1/22. Dysgenesis of the internal carotid artery associated with transsphenoidal encephalocele: a neural crest syndrome?We describe two original cases of internal carotid artery dysgenesis associated with a malformative spectrum, which includes transsphenoidal encephalocele, optic nerve coloboma, hypopituitarism, and hypertelorism. Cephalic neural crest cells migrate to various regions in the head and neck where they contribute to the development of structures as diverse as the anterior skull base, the walls of the craniofacial arteries, the forebrain, and the face. Data suggest that the link between these rare malformations is abnormal neural crest development.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
2/22. Craniofacial reconstruction of a giant cystlike temporal encephalocele.A 4-year-old girl underwent craniofacial reconstruction for giant cystlike encephalocele deriving from the temporo-maxillary region and giving impression of the duplicated head. The case of temporal encephalocele in this report is especially unusual in the extent of encephalocele, the degree to which it had expanded the zygomatic arch, mandible, cranial vault, and the radiologically undetectable bony defect. The use of craniofacial principles in the resection and reconstruction of the temporal encephalocecle are described.- - - - - - - - - - ranking = 2keywords = craniofacial (Clic here for more details about this article) |
3/22. Surgical treatment of frontoethmoidal encephalocele: a case report.This study reports a patient affected by congenital frontoethmoidal encephalocele. The cause of this malformation is unknown. A preoperative selective diagnosis evaluation is necessary. The workup should include an accurate clinical examination associated with radiological study (two- and three-dimensional computed tomography, magnetic resonance imaging, etc). The aim of the surgical treatment is to restore the functional brain tissue in the cranial cavity, perform dural repair, correct bone lack and other associated facial malformations (hypertelorism, orbital dystopia, etc.). A multidisciplinary team approach is necessary to resolve the brain herniation and to correct splanchnocranium malformations frequently associated with encephalocele. Cranial flap with orbital osteotomies has been performed; this operation permits correction of the hypertelorism and of the orbital dystopia associated with this malformation. In bone reconstructions, miniplates have been used as fixation devices. In adults we generally use titanium, but resorbable devices are required in children because of growing tissues. A restoration of craniofacial malformations with good aesthetic and functional results is achieved with early surgery.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
4/22. Sincipital encephalocele mismanaged as a facial hemangioma.A 3-month-old male infant was found to have a protruding mass with irregular contour at the root of his nose since birth. The mass protruded more when he cried. It was diagnosed and treated as a hemangioma by laser surgery in another hospital. However, the lesion recurred 1 week later. Reviewing the previous computed tomographic study of the brain, it revealed that the protruding mass communicated with the brain. Sincipital encephalocele was diagnosed, and craniotomy for excising the encephalocele and repair of the dura were undertaken successfully. We report this patient with sincipital encephalocele that was misdiagnosed and treated as a vascular malformation on the face. Fortunately, there was no complication. The authors stress the importance of accurate diagnosis of a lesion near the craniofacial junction to avoid unnecessary surgery and possible catastrophes.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
5/22. Morning glory syndrome and basal encephalocele.BACKGROUND: Morning glory syndrome (MGS) is a congenital optic disc dysplasia often associated with craniofacial anomalies, especially basal encephalocele. Clinical presentations are varied and often occult. CASE REPORT: We describe a case of bilateral MGS associated with basal encephalocele that was detected by chance when treatment was sought for respiratory distress. CONCLUSIONS: MGS and basal encephalocele should always be suspected in cases of midline deficiencies, particularly when ophthalmic signs of strabismus or poor vision are present. CT and MRI should be performed to delineate the extent of the lesion and a complete hormone screening should be carried out to exclude pituitary deficiency. The pathogenesis of MGS and basal encephalocele are unknown; however, it is thought to occur during the 5th week of embryonic development.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
6/22. Chronic tonsillar herniation and Crouzon's syndrome.patients born with craniofacial syndromes such as Crouzon's syndrome will often develop hydrocephalus after their initial craniofacial reconstructive procedures. We have treated 10 patients with Crouzon's syndrome; 5 patients required a shunting procedure after cranial remodeling. Each of these 5 shunted patients later demonstrated chronic tonsillar herniation on magnetic resonance imaging studies. One of these patients exhibited signs of pseudotumor cerebri and 1 had a spastic quadriparesis. Of the 5 patients who did not require a shunt, none displayed chronic tonsillar herniation. Our evidence suggests that jugular foramen stenosis produces an increased cerebral venous turgor that leads to a cerebrospinal fluid absorption defect and hydrocephalus. After the hydrocephalus is treated the increased venous turgor remains and provides the driving force for the development of chronic tonsillar herniation.- - - - - - - - - - ranking = 2keywords = craniofacial (Clic here for more details about this article) |
