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Published on: January 17, 2018
[Diagnosis, evolution and prognosis of prenatally diagnosed suprasellar cysts]
F Di Rocco1, A André1, T Roujeau1
1Service de neurochirurgie pédiatrique, hôpital Necker-Enfants-Malades, AP-HP, 149, rue de Sèvres, 75743 Paris cedex 15, France.
Insights
Suprasellar arachnoid cysts (SAC) in children are increasingly diagnosed prenatally. A new classification aids in predicting prognosis and guiding treatment for these rare cysts.
Area of Science:
- Pediatric Neurosurgery
- Fetal Medicine
- Developmental Neurology
Background:
- Suprasellar arachnoid cysts (SAC) are rare but increasingly detected prenatally.
- Improved prenatal imaging has led to a rise in SAC diagnoses.
Purpose of the Study:
- To retrospectively analyze 15 antenatally diagnosed SAC cases.
- To characterize radiological features, treatment strategies, and outcomes of pediatric SAC.
- To propose a classification system for improved prognosis and treatment decisions.
Main Methods:
- Retrospective review of 15 antenatal SAC cases (2005-2015).
- Analysis of radiological characteristics, treatment modalities (ventriculo-cysto-cisternostomy, fetoscopy, shunt, craniotomy, observation), and long-term follow-up (mean 71 months).
- Classification into three subtypes: SAC-1 (hydrocephalus), SAC-2 (interpeduncular cistern expansion), and SAC-3 (asymmetrical).
Main Results:
- Three subtypes identified: SAC-1 (13%), SAC-2 (53%), SAC-3 (33.5%).
- Surgical outcomes were initially favorable in 100% of treated cases, with one requiring re-treatment.
- Non-operated SAC-2 patients showed no radiological changes; long-term issues included endocrine disruption, developmental delay, neuropsychological impairments, and epilepsy in a minority of cases.
Conclusions:
- Prenatal MRI and ultrasound can differentiate three SAC subtypes.
- SAC-1 and SAC-3 have excellent prognoses with perinatal treatment.
- SAC-2 often benefits from monitoring and typically remains asymptomatic, allowing for better prognosis estimation and tailored treatment.
Abstract:
Suprasellar arachnoid cysts (SAC) in children are considered rare, but the incidence is increasing due to the improvement of prenatal diagnosis. We present 15 cases of SAC diagnosed during the antenatal period between 2005 and 2015. The records were reviewed retrospectively by specifying the radiological characteristics, treatment modalities, outcomes, and long-term monitoring. Mean follow-up was 71 months. The forms (SAC-1) accounted for 2 cases (13%) with hydrocephalus. We observed 8 (53%) lower forms (SAC-2) with interpeduncular cistern expansion without hydrocephalus. The 5 (33.5%) remaining patients showed asymmetrical forms (SAC-3). Six patients (40%) were treated by ventriculo-cysto-cisternostomy, 1 by fetoscopy, 1 (6.5%) by ventriculo-peritoneal shunt, 2 (13.5%) by pterional craniotomy, and 6 (40%) were simply followed. The surgical outcomes were initially favorable in 9 cases (100%), 1 patient (13%) had to be re-treated later. Non-operated patients were all type 2 and showed no radiological changes. In the long-term, 1 patient (6.5%) had endocrine disruption, 1 had delayed development, 2 (13.5%) had minor neuropsychological impairments, and 1 had epilepsy. Combined monitoring with prenatal MRI and ultrasound can be used to distinguish three subtypes of SAC. SAC-1 and SAC-3 have an excellent prognosis after treatment in the perinatal period. SAC-2 can benefit from simple monitoring and remain asymptomatic in their majority. This classification allows a better prognosis estimation and better treatment decision.
