Chiari III malformation in a neonate.

Area of Science:

• Neurology
• Pediatric Surgery
• Developmental Pediatrics

Background:

• Chiari malformation (CM) encompasses complex hindbrain deformities.
• CM type III (CMIII) represents the rarest and most severe form.
• Antenatal diagnosis of occipital encephalocele is crucial for early intervention planning.

Observation:

• A term neonate presented with an antenatal diagnosis of occipital encephalocele.
• The infant underwent surgical resection of the encephalocele, vessel ligation, and dural reconstruction on day 4 of life.
• Initial prognosis was guarded due to the severity of the condition.

Findings:

• The neonate experienced a good postoperative course without immediate complications.
• Despite successful surgical repair, the child developed cortical visual impairment.
• Global developmental delay was observed as a significant long-term outcome.

Implications:

• Early surgical intervention in CMIII cases can lead to favorable short-term outcomes.
• Long-term neurodevelopmental surveillance is essential for infants with CMIII.
• This case underscores the complex interplay between surgical repair and persistent neurodevelopmental deficits in rare congenital anomalies.