Basal encephalocele: surgical strategy and functional outcomes in the Tokyo experience

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  • 1 Department of Neurosurgery, Division of Pediatric Neurosurgery, Kitasato University School of Medicine, Sagamihara;
  • 2 Divisions of Neurosurgery and
  • 3 Plastic and Reconstructive Surgery, Tokyo Metropolitan Children’s Medical Center, Tokyo; and
  • 4 Divisions of Neurosurgery and
  • 5 Plastic Surgery, National Center for Child Health and Development, Tokyo, Japan
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OBJECTIVE

The basal encephalocele (BEC) is the rarest form of encephalocele, with an incidence of about 1/35,000 live births. The incidence of its subtype, sphenoidal BEC, is even lower at about 1/700,000 live births. The aim of this study was to propose the optimal surgical approach to repairing BEC, with special attention to the reconstruction of the skull base bone defect.

METHODS

Fourteen consecutive pediatric patients with BEC who underwent surgical repair between March 2004 and March 2020 (10 boys and 4 girls, age 25 days to 7 years, median age 4 months) were enrolled. The follow-up period of the surviving patients ranged from 53 to192 months (mean 119.8 months). The patient demographics, BEC subtypes, preoperative clinical condition, radiographic findings, surgical procedures, and postoperative course were retrospectively analyzed.

RESULTS

There were 4, 8, and 2 cases of sphenoidal BEC, sphenoethmoidal BEC, and ethmoidal BEC, respectively. The size of the bone defect was small in 3 patients, medium in 7, and large in 4 patients. All the patients with sphenoethmoidal and ethmoidal BEC showed associated congenital anomalies other than cleft palate. In total, 25 operations were performed. Two patients underwent multiple operations, whereas the remaining 9 patients received only 1 operation. The transoral transpalatal approach was the initial procedure used in all 14 patients. The transfrontobasal approach was applied as an additional procedure in 2 patients and as part of a 1-stage combined operation in 2 patients. Autograft bone alone was used for skull base reconstruction in 17 early operations. A titanium mesh/plate was used in the remaining 8 operations without any perioperative complications. All BECs were successfully repaired. Three patients died during the clinical course due to causes unrelated to their surgery. All but one of the surviving patients started growth hormone replacement therapy before school age.

CONCLUSIONS

Based on the authors’ limited experience, the key to successful BEC repair involves circumferential dissection of the BEC and a firm reconstruction of the skull base bone defect with a titanium plate/mesh. The transoral transpalatal approach is a promising, reliable procedure that may be used in the initial operation. When a cleft palate is absent, transnasal endoscopic repair is recommended. The transfrontobasal approach should be reserved for cases with a huge BEC and other anomalies. Long-term prognosis is apparently favorable in survivors.

ABBREVIATIONS BEC = basal encephalocele; ICA = internal carotid artery; LD = lumbar drain; TFB = transfrontobasal; TOTP = transoral transpalatal; VP = ventriculoperitoneal.

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Contributor Notes

Correspondence Nobuhito Morota: Kitasato University School of Medicine, Sagamihara, Kanagawa, Japan. nobu.m01@gmail.com.

INCLUDE WHEN CITING Published online October 30, 2020; DOI: 10.3171/2020.6.PEDS20315.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

  • 1

    Lewin ML. Sphenoethmoidal cephalocele with cleft palate: transpalatal versus transcranial repair. Report of two cases. J Neurosurg. 1983;58(6):924931.

    • Search Google Scholar
    • Export Citation
  • 2

    Yokota A, Matsukado Y, Fuwa I, Anterior basal encephalocele of the neonatal and infantile period. Neurosurgery. 1986;19(3):468478.

  • 3

    Suwanwela C, Suwanwela N. A morphological classification of sincipital encephalomeningoceles. J Neurosurg. 1972;36(2):201211.

  • 4

    Ogiwara H, Morota N. Surgical treatment of transsphenoidal encephaloceles: transpalatal versus combined transpalatal and transcranial approach. J Neurosurg Pediatr. 2013;11(5):505510.

    • Search Google Scholar
    • Export Citation
  • 5

    Sanjari R, Mortazavi SA, Amiri RS, Intrasphenoidal meningo-encephalocele: report of two rare cases and review of literature. Surg Neurol Int. 2013;4:5.

