Brian Watson's Simple Home Page The letter stated that the xray showed all the indications of bilateral coronalsynostosis, with probable sagittal synostosis, and was inconclusive on the http://www.ghg.net/watson/cranio2.htm
Extractions: When Molly was born on February 19th, we could tell immediately that something was not right with her head. The back of her skull had noticeable ridges along the lambdoid sutures (oddly enough these ridges were not where the fusion was - the sutures were open under the ridges but closed elsewhere). Our pediatrician, Dr. Gordon Bellah, told us that the skull plates may have just overlapped during birth, which would be OK once they got back into place. After two days he told us that if they were not overlapping, that she could possibly have to have surgery (he never said the word craniosynostosis, but that's what he was referring to). He said we would re-evaluate it at the two week check-up. THE TWO WEEK CHECK-UP At two weeks, the problem was still there, so we were sent to the X-Ray lab to have X-rays done. This is the first step in confirming craniosynostosis. Several hours later, while we were at home, Dr. Bellah's office called us and told us that the X-Rays indicated that she probably did have craniosynostosis, but that we would have to have a CT scat to confirm how bad it was. The next day, they made an appointment for us at Texas Children's Hospital for the following Tuesday, to see Dr. Robert Dauser. They gave us a letter from the doctor that read the X-Rays, which they said was "the reason we got an appointment so soon at Texas Children's Hospital". That didn't sound good at all. THE LETTER I had been doing a lot of internet research to learn more about craniosynostosis. I felt certain that, although Lambdoid synostosis is extremely rare, that it was indeed what Molly had, given the look and feel compared to internet examples. I had learned that bilateral coronal synostosis was the second most common type of craniosynostosis, but Molly didn't look like the examples for the coronal case. Bilateral coronal synostosis is MUCH harder to fix than sagittal and lambdoid synostosis.
Extractions: Table of Contents I. Basic Concepts and Future directions for the Treatment of Craniomaxillofacial Deformities 1. Looking Back, Looking Ahead: How We Arrived at Our Current Thinking About Craniofacial and Maxillofacial Surgery 2. Craniofacial Growth and Development: Normal and Deviant Patterns 3. Quantitative Computed Tomographic Scan Analysis: Normal Values and Growth Patterns 4. Anthropometric Surface Measurements in the Analysis of Craniomaxillofacial Deformities: Normal Values and Growth Trends 5. Dysmorphology, Syndromology, Genetics, and Ethical Considerations 6. Fetal Surgery (Repair) and wound Healing Enhancement: Now and in the Future 7. Engineering Tissues for the Face and Facial Skeleton 8. Craniofacial Reconstruction Through Osteotomies and Gradual Distraction of the Skeletal Parts: Biologic Basis, Current Problems, and Future Directions
Wayne Ozaki, MD, DDS--Publications Closures Surgical Forum, Volume 48 2703, 1997 Buchman, SR and Ozaki, W. ThreeDimensional Micro-Analysis of Human sagittal synostosis Craniofacial Surgery http://www.ohsu.edu/som-plasticsurg/faculty/wo_pub.shtml
Cranial Synostosis sagittal synostosis causes elongation and narrowing of the skull that can attaingrotesque proportions in childhood and are only partly mitigated by normal http://www.ohsu.edu/som-pedneurosurg/synostosis.html
Extractions: Premature ossification of a suture, or synostosis, is sometimes attributed to intrauterine restraint of head growth by twinning or by early engagement of the head in the pelvis. It is commonly seen in craniofacial disorders such as Crouzon, Apert, Pfeiffer, or Saethrre-Chotzen syndromes, or in the setting of fetal exposure to phenytoin, valproic acid, or other teratogens. Most often, however, there is no identifiable cause. Sporadic cases typically involve a single suture; syndromic cases typically demonstrate more complex, multi-sutural involvement, often bilateral coronal synostosis. The expansion of the head is driven from within by growth of the brain, and craniosynostosis causes deformity by restricting expansion of the head in the dimension perpendicular to the affected suture. Compensatory pressure by the growing brain results in expansion, or bossing, in adjacent areas of the skull. The characteristic patterns of deformity caused by synostosis of each individual suture are readily recognized by the trained eye. Thus, physical examination is the gold standard for evaluation of this problem. Misinterpretation of skull radiographs and computed tomographic (CT) scans is a common source of confusion and alarm for parents. CT scanning of the skull and brain has a role principally for surgical planning and to rule out any associated cerebral abnormality or hydrocephalus, which are extremely uncommon in sporadic synostosis.
