Oral-nasal-ocular cleft: the greatest challenge among the rare clefts.
BACKGROUND
Number 3 cleft or oral-nasal-ocular cleft is a well-known entity that was described by Morian over a century ago. This malformation is a paranasal-medial orbitomaxillary cleft running across the lacrimal segment of the lower eyelid and over the lacrimal groove. The Tessier number 3 naso-ocular cleft represents one of the most difficult and challenging malformations to correct for the reconstructive surgeon. We have conducted a retrospective analysis of our series consisting of 21 cases.


OBJECTIVE
The objective was to review the functional outcome and aesthetic results of the different techniques applied for each case.


METHODS
From 1997 to 2007, 21 patients with a Tessier number 3 cleft were treated in our craniofacial units. The clinical findings, tomographic studies, and surgical procedures were reviewed and analyzed. We have discussed our protocol of the treatment.


RESULTS
We have treated facial malformation in 2 craniofacial centers. Fourteen patients were evaluated in the first year of their life, with an average age at presentation of 3 years. Twelve patients were female, and 9 were male; 6 patients had amniotic bands in limbs, 5 patients had an association with Tessier number 11 cleft, 3 patients with number 9 cleft, and 1 with number 7 cleft. Related to cleft lip, 10 patients had bilateral cleft lip, and 8 patients had unilateral cleft lip. Three patients did not have any involvement of the upper lip. The alar base was deviated upward in 19 patients, 11 cases had severe anatomic alteration with the lateral border of the ala above the medial canthus, and 8 cases had a mild dislocation. Nine cases of lacrimal duct obstruction and 8 cases of lacrimal duct extrophy were identified. Twelve patients had a lower eyelid coloboma of varying grades, and there were 2 cases of microblepharia. Aiming the soft tissue reconstruction, eyelid, nose, and upper lip were evaluated regarding their position, absence of tissue, and position of medial canthus and ala. Twelve of our patients underwent correction in the same moment, their medial canthus rotated upward and the ala downward, using the contralateral side as the reference. The lip was treated using a Millard-like technique. Neo-conjunctivorhinostomy was performed in the same moment in 2 patients or later in 1 case. Four patients had plagiocephaly due to the cranial involvement, and they were submitted to cranioplasty. Three had neurosurgical approach and advancement of the frontal bandeau. One adult patient received an acrylic plate to reshape the frontal area.


CONCLUSIONS
Tessier number 3 cleft is one of the most difficult and challenging malformations to correct for the reconstructive surgeon. Besides the difficulties of its treatment, patients with Tessier number 3 cleft may achieve good results when the team has good skills.
da Silva Freitas
Renato
R
Section of Plastic and Reconstructive Surgery, Federal University of Paraná, Curitiba, Paraná. dr.renato.freitas@gmail.com
Alonso
Nivaldo
N
Busato
Luciano
L
Ueda
Wellington Keity
WK
Hota
Thiago
T
Medeiros
Simone Helena
SH
Kunz
Regiane Tiemi
RT
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D002971:Cleft Lip / Q000601:surgery; D003103:Coloboma / Q000601:surgery; D003608:Dacryocystorhinostomy; D004954:Esthetics; D005143:Eyelids / Q000002:abnormalities / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007766:Lacrimal Apparatus Diseases / Q000601:surgery; D008297:Male; D008437:Maxilla / Q000002:abnormalities* / Q000601:surgery; D019767:Maxillofacial Abnormalities / Q000145:classification / Q000601:surgery; D009666:Nose / Q000002:abnormalities* / Q000601:surgery; D009915:Orbit / Q000002:abnormalities* / Q000601:surgery; D019651:Plastic Surgery Procedures / Q000379:methods*; D012189:Retrospective Studies; D016896:Treatment Outcome