tissue classified into Hodgkin's and non- Hodgkin's types. It mostly affects lymph nodes although a considerable proportion of Non-Hodgkin's cases occur in extranodal sites. Materials and Methods: Selected cases diagnosed as non-Hodgkin's lymphoma (NHL) during the period of 1980 to 2012 were retrieved from the archives of the Oral Pathology Diagnostic Laboratory, Faculty of Dentistry, University of Malaya. The sections from the formalin-fixed paraffin embedded tissue blocks were stained with H&E as well as with LCA, CD20, and CD3. Results: The mean age was 41.6 years with a male: female ratio of 1.3:1. Out of the forty two cases, nineteen were Malays, eighteen were Chinese, followed by Indians (3) and Indonesians (2). The most common site of involvement was the mandible (22.2%), followed by the maxilla and palate (19.4% each). Most of the lesions presented as a painless progressive swelling. Only thirty six cases were further subdivided into B or T cell types. The majority were B-cell type (26 cases), of these 6 cases were Burkitt's lymphomas. Only ten cases were T-cell lymphoma, with three cases of NK/T-cell lymphoma. Conclusions: In this series of 42 patients diagnosed as extranodal non-Hodgkin's lymphoma, the lesions appeared as painless swellings, mostly in men with the mandible as the most frequent site of involvement. Majority were B-cell lymphomas with Malays and Chinese being equally affected whereas lymphomas were rare in the Indian ethnicity. T-cell lymphomas were found to be common in the Chinese ethnic group.

• Korean Journal of Cleft Lip And Palate
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• v.8 no.1
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• pp.31-37
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• 2005

In alveolar deformity of cleft patient, the flap design is very important to make the functional and esthetic outcome. Especially in bilateral cleft alveolus with wide defect, deficiency of covering tissue is a greatest problem. Wound dehiscence may develop oronasal fistula of palatal and labial region and loss of the bone graft. We report 2 cases with bilateral cleft alveolus. In both case, bilateral buccal mucosal flap was used for closure of bilateral cleft alveolus with wide defect. The one was operated with iliac bone graft according to secondary grafting method, the other was closed without bone grafting. The patient was 3 years old. So, secondary alveolar bone graft will be required some years later for the establishment of bony continuity and esthetic advantage. In both cases, we found the entire soft tissue closure without the lack of covering flap. In these case, the closure of alveolus defect was accomplished successfully by the use of bilateral buccal mucosal flap. There was no complication, secondary fistula. The most important thing is the tension-free closure of the bilateral buccal mucosal flap. So, we report these cases with literatures.

• Korean Journal of Cleft Lip And Palate
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• v.7 no.2
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• pp.77-91
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• 2004

본 연구의 목적은 반안면왜소증 환자의 하악골 신장술시 초기 치아골격 특성들 중에서 치료결과의 차이에 기여하는 인자를 찾는 것이다. 치료전의 치아골격 특성, 골신장술의 효과와 그 유지상태를 관찰하기 위하여 골신장술 직전(T0), 직후(T1), 추적 2년후(T2)에 측모와 정모 두부방사선 계측사진을 촬영하여 전후방, 수직치아, 비대칭 항목들을 계측하였다. T2 시기의 계측 결과에 따라서 환자들을 1군(양호군, 10명)과 2군(불량군, 9명)으로 분류하였다. 두 군에서 각 시기와 T0-T1, T1-T2 동안의 변화량의 차이를 Mann-Whitney U test, Wilcoxon signed independent t-test, rank test, ANOVA test를 사용하여 분석하였다. pruzansky type이 골신 장술의 성공과 실패 여부와 관계가 깊게 나타났다. T0 시기에 2군은 1군에 비하여 하악골이 후방위치되었고, 하악지 고경(ramus height)이 짧았고, 하악각(gonial angle)이 컸으며, 이환측 하악지가 내측경사되었고, 이환측으로의 이부변위(chin point deviation)가 크게 나타났다. 1군에서 골신장술의 주요한 효과는 하악지 고경의 증가, 하악골의 전방위치, 하악각의 증가, articular angle의 감소에 따른 하약골의 반시계방향 회전, 이환측의 하악지 경사의 증가, 교합면경사와 이부변위의 개선으로 나타났다. 그러나 2군에서는 골신장술을 시행했을 때 1군에 비하여 하악골이 시계방향으로 회전되었고 하악지 고경의 증가량이 작게 나타났다. T2 시기에 2군에서는 하악골의 반시계방향 회전이 나타났고, 하악지 성장이 일어나지 않았으나, 1군은 반대의 경향을 보였다. 이러한 인자들이 골신장술 결과의 차이에 기여하는 것으로 생각된다.

