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Primary intracranial arachnoidal cysts. A study of 67 childhood cases
I Pascual-Castroviejo1, M C Roche, A Martínez Bermejo
1Service of Pediatric Neurology, Hospital La Paz, Madrid, Spain.
Insights
Pediatric arachnoid cysts, often diagnosed in infancy, commonly present with macrocephaly. Treatment varies by size and location, with shunts and fenestration showing effectiveness.
Area of Science:
- Pediatric Neurosurgery
- Neurology
- Developmental Pediatrics
Background:
- Arachnoid cysts are congenital abnormalities that can cause significant neurological issues in children.
- Early diagnosis and understanding of cyst location and associated conditions are crucial for effective management.
Purpose of the Study:
- To analyze the clinical presentation, location, associated anomalies, and treatment outcomes of arachnoid cysts in children under 11 years.
- To provide insights into the management strategies for different types of pediatric arachnoid cysts.
Main Methods:
- Retrospective case series analysis of 67 children diagnosed with arachnoid cysts.
- Review of clinical data including presenting symptoms, cyst location, associated conditions, diagnostic methods, and treatment interventions.
Main Results:
- Macrocephaly was the most common presenting symptom (71.5%).
- Supratentorial and infratentorial cysts were equally prevalent.
- Associated anomalies included aqueductal stenosis and agenesis of the corpus callosum.
- Treatment involved shunts (cystoperitoneal/ventriculoperitoneal) and fenestration, with good outcomes reported.
Conclusions:
- Arachnoid cysts in children frequently present with macrocephaly and can have associated neurological abnormalities.
- Treatment strategies should be tailored to cyst size, location, and associated complications.
- Surgical interventions like shunting and fenestration are effective in managing symptomatic pediatric arachnoid cysts.
Abstract:
Sixty-seven cases (41 males and 26 females) of arachnoidal cysts in children under 11 years are reported. About 53% of cases were diagnosed before 1 year of life. Thirty-one (42.2%) were supratentorial (interhemispheric 9, temporal fossa 10, convexity 5, sylvian fissure 3, supra- and/or retrosellar 4); 31 (46.2%) infratentorial (supra- and/or retrocerebellar 22, foramen of Magendie 3, quadrigeminal cistern 5, pontocerebellar 1); 5 (7.5%) supra- and infratentorial. Macrocephaly was the presenting symptom in 48 cases (71.5%). Associated features were frequent: cranial asymmetry in 24; aqueductal stenosis in 10; agenesis of corpus callosum in 8; deficient cerebellar lobullation in 4; Chiari I malformation in 2; neurofibromatosis type 1 with dysgenetic zones of the brain in 1; arteriovenous malformation in 1. Diagnosis was made at autopsy in six cases in the days before computed tomography and magnetic resonance: three patients had a cyst in the supra- and retrocerebellar midline; two had a cyst in the quadrigeminal cistern and the sixth was a rare case with the cyst passing from the posterior fossa to the left lateral ventricle through a hole in the basal surface of the brain. Small and some middle-sized cysts were not treated. Big and some middle-sized cysts were usually treated by cysto- and/or ventriculoperitoneal shunts. Arachnoidal cysts of the quadrigeminal cistern usually present with aqueductal stenosis and have to be treated with ventriculoperitoneal shunt. Craniotomy and fenestration of the cysts were performed in some cases with good results. The average mental level of these children is usually moderately low.