Extractions: First Steps for Families Team Member Roles Duke Team Members Examples of Pediatric and Craniofacial Conditions Treated: Apert Syndrome Crouzon Syndrome Pfeiffer Syndrome Treacher Collins Syndrome Nager Syndrome Saethre Chotzen Syndrome Robin Sequence (Pierre Robin) Fronto-nasal Dysplasia Amniotic Band Syndrome Moebius Syndrome Hemifacial Microsomia Goldenhar Syndrome Parry Romberg Hemifacial Atrophy First Steps for Families Team Member Roles Duke Team Members
Research Programs - Scott P. Bartlett, M.D. Weinweig, J., Baker, SB, Bartlett, SP and Whitaker, LA Delayed cranial vaultreconstruction for sagittal synostosis in older children An algorithm for http://www.uphs.upenn.edu/surgery/res/res-spb.html
Extractions: Scott P. Bartlett, M.D. - Plastic Surgery Research is continuing on a longitudinal study of growth and development of patients having craniofacial surgery. The study is in conjunction with a physical anthropologist in the Facial Reconstruction Center and the Center for Human Appearance. Research in conjunction with genetics is also continuing indefinitely on studies to match the fibroblast growth factor receptors status with clinical manifestations of synostosis syndrome. Clinical studies of an analysis and modification of nasal reconstruction principles. Development of a partially buried mandibular distraction device. The design and implementation stages are complete, and the device will be marketed in conjunction with the Synthes Maxillofacial Company, Inc. of Paoli, Pennsylvania. Research is continuing, also with the Synthes Maxillofacial Company, on an implantable mid-face distraction device. The long term effects of facial trauma in infants and children on craniofacial growth.
Posterior Plagiocephaly- Flathead sagittal synostosis after shunting for hydrocephalus. Treatment Surgical. Deformationalabnormalities secondary to positional influences on skull growth. http://pedclerk.bsd.uchicago.edu/craniosynostosis.html
Extractions: Back to table of contents Posterior Plagiocephaly- Flathead The incidence of posterior flattening of the skull has become more common with the Back to Life program to help prevent SIDS. Positioning the infant in the supine position has increased the number of infants presenting to the pediatrician to evaluate unusual skull shapes. Other causes of a flattened occipital area include growth restrictions in-utero and torticollis. It is imperative to differentiate true synostosis of a suture from positional deformities. Craniosynostosis- defined as premature closure of the cranial sutures. Primary synostosis is defined as closure of one or more suture secondary to abnormality of skull development. Usually recognized at birth If only one suture is affected, skull and brain growth will be normal and will result in cosmetic deformity If two or more sutures are closed, brain growth will be impaired and there will be neurologic deficits. The closure of a suture will cause a bony prominence ridge along the suture line. Compensatory growth is in the direction of or parallel to the closed suture. For example, premature closure of the sagital suture will result in an elongated head (scaphalocephaly).
Plastic & Reconstructive Surgery Department Board. Six of the children had sagittal synostosis, three had metopicsynostosis, and one had coronal craniosynostosis. Each of http://www.wfubmc.edu/plastic/sub_pages/dr_david.html
Extractions: Louis Argenta, MD* David LR, Genecov DG, Camastra AA, Wilson JA, Argenta LC. Positron emission tomography studies confirm the need for early surgical intervention in patients with single-suture craniosynostosis. Journal of Craniofacial Surgery. Vol 10;(1) 38-42, January 1999. From the Department of Plastic and Reconstructive Surgery and of Neurosurgery, Wake Forest University School of Medicine, Winston-Salem, NC
Lambdoid Synostosis And Occipital Plagiocephaly: Clinical Decision... Marsh JL Discussion of Posnick JC, Armstrong D, Bite U Metopic and sagittal synostosisintracranial volume measurements prior to and after cranioorbital http://www.neurosurgery.org/journals/online_j/feb97/2-2-5.html
Extractions: Benjamin S. Carson, M.D., Carol S. James, P.A.C., Craig A. VanderKolk, M.D., and Michael Guarnieri, Ph.D. Departments of Neurosurgery and Plastic Surgery, Johns Hopkins Medical Institutions, Baltimore, Maryland Lambdoid craniosynostosis has been regarded as one of the least common categories of premature fusion of the cranial sutures, yet reports have suggested the incidence may be increasing. To guide treatment decisions, the authors describe a set of rules based on radiographic indicators and clinical assessment in the child. Experience suggests that children can have abnormal-appearing cranial sutures with normal neurological status and normal-appearing sutures with neurological deficits or marked cerebral compression. Early evaluation and follow-up treatment is essential for children with suspected craniosynostosis. Key Words * craniosynostosis * lambdoid synostosis * occipital plagiocephaly * pediatric craniofacial surgery * clinical decision rules * pediatric neurosurgery * children A wide range of poorly characterized genetic, nutritional, toxicological, and mechanical influences may cause occipital skull deformities in the neonatal skull. These deformities are often associated with increased intracranial pressure (ICP) and visual impairment.