1/35. 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.- - - - - - - - - - ranking = 1keywords = tube (Clic here for more details about this article) |
2/35. An unusual case of cephalocele associated with lipoma of corpus callosum.Cephalocele is a part of the family of neural tube defects. Although the frequency has ranged from 1 per 2,400 to 1 per 12,500 live births, the true incidence has been hidden in stillborns, underreferral of massive defects and early pregnancy losses. It has been suggested that the size, the content of the sac and associated hydrocephalus were unfavorable factors for the prognosis. Cephaloceles may occur as isolated malformations or together with other anomalies, and associated abnormalities are present in up to 50% of the cases. We report an unusual case of encephalocele associated with lipoma of corpus callosum, and the features of magnetic resonance imaging are discussed.- - - - - - - - - - ranking = 1keywords = tube (Clic here for more details about this article) |
3/35. Spontaneous cerebrospinal fluid otorrhea from a tegmen defect: transmastoid repair with minicraniotomy.Spontaneous cerebrospinal fluid (CSF) otorrhea is a rare condition that presents in 2 clinical categories. In congenital labyrinthine malformations, it leads to bouts of meningitis in a hearing-impaired child. In the adult age group, a spontaneous CSF leak almost always results from a dural and bony defect in the tegmen area. Possible pathogenic mechanisms include progressive sagging and rupture of dura through a congenital tegmen dehiscence and progressive bone erosion by aberrant arachnoid granulations. These patients usually present with a middle ear effusion, resulting in clear discharge after myringotomy with tube insertion. Based on 4 patients with a CSF leak from a tegmen defect, this report reviews the clinical findings and diagnostic approach. The surgical management by a 5-layer closure using a transmastoid approach with minicraniotomy is outlined. This procedure offers a relatively simple and reliable method for repair without the inherent risks of a middle fossa craniotomy.- - - - - - - - - - ranking = 1keywords = tube (Clic here for more details about this article) |
4/35. Spontaneous cerebrospinal fluid leakage and middle ear encephalocele in seven patients.Isolated cases of spontaneous cerebrospinal fluid (CSF) leakage with and without middle ear encephalocele have been reported. These leaks are usually accompanied by episodes of recurrent meningitis, hearing loss, or chronic headache. In this article, we report seven new cases of spontaneous CSF leakage. Six of these patients had conductive hearing loss and serous otitis media, and three had recurrent meningitis. Prior to a definitive diagnosis, six patients had received myringotomy tubes, which produced profuse clear otorrhea. Three patients had positive beta-2 transferrin assays. Computed tomography and magnetic resonance imaging confirmed a defect in the temporal bone tegmen. A combined transmastoid and middle fossa surgical approach with a three-layer closure was used to repair the tegmen defect. All patients had a lumbar drain placed prior to surgery. In addition to describing the seven new cases, we review the history of CSF leakage and discuss diagnostic methods, surgical findings, and our recommendations for management.- - - - - - - - - - ranking = 1keywords = tube (Clic here for more details about this article) |
5/35. Extramedullary hematopoiesis within a frontoethmoidal encephalocele in a newborn with holoprosencephaly.We present the first report of extramedullary hematopoiesis (EMH) in an encephalocele. The patient was a new-born with semilobar holoprosencephaly, a frontoethmoidal encephalocele, and a large subdural hematoma. The encephalocele appeared as a hemorrhagic mass, protruding from the forehead to cover the right eye, without involvement of the sinuses or nasopharynx. Computerized tomography and magnetic resonance imaging studies ruled out other forms of holoprosencephaly and confirmed the continuity of the brain with the extruded mass. immunohistochemistry confirmed the presence of an atrophic epithelium covering the mass. Histologic examination of the encephalocele revealed EMH both within and adjacent to malformed cerebral cortex, with a tendency for the hematopoietic cells to line up in columns within malformed cerebral cortex. We propose that a single event during the fourth week of gestation could both interrupt closure of the neural tube, giving rise to the encephalocele, and impair migration of the neural crest, leading to holoprosencephaly secondary to failure of neural crest derivatives to induce basomedial telencephalic differentiation. EMH may have been induced from hematopoietic stem cells in the richly vascular meningeal component of the encephalocele, in response to anemia and hypoxia.- - - - - - - - - - ranking = 1keywords = tube (Clic here for more details about this article) |
6/35. Meckel Gruber syndrome--a single gene cause of recurrent neural tube defects.Meckel Gruber syndrome (MGS), an autosomal recessive disorder characterised by posterior encephalocoele, multicystic kidneys and post-axial polydactyly should be recognised by obstetricians and paediatricians to counsel parents regarding the 25% recurrence risk. We report a consanguineous family with MGS affecting three infants.- - - - - - - - - - ranking = 4keywords = tube (Clic here for more details about this article) |
