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At least 1,045 records · Page 58Linked to original sources
PubMed1988

Parietal cephalocele: clinical importance of its atretic form and associated malformations.

In this study of atretic cephaloceles, the authors have considered the pedunculated or sessile type of cephalocele and also small nonpedunculated scalp defects developing in the vertex midline. Parietal cephaloceles were found in 15 infants (10 boys and five girls), and accounted for 37.5% of all cephaloceles. They consisted of four encephaloceles, six meningoceles, and five atretic cephaloceles. The clinical and morphological characteristics of parietal cephaloceles were investigated and compared with those arising at other locations. Parietal cephaloceles carried a much less favorable prognosis than those in the occipital region, regardless of the type of cephalocele; they were associated with cerebral malformations more frequently and were more severe than occipital cephaloceles. Grave congenital anomalies were found in 87% of patients with parietal cephalocele, and only two patients (neither of whom had any other malformation) attained normal development. Brain malformations were closely related to the site from which the cephalocele issued, and dorsal cyst malformation was found in eight patients with parietal cephalocele. Two types of atretic cephaloceles were found, each in a different location. The first type was an alopecic lesion occurring in the parietal midline; all five patients with this type had dorsal cyst malformations and none developed normally. The second type was a nodular lesion developing at the occipital midline, not associated with cerebral anomalies; all five patients with this type showed normal development. The pathogenesis of atretic cephaloceles and their associated intracranial malformations are discussed.

Alopecia↗
PubMed1992

Bilateral morning glory syndrome associated with sphenoid encephalocele. Case report.

Morning glory syndrome is a congenital anomaly of the optic disc in which the disc is enlarged and excavated, with white glial tissue in the center. A case is presented of morning glory syndrome associated with sphenoid encephalocele, median cleft lip, and agenesis of the corpus callosum. A 22-day-old boy was referred to the Wakayama Medical College Hospital for management of dyspnea due to a soft-tissue mass in the oral cavity. Magnetic resonance imaging revealed a mass extending through a bone defect in the sphenoid region and into the oral cavity. Surgical repair was attempted through a bifrontal craniotomy. A bone defect was identified in the sphenoid plate, through which the arachnoid membrane was connected to the oral cavity. Both optic nerves were elongated and adhered to the encephalocele. The wall of the meningocele was compressed digitally through the oral cavity and sutured to the dura mater of the bone defect. The operative findings suggest that a basal encephalocele protruding from a bone defect in the sphenoid plate may disturb the normal development of the optic nerve.

Abnormalities, Multiple↗
PubMed1994

Terminal syringohydromyelia and occult spinal dysraphism.

Terminal syringohydromyelia is a cystic dilatation of the lower third of the spinal cord. The authors describe its incidence and characteristics, its frequent association with occult spinal dysraphism, and its clinical significance and need for surgical treatment. All 143 cases of occult spinal dysraphism treated at the Duke University Medical Center between 1972 and 1992 were reviewed. A terminal syrinx was found in 24 (27%) of the 90 cases that were evaluated by magnetic resonance (MR) imaging. In contrast three (6.2%) of 48 cases evaluated by myelography and postmyelographic computerized tomography had a syrinx documented. The relative radiographic severity of the different syringes was estimated by using measurements of the syrinx and spinal cord on the MR images, classifying the cysts into large and small. Large syringes were frequently symptomatic, commonly presenting with pain, motor and sensory deficits of the lower extremities, scoliosis, and bowel and bladder dysfunction. Terminal syringohydromyelia with occult spinal dysraphic lesions was most often associated with tethered spinal cord from a tight filum terminale in the presence of an anorectal anomaly (67% of cases), meningocele manqué (54%), and diastematomyelia (38%). An infrequent association was seen with other spinal cord anomalies. The results of surgical management of terminal syringohydromyelia were analyzed, highlighting the necessity and effectiveness of shunting the large cysts, especially in the setting of a progressive symptomatology. Of the 11 patients with shunts who underwent MR imaging, 10 showed either complete or significant resolution of the syrinx; all five patients who had presented with pain (mainly back pain) showed complete resolution of the pain after shunting; finally, one-third of patients with shunt placement had significant postoperative improvement in their neurological examination, whereas none worsened. It is stressed that terminal syringohydromyelia is an important pathological entity that should be considered in patients with occult spinal dysraphism, and treated surgically when clinically or radiographically significant.

Adolescent↗
PubMed1996

Management and outcome of posttraumatic syringomyelia.

