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At least 901 records · Page 50Linked to original sources
PubMed2000

Secondary tethered cord syndrome in spinal dysraphism.

Secondary tethered cord syndrome following initial repair for spinal dysraphism is an important area of interest. In this study, 32 cases with spinal dysraphism in the lumbosacral region were enrolled, in whom radical repair with autologous material had been carried out in the early stage soon after birth. During the follow-up period of up to 19 years 10 months, surgery was considered to be indicated in 2 of the 8 lipomeningocele cases and in 6 of the 24 meningocele and meningomyelocele cases, because of the presence of tethered cord syndrome 4-19 years after the primary operation. In all 8 of these cases, MR imaging demonstrated tethered spinal cord in the form of low conus medullaris. In 6 of the 8 operated cases surgery was followed by improvement of the spinal neurological deterioration. According to our experience, early untethering for secondary tethered cord syndrome is essential. In addition, since the complications of Silastic duraplasty at untethering were all minor and the operative outcome was satisfactory, the use of silicone rubber sheeting as a dural substitute might be recommended to prevent adhesion of the spinal cord.

Adolescent↗
PubMed1997

[Imaging of the middle ear. CT and MRI].

High-resolution computed tomography (HRCT) provides excellent contrast between osseous structures, air and soft tissue in conjunction with high spatial resolution. Therefore, thin-section HRCT with bone window setting is the method of choice for the examination of the middle ear structures. The indications are acute and chronic inflammatory changes, cholesteatoma and tumor, the "postoperative middle ear", and malformations. In most cases, HRCT enables differentiation between inflammatory changes, cholesteatoma, and tumor. The excellent depiction of subtle osseous details enables the identification of erosions of the ossicles or of the bony walls of the mastoid cells, of osseous defects of the tegmen, of the bony labyrinth, and of the tympanic course of the facial canal. In addition, HRCT enables excellent depiction of reconstructions of the ossicles or prosthesis of the ossicles. Although HRCT is the first method of choice, magnetic resonance imaging (MRI) may provide additional information and lead to a more accurate diagnosis in some cases. This is explained by the excellent soft tissue contrast provided by MRI. In addition, MRI offers the possibility of using various pulse sequences and the administration of i.v. contrast material. Therefore, MRI may allow the differentiation between inflammatory changes, cholesteatoma, and tumor in those cases in which accurate diagnosis cannot be made by HRCT. The differentiation between a meningocele or meningoencephalocele and other entities such as tumors or cholesteatoma can be established by MRI. Furthermore, MRI can accurately depict cases of labyrinthitis or of neuritis of the facial nerve or of intracranial disease caused by middle ear processes, while this is not always possible by HRCT. In summary, HRCT of the middle ear is the method of choice, but MRI may provide supplementary information in those cases in which accurate diagnosis cannot be established by HRCT.

Ear Diseases↗
PubMed2000

Analysis of 100 pregnancy outcomes in women treated systemically with tacrolimus.

The aim of this paper is to provide a summary of clinical findings regarding the safety of tacrolimus in pregnancy. From 1992 to 1998 data were collected on 100 pregnancies from 84 mothers who received tacrolimus systemically; 83 cases of solid organ transplantation, and 1 case of Behçet's disease. Maternal mean age at conception was 28 years and pregnancy outcome was live birth in 68%, spontaneous abortion in 12%, induced abortion in 12%, stillbirth/perinatal death in 3%, ongoing pregnancy in 2%, and lost to follow up in 3%. Fifty-nine percent of the neonates were delivered prematurely (< 37 weeks of gestation). Birth weight was appropriate for the gestational age in 90% of the cases. Malformations occurred in 4 neonates: case 1, meningocele and urogenital defects; case 2, alcoholic embryopathy; case 3, ear defect, cleft palate and hypospadia; case 4, multicystic dysplastic kidney. There was no consistent pattern of malformations and 2 mothers subsequently delivered a healthy neonate while on tacrolimus therapy. Nearly 70% of pregnancies following systemic tacrolimus administration resulted in a favourable outcome without any significant effect on intrauterine growth. The incidence of malformations was similar to that reported with other immunosuppressants in transplant recipients.

Adolescent↗
PubMed2000

Spinal dysraphism: a review of neuroradiological features with embryological correlations and proposal for a new classification.

Our purpose was to review the neuroradiological features of spinal dysraphism and to correlate them with clinical findings and up-to-date embryological theory. We also aimed to formulate a working classification which might prove useful in clinical practice. We reviewed series of 986 children referred to our Spina Bifida Centre in the past 24 years. There were 353 children with open spinal (OSD) and 633 with closed (skin-covered) spinal (CSD) dysraphism. By far the most common open abnormality was myelomeningocele, and all patients with OSD had a Chiari II malformation. CSD was categorised clinically, depending on the presence of a subcutaneous mass in the back. CSD with a mass mainly consisted of lipomas with dural defects and meningoceles, and accounted for 18.8 % of CSD. CSD without a mass were simple (tight filum terminale, intradural lipoma) or complex (split cord malformations, caudal regression). Our suggested classification is easy to use and to remember and takes into account clinical and MRI features; we have found it useful and reliable when making a preoperative neuroradiological diagnosis in clinical practice.

