1/10. Clinical findings precede objective diagnostic testing in the identification of increased ICP in syndromic craniosynostosis.Crouzon syndrome is an autosomal dominant disorder resulting in facial dysmorphism and craniosynostosis involving multiple cranial sutures. A common but often unrecognized early complication associated with craniosynostosis is a finding of increased intracranial pressure (ICP). This increase in ICP can lead to optic atrophy, neuronal damage, and mental deficits. The case of a 21-month-old girl with Crouzon syndrome is described. Although the child was clinically asymptomatic, a routine ophthalmic exam revealed papilledema and subsequently increased intracranial pressure and craniosynostosis were found. Cranial expansion and bicanthal advancement were performed to relieve the increased pressure. In cases such as these, long-term follow-up is essential because of the progressive nature of the disorder as well as the possibility of a recurrence of elevated intracranial pressure and a need for secondary decompressive surgery.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
2/10. Intracranial hemorrhage resulting from skull base fracture as a complication of Le Fort III osteotomy.Various complications of Le Fort osteotomies have been reported. We describe a lethal complication of Le Fort III osteotomy we encountered in a 9-year-old boy with Crouzon syndrome. A standard Le Fort III osteotomy, including pterygomaxillary dysjunction with a curved osteotome and down-fracture manipulation, was performed uneventfully. When the intraoral buccal wound was closed after fixation of the external midface distraction devices, we discovered hemorrhage originating from the right posterior maxillary region. Although it was stopped with pressure on the osteotomized maxilla, the volume of intraoperative blood loss was nearly 2,000 ml. During the observation period in the intensive care unit, the patient suffered brain death, and he died 3 months later. A computed tomography scan obtained the day after surgery revealed vigorous subarachnoid and intraventricular hemorrhage and transverse fracture of the middle cranial fossa. This skull base fracture was believed to result from intraoperative maneuvers, including the pterygomaxillary dysjunction and down-fracture manipulation. We emphasize the risk of intracranial hemorrhage with Le Fort osteotomy and advise discussing this risk with patients and family members during preoperative consultations.- - - - - - - - - - ranking = 0.25keywords = pressure (Clic here for more details about this article) |
3/10. 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 = 2.25keywords = pressure (Clic here for more details about this article) |
4/10. Nasal continuous positive airway pressure (CPAP) therapy for obstructive sleep apnea in Hallermann-Streiff syndrome.An 8-year-old girl with Hallermann-Streiff syndrome (oculomandibulofacial syndrome) was examined. She had a history of severe snoring, reported nocturnal apnea, excessive daytime hypersomnolence, nocturnal enuresis, and failure to thrive. Overnight polysomnography confirmed severe obstructive sleep apnea. Long-term nasal continuous positive airway pressure (CPAP) therapy completely relieved the obstructive sleep apnea and was associated with improved weight gain and growth.- - - - - - - - - - ranking = 1.25keywords = pressure (Clic here for more details about this article) |
5/10. projection geometry and stress-reduction techniques in craniofacial surgery.Since 1981, we have been able to modify the mathematical patterns of projection geometry to reshape the skull in craniofacial surgery. Unlike burring, morcellization, rotation, and plate switching, this technique actually changes the shape of individual sections of the skull by changing their radius of curvature. The technique is an adaptation of the principles used by engineers to build complex structures such as ships' hulls, airfoils, and domes. The result is a rigid form of the desired shape that becomes permanent with healing. This has several advantages: 1. An increase in the level of safety of craniofacial procedures for remodeling the skull. This is so because there is no need to dissect normal areas as in the standard plate-switching techniques. 2. Decreased operating room time. 3. An increased range of surgical manipulations. No longer is the surgeon limited to the shape of the material present. 4. Relief of edge pressure on the frontal lobes during scalp closure. 5. Creation of a solid bony form over which the pericranial scalp flap can be draped to form new layers of bone.- - - - - - - - - - ranking = 0.25keywords = pressure (Clic here for more details about this article) |
6/10. craniofacial dysostosis: airway obstruction and craniofacial surgery.Craniofacial surgery in craniofacial dysostosis on airway obstruction was studied retrospectively in a consecutive series of patients. The records of 76 patients were reviewed, 27 with Apert syndrome, 47 with Crouzon's syndrome, and two with Pfieffer's syndrome. Of 172 operations, 148 were done for cosmetic reasons, hydrocephalus, or papillary oedema, and 24 were done for airway distress. Forty patients (23%) were children less than 13 years of age, and 22 underwent midface advancement procedures. Only 13 of these operations had been done for airway distress. Two were cured by operation and seven improved. After operation had failed to relieve the airway obstruction, a nasal continuous positive airway pressure device (nCPAP) was fitted to seven patients. The nCPAP relieved or improved airway obstruction recorded by a sleep study. As midface advancement in childhood rarely results in lasting improvement in breathing or aesthetics, it may well be advisable to postpone operation until the early teens. We conclude that with nCPAP operation can in most cases be deferred until a time when a permanent result can be achieved.- - - - - - - - - - ranking = 0.25keywords = pressure (Clic here for more details about this article) |
