Sirenomelia:The mermaid syndrome-A Rare case report
Sehereen Siddiqua (BD), Sharmin Abbasi (BD)
[Siddiqua] Anwer Khan Modern Medical College Hospital, [Abbasi] Anwer Khan Modern Medic
Background:Sirenomelia “The Mermaid Syndrome” is 1:1,00000 live births and is fatal within the firstday of life because of complications of abnormal kidney, urinary system and anorectal development.This syndrome has strong association (22%) with maternal Diabetes mellitus and relative risk 1:200-250. Case Report: A 30 years old primi wt 115 kg,non diabetic, normotensive,euthyroid delivered a live baby at 38 weeks of gestation with fused lower limbs.The baby cried immediately after birth. After consultation with the parents baby kept under oxygen and they refused to take NICU care. And the baby died 4 hours after delivery.It was her spontaneous pregnancy and she was in regular antenatal check-up. At 13weeks her down’s screening was normal.In 20 weeks anomaly scan done AFI was almost nil and the lower limbs did not visualized properly.Every 2-3 weeks interval we did USG but she remained anhydramnios throughout the pregnancy. Pt party was counseled that the baby may had multiple congenital anomaly and about the fate of the baby also and advised for termination. But pt refused and preg continued as per patients choice. There was no history of taking any teratogenic agents. X-ray of lower limbs of the baby showed –completely fused lower limb and one femur and one tibia,incomplete development of pelvis with absent acetabulum , ischium and pubic bones.USG of whole abdomen showed-bilateral agenesis of kidney and urinary bladder. Echocardiography showed-large VSD,ASD. Conclusion: Sirenomelia is fatal congenital anomaly and has a strong association wirth maternal diabetes. Early diagnosis and termination should be in mind. Regular antenatal check-up with optimum maternal blood glucose level in preconceptional period and in first trimester should be maintained to prevent this anomaly.