1/13. Microvascular temporomandibular joint and mandibular ramus reconstruction in hemifacial microsomia.Microvascular temporomandibular joint (TMJ) and mandibular ramus reconstruction was performed in a 4-year-old hemifacial microsomia patient with multiple craniofacial and extracranial anomalies (goldenhar syndrome). Her major craniofacial anomalies included bilateral cleft lip and palate, left macrostomia, left microtia, and complete absence of the left vertical mandibular ramus and TMJ. Most of her other anomalies had been corrected surgically before TMJ and vertical mandibular ramus reconstruction, which was accomplished with a metatarsophalangeal (MTP) joint transplantation. The MTP joint was placed in hyperextended position in the skull base inserting the proximal phalanx under the remnants of the zygomatic arch and replacing the vertical mandibular ramus with the metatarsal bone. Straight mouth opening, correction of the midline, and normalized lateral movements of the mandible were accomplished. The graft includes two epiphyseal plates, which should maintain growth of the transplant. During the follow-up period (16 months) the achieved results have been maintained without adverse effects. The present technique appears to be a promising alternative in the treatment of children with Pruzansky type 3 hemifacial microsomia.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
2/13. prenatal diagnosis of hemifacial microsomia and ipsilateral cerebellar hypoplasia in a fetus with oculoauriculovertebral spectrum.Oculoauriculovertebral spectrum, or goldenhar syndrome, is characterized by varying degrees of prevalently unilateral underdevelopment of craniofacial structures (orbit, ear, mandible) and spinal anomalies. We report the prenatal ultrasonographic diagnosis made at 24 weeks' gestation in a family with a negative history. The prenatal diagnosis was suspected due to the presence of marked hemifacial microsomia and moderate ipsilateral cerebellar hemisphere hypoplasia in the absence of facial clefting.- - - - - - - - - - ranking = 0.5keywords = craniofacial (Clic here for more details about this article) |
3/13. Vertically fused tracheal cartilage. An underrecognized anomaly.Anterior vertical fusion of the tracheal cartilages with normal pars membranacea is a rarely described tracheal anomaly. We report four cases of this anomaly, three associated with craniosynostosis syndromes and one with Goldenhar's syndrome. The anomaly was documented at postmortem examination in one case, both endoscopically and at tracheotomy in two cases, and endoscopically in one case. documentation from gross postmortem photographs, photomicrographs, and intraoperative endoscopic photographs is presented so that the anatomical aspects of this anomaly can be thoroughly understood and recognized. All four newborn patients developed recurrent lower respiratory tract infections, reactive airway disease, and chronically retained secretions attributable to this anomaly. Neither tracheal stenosis nor stridor was encountered. In the three surviving patients, the infections grew less frequent and less severe in later childhood. These observations suggest that this tracheobronchial anomaly may be associated with a variety of craniofacial syndromes and may alter airflow dynamics in a way that predisposes to retained secretions and chronic pulmonary disease.- - - - - - - - - - ranking = 0.5keywords = craniofacial (Clic here for more details about this article) |
4/13. Management of the difficult pediatric airway with Shikani Optical Stylet.The airways of children with many craniofacial and mandibulofacial malformations often present unique challenges to the anesthesiologists. We report the application of Shikani Optical Stylet (SOS) in facilitating the tracheal intubation of four children with history of difficult airway management. The SOS combines the benefits of the lightwand and fiberoptic bronchoscope.- - - - - - - - - - ranking = 0.5keywords = craniofacial (Clic here for more details about this article) |
5/13. Mandibular distraction: evolution of treatment protocols in hemifacial microsomy.Osteodistraction is currently used in those disorders presenting with osseus tissue deficit, excellent results having been obtained in the craniofacial complex. The factors contributing to the success of this procedure are a thin layer of subcutaneous tissue, minimum movement of the cutis, good vascularization of the soft tissues, and the good healing that ensues. The good plastic effects on the soft tissues and the possibility of constantly modulating the strength and monitoring the results have led to distraction osteogenesis gaining increasing consensus in the treatment of congenital and acquired deformities and in some selected cases, for the treatment of osseous deficits caused by trauma, postsurgical outcomes, or even severe alveolar deficits. Furthermore, in the last few years, this surgical technique has been successfully used in the treatment of pediatric deformities such as hemifacial microsomia, some severe class II skeletal deformities, and some syndromic cases, such as the treatment of temporomandibular ankylosis outcomes. In our opinion, guidelines in the indications for distraction are mandatory to select the procedure best suited to the pathologic situation of each individual patient.- - - - - - - - - - ranking = 0.5keywords = craniofacial (Clic here for more details about this article) |
