1/15. On the assets of CAD planning for craniosynostosis surgery.SkullWiz is a computer-aided design program that transforms computer tomographic data of the neurocranium into a mathematical model that can be interactively manipulated to plan craniosynostosis surgery. Proper planning of this type of surgery involves reference to the underlying viscerocranium and to normal neurocranial dimensions, simulation of all basic surgical actions (closed and open osteotomy, translation, rotation, bending, removal, burring), and reference to the mechanical properties of calvarial bone at a given age. With SkullWiz, infinite trials are possible to develop a surgical plan that combines minimal action with maximum morphologic result. In contrast, physical models, e.g., foam milled or stereolitographic, provide just a single (or double, after gluing) opportunity to visualize three-dimensional morphology and simulate a treatment plan, without reference support. Validation of SkullWiz is difficult due to parameter variability. Its assets are therefore graphically exemplified in two common types of nonsyndromatic single-suture craniosynostosis-trigonocephaly and anterior plagiocephaly. SkullWiz is one of the most accurate planning tools currently available for craniosynostosis surgery. Accurate transfer of the planning by aluminium templates results in efficient and precise surgery by avoiding per-operative "chipping and fitting."- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/15. encephalocele as a late complication of cranial vault reconstruction in a patient with Crouzon's syndrome.encephalocele is a rare late complication of craniosynostosis repair. An undiagnosed nick to the dura is the etiology of this complication. It usually presents within a few months to years after repair. This case report describes a patient with Crouzon's disease who had a craniosynostosis repair as an infant and presents 22 years later with an encephalocele. The patient had a thorough physical exam, 2D, and 3D CT Scans as a preoperative workup. Surgical intervention included repair of the encephalocele, cranialization of the frontal sinus with bone grafting, and Lefort III osteomies for mid face advancement. The patient benefited from a two-team simultaneous approach between neurosurgery and Plastic Surgery. The patient also benefited from an aggressive one-stage repair of all her defects and deformities.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
3/15. Unilateral fusion of the frontosphenoidal suture: a rare cause of synostotic frontal plagiocephaly.Unilateral coronal synostosis is the common appellation for premature, one-sided fusion of the frontoparietal suture-the most common cause of synostotic frontal plagiocephaly. However, frontal asymmetry can also result from isolated fusion across the anterior cranial base without involvement of the frontoparietal suture. This article describes three patients with localized synostosis of the frontosphenoidal suture, the medial extension of the coronal ring. Two patients were initially misdiagnosed as having unilateral coronal synostosis and the other as having deformational frontal plagiocephaly. The patients had variable frontal flattening, with depression and recession of the ipsilateral orbital rim. The nasal root was midline or slightly deviated to the contralateral side. The sagittal position of the ipsilateral malar eminence was slightly retruded in one patient and symmetric in the other two. The auricular position was symmetric in the sagittal plane for all patients. In all three patients, computed tomography examination demonstrated a patent frontoparietal suture and fusion of the frontosphenoidal suture (basilar hemicoronal ring). Two patients had involvement of contiguous sutures: one had fusion extending to the sphenoethmoidal suture and the other's involved part of the sphenozygomatic suture. The sagittal suture was midline in all patients. In summary, synostotic frontal plagiocephaly denotes a relatively broad phenotypic spectrum that includes unilateral coronal synostosis and more isolated fusions in the basilar coronal ring. The physical findings resulting from frontosphenoidal synostosis are unique, yet careful evaluation will minimize confusion with other causes of asymmetric frontal flattening. Proper diagnosis necessitates awareness of this uncommon entity and requires focused computed tomographic assessment.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
4/15. diagnosis and management of positional head deformity.In children with positional head deformity (posterior plagiocephaly), the occiput is flattened with corresponding facial asymmetry. The incidence of positional head deformity increased dramatically between 1992 and 1999, and now occurs in one of every 60 live births. One proposed cause of the increased incidence of positional head deformity is the initiative to place infants on their backs during sleep to prevent sudden infant death syndrome. With early detection and intervention, most positional head deformities can be treated conservatively with physical therapy or a head orthosis ("helmet").- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
5/15. Progressive postnatal craniosynostosis and increased intracranial pressure.Since its first description by Virchow in 1851, craniosynostosis has been known as a potentially serious condition resulting in premature fusion of skull sutures. Traditionally, craniosynostosis has been regarded as an event that occurs early in fetal development, resulting in a skull shape at birth that is determined by the suture or sutures involved. In recent years, a different form of craniosynostosis has been observed. patients initially come to the attention of physicians because they exhibit midface hypoplasia or occasionally hypertelorism. The affected individuals all have a normal skull shape and open sutures in infancy but develop multiple-suture craniosynostosis postnatally, ultimately requiring surgical correction. These cases are significant because, although the patients do not initially display the physical manifestations of craniosynostosis, they frequently develop increased intracranial pressure, which can have devastating consequences. Unless these patients