Related Experiment Video
Updated: Aug 6, 2026
Full-Endoscopic Surgery for Hypothalamic Hamartoma Resection
Published on: April 12, 2024
A 3-Month-Old Boy with a Giant Encephalocele-Resection of the Herniated Left Supra-Insular Hemisphere Without New
Denis Ehrl1,2, Vadym Burchak1, Andrea Szelenyi3
1Department of Plastic, Reconstructive and Hand Surgery, Burn Centre for Severe Burn Injuries, Nuremberg Clinics, Paracelsus Medical University, 90419 Nuremberg, Germany.
Insights
Large congenital encephaloceles can be safely resected with excellent outcomes. Intraoperative electrophysiology helps identify non-functional brain tissue, preventing post-operative deficits in neural tube malformations.
Area of Science:
- Pediatric Neurosurgery
- Developmental Neuroscience
- Congenital Malformations
Background:
- Congenital encephalocele, a rare neural tube malformation, involves CNS tissue protrusion through skull defects.
- Limited clinical evidence exists for large encephaloceles, making management challenging.
Abstract:
Background and Importance: Congenital encephalocele refers to the protrusion of CNS tissue through a skull defect. As a rare neural tube malformation, clinical evidence is particularly limited for large encephaloceles, and management is subject to ongoing discussions. Clinical Presentation: A 3-month-old boy presented with a left hemisphere herniation above the level of the Sylvian fissure into a congenital parietal encephalocele. No focal deficits were appreciated. We hypothesized that early prenatal damage due to protruding brain tissue may have resulted in unihemispheric motor control of both body sides. As such, surgical repair guided by intraoperative electrophysiology and plastic reconstruction was scheduled. Intraoperatively, bilateral and symmetric extremity response upon transcranial electric stimulation of the contra-lesional right hemisphere was detected, whereas no responses from direct cortical and subcortical stimulation of the herniated brain parenchyma were elicited. Complete resection of the herniated supra-insular hemisphere was provided, and no ischemic changes or new deficits occurred. At 24-month follow-up, the patient showed voluntary movements with both upper extremities and voluntary grasping with his left (non-paretic) hand, no mirror movements, no signs of spasticity, good eye contact, and could speak several words. Conclusions: Safe resection with excellent outcome can be provided even for large encephaloceles. Intraoperative electrophysiological findings aid in identifying the absence of cortico-spinal projections and appear helpful to avoid post-operative deficits.
