Interdisciplinary Fetal/Neuro Approach to ACC

The corpus callosum is the structure that connects the two hemispheres of the brain. ACC is a heterogeneous condition resulting from disruption of multiple developmental steps. The reported prevalence of ACC is 1 in 4,000 to 5,000 live births; however, rates of 2% to 3% have been reported among patients with neurodevelopmental disabilities. The cause of the disruption may be genetic, infectious (TORCH infections, Zika virus), vascular or toxic (fetal alcohol syndrome). Genetic factors are most common. 

The effects of the disorder range from subtle or mild to severe, depending on associated brain abnormalities. Children with the most severe brain malformations may have intellectual impairment, seizures, hydrocephalus and spasticity. Individuals with dysgenesis and hypoplasia are also at risk for hearing deficits and cardiac abnormalities.

Families often arrive to their initial consultation at the CFDT profoundly stressed at the thought of their unborn child missing part of their brain. They are often only aware of the worst-case scenarios for their unborn child until they meet with the CFDT’s multidisciplinary team.

Case in point

A family received a diagnosis of ACC in their unborn child. Based on the limited counseling they were given and the lack of a multidisciplinary approach near their home, the family flew across multiple states to New Jersey for an appointment at a pregnancy termination clinic. 

During their appointment, the clinic team recommended the family get a second opinion. They referred the family to Nahla Khalek, MD, MPH, FACOG, MSEd, a maternal-fetal medicine specialist with the CFDT.

At CHOP, the family had a well-informed multidisciplinary consultation with Dr. Khalek and the CFDT team that involved careful review of neuroimaging and genetic testing, as well as a meeting with CHOP Neurologist Sonika Agarwal, MBBS, MD. Dr. Agarwal is one of a handful of CHOP neurologists who specialize in fetal and neonatal neurology. She was recently featured on a podcast focused on ACC that is available on the SAGE for Neuroscience & Neurology website.

Through joint counseling with Dr. Khalek and Dr. Agarwal, the family learned more thorough information about their baby’s condition. They learned that, while there were risks, positive outcomes with this condition are possible. They left the evaluation with a clearer understanding of the path forward for their unborn child, and went on to have what was, ultimately, a very healthy pregnancy.  

Their baby was born uneventfully at a local hospital and is now a young infant who is being followed for neurodevelopmental progress. The family lives out of state, but they still connect with Dr. Agarwal and her team in CHOP Neurology for advice, as needed.

A holistic approach

From the first phone call, the CFDT coordinates all of a patient’s treatment. Multidisciplinary consultation includes members from genetics, maternal-fetal medicine, fetal and pediatric neurology, neurosurgery, and fetal radiology. The initial consultation also includes a meeting with a dedicated psychosocial service team. A clinical psychologist, psychiatrist and social worker provide emotional support, individualized and couple's counseling, supportive therapy, and social work services.

Part of the CFDT approach to evaluating fetuses with neurologic differences is evaluation with fetal neurologic MRI. A team of fetal neuroradiologists has developed optimal sequences for ultrafast fetal neurologic MRI. The CFDT has found that the combination of fetal neurosonography and ultrafast neurologic MRI are complementary and aid in the diagnosis of complex neurologic differences.

After a thorough evaluation, the team discusses the imaging and clinical history, confirms the diagnosis, explains options for the pregnancy, reviews pregnancy and delivery management options, and makes recommendations for ongoing care in partnership with the patient’s referring maternal-fetal medicine provider. Genetic counseling is offered to all families, as well as additional diagnostic testing including amniocentesis, maternal testing for cell-free DNA and infectious screening.

Experience in fetal neurology and review of imaging and genetic information to guide the counselling process is an important component of this comprehensive approach. CHOP neurologists have unique expertise in fetal neurology consultations.  

The entire CHOP team the family meets during the initial prenatal evaluation gathers with families for the consultation and communicates with the patient’s OB/MFM to guide the plan for a safe delivery. Coordination is done with various specialists for postnatal/post-birth neuroimaging including a brain MRI and long-term follow-up. 

Longitudinal follow-up and trajectory are important. CHOP’s team of fetal neurologists follows these infants over the long term in our Neurology Clinic to monitor their neurodevelopmental outcomes. We offer multidisciplinary care along with a team of early intervention and developmental specialists collaborating with their primary care/referring clinicians.

The importance of psychosocial support

ACC is a life-changing diagnosis. It’s important to support families through this challenging time from the very beginning.

All families cared for by the CFDT have access to the full range of psychosocial services and resources CHOP offers, from the prenatal course, through the time of delivery, throughout the postpartum period, and beyond. These services are available to the entire family. 

As patients and families transition from fetal care to early childhood programs, CHOP’s Family StEPS (Starting Early with Psychosocial Support) program streamlines care and unifies the process of assessment, intervention and referral. The goal is to provide consistent physical, emotional, spiritual, mental and social health supports to help families thrive at every step in their journey.

This dedicated approach increases access to psychosocial care services and better addresses the well-being of expectant parents and parents of young children through all the steps of their care journey, from pregnancy through early childhood.

To make a referral, please contact the Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment at 800-IN-UTERO (468-8376) or via our online form.