1/26. 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 = hypertelorism (Clic here for more details about this article) |
2/26. 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.- - - - - - - - - - ranking = 1keywords = hypertelorism (Clic here for more details about this article) |
3/26. 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 = 2keywords = hypertelorism (Clic here for more details about this article) |
4/26. Craniofacial surgery for leaking encephalocele in a newborn baby.A newborn baby with a leaking frontoethmoidal encephalocele is reported. The 1-day-old baby was referred to our center with a swelling on the nose leaking CSF. The child was the third baby of a consanguinous marriage. The baby had a swelling over the nose, 8 cm in diameter. The center of the swelling looked unhealthy, with a small raw area seeping CSF. CT scan showed a swelling containing tissue isodense with normal brain. The bone window and the 3D CT revealed a basal bone defect in front of the crista galii and an outer bone defect at the frontonasal junction over the nasion. The baby was operated on the 5th day after birth. One-stage repair of the encephalocele was performed, along with correction of hypertelorism and bone grafting over the forehead. The baby withstood the surgery well and was discharged from the hospital on the 10th postoperative day.- - - - - - - - - - ranking = 1keywords = hypertelorism (Clic here for more details about this article) |
5/26. Trans-sellar trans-sphenoidal encephaloceles: report of two cases.Trans-sphenoidal encephaloceles are rare entities. Only about 20 such cases have been reported in the world literature to date. Among these, the trans-sellar variety is rarest (five cases). We present two cases of trans-sellar trans-sphenoidal encephaloceles seen over the last 12 years. The first case was a 5 month old male infant presenting with nasal obstruction and a mass in the mouth. The second case was a 14 year old boy presenting with chronic nasal obstruction since early childhood. In the first case, the mass was coming to the mouth through the split palate. In the second case, the mass could be seen in the posterior half of the nose by rhinoscopy. The computed tomography scans including coronal and sagittal reconstructions had revealed trans-sellar trans-sphenoid encephaloceles in both the patients. The magnetic resonance imaging in the second case showed the cavity and the structures of the floor of the third ventricle forming the main fundal contents. The magnetic resonance angiography revealed dipping of the ectatic anterior cerebral arteries in the neck of the encephalocele sac in this case. Extracranial trans-palatal approach and the repair of encephalocele was done in the first case with the palatal reconstruction at the same sitting. In the second patient, a trans-nasal approach with bilateral maxillotomies was performed to repair the encephalocele, and the skull base defect was repaired by a split rib graft, which was fixed with mini-plate and screws posteriorly at the clivus and anteriorly at the anterior edge of the bony defect. The cleft lip and hypertelorism also were corrected at the same sitting. Our first case had CSF leak on the 10th postoperative day and developed fulminant meningitis, despite appropriate antibiotics, and succumbed. The second patient had a good postoperative recovery and a satisfactory follow up for 2 years.- - - - - - - - - - ranking = 1keywords = hypertelorism (Clic here for more details about this article) |
6/26. Craniofacial surgery for giant frontonasal encephalocele in a neonate.A 5-day-old neonate with a frontonasal encephalocele is reported. He was referred to our institute with a swelling on the glabella not associated with cerebrospinal fluid (CSF) leak. The baby was the first born of a non-consanguinous marriage. The baby had a swelling over the glabella, 7 cm in diameter. The swelling had healthy covering with a raw area at the centre without any CSF leak. A magnetic resonance imaging scan showed a soft tissue swelling containing tissue iso-intense to normal brain. The internal bony defect was at the junction of the frontal and ethmoid bones, in front of the crista galii, in the floor of the anterior cranial fossa. The baby was operated on the 11th day after birth. A one-stage repair of encephalocele was performed, along with correction of hypertelorism and reconstruction of the nasal bridge. The postoperative period was unremarkable. The baby was discharged from hospital on the 10th postoperative day.- - - - - - - - - - ranking = 1keywords = hypertelorism (Clic here for more details about this article) |
7/26. Frontoethmoidal encephalocele with ocular leakage of cerebrospinal fluid. Case report.The authors present the case of a 2-year-old boy who was born with a small bulging mass in the middle frontonasal area and hypertelorism. In the neonatal period he suffered from a continuous lacrimal secretion mistaken for recurrent conjunctivitis. Cranial computerized tomography scanning and magnetic resonance imaging revealed a frontoethmoidal encephalocele associated with an ocular leakage of cerebrospinal fluid. One-stage repair of the encephalocele, along with correction of hypertelorism and bone grafting of the forehead, was performed with good result. During a 2-year follow-up period no neurological deficits appeared.- - - - - - - - - - ranking = 2keywords = hypertelorism (Clic here for more details about this article) |
8/26. meningitis and midline facial deformity.A baby with unilateral cleft lip, midline cleft palate and hypertelorism developed meningitis in the first 48 h of life. Examination of the nasopharynx showed a soft tissue mass, which was confirmed as a basal encephalocele by computed tomography. There was also congenital hydrocephalus and the corpus callosum was absent. Surgical treatment included repair of the anterior basal skull defect, repair of the lip and palate, and ventriculo-peritoneal shunt. There is currently evidence of developmental delay and right-sided visual impairment due to Morning Glory syndrome. This case demonstrates that basal encephalocele should be considered in any baby with midline facial deformity who develops meningitis.- - - - - - - - - - ranking = 1keywords = hypertelorism (Clic here for more details about this article) |
9/26. Midline facial defects with hypertelorism and low-grade astrocytoma: a previously undescribed association.We report on a child with midline facial defects with hypertelorism (MFDH), median cleft lip, sphenoidal ventriculocele, partial agenesis of the corpus callosum, and low-grade astrocytoma in the cervicomedullary junction. This combination of findings has not been reported previously. Although this association might be casual, it demonstrates a relationship between disorders of frontonasal process and posterior fossae. It also suggests that individuals with MFDH might require a prospective follow-up with central nervous system magnetic resonance imaging.- - - - - - - - - - ranking = 5keywords = hypertelorism (Clic here for more details about this article) |
10/26. The Morning Glory syndrome associated with sphenoethmoidal encephalocele.A 10-month-old infant was referred to the ophthalmic department of the Royal victoria Hospital for assessment. pregnancy and delivery had been normal. At birth, cleft lip, cleft palate, hypertelorism and a soft tissue mass in the mouth were noted. At two days of age he developed meningitis, axial computerized tomography showed a sphenoethmoidal encephalocele, and agenesis of the corpus callosum. The basal encephalocele was surgically repaired. On examination in the ophthalmic department he was able to follow a light with the left eye. He perceived light with the right eye but did not follow. There was a manifest right divergent squint, measuring -10 degrees, and a right afferent nerve defect. Examination under anaesthetic was performed. The right fundus showed a Morning Glory syndrome. The disc was pink and deeply excavated, and surrounded by a ring of chorioretinal pigmentary disturbance. There was a central tuft of glial tissue. The left fundus was normal. The association of Morning Glory syndrome and basal encephalocele is rare; four previous cases have been reported. In all patients there were associated mid-facial congenital anomalies such as cleft lip and cleft palate. The presence of a basal encephalocele should be suspected if the Morning Glory syndrome occurs in association with mid-facial congenital anomalies.- - - - - - - - - - ranking = 1keywords = hypertelorism (Clic here for more details about this article) |
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