A congenital cranial dysinnervation disorder: Möbius’ syndrome

A congenital cranial dysinnervation disorder: Möbius' syndrome.

Möbius' syndrome, also known as Möbius' sequence, is a nonprogressive cranial dysinnervation disorder characterized by congenital facial and abducens nerve paralysis. Here, we report a 5-day-old girl who was conceived after in vitro fertilization with poor suck and facial paralysis. She had bilaterally ptosis and lateral gaze limitation, left-sided deviation of the tongue, dysmorphic face, hypo...

Congenital crocodile tears with Duane's syndrome--congenital cranial dysinnervation syndrome.

Chromosome 6 microdeletion in a patient with syndromic congenital cranial dysinnervation disorder

T congenital cranial dysinnervation disorders (CCDDs) encompass most congenital, static restrictions of ocular motility, often associated with ptosis, and retraction of the globe.1 The CCDDs can be monogenic or chromosomal in origin. Although chromosomal copy number variations (CNVs) has been reported in patients with syndromic CCDDs,2 but there are still patients for whom the genetic basis has...

Recent progress in understanding congenital cranial dysinnervation disorders.

BACKGROUND In 2002, the new term congenital cranial dysinnervation disorder (CCDD) was proposed as a substitute for the traditional concept of congenital fibrosis of the extraocular muscles (CFEOM) based on mounting genetic, neuropathologic, and imaging evidence, suggesting that many, if not all, of these disorders result from a primary neurologic maldevelopment rather than from a muscle abnorm...

Successful alignment following multiple surgeries in a child with severe esotropia and a congenital cranial dysinnervation disorder

Aim: To report the alignment, visual outcome and surgical strategy for severe bilateral congenital esotropia caused by a congenital cranial dysinnervation disorder (CCDD). This was successfully treated with a combination of interventions that included alternate occlusion, botulinum toxin, and conventional and augmented transposition procedures. Methods: A girl presented at 2 months of age with ...