scholarly journals Fetal Sirenomelia Associated with an Abdominal Cyst Originating from a Saccular Cloaca

2018 ◽  
Vol 2018 ◽  
pp. 1-4
Author(s):  
Yui Kinjo ◽  
Hitoshi Masamoto ◽  
Hayase Nitta ◽  
Tadatsugu Kinjo ◽  
Tomoko Tamaki ◽  
...  
Keyword(s):  
Umbilical Artery ◽  
Color Doppler ◽  
Abdominal Cavity ◽  
External Genitalia ◽  
Duodenal Atresia ◽  
Lower Limbs ◽  
Single Umbilical Artery ◽  
Normal Bladder ◽  
Anorectal Atresia ◽  
Abdominal Cyst

A 40-year-old pregnant woman presented with a fetal abdominal cyst and oligohydramnios. Color Doppler scan revealed a single blood vessel from the fetal aorta into a single umbilical artery. Severe oligohydramnios limited ultrasonographic evaluation of the fetal lower limbs, kidneys, or bladder. The pregnancy was terminated; the fetus showed fused lower limbs, bulging abdomen, and absent external genitalia and was diagnosed with type III sirenomelia. On autopsy, no normal bladder was observed, but duodenal atresia, anorectal atresia, and right renal agenesis were found. An intra-abdominal cyst, diagnosed histologically as a saccular cloaca, occupied the abdominal cavity. Ultrasonographic diagnosis of fetal sirenomelia is difficult due to poor depiction of the lower limbs. A vitelline artery leading to a single umbilical artery and a fetal abdominal cyst occupying most of the abdominal cavity are considered fetal sirenomelia associated with large defects of the gastrointestinal and genitourinary tracts.

scholarly journals Sirenomelia, the mermaid baby: a case report

2019 ◽  
Vol 8 (11) ◽  
pp. 4609
Author(s):  
Reetika Joshi ◽  
Veino Kuveio Duomai ◽  
Bianchi Sangma
Keyword(s):  
Intrauterine Growth Restriction ◽  
Umbilical Artery ◽  
External Genitalia ◽  
Lower Limbs ◽  
Single Umbilical Artery ◽  
Female Ratio ◽  
Fetal Movements ◽  
Anorectal Atresia ◽  
Male To Female ◽  
Live Baby

Sirenomelia sequence or mermaid syndrome is a rare congenital structural anomaly characterized by different degrees of fusion of lower limbs, thoracolumbar spinal anomalies, sacrococcygeal agenesis, genitourinary and anorectal atresia. The incidence of sirenomelia is 0.8-1 cases/ 105 births with male to female ratio being 3:1. A malformations patient with 40 weeks of gestation admitted with complaints of decreased fetal movements for one week. An emergency C-section was done in view of term pregnancy with severe oligohydramnios and intrauterine growth restriction with poor bishop’s score. A 1.9 kg live baby with fused lower limbs was delivered. The new born baby had narrow chest, fused both lower limbs and feet with 11 toes, small rudimentary external genitalia, imperforate anus and single umbilical artery. The baby also had features of potter’s facies including low set large ears, receding chin and prominent infraorbital folds. Sirenomelia is a rare fatal congenital anomaly. Early detection by prenatal ultrasound will help in timely termination of pregnancy.

scholarly journals Sirenomelia - Revisited

2018 ◽  
Vol 07 (01) ◽  
pp. 047-051
Author(s):  
Gaddam Vijaya Lakshmi ◽  
Jacob Abraham ◽  
Geena Benjamin
Keyword(s):  
Umbilical Artery ◽  
External Genitalia ◽  
Maternal Diabetes ◽  
Renal Tissue ◽  
Severe Form ◽  
Lower Limbs ◽  
Single Umbilical Artery ◽  
Mermaid Syndrome ◽  
Caudal Regression ◽  
Congenital Condition

AbstractSirenomelia, commonly known as mermaid syndrome is a rare congenital condition, featured by fusion of the lower limbs. This represents the most severe form of caudal regression syndrome, in which all the structures from the caudal mesoderm fail to develop properly. There is a marked deformity in the musculoskeletal system at the caudal end of the trunk and lower limbs. It is invariably associated with visceral malformations of the urogenital and gastrointestinal systems like agenesis or hypoplasia of renal tissue, blind termination of the colon, absent external genitalia and imperforate anus. It is usually associated with single umbilical artery. Though association with maternal diabetes has been described, the causative mechanisms remain unclear. The condition is generally fatal, due to associated developmental anomalies ofkidneys and other viscera.

