Cases reported "Hydrocephalus"

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1/96. Management of the ventricular shunt in posterior deformities of the skull in craniosynostosis.

    The existence of an occipitoparietal cerebrospinal fluid shunt complicates the attempt to remodel posterior skull deformities in craniosynostosis. A composite method to reposition the shunt hardware is described and discussed.
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ranking = 1
keywords = skull
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2/96. Cloverleaf skull anomaly with extreme orbitostenosis.

    A neonate presented with cloverleaf skull anomaly and severe proptosis requiring urgent cranioplasty to attain eyelid closure. Despite this, she experienced exposure keratitis and corneal perforations. A ventriculoperitoneal shunt was performed subsequently to relieve hydrocephalus, but respiratory problems eventually led to her death at 6 months. This case highlights the complexity of the problems encountered in the cloverleaf skull anomaly, and a brief review discussing its management is included. Despite improvements in treatment of this condition, the overall prognosis remains poor.
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ranking = 1.2
keywords = skull
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3/96. cleidocranial dysplasia with neonatal death due to central nervous system injury in utero: case report and literature review.

    cleidocranial dysplasia (CCD), an uncommon disorder involving membranous bones, is rarely lethal in early life. The calvaria is defective and wormian bones are present. Abnormalities of the clavicles vary in severity from a minor unilateral defect to bilateral absence. This report concerns pre- and postmortem anatomical and radiological findings in a 15-day-old female neonate with CCD. Her postnatal course was characterized by seizures and recognition of hydrocephalus during the first day of life. The calvaria was hypoplastic with numerous wormian bones. A pseudofracture of the right clavicle was present. hydrocephalus was present in the brachycephalic brain which had a severely thinned cerebral cortex. hemosiderin in the ventricular lining and marked subependymal gliosis were interpreted as evidence of old intraventricular hemorrhage that had occurred in utero. A CCD-related condition, Yunis-Varon syndrome (YVS), is noted for early lethality and for developmental and secondary abnormalities of the central nervous system. The present case only partially matches the phenotype of YVS and might represent a part of a spectrum of phenotypic variants ranging from viable CCD to lethal YVS.
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ranking = 0.014570082724277
keywords = fracture
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4/96. Neonatal craniopharyngioma. Case report.

    The authors report a case of craniopharyngioma in a newborn infant. Marked hydrocephalus of the fetal head was recognized in the pelvimetric roentgenograms of the mother and massive suprasellar calcification was seen in the plain skull films.
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ranking = 0.2
keywords = skull
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5/96. Traumatic acute giant epidural hematoma in a hydrocephalic shunted child.

    Extradural hematoma (EDH) is considered to be a rare complication of head trauma in children, and represents a serious and urgent pathology from which complete recovery can be expected if specialized treatment is instituted in time. In this article, the authors report the potential danger to a hydrocephalic shunted child who was apparently asymptomatic at the time of hospital admission with a mild head injury and developed an EDH of venous origin. This child had a rapid (time interval from injury to decerebrate posture of about 2 h), atypical (remained asymptomatic most of the time until abruptly deterioration) and fatal course, stressing the importance of early diagnosis and rapid therapy in order to avoid the death of the patient. The authors discuss the role of the ventriculoperitoneal shunting system in the lack of clinical symptoms associated with the presence of a giant EDH and a rapid and fatal course, and stress the importance of computed tomographic (CT) scanning in these patients, even if they are asymptomatic. If a skull fracture is suspected, a CT scan must be performed without delay.
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ranking = 2.0106176356845
keywords = skull fracture, skull, fracture
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6/96. Complications of venous insufficiency after neurotologic-skull base surgery.

    OBJECTIVE: To characterize the incidence and complications resulting from venous insufficiency after neurotologic-skull base surgery. STUDY DESIGN: Retrospective case review of >3,500 cases. SETTING: Tertiary referral center, inpatient surgery. patients: Six patients: four with complications related to chronic venous insufficiency and two with complications related to acute venous insufficiency. INTERVENTION(S): Medical (steroids, acetazolamide, hyperventilation, mannitol) and surgical (lumboperitoneal shunt, optic nerve decompression, embolectomy) interventions were undertaken. MAIN OUTCOME MEASURE(S): Chronic venous insufficiency: nonobstructive hydrocephalus manifested by headache, disequilibrium, and papilledema with resultant visual loss. Acute venous insufficiency: acute nonobstructive hydrocephalus resulting in mental status abnormalities in the postoperative period. CONCLUSIONS: (1) incidence of 1.5 per 1,000 cases. (2) Acute and chronic forms with different pathogenesis. (3) Acute form presents postoperatively with change in consciousness and herniation, and may proceed to death. (4) Chronic form presents months or years postoperatively with headache, disequilibrium, and visual changes from papilledema. (5) Occurs almost solely in patients with preoperative abnormalities of the venous collecting system. (6) Causes mental status changes postoperatively.
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7/96. Yunis-Varon syndrome: evidence for a lysosomal storage disease.

    We present clinical and neuropathological findings in a female infant with Yunis-Varon syndrome (YVS) comprising absence of thumbs and halluces, aphalangia of fingers and toes, hypoplasia of clavicles, severely undermineralized skeleton (especially skull), microcephaly, and multiple nonskeletal anomalies. The patient also had a Dandy-Walker malformation, hydrocephalus, and hypertension, which were not reported previously in YVS. The infant excreted an abnormal unidentified oligosaccharide. The patient died at day 108 with severe neurological impairment. autopsy showed prominent intraneuronal inclusions with vacuolar degeneration, mainly in the thalamic, dentate nuclei, cerebellar cortex, and inferior olivary nuclei. No storage phenomena were observed in other tissues. These findings strongly suggest that a lysosomal storage disorder is involved in the pathogenesis of Yunis-Varon syndrome.
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ranking = 0.2
keywords = skull
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8/96. 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.
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ranking = 0.014570082724277
keywords = fracture
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9/96. Type III occipital condylar fracture presenting with hydrocephalus, vertebral artery injury and vasospasm: case report.

    Occipital condylar fractures (OCF) are rare and have a high mortality rate. We report a patient with OCF who presented with acute hydrocephalus and died from diffuse vasospasm secondary to vertebral artery injury. A 45-year-old man fell 20 feet from a deer stand and landed on his head. CT showed a type III OCF continuing to the anterior rim of the foramen magnum on the left, with a bone fragment pushing into the medulla, causing hydrocephalus. The patient was stabilized, and a four-vessel arteriogram showed diffuse vasospasm with complete occlusion of the left vertebral artery at the level of the OCF. To our knowledge, this is the first documented case of the conjunction of OCF, hydrocephalus, and vasospasm.
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ranking = 0.072850413621384
keywords = fracture
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10/96. 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.
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keywords = skull
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