are recognized and vigilant follow-up monitoring is instituted at an early age, permanent impairment can result. A retrospective chart review study was conducted, and patients with multiple-suture craniosynostosis who developed symptoms of increased intracranial pressure were selected. The patients were divided into two groups, namely, those with normal sutures and/or head shape at birth (progressive craniosynostosis) (n = 15) and those with abnormal head shapes at birth (classic syndromic craniosynostosis) (n = 12). Clinical and radiological findings typically used to monitor the development of increased intracranial pressure were reviewed for both groups and compared. In addition, mutational analyses were performed. All patients with progressive postnatal craniosynostosis demonstrated clinical, radiological, or ophthalmological evidence of increased intracranial pressure, requiring skull expansion. Those patients displayed papilledema, anterior fontanelle bulge, and thumbprinting more often than did the patients with classic craniosynostosis. Thirteen of 15 patients were given the clinical diagnosis of Crouzon syndrome, which raises the question of whether such patients represent a subset of patients with this syndrome. Mutational analyses for the patients with progressive craniosynostosis demonstrated that, of 13 patients tested, 11 had mutations in exon 7 or 9 of FGFR2, which is a common site of mutations in Crouzon syndrome. The traditional indications of increased intracranial pressure used to monitor patients with classic craniosynostosis can be used to monitor patients with progressive postnatal craniosynostosis, particularly anterior fontanelle bulge, papilledema, and thumbprinting. It is thought that regular monitoring of these characteristics may lead to earlier diagnosis and allow for surgical intervention before the development of undesirable outcomes. It is important for clinicians to be aware of this group of patients, because any delay in diagnosis and treatment can result in severe consequences for the patients.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
6/15. A case of unilateral coronal synostosis in a child with craniofacial microsomia.Craniofacial microsomia is a congenital malformation complex associated with a wide array of craniofacial and extracraniofacial anomalies. Frontal plagiocephaly has been shown to occur in approximately 5% to 12% of patients with craniofacial microsomia. The etiology of craniofacial microsomia-associated frontal plagiocephaly is unclear; of the cases described in the literature, all but one had physical findings suggestive of deformational plagiocephaly. In the case with equivocal physical findings, radiographic studies showed no evidence of craniosynostosis. Unlike the above cases, we report a documented case of radiologically-confirmed unilateral coronal synostosis in a child with craniofacial microsomia.- - - - - - - - - - ranking = 2keywords = physical (Clic here for more details about this article) |
7/15. body image and the process of reconstructive surgery.Newer craniofacial operative techniques produce rapid changes in objective appearance and permit the study of body-image change. In 21 patients with either Crouzon's disease or Alpert's syndrome, a four-factor model of body-image development was applied that emphasizes cognitive growth, perception of body stimuli, stimuli from the environment in the form of comparison, and the response from others. Before a child is of school age, he has substantially defined his body image, therefore corrective surgery must be considered earlier. Although surgical intervention may produce significant objective physical change, there is not a correspondingly rapid change in body image. Four phases in the modification of body image are (1) the decision to undergo surgery, (2) the operative experience, (3) the immediate postoperative period, and (4) the reintegration stage. Recognition of this phasic process will help integrate care of these patients.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
8/15. trisomy 6q25 to 6qter in a severely retarded 7-year-old boy with turricephaly, bow-shaped mouth, hypogenitalism and club feet.trisomy for a small terminal segment of chromosome 6q produces a characteristic syndrome of malformations and dysmorphic signs which, on the basis of comparison with a previously published case, may be suspected on clinical grounds. The present case concerns a 7-year-old boy, the son of a carrier mother t(6;14)(q25;qter). The main symptoms are: very severe physical and mental retardation, turricephaly, Cupid's bow mouth with narrow lips, almond-shaped eyes with narrow palpebral fissures and ptosis, micropenis with absence of scrotum, club feet, hammer toes, and extension contractures. In addition, there are a great many minor dysmorphic features.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
9/15. rehabilitation aspects of Apert's syndrome.Two children with the unusual condition known as Apert's syndrome are presented. Both have the typical manifestations of the syndrome: craniosynostosis, exorbitism, hypertelorism, maxillary hypoplasia, dental malocclusion, cleft palate, compound syndactyly of the hands and simple syndactyly of the feet. A review of the latest surgical procedures for correction of these defects is presented and the importance of early physical and social rehabilitation is stressed.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
10/15. Baller-Gerold syndrome craniosynostosis-radial aplasia syndrome.A new case of the Baller-Gerold syndrome is described in a 6 1/2-year-old, black male who presented at birth with bilateral synostoses of the coronal and lambdoidal sutures, bilateral radial aplasia, vertebral anomalies and genito-urinary malformations. The parents and siblings were unaffected, and there was no history of consanguinity. A review of the history and physical findings in our patient and in the four other patients previously reported in the literature is provided, with a discussion on pathogenesis, prognosis and the possible autosomal recessive mode of inheritance of the syndrome.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
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