Sirenomelia (mermaid syndrome): A rare anomaly

Kadian, Yogender Singh; Duhan, Nirmala; Rattan, Kamal Nain; Rawal, Manoj

doi:10.4103/0189-6725.44190

Cited by 8 publications

(15 citation statements)

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“…Kaudal disgenezinin bir tipi mi yoksa tamamen ayri bir durum mu oldugu halen tarti §ma konusudur (3,6,(9)(10)(11)(12)(13). Patojenezi tam olarak açiklanamamakia birlikte bazi teoriler ileri sürülmü §tür.…”

Section: Tartiçmaunclassified

“…Patojenezi tam olarak açiklanamamakia birlikte bazi teoriler ileri sürülmü §tür. Bir dü §ünceye göre mezodermal hücre göcünün uygun §ekilde yapilamamasi sorumlu tutulmuçtur (9,11). Daha yaygin olan ba §ka bir dü §ünceye göre ise göbek kordonunda tek arter bulunmasi nedeniyle embriyonal dönemde alt taraf doku ve organlannin yetersiz kanlanmasi bacaklarm yapi §ikligina ve diger bulgulara neden olur (6,10,11,14,15).…”

Section: Tartiçmaunclassified

“…Bir dü §ünceye göre mezodermal hücre göcünün uygun §ekilde yapilamamasi sorumlu tutulmuçtur (9,11). Daha yaygin olan ba §ka bir dü §ünceye göre ise göbek kordonunda tek arter bulunmasi nedeniyle embriyonal dönemde alt taraf doku ve organlannin yetersiz kanlanmasi bacaklarm yapi §ikligina ve diger bulgulara neden olur (6,10,11,14,15). Sendromun nedenleri tam olarak bilinmemekle birlikte anne yaçmin 2O'nin altinda veya 4O'in üzerinde olmasi, tek yumurta ikizi olma, gebelikte çe §itli zararli etkenlere maruz kalma, diyabetik anne bebegi olma ve erkek cinsiyet risk etmenleri olarak bildirilmi §tir (3,4,15).…”

Section: Tartiçmaunclassified

“…Denizkizi sendromunda genellikle di § ve iç cinsiyet organlari tam olarak geli §mez (3,(9)(10)(11)(12)14). Bizim olgumuzda penis görünümlü bir yapi bulunmakia birlikte, skrotum geli §memi §ti ve testisler ele gelmiyordu.…”

Section: Tartiçmaunclassified

“…Bebegin ya §am süresini böbrek geli §imindeki bozuklugun derecesi belirler (13). Bebegin böbrek geli §iminde agir derecede bozukluk varsa oligohidramniyos ve akciger hipoplazisi geli §ir, ölü dogum veya erken dönemde ölümle sonuçlanir (3,11). Literatürde çok az sayida ya §ayan olgu bildirilmi §tir ve bunlar genellikle i §lev gösterebilen böbrekleri bulunan olgulardir (4,5).…”

Section: Tartiçmaunclassified

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Mermaid syndrome

Çelik¹,

Turhan²,

Gülaşı³

et al. 2013

Turk Pediatri Ars

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ÖzetSirenomeli olarak da adlandinlan denizkizi (mermaid) sendromu oidukça nadir görülen dogumsal bir bozukiuktur. Bu sendromda bebege denizkizi görünümünü veren yapifik bacakiann yam sira, agir üreme-bo §altim, mide-bagirsak, kalp-damar ve merkezi sinir sistemi bozukiuklari bulunur. Bu yazida yapi §ik bacakiann yam sira göbek kordonunda tek arter, böbrek yokiugu, akciger hipoplazisi, özofagus atrezisi, ventriküier septai defekt, anai atrezi, bagirsak atrezisi saptanan ve dogumunun beçinci saatinde kaybedilen denizkizi sendromu olgusu sunuimuçtur. (Türi< Ped Ar § 2013; 48:65-7) Anahtar sözcükler: Denizkizi, kaudal disgenezi, sirenomeli Summary Sirenomelia, also known as the mermaid syndrome, is a very rare congenitai anomaly characterized by iower iimb fusion and severe urogenital, gastrointestinal, cardiovascuier, central nervous system maiformations. We report a case of sirenomelia who had a singie umblicai artery, renal agenesis, pulmoner hypoplasia, esophageai atresia, ventricular septai defect, anai atresia, intestinal atresia and who was iost at fifth hour of life. (Turi< Arcti Ped 2013; 48; 65-7)

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Section: Tartiçmaunclassified

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Mermaid syndrome

Çelik¹,

Turhan²,

Gülaşı³

et al. 2013

Turk Pediatri Ars

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Sirenomelia: An epidemiologic study in a large dataset from the International Clearinghouse of Birth Defects Surveillance and Research, and literature review

Orioli¹,

Amar²,

Arteaga‐Vázquez

et al. 2011

American J of Med Genetics Pt C

139

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Sirenomelia is a very rare limb anomaly in which the normally paired lower limbs are replaced by a single midline limb. This study describes the prevalence, associated malformations, and maternal characteristics among cases with sirenomelia. Data originated from 19 birth defect surveillance system members of the International Clearinghouse for Birth Defects Surveillance and Research, and were reported according to a single pre-established protocol. Cases were clinically evaluated locally and reviewed centrally. A total of 249 cases with sirenomelia were identified among 25,290,172 births, for a prevalence of 0.98 per 100,000, with higher prevalence in the Mexican registry. An increase of sirenomelia prevalence with maternal age less than 20 years was statistically significant. The proportion of twinning was 9%, higher than the 1% expected. Sex was ambiguous in 47% of cases, and no different from expectation in the rest. The proportion of cases born alive, premature, and weighting less than 2,500 g were 47%, 71.2%, and 88.2%, respectively. Half of the cases with sirenomelia also presented with genital, large bowel, and urinary defects. About 10-15% of the cases had lower spinal column defects, single or anomalous umbilical artery, upper limb, cardiac, and central nervous system defects. There was a greater than expected association of sirenomelia with other very rare defects such as bladder exstrophy, cyclopia/holoprosencephaly, and acardia-acephalus. The application of the new biological network analysis approach, including molecular results, to these associated very rare diseases is suggested for future studies.

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Sirenomelia in a Nigerian triplet: a case report

Ugwu¹,

Eneh²,

Wonodi³

2011

J Med Case Reports

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IntroductionSirenomelia, also known as mermaid syndrome, is a very rare fatal congenital abnormality in which the legs are fused together, giving them the appearance of a mermaid's tail. It is commonly associated with abnormal kidney development, genital and rectal abnormalities. A handful of cases have been reported in other parts of the world, however, no cases have previously been reported in a Nigerian neonate. To the best of our knowledge, we believe that this is the first case reported from West Africa and in a triplet.Case presentationA 16-hour-old baby boy, the second of a set of Nigerian triplets, presented to our facility with fusion of the entire lower limbs, imperforate anus, indiscernible genital structures, single umbilical artery and a neural tube defect. His parents were from the Hausa ethnic group and not related.ConclusionSirenomelia has not been previously described in a set of triplets, and it is hoped that this report from West Africa will give information about the non-racial predilection of this condition.

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Sirenomelia (mermaid syndrome): A rare anomaly

Cited by 8 publications

References 0 publications

Mermaid syndrome

Mermaid syndrome

Sirenomelia: An epidemiologic study in a large dataset from the International Clearinghouse of Birth Defects Surveillance and Research, and literature review

Sirenomelia in a Nigerian triplet: a case report

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