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.

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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
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article
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article

Clinical Characteristics and Surgical Management of Symptomatic Trans-sellar Encephalocele: Two Case Reports and a Review of the Literature

Shinichiro Koizumi, Kosaku Amano, Takakazu Kawamata, Yukihiro MATSUYAMA et al.
NMC Case Report Journal
Head and Neck Surgical Oncology
article

Clinical Characteristics and Surgical Management of Symptomatic Trans-sellar Encephalocele: Two Case Reports and a Review of the Literature

Shinichiro Koizumi, Kosaku Amano, Takakazu Kawamata, Yukihiro MATSUYAMA, Kazuhiko Kurozumi, Dinh Thi Phuong Hoai
article en

Abstract

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.

NMC Case Report JournalVol. 12(0)
Hamamatsu University School of Medicine (JP), Tokyo Women's Medical University (JP)
Openalex Percentile: Top 42%
Head and Neck Surgical Oncology
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