BACKGROUNDAlthough neuroradiological findings of Möbius syndrome have been reported as a result of brain and brainstem abnormalities, magnetic resonance imaging (MRI) now permits the direct imaging of the cranial nerve (CN) and branches in the orbits. This study presents the MRI findings in patients with sporadic Möbius syndrome.

METHODSProspectively, CNs were imaged in the cistern using head coils and three dimensional fast imaging employing steady-state acquisition (3D-FIESTA), yielding a 0.5 mm(2) resolution in planes of 0.8 mm thickness in seven patients with sporadic Möbius syndrome. The cavernous and intraorbital segment of the CN and the extraocular muscles (EOMs) were imaged with T1 weighting in all patients. The cavernous segment was imaged in coronal planes, while the intraorbit in quasicoronal planes were imaged using surface coils. Intraorbital resolution was 0.16 mm(2) within 2.0 mm thick planes.

RESULTSIn the seven patients, the CN were absent or showed hypoplasia in the cistern, cavernous sinus, and orbit. Abducens (CN VI) and facial (CN VII) nerves were absent on the affected sides. Unilateral CN IX (glossopharyngeal nerve) in two cases displayed dysplasia. Branches from the inferior division of CN III were observed to innervate the lateral rectus (LR) bilaterally in three cases and unilaterally in one case, and had intimate continuity with the LR muscle in two cases bilaterally and two cases unilaterally. Hypoplasia of EOMs was shown in five cases. Dysplasia of the medulla on the left side was found in one patient.

CONCLUSIONSDirect imaging of CNs and EOMs by MRI is useful in diagnosis of Möbius syndrome. It can directly demonstrate the abnormalities of the CN and orbital structures. The absence or hypoplasia of CN VI and CN VII may be the most common radiologic features in sporadic Möbius syndrome, and hypoplasia of CN IX may be an associated feature. The abnormality of EOMs and aberrant innervations in the orbit should be observed, and may be important for the study of the etiology.

Cranial Nerves ; pathology ; Humans ; Imaging, Three-Dimensional ; Magnetic Resonance Imaging ; methods ; Mobius Syndrome ; pathology ; Oculomotor Nerve ; pathology

Child, Preschool ; Echocardiography ; methods ; Heart Septal Defects, Atrial ; complications ; physiopathology ; Humans ; Male ; Mobius Syndrome ; complications ; physiopathology ; Phenotype

Poland syndrome is characterized by unilateral absence or hypoplasia of the pectoralis muscle and variable degree of ipsilateral hand anomalies. Mobius syndrome is a congenital neurological disorder characterized by complete or partial facial paralysis. Although the pathogeneses of these diseases are not well-characterized, diminished blood flow to the affected side is thought to play a role. A male infant weighing 2.670 g was born at 38+3 weeks of gestation with left facial paralysis, left chest wall defect with dextrocardia, and symbrachydactyly between the second and third fingers. The combination of Poland-Mobius syndrome is rare, and only 2 cases associated with dextrocardia have been reported worldwide. Here, we report the first case of Poland-Mobius syndrome associated with dextrocardia in Korea.
Dextrocardia ; Facial Paralysis ; Fingers ; Hand ; Humans ; Infant ; Korea ; Male ; Mobius Syndrome ; Nervous System Diseases ; Pectoralis Muscles ; Poland Syndrome ; Pregnancy ; Thoracic Wall

