Bilateral Congenital Facial Paralysis

Abstract

The term congenital facial paralysis is applied to a group of cases in which the paralysis is noticed at or shortly after birth. The definition is qualified in this way to include certain cases of undoubted congenital origin in which symptoms are not recognized at birth. The subject of congenital facial paralysis has had sufficient attention for one to assume a clearly defined picture for it, but, unfortunately on review of the literature one finds little clarity about the condition. This confusion has come about more from lack of understanding of the mechanism of development, together with the absence of a simple working outline, than from any other reason. In attempting to formulate such a classification, several factors must be taken into consideration. From a scientific standpoint, an anatomic classification might be more accurate, for instance, the division into supranuclear or nuclear lesions, lesions at the exit of the nerve from the pons, lesions in the aqueduct of Fallopius, and lesions peripheral to the exit of the nerve from the stylomastoid foramen, as is commonly given in treatises on facial paralysis in the adult. But this is not practical in small infants because of the difficulty in determining the status of hearing, taste and smell at this age. On the other hand, a classification based more or less on the cause makes the diagnosis more simple, localizes the lesion fairly accurately, and makes it possible to offer a definite prognosis with greater ease, a vitally important affair to the parents. From an etiologic consideration, there are two general groups into which these cases may be divided : Obstetric

The neuropathology of hereditary congenital facial palsy vs Möbius syndrome

“…The degree of clumsiness is greater than could be explained by foot and hand deformities, and is probably the result of hypoplasia of either the corticospinal or corticopontocerebellar tracts, or of hypoplasia of the cerebellum. 19 The great majority of cases of congenital facial palsy, especially unilateral, are, however, the consequence of antepartum or intrapartum injury to the facial nerve or nucleus, 27,28 and might be misnamed in some instances as Möbius syndrome. We found support for our long tract hypothesis in two cases with Möbius syndrome who have been described previously, 25 and in the present sporadic case in whom the architecture of the entire brainstem, including part of the long tracts, was thoroughly affected by dysgenesis, degeneration, and necrosis (see figure 1).…”

Facial nerve anomalies in association with congenital hearing loss

“…Congenital FN paralysis is defined as facial palsy of the seventh cranial nerve that is present at birth or shortly after [20]. Congenital FN paralysis is uncommon.…”

The abnormal nucleus as a cause of congenital facial palsy

“…This study was undertaken to review the frequency of congenital nuclear aplasia, considered a very rare cause of CFP1 until now. Using MRI scans we found that an underlying CNS abnormality was evident in four of 15 (27%) patients with CFP as their sole symptom.…”

“…Congenital facial palsy (CFP) is clinically defined as facial palsy of the 7th cranial nerve which is present at birth or shortly thereafter 1. The most recent estimate of the incidence of CFP derives from the United States where the incidence was found to be 2.1 per 1000 live births 2.…”