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[Median congenital fistula of the upper lip associated with Goldenhar syndrome: Report of a case].

Authors :
Baptista C
Bertrand B
Bardot J
Casanova D
Degardin N
Source :
Annales de chirurgie plastique et esthetique [Ann Chir Plast Esthet] 2016 Apr; Vol. 61 (2), pp. 148-52. Date of Electronic Publication: 2015 May 23.
Publication Year :
2016

Abstract

Aim of the Study: We present the case of a patient with Goldenhar syndrome associated with congenital fistula in the middle of philtrum at the upper lip.<br />Patients and Methods: The patient was supported from birth for Goldenhar syndrome. Several procedures were performed in childhood to correct a macrostomia and mandibular hypoplasia right. At 14 years old, she has a rhinoplasty to correct a complex nasal malformation. During the procedure, a hole in the upper central incisor inter-space is found. It is extended by a fistula which runs through the front palate towards the vomer rail at the base of the partition. This fistula is blind behind the vomer and can be completely resected. Pathological examination of the resection reveals a squamous lining.<br />Results: The median fistula of the upper lip are extremely rare : less than 30 cases reported in the literature. The unusual run of the fistula and the recent appearance of a pituitary syndrome in this patient makes us look for a continuity between it and Rathke's pocket by computed tomography imaging type.<br />Conclusion: The association of Goldenhar syndrome and median congenital fistula of the upper lip was never yet described in the literature. The association with hypopituitarism was suspected with the posterior extension of the malformation, not confirmed by the scanner, but strongly suspected jointly by the neurosurgical and plastic surgery team.<br /> (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Details

Language :
French
ISSN :
1768-319X
Volume :
61
Issue :
2
Database :
MEDLINE
Journal :
Annales de chirurgie plastique et esthetique
Publication Type :
Academic Journal
Accession number :
26006304
Full Text :
https://doi.org/10.1016/j.anplas.2015.04.008