Fetüs Papyraceus ile İlişkili Aplazia Kutis Konjenita

Year 2015, Volume: 13 Issue: 2, 147 - 150, 01.09.2015

Ahmet Özdemir Osman Baştuğ Serra Alçı Levent Korkmaz Hülya Halis Sabriye Korkut Mehmet Adnan Öztürk Tamer Güneş

https://doi.org/10.4274/jcp.04274

Abstract

Aplazia kutis konjenita AKK derinin lokalize veya geniş alanlar şeklinde yokluğu ile karakterize nadir bir anomalidir. Genellikle skalpta görülmekle birlikte, tek veya multipl lezyonlar şeklinde vücudun herhangi bir yerinde bulunabilir. On bin doğumda bir görülen AKK izole bir lezyon olarak ortaya çıkabildiği gibi, yarık damak/dudak, sindaktili, parmakların yokluğu, göz anomalileri ve konjenital kalp hastalıkları ile birlikte görülebilir. AKK’nin oldukça nadir görülen bir formu da intrauterin ex olan ikiz eşine bağlı gelişen formudur. Büyük lezyonlar cerrahi onarıma gerek duyarken, küçük defektler spontan re-epitelizasyon ile iyileşebilir. Bu yazıda; nadir olarak görülen, fetüs papyraceus ile ilişkili olarak gövdesinde AKK gelişen erkek olgu sunulmuştur

Keywords

Aplazia kutis konjenita, re-epitelizasyon, ikiz

Aplasia Cutis Congenita Association with Fetus Papyraceus

Abstract

Aplasia cutis congenita ACC is a rare malformation characterized by localized or a wide area absence of tissue. The disease may involve any site of the body, although usually seen on the scalp. Observed one in 10.000 births, it can occur as the ACC as an isolated lesion and may be associated with cleft palate/lip, syndactyly, absence of fingers, eye abnormalities and congenital heart disease. ACC’s a rare form due to intrauterine twin, which is a form of ex. Large lesions that require surgical repair small defects heal spontaneously with re-epithelialization. We present a rare case of newborn male with truncal ACC associated with fetus papyraceus

Keywords

Aplasia cutis congenita, re-epithelialization, twins

References

• 1. Koshy CE, Waterhouse N, Peterson D. Large scalp and skull defects in aplasia cutis congenita. Br J Plast Surg 200;54:276-7.
• 2. Komuro Y, Yanai A, Seno H, Ichida M, Inoue M, Miyajima M, et al. Surgical treatment of aplasia cutis congenita of the scalp associated with bilateral coronal synostosis. J Craniofac Surg 2002;13:513-9.
• 3. Blouin MM, Bernard J, Caron F, Auger I. Aplasia cutis congenita of the trunk and scalp associated with fetus papyraceus. Int J Dermatol 2011;50:733-5.
• 4. Loreti A, Bracaglia R, Selvaggi G, Lahoud P, Sturla M, Farallo E. Aplasia cutis congenita: report of four cases and literature review. Eur J Plast Surg 2004;27:114-9.
• 5. Rodriguez-Garcia C, Gonzalez-Hernandez S, HernandezMartin A, Perez-Robayna N, Sanchez R, Torrelo A. Aplasia cutis congenita and other anomalies associated with methimazole exposure during pregnancy. Pediatr Dermatol 2011;28:743-5.
• 6. Pajaziti L, Rexhepi S, Shatri-Muça Y, Ferizi M. The role of diclofenack on inducing of aplasia cutis congenita: a case report. Cases J 2009;2:150.
• 7. Landy HJ, Keith LG. The vanishing twin: a review. Hum Reprod Update 1998;4:177-83.
• 8. Livnat EJ, Burd L, Cadkin A, Keh P, Ward AB. Fetus papyraceous in twin pregnancy. Obstet Gynecol 1978;51:41-5.
• 9. Benirschke K. Intrauterine death of a twin: mechanisms, implications for surviving twin, and placental pathology. Semin Diagn Pathol 1993;10:222-31.
• 10. Tempark T, Shwayder TA. Aplasia cutis congenita with fetus papyraceus: report and review of the literature. Int J Dermatol 2012;51:1419-26.
• 11. Mazza JM, Klein JF, Christopher K, Silverberg NB. Aplasia cutis congenita in a setting of fetus papyraceus associated with small fetal abdominal circumference and high alpha-fetoprotein and amniotic acetylcholinesterase. Pediatr Dermatol 2013;32:138- 40.
• 12. Frieden IJ. Aplasia cutis congenita: a clinical review and proposal for classification. J Am Acad Dermatol 1986;14:646-60.
• 13. Ong SS, Zamora J, Khan KS, Kilby MD. Prognosis for the cotwin following single-twin death: a systematic review. BJOG 2006;113:992-8.
• 14. Rasheed T, Orlando A, Gordon DJ. Basal cell carcinoma in aplasia cutis congenita. Br J Plast Surg 2000;53:351-2.
• 15. Wexler A, Harris M, Lesavoy M. Conservative treatment of cutis aplasia. Plast Reconstr Surg 1990;86:1066-71.
• 16. Chan RK, Liu AS, Rogers GF. Aplasia cutis congenita of the trunk associated with fetus papyraceous. J Craniofac Surg 2012;23:995-7.