The external ear, mandible and other components of hemifacial microsomia

Figueroa, Álvaro A.; Pruzansky, Samuel

doi:10.1016/s0301-0503(82)80042-8

Cited by 92 publications

(50 citation statements)

References 15 publications

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“…Über die Vielfalt und Variabilität der klinischen und radiologischen Symptomatik im KieferGesichts-Bereich wurde im internationalen Schrifttum häu-fig berichtet [2,3,6,7,9,10,12,13,16,18,19,22,24,31,33,[40][41][42].…”

Section: Introductionunclassified

Abnormalities of the TMJ and the Musculature in the Oculo-auriculo-vertebral Spectrum (OAV)

Hirschfelder

Piechot

Schulte

et al. 2004

Journal of Orofacial Orthopedics/Fortschritte der Kieferorthop�

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The abnormalities covered by the generic term "oculo-auriculovertebral spectrum" (OAV) form an exceptional heterogeneous dysmorphic complex characterized by unilateral malformations of miscellaneous craniofacial structures. The aim of the present study was to analyze morphometrically the developmental deficits concerning the temporomandibular joints, the mandibular rami and the mastication muscles by comparing the affected with the non-affected side. The volume, maximum horizontal diameter, and trabecular bone density of the head of the mandible, as well as the ramus height were acquired from CT data of 16 patients suffering from this syndrome. The volume and maximal cross-sectional area of the masseter muscles and the pterygoid muscles were defined by quantifying CT data of nine patients. The skeletal parameters were evaluated with VoXim software. The comparison of the mandibular structures on the affected and on the non-affected side was based on the Wilcoxon signed rank test. The results recorded on the affected side showed that the TMJ volume was reduced by a mean factor of 7.98, and the spongiodensity of the head of the mandible by a mean factor of 1.33, while the mean reduction in ramus height was 14.89 mm. The muscle volumes on the affected side had also undergone a mean reduction of 9.10 cm(3). The CT-based data analysis proved to be an excellent tool for quantitative three-dimensional evaluation of both the skeletal and the muscular parameters.

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Section: Introductionunclassified

Abnormalities of the TMJ and the Musculature in the Oculo-auriculo-vertebral Spectrum (OAV)

Hirschfelder

Piechot

Schulte

et al. 2004

Journal of Orofacial Orthopedics/Fortschritte der Kieferorthop�

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“…Numerous case reports [Sugar, 1966;Berkman and Feingold, 1968;Darling et al, 1968;Sugar and Berman, 1968;Ide et al, 1970;Pashayan et al, 1970;Bowen et al, 1971;Cohen, 1971;Mandelcorn et al, 1971;Friedman and Saraclar, 1974;Greenwood et al, 1974;Michaud and Sheridan, 1974;Abe et al 1975;Aleksic et al, 1976;Miyamoto et al, 1976;Luce et al, 1977;Kallor and Hendricks, 1979;Russell et al, 1979;Starzak and Fujimoto, 1979;Thomas, 1980;Wilson and Schmickel, 1980;Setzer et al, 1981;Wells et al, 1983;Rollnick and Kaye, 19851, fewer clinical reports of large numbers of patients [Grabb, 1965;Converse et al, 1973Converse et al, , 1974Obwegeser, 1974;Ross, 1975;Shokeir, 1977;Feingold and Baum, 1978;Figueroa and Pruzansky, 1982;Phelps et al, 1983;Tenconi and Hall, 1983;Rollnick and Kaye, 1983;Figueroa and Friede, 1985;Loevy and Shore, 19851, and literature reviews [Gorlin et al, 1963;Baum and Feingold, 1973;Gorlin et al, 1976;Pierport et al, 1982;Wilson, 19831…”

Section: Introductionmentioning

confidence: 99%

Oculoauriculovertebral dysplasia and variants: Phenotypic characteristics of 294 patients

et al. 1987

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Here we describe the phenotypic characteristics of a single craniofacial clinic population of 294 individuals affected with oculoauriculovertebral dysplasia (OAV) and variants. To our knowledge, this is the largest population so described in the literature. The study population was divided into five subgroups based on the presence of combinations of minimal diagnostic criteria: microtia, mandibular hypoplasia, anomalies of the cervical spine and/or epibulbar or lipodermoids. The following data were recorded: sex (M:F 191:103); race (78% Caucasian); the presence of unilateral or bilateral microtia (193 unilateral, 98 bilateral); the presence of symmetric microtia in bilateral cases (34/98); the presence of mandibular hypoplasia ipsilateral or contralateral to the microtic ear or most severely microtic ear in bilateral cases (135/137 were ipsilateral in unilateral cases, 55/62 were ipsilateral in bilateral cases); the number of individuals with no other congenital anomaly in addition to the minimal diagnostic criteria (154/294), with only one other congenital anomaly (51/294), and with two or more other congenital anomalies (89/294); and the type of other congenital anomalies. Finally, we compared our results with other studies. Findings from our study include: mandibular asymmetry should be expected in patients with unilateral or bilateral microtia; bilateral involvement is frequent in patients with microtia; other malformations are seen frequently in all subgroups; anomalies of the cervical spine are more likely to be associated with other anomalies; and other malformations are seen in all systems and should be searched for to provide optimal management.

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“…Hemifacial microsomia (HFM) is currently viewed as a nondiscrete disorder within a broad spectrum of associated facio-auriculo-vertebral defects involving principally structures derived from the first and second branchial arches [Figueroa and Pruzansky, 1982;Jones, 19881. The term HFM is generally applied when the constellation of defects within this spectrum is predominately unilateral.…”

Section: Introductionmentioning

confidence: 99%

Enamel defects: A developmental marker for hemifacial microsomia

et al. 1990

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We have observed primary tooth enamel defects in 4 children with hemifacial microsomia. The distribution of enamel defects was concordant with the laterality of craniofacial anomalies in these patients and was most pronounced on the maxillary incisors. Since the location of enamel defects serves as a chronicle for the events of tooth formation, we propose that enamel defects may serve as a developmental marker for the events leading to hemifacial microsomia.

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The external ear, mandible and other components of hemifacial microsomia

Cited by 92 publications

References 15 publications

Abnormalities of the TMJ and the Musculature in the Oculo-auriculo-vertebral Spectrum (OAV)

Abnormalities of the TMJ and the Musculature in the Oculo-auriculo-vertebral Spectrum (OAV)

Oculoauriculovertebral dysplasia and variants: Phenotypic characteristics of 294 patients

Enamel defects: A developmental marker for hemifacial microsomia

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