• Archives of Plastic Surgery
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• 제32권4호
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• pp.474-478
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• 2005

Craniosynostosis is a congenital anomaly characterized by premature closure of cranial sutures. Surgical intervention should be performed during infancy. However, surgical correction of craniosynostosis remains bone defect and secondary angle occasionally. Currently, publications investigating solutions to bone defect and secondary angle created by cranioplasty are getting much interest. We have used $BoneSource^{(R)}$ which is relatively safe as an implantable substance for providing solutions for this problem. From June 2002 to January 2004, five children with craniosynostosis underwent frontocalvarial contouring using $BoneSource^{(R)}$ and concurrent cranial vault remodeling. The patient ages ranged from 8.0 months to 4.9 years(mean, 2.5 years). The quantity of $BoneSource^{(R)}$ implanted ranged from 10 to 25g, with a mean of 13g. This paper presents the first series of children treated with $BoneSource^{(R)}$ for frontocalvarial contouring in the surgical correction of craniosynostosis. No patients experienced any complications. Our results shows excellent retention of contour without causing asymmetry or irregularity. No visible evidence of interference with craniofacial growth were observed. Through our experiences, $BoneSource^{(R)}$ is found to be very useful for frontocalvarial contouring in children undergoing correction of craniosynostosis.

• Archives of Plastic Surgery
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• 제32권2호
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• pp.189-193
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• 2005

Reconstruction of calvarial bone defects from congenital anomaly or from bone loss due to traumatic or neoplastic processes remains a significant problem in craniofacial surgery and neurosurgery. To facilitate bone regeneration, there have been many trials such as autologous bone graft or allograft, and the addition of demineralized bone matrix and matrix-derived growth factor. Guided bone regeneration is one of the methods to accelerate bone healing for calvarial bone defects especially in children. Pericranium is one of the most usable structure in bone regeneration. It protects the dura and sinus, and provides mechanical connection between bone fragments. It supplies blood to bone cortex and osteoprogenitor cells and enhances bone regeneration. For maximal effect of pericranium in bone regeneration, authors used pericranium as a flap for covering calvarial defects in surgeries of 11 craniosynostosis patients and achieved satisfactory results: The bone regeneration of original cranial defect in one year after operation was 74.6%(${\pm}8.5%$). This pericranial flap would be made more effectively by individual dissection after subgaleal dissection rather than subperiosteal dissection. In this article, we reviewed the role of pericranium and reported its usefulness as a flap in surgery of craniosynostosis to maximize bone regeneration.

• 대한구순구개열학회지
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• 제6권1호
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• pp.53-58
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• 2003

Lateral facial cleft is a rare congenital craniofacial anomaly. Their incidence is about 1 in 60,000 births and early treatment with closure in layers is important. Surgical treatment must reconstruct an anatomic and functional situation which give a good esthetic result. This study reports two cases of male at 31 months and 9 months suffered from esthetic problems due to lateral displacement of the oral commisure on the right side. Surgical treatment; the mucosa is closed in with a Z-plasty to avoid intraoral band contracture, the orbicularis oris muscle is closed after interfiber reorientation, and the skin is closed with multiple Z-plasty. So we report two cases of unilateral lateral facial cleft and review of etiopathogenesis in children with lateral facial cleft.

• Clinical and Experimental Pediatrics
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• 제57권6호
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• pp.292-296
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• 2014

The deletion of the distal long arm of chromosome 1 is associated with a characteristic facial appearance and a pattern of associated malformations. Characteristic manifestations include a round face with prominent 'cupid's bow' and downturned corners of the mouth, thin vermilion borders of lips, a long upper lip with a smooth philtrum, a short and broad nose, epicanthal folds, apparently low-set ears, micrognathia, microcephaly, abnormal hands and feet, variable cardiac or genital anomalies, moderate to severe mental retardation, and growth retardation. Using fluorescent in situ hybridization (FISH) analysis to map precisely the deletion, we present a case of chromosome 1q44 deletion with craniofacial characteristics, multiple congenital anomalies, and growth and psychomotor retardation. In comparison with other reported cases of 1q43-44 deletion, the subject does not show hydrocephalus, seizure, syn- or polydactyly of hands, and a urogenital anomaly. However, an arachnoid cyst, pinpoint dimple on the midline of the forehead, a right-sided supernumerary nipple and auricular pit, polydactyly of the right foot, adducted thumb, and flexion restriction of the proximal interphalangeal joint with a simian line in both hands were observed additionally.

