Sublabial transsphenoidal microsurgical technique to treat congenital transsphenoidal encephalocele: a technical note

  • Luigi Albano
  • Veronica Parisi
  • Stefania Acerno
  • Lina Raffaella Barzaghi
  • Marco Losa
  • Pietro Mortini
Technical Note


Encephalocele is a rare malformation consisting in herniation of cranial contents through a cranial defect. A transsphenoidal location is uncommon, representing 5% of all basal encephaloceles. The surgical treatment of transsphenoidal encephaloceles is challenging. An optimal approach has not yet been determined, and it varies according to the surgical experience. We report the surgical management of a transsphenoidal encephalocele. The encephalocele and the sellar defect were repaired through a sublabial transsphenoidal microsurgical approach (TSM). Preoperative magnetic resonance imaging (MRI) and computed tomography (CT) scans were crucial for surgical planning. The sublabial transsphenoidal microsurgical approach offered a good and complete exposure of both the sac and the bone defect. Therefore, the congenital defect was successfully repaired with complete resolution of the encephalocele without any surgical or medical complications. Postoperative CT scan and MRI showed the restoration of the bone defect and the recovery of a normal anatomy with herniated structures pushed back into the sella. The described sublabial transsphenoidal microsurgical approach represents a minimally invasive, safe, and effective treatment strategy for transsphenoidal encephalocele.


Transsphenoidal encephalocele Basal meningoencephalocele Transsphenoidal approach Congenital Microsurgery 


Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

The study has been performed in accordance with the ethical standards as laid down in the 1964 Declaration of Helsinki and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from parents of the child included in the study.


  1. 1.
    Abe T, Ludecke DK, Wada A, Matsumoto K (2000) Transsphenoidal cephaloceles in adults. A report of two cases and review of the literature. Acta Neurochir 142:397–400CrossRefGoogle Scholar
  2. 2.
    Ammirati M, Wei L, Ciric I (2013) Short-term outcome of endoscopic versus microscopic pituitary adenoma surgery: a systematic review and meta-analysis. J Neurol Neurosurg Psychiatry 84:843–849. CrossRefGoogle Scholar
  3. 3.
    BN F (1982) Midline fusion defects and defects of formation. Saunders, PhiladelphiaGoogle Scholar
  4. 4.
    David DJ (1993) Cephaloceles: classification, pathology, and management--a review. J Craniofac Surg 4:192–202Google Scholar
  5. 5.
    Formica F, Iannelli A, Paludetti G, Di Rocco C (2002) Transsphenoidal meningoencephalocele. Childs Nerv Syst 18:295–298. CrossRefGoogle Scholar
  6. 6.
    Herman P, Sauvaget E, Guichard JP, Tran Ba Huy P (2003) Intrasphenoidal transsellar encephalocele repaired by endoscopic approach. Ann Otol Rhinol Laryngol 112:890–893. CrossRefGoogle Scholar
  7. 7.
    Jho HD, Carrau RL (1997) Endoscopic endonasal transsphenoidal surgery: experience with 50 patients. J Neurosurg 87:44–51. CrossRefGoogle Scholar
  8. 8.
    Kennedy EM, Gruber DP, Billmire DA, Crone KR (1997) Transpalatal approach for the extracranial surgical repair of transsphenoidal cephaloceles in children. J Neurosurg 87:677–681. CrossRefGoogle Scholar
  9. 9.
    Koc K, Anik I, Ozdamar D, Cabuk B, Keskin G, Ceylan S (2006) The learning curve in endoscopic pituitary surgery and our experience. Neurosurg Rev 29:298–305; discussion 305. CrossRefGoogle Scholar
  10. 10.
    Kohan E, Lazareff J, Kawamoto H, Bradley JP (2010) Successful staged correction of transsphenoidal encephaloceles. Plast Reconstr Surg 126:197–204. CrossRefGoogle Scholar
  11. 11.
    Spacca B, Amasio ME, Giordano F, Mussa F, Busca G, Donati P, Genitori L (2009) Surgical management of congenital median perisellar transsphenoidal encephaloceles with an extracranial approach: a series of 6 cases. Neurosurgery 65:1140–1145; discussion 1145-1146. CrossRefGoogle Scholar
  12. 12.
    Starke RM, Raper DM, Payne SC, Vance ML, Oldfield EH, Jane JA, Jr. (2013) Endoscopic vs microsurgical transsphenoidal surgery for acromegaly: outcomes in a concurrent series of patients using modern criteria for remission. J Clin Endocrinol Metab 98:3190–3198. doi:
  13. 13.
    Steven RA, Rothera MP, Tang V, Bruce IA (2011) An unusual cause of nasal airway obstruction in a neonate: trans-sellar, trans-sphenoidal cephalocoele. J Laryngol Otol 125:1075–1078. CrossRefGoogle Scholar
  14. 14.
    Suwanwela C (1972) Geographical distribution of fronto-ethmoidal encephalomeningocele. Br J Prev Soc Med 26:193–198Google Scholar
  15. 15.
    Tirumandas M, Sharma A, Gbenimacho I, Shoja MM, Tubbs RS, Oakes WJ, Loukas M (2013) Nasal encephaloceles: a review of etiology, pathophysiology, clinical presentations, diagnosis, treatment, and complications. Childs Nerv Syst 29:739–744. CrossRefGoogle Scholar
  16. 16.
    Yokota A, Matsukado Y, Fuwa I, Moroki K, Nagahiro S (1986) Anterior basal encephalocele of the neonatal and infantile period. Neurosurgery 19:468–478CrossRefGoogle Scholar
  17. 17.
    Zeinalizadeh M, Sadrehosseini SM, Habibi Z, Nejat F, Silva HB, Singh H (2017) Endonasal management of pediatric congenital transsphenoidal encephaloceles: nuances of a modified reconstruction technique. Technical note and report of 3 cases. J Neurosurg Pediatr 19:312–318. CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Neurosurgery and Gamma Knife RadiosurgerySan Raffaele Health Institute, Vita-Salute UniversityMilanItaly

Personalised recommendations