Mermaid syndrome

Öz

Sirenomelia also known as the mermaid syndrome is a very rare congenital anomaly characterized by lower limb fusion and severe urogenital gastrointestinal cardiovasculer central nervous system malformations We report a case of sirenomelia who had a single umblical artery renal agenesis pulmoner hypoplasia esophageal atresia ventricular septal defect anal atresia intestinal atresia and who was lost at fifth hour of life Turk Arch Ped 2013; 48: 65 7

Anahtar Kelimeler

• Caudal dysgenesis, mermaid, sirenomelia

Denizkızı sendromu

Öz

Sirenomeli olarak da adlandırılan denizkızı mermaid sendromu oldukça nadir görülen doğumsal bir bozukluktur Bu sendromda bebeğe denizkızı görünümünü veren yapışık bacakların yanı sıra ağır üreme boşaltım mide bağırsak kalp damar ve merkezi sinir sistemi bozuklukları bulunur Bu yazıda yapışık bacakların yanı sıra göbek kordonunda tek arter böbrek yokluğu akciğer hipoplazisi özofagus atrezisi ventriküler septal defekt anal atrezi bağırsak atrezisi saptanan ve doğumunun beşinci saatinde kaybedilen denizkızı sendromu olgusu sunulmuştur.

Anahtar Kelimeler

• Denizkızı
• kaudal disgenezi
• sirenomeli

Kaynakça

1. Lutz N, Meyrat BJ, Guignard JP, Hohlfeld J. Mermaid syndrome: virtually no hope for survival. Pediatr Surg Int 2004; 20(7): 559-61.
2. Browne M, Fitchev P, Adley B, Crawford SE. Sirenomelia with an angiomatous lumbosacral myelocystocele in a full-term infant. J Perinatol 2004; 24(5): 329-31.
3. Taghavi MM, Jafari Naveh HR, Shariati M, Morteza Zade F. Sirenomelia (mermaid syndrome): an infant from parents who used a special form of snuff. Pak J Biol Sci 2009; 12(9): 722-5.
4. Romano S, Esposito V, Fonda C, Russo A, Grassi R. Beyond the myth: the mermaid syndrome from Homerus to Andersen. A tribute to Hans Christian Andersen's bicentennial of birth. Eur J Radiol 2006; 58(2): 252Stanton MP, Penington EC, Hutson JM. A surviving infant with sirenomelia (Mermaid syndrome) associated with absent bladder. J Pediatr Surg 2003; 38(8): 1266-8.
5. Pinette MG, Hand M, Hunt RC, Blackstone J, Wax JR, Cartin A. Surviving sirenomelia. J Ultrasound Med 2005; 24(11): 1555-9.
6. Sikandar R, Munim S. Sirenomelia, the mermaid syndrome: case report and a brief review of literature. J Pak Med Assoc 2009; 59(10): 721-3. Goodlow OG, Sibley RI, Allen BG, Kamanda WS, Gullattee AC, Rayfield WC. Sirenomelia: mermaid syndrome. J Natl Med Assoc 1988; 80(3): 343-6.
7. Thottungal AD, Charles AK, Dickinson JE, Bower C. Caudal dysgenesis and sirenomelia-single centre experience suggests common pathogenic basis. Am J Med Genet A 2010; 152A(10): 2578-87.
8. Duesterhoeft SM, Ernst LM, Siebert JR, Kapur RP. Five cases of caudal regression with an aberrant abdominal umbilical artery: Further support for a caudal regression-sirenomelia spectrum. Am J Med Genet A 2007; 143A(24): 3175-84.

9. Kadian YS, Duhan N, Rattan KN, Rawal M. Sirenomelia (mermaid syndrome): a rare anomaly. Afr J Paediatr Surg 2008; 5(2): 105-6.
10. Bruce JH, Romaguera RL, Rodriguez MM, González-Quintero VH, Azouz EM. Caudal dysplasia syndrome and sirenomelia: are they part of a spectrum. Fetal Pediatr Pathol 2009; 28(3): 109-31.
11. Messineo A, Innocenti M, Gelli R, Pancani S, Lo Piccolo R, Martin A. Multidisciplinary surgical approach to a surviving infant with sirenomelia. Pediatrics 2006; 118(1): e220-3.
12. Wasnik A, Lalchandani UR. Sirenomelia-sympus unipus (mermaid deformity). Pediatr Radiol 2010; 40(Suppl 1): S14.
13. Schiesser M, Holzgreve W, Lapaire O, et al. Sirenomelia, the mermaid syndrome--detection in the first trimester. Prenat Diagn 2003; 23(6): 493-5. Kırımi E, Peker E, Tuncer O, Akgün C, Kurdoğlu Z. Sirenomelia ve polidaktili birlikteliği: bir vaka sunumu. Anatol J Clin Investig 2010: 4(1): 67-9. 67 Türk Ped Arfl 2013; 48: 65-7 Turk Arch Ped 2013; 48: 65-7 Çelik ve ark.
14. Denizkızı sendromu / Mermaid syndrome