cornelia de lange syndrome (cdls) is an uncommon multiple congenital anomaly with unknown cause and recurrent risk and may be the result of an inheritance metabolic error. in classical form of the syndrome there is a recognizable facial appearance at birth although in children with mild disease this may be less obvious at birth but become more noticeable over the first three years of life. in t...

holoprosencephaly (hpe) is a rare congenital brain malformation associated with multiple midline facial defects. this anomaly is resulted from the failure of diverticulation and cleavage of primitive prosencephalon during weeks 4-8 of gestation. hpe is the most common forebrain developmental anomaly in human with the incidence rate of 0.49-1.2 cases per 10,000-20,000 term births. in this study,...

talon cusp is a rare developmental anomaly that occurs most commonly on the lingual side of the incisors. occurrence of the labial talon cusp is rare in the dental literature. till date only seven cases of isolated nonsyndromic labial talon cusps have been reported in the maxillary permanent dentition. however, a few cases of syndromic labial talon cusps and faciolingual talon cusps have been r...

OBJECTIVE To review our experience with branchial cleft anomalies, with special attention to their subtypes and anatomical relationship to the facial nerve. STUDY DESIGN Case series. SETTING Tertiary care center. PATIENTS Ten patients who underwent resection for anomalies of the first branchial cleft, with at least 1 year of follow-up, were included in the study. The data from all cases w...

The Wildervanck (or cervico-oculo-acoustic) syndrome consists of the characteristic triad of Klippel-Feil anomaly, abducens nerve palsy, and congenital deafness. It is a rare "disorder of polygenic heredity with sex limitation predominant to the female" (female-male ratio is 10: 1) ( 1 ). Hearing loss is most commonly neurosensory due to developmental inner ear anomalies. Only a single case of ...

Anomalies of the course of the facial nerve have been reported in association with middle and inner ear malformations. Bifurcation of its intratemporal portion is a rare malformation in which focal splitting of one or more facial nerve segments occurs. We describe the CT appearance of this anomaly and discuss its possible embryology. Facial nerve bifurcation is important to recognize in patient...

Anomalous facial nerve (FN) course can be found in a significant number of cases with aural anomalies. The most common anomaly of the FN involves the tympanic portion overlying the oval window.1--3 Facial canal dehiscence of the tympanic portion may be responsible for the anomalous course of FN over the oval window. The incidence of facial canal dehiscence found during otologic surgery is relat...

we report five cases of duplicated external ear canal with especial attention to its histology and surgical care of facial nerve for their complete resection. these five patients were selected from 50 cases of first bronchial cleft anomalies including atretic external ear canals, preauricular fistula, cyst and sinuses. the characteristic histopathology and clinical features such as external ope...

We report five cases of duplicated external ear canal with especial attention to its histology and surgical care of facial nerve for their complete resection. These five patients were selected from 50 cases of first bronchial cleft anomalies including atretic external ear canals, preauricular fistula, cyst and sinuses. The characteristic histopathology and clinical features such as external ope...