7/22. Heminasal proboscis with associated microphthalmos and encephalocele.Heminasal proboscis is a rare congenital malformation that presents complex management issues when associated with other craniofacial abnormalities. A newborn male, known to have a facial mass on prenatal ultrasonography, was delivered by planned induction at 37 weeks' gestation. He was intubated immediately because of anticipated respiratory difficulty. He had a right-sided proboscis, pedicled on the right medial canthal region. Additional airway evaluation identified a piriform aperture and choanal stenosis. magnetic resonance imaging confirmed a hypoplastic orbit with aplastic optic nerve and a naso-orbito-ethmoidal encephalocele. Right upper and lower eyelid colobomas were present. The airway was initially stabilized with a tracheostomy. Beginning at the age of 10 weeks, he underwent several staged procedures to excise the encephalocele and to reconstruct the nose, orbit, eyelid, and lacrimal drainage system. The encephalocele was exposed via a bicoronal approach and frontal craniotomy. The dural defect was repaired with a pericranial flap. Nasal reconstruction provided an adequate nasal passage and tip after three staged procedures.Creation of a conjunctival flap prepared the right eye for later insertion of a prosthetic shell. The patient remained successfully decannulated 3 years after his last reconstructive procedure. Identification of all associated anomalies is important before initiating surgical repair. The presence of coexisting craniofacial abnormalities requires a multispecialty approach and coordination of surgical procedures.- - - - - - - - - - ranking = 2keywords = craniofacial (Clic here for more details about this article) |
8/22. Hereditary cranium bifidum persisting as enlarged parietal foramina (Catlin marks) on cephalometric radiographs.Cranium bifidum occultum is a rare skull ossification disorder referred to as the Catlin mark characterized by ossification defects in the parietal bones. Evidence suggests that this condition has a strong genetic heterogenicity. It is believed that, as calvarial growth continues, ossification in parietal bones fills these defects, and they can remain as parietal foramina on either side of the sagittal suture. During the conversion phase of cranium bifidum to the persistent parietal foramen, there will be periods when the brain is unprotected because of the delay in the ossification of the parietal bones. This report describes cranium bifidum occultum diagnosed as an incidental finding in a 14-year-old boy who initially had large bilateral unossified parietal bones and many congenital abnormalities. The patient underwent various surgical procedures over 6 years for the correction of cleft lip and palate. With craniofacial corrections and orthodontic treatment, the patient now has stable dentition and a firm palate with most of the parietal bones ossified. Cranioplasty was not recommended by his family physician after consultation with a neurosurgeon. Orthodontists should be familiar with this genetic abnormality because it causes delay in parietal bone ossification, and they should be able to distinguish between anatomic parietal foramina and enlarged parietal foramina (persistent unossified areas of cranium bifidum occultum), especially when craniofacial abnormalities are noticed.- - - - - - - - - - ranking = 2keywords = craniofacial (Clic here for more details about this article) |
9/22. A complex therapy for treatment of frontoethmoidal meningoencephalocele in a developing third world country: neurosurgical aspects.OBJECT: Meningoencephaloceles are congenital malformations that have a high incidence in the population of Southeast asia. Frontoethmoidal meningoencephaloceles, the most common variety, require surgical treatment. The authors combined neurosurgical and craniofacial approaches for the development of a simple technique that corrects this type of meningoencephalocele in a one-step procedure that has not been discussed in the literature previously. methods: In three visits of approximately 1 week each, 30 patients suffering from a frontoethmoidal meningoencephalocele underwent surgery successfully at the Rose charities Surgical rehabilitation Center, Kien Khleang, Phnom Penh, cambodia. To the authors' knowledge, this is the first reported series of operations in this geographical region to treat meningoencephaloceles at a relatively primitive surgical center. Difficulties faced in this series included tropical conditions, problems ensuring sterility, and limited technical support. CONCLUSIONS: The authors present the neurosurgical highlights and the outcomes in this series of patients. The single approach, via a bicoronal skin incision and small frontobasal trepanation, facilitates closure of the frontal skull defect and resection of the meningoencephalocele (including its extension into the facial area), as well as a satisfactory, one-step correction of the nasal skeleton and telecanthus.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
10/22. Craniofacial clefting and sutural dystopia.Sutural anomalies in conjunction with craniofacial clefting are unusual. A case of median frontal clefting is presented in which there was an absence of a normal metopic suture and replacement by paramedian frontal sutures. The association of an underlying brain anomaly, with attendant surgical difficulties, is noted, as are the radiological techniques of preoperative diagnosis.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
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