Sirenomelia
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Sirenomelia (also known as the mermaid syndrome) is a rare congenital malformation characterised by the fusion of lower limb structures.
Epidemiology
The estimated incidence is at ~ 1 in 60,000 - 70000-70,000 of pregnancies 9. There may be greater male predilection (somewhat paradoxical given the usage of mermaid)
Pathology
While it was previously thought as beloning the same spectrum as the caudal regression syndrome, this proposition is now heavily debated and it is now thought to result from a seperate pethogenesis. A vascular steal phenomenon causing severe ischemia to the caudal portion of the fetus is often accepted.
Associations
- maternal diabetes
- renal agenesis
- single umbilical artery
- imperforate anus
- oligohydramnios
- congenital cardiac anomalies
- abdominal wall defects
Radiographic features
Antenetal ultrasound
The lower extrametities typically appear fused into a single limb and there can be sacral agenesis to varying degrees.
Ancillary sonographic features include
- presence of a single umbilical artery
- presence of oligohydramnios 6
Treatment and prognosis
The condition of often fatal and incompatible with life (in contrary(contrary to popular fairy tales). Exceptional cases without renal agenesis may survive. Depending on the country you live in, a termination of pregnancy could be considered in appropriate situations.
EtymologyHistory and etymology
Derives from the Latin words: siren meaning "a partly female creature in Greek legend whose beautiful singing lured sailors to their deaths" and melia meaning limb
Differential diagnosis
For mild forms consider
- caudal regression sequence : limbs not fused and often has polyhydramnios
-<p><strong>Sirenomelia </strong>(also known as the <strong>mermaid syndrome</strong>) is a rare congenital malformation characterised by the fusion of lower limb structures.</p><h4>Epidemiology</h4><p>The estimated incidence is at ~ 1 in 60,000 - 70000 of pregnancies <sup>9</sup>. There may be greater male predilection (somewhat paradoxical given the usage of mermaid)</p><h4>Pathology</h4><p>While it was previously thought as beloning the same spectrum as the <a href="/articles/caudal-regression-syndrome" title="Caudal regression syndrome">caudal regression syndrome</a>, this proposition is now heavily debated and it is now thought to result from a seperate pethogenesis. A vascular steal phenomenon causing severe ischemia to the caudal portion of the fetus is often accepted.</p><h5>Associations</h5><ul>-<li><a href="/articles/fetal-conditions-associated-with-maternal-diabetes" title="Conditions associated with maternal diabetes">maternal diabetes</a></li>-<li><a href="/articles/renal-agenesis" title="Renal agenesis">renal agenesis</a></li>-<li><a href="/articles/single-umbilical-artery" title="Single umbilical artery">single umbilical artery</a></li>-<li><a href="/articles/anal-atresia" title="Imperforate anus">imperforate anus</a></li>-<li><a href="/articles/oligohydramnios" title="Oligohydramnios">oligohydramnios</a></li>-<li><a href="/articles/congenital-cardiovascular-anomalies" title="Congenital cardiac anomalies">congenital cardiac anomalies</a></li>-<li><a href="/articles/fetal-anterior-abdominal-wall-defects" title="Fetal anterior abdominal wall defects">abdominal wall defects</a></li>-</ul><h4>Radiographic features</h4><h5>Antenetal ultrasound</h5><p>The lower extrametities typically appear fused into a single limb and there can be <a href="/articles/sacral-agenesis" title="Sacral agenesis">sacral agenesis </a>to varying degrees.</p><h6>Ancillary sonographic features include</h6><ul>-<li>presence of a <a href="/articles/single-umbilical-artery" title="Single umbilical artery">single umbilical artery</a>- +<p><strong>Sirenomelia </strong>(also known as the <strong>mermaid syndrome</strong>) is a rare congenital malformation characterised by the fusion of lower limb structures.</p><h4>Epidemiology</h4><p>The estimated incidence is at ~ 1 in 60,000-70,000 of pregnancies <sup>9</sup>. There may be greater male predilection (somewhat paradoxical given the usage of mermaid)</p><h4>Pathology</h4><p>While it was previously thought as beloning the same spectrum as the <a href="/articles/caudal-regression-syndrome">caudal regression syndrome</a>, this proposition is now heavily debated and it is now thought to result from a seperate pethogenesis. A vascular steal phenomenon causing severe ischemia to the caudal portion of the fetus is often accepted.</p><h5>Associations</h5><ul>
- +<li><a href="/articles/fetal-conditions-associated-with-maternal-diabetes">maternal diabetes</a></li>
- +<li><a href="/articles/renal-agenesis">renal agenesis</a></li>
- +<li><a href="/articles/single-umbilical-artery">single umbilical artery</a></li>
- +<li><a href="/articles/anal-atresia">imperforate anus</a></li>
- +<li><a href="/articles/oligohydramnios">oligohydramnios</a></li>
- +<li><a href="/articles/congenital-cardiovascular-anomalies">congenital cardiac anomalies</a></li>
- +<li><a href="/articles/fetal-anterior-abdominal-wall-defects">abdominal wall defects</a></li>
- +</ul><h4>Radiographic features</h4><h5>Antenetal ultrasound</h5><p>The lower extrametities typically appear fused into a single limb and there can be <a href="/articles/sacral-agenesis">sacral agenesis </a>to varying degrees.</p><h5>Ancillary sonographic features </h5><ul>
- +<li>presence of a <a href="/articles/single-umbilical-artery">single umbilical artery</a>
-<li>presence of <a href="/articles/oligohydramnios" title="Oligohydramnios">oligohydramnios</a> <sup>6</sup>- +<li>presence of <a href="/articles/oligohydramnios">oligohydramnios</a> <sup>6</sup>
-</ul><h4>Treatment and prognosis</h4><p>The condition of often fatal and incompatible with life (in contrary to popular fairy tales). Exceptional cases without renal agenesis may survive. Depending on the country you live in, a termination of pregnancy could be considered in appropriate situations.</p><h4>Etymology</h4><p>Derives from the Latin words : <strong>siren</strong> meaning "a partly female creature in Greek legend whose beautiful singing lured sailors to their deaths" and <strong>melia</strong> meaning limb</p><h4>Differential diagnosis</h4><p>For mild forms consider</p><ul><li>-<a href="/articles/caudal-regression-syndrome" title="Caudal regression syndrome (CRS)">caudal regression sequence</a> : limbs not fused and often has polyhydramnios</li></ul>- +</ul><h4>Treatment and prognosis</h4><p>The condition of often fatal and incompatible with life (contrary to popular fairy tales). Exceptional cases without renal agenesis may survive. Depending on the country you live in, a termination of pregnancy could be considered in appropriate situations.</p><h4>History and etymology</h4><p>Derives from the Latin words: <strong>siren</strong> meaning "a partly female creature in Greek legend whose beautiful singing lured sailors to their deaths" and <strong>melia</strong> meaning limb</p><h4>Differential diagnosis</h4><p>For mild forms consider</p><ul><li>
- +<a href="/articles/caudal-regression-syndrome">caudal regression sequence</a> : limbs not fused and often has polyhydramnios</li></ul>
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