• Korean Journal of Cleft Lip And Palate
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• v.7 no.2
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• pp.133-139
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• 2004

Congenital bilateral idiopathic hyperplasia of the coronoid processes presents with limited mouth-opening without visible maxillofacial deformity or temporomandibular joint dysfunction / disorder. According to Blanchard et al and McLoughlin et al, it was lnitially described in 1853 by Langenbeck, and the first cases were reported by Holmes in 1956. Since then, there have been regular reports of a certain number of cases. In 1995, McLoughlin et al recorded 79 published cases of bilateral hyperplasia of the coronoid processes. Among them, Fabie et al have found only 3 published cases relating to children younger than 8 years, and have presented the first case of objectively diagnosed restricted mouth opening from birth by pediatricians. Authors have experienced 2 child patients with mouth opening limitation who was diagnosed congenital bilateral idiopathic hyperplasia of the coronoid processes without any other TNJ morphology in Dept of Oral and Maxillofacial surgery, Seoul National University Dental Hospital in 2004. Coronoidectomy was performed and postoperative active mouth opening exercise is indicated. Authors report 2 cases of congenital bilateial idiopathic hypeiplasia of the coronoid processes with literature liview.

• Korean Journal of Cleft Lip And Palate
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• v.14 no.1_2
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• pp.1-18
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• 2011

Skeletal malocclusion is the result of abnormal dimension and alignment of each skeletal component. Understanding on these mechanisms may help to elucidate the etiology of skeletal malocclusion and to establish population-oriented treatment plans. Attempts to subdivide the Angle's classification have been performed for Class III malocclusion, while few studies have been conducted for Class II malocclusion despite recent growing interests in Class II malocclusion. 200 adults (88 male, 112 female) with skeletal Class II malocclusion were collected and subdivided using cluster analysis, using the measurements representing the dimension and the alignment of each facial skeletal component. The properties of each cluster was grouped within the subjects and a comparison between the subjects and the control group (38 male, 35 female) with normal occlusion was performed. Six clusters were finally recognized in each male and female groups. The clusters in both genders were mainly characterized by the cranial base alignment, dimension of the posterior cranial base, dimension of the mandibular ramus and the degree of mandibular rotation. The results implicate that active treatment of mandible rather than the nasomaxillary complex may be primarily considered for the correction of Korean Class II skeletal pattern.

• Korean Journal of Cleft Lip And Palate
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• v.16 no.1
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• pp.37-49
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• 2013

Cleft lip is a common congenital facial deformity which might cause speech, hearing, appearance, and psychological disorder. For the purpose of appropriate management for the cleft lip patients according to their individual situations, reviews of the historical evolution for the cleft lip treatment were summarized. More than 15 English written articles with 4 related historical books were reviewed, and the chronology of the cleft lip management from ancient to recent twenty first century, via middle ages and Renaissance, were summarized. Multifactorial causes of cleft lip, before the modern understanding of embryological background of it, most management of cleft lip has been explained under the basis of religions and/or superstitions. As the anatomic and embryologic knowledges were known and revealed, various misconceptions were corrected continously, and the simple closure of the lip defect was also evoluted to the applications of plastic concept. Recently, cosmetic outcomes with functional results, such as speech, hearing, psychological status, have been considered importantly, under the multidiciplinary care system. For the better understanding of cleft lip management as a routine esthtetic and funtional reconstructive procedure, the various historical treatment trends were reviewed and summarized as time goes on. This review presentation will discuss the appropriate management for cleft lip patients.