Learning From a Missed Diagnosis: Complete Agenesis of the Corpus Callosum With Benign Hydrocephalus

Abstract

In agenesis of the corpus callosum, ventriculomegaly without evidence of cerebrospinal fluid obstruction often requires no surgical intervention. Regular follow-up every 3-6 months, then annually, is essential to monitor for signs of raised intracranial pressure or neurological decline-prompting advanced imaging and consideration of surgical management if deterioration occurs.

Keywords: arrested hydrocephalus; benign hydrocephalus; corpus callosum; corpus callosum agenesis.

FIGURE 1

The parts of the corpus callosum and their complex connections with specific brain regions. Clinical tests used to assess intricate neurocognitive functions related to specific parts of the corpus callosum are listed in the boxes.

FIGURE 2

MRI images of the patient with agenesis of corpus callosum. (A) T2‐weighted sagittal MRI, (B) T1‐weighted coronal MRI, (C) FLAIR axial, (D) T2‐weighted coronal MRI (at the level of anterior horns). Yellow arrows indicate enlarged lateral ventricles, while the blue arrow points to an interhemispheric cyst.

FIGURE 3

Management protocol for agenesis of corpus callosum (AgCC), developed based on current published evidence in the absence of universal guidelines. Following the diagnosis of AgCC, if there are no signs of raised intracranial pressure or clinical deterioration, patients may be monitored with neurocognitive assessments conducted initially every 3 months, then every 6 months, and subsequently on an annual basis. Any clinical decline should prompt advanced neuroimaging before considering surgical intervention.