Extractions: The next most common form of synostosis involves one or both coronal sutures. In the case of one-sided involvement, the forehead just above the eye becomes flattened and is taller than normal (see Figure 3). The opposite side of the forehead may stick out a bit more than normally. The top of the nose is shifted towards the side that is flattened and the tip is pointed towards the opposite side. If both coronal are fused, the head is very short from front to back and tall, particularly in the front. Both sides of the forehead are quite flattened (Figure 4). True lambdoid synostosis is actually quite rare and produces a head shape that is quite different from the head shape that is typical of occipital plagiocephaly (OP). Whereas the head in OP is shaped like a parallelogram the head shape and true lambdoid synostosis is akin to a trapezoid. The ear on the affected side is pushed further back and the forehead on the affected side is pulled back rather than pushed forward as in OP. Craniosynostosis can be diagnosed by skull x-rays which usually reveal that the affected suture is not visible on x-ray (normal sutures appear as dark gaps between the whitish bone on each side) (see Figure 6). There is also often heaping of the bone at the site of the fused suture. A computed tomography (CT) scan, as well as 3-dimentional CT can supplement skull x-rays. In addition to the primary changes seen at the suture there can also be secondary changes seen in the alteration of how the skull has formed, particularly in its shape.
Department Of Neurosurgery Of Mount Sinai School Of Medicine sagittal synostosis results from premature closure of the midline suture, calledthe sagittal suture. This suture runs from the front to the back of the skull. http://www.mssm.edu/neurosurgery/germano/craniosynostosis.shtml
Extractions: The deformity that results depends on which suture(s) is involved. The cause of this condition is not known. Some of the craniosynostosis conditions can be passed between generations of families. Consequently, it is important that all affected children have a genetics evaluation. What are the different types of craniosynostosis Sagittal synostosis results from premature closure of the midline suture, called the sagittal suture . This suture runs from the front to the back of the skull. The skull becomes elongated and narrow. The condition of this abnormal head shape is called scaphocephaly Metopic synostosis results from closure of the forehead suture
UAMS Plastic Surgery - Publications Synostectomy Versus Complex Cranioplasty for the Treatment of sagittal synostosis.Boop FA, Shewmake K, Chadduck W. Child's Nerv Syst, 1996;12371375. http://www.uams.edu/dps/publications.htm
Genes At Work - Topics In Genetics on clinical examination. sagittal synostosis is most commonly diagnosedand represents over 50% of the synostoses. This type of http://www.umdnj.edu/genesatwork/topics/pediatrics/02_pediatrics.htm
Extractions: by Beth A. Pletcher, MD, May 1999 In addition to simple synostosis, there are over 75 syndromes described where craniosynostosis is a feature. When craniosynostosis occurs in the context of growth retardation, multiple congenital anomalies and/or mental retardation, one should always consider a cytogenetic cause. For example, metopic suture synostosis is quite common in deletion 9p which is also associated with dysmorphic facial features and developmental delay. A cloverleaf cranial deformity is a severe defect resulting in a trilobular head shape and is seen in both thanatophoric dysplasia and two subtypes of Pfeiffer syndrome. Recent advances in molecular genetics have greatly expanded our understanding of some of the more well known syndromic craniofacial syndromes. While in the past patients have been categorized by clinical features alone, the discovery of several "craniofacial genes" in recent years has forced clinicians to rethink their diagnostic approach. It has been shown that mutations (genetic typos) within the same gene can result in several different craniofacial syndromes. Furthermore, mutations in different genes can also result in the same collection of clinical features and identical diagnoses. In order to put this in perspective, it may be useful to review some of the more common craniofacial disorders and their clinical descriptions.