6/13. An interesting case presentation: pulmonary malformations associated with oculoauriculovertebral dysplasia (Goldenhar anomalad).Oculoauriculovertebral dysplasia (OAV) is a heterogeneous field defect involving the first and second branchial arches and is characterized by microtia, mandibular hypoplasia, vertebral anomalies, and epibulbar dermoids. We report a case of OAV with pulmonary manifestations and review the literature regarding this association. Anomalies identified were previously undescribed tracheal stenosis, along with tracheoesophageal cleft and unilateral pulmonary agenesis. Recognition of the pulmonary malformations associated with OAV may lead clinicians to consider a diagnostic measure such as flexible fiberoptic endoscopy in the evaluation of infants with craniofacial malformations and respiratory distress.- - - - - - - - - - ranking = 0.5keywords = craniofacial (Clic here for more details about this article) |
7/13. External cranial vault molding after craniofacial surgery.Many congenital cranial vault deformities are only incompletely corrected by currently available surgical techniques. The supplementary use of external cranial vault molding devices after these surgical techniques, however, has resulted in consistently improved cranial vault from over what could be achieved by operation alone. These devices are inexpensive, easily individualized to abnormal skull shapes, and well tolerated by young patients.- - - - - - - - - - ranking = 2keywords = craniofacial (Clic here for more details about this article) |
8/13. Goldenhar's syndrome: a case study.Goldenhar's Syndrome, a rare symptom complex involving craniofacial and vertebral malformations, is reviewed and a detailed case history of a 19-mo-old exhibiting the syndrome is described. This multiple-problem child exhibited a 6-mo deficit in communication skills at 12 mo of age. After 6 mo of participation in a multidisciplinary early intervention program, including speech-language therapy, the child exhibits normal language although he has articulation problems consistent with his craniofacial defects.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
9/13. Retinal and optic nerve findings in Goldenhar-Gorlin syndrome.Involvement of the posterior segment of the eye in Goldenhar-Gorlin syndrome is more common than is generally appreciated. We examined seven patients with this syndrome. Abnormalities included diminished visual acuity, tilted optic disc, optic nerve hypoplasia, tortuous retinal vessels, macular hypoplasia and heterotopia, microphthalmia and anophthalmia. In one case, pathologic study showed agenesis of the optic nerve. It is proposed that retinal, optic nerve and craniofacial abnormalities in this condition may reflect an asynchrony in the migration of the neural crest cells in the early stages of embryonal development.- - - - - - - - - - ranking = 0.5keywords = craniofacial (Clic here for more details about this article) |
10/13. Surgical correction of Goldenhar's syndrome.Goldenhar's syndrome is a variant of hemifacial microsomia - one of the more common congenital syndromes of the first and second arch. The Goldenhar's type is characterized by varying degrees of underdevelopment of craniofacial structures. The major deformities involve the mandible and the ear; but they also include the orbit and vertebral column, specific defects pathognomonic of this condition. Medical management requires reconstructive surgery of which treatment must take into consideration the timing of surgery and the effects of surgery upon the growth and development of the poorly formed structures. This paper presents a discussion of the pathogenesis and the favorable and unfavorable factors affecting the selection and timing of surgery. The literature is reviewed and two additional cases are added with an evaluation of the effects of early reconstruction. A single-stage procedure, utilizing rib autograft, is described for correction of the defective hemimandible, glenoid fossa, and zygoma. The results are evaluated by means of photographs and radiographs with a follow-up period of at least one and one-half years. The data suggest a remodeling of the grafts to resemble normal structures, and a downward growth of the maxilla to fill a space created by the reconstruction of the mandible and expansion of the lower portion of the face. Multiple stages of surgery as well as unnecessary delays in performing surgical procedures are challenged by the report.- - - - - - - - - - ranking = 0.5keywords = craniofacial (Clic here for more details about this article) |
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