Tags

Type your tag names separated by a space and hit enter

Anterior basal encephalocele in the median cleft face syndrome. Comments on nosology and treatment.
Genet Couns. 1990; 1(2):103-9.GC

Abstract

Two cases of median cleft syndrome with associated basal encephalocele are presented. The median cleft face syndrome consists of several craniofacial defects and occurs, as far as we know, sporadically. Extracranial defects should be looked for carefully since they may indicate the presence of a specific syndrome with its own prognosis and mode of inheritance. Basal encephaloceles are sometimes part of the syndrome and may cause serious, or even life-threatening, symptoms. With regard to the treatment of basal encephaloceles there still exists a lot of controversion, but most authors agree that it is better to postpone operation until after the age of three because mortality is lower then.

Authors+Show Affiliations

Grubben C
Centre for Human Genetics, University Hospital Leuven, Belgium.
Fryns JP
No affiliation info available
De Zegher F
No affiliation info available
Van Den Berghe H
No affiliation info available

MeSH

Abnormalities, MultipleChild, PreschoolCleft LipCleft PalateEncephaloceleFacial BonesFemaleFollow-Up StudiesHumansInfantMaleSkullSyndrome

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

2080994

Citation

Grubben, C, et al. "Anterior Basal Encephalocele in the Median Cleft Face Syndrome. Comments On Nosology and Treatment." Genetic Counseling (Geneva, Switzerland), vol. 1, no. 2, 1990, pp. 103-9.
Grubben C, Fryns JP, De Zegher F, et al. Anterior basal encephalocele in the median cleft face syndrome. Comments on nosology and treatment. Genet Couns. 1990;1(2):103-9.
Grubben, C., Fryns, J. P., De Zegher, F., & Van Den Berghe, H. (1990). Anterior basal encephalocele in the median cleft face syndrome. Comments on nosology and treatment. Genetic Counseling (Geneva, Switzerland), 1(2), 103-9.
Grubben C, et al. Anterior Basal Encephalocele in the Median Cleft Face Syndrome. Comments On Nosology and Treatment. Genet Couns. 1990;1(2):103-9. PubMed PMID: 2080994.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Anterior basal encephalocele in the median cleft face syndrome. Comments on nosology and treatment. AU - Grubben,C, AU - Fryns,J P, AU - De Zegher,F, AU - Van Den Berghe,H, PY - 1990/1/1/pubmed PY - 1990/1/1/medline PY - 1990/1/1/entrez SP - 103 EP - 9 JF - Genetic counseling (Geneva, Switzerland) JO - Genet Couns VL - 1 IS - 2 N2 - Two cases of median cleft syndrome with associated basal encephalocele are presented. The median cleft face syndrome consists of several craniofacial defects and occurs, as far as we know, sporadically. Extracranial defects should be looked for carefully since they may indicate the presence of a specific syndrome with its own prognosis and mode of inheritance. Basal encephaloceles are sometimes part of the syndrome and may cause serious, or even life-threatening, symptoms. With regard to the treatment of basal encephaloceles there still exists a lot of controversion, but most authors agree that it is better to postpone operation until after the age of three because mortality is lower then. SN - 1015-8146 UR - https://www.unboundmedicine.com/medline/citation/2080994/Anterior_basal_encephalocele_in_the_median_cleft_face_syndrome__Comments_on_nosology_and_treatment_ DB - PRIME DP - Unbound Medicine ER -