[11,12,17,18,61,73] Data on the incidence of such impairments are uncertain and difficult to assemble because cranial deformities are not often lethal, not always recognized at birth, and not recorded in adults.[10] Studies have indicated a baseline of 0.2 to 0.5 cases of craniosynostosis per 1000 births.[10,15] A subset of the craniosynostoses, unilambdoid synostosis, accounted for approximately 1% of cases in three series,[14,43,62] zero cases in a review of 204 patients,[2] and four cases (3%) in a series of 130 patients with craniosynostosis.[30] Other reports suggest that lambdoid deformities may have been underdiagnosed in earlier series.[20,25,47,49]
Craniosynostosis Therefore, infants with sagittal synostosis present with a very narrowhead when viewed from the front and very long on side view. http://viper.med.unc.edu/surgery/neurosurgery/craniosynostosis.html
Extractions: (back to the top) The human skull is formed by bone plates, which are connected by fibrous regions called sutures (Rad 1a-1d). These sutures remain open and soft during infancy to allow the skull to expand as the brain grows underneath the bone plates. The open sutures will allow the child's brain to triple in size by age two. The sutures will fuse shut later in childhood. Craniosynostosis is defined as premature closure of the sutures while the baby is still in the womb. This problem occurs during fetal development and affects approximately 1 in 2,000 infants. When the suture(s) closes too early, the bones along the fused suture(s) will not grow and there will be compensatory overgrowth along the other sutures that remain open. Craniosynostosis can cause problems that are neurologic (brain related) and morphologic (skull shape related). The lack of bone growth at a fused suture can affect brain growth and lead to subsequent injury to the brain. Also, the abnormal growth patterns related to these fused sutures can cause malformations of the skull and surrounding structures of the orbits (eye sockets) and face. The treatment of craniosynostosis requires surgery in order to release the involved suture and reshape the malformed bones of the skull. The goals of surgery remove the involved suture so that brain growth can occur normally and improve the facial appearance. A team that includes a pediatric neurosurgeon, pediatric craniofacial surgeon, pediatric anesthesiologist, and a pediatric intensive care specialist is essential in order to achieve a successful surgical result while minimizing risk and morbidity.
AOL Hometown - Children Getting Well You can read about Chandler s diagnosis with sagittal synostosis, surgery recoveryas well as plans for future surgery. Chandler s Before After Photos, http://hometown.aol.com/ht.ssp?cid=17202&p=2&bt=h&idx=28
J Med Genet -- JANECKE Et Al. 38 (4): 265 The infant had dysmorphic features including an anteriorly enlarged fontanelle,premature sagittal synostosis, brachycephaly, a low anterior hairline, a broad http://jmg.bmjjournals.com/cgi/content/full/38/4/265
Extractions: Genetics J Med Genet 265-269 ( April ) E DITOR An adducted thumb-club foot syndrome was originally reported by Dundar et al in 1997 (MIM 601776). The patients presented with facial dysmorphism comprising a broad and bossed forehead, widely patent anterior fontanelle, telecanthus, downward slanting palpebral fissures, deep set ears, arachnodactyly, severely adducted thumbs, and club feet. Dundar et al discussed several known conditions featuring adducted thumbs, such as the Freeman-Sheldon syndrome (MIM 193700 and 277720), multiple pterygium syndrome (MIM 265000), congenital contractural arachnodactyly (MIM 121050), the Christian adducted thumb syndrome (201550), and the MASA syndrome/X
Sagittal Suture, Craniosynostosis, Page 5, A New Technique sagittal suture synostosis. The youngest baby at time of surgery was 12 days. Seethe results of endoscopic strip craniectomy for sagittal suture synostosis. http://www.muhealth.org/~neuromedicine/craniosynostosis05.shtml
Synostosis The Cranial Synostoses. synostosis is the premature closure of one or more of the calvarial sutures. Such fusion limits the abilty of the skull to expand in a direction perpendicular to the suture http://www.family.georgetown.edu/welchjj/netscut/neurology/synostosis.html
