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This site is in-development and may not reflect the final version. Preview the new GARD site. Other Names:. Muenke nonsyndromic coronal craniosynostosis; Syndrome of coronal craniosynostosis. Congenital and Genetic Diseases ; Musculoskeletal Diseases.
Summary Summary. Symptoms Symptoms. Many people with Muenke syndrome have a premature fusion of skull bones along the coronal suture, the growth line which goes over the head from ear to ear. Other parts of the skull may be affected as well. These changes can result in an abnormally shaped head, wide-set eyes, and flattened cheekbones. About 5 percent of affected individuals have an enlarged head macrocephaly.
Some people with Muenke syndrome have mild abnormalities of the hands or feet. Showing of 45 View All. Short and broad skull. Cone-shaped end part of bone. Narrow, high-arched roof of mouth. Narrow, highly arched roof of mouth. Wide-set eyes. Widely spaced eyes. Rise in pressure inside skull. Zygomatic flattening. Flat head syndrome. Flattening of skull.
Rhomboid shaped skull. Bulging eye. Eyeballs bulging out. Prominent eyes. Prominent globes. Protruding eyes. Drooping upper eyelid. Short feet. Small feet. Fused ankle bones. Too much cerebrospinal fluid in the brain. Hyperpigmented spots. Loss of hair color. Patchy loss of skin color.
Increased size of skull. Large head. Large head circumference. Lazy eye. Wandering eye. Attention deficit. Attention deficit disorder. Attention deficit-hyperactivity disorder. Attention deficits. Short fingers or toes. Broad big toe. Wide big toe.
Broad thumbs. Differential diagnoses include other types of syndromic craniosynostosis such as Crouzon and Saethre-Chotzen syndromes and Pfeiffer syndrome type 1. Prenatal diagnosis is possible in families with a known disease-causing mutation. Prenatal ultrasound examination may be used as an adjunct to prenatal genetic testing. Management of MS should be multidisciplinary. Patients should be tested for hearing loss and be subsequently monitored, even if initial evaluation is normal.
Surgical management is offered based on craniosynostosis severity. Typically, an initial craniosynostosis repair usually at months of age , that consists of fronto-orbital advancement and cranial vault remodeling, is performed. A secondary or tertiary transcranial repair, with or without extracranial contouring, may be needed. Endoscopic strip craniectomy is a newer and less invasive procedure, usually performed before the age of 3 months.
The prognosis is variable. Good outcomes are observed if patients receive early surgical reconstruction based on severity , as well as early and continued medical management of the neurologic, auditory, ocular, developmental and behavioral manifestations. Life-expectancy is normal. Expert reviewer s : Yonit. Other search option s Alphabetical list. Suggest an update. Summary and related texts. However, this will not benefit people whose hair cells are too damaged to pick up any sound and in these cases a cochlear implant may be suggested.
As the bone continue to grow during childhood and adolescence, further surgery may be needed to make minor corrections to the skull shape. The outlook for children with Muenke syndrome is good with the vast majority growing up to lead a normal life, working and raising a family. Most children are of normal intelligence so usually do well at school, college and university. Hearing support early in life will help with speech and language development.
Children with Muenke syndrome will require long term monitoring, particularly during periods of growth in childhood and adolescence, but further craniofacial surgery is unusual.
Some children and families benefit from psychological input at various stages throughout childhood and adolescence. Headlines — the Craniofacial Support Group — is the main support organisation in the UK for families of children and young people affected by a craniofacial disorder. Changing Faces is another organisation that will be able to offer help and support to anyone living with a condition that affects their appearance.
Visit their website or telephone their helpline on Muenke syndrome Muenke syndrome also known as FGFR3 associated craniosynostosis or Parg mutation is a type of complex craniosynostosis named after the doctor who first described it in the mids. Medical information Craniofacial information for parents and visitors.
Compiled by:. Last review date:. Disclaimer Please note this is a generic GOSH information sheet so should not be used for the diagnosis or treatment of any medical condition. If you have specific questions about how this relates to your child, please ask your doctor.
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