Congenital sixth nerve palsy with associated anomalies.

Congenital abduction deficit is most likely due to Duane's retraction syndrome as congenital abducens nerve palsy is very rare. We report two cases of infantile abduction deficit due to sixth nerve palsy associated with other anomalies to highlight the importance of including neuroimaging in the evaluation of an infant presenting with a limitation of abduction.

Clinical correlation of imaging findings in congenital cranial dysinnervation disorders involving abducens nerve.

PURPOSE: High-resolution magnetic resonance imaging (MRI) of intracranial parts of sixth nerve and seventh nerve and the extraocular muscles (EOMs) in orbit to correlate the clinical characteristics in patients with two special forms of strabismus in congenital cranial dysinnervation disorders which are Duane's retraction syndrome (DRS) and Mobius syndrome. MATERIALS AND METHODS: Morphological analysis by 3T MRI of orbit (using surface coils) and brain (using 32 channel head coil) was performed on 6 patients with clinical DRS (1 bilateral), 2 cases with Mobius syndrome, and 1 case with congenital sixth nerve palsy. These were compared with findings in five controls. RESULTS: We observed absence/hypoplasia of sixth nerve in five out of seven eyes with DRS (71.42%), anomalous course in one eye, sixth and seventh nerve absence/hypoplasia in affected eyes with Mobius syndrome and bilateral absence/hypoplasia of the sixth nerve in congenital sixth nerve palsy. For EOMs we calculated maximum diameter, area, and circumference of muscles using Osirix software, and noticed significant hypoplasia of lateral rectus in comparison to controls (P < 0.001). CONCLUSIONS: MRI gives useful information regarding confirmation of clinical diagnosis and its neurological anomalies in complex cases and helps to plan tailor made surgical management.

Inferior oblique transposition for large hypertropia after vertical rectus transposition.

Consecutive vertical deviations occur in 13% to 30% of cases after full tendon vertical rectus transposition (VRT) surgery in adult patients with abducens nerve palsy. We report a 7-year-old boy with congenital abducens nerve palsy who developed a large hypertropia following VRT with posterior fixation sutures. During subsequent surgery, extensive scarring of the vertical rectus muscles was observed, and an inferior oblique nasal anterior transposition was performed. The patient was orthotropic in primary position through 18 months of follow-up. The causes and management options for vertical deviation following VRT are discussed.

Síndrome de Moebius: ¿misoprostol como teratógeno? / Möbius syndrome: misoprostol as a teratogenic?

El síndrome de Moebius (SM) es una alteración congénita poco frecuente, caracterizada por la parálisis del nervio facial y del motor ocular externo, asociada a otras malformaciones craneofaciales y musculoesqueléticas. Su etiología no está clara, aunque en su aparición se asocian algunos agentes teratógenos, como el misoprostol. El mecanismo etiopatogénico se explicaría por la disrupción vascular secundaria al efecto vasoconstrictor del fármaco, en el territorio troncoencefálico. A continuación se describe el caso de un recién nacido afectado de SM, cuya madre usó misoprostol con fines abortivos durante el primer trimestre de la gestación. En los últimos años se ha documentado un número cada vez mayor de casos de SM asociados a esta práctica (AU)

The Möbius syndrome (Moebius syndrome) is an infrequent congenital disorder characterized by facial and abducens nerve palsy as well as the external ocular motor palsy. It is associated with other craniofacial and orthopedic anomalies. Its etiology is still unclear, although in its appearance teratogenics agents such as misoprostol have been related. Misoprostol’s etiopathogenic mechanism would be explained due to a secondary vascular disruption due to the vasoconstrictor effect of the medication, in the level of the area of the brain stem. Here we report a newborn with the Möbius syndrome whose mother had used misoprostol as an abortive during the first trimester of pregnancy. There has been a large number of Möbius syndrome associated with the use of misoprostol due to abortion attempt during the last years (AU)

The natural history of Möbius syndrome in a 32-year-old man.

Möbius syndrome (OMIM#157900) is an extremely rare congenital entity involving bilateral or unilateral palsy of the facial nerve, usually with dysfunction of other cranial nerves (second, third, fifth, sixth, ninth, tenth and twelfth). It was estimated that Möbius syndrome occurs in 1 of 50 000 live births. The aetiology and the pathogenesis of the syndrome remain unknown. The majority of published cases were sporadic. We report on the natural history of a 32-year-old man with de novo Möbius syndrome. The diagnosis was established at the age of 9 months due to partial bilateral facial and abducent nerve palsy. Additionally, the patient demonstrated failure to thrive during infancy and childhood, many dysmorphic features, lower limb anomalies, and hypogonadism in adulthood, but his intelligence was in the normal range. The low quality of life of the patient with Möbius syndrome is emphasized.

Normal abduction in a patient with duplicated abducens nerve.

PURPOSE: To our knowledge, there has been no report of ophthalmologic findings related with a duplicated abducens nerve in the ophthalmic literature. This study reports such findings. METHODS: An ophthalmologic examination was performed in one patient with a duplicated abducens nerve, revealed with thin-sectioned magnetic resonance imaging (MRI) across the brainstem level. RESULTS: The MRI disclosed a duplicated left abducens nerve. The patient was orthotropic in five cardinal positions, and her ductions and versions were full. CONCLUSIONS: One patient with a duplicated abducens nerve showed orthotropia and normal ocular movement.

Normal Abduction in a Patient with Duplicated Abducens Nerve

PURPOSE: To our knowledge, there has been no report of ophthalmologic findings related with a duplicated abducens nerve in the ophthalmic literature. This study reports such findings. METHODS: An ophthalmologic examination was performed in one patient with a duplicated abducens nerve, revealed with thin-sectioned magnetic resonance imaging (MRI) across the brainstem level. RESULTS: The MRI disclosed a duplicated left abducens nerve. The patient was orthotropic in five cardinal positions, and her ductions and versions were full. CONCLUSIONS: One patient with a duplicated abducens nerve showed orthotropia and normal ocular movement.

Paresia de VI nervo congênita / Congenital VI nerve palsy

Os autores relatam caso de uma criança de 2 anos com hipótese diagnóstica de paresia de VI nervo esquerdo, congênita. O paciente foi submetido a cirurgia no olho esquerdo, com amplo retrocesso do reto medial e grande ressecçäo do reto lateral, sendo que as características peroperatórias e resultado cirúrgico reforçam a hipótese diagnóstica inicial.

Imaging evaluation of cranial nerves 3, 4, and 6.

Neuropathies of the oculomotor, trochlear, and abducens nerves may present with isolated or complex neurologic findings. An understanding of the anatomy of these cranial nerves as they traverse the brainstem, basilar cisterns, and cavernous sinus on their way to the orbit can assist in localizing the suggested site of pathology and help to focus imaging protocols. Differential diagnostic possibilities for specific anatomic locations are reviewed.