Sirenomelia With Anhydramnios: A Clue From the Movements of Fetal Limbs

2021 ◽  
Vol 37 (2) ◽  
pp. 207-212
Author(s):  
Rahul Dev Chauhan ◽  
Ipsita Sahoo ◽  
Yashvir Mathur
Keyword(s):  
Umbilical Artery ◽  
External Genitalia ◽  
Lower Limbs ◽  
Lumbosacral Spine ◽  
Rare Entity ◽  
Single Umbilical Artery ◽  
Normal Morphology ◽  
Anal Orifice ◽  
First Case ◽  
Antenatal Detection

Sirenomelia is a rare, lethal congenital malformation of the fetus, typically characterized by the fusion of lower limbs and additional malformations involving the lumbosacral spine and urogenital and gastrointestinal tracts. We report a case of sirenomelia with anhydramnios detected during anomaly scan where the fetus had normal morphology of long bones of the lower limbs. There were associated abnormalities of the lumbosacral spine (caudal dysgenesis). The abortus had fused lower limbs, absent external genitalia and anal orifice, single umbilical artery, and Potter facies. We also highlight the interesting diagnostic challenges faced by us during this antenatal ultrasound. To our knowledge, this is the first case report of sirenomelia where the synchronous movement of lower limbs was a clue that led to the timely antenatal detection of this rare entity. This ultimately helped in perinatal counseling of parents to decide on termination of pregnancy.

scholarly journals Mermaid syndrome

2017 ◽  
Vol 6 (5) ◽  
pp. 2104
Author(s):  
Nisha Marhatta ◽  
Deepali Raina
Keyword(s):  
World Literature ◽  
Umbilical Artery ◽  
Renal Agenesis ◽  
External Genitalia ◽  
Congenital Deformity ◽  
Intestinal Loop ◽  
Single Umbilical Artery ◽  
Live Births ◽  
Mermaid Syndrome ◽  
The World

Sirenomelia, alternatively known as Mermaid Syndrome, is a very rare congenital deformity in which the legs are fused together, giving them the appearance of a mermaid's tail. This syndrome was originally stated by Rocheas and Palfya in 16th century. It occurs in about 1 in 100,000 live births. It is also associated with multiple anomalies like renal agenesis, ambiguous external genitalia, imperforate anus, blind intestinal loop and single umbilical artery. Occasionally double inferior Venacava, dextrocardia and angiomatous lumbosacral myelocystocele are reported as well. Most of the Sirenomelia come to an end as stillbirth. Only a few are born alive and survival beyond few hours after delivery is extremely rare. About 300 cases have been reported in the world literature so far.

scholarly journals A case report of sirenomelia with two umbilical arteries: an unusual finding in a rare case

2021 ◽  
Vol 10 (9) ◽  
pp. 3640
Author(s):  
Swati Trivedi ◽  
Oby Nagar ◽  
J. P. Soni ◽  
Shashank Trivedi ◽  
Prasoon Rastogi
Keyword(s):  
Umbilical Artery ◽  
Tertiary Care ◽  
Lower Limbs ◽  
Care Hospital ◽  
Single Umbilical Artery ◽  
High Risk Factors ◽  
Mermaid Syndrome ◽  
Lower Segment Cesarean Section ◽  
History Of ◽  
Umbilical Arteries

Sirenomelia also known as Mermaid syndrome is a rarely encountered fusion anomaly of the caudal region of body often associated with Potter’s facies, single umbilical artery and various visceral abnormalities which make it irreconcilable with life. Here we report a case of sirenomelia delivered in our tertiary care hospital by lower segment cesarean section to a 24 year old third gravida with no previous live issues. No high risk factors could be identified with the mother including diabetes mellitus. Baby was born alive with Potter’s facies. Both the lower limbs were merging into each other like a mermaid’s tail (hence called mermaid syndrome). Sex of the baby could not be identified and the urogenital and anal orifices were absent. Umbilical cord stump had two umbilical arteries. The baby died after 20 hours of life. There appears to be no definitive modality for diagnosing sirenomelia in the antenatal period. However, if a patient has consistently low AFI without any history of leaking per vaginum, high resolution USG or colour Doppler should be done at the earliest to look for the cause.

scholarly journals OP13.10: Perinatal outcome of 127 consecutive fetuses with single umbilical artery

2008 ◽  
Vol 32 (3) ◽  
pp. 353-353
Author(s):  
F. Vinals ◽  
S. Diaz ◽  
R. Naveas ◽  
A. Giuliano
Keyword(s):  
Perinatal Outcome ◽  
Umbilical Artery ◽  
Single Umbilical Artery

Explanation of Perinatal Mortality Rate in Collaborative Study

PEDIATRICS ◽  
1973 ◽  
Vol 52 (6) ◽  
pp. 891-891
Author(s):  
Arnold S. Goldstein ◽  
Henry H. Mangurten
Keyword(s):  
Mortality Rate ◽  
Perinatal Mortality ◽  
Umbilical Artery ◽  
Collaborative Study ◽  
The United States ◽  
Mortality Rates ◽  
Single Umbilical Artery ◽  
Neonatal Deaths ◽  
General Mortality ◽  
Future Management

The article by Froehlich and Fujikura1 on the prognosis of single umbilical artery is a much needed and highly informative addition to the literature. It presents a great deal of information and some important implications as to future management. We question the mortality rates quoted. They are given as percentages, and include stillbirths and neonatal deaths, i.e., perinatal mortality. The figure given as the general mortality rate is 3.8% or 38 per 1,000 births. Previous figures cited for perinatal mortality in the United States have varied from approximately 19 per 1,000 to approximately 26 per 1,000.2-4 We wonder how the figure of 38 per 1,000 was determined.

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