7/10. Le Fort III advancement with gradual distraction using internal devices.The purpose of this report is to demonstrate a practical clinical method for advancement of the midface using a combination of Le Fort III osteotomy and gradual distraction. Use of internal, submergible distraction devices and modification of the Ilizarov distraction protocol are presented. case reports show the effect of departing from the Ilizarov protocol by eliminating the latency period, initiating distraction intraoperatively, and rapidly distracting the segments postoperatively. A method of controlling fragment distraction rate by regulating pressures within the osteotomy site is described. The design and surgical application of internal distraction devices are shown. Nine patients with midface hypoplasia related to craniofacial anomalies underwent Le Fort III osteotomies assisted by gradual distraction. Ages ranged from 4 to 13 years. Custom internal distraction devices were employed to meet the unique requirements of each patient. Activation of the distraction device was accomplished by a percutaneous pin that remained in place for 3 to 5 days. The rate of transport was determined by regulating the forces within the distraction site. The internal devices remained in place for 6 months for fixation of the transported fragment. Midface advancement, measured as the anterior displacement of the infraorbital rim, ranged from 16 to 30 mm, with a mean of 20 mm. The typical force required to produce incremental movement of the Le Fort III osteotomy was found to be between 7 and 9 kg as transport exceeded 10 mm. Four patients with obstructive sleep apnea prior to surgery had resolution of the disorder. All the midface advancements remained clinically stable. Establishment of osseous continuity was verified by surgical examination in the four patients in whom the distraction devices have been removed. Combining intraoperative advancement of the Le Fort III osteotomy with gradual distraction consistently resulted in larger movements than could be achieved with conventional osteotomies and rigid fixation alone. The pediatric craniofacial skeleton responds differently to distraction than does the orthopedic skeleton. Eliminating the latency period, establishing a substantial bone gap intraoperatively, and rapidly distracting the fragment postoperatively did not adversely affect the osseous union in these patients. The internal, submergible feature of the device design offers advantages in patient acceptance, fixation stability, and wound management. This method of Le Fort III midface advancement has been shown to be clinically practical and effective.- - - - - - - - - - ranking = 0.25keywords = pressure (Clic here for more details about this article) |
8/10. Jugular foraminal stenosis in Crouzon syndrome.We describe a 4-year-old boy with Crouzon syndrome, with associated acanthosis nigricans and Chiari-I malformation, who developed increased intracranial pressure necessitating posterior cranial expansion. Postoperatively, an arteriovenous fistula appeared over the mastoid region. cerebral angiography demonstrated bilateral atresia of the jugular veins and occlusion of the left sigmoid and right transverse sinuses. We propose that increased intracranial venous pressure, secondary to bilateral jugular foraminal stenosis, caused hydrocephaly and venous dilation in the scalp vasculature. The latter set the stage for a traumatic arteriovenous fistula of the scalp, probably resulting from laceration of an adjacent artery and vein. Jugular atresia is a basilar malformation common to achondroplasia and certain eponymous syndromic craniosynostoses. Our patient has a mutation in fibroblast growth factor receptor 3, a different locus in the same gene mutated in achondroplasia.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
9/10. intracranial hypertension in a patient with craniofacial synostosis and patent sutures.Craniosynostosis is often associated with restrictive cranial vault deformities, diminished intracranial volume, and intracranial hypertension. Advances in imaging techniques have provided a method of intracranial volume analysis that has demonstrated a more complicated relationship between craniofacial abnormalities and elevated intracranial pressures. Studies have confirmed a decrease in intracranial volume in the presence of craniosynostosis, but this association is not found in every patient. We report an unusual case of elevated intracranial pressure in a patient with the clinical stigmata of Crouzon's syndrome but with patent cranial vault sutures.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
10/10. Obstructive sleep apnea syndrome in a family with Crouzon's syndrome.Craniofacial anomalies are recognized causes of obstructive sleep apnea syndrome (OSAS) in children. Current literature is limited due to rarity of cases. Furthermore, the mechanism of upper airway obstruction is not clearly understood. We would like to report a family (father and 2 sons) who are suffering from Crouzon's syndrome. The two brothers (ages 1 and 3) were found to have significant obstructive sleep apnea syndrome (OSAS) with failure to thrive. Nasal continuous positive airway pressure (CPAP) markedly improved their OSAS and resulted in accelerated weight gain. The nasoendoscopy and magnetic resonance imaging (MRI) scan taken during natural sleep showed that choanal stenosis, maxillary hypoplasia, posteriorly displaced tongue, lengthened soft palate and adenoid tissues were important in the pathogenesis of upper airways obstruction in Crouzon's syndrome. Nasal CPAP improved airway obstruction by opening a narrow slit as demonstrated by MRI. Our results suggest that OSAS occurred in children with Crouzon's syndrome and that nasal CPAP was a useful treatment modality.- - - - - - - - - - ranking = 0.25keywords = pressure (Clic here for more details about this article) |
| | Next -> |