Extractions: The calvarium is divided by sutures into bony plates. In this illustration, the brown metopic suture divides the orange frontal bones. The frontal bones are separated from the blue parietal bones by the yellow coronal suture, and the two parietal bones are separated from each other by the purple sagittal suture. The occipital bone is separated from the parital bones by the white lambdoidal sutures. The space where the metopic, coronal and sagittal sutures come together is the anterior fontanelle , and the posterior fontanelle is present where the lambdoidal and sagittal sutures meet. most common, 58% of all synostoses 2-3 x more males than females dolichocephalic scaphycephaly (long-headed/keel-headed) May be turricephalic (tower-headed) frontal and/or occipital bossing may be present frequently, early closure of anterior fontanelle about 10% associated with increased ICP
Craniosynostosis, Cincinnati Children's Hospital Medical Center Strip sagittal Craniectomy. This technique is used for children with sagittalsynostosis but is being replaced by endoscopic guided procedures. http://www.cincinnatichildrens.org/health/info/neurology/diagnose/craniosynostos
Extractions: Home Contact Us Site Map Go to Advanced Search ... Chiari Malformation Craniosynostosis Epilepsy and Seizures Fever-related Seizures Headaches Hydrocephalus ... Treatment Related Services Neurosurgery Plastic Surgery Craniosynostosis, or simply synostosis, is the early growing together or fusion of two or more bones of the skull. A newborn's skull is made up of many separate bones that are not yet fused together. Because the brain grows quickly in the first two years of life, it is important that the skull bones not be fused yet. In fact, complete fusion of the bones normally occurs late in the teen years. Synostosis interferes with normal growth of the brain and skull. Doctors haven't found all the causes for synostosis. In some case the cause appears to be genetic. Craniosynostosis causes a change in the normal shape of the head. If a suture the seam between two skull bones is fused, it cannot grow, and the bones with open sutures then grow more than usual to allow enough room for brain growth. In many children the only symptom may be an irregularly shaped head. Some, however, will experience unusually high pressure on the brain, initially causing headaches. If left untreated, some children may then develop learning disabilities or problems with their eyesight. Early in life the brain is spared damage from this pressure by the other bones growing to accommodate the growing brain.
Craniosynostosis 2. Risk to siblings with two unaffected parents = sagittal 1%. VI. sagittalsynostosis. A. Definition when the sagittal suture fuses prematurely. http://www.genesoc.com/counseling/Outlines/craniosynostosis.htm
Extractions: Resources for Genetic Counselors site updated May 10, 2004 outlines links search Overview: Craniosynostosis refers to the premature fusion of the cranial sutures (growth centers between the bones of the skull) The skull is made up of a number of bones and when those bones fuse together too soon it doesnt allow the head to grow in the usual fashion and can lead to changes in head shape Usually cranial sutures do not fuse completely until the fourth decade Primary vs. secondary craniosynostosis: Primary suture growth is altered head frequently assymmetric the brain continues to grow in areas where sutures are open most individuals are normal neurologically benefit from surgery Secondary growth of brain is impaired neurologically abnormal usually Isolated vs. Syndromic: Isolated usually presents during first year of life usually involves only one cranial suture (may involve more than one though) no other associated abnormalities except those produced secondarily as a result of premature fusion of suture sagittal suture is most common site for isolated craniosynostosis suture that runs from front to back and separates parietal bones results in head elongated from front to back due to limited growth laterally (scaphocephaly) intracranial pressure is usually in the normal range cranial surgery usually recommended to give a more usual head shape usually babies with isolated craniosynostosis grow and develop normally Syndromic Other characteristic features present
MCG Craniofacial Center - Craniofacial Anomalies Scaphocephaly usually results from premature closure of the sagittal suture (sagittalsynostosis.) It may require surgical correction depending upon its http://www.mcg.edu/centers/cranio/anomal.htm
