Síndrome de Marcus-Gunn / Marcus Gunn syndrome

Introducción: El síndrome de Marcus-Gunn se manifiesta con retracción o elevación del párpado ptótico ante la estimulación del músculo pterigoideo del mismo lado y el término "guiño mandibular" es inapropiado ya que el párpado no siempre desciende. Puede producirse en la apertura bucal, masticación, avance mandibular, al sonreír, silbar, bruxar, sacar la lengua, deglutir, chupar, cantar, en la contracción esternocleidomastoidea, maniobra de Valsalva, respirar o inhalar. Es congénito y de modalidad rara y autosómica dominante. Objetivo: Contribuir al conocimiento de esta rara enfermedad y a su relación con el ámbito odontológico. Presentación del caso: Se trata de un niño de 3 años diagnosticado de síndrome de Marcus-Gunn en el periodo neonatal, por la observación de la madre de una apertura palpebral izquierda durante la succión nutritiva. No presenta otras enfermedades ni antecedentes de interés. En la exploración extraoral se advierte una ptosis palpebral derecha y apertura palpebral izquierda en los movimientos mandibulares y deglución. La intensidad del reflejo se incrementa en estados de ansiedad derivados del tratamiento dental. Conclusiones: el síndrome de Marcus-Gunn es una entidad rara en pediatría, en la cual sus hallazgos clínicos determinan el diagnóstico. Teniendo en cuenta que en ocasiones presentan alteraciones oculares, nada nos hace sospechar la presencia de una enfermedad oral específica(AU)

Introduction: Marcus Gunn syndrome manifests with retraction or elevation of the eyelid ptotico while stimulation of the pterygoid muscle on the same side and the term jaw-winking is inappropriate because the eyelid does not always goes down. It can occur during mouth opening, mastication, mandibular advancement, while smiling, whistling, bruxing, sticking out the tongue, swallowing, sucking, singing, during the sternocleidomastoid contraction, the Valsalva maneuver, breathing or inhaling. This syndrome is congenital and rare, and of autosomal dominant modality. Objective: To contribute to the knowledge of this rare disease and its relationship with the odontologic field. Presentation of the case: 3 years old boy diagnosed with Marcus Gunn syndrome in the neonatal period by the observation of the mother of a left palpebral opening during the nutritive sucking. He does not present other diseases or a background of interest. In the extraoral exploration, it is noticed a right palpebral ptosis and a left palpebral opening in the jaw movements and in swallowing. The intensity of the reflex increases in anxiety states arising from the dental treatment. Conclusions: Marcus Gunn syndrome is a rare entity in pediatrics, in which its clinical findings determine the diagnosis. Taking into account that sometimes it presents ocular alterations, nothing makes us suspect the presence of a specific oral disease(AU)

Jaw ‑ winking phenomenon: Report of a case with review of literature.

Jaw‑winking or the Marcus Gunn phenomenon (MGP), a congenital uncommon condition characterized by involuntary elevation of a drooping upper eyelid concomitant with various movements of the mandible, is well‑documented in medical literature, but very little information is available in dental literature. This is an interesting case report of a 17‑year‑old female with MGP, who reported for orthodontic treatment of her proclined upper anterior teeth, severe lower crowding, and mandibular micrognathia. Review of the literature revealed only two reported cases of dental anomalies in conjunction with MGP. The patient also gave a positive family history of MGP with a maternal cousin having a similar problem. Dentists and other oral health professionals can play a significant role in detection and diagnosis of this rare condition and should take protective measures during dental and oral surgical procedures.

Fenômeno de Marcus Gunn recidivado: relato de caso e revisão bibliográfica / Surgical treatment of relapsed Marcus Gunn syndrome: a case report and literature review

INTRODUÇÃO: O fenômeno de Marcus Gunn é uma ptose congênita rara e pouco conhecida, de origem neurogênica, de fisiopatogenia ainda não esclarecida. Os autores relatam um caso de ptose acentuada e recidivada após quatro correções cirúrgicas, e fazem uma breve revisão bibliográfica. RELATO DE CASO: Adulto jovem de 19 anos, sexo masculino, foi submetido a quatro tentativas de correção de ptose palpebral a direita em outros serviços, sem diagnóstico da origem da ptose congênita. O paciente apresentava abertura palpebral do olho direito ao abrir a boca, ao lateralizar o olhar para o lado ipse-lateral, e ao fechamento do olho contra-lateral. A última cirurgia foi realizada 2 anos antes, com posicionamento e fixação de fitas de silicone. O paciente não apresentava nenhum déficit neurológico ou visual. Foi submetido à cirurgia para remoção das fitas de silicone anteriormente posicionadas, confecção de enxertos em fita de técido fibroconectivo da fáscia lata do membro inferior direito, miectomia do musculo elevador da pálpebra ptosada, fixação dos enxertos da fáscia lata na borda superior do tarso com tração e fixação ao musculo frontal ipsilateral. Evoluiu com movimentos sincrônicos das pálpebras bilateralmente, e sem recidiva do ptose até o presente momento.

