Cases reported "Encephalocele"

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1/83. Anatomical and embryological considerations in the repair of a large vertex cephalocele. Case report.

    The case of a neonate with a large vertex cephalocele is presented. The anatomical features of this anomaly were evaluated by means of magnetic resonance imaging and magnetic resonance angiography. Fusion of the thalami, dysgenesis of the corpus callosum, and failure of adequate formation of the interhemispheric fissure were characteristics of the major cerebral anomalies associated with the cephalocele. The absence of a falx in the midline, a split configuration of the superior sagittal sinus, and a dysgenetic tentorium with a concomitant abnormal venous drainage pattern were found in association with a large dorsal cyst. Repair of the anomaly was undertaken on the 3rd postnatal day. A cerebrospinal fluid shunt was required to treat hydrocephalus on Day 30. The child is well at age 3 years, but with significant developmental delay. The pathogenesis of this vertex cephalocele relates to semilobar holoprosencephaly and dorsal cyst formation. In addition, a disturbance in the separation of the diencephalic portion of the neural tube from the surface ectoderm or skin during the final phases of neurulation had occurred to help create the large cephalocele. Detailed preoperative imaging studies and awareness of the embryology and anatomy of this lesion facilitated the repair of the cephalocele. The prognosis of the child is determined not only by the presence of hydrocephalus, but also by the number of associated major cerebral anomalies. Options for treatment are discussed.
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ranking = 1
keywords = operative
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2/83. Transcranial approach for transsphenoidal encephalocele: report of two cases.

    BACKGROUND: Whereas the transcranial approach has been regarded as the therapy of choice for transethmoidal encephalocele, its feasibility for transsphenoidal encephalocele has remained controversial, particularly in neonates and infants. CASE REPORT: Two cases of transsphenoidal encephalocele operated transcranially are presented. In the first case, this 6-year-old boy underwent a transpalatal operation with repair of a cleft palate in another hospital before admission. reoperation via the transcranial route was carried out because of postoperative recurrent meningitis. With partial resection of the anterior wall, the encephalocele could be separated from the underlying tissue, and the interspace was filled with the pericranial flap. He made an uneventful recovery and has been well for the past 3 years. The second was a 3-month-old baby with a large encephalocele filling the nasopharyngeal space. As the cleft palate was absent, the transcranial approach was employed. In this case, the herniated tissue was excised at the lowest level possible. Postoperatively, panhypopituitarism became manifest. Re-evaluation of the preoperative magnetic resonance imaging (MRI) disclosed a small mass far below the dorsum sellae, which turned out to be an anomalous pituitary gland on histologic examination. CONCLUSIONS: The transcranial approach is considered a valid alternative for the therapy of transsphenoidal encephalocele, particularly when the transpalatal approach is unfeasible. While the anterior wall of the herniated sac may be safely resected, the posterior wall should under no circumstances be sacrificed. The preoperative MRI is essential, as it may provide valuable information as to the location of vital structures within the herniated tissue.
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ranking = 4
keywords = operative
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3/83. Split-thickness calvarial grafts in young children.

    Sixteen patients (mean age, 1.9 years) underwent split-thickness calvarial bone grafting using a full-thickness craniotomy, high-speed drill, and osteotomes for bone harvesting. The minimal calvarial bone thickness for a successful graft was found to be 7 mm. All patients were diagnostically studied preoperatively with computed tomographic scans and skull films. The mean follow-up was 2.9 years (range, 0.75-7.9 years). All patients had successful graft take. There were no infections or mortalities. Continued graft growth was seen in all patients. There was one dural tear during harvesting with no cerebrospinal fluid leak or postoperative sequelae. This analysis indicates that split-thickness calvarial grafts can be safely and adequately performed in children as young as 13 months of age.
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ranking = 2
keywords = operative
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4/83. Meningoencephalic herniation into the middle ear.

    Meningoencephalic herniation into the middle ear (MHME) is a rare condition. It can result from ear surgery, infection, head trauma or can be spontaneous. diagnosis requires a high degree of clinical suspicion. The presentation may suggest the condition, but sometimes the intraoperative discovery of an occult meningoencephalic herniation may be a frightening situation. Treatment planning must avoid intra-cranial complications. Transmastoid (TM) and middle cranial fossa (MCF) are alternative or complementary approaches, determined by several factors, including the size and the site of the bony defect and the presence or absence of middle ear infection. Three case reports are presented and a review of the literature is performed, to explain some aspects related to MHME, including aetiopathogenesis, clinical presentation, histopathology, diagnosis and treatment.
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keywords = operative
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5/83. Cephaloceles and abnormal venous drainage.

    Seven cases of parietal cephalocele and three cases of occipital cephalocele associated with abnormal venous drainage were evaluated, and the mechanism of the development for venous system was discussed from the embryological viewpoint. In parietal cephaloceles the abnormally draining vein, which corresponds to the straight sinus, ascended along the interhemisphere apart from the tentorium and emptied into the superior sagittal sinus below the cephalocele. The superior sagittal sinus formed the confluence, which was positioned high up. Intraoperative findings revealed that cephalocele penetrated the superior sagittal sinus at the midline. In the case of occipital cephalocele, the straight sinus followed a postero-superior course, to drain into the confluence just above the neck of cephalocele. The results of our study suggest that the causation of abnormal venous drainage in the great vein of Galen, the straight sinus and the superior sagittal sinus may be secondary, through interaction with a developmentally pre-existing cephalocele.
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keywords = operative
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6/83. A rare case of upper airway obstruction in an infant caused by basal encephalocele complicating facial midline deformity.

