A rare case of sirenomelia with total bilateral cleft lip palate and kyphoscoliosis

Authors

  • Sangeeta Arya Department of Obstetrics & Gynecology, GSVM Medical College, Kanpur- 208002, U.P., India
  • Anil Kumar Verma Department of Obstetrics & Gynecology, GSVM Medical College, Kanpur- 208002, U.P., India
  • Kiran Pandey Department of Obstetrics & Gynecology, GSVM Medical College, Kanpur- 208002, U.P., India
  • Sunil Kumar Verma Department of Obstetrics & Gynecology, GSVM Medical College, Kanpur- 208002, U.P., India

Keywords:

Sirenomelia, Caudal dysgenesis, Cleft-lip-palate, kyphoscoliosis

Abstract

Sirenomelia, a rare congenital deformity, has fused lower limbs with variable combination of visceral anomalies. This condition is fatal within a day or two of birth so early antenatal detection and pregnancy termination is advocated. Antenatally it can be diagnosed by ultrasonography showing oligohydramnios, renal agenesis and fibulae positioned between the tibiae. A 24 years old Muslim primigravida came for first antenatal check up at 18 weeks of pregnancy. She didn’t turn up and came directly in emergency at 37 weeks in active labor and delivered a baby weighing 1.8 kg with a small sized placenta, complete bilateral cleft lip palate, Kyphoscoliosis and fused lower limbs upto ankles. Baby expired after 25 minutes of birth. Till now around 300 cases of Sirenomelia have been reported worldwide. All human cases of Sirenomelia have been found to be associated with variable degree of renal and urethral dysplasia or renal agenesis. External genitalia are affected, whereas gonads remain unaffected. In rare cases Sirenomelia is associated with upper body defects including cleft palate, cervical and upper thoracic vertebral abnormalities, pulmonary hypoplasia and cardiac defects.

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Published

2016-12-09

Issue

Section

Case Reports