Hostname: page-component-586b7cd67f-r5fsc Total loading time: 0 Render date: 2024-11-24T16:35:28.625Z Has data issue: false hasContentIssue false
  • English
  • Français

Frontoethmoidal encephalomeningocoele with colpocephaly: case report and clinical review

Published online by Cambridge University Press:  01 August 2007

Abhinandan Bhattacharjee ,
A Chakraborty  and
P Purkaystha
Get access Rights & Permissions [Opens in a new window]

Abstract

Frontoethmoidal encephalomeningocoele is a rare congenital disease in which an intracranial mass protrudes through a midline defect from the anterior cranial fossa into the facial skeleton. The condition affects patients in South East Asian countries, such as Thailand, Burma, Malaysia and Indonesia, with frequency of 1 in 5000. The pathogenesis of encephalocoeles may be regarded as a ‘late’ neurulation defect during the fourth gestational week.

We present a case of frontoethmoidal encephalomeningocoele with corpus callosal agenesis and colpocephaly; this may well be the first report of this combination. The patient had a bulging mass in the middle frontonasal area, with broadening of the nasal bridge and hypertelorism. Computed tomography scans delineated the skull defect and associated brain anomalies. A one-stage, combined transfacial–transcranial approach, correctional procedure was performed. We present here a discussion of the findings, with special reference to the condition's pathogenesis, morphological classification and evolving surgical treatments.

Early diagnosis and referral, involving multidisciplinary teamwork, are of paramount importance because of the distorting influence of the extruding mass on facial growth.

Keywords

Encephalocele, FrontalCongenital Malformations, Nervous SystemSkull BaseTomography, Computerized
Type
Research Article
Information
The Journal of Laryngology & Otology , Volume 122 , Issue 3 , March 2008 , pp. 321 - 323
Copyright
Copyright © JLO (1984) Limited 2007

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1 Ramachandran, CRS, Phelps, PD. Nasal encephaloceles associated with unilateral absence of cochlea. J Laryngol Otol 1991;9:813–17Google Scholar
2 Hoving, EW, Vermeij-Keers, C, Mommaas-Kienhuis, AM, Hartwig, NG. Separation of neural and surface ectoderm after closure of the rostral neuropore. Anat Embryol (Berl) 1990;182:455–6310.1007/BF00178910Google Scholar
3 Meling, TR, Due-Tonnessen, BJ, Helseth, E, Skjelbred, P, Arctander, K. Frontoethmoidal meningoencephaloceles. Tidsskr Nor Laegeforen 2000;120:2250–2Google ScholarPubMed
4 Mahapatra, AK, Suri, A. Anterior encephaloceles: a study of 92 cases. Pediatr Neurosurg 2002;36:113–18Google Scholar
5 Sadewa, AH, Sutomo, R, Istiadjid, M, Nishiyama, K, Shirakawa, T, Matsuo, M et al. C677T mutation in the MTHFR gene was not found in patients with frontoethmoidal encephalocele in East Java, Indonesia. Pediatr Int 2004;46:409–1410.1111/j.1442-200x.2004.01927.xCrossRefGoogle Scholar
6 Hoving, EW, Vermeij-Keers, C. Frontoethmoidal encephaloceles: a study of their pathogenesis. Pediatr Neurosurg 1997;27:246–56CrossRefGoogle ScholarPubMed
7 David, DJ, Sheffield, L, Simpson, D, White, J. Fronto-ethmoidal meningoencephaloceles: morphology and treatment. Br J Plast Surg 1984;37:271–84CrossRefGoogle ScholarPubMed
8 Boonvisut, S, Ladpli, S, Sujatanond, M, Tandhavadhana, C, Tisavipat, N, Luxsuwong, M et al. Morphologic study of 120 skull base defects in frontoethmoidal encephalo-meningoceles. Plast Reconstr Surg 1998;101:1784–95Google Scholar
9 Chari, R, Bhargava, R, Hammond, DI, Ventureyra, EC, Lalonde, AB. Antenatal unilateral hydrocephalus. Can Assoc Radiol J 1993;44:57–9Google ScholarPubMed
10 Moore, MH, Blackwell, S, Butterworth, MJ. The frontal sinus in frontoethmoidal meningoencephaloceles. Craniofac Surg 1994;5:185–710.1097/00001665-199407000-00010CrossRefGoogle ScholarPubMed
11 Shah, AK, Desai, AA, Sharma, SN, Udwadia, RB. Frontoethmoidal meningoencephalo-cystocele. Ann Plast Surg 1989;22:523–7, 528CrossRefGoogle Scholar
12 Lundorf, E, Kjeldgaard, A, Halaburt, H. Coronal CT examination in the evaluation of small frontoethmoidal encephalocoeles. J Laryngol Otol 1988;102:1119–21Google Scholar
13 Rojvachiranonda, N, David, DJ, Moore, MH, Cole, J. Frontoethmoidal encephalomeningocele: new morphological findings and a new classification. J Craniofac Surg 2003;14:847–5810.1097/00001665-200311000-00006CrossRefGoogle ScholarPubMed
14 David, DJ, Simpson, DA. Frontoethmoidal meningoencephaloceles. Clin Plast Surg 1987;14:83–9Google Scholar
15 Mahatumarat, C, Rojvachiranonda, N, Taecholarn, C. Frontoethmoidal encephalo-meningocele: surgical correction by the Chula technique. Plast Reconstr Surg 2003;111:556–65, 566–710.1097/01.PRS.0000040523.57406.94CrossRefGoogle Scholar
16 Boonvisut, S, Ladpli, S, Sujatanond, M, Tisavipat, N, Luxsuwong, M, Nunta-aree, S et al. A new technique for the repair and reconstruction of frontoethmoidal encephalo-meningoceles by medial orbital composite-unit translocation. Br J Plast Surg 2001;54:93–10110.1054/bjps.2000.3487Google Scholar
17 Holmes, AD, Meara, JG, Kolker, AR, Rosenfeld, JV, Klug, GL. Frontoethmoidal encephaloceles: reconstruction and refinements. J Craniofac Surg 2001;12:6–18Google Scholar
18 Mahatumarat, C, Taecholarn, C, Rojvachiranonda, N. Spontaneous closure of bony defect in a frontoethmoidal encephalomeningocele patient. J Craniofac Surg 1999;10:149–54Google Scholar
19 Hoving, EW. Nasal encephaloceles. Childs Nerv Syst 2000;16:702–610.1007/s003810000339CrossRefGoogle ScholarPubMed
20 Van Den Abbeele, T, Elmaleh, M, Herman, P, François, M, Narcy, P. Transnasal endoscopic repair of congenital defects of the skull base in children. Arch Otolaryngol Head Neck Surg 1999;125:580–4CrossRefGoogle ScholarPubMed
21 Donnenfeld, AE, Hughes, H, Weiner, S. Prenatal diagnosis and perinatal management of frontoethmoidal meningoencephalocele. Am J Perinatol 1988;5:51–3Google Scholar
22 Murali, R, Douyon, P, Omeis, I. Large anterior skull base lesions in patients presenting with obstructive sleep apnea. Report of two cases and review of the literature. J Neurosurg 2004;101:163–6Google Scholar
23 Rao, BD, Subhramanyam, MV, Chari, KVN. Intranasal meningo-encephalocele. Int Surg 1974:59;421–2Google Scholar
24 Hafeez, AB, Sharma, R, Faillace, WJ. Congenital midline nasofrontal mass – two case reports with clinical review. Clin Pediatr (Phila) 1995;34:482–6Google Scholar