Caudal Regression Syndrome Versus Sirenomelia: A Case Report

Das, Bibhuti B.; Rajegowda, Benamanahalli; Bainbridge, Ronald; Giampietro, Philip F.

doi:10.1038/sj.jp.7210598

Cited by 32 publications

(31 citation statements)

References 10 publications

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“…(3) Öte yandan prenatal dönemde yapılan ultrasonografik incelemelerde KRS'da iki umblikal arter, ölümcül olmayan böbrek anormallikleri, füzyona uğramamış alt ekstremiteler görülmesi sirenomeli'nin farklı bir sendrom olduğunu düşündür-mektedir. (6) Sirenomeli etiyolojisi multifaktöriyel olup; kaudal mezoderm blastogenezis yetersizliği, vasküler çalma fenomeni, amniyotik bantlar veya oligohidramniosa bağlı mekanik sıkıştırma, kadmiyum, retinoik asit gibi teratojenler, annede diyabet ve genetik mekanizmalar sayılabilir. (1) Prenatal dönemde sirenomeli tanısında kullanılabilecek bilinen bir serum belirteci henüz yoktur ve teşhisinde prenatal ultrasonografi kullanılır.…”

Section: Discussionunclassified

“…(1) Prenatal dönemde sirenomeli tanısında kullanılabilecek bilinen bir serum belirteci henüz yoktur ve teşhisinde prenatal ultrasonografi kullanılır. (6) Yaptığımız literatür taramasında sirenomeli'nin anestezi yönetimi ile ilgili yazılar sınırlı idi. Bu olgularda kısmi veya tam ekstremite anomalilerinden dolayı damar yolu açılamaması, kan basıncı monitörizasyonunun yapılamaması, bilateral renal agenezi varlığında olası metabolik sorunlar anestezi yönetimini özellikli kıl-maktadır.…”

Section: Discussionunclassified

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Anesthetic Management of a Patient With Sirenomelia A Case Report

Yücel¹,

Begeç²,

Erdoğan³

et al. 2012

J Turk Anaesth Int Care

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ÖZETSirenomeli veya mermaid sendromu ender görülen kalıtsal bir anormallik olup, alt ekstremitelerin kısmen veya tamamen gelişmemesi veya füzyonu ile karakterizedir. Gastrointestinal ve genitoüriner kusurlar sıklıkla bu doğumsal bozukluğa eşlik eder. Periferik venlerin yerini belirlemedeki zorluk ve anormal ven drenajı nedeniyle intravenöz yol için ekstremite kullanımı sınırlı olabilir. Preoperatif dönemde eşlik eden anormallikler tespit edilmeli ve gereken önlemler alınmalıdır. Bu sunumuzda sirenomeli sendromu tanısı koyulan olguda anestezi uygulaması ve anestezi yönetimi tartışılmıştır.Anahtar kelimeler: Kaudal regresyon sendromu, sirenomeli, anestezi SUMMARY Anesthetic Management of a Patient with Sirenomelia: A Case ReportSirenomelia or mermaid syndrome is an extremely rare developmental malformation characterized by complete or partial hipotrophy or fusion of the lower limbs. Gastrointestinal and urogenital anomalies are often accompanies with this congenital syndrome. Difficulty in determining the location of the peripheral veins and abnormal venous drainage may limit the use of limb for intravenous access. Associated anomalies should be identified and measures should be taken during the preoperative period. In this report we discussed anesthetic management of a patient diagnosed as sirenomelia syndrome.

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Section: Discussionunclassified

Anesthetic Management of a Patient With Sirenomelia A Case Report

Yücel¹,

Begeç²,

Erdoğan³

et al. 2012

J Turk Anaesth Int Care

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“…La gran mayoría de los casos son esporádicos, sin embargo, existen reportes que sugieren la existencia de una contribución genética, al menos parcial, en el desarrollo de este síndrome 3,8,14,21 . Existen casos de SRC familiar, pero ningún patrón de herencia Mendeliana ha sido establecido 22,23 . Estudios recientes entregan evidencia sobre la participación de un gen "homeobox" (HLXB9) en el desarrollo de la agenesia sacra de herencia dominante, pero su posible relación con el origen del SRC no ha sido establecida 22,23 .…”

Section: Discussionunclassified

Síndrome de Regresión Caudal: Caso Clínico

et al. 2010

Rev. chil. pediatr.

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“…It is a condition which is characterised by fused lower limbs, genitourinary abnormalities, imperforate anus and a single umbilical artery [1]. It has an incidence of about 1:1,00,000 births [2].…”

Section: Introductionmentioning

confidence: 99%

Sirenomelia: A Case Report

Sharma¹,

Singh²

2017

IJAR

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Sirenomelia or Mermaid syndrome is a rare congenital malformation characterised by fused lower limbs, genitourinary abnormalities, imperforate anus and a single umbilical artery. Its incidence is about 1:1,00,000 births. This is a case of sirenomelia which was detected in a fetus of about 35 wks. The fetus was delivered by emergency Caesarean section with poor Apgar score and died soon after birth. The fetus had fused lower limbs, a single foot and four digits. The urinary system and adrenal glands were absent. The gonads couldn't be detected. The terminal part of the colon was blind-ending and distended with meconium. The abdominal aorta below the umbilical artery was rudimentary and gave off two iliac arteries. The right iliac artery continued into the thigh. The left iliac artery was small. There was right pulmonary aplasia and left pulmonary hypoplasia. Radiograph of the fetus revealed partially fused femurs, separate tibiae, but no fibulae. There were only 3 metatarsals, each of them having a single phalanx. Sirenomelia cases usually die because of pulmonary hypoplasia, aplasia or renal agenesis. Though there are a few theories regarding aetiopathogenesis, there is still no such one which can fully explain all the features. The sirenomelia cases can be detected by ultrasound antenatally. Such babies cause difficult labor and are usually delivered by Caesarean section but death usually occurs soon after birth. Once the diagnosis is made during early pregnancy, termination of pregnancy is advisable.

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Caudal Regression Syndrome Versus Sirenomelia: A Case Report

Cited by 32 publications

References 10 publications

Anesthetic Management of a Patient With Sirenomelia A Case Report

Anesthetic Management of a Patient With Sirenomelia A Case Report

Síndrome de Regresión Caudal: Caso Clínico

Sirenomelia: A Case Report

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