Aplazia Kutis Konjenita'lı Olguların Değerlendirilmesi ve İzlemi
SUMMARY: Aplasia cutis congenita (ACC) is part of a heterogeneous group of disorders which is characterized by the absence of a portion of skin in a localized or widespread area effecting approximately 1 in 10,000 newborns. There is no sex predilection or increase in the frequency depending on ethnic origin. Lesions are mostly seen on the scalp as a solitary, small (0.5-3 cm) lesions with varying degrees in deepness. Although the association with various syndromes has been defined, it is mostly seen as isolated lesions. If careful examination is not performed and the consequences of the aplasia cutis congenita is not known, underlying bone defects in nonsyndromic cases is unrecognized. Here in we reviewed the diagnosis and management of aplasia cutis congenita with relevant literatüre, by presenting two newborn casesKEY WORDS: Newborn, scalp, skin defects, aplasia cutis congenitaÖZET: Aplazia kutis konjenita (AKK) nadir görülen, doğumda izole ya da yaygın alanlarda cilt yokluğu ile karakterize heterojen hastalık grubudur. İnsidansı 1:10 000’dir. Kız ve erkekleri eşit oranda etkiler. Etnik kökene bağlı sıklığında artış tanımlanmamıştır. Sıklıkla tek, küçük (0.5-3 cm), skalp yerleşimlidir. Çeşitli sendromlarla birlikteliği olsa da, sıklıkla izole lezyon olarak görülür. Kemik yokluğunun da eşlik ettiği olgularda komplikasyon sıklığında artış olabilir. Burada skalpta, kemik yokluğunun eşlik etmediği aplazia kutis konjenita tanısı alan iki yenidoğan sunulmuştur. Özellikle sendromik bulguları olmayan vakalarda, saçlı deride gözden kaçabilecek bir lezyon olması, eşlik edebilecek kemik defektleri açısından değerlendirmenin önemi vurgulanmıştır.ANAHTAR KELİMELER: Yenidoğan, skalp, cilt defekti, aplazia kutis konjenita
Anahtar Kelimeler:
ANAHTAR KELİMELER: Yenidoğan, skalp, cilt defekti, aplazia kutis konjenita
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- Bajpai, M. Pal, K. (2003). Aplasia cutis cerebri with partial acrania total reconstruction in a severe case and review of the literature. J Pediatr Surg.38(2): e4.
- Campbell, W. (1826). Case of a congenital ulcer on the cranium of fetus terminating in haemorrhage on the 18th day after birth. J Med Sci. 2, 82. f a t a l
- Adams, F. H. Oliver, C.P. (1945). Hereditary deformities in man due to arrested development.J Hered.36,3-10.
- Frieden, I.J. (1986). Aplasia cutis congenita: a clinical review and proposal for classification. J Am Acad Dermatol. 14(4), 646-660.
- Moriya, J. Kakeda, S. Korogi, Y. (2006). An unusual case of split cord malformation. Am J Neuroradiol. 27, 1562‑1564.
- Burkhead, A. Poindexter, G. Morrella, D.S. (2009). Case of extensive Aplasia Cutis Congenita with underlying skull defect and central nervous system malformation: discussion of large skin defects, complications, treatment and outcome. J Perinatol. 29, 582-585.
- Verhelle, N.A. Heymans, O. Deleuze, J.P. (2004). Abdominal studies in the pathology and development. Am aplasia cutis congenita: case report and review of the literature. J Pediatr Surg, 39, 237-239.
- Gazioglu, N. Abuzayed, B. Aslan, H. (2012). Repair of the venous sinus defect with bovine pericardium dura substitute in a case aplasia cutis congenita. Neurosurg Quart,22,19-21.
- Elliott, A.M. Teebi, A.S. (1997). Further dominant nonsyndromic aplasia cutis congenita. Am. J. Med. Genet, 73, 495-496. of
- Croce, E.J. Purohit, R.C. Janovski, N.A. (1973). Congenital absence of skin (Aplasia cutis congenita). Arch Surg, 106, 732.
- Chitnis, M.R. Karachi, R. Galea, P. (1996) congenita. Eur. J. Pediatr Surg, 6,100. aplasia cutis
- Glasson, D.W. Duncan, G.M. (1985). Aplasia cutis congenita of the scalp: delayed massive hemorrhage. Reconstr Surg, 75,423. complicated by
- Kruk-Jeromin, J. Janik, J. Rykala, J.
- (1998). Aplasia cutis congenita of the scalp. Report of 16 cases. Dermatol Surg, 24, 549-553.
- Moros, P.M. Labay, M.M. Valle, S.F.
- (2000). Aplasia cutis congenita in a newborn: Etiopathogenic review and diagnostic approach. An Esp Pediatr, 52, 453-456.
- Dallapoiccola, B. Giannotti, A. Marino, B. (1992). Familial aplasia cutis congenita and coarctation of the aorta. Am J Med Genet, 43, 762.
- Levine, S.M. Reformat, D.D. Thorne, C.H. Perioperative Management and Case Report Am J Crit Care, 21, 212-215.
- ISSN: 1305-4953
- Yayın Aralığı: Yılda 6 Sayı
- Başlangıç: 2013
- Yayıncı: Eskişehir Osmangazi Üniversitesi Rektörlüğü