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Abstract Ref Number = APCP1015
Poster Presentation
Sirenomelia, The Mermaid Syndrome - A Rare Case Report
Agniya Ali Fahmi Hikmat,Muhammad Rizki DM,Heka Majasari
Cianjur Hospital West Java
Background : Sirenomelia, which is also known as Mermaid Syndrome is an extremely rare congenital developmental disorder characterized by fusion of lower limbs, urogenital and gastrointestinal tract malformations, Potters facies and pulmonary hypoplasia. The incidence is 0.8-1 case/100.000 births. We are reporting rare case of mermaid baby which was delivered in our hospital.
Case Presentation Summary : A 28 year old female, G2P1L1A0 with 37 weeks of gestational age with previous one vaginal delivery was admitted in labor room in second stage of labor. She was from poor socioeconomic background with no antenatal care or investigations. There was history of tobacco use both before and during antenatal period. There was no history of ingestion of other teratogenic agents or diabetes mellitus and hypertension. Her pregnancy was uneventful with no history of medical disorder in mother or congenital anomaly in her family. She delivered a term 2,5 kg baby with single fused lower limbs. The baby cried immediately after birth. The Apgar score was 4 at 1 min and 1 at 5 min. On physical examination, the infant showed narrow chest, fused lower limbs with single foot and 3 toes, absent external genitalia, inferforate anus and umbilical cord with single umbilical artery. There were also prominent epicantal folds, downward curved nose, receding chin, low-set soft dysplastic ears and small slit-like mouth suggestive of Potters facies. The baby died within 4 hours post birth. Autopsy was declined by parents. Intrapartum and the postpartum period of mother was uneventful.
Learning Points/Discussion : Sirenomelia or mermaid syndrome is a rare and lethal congenital anomaly associated with varying degrees of visceral defects. The etiology is still a matter of controversy and is not clearly defined. Early diagnosis and termination should be in mind. However prevention is possible and should be goal. A regular antenatal check-up is essential for all pregnant women.
Keywords: sirenomelia mermaid syndrome congenital disorder
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