국민건강보험공단 자료에 따른 다지증 및 합지증의 통계 분석

김종명,박명철,이승헌,나동균,정재호 대한성형외과학회 2003 Archives of Plastic Surgery Vol.30 No.6

The feature of congenitial hand anomalies is multiple and complex. Polydactyly and syndactyly is one of the most common congenital anomalies of the upper limb. The statistical analysis of these anomaly is the basic study of congenital hand anomalies. However, the number of reports is small. The purpose of this study is to compare and analyze objective data and make a nation-wide statistical report on polydactyly and syndactyly. We used data from the National Health Insurance Corporation for reviewing polydactyly and syndactyly. We reviewed 3 years of data from 1998 to 2000 focusing on age, the number of operations, male to female ratio, the duration of hospital care and cost.The number of operations for polydactyly was 694 cases and for syndactyly was 574 cases. Therefore we may assume that polydactyly occurs 1:910 births and syndactyly occurs 1:1101 births. The duration of hospital care for polydactyly was 12 days and for syndactyly was 17 days. The full cost for the correction of polydactyly was 770,000 won and syndactyly was 1,220,000 won. The ratio of polydcatyly and syndactyly was 1.12:1. The operation for polydactyly and syndactyly was performed between the age of 1 and 2 in most cases. Male to female ratio of polydactyly was 1.14 :1 and syndactyly was 1.39:1. This study will be helpful to find appropriate treatment for congenital hand anomalies.

오각형피판과 최소한의 피부이식을 이용한 합지증의 교정

배병만,어수락,김인규,고성훈,Neil F. Jones 대한성형외과학회 2007 Archives of Plastic Surgery Vol.34 No.1

Purpose: The key of treatment in syndactyly is to separate the fused digits safely, and to create a normal web space with enough cutaneous coverage. Despite many techniques have described the correction of syndactyly, skin graft still remains the annoying one. We designed the pentagonal flap from hand dorsum to reconstruct the web space reliably and try to minimize the need for skin graft. Methods: Between July 2003 and August 2005, six cases of syndactyly were corrected at UCLA Medical Center and Hallym University Sacred Heart Hospital using dorsal pentagonal flap for web space reconstruction and straight incisions for the sides of digits to minimize the need for skin graft. The proximal edge of the pentagonal flap was designed in V shape to allow for easy closure of the donor site after advancement. The pentagonal flap was advanced volarly with the underlying dermofat tissues to form a digital web. In some cases, skin defects were unavoidable and covered with full thickness skin graft from the inguinal area. Results: Syndactyly were seen in 4 cases of Apert syndrome, 1 postburn scar webbing with PIP joint contracture and 1 recurrence after the incomplete reconstruction. In all Apert syndrome, straight line incision was used along the sides of the fingers and skin graft was needed. But, in 2 cases of incomplete type, we could save the need for skin graft only for the correction of syndactyly. We could get a good looking web space without any complications such as flap or graft loss.Conclusion: As a modification of Sherif's V-Y dorsal metacarpal flap, we believe pentagonal flap could be one of the easiest and safest way to reconstruct the web space of syndactyly in functional and cosmetic standpoint.

Foot Syndactyly: A Clinical and Demographic Analysis

김종호,김병준,권성택 대한성형외과학회 2016 Archives of Plastic Surgery Vol.43 No.6

Background Syndactyly of the foot is the second most common congenital foot anomaly. In East Asia, however, no large case study has been reported regarding the clinical features of isolated foot syndactyly. In this study, we report a review of 118 patients during the last 25 years. Methods We conducted a chart review of patients who underwent surgical correction for foot syndactyly between January 1990 and December 2014. Operations were performed with a dorsal triangular flap and a full-thickness skin graft. The demographics of included patients and their clinical features were evaluated. Surgical outcomes and complications were analyzed. Results Among 118 patients with 194 webs (155 feet), 111 patients showed nonsyndromic cases and 7 patients showed syndromic cases. In 80 unilateral cases (72.1%), the second web was the most frequently involved (37.5%), followed by the fourth (30%), the first (15%), the third (15%), the first and second in combination (1.3%), and the second and third in combination (1.3%). Among 31 bilateral cases, 2 cases were asymmetric. Among the remaining 29 symmetric bilateral cases, the second web was the most frequently involved (45.2%), followed by the first (22.6%), and the fourth (6.5%). No specific postoperative complications were observed, except in the case of 1 patient (0.51%) who required a secondary operation to correct web creep. Conclusions This retrospective clinical study of 118 patients with both unilateral and bilateral foot syndactyly revealed that the second web was the most frequently involved. In addition, complete division and tension-free wound closure with a full-thickness skin graft of sufficient size showed good postoperative results.