INTRODUCTION: Marcus Gunn syndrome is a rare and little known congenital ptosis of neurogenic origin. Its physiopathological mechanism has not been clarified. The authors report a case of pronounced ptosis that relapsed after four surgical corrections, along with a brief literature review. CASE REPORT: A 19-year-old young adult underwent four surgical corrections of the right palpebral ptosis in other services, without a definite diagnosis of the origin of the congenital ptosis. The patient showed eyelid opening in the right eye on opening the mouth, when the eye is lateralized to the ipsilateral side, and with contralateral eye closure. The last surgery was performed 2 years prior, during which silicone bands were placed and fixated. The patient did not present any visual or neurological deficit. He underwent surgery in order to remove the silicone bands that were previously placed, to prepare the strip grafts made of fibrous connective tissue from the fascia lata of the right inferior limb, and to perform myectomy of the levator muscle of the palpebral ptosis, fixation of the fascia lata grafts at the upper edge of the tarsus with traction and fixation to the ipsilateral frontal muscle. The patient progressed with synchronic movements of the eyelids bilaterally, and without relapse of the ptosis until the present date.

Marcus Gunn Jaw-Winking Phenomenon: Brief Communication.

Marcus Gunn jaw-winking phenomenon is the most common form of congenital synkinetic neurogenic ptosis. The unilaterally ptotic eyelid elevates with jaw movements due to cross innervations between oculomotor nerve and mandibular branch of trigeminal nerve. We report a case of a 12-year-old female who presented with complaint of drooping of left upper lid since birth associated with elevation of left upper lid while chewing. She was diagnosed to have Marcus Gunn jaw-winking syndrome.

A Case of Bilateral Marcus-Gunn Syndrome

PURPOSE: To report a case of bilateral Marcus Gunn syndrome, which can be easily overlooked. CASE SUMMARY: A 6-year-old boy visited our clinic complaining of right ptosis. On ocular examination, the palpebral fissure width was 4 mm in the right lid and 7 mm in the left lid. Marginal reflex distance 1 (MRD 1) was -1 mm in the right lid and 2 mm in the left lid. Bilateral levator muscle function was good. When the patient moved the jaw to the right side, the left eyelid was retracted by 2 mm, and when the jaw was moved to the left side, the right upper eyelid retracted by 4 mm, which showed a moderate bilateral jaw-winking phenomenon. There was no change in bilateral palpebral fissure width when only opening the mouth without jaw movement. CONCLUSIONS: Asymmetrical bilateral ptosis can be easily misdiagnosed for unilateral ptosis, and concurrent Marcus Gunn phenomenon can exist. Therefore, a thorough examination is necessary when examining a ptosis patient.

Optic Nerve Injury Associated with Facial Trauma

The traumatic optic neuropathy occurs in 0.5 to 5% of closed head trauma cases. Because it may cause permanent blindness, the early diagnosis and proper treatment are very important. We have treated 6 traumatic optic neuropathy patients among 433 head trauma cases from Sep, 1997 to Aug, 1998. Four patients showed visual recovery with steroid therapy. The diagnosis can be made clinically when the patient suffers from acutely decreased vision, Marcus-Gunn pupil, with its relatively normal fundoscopic finding, is considered as the most sensitive indicator and the first herald of visual tract injury, even though visual acuity is normal at the initial stage. The megadose steroid therapy must be diagnosed for the recovery of incompletely injured axons rather than regeneration. The optic canal decompression surgery must be spared for the delayed onset optic neuropathy patients who do not respond to the steroid therapy. In a case of indirect optic neuropathy, the associated orbital fracture reduction should not be undertaken until the optic neuropathy has stabilized. But severely displaced blow-in fracture of the orbit that decreases orbital volume should repaired early for orbital decompression.

A Case of Marcus Gunn Jaw Winking and Pseudo Inferior Oblique Overaction

The most common type of ocular miswiring is Marcus Gunn jaw winking phenomenon and Duane's retraction syndrome and the next most common is pseudo inferior oblique overaction. The association of Marcus Gunn jaw winking and pseudo inferior oblique overaction has not been previously reported. The authors describe a 21-year-old woman with both Marcus Gunn jaw winking phenomenon and pseudo inferior oblique overaction. This coexistence in the present case may provide some support to the existing concept that both phenomena are caused by an ocular aberrant innervation. This unusual combination suggests that multiple aberrant innervation patterns can occur in the same patient. The occurrence of ocular miswiring is more common than is indicated by the few reports in the literature.

The Clinical Study On the Surgical Methods of Synkinetic Ptosis Treatment

Synkinetic ptosis refers to abnormal vertical movement of upper eyelid. This abnormal elevation of the eyelid results from opening or closing of the mouth, chewing, sucking, and movement of jaw outward or toward the contralateral side. A number of other stimuli for the phenomenon have been described. These include smile, sternocleidomastoid contraction or tongue protrusion, inspiraion, and voluntary nystagmus. This type of ptosis was most commonly seen with a congenital Marcus-Gunn jaw-winking syndrome. Usually this movement is thought to be due to a congenital misdirection of some of the 5th cranial nerve fibers into the branch of the 3rd cranial nerve that supplied the levator muscle. We experienced 6 cases of synkinetic ptosis, among them, interesting enough, one was typical elevation of upper lid in smile due to misdirection between the oculomotor and facial nerve fibers. The cosmetic problem was the first purpose of the surgery. So, we performed a levator extirpation with frontalis suspension on 4 cases who had the severe jaw-winking phenomenon and undertook only fron talis suspension on 2 cases and one of whom had poor superior rectus function. In the cases who have undergone levator extirpation with frontalis suspension, synkinetic movement was improved in all. However, in those who underwent the frontalis suspension procedure, the synkinetic phenomenon still remained in spite of acceptable improvement of blepharoptosis in primary position.