Children diagnosed with suprasellar arachnoid cysts often concurrently have hydrocephalus. This study aims to classify the relationship between suprasellar arachnoid cysts and hydrocephalus, discussing surgical strategies-shunting or neuroendoscopic approaches-and their sequence, based on this classification. A retrospective analysis was conducted on 14 patients diagnosed with suprasellar arachnoid cysts and hydrocephalus, treated surgically by the first author between January 2016 and December 2020. Clinical features, radiological findings, surgical strategies, and outcomes were reviewed. The classification of the relationship between the suprasellar arachnoid cysts and hydrocephalus was developed and illustrated with specific cases. Recommendations for future surgical management based on this classification are provided. We classified the relationship between suprasellar arachnoid cysts and hydrocephalus into three categories. SACH-R1, the direct type, represents cases where the cysts cause obstructive hydrocephalus. Here, neuroendoscopic ventriculocystocisternostomy (VCC) effectively treats both conditions. SACH-R2, the juxtaposed type, involves concurrent occurrences of cysts and hydrocephalus without a causative link. This is further subdivided into SACH-R2a, where acute progressive communicating hydrocephalus coexists with the cyst, initially managed with a ventriculoperitoneal shunt, followed by VCC upon stabilization of hydrocephalus; and SACH-R2b, where the cyst coexists with chronic stable communicating hydrocephalus, first addressed with VCC, followed by monitoring and potential secondary shunting if needed. Key factors differentiating SACH-R2a from SACH-R2b include the patient's age, imaging signs of fourth ventricle and cisterna magna enlargement, and the rapid progression or chronic stability and severity of hydrocephalus symptoms. SACH-R3, the reverse type, describes scenarios where shunting for hydrocephalus leads to the development or enlargement of the cyst, managed via neuroendoscopic VCC with precautions to prevent infections in existing shunt systems. The simultaneous presence of suprasellar arachnoid cysts and hydrocephalus requires a nuanced understanding of their complex relationship for optimal surgical intervention. The analysis and classification of their relationship are crucial for determining appropriate surgical approaches, including the choice and sequence of shunting and neuroendoscopic techniques. Treatment should be tailored to the specific type identified, rather than blindly opting for neuroendoscopy. Particularly for SACH-R2a cases, we recommend initial ventriculoperitoneal shunting.
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