Child, Preschool↗
PubMed2005

Contribution of fetal MRI to the diagnosis of inner ear abnormalities: report of two cases.

We report two cases of fetal inner ear abnormalities diagnosed by MRI. Cerebral MRI was performed on two fetuses, at 32 and 30 weeks gestation, following US that demonstrated multiple malformations suggestive of CHARGE syndrome in one fetus and ventriculomegaly and poor visibility of the posterior fossa in the other. MRI revealed vestibular hypoplasia and agenesis of the semicircular canals in one fetus and cystic cochleas, partial vermian agenesis and an occipital meningocele in the second fetus. Both pregnancies were terminated and there was good correlation between fetal MRI, ex utero CT and fetopathological findings. The inner ears should be carefully examined when performing fetal cerebral MRI because abnormalities of the inner ear may be associated with cerebral anomalies.

Abnormalities, Multiple↗
PubMed2003

Split spinal cord malformations: report of 22 cases and review of the literature.

OBJECT: The aims of this study were to describe the embryological changes that drive the formation of a split spinal cord malformation, analyse the forms of presentation, evaluate the diagnostic procedures and discuss the indications for the different forms of therapy for each case. METHODS: Clinical and radiological features of 22 cases of split spinal cord malformations (SSCM) are reported. Three groups of patients are considered: group a, patients with signs of tethered cord or scoliosis; group b, patients with midline cutaneous stigmata and group c, patients with a meningocele or meningomyelocele. CONCLUSION: While CT scan is particularly useful for the evaluation of vertebral bodies and posterior arch abnormalities and spur characteristics in SSCM, MRI gives complementary information on the anatomy of spinal cord, dural sac, conus and filum terminale and permits the exclusion of associated lesions such as hydro-syringomyelia, dermal sinus or dermoid and epidermoid cysts. Surgery should be considered indicated in all cases of SSCM, even the asymptomatic ones, except in very badly handicapped meningomyelocele patients with nonprogressive disability and type II SSCM.

Adolescent↗
PubMed2003

Multiple coexistent dysraphic pathologies.

INTRODUCTION: Four distinct dysraphic anomalies were observed in a single child. While combinations of such anomalies are well recognised, quadruple dysraphic pathology, nevertheless, is extremely uncommon. To our knowledge, no previous cases have been reported in the literature. CASE REPORT: We present the management of a child with a concurrent segmental meningocele, a type-1 split cord malformation (SCM) associated with hemivertebrae, lipomyelomeningoceles in each hemicord of the SCM and a terminal myelocystocele, and we review the literature on potential mechanisms of dysmorphogenesis. DISCUSSION: Existing embryologic hypotheses for the dysraphic spectrum lack experimental evidence and studies in animal models. This case challenges the existing hypotheses and illustrates our incomplete understanding of human terminal spinal cord embryogenesis. Further studies on the morphogenetic basis for these anomalies are required.

Abnormalities, Multiple↗
PubMed2000

Endoscopic approaches to the cranial base: perspectives and realities.

We describe the development of transnasal endoscopic approaches to the cranial base in an interdisciplinary series of 103 patients, including 13 in the pediatric age group. Our aim was to define, with the aid of different case reports, the possibilities of endoscopic techniques in tumor resection, fistula repair, the treatment of mucoceles and meningoceles, and of combined intracranial and endoscopic approaches. The advantages of these minimally invasive approaches are panoramic visualization, rapidity and reduction of the cosmetic and functional disabilities in comparison with other conventional approaches, and a better capacity for identifying and developing key landmarks for surgery. On the other hand, endoscopic surgery requires an exsanguine operation field, technical improvements in instruments, and specific skills. Interdisciplinary collaboration in endoscopic approaches has proved useful in integrating experiences without overlap between fields, and in broadening possibilities: in our opinion endoscopic approaches will certainly be important in the future of cranial base surgery.

Adolescent↗
PubMed2001

Atretic cephalocele communicating with lateral ventricles.

Atretic cephaloceles are thought to be involuted true meningoceles or encephaloceles. They are located at the midline of the parietal or occipital region and usually joined to the subarachnoidal space. We present a 6-month-old girl with atretic cephalocele communicating with the lateral ventricles.

Encephalocele↗
PubMed2006

Perineal lipomas associated with anorectal malformations.