Foot Syndactyly: A Clinical and Demographic Analysis

Kim, Jong Ho,Kim, Byung Jun,Kwon, Sung Tack Korean Society of Plastic and Reconstructive Surge 2016 Archives of Plastic Surgery Vol.43 No.6

Background Syndactyly of the foot is the second most common congenital foot anomaly. In East Asia, however, no large case study has been reported regarding the clinical features of isolated foot syndactyly. In this study, we report a review of 118 patients during the last 25 years. Methods We conducted a chart review of patients who underwent surgical correction for foot syndactyly between January 1990 and December 2014. Operations were performed with a dorsal triangular flap and a full-thickness skin graft. The demographics of included patients and their clinical features were evaluated. Surgical outcomes and complications were analyzed. Results Among 118 patients with 194 webs (155 feet), 111 patients showed nonsyndromic cases and 7 patients showed syndromic cases. In 80 unilateral cases (72.1%), the second web was the most frequently involved (37.5%), followed by the fourth (30%), the first (15%), the third (15%), the first and second in combination (1.3%), and the second and third in combination (1.3%). Among 31 bilateral cases, 2 cases were asymmetric. Among the remaining 29 symmetric bilateral cases, the second web was the most frequently involved (45.2%), followed by the first (22.6%), and the fourth (6.5%). No specific postoperative complications were observed, except in the case of 1 patient (0.51%) who required a secondary operation to correct web creep. Conclusions This retrospective clinical study of 118 patients with both unilateral and bilateral foot syndactyly revealed that the second web was the most frequently involved. In addition, complete division and tension-free wound closure with a full-thickness skin graft of sufficient size showed good postoperative results.

V-M 성형술을 이용한 지간구축 및 합지증의 교정 임상례

김의식,박상렬,황재하,김광석,이삼용,Kim, Eui-Sik,Park, Sang-Ryul,Hwang, Jae-Ha,Kim, Kwang-Seog,Lee, Sam-Yong 대한성형외과학회 2010 Archives of Plastic Surgery Vol.37 No.1

Purpose: The loss of web space is caused by congenital syndactyly or acquired burn injury, trauma or surgery. Numerous surgical procedures have been described for restoration of the web space. Local flaps are usually preferred because of the easiness to perform and tolerable postoperative outcome. Among the various local flaps, the authors introduce V-M plasty for correction of web space contracture and syndactyly. Method: From March 2007 to Jun 2008, 4 patients underwent V-M plasty for correction of web space contracture and syndactyly. V-M plasty consists of 3 distinct triangular flaps. One triangular flap is designed next to the web region on the dorsal site of the hand, whereas the remaining 2 triangular flaps are placed on the volar site. The dorsal triangular flap is then placed between the volar adjacent triangular flaps. At the end of the operation, the involved fingers or toes are positioned in abduction to avoid kinking of the triangular flaps. Result: All the patients gained web functions with good esthetic appearance without any recurrence or complications. Mean follow-up was 8 months. Conclusion: V-M plasty is a safe, easy and rapid procedure to design and apply by using local tissues without the needs for a skin graft or risk of linear scarring and recurrence. The authors advise this versatile technique both in primary and recurrent cases of web space contracture and syndactyly.

Surgical Treatment of Syndactyly of Harlequin Ichthyosis

Sung Jae Ahn,Jong Won Hong 대한수부외과학회 2018 대한수부외과학회지 Vol.23 No.3

Harlequin ichthyosis (HI) is a rare and most severe form of autosomal recessive congenital ichthyoses characterized by excessive hyperkeratosis. The skin anomalies affect the shape of eyes, ears, nose, mouth, and distal extremities. Since HI is very fatal, there is a lack ofdata that documents successful treatment of HI patients with syndactyly. We successfully managed syndactyly in a HI patient by using aconventional method without any complication and we noticed an improvement functionally and aesthetically. Harlequin ichthyosis (HI) is a rare and most severe form of autosomal recessive congenital ichthyoses characterized by excessive hyperkeratosis. The skin anomalies affect the shape of eyes, ears, nose, mouth, and distal extremities. Since HI is very fatal, there is a lack of data that documents successful treatment of HI patients with syndactyly. We successfully managed syndactyly in a HI patient by using a conventional method without any complication and we noticed an improvement functionally and aesthetically.