Curriculum Vitae For Dr. Todd Maugans - UVM Neurosurgery Maugans TA, McComb JG, Levy ML Surgical management of sagittal synostosis acomparative analysis of strip craniectomy and calvarial vault remodeling. http://www.med.uvm.edu/neurosurgery/cv_maugans.html
Web Hosting, Domain Name, Free Web Site, Email Address Web sagittal synostosis Tanner Buckner (before) sagittal synostosis Tanner's PicturesBefore click on the picture to view a larger image side view top view. http://www.fortunecity.com/millenium/greendale/231/buckner/before.html
CRANIOSYNOSTOSIS sagittal synostosis refers to premature closing of the midlinesuture that runs from front to back of the skull. This results http://www.forwardface.org/misc_text/conditions/cranio.htm
Extractions: CRANIOSYNOSTOSIS Craniosynostosis is a condition that children are either born with or develop. It literally means fusion of the skull bones and may occur singularly or as part of a genetic syndrome. At birth, the human skull is not a solid mass of bone. Rather, soft spots or sutures separate the seven bones that comprise the skull. These separations allow for brain growth. When one or more of these sutures close prematurely, the result is called a craniosynostosis condition. The deformity that results depends on which suture(s) is involved. What actually happens is that as a baby grows, the brain increases rapidly in size. When the suture(s) fuse, there is no room for the brain to develop at that area. This growth inhibition is compensated for by overgrowth in another area resulting in an abnormally shaped skull. Types of Craniosynostosis (a) Isolated craniosynostosis is non-inherited and usually involves only one suture. Sagittal synostosis refers to premature closing of the midline suture that runs from front to back of the skull. This results in an elongated and narrow skull shape called scaphocephaly Metopic synostosis refers to the early closing of the forehead suture.
Johns Hopkins Magazine June 1998 CT, Richtsmeier and several collaborators at the Craniofacial Center embarked onan ambitious experiment to understand a condition called sagittal synostosis. http://www.jhu.edu/~jhumag/0698web/joan.html
Extractions: BEFORE EVERYTHING IN HER WORLD CHANGED, Joan Richtsmeier had been listening raptly to a speaker in a stately auditorium at the University of Chicago. It was the summer of 1994 and the finest minds in the field of vertebrate structure had come from all over the world to attend the conference. Richtsmeier, who had been invited to lead a workshop, was elated. At the workshop she planned to unveil to her international colleagues a new technique for measuring structure and growth. She had already used these methods to study skull growth in patients with severely disfigured faces and skulls, and hoped that the new tools would one day help refine surgical procedures for correcting such psychologically devastating conditions. There were other measures of success in her life. At age 38, Richtsmeier, a biological anthropologist, had already been named associate professor at the Johns Hopkins School of Medicine. She had recently published several articles in major research journals. Her students praised her in their written evaluations.
Craniosynostosis Isolated sagittal synostosis (the suture that runs from the anterior fontanelle tothe posterior fontanelle) occurs in about 55% of cases and isolated coronal http://www.drhull.com/EncyMaster/C/craniosynostosis.html
Extractions: Help for sleepless parents Encyclopedia Index C craniosynostosis Search craniosynostosis Craniosynostosis, or premature fusion of the skull plates, occurs in about one in 2,000 infants. It can be an isolated abnormality or part of a broader malformation syndrome The joints between the plates are called the sutures . Craniosynostosis occurs when for whatever reason, sporadic or as part of a larger problem, one or more of these sutures fuse prematurely. Constrained abnormally in one part of the skull, brain growth forces the remainder of the skull to expand out of proportion, leading to abnormal skull shape. Isolated sagittal synostosis (the suture that runs from the anterior fontanelle to the posterior fontanelle) occurs in about 55% of cases and isolated coronal suture synostosis (the suture that runs down laterally from the anterior fontanelle) occurs in 20% of cases. Diagnosis of craniosynostosis is by xrays, and increasingly by CT scan . Early evaluation and possible neurosurgical intervention is imperative, since the synostosis may be a marker for a larger pattern of abnormalities, and if synostosis is left uncorrected, head and facial shape can be severely affected. There is also concern that some forms of synostosis can cause damage to the infant brain by constraint of normal brain growth.