Child ; Female ; Humans ; Mobius Syndrome

PURPOSE: Mobius syndrome is a rare congenital disorder characterized by facial diplegia and bilateral abducens palsy, which occasionally combines with other cranial nerve dysfunction. The inability to show happiness, sadness or anger by facial expression frequently results in social dysfunction. The classic concept of cross facial nerve grafting and free muscle transplantation, which is standard in unilateral developmental facial palsy, cannot be used in these patients without special consideration. Our experience in the treatment of three patients with this syndrome using transfer of muscles innervated by trigeminal nerve showed rewarding results. METHODS: We used bilateral temporalis muscle elevated from the bony temporal fossa. Muscles and their attached fascia were folded down over the anterior surface of the zygomatic arch. The divided strips from the attached fascia were passed subcutaneously and anchored to the medial canthus and the nasolabial crease for smiling and competence of mouth and eyelids. For the recent 13 years the authors applied this method in 3 Mobius syndrome cases- 45 year-old man and 13 year-old boy, 8 year-old girl. RESULTS: One month after the surgery the patients had good support and already showed voluntary movement at the corner of their mouth. They showed full closure of both eyelids. There was no scleral showing during eyelid closure. Also full closure of the mouth was achieved. After six months, the reconstructed movements of face were maintained. CONCLUSION: Temporalis muscle transfer for Mobius syndrome is an excellent method for bilateral reconstruction at one stage, is easy to perform, and has a wide range of reconstruction and reproducibility.
Adolescent ; Anger ; Child ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Cranial Nerves ; Eyelids ; Facial Expression ; Facial Nerve ; Facial Paralysis ; Fascia ; Female ; Happiness ; Humans ; Male ; Mental Competency ; Middle Aged ; Mobius Syndrome* ; Mouth ; Muscles ; Paralysis ; Reward ; Smiling ; Transplants ; Trigeminal Nerve ; Zygoma

No abstract available.
Duane Retraction Syndrome* ; Facial Paralysis ; Mobius Syndrome*

Brain ; pathology ; Central Nervous System Diseases ; congenital ; diagnosis ; pathology ; physiopathology ; Diagnosis, Differential ; Humans ; Infant, Newborn ; Magnetic Resonance Imaging ; Male ; Mobius Syndrome ; diagnosis ; pathology ; physiopathology

Mobius syndrome is a rare congenital disease characterized by unilateral or bilateral limitation of abduction and facial palsy due to the 6th and 7th cranial nerve paralysis. In addition, it may be associated with limb anomalies and other cranial nerve paralysis. We experienced three cases of Mobius syndrome characterized by esodeviation associated with bilateral limitation of abduction, bilateral facial atroph, tongue atrophy, congenital amputation of limb, and congenital clubfoot. We performed a large amount of bilateral medial rectus recession and unilateral lateral rectus resection, and obtained cosmetically successful results that orthophoric or less than 10 prism diopter of esotropia in primary postition, but mild limitation of adduction was observed as a complication.
Amputation ; Atrophy ; Clubfoot ; Cranial Nerves ; Esotropia ; Extremities ; Facial Paralysis ; Mobius Syndrome* ; Paralysis ; Tongue

Mobius syndrome is generally considered to be a static disorder of congenital origin, and is manifested as unilateral or bilateral facial weakness and lateral gaze limitation. In most instances the syndrome occurs sporadically, but rarely familial cases have been reported. We report a family of three members with Mobius syndrome; a 7-year-old girl, a 6-year-old boy, and their 29-year-old mother. Each patient revealed facial diplegia, and unilateral or bilateral lateral rectus palsy. Brain MRI scans showed normal and there were no definite brainstem dysfunctions on electrophysiologic studies.
Abducens Nerve Diseases ; Adult ; Brain ; Brain Stem ; Child ; Female ; Humans ; Magnetic Resonance Imaging ; Male ; Mobius Syndrome* ; Mothers

Mobius syndrome is generally considered to be a static disorder of congenital origin, and is manifested as unilateral or bilateral facial weakness and lateral gaze limitation. In most instances the syndrome occurs sporadically, but rarely familial cases have been reported. We report a family of three members with Mobius syndrome; a 7-year-old girl, a 6-year-old boy, and their 29-year-old mother. Each patient revealed facial diplegia, and unilateral or bilateral lateral rectus palsy. Brain MRI scans showed normal and there were no definite brainstem dysfunctions on electrophysiologic studies.
Abducens Nerve Diseases ; Adult ; Brain ; Brain Stem ; Child ; Female ; Humans ; Magnetic Resonance Imaging ; Male ; Mobius Syndrome* ; Mothers