Almost Unilateral Focal Dermal Hypoplasia

( Solam Lee ),( Sung Jay Choe ),( Sung Ku Ahn ) 대한피부과학회 2017 Annals of Dermatology Vol.29 No.1

Focal dermal hypoplasia, caused by mutations in PORCN, is an X-linked ectodermal dysplasia, also known as Goltz syndrome. Only seven cases of unilateral or almost unilateral focal dermal hypoplasia have been reported in the English literature and there have been no previously reported cases in the Republic of Korea. A 19-year-old female presented with scalp defects, skin lesions on the right leg and the right trunk, and syndactyly of the right fourth and fifth toes. Cutaneous examination revealed multiple atrophic plaques and a brown and yellow mass with fat herniation and telangiectasia that was mostly located on the lower right leg. She had syndactyly on the right foot and the scalp lesion appeared to be an atrophic, membranous, fibrotic alopecic scar. A biopsy of the calf revealed upper dermal extension of fat cells, dermal atrophy, and loss of dermal collagen. A diagnosis of almost unilateral focal dermal hypoplasia was made on the basis of physical and histologic findings. Henceforth, the patient was referred to a plastic surgeon and an orthopedics department to repair her syndactyly. (Ann Dermatol 29(1) 91∼94, 2017)

Free Fat Graft for Congenital Hand Differences

Toshihiko Ogino,Daisuke Ishigaki,Hiroshi Satake,Kousuke Iba 대한정형외과학회 2012 Clinics in Orthopedic Surgery Vol.4 No.1

Background: Free fat graft has been used for the treatment of congenital hand differences. However, there have been a fewreports about the outcome of that treatment. In this study, the outcome of free fat grafts for congenital hand and foot differenceswas investigated. Methods: Fourteen bones with longitudinal epiphyseal bracket, 3 wrists with Madelung deformity, and 5 cases of osseous syndactylywere treated with free fat graft with osteotomy, physiolysis, or separation of osseous syndactyly. Of the fourteen boneswith longitudinal epiphyseal bracket, 9 were treated with open wedge osteotomy with free fat graft and 5 with physiolysis andfree fat graft. The Madelung deformity was treated with physiolysis with free fat graft. For osseous syndactyly, syndactyly releasewith free fat graft was performed fi ve times on four hands. Results: In the fourteen cases with longitudinal epiphyseal bracket, lateral deviation improved in all except two cases after surgery. The average lateral deviation angle changed from 32.5 degrees before surgery to 15.2 degrees after surgery. The average improvementof the lateral deviation angle was 12.2 degrees in the osteotomy group and 20.6 degrees in the physiolysis group. Themean ratio of improvement of the lateral deviation angle to the lateral deviation angle before surgery was 39.4% in the osteotomygroup and 51.2% in the physiolysis group. The Madelung deformity improved after surgery in two cases but there was no improvementin one case. For these conditions, the results were not good enough when surgery was done after age 13 or at age four forseverely hypoplastic brachymesophalangy. Of the 5 cases of osseous syndactyly, reunion of the separated bones occurred in onecase. The grafted free fat should be deep enough to cover the osteotomy site of the bones to prevent reunion of the separatedbones. Conclusions: Physiolysis and free fat graft performed during the growth period can correct the deviation due to longitudinalepiphyseal bracket and Madelung deformity. Free fat graft is also useful to prevent reunion of the bones after separation of osseoussyndcatyly, if the grafted fat is securely fi lled into the space between the separated bones.

Sudden Cardiac Arrest during Anesthesia in a 30-Month-Old Boy with Syndactyly: A Case of Genetically Proven Timothy Syndrome

안효순,최은영,권보상,김기범,배은정,노정일,최정연,박성섭 대한의학회 2013 Journal of Korean medical science Vol.28 No.5

Timothy syndrome, long QT syndrome type 8, is highly malignant with ventricular tachyarrhythmia. A 30-month-old boy had sudden cardiac arrest during anesthesia induction before plastic surgery for bilateral cutaneous syndactyly. After successful resuscitation, prolonged QT interval (QTc, 0.58-0.60 sec) and T-wave alternans were found in his electrocardiogram. Starting β-blocker to prevent further tachycardia and collapse event, then there were no more arrhythmic events. The genes KCNQ1, KCNH2, KCNE1and 2, and SCN5A were negative for long QT syndrome. The mutation p.Gly406Arg was confirmed in CACNA1C, which maintains L-type calcium channel depolarization in the heart and other systems.

Poland 증후군 : 3례 보고

최경수,정의섭,양창렬,김봉천 예수병원 1992 예수병원학술지 Vol.13 No.-