Alobar Holoprosencephaly: Case Report

Holoprosencephaly is a congenital induction disorder of the brain occurring at 3-6 weeks` gestation, with failed segmentation of the neural tube.The alobar form of Holoprosencephaly is rare. It is defined by a single ventricle, fusion of the thalami and basal ganglia, and absence of the interhemispheric fissure and corpus callosum.

We reported here the case of alobar holoprosencephaly with hydrocephalus and facial malformations in newborn patient.

We admitted in the Pediatric Emergency Department a female newborn diagnosed with alobar holoprosencephaly by brain CT. She was born via caesarean with 40 weeks of gestational age and birth weight of 3500 grams. She was born with a severe neonatal respiratory distress .The mother was 30-year-old,multiparous (4G,4P), She took fenugreek at the first trimester of pregnancy. Clinical examination objectified severe neonatal respiratory distress, facial dimorphism with a single external nasal orifice, a cleft palate, and macrocephaly. The rest of the examination was normal.

The brain CT revealed alobar holoprosencephaly associated with median facial anomalies (cleft palate, choanal atresia).The echocardiography objectified a 3mm interventricular communication. The abdominal echography and the chest CT were normal.

Alobar holoprosencephaly is a rare subtype of holoprosencephaly. It is the most severe of the classical three subtypes. Holoprosencephaly can frequently be diagnosed via screening ultrasound at 20 weeks gestation. The diagnosis should be considered in fetuses and infants who have midline abnormalities.