Traumatic paraplegia is the most common cause of nonhindbrain-related syringomyelia. Fifty-seven patients with a mean age of 34.3 years at presentation were treated at the Midland Centre for Neurosurgery and Neurology between 1973 and 1993. A variety of treatment strategies have been used over the years, including syringosubarachnoid and syringopleural shunts, spinal cord transection, and pedicled omental graft transposition. More recently decompressive laminectomy, subarachnoid space reconstruction and formation of surgical meningocele have been used. A total of 81 operations were performed in these patients, 69 of them at the Syringomyelia Clinic. Combinations of strategies were often chosen; the use of one strategy such as drainage did not preclude another such as transection or augmentation of the cerebrospinal fluid pathways. The overall postoperative complication rate was 12%. Problems specific to the operation type included dislodged, blocked, and infected drains (10 patients). Acute gastric dilation was seen following pedicled omental graft (one patient). At 6 years only 49% of the drains inserted still functioned. A higher than expected rate of cervical spondylotic myelopathy has been noted. Two patients developed Charcot's joints. Thirty-six patients were asked to score themselves with regard to limb function and performance of daily living activities and 30% reported improvement, particularly ion arm function. Since the use of magnetic resonance imaging has become widespread, it has become apparent that decompressive laminectomy with subarachnoid space reconstruction is effective in controlling the syrinx cavity. In complete paraplegia, spinal cord transection is an effective alternative. Pedicled omental grafting was associated with poor outcome and an increased complication rate and has been abandoned.

Activities of Daily Living↗
PubMed1997

Transpalatal approach for the extracranial surgical repair of transsphenoidal cephaloceles in children.

The surgical treatment of transsphenoidal cephaloceles in children is controversial. Reduction and repair via a transcranial approach are associated with high postoperative rates of morbidity, mortality, and hypothalamic dysfunction. In this study, four patients, aged 3 to 35 months at surgery, underwent successful transpalatal repair of two encephaloceles and two meningoceles. Two patients presented with nasal obstruction in infancy, one presented with unexplained meningitis, and in one patient the lesion was found incidentally during evaluation for seizures. Two children had median cleft face syndrome, another had an associated Arnold-Chiari type I malformation, and the fourth had no other cranial abnormalities. All patients underwent preoperative evaluation including magnetic resonance (MR) imaging. Auditory, ophthalmological, genetic, endocrinological, or other evaluation was undertaken as indicated. Lesions were approached through the median raphe of the hard and soft palates. All cephaloceles were easily visualized and dissected after division of the nasal palatal mucosa. The dural sac and its contents were reduced by surface coagulation after division and dissection of the overlying mucosa. Once reduced, the bone defect was obliterated in three of four patients. The dura was not opened and anomalous neural elements were not resected. At follow-up evaluation, all patients demonstrated resolution of preoperative symptoms without evidence of infection or lasting morbidity. Follow-up MR imaging showed reduction in all cases. The authors conclude that this transpalatal approach is safe and reliable for the treatment of transsphenoidal cephaloceles in young children.

Child, Preschool↗
PubMed2000

Use of titanium mesh for reconstruction of large anterior cranial base defects.

The authors evaluated the role of titanium mesh used in combination with vascularized pericranium to provide rigid support during reconstruction of anterior skull base defects. Thirteen patients with large anterior skull base defects caused by tumor invasion or traumatic injury involving the cribriform plate, orbital roof, and planum sphenoidale were included in the study. The reconstruction technique involved placement of titanium mesh between two layers of continuous vascularized pericranium. Surgical glue and routine lumbar cerebrospinal fluid (CSF) drainage were not used in any patient. At a mean postoperative follow-up time of 22 months (range 8-39 months), none of the patients had developed infection or meningocele. Postoperative CSF rhinorrhea occurred in two patients with extensive dural defects, which resolved with temporary lumbar drainage. Use of titanium mesh and a two-layer vascularized pericranial graft is a safe, reproducible, and feasible method for reconstructing the anterior skull base. Patients with large dural defects may need temporary CSF diversion to avoid postoperative fistula formation.

Adult↗
PubMed2002

Cervical nerve root avulsion in brachial plexus injuries: magnetic resonance imaging classification and comparison with myelography and computerized tomography myelography.

