Fetal macrocrania: diagnosis, delivery and outcomes

To describe fetal macrocrania including prenatal diagnosis, delivery considerations and clinical outcomes.

A retrospective case series was developed by reviewing 26 885 ultrasounds performed between 1 March 2003 and 30 June 2007 for the prenatal diagnosis of macrocrania. Medical records of each mother/infant pair were reviewed for demographic information, ultrasound findings, obstetric management and outcomes.

Twenty-three fetuses were diagnosed with macrocrania. Median gestational age at diagnosis was 31.1 weeks (range 18.3-38.1) and at delivery was 36.9 weeks (range 30.7-39.9). Fifteen patients (65%) underwent amniocentesis for karyotype; none were aneuploid but one had a duplication on chromosome 7. All the 23 infants were liveborn. Twenty-one deliveries were by Cesarean (91%), with thirteen of these by classical incision (62%). Of the infants, 5 (22%) died shortly after birth, 16 (70%) were stabilized in the neonatal intensive care unit and were discharged alive and 2 (8%) were transferred to another center and subsequently died. Eighteen babies required ventriculoperitoneal shunting (78%).

Macrocrania is a diagnosis usually made in children but can also be made prenatally. Fetal macrocrania is usually a result of ventriculomegaly due to an obstructive process to cerebrospinal fluid flow. Abdominal delivery is usually required, often necessitating a classical uterine incision. Targeted ultrasonography, extensive counseling of parents and delivery at a tertiary care center with availability of neurosurgery is recommended.