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At least 1,333 records · Page 74Linked to original sources
PubMed2005

Transethmoidal intranasal meningoencephalocele in an adult with recurrent meningitis.

Intranasal meningoencephalocele is a rarely encountered congenital malformation. We report a case of transethmoidal intranasal meningoencephalocele in a 52-year old man with recurrent purulent meningitis. After treatment of the acute meningitis, frontal craniotomy followed by the removal of the stalk of the meningoencephalocele and repair of the bony defect was successfully performed. He has had no further meningitis or CSF rhinorrhea post-operatively. Detailed neuroradiological examination and appropriate surgical treatment are important to prevent fatal neurological complications of intranasal meningoencephalocele.

Cerebral Angiography↗
PubMed2005

Adult intranasal meningoencephalocele presenting as a nasal polyp.

We present an isolated purely intranasal meningoencephalocele, presenting as a nasal polyp in an adult, which is uncommon. Based on a review of the literature, we emphasize that nasal obstruction may be the only presenting symptom of an intranasal meningoencephalocele.

Adult↗
PubMed2004

Joubert-like syndrome unlinked to known candidate loci.

We observed the Joubert syndrome (JS) associated with bilateral morning glory disk anomaly and cystic dysplastic kidneys in three patients from a consanguineous kindred. Homozygosity mapping excluded three JS candidate loci as sites harboring the disease gene. We thus delineate an autosomal recessive disorder, distinct from JS and related conditions.

Adult↗
PubMed2004

Currarino syndrome: proposal of a diagnostic and therapeutic protocol.

BACKGROUND/PURPOSE: The Currarino syndrome (CS) is a peculiar form of caudal regression syndrome (CRS) characterized by the association of hemisacrum, anorectal malformation (ARM), and presacral mass. The authors analyzed retrospectively their series, and they propose a multidisciplinary diagnostic and therapuetic protocol that until now has not been introduced. METHODS: A series of 6 patients with CS is presented. Five of them were treated initially in other centers. None of them had an early diagnosis. All presented associated anomalies; in 50%, Hirschsprung's disease (HD) and other dysganglionoses were present. One patient died of a presacral ectopic nephroblastoma. RESULTS: Depending on the expressivity, 3 types of CS can be identified, complete, mild, and minimal. Dysganglionoses and HD can be considered part of CS. A multidisciplinary diagnostic and therapeutic protocol is presented. Main points are sacrum x-Ray, molecular genetic diagnosis, radiologic evaluation of every member of CS families, magnetic resonance (MR) evaluation of patient spine and pelvis, suction rectal biopsies, and search for associated anomalies. CONCLUSIONS: This protocol could give a valid contribution to the treatment of CS, allowing an early diagnosis and proposing a rational timing of multidisciplinary surgical procedures. Early diagnosis and treatment are essential to avoid morbidity and mortality from an undiagnosed presacral mass.

Abnormalities, Multiple↗
PubMed2004

Three new cases of disorganizationlike syndrome: one with accessory extrophia vesicalis.

The authors report 3 unrelated Turkish cases of disorganizationlike syndrome. All of these patients have accessory limbs, and 2 of them have accessory genitourinary structures. Interestingly, one of these patients has extrophia vesicalis of accessory bladder. None of them have chromosomal abnormality. Here the authors present distribution of findings of these cases.

Abnormalities, Multiple↗
PubMed2004

Orbital varices, cranial defects, and encephaloceles: an unrecognized association.

PURPOSE: To highlight an apparently unreported association between orbital varices and malformations of the cranial skeleton with or without abnormalities of the underlying cerebral parenchyma. DESIGN: Retrospective, noncomparative review of case notes and radiologic imaging. PARTICIPANTS: Three hundred ten patients attending the Orbital Clinic at Moorfields Eye Hospital, London, with a diagnosis of low-pressure, low-flow orbital venous anomalies. METHODS: All available orbital imaging for patients with orbital venous anomalies was examined. For those with defects of the cranial base, the radiologic characteristics were noted and the clinical details were reviewed. MAIN OUTCOME MEASURES: The presence and type of orbital roof or medial wall defects and associated nasal or other cranial anomalies. RESULTS: Imaging was adequate for review in 222 of 310 patients (72%), and anomalies of the neighboring cranium or cerebral structure were found in 10 of 222 patients (4.5%). In the group with cranial anomalies, the proportion of men (7/10; 70%) did not significantly differ from that in the group with varices but without cranial anomalies (93/212; 44%; P = 0.19, Fisher exact test). Orbital varices were associated with 3 types of cranial anomaly: major midline encephaloceles (type I anomaly; 4 cases), large superomedial defects of the orbital wall (type II; 3 cases), or defects of the greater wing of the sphenoid (type III; 3 cases). CONCLUSIONS: Clinicians should be aware of the possibility of significant cranio-orbital or cranionasal anomalies in patients with orbital venous anomalies; these anomalies can vary from minor defects in the cranial base to large encephalomeningoceles. This rare association should not be overlooked when orbital varices become markedly inflamed, because intranasal encephaloceles are a known predisposition to recurrent meningitis.

Adult↗
PubMed2004

Management of pseudomeningocele following neurotologic procedures.

OBJECTIVES: A pseudomeningocele results from the escape of cerebrospinal fluid through a dural defect with trapping of subarachnoid fluid in surrounding soft tissue. The present study evaluates the incidence of pseudomeningocele following neurotologic procedures and delineates an algorithm for management. METHODS: A retrospective review of 375 consecutive patients undergoing neurotologic procedures at a single institution identified 17 patients with the postoperative complication of pseudomeningocele. RESULTS: The incidence of pseudomeningocele formation was 4.5% in the present study. Fourteen pseudomeningoceles resolved with nonoperative management including pressure dressing, bed rest, and lumbar spinal drainage. Three patients failed nonoperative management and required surgical procedures for resolution. All patients ultimately had resolution of their pseudomeningocele. CONCLUSION: Skull-base pseudomeningoceles occur as a complication following neurotologic procedures and can cause complications as they enlarge. The majority of these cases can be dealt with in a nonsurgical manner, but those failing to respond to conservative management should be considered for surgical intervention.

