Author/Authors :
Elgamal, Essam A. Sheikh Khalifa Medical City - Institute of Surgery - Neurosurgery Division, United Arab Emirates , Elwatidy, Sherif M. King Saud University - College of Medicine - Department of Pediatrics, Division of Neurosurgery, Saudi Arabia , Alhabib, Amro F. King Saud University - College of Medicine - Department of Pediatrics, Division of Neurosurgery, Saudi Arabia , Jamjoom, Zain B. King Saud University - College of Medicine - Department of Pediatrics, Division of Neurosurgery, Saudi Arabia , Murshid, Waleed R. Taibah University - College of Medicine - Department of Neurosurgery, Saudi Arabia , Hassan, Hamdy H. King Saud University - College of Medicine - Department of Radiology and Medical Imaging, Saudi Arabia , Salih, Mustafa A. King Saud University - Department of Pediatrics, Saudi Arabia
Abstract :
Objective: To ascertain the incidence and clinical implications of agenesis of the corpus callosum (ACC) in spinal open neural tube defects (SONTD). Methods: All cases of SONTD registered at the Spina Bifida Clinic in King Khalid University Hospital, Riyadh, Saudi Arabia between 1995 and 2010 were retrospectively reviewed, and mid-sagittal MRI of the corpus callosum (CC) area was analyzed in each case. Neurodevelopmental outcome was classified as poor in children with seizures, severe neurodevelopmental impairment, or death. Results: Thirty-eight patients (45.8%) with ACC were identified among 83 cases with SONTD. Patients’ age ranged between one and 16 years. Total ACC was found in 10 patients, partial ACC in 25, and in 3 patients, the CC was hypoplastic. Active hydrocephalus was an associated finding in 9 out of 10 patients with total ACC, 22 out of 25 with partial ACC, and in all patients with hypoplasia of the CC. Thirteen patients (34.2%) had normal intellectual function, whereas 24 patients presented with learning disability, epilepsy, or poor intellectual function; and one patient died of respiratory failure. Conclusion: Agenesis of the corpus callosum is found in a significant portion of patients with SONTD. When associated with hydrocephalus, its presence affects neuro-developmental outcome.
