Frontoethmoidal encephalocele: clinical presentation, diagnosis, treatment, and complications in 400 cases

Childs Nerv Syst. 2018 Jun;34(6):1161-1168. doi: 10.1007/s00381-017-3716-3. Epub 2018 Jan 5.


Purpose: The purpose of this study is to review a large series of frontoethmoidal encephalocele (FEE) regarding their clinical presentation, the progressiveness of the mass volume, the skin stigmata as well as its surgical approach and post-surgical complications.

Method: Records of all FEE patients treated in Soetomo General Hospital, Surabaya, and Charity Foundation Program from 2008 to 2015 were reviewed. Detailed patient's demography, clinical findings, radiology results, operative procedures, and complications were documented. Follow-up was organized in weekly basis for the first 1 month after surgery or more often when situation or complication occurred. Wound healing, neurological assessment for new or progressive deficit, pseudomeningocele, skin breakdown, cerebrospinal fluid (CSF) leakage, exposed implant, recurrent mass, and cosmetic results were documented. Since most of the patients had no direct phone line at their hometown, we relied on social worker to contact them.

Results: One-stage surgery was performed for 400 patients with FEE (212 were male and 188 were female). Of 400 patients, 388 (97%) were younger than 18 years old. Most FEEs were nasoethmoidal, either isolated or combined with nasoorbital type (347 cases [86.75%]); nasofrontal subtypes were seen in 34 cases (8.5%) and nasoorbital in 14 cases (1.5%). The mean operative time was 2 h (range 30 min-3 h). There were only two patients (0.5%) needed postoperative blood transfusions. Mean hospitalization time was 5 days (range 4-7 days). Overall, complication rate in our series was 12.5%, mostly was CSF leakage and wound dehiscence.

Conclusion: The current socioeconomic conditions and local facility should be considered to treat these specific disease processes. The refined and meticulous technique, especially in choosing the approach and handling the dural closure, is essential in lowering the complication rate.

Keywords: Developing country; Neural tube defect; Pediatric neurosurgery; Surgical technique.

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Encephalocele / diagnosis*
  • Encephalocele / pathology*
  • Encephalocele / surgery*
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Neurosurgical Procedures / adverse effects
  • Neurosurgical Procedures / methods*
  • Osteotomy / methods*
  • Plastic Surgery Procedures / methods
  • Postoperative Complications / epidemiology
  • Young Adult