    • Search Google Scholar
    • Export Citation
  • 6

    Tirumandas M, Sharma A, Gbenimacho I, Nasal encephaloceles: a review of etiology, pathophysiology, clinical presentations, diagnosis, treatment, and complications. Childs Nerv Syst. 2013;29(5):739744.

    • Search Google Scholar
    • Export Citation
  • 7

    Kennedy EM, Gruber DP, Billmire DA, Crone KR. Transpalatal approach for the extracranial surgical repair of transsphenoidal cephaloceles in children. J Neurosurg. 1997;87(5):677681.

    • Search Google Scholar
    • Export Citation
  • 8

    Lewin ML, Shuster MM. Transpalatal correction of basilar meningocele with cleft palate. Arch Surg. 1965;90(5):687693.

  • 9

    Gerhardt HJ, Mühler G, Szdzuy D, Biedermann F. Therapy problems in sphenoethmoidal meningoceles. Article in German. Zentralbl Neurochir. 1979;40(1):8594.

    • Search Google Scholar
    • Export Citation
  • 10

    Hoff SR, Edwards MSB, Bailey CM, Koltai PJ. The transpalatal approach to repair of congenital basal skull base cephaloceles. J Neurol Surg B Skull Base. 2014;75(2)(B2):96103.

    • Search Google Scholar
    • Export Citation
  • 11

    Zeinalizadeh M, Sadrehosseini SM, Habibi Z, Endonasal management of pediatric congenital transsphenoidal encephaloceles: nuances of a modified reconstruction technique. Technical note and report of 3 cases. J Neurosurg Pediatr. 2017;19(3):312318.

    • Search Google Scholar
    • Export Citation
  • 12

    Itakura T, Miyamoto K, Uematsu Y, Bilateral morning glory syndrome associated with sphenoid encephalocele. Case report. J Neurosurg. 1992;77(6):949951.

    • Search Google Scholar
    • Export Citation
  • 13

    Macfarlane R, Rutka JT, Armstrong D, Encephaloceles of the anterior cranial fossa. Pediatr Neurosurg. 1995;23(3):148158.

  • 14

    Spacca B, Amasio ME, Giordano F, Surgical management of congenital median perisellar transsphenoidal encephaloceles with an extracranial approach: a series of 6 cases. Neurosurgery. 2009;65(6):11401146.

    • Search Google Scholar
    • Export Citation
  • 15

    Kohan E, Lazareff J, Kawamoto H, Bradley JP. Successful staged correction of transsphenoidal encephaloceles. Plast Reconstr Surg. 2010;126(1):197204.

    • Search Google Scholar
    • Export Citation
  • 16

    Formica F, Iannelli A, Paludetti G, Di Rocco C. Transsphenoidal meningoencephalocele. Childs Nerv Syst. 2002;18(6-7):295298.

  • 17

    Faggin R, Pentimalli L, Grazzini M, Combined endoscopic-microsurgical approach for transsphenoidal (sphenopalatine) encephalocele with an intralesional pituitary gland. Case report. J Neurosurg Pediatr. 2009;4(3):262265.

    • Search Google Scholar
    • Export Citation
  • 18

    Abe T, Lüdecke DK, Wada A, Matsumoto K. Transsphenoidal cephaloceles in adults. A report of two cases and review of the literature. Acta Neurochir (Wien). 2000;142(4):397400.

    • Search Google Scholar
    • Export Citation
  • 19

    Di Rocco F, Couloigner V, Dastoli P, Treatment of anterior skull base defects by a transnasal endoscopic approach in children. J Neurosurg Pediatr. 2010;6(5):459463.

    • Search Google Scholar
    • Export Citation
  • 20

    Tan SH, Mun KS, Chandran PA, Combined transnasal and transoral endoscopic approach to a transsphenoidal encephalocele in an infant. Childs Nerv Syst. 2015;31(7):11651169.

    • Search Google Scholar
    • Export Citation
  • 21

    Tyler-Kabara EC. Sphenoidal encephaloceles. J Neurosurg Pediatr. 2013;11(5):504.

  • 22

    Reinard K, Basheer A, Jones L, Surgical technique for repair of complex anterior skull base defects. Surg Neurol Int. 2015;6:20.

  • 23

    Kai Y, Nagahiro S, Yoshioka S, Ushio Y. Application of the skull base technique to the repair of transsphenoidal meningoencephaloceles. Pediatr Neurosurg. 1996;25(1):5456.

    • Search Google Scholar
    • Export Citation

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