Nadir Görülen Bir Sendrom: Miad Gebede Sirenomeli

Yıl 2016, Cilt: 13 Sayı: 2, 176 - 179, 29.08.2016

Hacer Uyanıkoğlu , Kenan Gengeç Sibel Sak Muhammet Erdal Sak

Öz

Sirenomeli vücudun kaudal bölgesinin anormal gelişimi ile kendini gösteren nadir bir konjenital yapısal
anomaliler grubudur. Tanı erken gebelik haftasında ultrason kullanılarak dikkatli bir inceleme ile antenatal
olarak konulabilir. Biz bu yazıda 38. gebelik haftasında ilk kez doktora başvuran ve sancı tarifleyen hastanın
yapılan ultrasonografisinde bilateral böbrek agenezisi ve anhidroamniyoz saptanıp gebeliği sonlandırılan ve
doğum sonrası sirenomeli tanısı konulan bir olguyu literatür ışığında tartışmayı amaçladık.

Anahtar Kelimeler

Kaudal regresyon, Sirenomeli, Anhidroamniyoz

A Rare Seen Case Report: Sirenomelia in a Term Pregnant Woman

Öz

Sirenomelia is a rare congenital anomaly characterized by abnormal development of the caudal
body structures. The disease can be diagnosed antenatally by the careful ultrasound examination in early
gestational week. In this paper we aimed to discuss a case which had bilateral renal agenesia and
anhydramnios image detected by ultrasound examination at the 38' gestational week. She was admitted to the
hospital firstly at the 38' gestational week by described abdominal pain. We diagnosed sirenomelia after
cesarean section.

Anahtar Kelimeler

Caudal Regression, Sirenomelia, Anhydramnios

Kaynakça

• 1. Lutz N, Meyrat BJ, Guignard JP, Hohlfeld J. Mermaid syndrome: virtually no hope for survival. Pediatr SurgInt 2004;20(7):559-61.
• 2. Browne M, Fitchev P, Adley B, Crawford SE. Sirenomeliawith an angiomatous lumbosacral myelocystocele in a full-term infant. J Perinatol 2004;24(5):329-31.
• 3. Taghavi MM, JafariNaveh HR, Shariati M, Morteza Zade F. Sirenomelia (mermaid syndrome): an infant from parents who used a special form of snuff. Pak J BiolSci 2009;12(9):722-5.
• 4. Romano S, Esposito V, Fonda C, Russo A, Grassi R. Beyond themyth: the mermaid syndrome from Homerusto Andersen. Atributeto Hans Christian Andersen's bicentennial of birth. Eur J Radiol 2006;58(2):252-9.
• 5. Stanton MP, Penington EC, Hutson JM. A surviving infant with sirenomelia (Mermaid syndrome) associated with absent bladder. J Pediatr Surg 2003;38(8):1266-8.
• 6. Mohammed Z. Seidahmed, Omer B. Abdelbasit, Khalid A. Alhussein, Abeer M. Miqdad, Mohammed I. Khalil, Mustafa A. Salih. Sirenomelia and severe caudal regression syndrome. SaudiMed J 2014;35(1):36–43.
• 7. Duesterhoeft SM, Ernst LM, Siebert JR, Kapur RP. Fivecases of caudal regression with an aberrant abdominal umbilical artery: Further support for a caudal regressionsir e n ome l i a s p e c tr um. Am J Me dGe n e t A 2007;143(24):3175-84.
• 8. Dosedla E, Kalafusová M, Calda P. Sirenomelia apus after trimethoprim exposure: first-trimester ultrasound diagnosis-a case report. J Clin Ultrasound. 2012;40(9):594- 7.