Meckel-gruber syndrome in togo: Prenatal ultrasound and comp
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Meckel–Gruber syndrome (MGS) is a rare autosomal recessive lethal disorder involving multiple systems. It is characterized by occipital encephalocele, polycystic kidneys and post-axial polydactyly. We report a case of MGS with occipital meningocele, bilateral enlarged echogenic kidneys, polydactyly, and severe oligohydramnios.

A 20-year-old female G1P0 presented to the obstetric department for the first time, with history of 28 weeks amenorrhea for routine antenatal examination. There was history of third-degree consanguineous marriage. She had her antenatal examination at a peripheral hospital. She was taking iron and folic acid (dosages of iron and folic acid were normal). Ultrasound revealed a fetus with occipital meningocele, bilateral enlarged echogenic kidneys, polydactyly, and severe oligohydramnios. No liver abnormalities found. For the precise analysis of polydactyly, a CT scan of the uterine contents was made. It showed a postaxial polydactyly affecting the four extremities, with six fingers at each hand and six toes at each foot. The scanner also found an occipital bone defect from which the encephalon herniated.

The family was counseled and after getting consent, termination of pregnancy was planned immediately at 28 weeks’ gestation. Post-abortion macroscopic examination revealed multiple congenital anomalies including occipital encephalocele, and post-axial polydactyly...

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