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Porencephalic congenital cysts with hydrocephalus
Insights
Porencephalic congenital cysts in children often present with neurological deficits. Surgical intervention, including shunts and plastic surgery, improved patient morbidity.
Area of Science:
- Pediatric Neurology
- Neurosurgery
- Developmental Pediatrics
Background:
- Porencephalic congenital cysts are rare brain malformations.
- Early diagnosis and intervention are crucial for managing associated neurological deficits.
Purpose of the Study:
- To review therapeutic criteria for porencephalic congenital cysts based on tomographic findings.
- To evaluate the clinical presentation, diagnostic methods, and surgical outcomes in children with these cysts.
Main Methods:
- Retrospective review of 10 children (6 male, 4 female) with porencephalic congenital cysts.
- Analysis of clinical symptoms, preoperative imaging (X-ray, EEG, CT), and surgical procedures (peritoneal/atrial shunts, plastic surgery).
Main Results:
- Most children presented with symptoms within the first 6 months of life.
- Common symptoms included retarded psychomotor development, endocranial hypertension, macrocrania, motor deficits, and seizures.
- CT scans revealed porencephalic cysts, ventricular dilatation, and midline shift in most patients.
- Surgical interventions led to improved morbidity without mortality.
Conclusions:
- Tomographic findings are essential for diagnosing and planning treatment for porencephalic cysts.
- Surgical management, including shunting and plastic surgery, can effectively improve outcomes in affected children.
- Multidisciplinary rehabilitation and anticonvulsive treatment are vital components of postsurgical care.
Unlabelled:
The therapeutic criteria according to tomographic findings are reviewed. 10 children, 6 male and 4 female, with porencephalic congenital cysts were studied. Early symptoms began within the first 6 months of life in 9 cases, and at the age of 5 years in 1. The most frequent symptoms were: seizures in 3; motor deficit in 5; retarded psychomotor development in 7; endocranial hypertension syndrome in 7; symmetric macrocrania in 7; paresis of the motor ocular nerves in 4. Preoperative studies: X-ray films-1 each patient-showed diastasis of sutures in 6 and cranial asymmetry in 2. Electroencephalograms-6 patients-were abnormal and diffuse in 3, hipsarrhythmic in 2, and focal in 1. CT-1 each patient-showed porencephalic cysts in all the patients; ventricular dilatation in 9; a single ventricle in 1, and a shift of the midline in 5. Postoperative studies: EEG, 1 each patient; CT, 1 each patient.
Surgical Treatment:
8 patients underwent peritoneal shunts and 2 atrial shunts. Plastic surgery of the dysraphy was also performed. Postsurgical treatment: Rehabilitation and anti-convulsive treatment-4 patients. There were no deaths among the patients. The morbidity improved.
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