Clinical Characteristics and Surgical Management of Symptomatic Trans-sellar Encephalocele: Two Case Reports and a Review of the Literature
Trans-sellar encephalocele is a rare subtype of basal encephalocele characterized by herniation of neural tissue through a defect in the sellar floor, extending into the sphenoid sinus or nasopharynx. Due to its deep anatomical location and proximity to critical neurovascular and endocrine structures, trans-sellar encephalocele presents significant diagnostic and surgical challenges. We report two adult cases of symptomatic trans-sellar encephalocele with progressive visual impairment, both successfully treated via endoscopic endonasal surgery. In addition, to contextualize these cases, we conducted a comprehensive literature review of 47 previously reported symptomatic trans-sellar encephalocele that was surgically treated in the neurosurgical field over the past quarter century, resulting in a total 49 patients (33 males, 16 females). Clinical characteristics, imaging findings, surgical approaches, and outcomes were analyzed. Of the 49 patients, the common symptoms included nasal obstruction (59.2%), endocrine dysfunction (46.9%), cerebrospinal fluid leakage (30.6%), and visual impairment (24.5%). The majority (69.4%) were diagnosed at age six or younger. Surgery was performed primarily via transnasal approaches (49.4%). Outcomes were available in 44 cases; 81.8% experienced symptom resolution or improvement. In our two cases, postoperative imaging confirmed the reduction of the trans-sellar encephalocele and cessation of cerebrospinal fluid inflow. Visual symptoms improved without recurrence or complications. Early diagnosis and tailored surgical intervention are essential for optimizing outcomes in trans-sellar encephalocele. The endoscopic endonasal approach enables safe and effective reduction of the trans-sellar encephalocele while preserving critical structures. Modifying of cerebrospinal fluid dynamics appears to play a key role in halting symptom progression. Long-term follow-up and further studies are warranted to establish standardized management strategies.
Authors
- Shinichiro Koizumi (ORCID: https://orcid.org/0000-0003-1104-6824)
- Kosaku Amano (ORCID: https://orcid.org/0000-0002-5589-4852)
- Takakazu Kawamata (ORCID: https://orcid.org/0000-0002-6076-7065)
- Yukihiro MATSUYAMA
- Kazuhiko Kurozumi (ORCID: https://orcid.org/0000-0002-6942-9919)
- Dinh Thi Phuong Hoai (ORCID: https://orcid.org/0000-0002-9807-5953)
Institutions
- Hamamatsu University School of Medicine (JP)
- Tokyo Women's Medical University (JP)
Publication Details
- Journal
- NMC Case Report Journal
- Published
- 2025-12-04
- DOI
- https://doi.org/10.2176/jns-nmc.2025-0222
- Primary Topic
- Head and Neck Surgical Oncology
- Type
- article
- Field-Weighted Citation Impact
- 0.00