• Archives of Plastic Surgery
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• 제33권2호
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• pp.237-240
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• 2006

The simultaneous correction of the hypertelorism and exophthalmos combined with craniosynostosis is very rarely performed operative procedures in the world. The craniosynostosis is the congenital anomaly that designates premature fusion of one or more sutures in either cranial vault or cranial base. Hypertelorism is not a distinct clinical syndrome in itself, but is a physical finding secondary to facial and cranial maldevelopment and it is defined as a increase in the distance between the medial orbital walls. Exophthalmos can occur following the decrease in the size of the orbit in patients with developmental skeletal disorders such as craniofacial synostosis. The authors experienced 9-year-old male patient, who has complex cranio-facial abnormality. The craniosynostosis was oxycephaly type and primary fronto-orbital advancement surgery had been performed in other hospital. The abnormal cranial vault combined with hypertelorism and exophthalmos due to maldeveloped both orbital walls. Surgical correction was obtained by various cranio-fronto-orbital remodeling technique such as calvarial bone craniotomy, fronto-orbital advancement, paramedian resection, medial canthopexy, Tessier-Wolfe three wall orbital expansions. We achieved a quite satisfactory result both functionally and aesthetically in a complex cranio-facial deformity patient by combination and modification of previously developed various cranio-facial plasty technique and hereby report the case with brief discussion and review of literature.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제37권
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• pp.3.1-3.7
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• 2015

Hemifacial microsomia (HFM) is the most common craniofacial anomaly after cleft lip and cleft palate; this deformity primarily involves the facial skeleton and ear, with either underdevelopment or absence of both components. In patients with HFM, the management of the asymmetries requires a series of treatment phases that focus on their interception and correction, such as distraction osteogenesis or functional appliance treatment during growth and presurgical orthodontic treatment followed by mandibular and maxillary surgery. Satisfactory results were obtained in a 9-year-old girl with HFM who was treated with distraction osteogenesis. At the age of 19, genioplasty and mandible body augmentation with a porous polyethylene implant (PPE, $Medpor^{(R)}$, Porex) was sequentially performed for the functional and esthetic reconstruction of the face. We report a case of HFM with a review of the literature.

• Clinical and Experimental Pediatrics
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• 제60권11호
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• pp.353-358
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• 2017

Purpose: To develop and evaluate a simple screening tool to assess hearing loss in newborns. A derived score was compared with the standard clinical practice tool. Methods: This cohort study was designed to screen the hearing of newborns using transiently evoked otoacoustic emission and auditory brain stem response, and to determine the risk factors associated with hearing loss of newborns in 3 tertiary hospitals in Northern Thailand. Data were prospectively collected from November 1, 2010 to May 31, 2012. To develop the risk score, clinical-risk indicators were measured by Poisson risk regression. The regression coefficients were transformed into item scores dividing each regression-coefficient with the smallest coefficient in the model, rounding the number to its nearest integer, and adding up to a total score. Results: Five clinical risk factors (Craniofacial anomaly, Ototoxicity, Birth weight, family history [Relative] of congenital sensorineural hearing loss, and Apgar score) were included in our COBRA score. The screening tool detected, by area under the receiver operating characteristic curve, more than 80% of existing hearing loss. The positive-likelihood ratio of hearing loss in patients with scores of 4, 6, and 8 were 25.21 (95% confidence interval [CI], 14.69-43.26), 58.52 (95% CI, 36.26-94.44), and 51.56 (95% CI, 33.74-78.82), respectively. This result was similar to the standard tool (The Joint Committee on Infant Hearing) of 26.72 (95% CI, 20.59-34.66). Conclusion: A simple screening tool of five predictors provides good prediction indices for newborn hearing loss, which may motivate parents to bring children for further appropriate testing and investigations.

• Journal of Genetic Medicine
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• 제8권1호
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• pp.67-70
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• 2011

체질성 8삼염색체 모자이시즘은 비교적 드문 염색체 이상으로 특이한 두개안면 모양 (변형 두개골, 돌출된 이마, 낮거나 변형된 귀), 골격계 기형, 심장 기형, 신장 기형, 잠복고환, 다양한 정도의 발달지체 등의 특징적인 표현형을 갖는다. 그 표현형과 세포유전학적 표현의 다양성 때문에 특정 질환이 생기기 전까지 진단이 늦어지기도 한다. 전형적인 표현형 없이 속발성 무월경을 동반한 28세 여성에서 발견된 체질성 8삼염색체 모자이시즘(47,XX,+8[9]/46,XX[41])을 보고한다.

