El ve ayağın polidaktilisi: Üç olgunun sunumu ve polidaktili olgularında güncel prenatal ve postnatal yaklaşımların gözden geçirilmesi

Öz

Polidaktili el ve ayağın yaygın doğumsal bir anomalisidir ve 500 doğumda 1 görülür. Polidaktili tek başına olabileceği gibi bir sendromun parçası olabilir ya da kromozomal bir anomaliye eşlik edebilir. Polidaktili ile ilgili bazı bilinmeyenler mevcuttur. Biz bu yazıda polidaktilinin bulunabileceği üç formu kapsayan olgular sunduk. İlki bilateral multikistik displastik böbrek ve polidaktili, ikincisi izole polidaktili tip B ve üçüncü olgu Trizomi 13'e eşlik eden bir polidaktili vakasıdır. Amacımız, polidaktilili hastalarda prenatal danışmanlık ve postnatal yönetimde en iyi multidisipliner yaklaşımı değerlendirmede yardımcı olabilecek güncel bilgileri sunmak için literatürü gözden geçirmektir.

Anahtar Kelimeler

• Polidaktili,Kol ve bacak deformasyonları, doğumsal,Düşük, istemli,Doğum öncesi tanı,Amniyosentez,Ultrasonografi, prenatal,Süt çocuğu, yenidoğan,Otopsi

Polydactyly oh the hands and feet: Report of three cases and review of the literature for current

Öz

Prenatal and postnatal optimal approaches in cases with polydactyly Polydactyly is a common birth deformity of the hands or feet with a prevalence of 1 in 500 births. It can occur in isolation or as part of a syndrome. It may also be associated with a chromosomal anomaly. Some facts about polydactyly still remain unknown. In this paper, we presented cases including the three forms in which polydactyly could be found. The first patient was a case of bilateral multicystic dysplastic kidney. The second patient was isolated polydactyly type B. The third patient was Trisomy 13. Our aim was to review the literature to present current knowledges that may help to assess the best multidisciplinary approach in prenatal counselling and in postnatal management in patients with polydactyly.

Anahtar Kelimeler

• Polydactyly,Limb Deformities, Congenital,Abortion, Induced,Prenatal Diagnosis,Amniocentesis,Ultrasonography, Prenatal,Infant, Newborn,Autopsy

Kaynakça

1. 1.Castilla EE, Lugarinho da Fonseca R, da Graca Dutra M, et al. Epidemiological analysis of rare polydactylies. AmJMed Genet 1996;65(4):295
2. 2.Kumar P, Manimegalai M, Premalatha S. Ainhüm in supernumery fingers. Indian JDermatol Venereol Leproll995;61(6):376
3. 3.Castilla EE, Lugarinho R, da Graca Dutra M, et al. Associated anomalies in individuals with polydactyly. Am J Med Genet 1998;80(5):459
4. 4.Mungen E, Dündar O, Muhcu M, et al. Placental mesenchymal dysplasia associated with trisomy 13: sonographic findings. J Clin Ultrasound 2008;36(7):454
5. 5.Phelps DA, Grogan DP. Polydactyly of the foot. JPediatr Orthop 1985;5(4):446
6. 6.Philip-Sarles N. Genetics of congenital hand malformations. Chir Main 2008;27 Suppl 1:S7
7. 7.Venn-Watson E A. Problems in polydactyly of the foot. Orthop Clin North Am 1976;7(4):909
8. 8.Zguricas J, Bakker WF, Heus H, et al. Genetics of limb development and congenital hand malformations. Plast Reconstr Surg 1998;101(4):1126

9. 9.Kapetanos GA. Mixed polydactyly. An unusual case of a patient with seven-toed feet. Clin Orthop RelatRes 1984(186) :220
10. 10.Zhao H, Tian Y, Breedveld G, et al. Postaxial polydactyly type A/B (PAP-A/B) is linked to chromosome 19pl3.1-13.2 in a Chinese kindred. EurJHum Genet 2002; 10(3): 162
11. 11.Netscher DT, Baumholtz MA. Treatment of congenital upper extremity problems. Plast Reconstr Surg2007;119(5):101e
12. 12.Wassel HD. The results of surgery for polydactyly of the thumb. Clin Orthop RelatRes 1969;64:175