OBJECT: The authors describe a new magnetic resonance (MR) imaging technique to demonstrate the status of the cervical nerve roots involved in brachial plexus injury. They discuss the accuracy and reproducibility of a MR imaging-derived classification for diagnosis of nerve root avulsion compared with those of myelography combined with computerized tomography (CT) myelography. METHODS: The overlapping coronal-oblique slice MR imaging procedure was performed in 35 patients with traumatic brachial plexus injury and 10 healthy individuals. The results were retrospectively evaluated and classified into four major categories (normal rootlet, rootlet injuries, avulsion, and meningocele) after confirming the diagnosis by surgical exploration with or without spinal evoked potential (EP) measurements and by referring to myelography and CT myelography findings. The reliability and reproducibility of the MR imaging-based classification was prospectively assessed by eight independent observers, and its diagnostic accuracy was compared with that of traditional myelography/CT myelography classification, correlated with surgical and spinal EP findings in another 50 cervical roots in 10 patients with traumatic brachial plexus injury. CONCLUSIONS: In the retrospective study in which MR imaging and myelography/CT myelography findings involving 175 cervical roots in 35 patients were compared, the sensitivity of detection of the cervical nerve root avulsion was the same (92.9%) with both modalities. In the prospective study, interobserver reliability and intraobserver reproducibility showed that there was no statistically significant difference between MR imaging and myelography/CT myelography and that their accuracy for detecting cervical root avulsion was the same as that in the retrospective study. The overlapping coronal-oblique slice MR imaging technique is a reliable and reproducible method for detecting nerve root avulsion. The information provided by this modality enabled the authors to assess the roots of the brachial plexus and provided valuable data for helping to decide whether to proceed with exploration, nerve repair, primary reconstruction, or other imaging modalities.

Adult↗
PubMed2005

Diagnosis-related pitfall of a lateral sacral cyst. Case report.

Because physical examination typically demonstrates normal findings in cases of low-back pain, diaglosis of the cause can be challenging. Frequent magnetic resonance imaging studies of the lumbosacral spine can typically lead to discovery of benign diseases and thus misinterpretation of these images. The authors report an unusual case in which a functional ovarian cyst was incidentally associated with a perineural cyst and mimicked a lateral sacral meningocele. In light of this, the authors recommend repeated examinations to avoid mistakes.

Adolescent↗
PubMed1991

Neurosurgical application of fibrin adhesive.

We have tried fibrin adhesive, which mimics the end stage of plasmatic coagulation, in 26 patients with various neurosurgical problems such as: repair of cerebrospinal fluid (CSF) leaks, sealing of the vascular anastomosis sites, reinforcements of aneurysmal clippings, and hemostasis after resection of brain tumors. Presented in this report are 11 intracranial aneurysms, 11 brain tumors, 2 lipomyelo-meningoceles, and one each of cerebral arteriovenous malformation and torn dura resulting from a mastoidectomy. Procedures which seemed to be impossible or very difficult by conventional neurosurgical techniques could be accomplished in all cases without any complication. Our experience with fibrin adhesive suggests that it is a valuable adjuvant to various microneurosurgical procedures, and it may be potentially useful for protection of major cerebral veins and venous sinuses during cerebral retraction.

Adolescent↗
PubMed1999

Vertex hemangioma mimicking an encephalocele.

Most extracranial masses detected prenatally by use of ultrasonography are either encephaloceles or meningoceles. This article describes an extracranial mass detected prenatally that was initially thought to be an encephalocele, but was subsequently found to be a large hemangioma.

Adolescent↗
PubMed1990

Screening spinal ultrasound in newborns with neural tube defects.

Twenty-six newborns with spina bifida had complete spinal ultrasound (US) prior to initial surgical repair of the neural tube defects. Associated anomalies were found on screening US in seven infants and included diastematomyelia (two cases), hydromyelia (two cases), and dural fat deposits (three cases). Initial surgical therapy was altered in one infant with diastematomyelia. Four closed simple meningoceles showed no internal neural elements on US and none were found at surgery.

Humans↗
PubMed1999

[Chiari malformation (type I) associated with bilateral abducens nerve palsy: case report].

There are still many questions concerning the pathology and therapeutic strategy for Chiari malformation. We recently encountered a case of Chiari malformation associated with only bilateral abducens nerve palsy which was successfully treated by foramen magnum decompression. The pathology and mechanisms of Chiari malformation (type I) were evaluated in this case. The patient was a 12-year-old girl who had visited our institute 1 month previously complaining of diplopia. She had no particular past or family history and her general appearance was also normal. Bilateral abducens nerve palsy was the only neurological deficit. Her cerebrospinal pressure was 9 cm H2O. Neuroradiological findings revealed basilar impression, C4-6 syringomyelia and Chiari malformation (type I), but no hydrocephalus or meningocele. Foramen magnum decompression with lateral extension, upper cervical laminectomy and open duraplasty were performed. The position of both eyes recovered fully shortly after surgery, and diplopia occurred only when the patient looked sideways. Eye movements also returned completely to normal 3 months after surgery. MRI showed no syringomyelia 4 months after surgery, the upward shift of the pendulous portion of the cerebellar tonsil, and the expansion of the subarachnoidal space of the pons-oblongata junction. In conclusion, it was thought that the mechanism of bilateral abducens nerve palsy in this case was due to the strangulation and the downward traction of the pons-oblongata junction. The nerve was relieved from this traction by foramen magnum decompression.

