Naso-orbital encephalocele

MedGen UID:: 82875
•Concept ID:: C0271330
•: Congenital Abnormality

Synonym:	Orbital encephalocele
SNOMED CT:	Encephalocele of orbit (15671007); Orbital encephalocele (15671007)

HPO:	HP:0007115

Definition

A type of encephalocele that occurs along the medial orbit wall at the level of the frontal process of the maxilla and the ethmoid-lacrimal bone junction. [from HPO]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVNaso-orbital encephalocele

Fetal anomaly
- Congenital Systemic Disorder
  - Abnormality of the nervous system
    - Encephalocele
      - Anterior encephalocele
        Frontal encephalocele
        Naso-orbital encephalocele

Conditions with this feature

Oculocerebrocutaneous syndrome

MedGen UID:: 163214
•Concept ID:: C0796092
•: Disease or Syndrome

A rare neurologic disease typically characterized by the triad of eye, central nervous system and skin malformations, and often associated with an intellectual disability.

See: Condition Record

Oculocerebrocutaneous syndrome

Recent clinical studies

Etiology

Internasal: A New Type of Frontoethmoidal Encephalomeningocele.

Sirimaharaj W
Ann Plast Surg 2018 Oct;81(4):423-426. doi: 10.1097/SAP.0000000000001580. PMID: 30067526

Frontoethmoidal encephalomeningocele: surgical correction by the Chula technique.

Mahatumarat C, Rojvachiranonda N, Taecholarn C
Plast Reconstr Surg 2003 Feb;111(2):556-65; discussion 566-7. doi: 10.1097/01.PRS.0000040523.57406.94. PMID: 12560676

Frontoethmoidal encephaloceles: reconstruction and refinements.

Holmes AD, Meara JG, Kolker AR, Rosenfeld JV, Klug GL
J Craniofac Surg 2001 Jan;12(1):6-18. doi: 10.1097/00001665-200101000-00003. PMID: 11314190

Anterior encephaloceles: a report of 30 cases.

Mahapatra AK, Tandon PN, Dhawan IK, Khazanchi RK
Childs Nerv Syst 1994 Nov;10(8):501-4. doi: 10.1007/BF00335071. PMID: 7882371

See all (4)

Diagnosis

Rare combination of frontonasal and bilateral naso-orbital encephaloceles.

Alexander AA, Saettele MR, L'heureux D, Shah PA, Fickenscher KA
J Radiol Case Rep 2011;5(11):1-7. Epub 2011 Nov 1 doi: 10.3941/jrcr.v5i11.715. PMID: 22470768 Free PMC Article

Transilluminant naso-orbital dermoid cyst masquerading as meningocele.

Wani BN, Jajoo SN, Bhole AM
J Craniofac Surg 2010 May;21(3):930-2. doi: 10.1097/SCS.0b013e3181d7f19f. PMID: 20485086

Frontoethmoidal encephalomeningocele: new morphological findings and a new classification.

Rojvachiranonda N, David DJ, Moore MH, Cole J
J Craniofac Surg 2003 Nov;14(6):847-58. doi: 10.1097/00001665-200311000-00006. PMID: 14600626

Nasal encephaloceles: definitive one-stage reconstruction.

Sargent LA, Seyfer AE, Gunby EN
J Neurosurg 1988 Apr;68(4):571-5. doi: 10.3171/jns.1988.68.4.0571. PMID: 3351585

Pseudoepiphora from cerebrospinal fluid leak: case report.

Dryden RM, Wulc AE
Br J Ophthalmol 1986 Aug;70(8):570-4. doi: 10.1136/bjo.70.8.570. PMID: 3741820 Free PMC Article

See all (5)

Therapy