Adult↗
PubMed2005

Endoscopic transpterygoid approach to the lateral sphenoid recess: surgical approach and clinical experience.

OBJECTIVE: Pathology within a lateral recess of a widely pneumatized sphenoid sinus is difficult to access with the use of traditional open and current endoscopic surgical approaches. A new surgical procedure, the endoscopic transpterygoid approach, directly accesses this region. A clinical experience over several year with this approach is reported as well as a refined and updated description of the technique. SETTING: Tertiary care center. METHODS: Retrospective review of medical records of patients treated with the endoscopic transpterygoid approach to the lateral sphenoid recess. RESULTS: An endoscopic transpterygoid approach was performed in 9 patients, 6 to resect a middle fossa meningoencephalocele and repair the CSF leak and associated skull base defect, 2 for possible invasive fungal sinusitis, and 1 to marsupialize a symptomatic epidermoid cyst. Patients tolerated the approach well and no significant complications occurred. CONCLUSIONS AND SIGNIFICANCE: In selected cases, the endoscopic transpterygoid approach enables the otolaryngologist to meet modern demands to treat conditions in the lateral sphenoid using minimally invasive techniques that are well-tolerated by patients. The surgical approach and the initial experience described herein is presented in the hopes that it can aid fellow otolaryngologists to help patients with challenging conditions in this region of the body.

Cerebrospinal Fluid Rhinorrhea↗
PubMed2005

The relationship between clinical signs and brain herniation associated with rostrotentorial mass lesions in the dog.

Retrospective analysis of magnetic resonance imaging and clinical findings in a referral population of dogs was used to determine the relationship between rostrotentorial space-occupying lesions and the development of secondary neurological signs. Brain herniation was detected in 54/153 cases of uni-focal rostrotentorial space-occupying lesions; of these 30 had caudal transtentorial herniation (CTH) and 24 had both transtentorial and foramen magnum herniation (CTH/FMH). Masses associated with herniation were larger and situated more dorsally and caudally within the cranial vault. Clinical signs classically associated with CTH, e.g., oculomotor nerve palsy, were seen in only one case of isolated CTH and seven (14%) of all herniation cases. Deficits in caudal cranial nerve function were detectable in 39% of cases with FMH. We conclude that severe shifts in brain parenchyma can exist in the absence of detectable localising signs.

Animals↗
PubMed2001

[Post-surgical and post-infectious meningoencephalic herniation in the middle ear].

Meningoencephalic hernia of the middle ear, due to the advent of antibiotics, the surgical microscope, and the new surgical techniques for the treatment of chronic otitis media, is an infrequent pathology in the presents days. The goal of this paper is to present four cases of meningoencephalic hernia of the middle ear, three of then postsurgical and the remaining of inflammatory origin. Diagnosis, treatment and complications are discussed. Meningoencephalic hernia of the middle ear is usually asymptomatic and diagnosis is accidental. Immediate surgical treatment is indicated in order to avoid the potential complications of this disease. Transmastoid approach has been used for three cases and a middle cranial fossa approach was used in the fourth patient.

Adult↗
PubMedDate not supplied

[Spontaneous middle ear meningoencephalic herniation. 2 case reports].

Two cases of idiopathic brain herniation into the middle ear without previous surgery are reported. Brain herniation is an unusual pathology, usually related to middle ear surgery. The idiopathic appearance of this disease is the secondly most frequent cause. Its origin is believed to be related to a congenital defect of tegmen timpani. Two elements, both rare, are emphazised: the spontaneous bilateral presentation in one of the cases, and the CSF leak as an initial symptom. We present a review of the literature, describing epidemiology results and the main accepted etiology theories. We discuss the diagnosis and treatment methods: magnetic resonance imaging and surgery.

Aged↗
PubMed1993

Orbital meningoencephalocele manifesting as a conjunctival mass.

Orbital meningoencephalocele is a rare congenital abnormality caused by a defect of the cranio-orbital bones that usually manifests soon after birth as a soft, cystic fullness in the superomedial canthal area with associated exophthalmos. We managed an unusual case of congenital orbital meningoencephalocele that manifested as a cystic conjunctival mass without proptosis or periorbital changes. Preoperative computed tomographic scans failed to demonstrate a bone defect. After suture ligature of the posterior stalk, excision of the lesion yielded an ependymal cyst surrounded by neuroglial and meningeal tissue and filled with cerebrospinal fluid. The patient had normal results of ophthalmic and neurologic examinations after transconjunctival resection of the lesion after three years of follow-up. An orbital approach may be appropriate for a few selected cases in which no bone defect is found on computed tomography. Orbital meningoencephalocele should be included in the differential diagnosis of isolated congenital conjunctival cystic masses.

Conjunctival Diseases↗
PubMed1999

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.

Abnormalities, Multiple↗
PubMed2003

Massive plexiform neurofibroma with associated meningo-encephalocoele and occipital bone defect presenting as a cervical mass.

The commonest skull manifestations in neurofibromatosis involve the orbit, with very few reports about occipital defects. We report a rare case of a 54-year-old lady with a massive plexiform neurofibroma extending from the auricular region down her left neck and into her shoulder, with an associated large left occipital and left petrous bone defect and extensive cerebellar meningo-encephalocoele, which presented with a relatively asymptomatic cervical mass and was treated with resection of the neurofibroma and advancement and rotational skin flaps.

Encephalocele↗