Abducens Nerve Diseases↗
PubMedDate not supplied

H-type rectovaginal fistula associated with the Currarino triad.

We describe a case of H-type rectovaginal fistula associated with the Currarino triad (anorectal stenosis, sacral defect, presacral mass). Presenting symptoms included passage of feces per vaginam, signs of intestinal subocclusion without perianal inflammation, left leg paresis and foul-smelling urine. An anterior sacral meningocele was repaired at the age of three months. At age 18 months the fistula was excised through a perineal approach after creation of a protective colostomy. Diagnostic and therapeutic aspects of this malformation are discussed.

Abnormalities, Multiple↗
PubMed1975

[First experiences with the incontinence operation Kaufman III (implantation of a silicongel prothesis covered with polyurethanfoam) (author's transl)].

For operative treatment of urinary incontinence after TUR of the prostate, radical prostatectomy and meningocele operation the silicongel prothesis described by Kaufman was implanted. By compression of the bulbus urethrae a sealing effect was achieved. The pressure of the detrusor opens the urethra enough to enable urination. Seven patients were operated. The results directly after the operations were: three very good, two good and two bad. We achieved very good results by implantation of the prothesis "large" -in the case of the two bad operative results we used the "medium" size prothesis. By filling these protheses with 5 ml liquid they proved to be leaky. One of these was operatively removed and replaced by the size "large". This patient later developed a urethra stricture in the implantation region with overflow bladder after having been continent. By internal urethrotomy this condition was restored; except for minor dripping after miction the patient was continent. The prothesis of a direct postoperative continent patient had to be filled. This led to and infection with the development of fistula. The prothesis had to be removed. A further patient who sat on a hard object developed a lesion of the prothesis with resulting incontinence. At least six months after the operations four patients remained continent and three incontinent.

Aged↗
PubMedDate not supplied

Petrous apex cephaloceles.

BACKGROUND AND PURPOSE: Petrous apex cephaloceles (PACs) are uncommon lesions that are usually incidental but may be symptomatic. We reviewed MR and CT studies in 10 patients with PACs to identify characteristic imaging features that facilitate their diagnosis. METHODS: MR and CT studies from 10 patients with PACs were reviewed retrospectively. In each case the PAC was characterized by lesion center, signal intensity or attenuation, adjacent petrous apex pneumatization, and its relationship to Meckel's cave. Intraoperative findings were reviewed in the three cases in which surgery was performed. RESULTS: All 10 patients had lobulated expansile cystic petrous apex lesions centered along the posterolateral margin of Meckel's cave. All cysts were contiguous with Meckel's cave. Three patients had bilateral PACs. Four patients had symptoms that could potentially be explained by the PAC, while findings in the other six were incidental observations. Three patients underwent surgery, during which two lesions were diagnosed as meningoceles while the third was diagnosed as an arachnoid cyst protruding through a dural defect. CONCLUSION: PACs represent a protrusion of meninges and CSF from the posterolateral portion of Meckel's cave into the petrous apex, which is their characteristic imaging appearance. PACs are usually incidental but may be symptomatic. Surgical intervention should be approached cautiously and undertaken only when symptoms are clearly linked to the presence of this lesion.

Adult↗
PubMed2001

Limb/pelvis hypoplasia/aplasia with skull defect (Schinzel phocomelia): distinctive features and prenatal detection.

Schinzel phocomelia syndrome is characterized by limb/pelvis hypoplasia/aplasia: specifically, intercalary limb deficiencies and absent or hypoplastic pelvic bones. The phenotype is similar to that described in a related multiple malformation syndrome known as Al-Awadi/Raas-Rothschild syndrome. The additional important feature of large parietooccipital skull defects without meningocele, encephalocele, or other brain malformation has thus far been reported only in children with Schinzel phocomelia syndrome. We recently evaluated a boy affected with Schinzel phocomelia born to nonconsanguineous healthy parents of Mexican origin. A third-trimester fetal ultrasound scan showed severe limb deficiencies and an absent pelvis. The infant died shortly after birth. Dysmorphology examination, radiographs, and autopsy revealed quadrilateral intercalary limb deficiencies with preaxial toe polydactyly; an absent pelvis and a 7 x 3-cm skull defect; and extraskeletal anomalies including microtia, telecanthus, micropenis with cryptorchidism, renal cysts, stenosis of the colon, and a cleft alveolar ridge. A normal 46,XY karyotype was demonstrated, and autosomal recessive inheritance was presumed on the basis of previously reported families. This case report emphasizes the importance of recognizing severe pelvic and skull deficiencies (either post- or prenatally) in differentiating infants with Schinzel phocomelia from other multiple malformation syndromes that feature intercalary limb defects, including thalidomide embryopathy and Roberts-SC phocomelia.

Abnormalities, Multiple↗