Extractions: Craniofacial Anomalies Before a child is born, the bones of the skull are not fused; this allows the bony plates of the skull to be more flexible, so the head can be molded during birth. Otherwise, it would be very difficult for a child's head to pass through the birth canal. Craniosynostosis is a condition that occurs when the bone junctions, or sutures, fuse prematurely. When some of the skull bones fuse in the womb, it can affect the baby's head shape. Sometimes the growth of the brain can also be influenced, and will benefit from surgical release of the constricted bones. Some examples follow. Click here to read about one family's experience. Plagiocephaly (plagio=oblique + cephaly=head) This condition presents with a lop-sided or twisted appearance to the head, with one side of the forehead or posterior head pushing out farther than the other. It can be associated with premature closure of one or both of the coronal or lambdoid sutures, or may come as a result of pressure of the mother's pelvic bones on the skull ( positional plagiocephaly Scaphocephaly (scapho=boat, skiff + cephaly=head)
Extractions: Home About Mayo Clinic Contact Us Mayo Clinic Locations: Arizona Florida Minnesota Mayo Clinic ... Medical Specialties Pediatrics Pediatrics Overview Patient Stories Diseases, Treatments and Services Appointments ... Other Specialties - Rochester The treatment of children with craniofacial disorders requires a comprehensive team approach. The combined expertise of the multidisciplinary Mayo Clinic craniofacial disorders team optimizes the holistic care of the child. The team includes specialists from Pediatric Neurosurgery, Plastic and Reconstructive Surgery, Child and Adolescent Neurology and Pediatric Medical Genetics. This team has the most current diagnostic and treatment options available. Premature closure of growth plates in the skull cause abnormal growth patterns of the face and skull. The altered growth results in an abnormal head shape. The actual shape of an affected infant's head depends on which growth plates closed prematurely. An infant's skull is made up of a collection of separate bones. The spaces between these bones are called sutures. The brain grows rapidly in an infant. In order to make room for the growing brain, the skull also grows rapidly. Growth of the skull takes place at the sutures. Premature fusion or closure of these sutures is called craniosynostosis . In a child with craniosynostosis, the brain continues to grow. However, the skull will not grow where the suture is closed. Compensatory growth occurs at adjacent sutures, resulting in an abnormal head shape.
Pediatric Advisor English Index: N To S Pharyngoplasty Orthognathic (jaw) Surgery Positional Head Deformity Rhinoplasty SagittalSynostosis (form of craniofacial anomalies) Videofluoroscopy (a moving http://www.med.umich.edu/1libr/pa/pa_index_3.htm
Extractions: This information is approved and/or reviewed by U-M Health System providers but it is not a tool for self-diagnosis or a substitute for medical treatment. You should speak to your physician or make an appointment to be seen if you have questions or concerns about this information or your medical condition. McKesson Clinical Reference Systems: Pediatric Advisor 2002.1 Child and Adolescent Health Topics A-Z Your Child
Classification Of Previously Unclassified Cases Of Craniosynostosis Ten of the 17 patients had the coup de serpe deformity in association with sagittalsynostosis, six with a large frontal bone and four with a normalsize http://www.neurosurgery.org/journals/online_j/dec96/1-6-p1.html
Extractions: Paul D. Chumas, F.R.C.S.(SN), Giuseppe Cinalli, M.D., Eric Arnaud, M.D., Daniel Marchac, M.D., and Dominique Renier, M.D. Cases of craniosynostosis usually fall into well-demarcated categories: those related to a syndrome or identified by a combination of suture involvement and morphological appearance. Between 1976 and 1995, 53 (3.6%) of 1474 cases in the craniofacial databank were assessed and designated as nonsyndromic but unclassifiable. The records and radiological studies obtained in these patients were retrospectively analyzed and comparisons were made with patients classified in the databank as having simple craniosynostoses. "Two-suture synostosis" is a relatively straightforward condition and is treatable with standard craniosynostosis techniques. However, possibly as a result of surgical compromise when two sutures are involved, the rate of reoperation is far higher than in simple suture cases. In contrast, patients in the "complex" group presenting with severe multisuture involvement require a more tailor-made approach to their management that often entails a second procedure. Key Words * craniosynostosis * complex disease * cranial suture The classification of craniosynostoses depends on a combination of criteria: 1) whether the sutural disease is part of a syndrome; 2) the morphological appearance of the patient; 3) the actual sutures involved; and 4) progression of the disease over time.[1] Using these criteria, it is possible to classify the vast majority of craniosynostoses. In a minority of cases the initial classification proves to be incorrect and other features indicating a more complex nature become apparent. It is thus necessary to have a sufficient follow-up period to be confident that the initial diagnosis still holds.
