Fuchs' theory Ernst Fuchs, who first described this disease in 1906, assumed that the syndrome was caused by a noxious factor of unknown origin, which was active from fetal or early postnatal life.' First, the normal development of uveal pigmentation would be inhibited, resulting in heterochromia (Fig 1). Later, the eye would respond to this pathological agent by a low grade inflammation. Many objections were raised against Fuchs' theory. The heterochromia was not always congenital and there were no signs of overt ocular inflammation.

https://bjo.bmj.com/content/bjophthalmol/78/4/307.full.pdf

Fuchs' heterochromic cyclitis: review of the literature on the pathogenetic mechanisms.

There exists a spectrum of syndromes characterized by cranial nerve palsies, limb anomalies, and craniofacial malformations. Criteria based on type and severity of limb anomaly or presence or absence of cranial nerve palsy may alter the syndrome nomenclature due to a selection bias but do not appear to benefit syndrome delineation to any extent. Patients with seventh nerve palsy and abduction weakness are usually diagnosed as having Mobius syndrome by ophthalmologists. The observed ocular motility findings range from primarily abduction deficiencies to patients who have a Duane or gaze palsy pattern of horizontal movements. Some cases previously described as "sixth nerve and partial third" may better fall into one of the latter groups, since an isolated adduction deficit as a manifestation of third nerve involvement is rare. The presence of clinical appearance of Duane syndrome in these Mobius-type patients raises the possibility of abnormal innervation of the lateral rectus as an explanation of some patterns of motility observed.

Möbius and Möbius-like syndromes (TTV-OFM, OMLH).

Idiopathic and Other Anterior Uveitis Syndromes

The explosion of knowledge in the area of genetics, and a greater desire by patients and their families to know the cause and risk of recurrence of genetic malformations, have resulted in greater attention to errors of morphogenesis. For physicians who do not confront these problems regularly, the vocabulary often is confusing and unclear. Increasing effort has been made to clarify definitions for better communication of ideas. Improved classification of malformations and clusters of associated anomalies will assist in better diagnosis and treatment and more accurate risk counseling. Spranger et al.219 Cohen,38–40 Herrmann and Opitz,100 and others have attempted to define more precisely commonly used terms.

Craniofacial Syndromes and Malformations

Association of keratoconus and Möbius' syndrome.

In a 16-year-old boy with congenital bilateral paralysis of the sixth cranial nerve and unilateral paralysis of the seventh nerve (on the right) heterochromia of the right eye was observed, characterized by a lighter color of the iris, precipitates on Descemet's membrane and absence of synechiae. The development of Fuch's heterochromic cyclitis in combination with Moebius' syndrome is discussed in connection with possible pathogenic factors, in particular immunopathologic processes which may contribute to an understanding of the origin and progression of the disease.

Heterochromiezyklitis Fuchs bei Moebius-Syndrom

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Heterochromiezyklitis Fuchs bei Moebius-Syndrom