Mobius syndrome is caused by abnormal development of the cranial nerves. This rare disorder has a number of causes. Most often affected are the cranial nerves VI and VII. Occasionally the cranial nerves V and VIII are affected.

If the cranial nerve VI is affected, the patient suffers from loss of lateral gaze. If cranial nerve VII is affected, the patient suffers from bilateral facial palsy — mask-like expressionless face with mouth constantly held open. If cranial VIII is affected the patient suffers from hearing loss.

Although its rarity often leads to late diagnosis, Infants with this disorder can be identified at birth: by a "mask-like" expression detectable during crying or laughing due to paralysis (palsy) of the sixth and seventh cranial nerves. Other characteristics include:

abnormalities in the limbs — their fingers may be webbed, shorter than usual or they may have more than 5 fingers on their hand
impaired sucking ability
inability to follow objects with the eye—instead the child turns his or her head to follow
crossed eyes
inability to smile
limitation of tongue movement

Later on, the child may develop speech difficulties, crossed eyes, abnormally small eyes, and fluid building up in the lungs, causing bronchopneumonia.

Treatment

There is no specific course of treatment for Mobius syndrome. Treatment is supportive and in accordance with symptoms. Infants may require feeding tubes or special bottles, such as the Haberman Feeder, to maintain sufficient nutrition. Surgery may correct crossed eyes and improve limb and jaw deformities. Physical and speech therapy often improves motor skills and coordination, and leads to better control of speaking and eating abilities. Plastic reconstructive surgery may be beneficial in some individuals. Nerve and muscle transfers to the corners of the mouth have been performed to provide limited ability to smile.

Pathological picture

The causes of Mobius syndrome are poorly understood. Many cases have no obvious cause. Others may be genetic.

Some cases are associated with reciprocal translocation between chromosomes or maternal illness. Some maternal trauma may result in impaired or interrupted blood flow (Ischemia) or lack of oxygen (Hypoxia) to a developing fetus. The use of drugs and a traumatic pregnancy may also be linked to the development of Mobius syndrome. The use of the drugs Misoprostol or Thalidomide by women during pregnancy has been linked to the development of Mobius syndrome in some cases.

Some researchers have suggested that the underlying problem of this disorder could be congenital hypoplasia or agenesis of the cranial nerve nuclei. Certain symptoms associated with Mobius syndrome may be caused by incomplete development of facial nerves, other cranial nerves, and other parts of the central nervous system.

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child with a limb deficiency, The
From Journal of Bone and Joint Surgery, 5/1/99 by Jackson, Andrew

The child with a limb deficiency. Edited by John A. Herring and John G. Birch. Pp 500. Rosemont, Illinois: American Academy of Orthopaedic Surgeons, 1998. ISBN: 0-89203-175-1. US$25.00

This book provides an essentially North American view of the management of limb deficiency in children and there is no doubt that it fills a substantial void in the orthopaedic literature. Since the authors all met at a symposium as part of the preparation of this volume, the editors have been able to insert short and succinct `consensus views' where controversy arises. Such an open-minded approach provides the reader with a sense of balance and a feeling for the direction in which the pendulum of opinion is swinging.

The highlight of the opening embryology section is a fascinating explanation of the hypothesis of the description sequence of the supply to the subclavian artery. Could the syndromes of Poland, Sprengle, Klippel-Feil and Mobius all share a similar aetiology of vascular occlusion, the precise syndrome produced depending on the site of vascular interruption? The fact that each of these syndromes may occur with some features of each other, seems to make the hypothesis likely.

Next and not surprisingly the problem of the classification of patterns of deformity is discussed. The term `congenital deficiency of the femur' is to be preferred to `proximal femoral focal deficiency'. While it is clear that the spectrum of congenital deformity can be split into ever more complex classifications, a strong plea is made for simpler and more useful classifications which are helpful in the treatment strategy. The Gillespie and Torode classification of congenital abnormalities of the femur is to be commended in this respect

The controversial van Nes rotationplasty, which has never been popular in the UK, is due for reconsideration particularly as the alternative to knee fusion and Syme's amputation when the ankle of the short leg lies at the level of the knee of the long leg. While the cosmetic appearance may seem grotesque to some, the functional result as judged by gait analysis and oxygen consumption shows advantages in favour of the Van Nes technique over the alternatives. Greater experience has allowed the procedure to be performed as early as the ages of two or three years and this leads to better acceptance by the patient. The problem of derotation of the leg with growth can be minimised by attention to surgical detail.

By contrast, Brown's fibulofemoral transfer for congenital absence of the complete tibia should probably be abandoned; it seldom works and the functional results of knee disarticulation are reasonably good. One author still felt that it has a place when there is an active quadriceps mechanism and the problem is bilateral.

There are competent sections on techniques of limb-lengthening, using either the Ilizarov or monolateral half-pin fixator methods. Particularly impressive in terms of lengthening are the results which may be achieved in severe fibular hemimelia, but it requires five or more sessions of treatment during the period of growth. Perhaps the impressive photographs do not state as much about function as one would wish to know. This does not sound like treatment for the faint-hearted.

In peacetime, traumatic limb amputations are much less common in our practice than the major congenital limb deficiencies. Nevertheless, it should be remembered that in other parts of the world this ratio is drastically reversed, particularly where modern warfare and landmines take their toll.

The upper limb is covered in slightly less detail but the problems here are different with the emphasis on preserving sensation and improving prehension. There are interesting contributions on limb salvage versus amputation for malignant tumours, stump capping for the prevention of spiky overgrowth that can compromise the tip of a stump and the lengthening of short residual limbs. Finally, there are up-to-date contributions on prosthetics and rehabilitation.

It is clear from this book that these patients do not just need specialist surgeons, they need specialist centres. I was delighted to see a contribution entitled `training the child with lower limb loss to run'. Having achieved that some then go on to ski, which emphasises what can be done when these patients really move into top gear.

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