palsy面神经麻痹课件

David Kuykendall, MD University of Nevada School of Medicine 2013 Caused by Malformation Damage Agenesis Affects the 7th Cranial Nerve Muscles for Facial Expression Taste of anterior 2/3 of tongue Often noticed in first 24 hours of life during babies first cries Can present as poor feeding Evaluate baby for stroke: Strength of movement, Reflexes, and Sensation of extremities on the affected side. If normal: suspect congenital facial palsy Ear tags/pits, malformed external ears, and/or facial asymmetry may indicate Brachial Arch Syndrome Family History Pregnancy Risks: infections, substance use/abuse, trauma, PMHx, pets/environment Associated symptoms: doesnt seem to hear/see well, neurological dysfunctions, fever, poor feeding, etc. Exam: otologic, neurologic, ocular, dermatologic and oral exams Birth Trauma (Most Common) Brachial Arch Syndromes Moebius Syndrome Hemifacial Microsomia Goldenhars Syndrome Bells Palsy Tumor Injuries that occur during birthing process or facial pressure on sacrum from position of baby in the uterus Risk Factors: LGA, Gestational diabetes, post-term, vaginal deliveries, intrauterine trauma, assisted deliveries (vacuum and forceps) Usually resolve in first week of life Few (less than 5%) cases of permanent nerve damage with no facial paralysis improvement (Lundstrom, 2002) Development (abridged) During fourth week of life the embryo develops brachial (pharyngeal) arches The Second Brachial Arch gives rise to CN VII During the sixth week of life the external ear develops from the 1st and 2nd Brachial Arch Malformations are often accompanied by facial nerve dysfunctions as malformation of the ear can result in disrupted growth of CN VII (Backous, 1991) Causes: Genetic Mutation Vascular Disruption Trauma or Blood Loss during pregnancy Bilateral congenital facial paralysis Cant smile, expressionless face, limited eye movement, feeding difficulties (most need feeding tubes), and high risk of aspiration Normal Intelligence Strongly associated with clubfoot, hand abnormalities, and Poland Syndrome (absence of chest musculature) Results from underdevelopment of Cranial Nerves 6 (eye movement) and 7 (Facial expresion) Epidemiology: 1 in every 1-1.5 million child births Symptoms: Corneal erosion (lack of blinking), strabismus, hand/foot abnormalities (webbing, clubbing), chest well abnormalities, difficulties breathing and swallowing Also have speech difficulties and need speech pathologists Have difficulty paralysis of lips, soft palate, and the tongue base Treatment: Supportive Surgery to correct strabismus, regular eye drops, speech therapy, G-tube for poor feeding “Smile Surgery” muscle grafts from thigh to mouth to facilitate facial expression This surgery is complex, expensive and tedious (twelve hours for one side of face) It is NOT a cure for Moebius Syndrome More severe brachial arch malformation Usually unilateral facial paralysis but most prominent sign is Underdevelopment of affected side of face Full affects noticed at 4-6 years of age: full growth of facial bones and slimming of face Absence or microtia (underdevelopment) of external ear Malformation of middle ear (hearing deficits) Underdevelopment of bony structures of affected face: Mandible, Orbit, Zygomatic bone, etc. Thinning/Atrophy of cheek muscles and tissue Variable in presentation and severity Difficulties breathing due to laryngeal obstruction Often involves tracheotomy Caused by malformation of brachial arches during 4-6th week of Fetal Development Evidence poor: possible trauma/blood loss, hereditary, and blood supply issues involved Severity graded by OMENS (orbit, mandible, ear, nerves, soft tissue) scale Hearing screening important to assess hearing dysfunctions Treatment: Best to wait until adulthood. Many times growth will develop to near normal facies. Rib to mandible bone grafts to correct facial shape may be used in adulthood Hearing aid to correct hearing deficits Unilateral facial paralysis with associated facial malformations Incomplete development of ear, mandible, nose, lip and soft palate Malformation of both 1st and 2nd brachial arch Epidemiology: Unknown but very rare. Few case studies exist. First described by Maurice Goldenhar in 1952 Limbal Dermoids Preauricular Skin Tags Strabismus This is distinguished from hemifacial microsomia in that this condition involves internal organ dysfunction Typically affects heart, lung, kidneys Organ(s) are usually absent or underdeveloped on affected side Also affects vertebrae (scoliosis) Can be associated with hearing loss, deafness, and blindness Cause: unknown Treatment: Supportive Surgery for strabismus, rib to mandible grafts, dermoid debulking, repairing cleft lip/palate Cardiology: repair heart malformation or manage symptomatic heart disease Hearing Screening important to assess need for hearing aids Vision test to assess need for corrective lenses Spinal surgery to correct severe cases of scoliosis Dysfunction of cranial nerve VII Presents as Unilateral facial paralysis Lower Motor Neuron Disease Previously considered idiopathic, links have been established to herpes simplex infections (Musani et al, 2009.) Other common causes less likely in newborn: Lyme Disease, TB, polio Diagnosis of exclusion (usually necessitates pediatric neurology consult) Should have no associated cochlear or neurological symptoms Onset of Bells Palsy is acute Cause: inflammation of the facial nerve causes impingement at point it exits the skull Categorized by House-Brackmann Score Graded I-VI: measure upward movement of eyebrow, outward movement of the angle of the jaw Treatment: Corticosteroids shown to be effective in treatment Acyclovir was shown not to be effective Surgery not shown to show improved outcomes, “smile surgery” may be helpful in cases that do not respond to medical management Prognosis: Most patients (85%) see improvement in 3 weeks with full return of function, of the remaining 15%, 10% found return to full function in 3week-6month period. Of the remaining 5% recovery was considered poor or absent A benign or malignant growth can cause impingement of the CN VII which can lead to facial paralysis Proper workup of intracranial or impinging mass should be done including imaging MRI or CT Treatment: Surgical removal of impinging mass usually curative No specific workup If suspect TORCH infections: viral titers If appears syndromic: start with chromosomal analysis Remember acousitc and visual screening to detect deficits Proper consults: Neurology, Surgery, ENT, speech therapy, audiologist, etc. MRI or CT if suspecting tumor; these tests also useful to analyze bone structure for surgical correction Backous, D. D. (1991, December 7). From the Grand Rounds Archive at Baylor: craniofacial microsomia. Gray, H. (1977). Anatomy, descriptive and surgical (15th ed.). New York: Portland House. Bascom, D. (2002). Facial nerve embryology. eMedicine. Retrieved February 16, 2004, Hay, W., Hayward, A., Levin, M., Paduga, R; American Academy of, Neurology