RESUMO
The Tessier no. 5 facial cleft is an extremely rare congenital malformation. Only 26 cases have been described in the English-language literature. The cleft begins in the upper lip just medial to the oral commissure, extending across the cheek as a groove ending at the junction of the middle and lateral thirds of the lower eyelid. The bone involvement usually includes an alveolar cleft in the premolar region, extends across the maxilla lateral to the infraorbital nerve, up to the infraorbital rim and orbital floor. The goals of the surgical procedure include reconstructing the lower eyelid, repositioning the lateral canthus, closure of the labiomaxillary cleft, and restoration of the skeletal continuity (including the orbital floor defect) with bone grafts. We present six patients with the Tessier no. 5 facial cleft who have been treated in our combined centers and discuss the surgical options and difficulties faced in the reconstruction of this rare and challenging craniofacial malformation. To date, we have treated six patients (two with bilateral and four with unilateral clefts). Three of the patients with unilateral clefting had an associated no. 4 cleft and one patient with a bilateral cleft had an associated no. 3 cleft. This paper represents the largest series to date documenting surgery for patients with the Tessier no. 5 facial cleft.
Assuntos
Anormalidades Craniofaciais/cirurgia , Face/anormalidades , Procedimentos de Cirurgia Plástica/métodos , Processo Alveolar/anormalidades , Alveoloplastia/métodos , Transplante Ósseo , Bochecha/anormalidades , Bochecha/cirurgia , Criança , Pré-Escolar , Fenda Labial/patologia , Fenda Labial/cirurgia , Pálpebras/anormalidades , Pálpebras/cirurgia , Face/cirurgia , Feminino , Humanos , Lactente , Recém-Nascido , Macrostomia/cirurgia , Masculino , Maxila/anormalidades , Maxila/cirurgia , Músculo Esquelético/transplante , Órbita/anormalidades , Órbita/cirurgia , Transplante de Pele , Retalhos Cirúrgicos , Resultado do Tratamento , Adulto JovemRESUMO
The no. 0-14 cleft involves the midline of the face and cranium. It may include both a true and a false median cleft lip, with or without associated hypotelorism or hypertelorism. The no. 0 cleft is the most common of the craniofacial clefts. The objective of this study was to review the functional outcome and aesthetic results of the different techniques applied for each case. We have conducted a retrospective analysis of our series consisting of 32 cases of Tessier no. 0 cleft, in the period between 1997 and 2007. The patients were divided into 2 groups: those with the true median cleft and those with the false median cleft. The clinical findings, lip malformation, alveolar cleft, nasal appearance, septal involvement, associated deformities, and surgical procedures, were all reviewed. Holoprosencephaly was present in 9 cases, with a false median cleft upper lip and an absence of the premaxilla, septum, and columella (only 1 patient underwent lip and columella reconstruction at 2 years of age). Nine patients had an incomplete median cleft lip. Seven of these cases had associated median alveolar cleft, and 1 had an intranasal tumor, associated with lipoma of corpus callosum, characteristic of the Pai syndrome. Six cases of a bifid nose were seen, 2 of which were associated with an alveolar median cleft and hypertelorism. An isolated median alveolar cleft was present in 7 cases, 2 of them associated with a no. 30 cleft. This article presents a large series of Tessier no. 0 cleft, describing the differences between the false and the true median cleft. The surgical procedures may vary in relation to the type of involvement.
Assuntos
Anormalidades Craniofaciais/classificação , Anormalidades Craniofaciais/cirurgia , Adolescente , Adulto , Criança , Pré-Escolar , Fissura Palatina/cirurgia , Holoprosencefalia/cirurgia , Humanos , Lactente , Recém-Nascido , Estudos Retrospectivos , Adulto JovemRESUMO
The monobloc frontofacial osteotomy provides aesthetic and functional improvement in the treatment of various craniofacial deformities. This procedure, through highly complex, has had some significant associated complication, such as cerebrospinal fluid leakage, hematoma, infection, and bone resorption. Distraction has been successfully used to gradually elongate bone and soft tissue. This method seems to provide improved results over conventional surgery, with less morbidity. We present a case of a patient with Apert syndrome who underwent monobloc advancement using the Rigid External Device (RED) device and who developed a transient bilateral amaurosis on the fourth postoperative day before distraction. A second procedure was performed to push back the frontal bandeau, maintaining the device in position. The blindness was resolved with this procedure as well as treatment with systemic steroids. The distraction was started thereafter, and the desired improvement was acquired. To our knowledge, this is the first case of transient bilateral amaurosis in a patient undergoing monobloc distraction.
Assuntos
Acrocefalossindactilia/terapia , Cegueira/etiologia , Ossos Faciais/cirurgia , Osso Frontal/cirurgia , Osteogênese por Distração/efeitos adversos , Complicações Pós-Operatórias/etiologia , Cegueira/terapia , Criança , Ossos Faciais/anormalidades , Feminino , Osso Frontal/anormalidades , Glucocorticoides/uso terapêutico , Humanos , Metilprednisolona/uso terapêutico , Osteogênese por Distração/métodos , Complicações Pós-Operatórias/terapia , Procedimentos de Cirurgia Plástica/efeitos adversos , Procedimentos de Cirurgia Plástica/métodosRESUMO
Craniofacial microsomia is assciated with hypoplasia of the facial skeleton and musculature. These primary defects cause a secondy alteration of the craniofacial skeleton. Current therapies do not attempt to correct the cranial base deformity in childhoold. Another cause of oblique deformities of the skull is deformational plagiocephaly. This common disorder is secundary to external deformational forces and tends to improve with time and may require only conservative treatment. We present two cases of deformational plagiocephaly superimposed upon hemifacial microsomia. Orthotic treatment was utilized to improve both the deformational plagiocephaly and the cranial base deformity. This novel therapy has the potential to correct the cranial base deformity in craniofacial microsomia.