• Archives of Plastic Surgery
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• 제38권4호
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• pp.485-489
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• 2011

Purpose: Median cleft of upper lip is defined as any congenital vertical cleft through the midline of the upper lip. It is uncommon, its embryological pathogenesis remains unexplained to date. The authors hereby report a rare case of median cleft of the upper lip associated with enlarged frenulum and palatal mass. This case offers some understanding of the possible embryologic development of this anomaly. Methods: A 10-month-old boy born by normal vaginal delivery at full-term had a notch in the midline of the upper lip with widened philtrum along with enlarged median frenulum, alveolar cleft, and mass of the hard palate. We performed en bloc resection of the enlarged frenulum and palatal mass and cheiloplasty under general anesthesia. Results: Histological examination revealed that the frenulum and palatal mass was consisted of fibrous tissue with normal mucous membrane. The postoperative course was satisfactory. Conclusion: A rare case of median cleft of the upper lip with associated enlarged frenulum and palatal mass was presented with proper surgical management. The surgical technique includes marginal excision of the clefted epithelium and reconstruction of orbicularis oris muscle, in addition to en bloc resection of the palatal mass and frenulotomy.

• Clinical and Experimental Pediatrics
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• 제49권5호
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• pp.507-512
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• 2006

목 적 : 신생아 난청은 신생아 1,000명당 1-3명에서 발생하며 조기에 발견하여 중재하지 않으면 유소아의 언어 습득과 발달 지연에 영향을 미친다. 저자들은 신생아 고위험군을 대상으로 청각 선별검사로서 DPOAE를 이용하여 난청의 유병률과 위험 인자와의 관련성에 대해 조사하였다. 방 법 : 2001년 5월부터 2004년 12월까지 순천향대학교 부천 병원 신생아 집중치료실에 입원하여 청각 선별검사로 변조 이음향방사를 시행받은 871명을 대상으로 의무기록을 후향적으로 검토하였다. 변조 이음향방사에서 통과된 경우 Pass 군으로, 통과되지 못한 경우를 Refer 군으로 나누어 출생 체중, 재태 기간, 산모의 위험 인자, 가족력, 인공호흡기 사용, 빌리루빈 수치, 이독성 약물의 사용 등 청각에 영향을 미치는 인자에 대해 연구하였다. 결 과 : 총 871명 중 106명(12.1%)이 Refer 군으로 우측 이상이 36명(4.1%), 좌측 이상이 23명(2.6%), 양측 이상이 47명(5.4%)이었다. Refer군 106명 중 20명(18.9%)이 3개월 후 변조 이음향방사를 재시행 하였으며 40명(37.7%)에서 ABR를 시행하였다. 총 871명 중 Pass 군과 Refer 군 사이에 성별, 출생 장소(본원 및 외부병원), 청각장애의 가족력, 재태 연령에 따른 출생 체중(부당 경량아, 부당 중량아, 적정 체중아), 산모의 위험 인자(임신성 고혈압, 임신성 당뇨, 조기 양막 파수), 빌리루빈 수치 및 gentamicin 사용 등의 인자와는 유의한 차이가 없었다. 총 871명 중 Pass 군과 Refer 군 사이에 출생 체중 1,500 g 미만, 재태 연령 37주 이하, 심폐 소생술 시행, 낮은 Apgar 점수(1분에 5점 이하, 5분에 6점 이하), 두경부 이상(구개순, 구개열, 이개 앞 피부 돌출), 인공호흡기 사용, 패혈증, vancomycin 사용 등의 인자에서 유의한 차이(P<0.05)를 보였다. 결 론 : 60 dB 이상의 난청이 18명(2%)에서 관찰되었으며 이는 전체 신생아 유병률(1-3명/1,000명)보다 높은 수치이다. 그러므로 신생아 집중치료실 입원 환자에게는 청각 선별검사를 반드시 시행하여야 하며 변조 이음향방사가 선별검사의 방법으로 이용될 수 있다. 또한 이번 연구를 기초로 하여 전체 신생아를 대상으로 한 신생아 청각 선별검사의 보편화 할 것을 추천하며 이상이 있는 경우 더욱 적극적인 추적 관찰이 필요할 것으로 사료된다.