Sirenomelia without Vitelline Artery Steal

Jaiyesimi, F; Gomathinayagam, T.; Dixit, Abhishek; Amer, Maged Z.

doi:10.5144/0256-4947.1998.542

Cited by 22 publications

(31 citation statements)

References 16 publications

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“…Maternal diabetes has been described as an important risk factor for caudal malformations in general [3]. However, with only about 0.5-3.7% of sirenomelia cases occurring in diabetic mothers [15,18,19] , the association between maternal diabetes and sirenomelia has been described as weak [20]. Our patient was not known to be diabetic.…”

Section: Discussionmentioning

confidence: 91%

Sirenomelia, the Mermaid Syndrome in Kuwait: A case Report

Alhadhoud¹

2017

SOJGOW

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Section: Discussionmentioning

confidence: 91%

Sirenomelia, the Mermaid Syndrome in Kuwait: A case Report

Alhadhoud¹

2017

SOJGOW

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“…VACTERL syndrome is characterised by vertebral anomalies, anal atresia, cardiac defects, tracheoesophageal fistula, renal and radial anomalies and limb defects. It shares many features with sirenomelia but the defects are more severe in the case of sirenomelia than in VACTERL syndrome [13]. The etiopathogenesis of sirenomelia is not clearly known.…”

Section: Case Reportmentioning

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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“…The single umbilical artery in our patient favours this theory. However Jaiyessimi et al 12 reported a case of sirenomelia without vitelline artery steal which indicates that factors other than vitelline artery steal could be responsible for sirenomelia in humans. In 1961 Duhamel 13 proposed Figure 2.…”

Section: Case Reportmentioning

confidence: 99%

“…VACTERAL syndrome involves vertebral, anal, cardiovascular, trachea oesophageal, renal and limb dysgenesis. There i s major overlap in phenotypic manifestation of sirenomelia and VACTERAL 12 . But as our patient was a live born, autopsy could not be done and we have no radiographic proof to ascertain any relationship with VACTERAL as we referred the baby to higher centre for better management.…”

Section: Case Reportmentioning

confidence: 99%