2000 Summer Research: M. Rosalynn De Leon 2000 Summer Research M. Rosalynn De Leon. Does sagittal synostosis ADverselyAffect Early Psychological Development? Research concerns http://depts.washington.edu/~bridges4/research_pages/roz.html
University Of Washington BRIDGES4 - Undergraduate Research Matthew Speltz Dept Psychiatry Behavioral Sciences, CHMC Research ProjectDoes sagittal synostosis Adversely Affect Early Psychological Development? http://depts.washington.edu/~bridges4/ResearchSummer00.html
Society For Pediatric Anesthesia The authors analyzed each type of synostosis separately. For sagittal synostosis,preop hct ranged between 24%37% with the postop(PACU) hct range 10%-37%. http://www.pedsanesthesia.org/newsletter/2002summer/commentary10.shtml
Extractions: Maugans T Pediatrics 2002:110;166-167 Ninety-seven patients were discharged on the first postoperative day. The authors report lower total cost for this procedure compared to cranial vault remodeling and conclude that their results are excellent with low morbidity. The authors describe their results as follows: "Most patients have achieved or are in the process of reaching normalization of their craniofacial deformities." The commentary by Dr. Maugans, a pediatric neurosurgeon associated with the University of Vermont, notes that the paper from Missouri does indeed show that the technique described can be applied safely, with little OR time and low EBL. He notes that the authors of the paper do not rigorously define their reported good cosmetic results, however. Dr. Maugans, writing for the pediatricians who will likely be seeing these patients in their offices, also comments on the use of custom helmets for weeks to months and does not minimize the costs of time, effort etc involved in this process. Commentary This paper is reviewed simply as a FYI. Yet another surgery being done with an endoscope. I cannot draw on personal experience with surgeons who use this technique, so I found the balanced comments in Dr. Maugans commentary very helpful in evaluating this report of a new surgical technique.
Cancer Center (Siteman Cancer Center): Physician's Directory Photographic assessment of head shape following sagittal synostosis surgery. Thevariants of sagittal synostosis Strategies for surgical correction. http://wuphysicians.wustl.edu/physician2.asp?PhysNum=757
Extractions: The Craniosynostosis Division of the Institute of Reconstructive Plastic Surgery At the Institute of Reconstructive Plastic Surgery at the NYU Medical Center, patients with Craniosynostosis are evaluated and treated by a multidisciplinary team emphasizing comprehensive care beginning at birth and continuing until the completion of facial growth. The team consists of specialists from Plastic Surgery, Dermatology, Genetics, Neurosurgery, Nursing, Ophthalmology, Otolaryngology, Orthodontics, Prosthodontics, Pediatrics, psychology and Speech Pathology. What is Craniosynostosis? Diagram Showing the Cranial Sutures The seven bones that compose the skull of a newborn are separated by soft spots called sutures. In the front of the skull these sutures intersect in the fontanelle, which is the large soft spot located above the baby's forehead. Craniosynostosis is a term that describes premature or earty closure of one or more of these sutures. The deformity which results depends on which suture(s) is involved. The cause of this condition is not known. Some of the craniosynostosis conditions can be inherited or passed between generations of families. Consequently, it is important that all affected children have a genetics evaluation.
Pediatric Neurosurgery, P.A. - Childhood Visual Pathway Glioma date has been a wound infection in a repeat operation done for pansynostosis ina patient born with unilateral coronal and sagittal synostosis who developed http://www.pedneurosurgery.com/bilateral_coronal_cranisynostosis.htm
Extractions: Chris A. Gegg MD DESCRIPTION INTRODUCTION CASE DISCUSSION ... CONCLUSION Craniosynostosis is a common congenital defect requiring surgical intervention by a trained team in order to maximize cosmetic outcome and minimize risk of neurological injury. Recent technological improvements in surgical fixation devices have allowed earlier repair of craniosynostosis, leading to better cosmetic outcomes. The incidence of neurological compromise in non-syndromic craniosynostosis diagnosed early approaches 0%. In this article, we discuss a recent case of bilateral coronal synostosis diagnosed immediately after birth. Radiographic imaging studies (axial and three-dimensional reconstruction) are reviewed pre- and post-operatively. Literature is then reviewed and justification for reducing the tine necessary to wait pre-operativey to maximize normal outcome in patients with congenital skull deformities is discussed. Ultra-early repair of bilateral coronal synostosis can be safely performed on infants within the first week of life. Back to top Craniosynostosis is premature closure of the cranial sutures and may affect any of the sutures. Coronal synostosis affects 1/10,000 live births. Untreated, coronal synostosis leads to progressive skull and facial abnormalities, classically ipsilateral frontal bone and orbital flattening, harlequin eye deformity, and a tendency for a towering configuration of the skull. The only treatment for true craniosynostosis is surgical reconstruction. The earlier the surgical reconstruction is performed, the less the long-term cosmetic deformity. Additionally, intracranial hypertension has been described in as many as 55% of patients with single suture synostosis. With each successive suture closure, the incidence of normal intracranial pressure is essentially halved. Elevated intracranial pressure has been shown to reduce intelligence over a prolonged period.