Newborns with anorectal malformations may have associated perineal lipomas. These lesions are rare but may make the repair of the anorectal malformation more complex. Knowledge about the functional consequences of these lesions is scanty. The purpose of this study was to review the experience of anorectal malformations with perineal lipomas in two Scandinavian paediatric surgical centres. Six patients with perineal lipomas and anorectal malformation treated in the two centres from 1991 to 2005 were retrospectively reviewed. Bowel function was evaluated in patients more than 4 years old. Three girls and three boys (age 4 months to 14 years) with anorectal malformations and perineal lipoma were included in the study. The boys all had high anorectal malformations with rectourethral or rectovesical fistulas. Two girls had a rectovestibular fistula and one girl had a cloaca. All patients had perineal lipomas, in one associated with an anterior meningocele. The lipomas were excised at time of anorectal reconstruction. One of the patients still has a colostomy, and two colostomies were recently closed. The bowel function was evaluated in three patients. Two patients have an ACE stoma to control constipation and soiling and one is soiling despite regular washouts. In conclusion, perineal lipomas associated with anorectal malformations are rare but may distort sphincter anatomy. Excision is best performed at time of anorectal reconstruction. Although our experience is limited, bowel function seems to be compromised by these lesions.

Adolescent↗
PubMed2003

Two occult skull base malformations causing recurrent meningitis in a child: a case report.

Occult malformations of the skull base are rare anomalies, but can cause severe complications such as meningitis. Detailed skull base investigations for detecting cerebrospinal fluid fistulas or celes are often not initiated until after a history of recurrent meningitis. We present a child first seen at the age of 12 with recurrent episodes of bacterial meningitis since early childhood, requiring antibiotic prophylaxis for years. High-resolution computed tomography revealed a chronic sinusitis and a bony defect on the right olfactory groove, while magnetic resonance imaging and CT-cisternography indicated no cerebrospinal fluid fistula or cele at that time. Endonasal surgery for chronic sinusitis was performed with a confirmed bony defect on the right olfactory groove and an olfactory fibre without its sleeve-like dura prolongation running into an adjacent ethmoidal cell, necessitating that it be covered. In the absence of any antibiotics a new episode of meningitis occurred 5 years after surgery. CT-cisternography and magnetic resonance imaging were repeated, now indicating a transclival bony defect with a meningocele in its proximal part, most probably presenting a canalis basilaris medianus. Endonasal surgery confirmed this bony defect after adenoidectomy, and closure was accomplished. No further meningitis has been observed for 2 years. Congenital skull base defects may be difficult to detect, but sufficient surgical closure after their precise delineation is mandatory to prevent infectious endocranial complications. The presence of more than one developmental skull base defect should be considered during careful radiological skull base evaluation, which has to include the clivus in order not to overlook rare basilar malformations.

Child↗
PubMed2006

Endoscopic endonasal sinus surgery: a review of 18 years of practice and long-term follow-up.

A study of 1,915 patients operated on with a total of 4,285 endoscopic endonasal ethmoidal procedures during a period of 18 years is presented. The study comprises five groups of patients with (1) acute recurrent and chronic sinusitis--long-standing symptoms >3 months--(n = 733), (2) nasal/paranasal polyposis (n = 710), (3) sinogenic headache (n = 325), (4) mucoceles (n = 59) and (5) other nasal/paranasal disorders (n = 88). This group includes acute sinus infection with complications (n = 38), acute trauma/injury (n = 22), Grave's disease with exophthalmus (n = 12) and olfactory dysfunction (n = 16). The need for a thorough examination leading to a final diagnosis is emphasized. Endoscopy and CT/MRI are standard tools in the diagnostic and postoperative process. Close cooperation and educational teaching of the patient are necessary to obtain highest compliance. Long-standing medical treatment of inflammatory diseases is important to the long-term outcome and is of utmost importance to surgical success. Surgery is recommended to be performed under general anesthesia (total intravenous anesthesia or laryngeal mask anesthesia) with additional local, topical anesthetics, and can be easily performed on a day-case out-patient basis. The surgery is based upon the Messerklinger technique (MT). Peroperative complications are reduced by a skilled and meticulous surgical conduct and an adequate medication preoperatively. During this period of time, necessary controls within the first postoperative month were reduced from 3-4 to only one. Long-term follow-up secures a long-standing optimal result. Endoscopic surgery for more rare conditions, such as orbital decompression, CSF leaks and meningoceles, is recommended to be performed in specialized centers with adequate trained staff and necessary equipment. The possibilities of the picture archiving communication system, giving a number of different projections and angles and video-guided imaging peroperatively, facilitates safe surgical performances in areas usually associated with great risks and hazards. The long-term results are very good during the above circumstances. Reoperations are few and the patient satisfaction is very high.

Adult↗
PubMed1998

Cochlear implantation after subtotal petrosectomies.