    A four-month-old male infant with basal encephalocele of the transsphenoidal type presented with upper airway obstruction and facial midline deformity, including cleft lip, cleft palate, hypertelorism and exophthalmos. Basal encephalocele is a rare disease, and usually not detectable from the outside. In this case, initially the cause of an upper airway obstruction was considered to be posterior rhinostenosis, and posterior rhinoplasty with inferior nasal conchectomy was scheduled. However, in preoperative examination, computed tomography (CT) and magnetic resonance imaging (MRI) revealed a bony defect in the sphenoidal bone and a cystic mass in communication with cerebrospinal fluid, herniating into the nasal cavity through the bony defect. The mass was diagnosed as a transsphenoidal encephalocele, the scheduled operation cancelled, and tracheostomy performed for airway management. The possibility of basal encephalocele should be considered in the case of upper airway obstruction with facial midline deformity.
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ranking = 1
keywords = operative
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7/83. Transsphenoidal cephaloceles in adults. A report of two cases and review of the literature.

    Transsphenoidal cephalocele rarely occurs in adults. We describe two adult cases with transsphenoidal cephaloceles. The first case was a 53-year-old female who presented with spontaneous cerebrospinal fluid (CSF) rhinorrhea. Magnetic resonance (MR) imaging demonstrated a transsphenoidal meningocele. Surgical repair was attempted via the transsphenoidal route. Rhinorrhea recurred at one month and also two years later. Transsphenoidal surgical repairs were repeated. There has been no evidence of CSF leakage in the four years of follow-up after the last surgery. Transsphenoidal repair seems to be the most suitable approach in this case. The second case was a 26-year-old female with secondary amenorrhea, diabetes insipidus, bitemporal hemianopsia, and see-saw nystagmus. MR imaging demonstrated a transsphenoidal encephalocele with the optic chiasma and infundibular recesses descending into the meningocele and an agenesis of the corpus callosum. The first operation was performed via the transnasal route and the second by sublabial transmaxillary transsphenoidal approach six months later. Postoperative MR imaging revealed reduction of the encephalocele. There was neither worsening nor noticeable improvement in the neurological or endocrinological function. However, in this type, complete repair is often impossible, and non-radical surgery such as transsphenoidal repair may be indicated as most suitable. They should be selected with careful assessment referring to MR findings and clinical symptoms and signs.
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ranking = 1
keywords = operative
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8/83. Preservation of olfaction in anterior skull base surgery.

    OBJECTIVES/HYPOTHESIS: In selected unilateral tumors and defects of the anterior skull base, the preservation of contralateral olfaction is achievable through a localized subcranial approach without compromising surgical objectives of resection or repair. STUDY DESIGN: Description of a functional adaptation of anterior skull base surgical techniques through a retrospective patient series. methods: Nine patients underwent anterior skull base surgery for unilateral cribriform plate disease including four malignant and two benign tumors, two encephaloceles, and one iatrogenic cribriform injury with cerebrospinal rhinorrhea. All nine patients consented to a localized subcranial approach to the anterior skull base to preserve the contralateral olfactory nerves. In four patients with benign disease a portion of the ipsilateral nerves was additionally conserved. Postoperative olfaction was assessed objectively with a commercially available smell test. Indications, technique, results, and complications are reported and discussed. RESULTS: All patients had eradication of disease with preservation of functional olfaction CONCLUSIONS: Conservation of olfaction is possible in selected cases of anterior skull base surgery when the lesion is unilaterally confined.
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ranking = 1
keywords = operative
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9/83. Atretic cephalocele: contribution of magnetic resonance imaging in preoperative diagnosis.

    We describe a case of parietal cephalocele. MRI and MR venography were useful for accurate anatomical depiction. We speculate on developmental pathogenesis in the context of the literature available.
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ranking = 4
keywords = operative
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10/83. An occipital encephalocystocele involving both sides of the lateral ventricles.

    We reported a rare case of an occipital encephalocystocele linked by bilateral posterior horns of the lateral ventricles. The subject was a newborn girl with a large occipital cephalocele (6 x 9 cm in diameter) covered with alopetic skin. cerebrospinal fluid leakage was not observed. The fontanel was soft, but a characteristic flat forehead was noted. Neither neurological deficits nor cardiopulmonary dysfunctions were evident at birth. Magnetic resonance images revealed that the sac contained the occipital lobes and that the cavity was linked by bilateral posterior horns. Agenesis of the corpus callosum and dysgenesis of the falx cerebri were associated. Fifteen days after birth, the cephalocele was removed surgically. Cutting sections revealed that the cephalocele contained enlarged bilateral posterior horns and cerebral hemispheres. The postoperative course was uncomplicated by hydrocephalus or infection, and the patient was discharged without any neurological deficit 22 days after surgery. Her milestone was survival; and at present 12 months after surgery, she is alive with moderate developmental retardation.
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ranking = 1
keywords = operative
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