1/36. Raised intracranial pressure in minimal forms of craniosynostosis.Most cases of craniosynostosis are diagnosed during early infancy, but occasionally craniosynostosis evolves with minimal cranial involvement and goes unnoticed until late childhood. Seemingly these mild forms of craniosynostosis cause few, if any, symptoms of neurological involvement. We describe the cases of a 9-year-old girl and a 6-year-old boy who presented with evident signs of raised intracranial pressure (ICP), together with a negligible skull deformity. We have termed these cases as occult craniosynostosis. Differential diagnosis in our patients was established against known causes of benign intracranial hypertension. Bilateral expanding craniotomies afforded total relief from the symptoms and signs of raised ICP. Neurosurgeons treating children with symptoms and signs of benign intracranial hypertension should be aware of the possibility of minimal forms of craniosynostosis evolving with marked manifestations of raised ICP.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
2/36. Coincident metopic synostosis and deformational plagiocephaly.A male infant is described in whom coincident pathologies of metopic synostosis and deformational plagiocephaly were observed. The role in causation of localized pressure (in particular, extreme constraint) is addressed.- - - - - - - - - - ranking = 0.2keywords = pressure (Clic here for more details about this article) |
3/36. Antley-Bixler syndrome, description of two new cases and review of the literature.OBJECTIVE: Antley-Bixler syndrome (ABS) is a rare disorder characterized by multiple malformations of cartilage and bone including multisynostotic osteodysgenesis, midface hypoplasia, choanal atresia or stenosis, femoral bowing, neonatal fractures and multiple joint contractures and, occasionally, urogenital, gastrointestinal or cardiac defects. Since the first report in 1975, at least 34 cases (including this report) have been described. We present 2 cases of congenital hydrocephalus, suggesting the cause of craniosynostosis and midface hypoplasia is associated with ABS. CLINICAL PRESENTATION: The first case was a 1-day-old female with arnold-chiari malformation, multiple cranial synostosis, obstructive hydrocephalus and radioulnahumeral synostosis. Ventriculoperitoneal (V-P) shunting was performed when she was 7 days old. She died 42 days later due to cardiopulmonary failure. The second case was a 2-month-old female with bilateral coronal synostosis, obstructive hydrocephalus and brachycephaly. V-P shunting was done soon after her admission, and bilateral lateral canthal advancement with the floating forehead procedure was performed 1 month later. She is still alive with good development. DISCUSSION: The main anomalies of the ABS can be divided into 4 categories: craniofacial, skeletal, extremity and urogenital anomalies. They may be the result of mutation in the fibroblast growth factor receptor 2 (Ser351Cys) gene, which was confirmed in our case 2. Craniosynostosis combined with hydrocephalus created congenital increased intracranial pressure (IICP). Early V-P shunt implantation and surgical release of the closed suture and lateral canthal advancement should be done as soon as possible, ideally when the patient is younger than 3 months. CONCLUSION: Early correction of craniosynostosis was feasible and safe for one of our patients. We offer our experience in the treatment of hydrocephalus and correction of craniosynostosis to relieve IICP.- - - - - - - - - - ranking = 0.2keywords = pressure (Clic here for more details about this article) |
4/36. Results of cranial vault reshaping.This study was designed to assess the outcome of cranial vault reshaping for correction of deformity of the skull and the upper face. A retrospective review of all children who underwent cranial vault reshaping by a single team of surgeons between 1993 and 1996 was performed. There were 10 children in the series. The age at surgery ranged from 6 to 62 months (mean age, 25 months). Five children in the series had untreated sagittal craniosynostosis with scaphocephaly, two had pansynostosis resulting in cloverleaf skull deformity, and three had turricephaly after shunt treatment of hydrocephalus. There was no operative mortality. blood loss ranged from 250 to 1,500 ml (mean, 422 ml). All patients needed transfusion. There were two major complications resulting from increased intracranial pressure, but both patients recovered completely with no neurological sequelae. titanium plates and screws were used in all patients, but were removed in two when they became palpable. The 5 children with sagittal craniosynostosis had a normal head shape. The 2 children with cloverleaf skull have improved head shape with persistent increased bitemporal width and round faces. The 3 children with turricephaly after shunting have marked improvement with mild persistent deformity. This study shows that cranial vault reshaping is safe and can lead to a long-term normal head shape in children with late correction of sagittal craniosynostosis. Children with more severe anomalies, particularly syndromic patients, can be improved but will have persistent mild deformity.- - - - - - - - - - ranking = 0.2keywords = pressure (Clic here for more details about this article) |
5/36. hyponatremia in the postoperative craniofacial pediatric patient population: a connection to cerebral salt wasting syndrome and management of the disorder.hyponatremia after cranial vault remodeling has been noted in a pediatric patient population. If left untreated, the patients may develop a clinical hypoosmotic condition that can lead to cerebral edema, increased intracranial pressure, and eventually, to central nervous system and circulatory compromise. The hyponatremia has traditionally been attributed to the syndrome of inappropriate secretion of antidiuretic hormone (SIADH); however, in our patients the treatment has been resuscitation with normal saline as opposed to fluid restriction (the accepted treatment of SIADH), thus placing the diagnosis of SIADH in question. patients who developed hyponatremia after intracranial injury or surgery were, until recently, grouped together as having SIADH. However, there are diagnosis and treatment differences between SIADH and another distinct but poorly understood disorder that is designated cerebral salt wasting syndrome (CSW). CSW is associated with increased urine output and increased urine sodium concentration and volume contraction, and it is frequently seen after a central nervous system