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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

It has been suggested that the complications associated with intracranial hypertension in craniosynostotic infants may be managed with surgical release of the synostosed sutures. However, both postoperative increases and decreases in intracranial pressure (ICP) have been reported in heterogeneous samples of infants with syndromic and nonsyndromic craniosynostoses. The present study was designed to describe longitudinal changes in ICP in a homogeneous sample of rabbits with uncorrected and corrected familial coronal suture synostosis and compare them with age-matched normal control rabbits. Fifty-three rabbits were divided into three groups: normal rabbits (n = 28), rabbits with uncorrected bilateral coronal suture synostosis (n = 9), and rabbits with bilateral coronal suture synostosis with coronal suturectomy at 25 days of age (n = 16). ICP was measured at 25 and 42 days of age using a Codman epidural microtransducer. Results revealed that rabbits with uncorrected craniosynostosis had significantly (P < 0.05) higher ICP at 25 days of age than normal control rabbits by approximately 86%. However, by 42 days of age, ICP in normal rabbits increased by 75%, whereas ICP in rabbits with uncorrected craniosynostosis decreased by 69% over the same time. Synostotic rabbits with coronal suturectomy showed a 50% decrease in ICP immediately after surgical release and then followed the normal, age-related ICP pattern, which significantly increased by 75% at 42 days of age. Results suggest that, in the rabbit model, the postsuturectomy rise in ICP may simply be normal, age-related changes, although a longer follow-up will be needed to determine the recurrence of pathological ICP. Possible multifactorial explanations for intracranial decompression and compensation in the craniosynostotic rabbit model are also discussed.
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PMID:Increased intracranial pressure after coronal suturectomy in craniosynostotic rabbits. 1038 9

Previous research has shown that dietary docosahexaenoic acid (DHA) attenuates the development of high blood pressure in young spontaneously hypertensive rats (SHR). The purpose of this study was to investigate the effects of dietary DHA on organ and vascular fatty acid composition in SHR. Given the important structural and functional role of fatty acids in cell membranes, alterations in fatty acid composition may contribute to the antihypertensive effect of DHA. SHR were fed a purified diet containing either a corn/soybean oil mixture (CSO, control) or a DHA-enriched oil for 6 weeks. The DHA diet markedly increased the levels of DHA in the aorta, renal artery, plasma, liver, heart, kidney, and lung by 5-, 15-, 7-, 6-, 3.8-, 3.5-, and 8.8-fold (P<0.001), respectively. The levels of eicosapentaenoic acid were also increased while there was a concomitant reduction in arachidonic and adrenic acids. Therefore, dietary DHA increases the incorporation of omega-3 polyunsaturated fatty acids in specific organs and vascular tissue in SHR at the expense of omega-6 polyunsaturated fatty acids.
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PMID:The effects of a diet rich in docosahexaenoic acid on organ and vascular fatty acid composition in spontaneously hypertensive rats. 1067 Jun 90

Although it is currently thought that surgery is indicated mainly for cosmetic reasons in isolated craniosynostoses, the functional aspects of the treatment must not be underestimated. Prospective studies on intracranial pressure and mental evolution of these children have shown that there were functional consequences in a significant proportion of cases even of single suture fusion. The frequency of increased intracranial hypertension and the risk of mental impairment depend on the age of the child and the type of craniosynostosis. In nonsyndromic cases, the higher risks are observed in multisutural craniosynostoses (brachycephaly, oxycephaly). In syndromic cases, the risk of intracranial hypertension is higher in Crouzon syndrome, and Apert syndrome carries the higher risk of mental retardation. The study of a personal series of 2,137 craniosynostoses shows that the functional and the cosmetic results are better after early surgery, and that the operative risks are not higher in infants than in older children.
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PMID:Management of craniosynostoses. 1115 14

Surgical procedures for correction of craniosynostosis are performed in young infants with a small blood volume and represent major surgery with extensive blood loss. An accurate determination and a precise restoration of blood losses represent the major concern for the anaesthetist during this surgery. The preoperative assessment of these patients is usually simple, except in the cases where the craniosynostosis is associated with other congenital malformations. The anaesthetist should keep in mind that intracranial hypertension may be associated with craniosysnostosis, which modify the anaesthetic management, especially the induction of anaesthesia. Even though the psychological impact of a craniosynostosis should be taken into consideration, surgery is most often indicated for functional considerations, therefore parents should be informed of the risks related to the procedure. During the postoperative period the major concerns are related to the possibility of a persistent bleeding, which usually decreases and disappears over the first 12 hours.
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PMID:[Anesthetic management for craniosynostosis]. 1191 69

