1/632. 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. ( info) |
2/632. Cranio-cerebral erosion: delayed diagnosis and treatment. Cranio-cerebral erosion is a well-known complication of calvarian fracture with underlying dural tear and cerebral injury in infancy and early childhood. The anatomy, pathogenesis and natural evolution of these lesions remain obscure. The common clinical symptoms are seizures, focal neurological deficits, impairment of consciousness and a soft subgaleal mass. Three patients of cranio-cerebral erosion who underwent delayed surgery in their adult lives are presented to illustrate the common and uncommon features, and their long-term outcome is discussed. ( info) |
3/632. 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. ( info) |
4/632. Ocular malformations, moyamoya disease, and midline cranial defects: a distinct syndrome. PURPOSE: To report a 10-year-old girl with developmental anomalies of both optic disks, a chorioretinal coloboma, sphenopharyngeal meningoencephalocele, and moyamoya disease. methods: A full ophthalmologic examination, cranial magnetic resonance imaging and magnetic resonance angiography, and cerebral angiography were performed. RESULTS: The patient had a morning glory disk anomaly and microphthalmos of the right eye and optic nerve hypoplasia and retinochoroidal coloboma in the left eye. She had a midfacial cleft and an episode of seizures and a stroke. magnetic resonance imaging showed a sphenopharyngeal meningoencephalocele. magnetic resonance angiography and cerebral angiography demonstrated a pattern consistent with moyamoya disease. CONCLUSIONS: This patient had a distinct syndrome of optic disk, retinochoroidal, and carotid circulation anomalies with midline cranial defects. The recognition and treatment of the vascular abnormalities and cranial defects may prevent complications such as strokes that may occur during or after general anesthesia. ( info) |
5/632. 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. ( info) |
6/632. 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. ( info) |
7/632. Localized vitiligo and frontoethmoidal meningoencephalocele. A child with frontoethmoidal meningoencephalocele in whom localized facial vitiligo developed after surgical correction of the encephalocele is presented. The potential role of the ocular disease accompanying the encephalocele in the development of the hypopigmentation is discussed. ( info) |
8/632. Idiopathic temporal encephalocele: report of two cases. BACKGROUND: Idiopathic brain herniation into the middle ear is a rare condition that represents diagnostic and therapeutic challenges. OBJECTIVE: The authors present here two new cases of idiopathic brain herniation with special clinical presentation and emphasis on radiographic studies, particularly computed tomographic scan and magnetic resonance imaging, which allowed the authors to detect the malformation. RESULTS: The two patients underwent surgical treatment with infratemporal approach and recovered perfectly. ( info) |
9/632. 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. ( info) |
10/632. Craniofacial correction of giant frontoethmoidal encephalomeningocele. The surgical treatment of a very large anterior encephalocele in an infant is presented. Because of the large size of the encephalocele, a combined transfacial-transcranial approach was used for correction of the associated intracranial, cranioorbitonasal bone, and facial skin deformities. ( info) |