trauma. We therefore developed a prospective study to evaluate the cause of the sodium imbalance.Ten consecutive pediatric patients who underwent intracranial surgery for various craniosynostotic disorders were postoperatively monitored in the pediatric intensive care unit for hemodynamic, respiratory, and fluid management. The first four patients were evaluated for electrolyte changes and overall fluid balance to determine the consistency with which these changes occurred. The remaining six patients had daily (including preoperative) measurement of serum electrolytes, urine electrolytes, urine osmolarity, serum antidiuretic hormone (ADH), aldosterone, and atrial natriuretic hormone (ANH). All patients received normal saline intravenous replacement fluid in the postoperative period.All of the patients developed a transient hyponatremia postoperatively, despite normal saline resuscitation. serum sodium levels as low as 128 to 133 mEq per liter (normal, 137 to 145 mEq per liter) were documented in the patients. All patients had increased urine outputs through the fourth postoperative day (>1 cc/kg/h). The six patients who were measured had an increased ANH level, with a peak value as high as 277 pg/ml (normal, 25 to 77 pg/ml). ADH levels were low or normal in all but one patient, who had a marked increase in ADH and ANH. aldosterone levels were variable. On the basis of these results, all but one patient showed evidence of CSW characterized by increased urine output, normal or increased urine sodium, low serum sodium, and increased ANH levels. The other patient had similar clinical findings consistent with CSW but also had an increase in ADH, thus giving a mixed laboratory picture of SIADH and CSW.The association of CSW to cranial vault remodeling has previously been ignored. This study should prompt reevaluation of the broad grouping of SIADH as the cause of all hyponatremic episodes in our postoperative patient population. An etiologic role has been given to ANH and to other, as yet undiscovered, central nervous system natriuretic factors. All of the patients studied required normal saline resuscitation, a treatment approach that is contrary to the usual management of SIADH. These findings should dictate a change in the postoperative care for these patients. After cranial vault remodeling, patients should prophylactically receive normal saline, rather than a more hypotonic solution, to avoid sodium balance problems.- - - - - - - - - - ranking = 0.2keywords = pressure (Clic here for more details about this article) |
6/36. 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 = 0.8keywords = pressure (Clic here for more details about this article) |
7/36. Three-dimensional cranial expansion using distraction osteogenesis for oxycephaly.Oxycephaly is associated with raised intracranial pressure as a result of the fusion of multiple cranial sutures. We have performed an effective and less invasive cranial expansion by means of three-dimensional cranial distraction for the treatment of oxycephaly with suspicion of increased intracranial pressure. We describe two oxycephaly cases and the surgical technique of three-dimensional cranial expansion using distraction osteogenesis.- - - - - - - - - - ranking = 0.4keywords = pressure (Clic here for more details about this article) |
8/36. Rapid development of Chiari I malformation in an infant with Seckel syndrome and craniosynostosis. Case report and review of the literature.To illustrate the rapidity with which a child can develop a severe, symptomatic Chiari I malformation, the authors present the case of a 3-month-old infant with Seckel syndrome (microcephaly, micrognathia, craniosynostosis, and multiple other abnormalities) and posterior sagittal and bilateral lambdoid synostosis. The infant underwent magnetic resonance (MR) imaging shortly after birth; the initial image demonstrated the cerebellar tonsils in the posterior fossa, with no herniation. He subsequently developed severe apneic episodes and bradycardia; repeated MR imaging at 3 months demonstrated severe tonsillar herniation with compression of the brainstem. The child underwent posterior fossa remodeling surgery, including release of the posterior sagittal and lambdoid sutures and decompression of the Chiari I malformation. The patient's apnea gradually improved; however, he died of complications of pneumonia and sepsis several weeks later. The authors identified from the literature 21 patients in whom there was a documented MR image or other neuroimage that did not reveal evidence of a Chiari I malformation, followed by a subsequent study with clear documentation of the presence of Chiari I malformation. The interval between the initial study and the development of the tonsillar herniation ranged from 11 days to 18.5 years. In most cases, a lumbar cerebrospinal fluid (CSF) diversion had been performed. This patient developed a severely symptomatic Chiari I malformation during a 3-month period. These reports illustrate that the Chiari I malformation can develop rapidly in the face of increased intracranial pressure, craniosynostosis, and spinal CSF diversion.- - - - - - - - - - ranking = 0.2keywords = pressure (Clic here for more details about this article) |
9/36. Sustained raised intracranial pressure implicated only by pattern reversal visual evoked potentials after cranial vault expansion surgery.Craniosynostosis, the premature fusion of cranial sutures, may be associated with raised intracranial pressure (ICP) with or without a reduced intracranial volume. Regardless of the aetiology, raised ICP may result in optic neuropathy, the timely detection of which can prevent further visual deterioration. Raised ICP is usually treated with craniofacial surgery such as cranial vault expansion. In this case study, we recorded serial pattern reversal visual evoked potentials (pVEPs) and obtained digital optic disc images before and after cranial vault expansion surgery. The amplitude of the pVEPs continued to decrease after cranial vault expansion surgery, prompting further neuroimaging that implicated a blocked ventriculo-peritoneal shunt. Only after shunt revision did the pVEP amplitude increase. Throughout the monitoring period, there was no change in the appearance of either the right or left optic disk, nor a consistent change in visual acuity.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
10/36. 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 = 1.8keywords = pressure (Clic here for more details about this article) |
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