In this study, we explored two strategies of assessing continuous intracranial pressure (ICP) recordings in children with craniosynostosis, namely either by computation of the mean ICP or by computation of the accurate numbers of ICP elevations of different durations. The ICP recordings of 121 consecutive patients with a tentative diagnosis of craniosynostosis who underwent continuous ICP monitoring were examined. The relationship between mean ICP and numbers of ICP elevations was defined. The distribution of numbers of ICP elevations between patients either undergoing surgery or conservative treatment was also compared, since the choice of treatment was heavily dependent on the results of ICP monitoring. At the time of ICP monitoring, calculation of mean ICP was the main parameter for assessment of ICP curves. After a median observation period of 16 months, the ICP curves were reexamined by means of the software Sensometrics Pressure Analyser, which presents the ICP curve as a matrix of numbers of ICP elevations of different levels (20-40 mm Hg) and durations (0.5- 20 min). Since the recording period differed between the cases, the numbers were standardized to a given recording time of 10 h, to allow for comparisons between patients. Cases with a borderline mean ICP during sleep (mean ICP 10-15 mm Hg) constituted 40.5% of the 121 patients. In this group, a rather weak relationship between mean ICP and the number of ICP elevations above 20 mm Hg was found, as well as a relatively high number of ICP elevations above 20 mm Hg of various durations. As compared to the patients undergoing surgery, a rather high number of ICP elevations above 20 mm Hg of various durations was found in patients undergoing conservative treatment. The study confirmed our hypothesis that in children with craniosynostosis, calculation of mean ICP does not describe the ICP curve in a reliable way. Decision-making should also include the computation of the distribution of numbers of ICP elevations, since this procedure represents a more sensitive strategy of detecting intracranial hypertension.
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PMID:Assessment of continuous intracranial pressure recordings in childhood craniosynostosis. 1242 46

Apert Syndrome, also called acrocephalosyndactylia type 1, is characterized by craniostenosis with early fusion of sutures of the vault and/or cranial base, associated to mid-face hypoplasia, symmetric syndactylia of the hands and feet and other systemic malformations. CNS malformations and intracranial hypertension are frequently observed in these patients. Early surgical treatment aims to minimize the deleterious effects of intracranial hypertension. Fronto-orbital advancement, the usual surgical technique, increases the intracranial Volume and improves the disposition of encephalic structures previously deformed by a short skull. This study analyzes CNS alterations revealed by magnetic resonance in 18 patients presenting Apert Syndrome, and the conformational alterations in the encephalic structures after surgical treatment. The patients' age in February 2001 ranged from 14 to 322 Months (m=107). Image study included brain magnetic resonance showing ventricular enlargement in five cases (27.8%), corpus callosum hypoplasia in five cases (27.8%), septum pellucidum hypoplasia in five cases (27.8%), cavum vergae in two cases (11.1%) and, arachnoid cyst in the posterior fossa in two cases (11.1%). Absence of CNS alterations was noted in 44.4% of cases. A corpus callosum morphologic index was established by dividing its height by its length, which revealed values that ranged from 0.4409 to 1.0237. The values of this index were correlated to the occurrence or absence of surgical treatment (p=0.012; t=2.83). Data analysis allowed the conclusion that the corpus callosum morphologic measure quantified the conformational alterations of the cerebral structures determined by the surgical treatment.
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PMID:Apert syndrome: analysis of associated brain malformations and conformational changes determined by surgical treatment. 1509 49

Intracranial pressure (ICP) is derived from cerebral blood and cerebrospinal fluid (CSF) circulatory dynamics and can be affected in the course of many diseases of the central nervous system. Monitoring of ICP requires an invasive transducer, although some attempts have been made to measure it non-invasively. Because of its dynamic nature, instant CSF pressure measurement using the height of a fluid column via lumbar puncture may be misleading. An averaging over 30 minutes should be the minimum, with a period of overnight monitoring in conscious patients providing the optimal standard. Computer-aided recording with online waveform analysis of ICP is very helpful. Although there is no "Class I" evidence, ICP monitoring is useful, if not essential, in head injury, poor grade subarachnoid haemorrhage, stroke, intracerebral haematoma, meningitis, acute liver failure, hydrocephalus, benign intracranial hypertension, craniosynostosis etc. Information which can be derived from ICP and its waveforms includes cerebral perfusion pressure (CPP), regulation of cerebral blood flow and volume, CSF absorption capacity, brain compensatory reserve, and content of vasogenic events. Some of these parameters allow prediction of prognosis of survival following head injury and optimisation of "CPP-guided therapy". In hydrocephalus CSF dynamic tests aid diagnosis and subsequent monitoring of shunt function.
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PMID:Monitoring and interpretation of intracranial pressure. 1514 91

