No abstract available.
Umbilical Cord*

With the ever- increasing number of intact aneurysms revealed by modern imaging, the options for their management are assuming greater importance. The surgical management of patients with unruptured intracranial aneurysms continues to be contoversial, and the criteria for withholding treatment or choosing between endovscular embolization and conventional microsurgery are not well delineated. In order to define the surgical result for unruptured intracranial aneurysms, 41 patients(from June 1989 to May 1995) with surgically treated unruptured aneurysms were analyzed. They were categorized as incidental, multiple or aneurysm with mass effect. Subarachnoid hemorrhage from another aneurysm(multiple) was the most common presentation(19 patients). Eleven patients were presented with incidental findings unrelated to aneurysmal subarachnoid hemorrhage or direct aneurysmal mass effect, and 11 patients were presented with mass effect such as cranial nerve palsy or brain stem compression. We could perform direct neck clipping presented with mass effect such as cranial nerve palsy or brain stem compression. We could perform direct neck clipping with without wrapping in 37 patients, trapping in 2, and wrapping in 1. One patient with giant vertebrobasilar artery aneurysm(greater than 25mm in diameter) which was presented with mass effect could not be treated adequately. Instances of morbidity included cranial nerve injury in 4 patients, hemiparesis in 3, hematoma in 2, and major hemispheric infarction in 1. One patient presented with mass effect, died from major hemispheric infarction after surgery of proximal internal carotid artery aneurysm with a size greater than 25mm in diameter. Two patients, who underwent surgery for giant vertebrobasilar artery aneurysms presented with mass effect, were in poor state due to persistent cranial nerve palsy and homiparesis. In general overall outcome was very good. Excellent or good outcome was achieved in 38 patients(92.7%) while 3 patients(7.3%) either died or was/were in poor condition. The aneurysm size was correlated well with the surgical outcome. We have achieved excellent or good out comes in 100% of patients with aneurysms 25mm or less in diameter. However, with aneurysms greater than 25mm in diameter, the outcomes were very poor with 75% of these patients in poor state or dead. "Surgery in unruptured aneurysms?" The answer was "Yes". We believe the size and location of the aneurysm are the key predictons of risk for sugical morbidity.
Aneurysm ; Arteries ; Brain Stem ; Carotid Artery, Internal ; Cranial Nerve Diseases ; Cranial Nerve Injuries ; Hematoma ; Humans ; Incidental Findings ; Infarction ; Intracranial Aneurysm* ; Microsurgery ; Neck ; Paresis ; Subarachnoid Hemorrhage ; Withholding Treatment

OBJECTIVE: Activating mutations in the fibroblast growth factor receptor-2 (FGFR2) have been shown to cause syndromic craniosynostosis such as Apert and Crouzon syndromes. The purpose of this pilot study was to investigate the resultant phenotypes induced by the two distinctive bone-targeted gene constructs of FGFR2, Pro253Arg and Cys278Phe, corresponding to human Apert and Crouzon syndromes respectively. METHODS: Wild type and a transgenic mouse model with normal FGFR2 were used as controls to examine the validity of the microinjection. Micro-CT and morphometric analysis on the skull revealed the following results. RESULTS: Both Apert and Crouzon mutants of FGFR2 induced fusion of calvarial sutures and anteroposteriorly constricted facial dimension, with anterior crossbite present only in Apert mice. Apert mice differed from Crouzon mice and transgenic mice with normal FGFR2 in the anterior cranial base flexure and calvarial flexure angle which implies a possible difference in the pathogenesis of the two mutations. In contrast, the transgenic mice with normal FGFR2 displayed normal craniofacial phenotype. CONCLUSION: Apert and Crouzon mutations appear to lead to genotype-specific phenotypes, possibly causing the distinctive sites and sequence of synostosis in the calvaria and cranial base. The exact function of the altered FGFR2 at each suture needs further investigation.
Acrocephalosyndactylia ; Animals ; Craniofacial Dysostosis* ; Craniosynostoses ; Fibroblast Growth Factors ; Humans ; Malocclusion ; Mice ; Mice, Transgenic ; Microinjections ; Phenotype ; Pilot Projects ; Skull ; Skull Base ; Sutures ; Synostosis