7/35. Closed head injury complicated by nonketotic hyperglycemic hyperosmolar coma.A case of nonketotic hyperglycemic hyperosmolar coma occurring in a patient with closed head injury and basilar skull fracture is reported. The metabolic abnormality masqueraded as a progression of central transtentorial herniation due to cerebral edema. It is suggested that the development of this complication is related to various predisposing factors normally employed in cases of critically ill, neurologically compromised patients. The anatomic lesions found at autopsy were considered inappropriate as a cause of death; the occurrence of central transtentorial herniation three days before death had been reversed. An awareness of this syndrome is emphasized in considering patients receiving steroids, mannitol, tube feedings, Dilantin, and dehydrative measures as part of their care.- - - - - - - - - - ranking = 1keywords = tube (Clic here for more details about this article) |
8/35. Posterior fontanelle giant encephalocele.A 4-day-old baby was admitted with a large posterior fontanelle encephalocele. The baby was the third child of a consanguineous marriage. Two older siblings, 5 and 3 years old, were normal. The baby had a small head with a circumference of 30 cm only and an encephalocele with a circumference of 37 cm. The baby was active and there was no other neural tube defect or any other congenital anomalies. Noncontrast CT scan of the head with bone window showed a large posteriorly located encephalocele above the occipital bone, containing a small amount of brain tissue and a large volume of CSF. The baby was operated on in the lateral position and the encephalocele was excised. The dural defect was closed directly. The baby had an uneventful postoperative recovery. Twenty months later, the baby was well with no gross neurological deficits.- - - - - - - - - - ranking = 1keywords = tube (Clic here for more details about this article) |
9/35. Orbital compartment syndrome mimicking cerebral herniation in a 12-yr-old boy with severe traumatic asphyxia.OBJECTIVE: To report a case of orbital compartment syndrome mimicking cerebral herniation in a boy with severe traumatic asphyxia. DESIGN: Case report. SETTING: A tertiary-care pediatric intensive care unit. SUBJECT: A 12-yr-old boy with traumatic asphyxia syndrome. INTERVENTION: Mechanical ventilation, chest tube drainage, nitric oxide, lateral canthotomies, intracranial pressure monitoring. MEASUREMENTS AND MAIN RESULTS: A patient is presented with severe traumatic asphyxia syndrome complicated by prolonged hypoxemia, massive capillary leak syndrome, and acute onset of pupillary dilation and loss of reactivity to light. Ophthalmologic examination confirmed bilateral orbital compartment syndrome, which was treated emergently with bilateral canthotomies at the bedside. The procedure was followed by prompt return of pupillary size and function and decrease in intraocular pressure. The patient experienced complete recovery of vision in the right eye, but vision in the left eye was severely impaired. CONCLUSIONS: Our case report emphasizes the importance of considering orbital compartment syndrome in patients with traumatic asphyxia syndrome. Recognition of orbital compartment syndrome is important in this setting because prompt operative intervention may reduce the likelihood of permanent vision loss.- - - - - - - - - - ranking = 1keywords = tube (Clic here for more details about this article) |
10/35. diagnosis and management of spontaneous cerebrospinal fluid-middle ear effusion and otorrhea.OBJECTIVES/HYPOTHESIS: Spontaneous leak of cerebrospinal fluid (CSF) into the middle ear can occur in adults without a history of temporal bone trauma or fracture, meningitis, or any obvious cause. Therefore, clues may be lacking that would alert the otolaryngologist that fluid medial to an intact eardrum, or fluid emanating from an eardrum perforation, is likely to be CSF fluid. A review of relevant medical literature reveals that herniation of the arachnoid membrane through a tegmen defect may be congenital, or CSF leak may occur when dynamic factors (i.e., brain pulsations or increases in intracranial pressure) produce a rent in the arachnoid membrane. Because tegmen defects may be multiple rather than single, identifying only one defect may not be sufficient for achieving definitive repair. Data on nine cases of spontaneous CSF leak to the ear in adult patients from four medical centers are presented and analyzed to provide collective information about a disorder that can be difficult to diagnose and manage. STUDY DESIGN: Retrospective review of nine cases of spontaneous CSF middle ear effusion/otorrhea. RESULTS: The majority of patients presented with symptoms of aural fullness and middle ear effusion. Many developed suspicious clear otorrhea only after insertion of a tympanostomy tube. Two patients had multiple defects in the tegmen and dura, and five patients had meningoencephaloceles confirmed intraoperatively. Five patients underwent combined middle cranial fossa/transmastoid repair. Materials used in repair included temporalis fascia, free muscle graft, Oxycel cotton, calvarial bone, pericranium, bone wax, and fibrin glue. CONCLUSIONS: CSF middle ear effusion/otorrhea can develop in adults without a prior history of meningitis or head trauma or any apparent proximate cause. Although presenting symptoms can be subtle, early suspicion and confirmatory imaging aid in establishing the diagnosis. Because surgical repair by way of a mastoid approach alone can be inadequate if there are multiple tegmen defects, a middle fossa approach alone, or in combination with a transmastoid approach, should be considered in most cases.- - - - - - - - - - ranking = 1keywords = tube (Clic here for more details about this article) |
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