In a retrospective study, the characteristics of a group of patients (n = 9) with a postnatally expressed Crouzon syndrome were described. Although they do not always display the physical signs of craniosynostosis, such patients are highly at risk of developing symptoms secondary to multiple suture synostosis. By reviewing the hospital files, radiographs, and three-dimensional computed tomography scans of these patients, it was possible to describe the pattern of suture obliteration chronologically. Furthermore, certain phenotypic signs and symptoms such as skull shape and development of digital impressions, a bulge at the bregma, and intracranial hypertension were inventoried as well as patients' genotypes. Interestingly, ossification started at the lambdoid sutures in at least four patients and most likely in three additional ones. The coronal sutures were the last to ossify in at least three of the patients. Various skull shapes were encountered. Furthermore, all nine patients developed digital impressions, starting occipitally in eight of them. Seven patients developed a bulge at the bregma, and four of them exhibited intracranial hypertension. The genotype varied in our patients. To recognize patients with postnatal Crouzon syndrome as soon as possible, special attention must be paid to 1) occipital development of digital impressions and/or ossification of sutures, 2) development of a prominent bregma, 3) development of intracranial hypertension, and/or 4) progressive characteristic "crouzonoid" features. Such patients can be considered as representing a subtype of Crouzon syndrome. To prevent or treat intracranial hypertension and/or loss of vision, surgical intervention should be performed at the onset of progressive craniosynostosis between 1 and 2 years of age.
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PMID:Crouzon syndrome: phenotypic signs and symptoms of the postnatally expressed subtype. 1516 38

Four children with syndromic craniosynostosis (Crouzon's syndrome and Apert syndrome) developed irreversible visual loss. Apart from papilledema, there were no other clinical symptoms of intracranial hypertension. Ventriculomegaly or hydrocephalus was present in all cases. Two children were known to have obstructive sleep apnea syndrome. These cases are reported to inform specialists involved in follow-up of children with syndromic craniosynostosis that visual loss can develop suddenly without other symptoms of intracranial hypertension. To prevent visual loss, papilledema should be detected at an early stage and intervention should be instituted promptly. Therefore, periodic funduscopy should be performed in children at risk, such as children with syndromic craniosynostosis and additional hydrocephalus or obstructive sleep apnea syndrome.
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PMID:Visual loss in syndromic craniosynostosis with papilledema but without other symptoms of intracranial hypertension. 1554 95

The Saethre-Chotzen syndrome (SCS) is an autosomal dominant craniosynostosis syndrome with uni- or bilateral coronal synostosis and mild limb deformities. It is caused by loss-of-function mutations of the TWIST 1 gene. In an attempt to delineate functional features separating SCS from Muenke's syndrome, we screened patients presenting with coronal suture synostosis for mutations in the TWIST 1 gene, and for the Pro250Arg mutation in FGFR3. Within a total of 124 independent pedigrees, 39 (71 patients) were identified to carry 25 different mutations of TWIST 1 including 14 novel mutations, to which six whole gene deletions were added. The 71 patients were compared with 42 subjects from 24 pedigrees carrying the Pro250Arg mutation in FGFR3 and 65 subjects from 61 pedigrees without a detectable mutation. Classical SCS associated with a TWIST 1 mutation could be separated phenotypically from the Muenke phenotype on the basis of the following features: low-set frontal hairline, gross ptosis of eyelids, subnormal ear length, dilated parietal foramina, interdigital webbing, and hallux valgus or broad great toe with bifid distal phalanx. Functional differences were even more important: intracranial hypertension as a consequence of early progressive multisutural fusion was a significant problem in SCS only, while mental delay and sensorineural hearing loss were associated with the Muenke's syndrome. Contrary to previous reports, SCS patients with complete loss of one TWIST allele showed normal mental development.
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PMID:Saethre-Chotzen syndrome caused by TWIST 1 gene mutations: functional differentiation from Muenke coronal synostosis syndrome. 1625 95


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