Among cochlear implant candidates there are patients who have abnormal middle and/or inner-ear conditions that make them unsuitable for implantation. Insertion of a foreign body may also be contraindicated in the setting of an existing or potential intracranial communication, or when the ear is prone to infection. Five patients presented with such unfavorable conditions. These included a Mondini dysplasia with persistent cerebrospinal fluid leak, an atretic mastoid with meningocele, chronic otitis media, a transverse petrous bone fracture, and a temporal bone adenoma. All patients underwent subtotal petrosectomies and cochlear implantations. In four cases implantation was performed concomitantly with subtotal petrosectomy, while the remaining case required a two-stage procedure. No complications occurred. The technique is described in detail, and the cases and the indications for surgery are discussed. By obliterating and isolating the tympanomastoid cleft from the outer environment and utilizing the technique of subtotal petrosectomy, a broader spectrum of patients can now be implanted safely.

Adult↗
PubMed1980

Fetal cranial and craniocervical masses: ultrasound characteristics and differential diagnosis.

Ultrasound is assuming an essential role in the detection of fetal cranial and spinal anomalies. Illustrated in this article is the sonographic appearance of cranial abnormalities wherein the diagnosis of encephalocele is clear cut because the anatomic defect of the neural tube is visualized. Additionally presented is a variety of cranial and craniocervical cystic masses, including meningocele, wherein the anatomic defect of the neural tube is not apparent and the diagnosis is reached by careful attention to the specific ultrasonic characteristics of each mass.

Adult↗
PubMed1991

Neurenteric cyst: surgery and diagnostic imaging.

Neurenteric cysts are rare, with fewer than 30 cases noted in the literature. We report the case of a newborn infant with respiratory distress caused by a large neurenteric cyst that was identified by prenatal ultrasound. Treatment consisted of excision of the mass through a right posterolateral thoracotomy. The cyst adhered to the spine at the level of the first thoracic vertebra and communicated with the jejunum through a posterior diaphragmatic defect. Postoperative studies with magnetic resonance imaging (MRI) and computed tomography (CT) disclosed an anterior meningocele and tethering of the spinal column. This is the second reported case of a neurenteric cyst demonstrated by prenatal ultrasound. The presence of an intrathoracic cyst associated with spinal abnormalities is characteristic of this anomaly. With imaging techniques such as MRI and CT, we may detect residual intraspinal disease associated with neurenteric cysts.

Female↗
PubMed1993

Long-term outcome in surgically treated spina bifida cystica.

This report describes the long-term operative outcome of 72 patients with spina bifida cystica. The period of follow-up was between 4 and 20 years. In our series, 17 patients died, with the mortality rate increasing as the lesions were more rostral. All cases involving only a meningocele are living without handicap. The cases of spina bifida cystica with hydrocephalus had higher morbidity and mortality when compared to those without hydrocephalus. We conclude that the rostro-caudal location, the content of the sac, and whether there is associated hydrocephalus are important factors influencing the long-term prognosis of spina bifida cystica.

Child↗
PubMed1996

Endoscopic repair of traumatic CSF rhinorrhea in a pediatric patient.

Cerebrospinal fluid (CSF) rhinorrhea typically results from trauma to the skull base, producing leaks through either the cribiform plate region or the sphenoid bone. Traditional approaches to the repair of such leaks include a frontal craniotomy or external ethmoidectomy. An endoscopic approach through the nose has also proven to be successful. A 7-year-old male suffered traumatic CSF rhinorrhea and development of a meningocele in the region of the cribiform plate. After demonstrating the site of the leak with fluorescein dye, the defect was repaired via a transnasal endoscopic approach. The evaluation of the child with CSF rhinorrhea, including the presenting symptoms and signs and the radiographic assessment, is presented. The variety of approaches, types of repair and post-operative care are also discussed.

Cerebrospinal Fluid Rhinorrhea↗
PubMed1987

Teratogenicity of flubendazole in rats.

Flubendazole, the p-fluoroderivative of mebendazole, was suspended in deionized water, and administered by gavage once daily to pregnant rats on days 8 through 15 of pregnancy at 0 (control), 2.5, 10, 40 or 160 mg/kg. Fetuses were removed on day 21 of pregnancy by caesarian section, and examined by routine teratological methods. The highest dose (160 mg/kg) was embryocidal and resulted in a significant increase in the fetal resorption rate. There was a dose-dependent decrease in fetal body weights which was significant at 40 mg/kg or more. The 40 and 160 mg/kg doses induced significant fetal (gross, skeletal and internal) malformations. A variety of gross malformations occurred, i.e. encephalocele, cranial meningocele, omphalocele, ectrodactyly, club foot, defects in tail, anal atresia, shortened backbone and Spina bifida occulta. The skeletal malformations mainly affected the vertebrae and ribs. The most frequently observed internal malformation was hydrocephaly, followed by anophthalmia and/or microphthalmia.

Abnormalities, Drug-Induced↗