|Year : 1963 | Volume
| Issue : 3 | Page : 76-78
MS Nirankari, Daljit Singh, Om Parkash
Department of Ophthalmology, Medical College, Amritsar, India
|Date of Web Publication||28-Jan-2008|
M S Nirankari
Department of Ophthalmology, Medical College, Amritsar
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Nirankari M S, Singh D, Parkash O. Mobius syndrome. Indian J Ophthalmol 1963;11:76-8
This relatively uncommon congenital anomaly has been given various names : Mobius syndrome, congenital facial diplegia, congenital oculofacial paralysis, nuclear agenesis and congenital nuclear aplasia.
Mobius (1892) collected and classified 43 cases of congenital and acquired cranial nerve palsies. One group consisted of 6 cases of bilateral abducens and facial nerve palsies. Since then the condition has been named after Mobius although it had been described earlier by von Grafe (1880), Harlan (1881) and Chisolm (1882).
Mobius syndrome comprises of bilateral facial and abducens palsies, hypoglossal involvement, underdeveloped jaw, club foot, chest malformations, arm malformation and a number of other minor congenital anomalies.
J. L. Henderson (1939) in a review of 60 cases showed 75%, abducens paralysis, 33% oculomotor nucleus involvement, 33% hypoglossal affection, 30% club foot, 20% arm malformations, 13% chest muscle defects and 10% mental deficiencies.
| Case Report|| |
F. R., 60 years old male was admitted to Ram Lal Eye Hospital for the treatment of mature senile cataract of right eye. Examination revealed following interesting features. [Figure - 1],[Figure - 2],[Figure - 3] and [Figure - 4]
1. Bilateral facial palsy, which was incomplete in the lower half of the left side of the face. The patient suffered from epiphora on both sides. The lower half of the left cornea showed a macular type of opacity, the result of exposure. The right cornea was clear.
2. Both eyes were in position of extreme convergence.
3. There was absence of abduction on both sides.
4. Only slight convergence was present.
5. Vertical movements of the eyes were unaffected. Bell's phenomenon
6. The left side of the tongue was shrunken and on protrusion the tongue deviated to the right. The auditory nerve and the motor root of the trigeminal nerve were unaffected.
7. Interesting developmental anomalies were present in the left arm, hand and chest.
The left tipper limb was shorter than the right by about 3 inches. The shortening was not uniform but was the result mainly of shortening of the arm and to a lesser extent, of the fingers. The left hand was less developed and had a poor grip, a result of the webbing of the fingers. The webbing involved the proximal two phalanges of the index, middle and ring lingers. The web extended to the middle of the thumb causing severe limitation of abduction and flexion. The grasp of the left hand for small objects was poor, for the same reason. On the skin of the back of the first metacarpophalangeal joint there was a pedunculated, firm dark brown nodule., having a size about 6 mm (supernumerary finger).
The musculature of the forearm of the left side was slightly less developed. The pectoralis minor and the sternal head of pectoralis major were absent. The clavicular head of pectoralis major and other muscles of the arm were well developed and had good power. All the movements at the shoulder joint were normal.
The nipple of the left side was absent.
8. The hearing power of the patient was normal.
9. The patient was reasonably intelligent.
Intracapsular extraction was done on the right eye by the modified Smith Indian technique. The recovery was uneventful. The corrected vision in the aphakic eye was 6/18.
Four months later the patient was persuaded for exploratory surgery in the unoperated left eye.
Forced duction test (Bedrossian and Lachman) was performed on both sides after retrobulbar anaesthesia. It was found that neither eye could he abducted beyond the middle by forceps, but the eyes could be elevated or depressed without difficulty.
Exploration of medial and lateral recti was done on the left side. The insertion of the medial rectus was found to be 6.5 mm from the limbus, whereas the lateral rectus was attached 13 mm away. The lateral rectus appeared very poorly developed. It had many check ligaments which were cut. But this did not improve the appearances or movements of the eye.
| Discussion|| |
The aetiology and primary site of lesion in this interesting condition are objects of controversy. Although the condition has been recognised for nearly eight decades yet these problems have escaped solution.
Kunn (1895) suggested that the lesions of Mobius syndrome were due to failure of development of nuclei i.e., nuclear aplasia. This theory has been substantiated by the pathological studies of Huebner (1900), Rainy and Fowl (1903), Spatz and Ullrich (1931) and Balint (1936). All these workers found hypoplasia of the affected cranial nerve nuclei and nerve trunks. Huebner in addition reported hypoplastic posterior longitudinal bundle.
Leszynsky (1897) and Henderson (1939) argued that it is not possible to decide that nuclear hypoplasia is the primary lesion, because failure of development or hypoplasia of the muscles can lead to secondary degenerative changes in the cranial nuclei and nerves.
In our case one may concede to the theory of nuclear hypoplasia in the case of facial, hypoglossal and abducens palsies, because it is easy enough for a noxious agent to act on a relatively restricted area of the nuclei in the floor of fourth ventricle and cause multiple lesions. But surely presence of posteriorly attached lateral rectus, absence of nipples and breast, absence of pectoralis minor, absence of sternal head of pectoralis major, shortening of the arm, syndactyly and supernumerary finger can not be explained by agenesis of any known nuclei. We agree with the suggestion of Reed and Grant (1957) based on the work of Duraswami (1955), that the diverse manifestations of Mobius syndrome are the result of some noxious agent acting on the embryo, perhaps at the time when the affected tissues are undergoing most rapid development.
| Summary|| |
A case of Mobius syndrome showing bilateral facial and abducens paralysis and a host of skeletal abnormalities has been described. The aetiology of the condition is briefly discussed.
| References|| |
Sobb R. L. (1961), A.M.A. Arch. of ophth., 65 : 40/16.
Yasuma (1955), A.M.A. Arch. of ophth., 54: 137.
Spaeth (1953), A.M.A. Arch. of ophth., 49: 49.
Bedrossian E. H. and Lachman, B. E. (1956), Amer. J. Ophthal., 41: 304.
Reed, H. and Grant, W. (1957), Brit. J. Ophthal., 41: 731.
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]