The prognosis of fetal ventriculomegaly (FVM) varies because of the disease’s heterogeneity and the diversity of accompanying anomalies. Moreover, the cases that are referred to neurosurgeons may have different clinical features from those typically encountered by obstetricians. The object of this study was to delineate the prognosis of FVM in cases for which neurosurgical consultation was sought.
Forty-four cases of FVM that were diagnosed before birth and referred to neurosurgeons for prenatal consultation were analyzed retrospectively. Twenty-five of the 44 patients had accompanying anomalies, but in only three (12%) of the cases were they detected prenatally. Postnatal imaging studies revealed that agenesis of the corpus callosum (nine cases) was the most common associated anomaly. Neuronal migration disorders, periventricular leukomalacia, and arachnoid cysts were present in four cases each, and aqueductal stenosis was present in three cases.
Thirty-three patients were followed up longer than 11 months; in 15 (45%) delayed cognitive and/or motor development was documented, and all had accompanying anomalies. All 10 of the patients with isolated FVM exhibited normal development during the follow-up period. Eleven (25%) of the 44 patients underwent neurosurgical interventions for ventriculomegaly, which included ventriculoperitoneal shunt placement in seven cases. Four patients (9%) died.
The authors conclude that delayed development and disturbed functional status in patients in whom FVM was diagnosed prenatally are closely related to the presence of certain accompanying anomalies. On postnatal examination, more than half of the patients in whom the diagnosis of FVM was based on ultrasonography findings and whose parents were offered prenatal neurosurgical consultation were found to have additional anomalies that were not detected prenatally. Because of the possibility of additional undiagnosed anomalies, consulting neurosurgeons should be cautious in giving a prognosis in cases of FVM, even when prenatal ultrasonography reveals isolated ventriculomegaly and tests for intrauterine infection and chromosomal abnormality yield negative results.