1/20. Spontaneous cerebrospinal fluid otorrhea from a tegmen defect: transmastoid repair with minicraniotomy.Spontaneous cerebrospinal fluid (CSF) otorrhea is a rare condition that presents in 2 clinical categories. In congenital labyrinthine malformations, it leads to bouts of meningitis in a hearing-impaired child. In the adult age group, a spontaneous CSF leak almost always results from a dural and bony defect in the tegmen area. Possible pathogenic mechanisms include progressive sagging and rupture of dura through a congenital tegmen dehiscence and progressive bone erosion by aberrant arachnoid granulations. These patients usually present with a middle ear effusion, resulting in clear discharge after myringotomy with tube insertion. Based on 4 patients with a CSF leak from a tegmen defect, this report reviews the clinical findings and diagnostic approach. The surgical management by a 5-layer closure using a transmastoid approach with minicraniotomy is outlined. This procedure offers a relatively simple and reliable method for repair without the inherent risks of a middle fossa craniotomy.- - - - - - - - - - ranking = 1keywords = tube (Clic here for more details about this article) |
2/20. Detection of skin over cysts with Spina bifida may be useful not only for preventing neurological damage during labor but also for predicting fetal prognosis.Spina bifida is one of the most common open neural tube defects. There are two common types of spina bifida cystica, myelomeningocele and meningocele. Special attention to the thickness of the cystic sac (presence of intact skin and subcutaneous tissue) on magnetic resonance imaging is advantageous for determination of whether the child will profit from cesarean section in order to prevent neurological change (infection and drying of nerve tissue) and for management of spina bifida (most meningocele) during the perinatal period. Furthermore, skin detection may help to predict the prognosis of spina bifida after birth. meningocele, with intact skin over the cyst, has a better clinical course than myelomeningocele. Some myelomeningoceles with neural tube defects in a lower position, also frequently having an intact skin over the cyst, have almost the same clinical course as a meningocele. From this, we hypothesize that a baby with spina bifida who has intact skin over the cyst might have a good prognosis neurologically. In this report, we concentrate attention on the skin over cysts in 3 cases (1 meningocele and 2 myelomeningoceles).- - - - - - - - - - ranking = 2keywords = tube (Clic here for more details about this article) |
3/20. Rudimentary meningocele: remnant of a neural tube defect?BACKGROUND: Rudimentary meningocele, a malformation in which meningothelial elements are present in the skin and subcutaneous tissue, has been described in the past under a variety of different terms and has also been referred to as cutaneous meningioma. There has been debate as to whether rudimentary meningocele is an atretic form of meningocele or results from growth of meningeal cells displaced along cutaneous nerves OBJECTIVE: We reviewed the clinical, histological, and immunohistochemical characteristics of rudimentary meningocele in an attempt to assess the most likely pathologic mechanism for it. DESIGN: Retrospective study. SETTING: University hospitals. patients: Thirteen children with rudimentary meningocele. MAIN OUTCOME MEASURES: medical records were reviewed and histopathologic examination as well as immunohistochemistry studies were performed for each case. A panel of immunoperoxidase reagents (EMA, CD31, CD34, CD57, S-100, and CAM 5.2) was used to assess lineage and to confirm the meningothelial nature of these lesions. RESULTS: Recent evidence indicating a multisite closure of the neural tube in humans suggests that classic meningocele and rudimentary meningocele are on a continuous spectrum. CONCLUSION: Rudimentary meningocele seems to be a remnant of a neural tube defect in which abnormal attachment of the developing neural tube to skin (comparable to that in classic meningocele) could explain the presence of ectopic meningeal tissue. In the majority of cases, no underlying bony defect or communication to the meninges could be detected. However, in light of the probable pathogenesis, imaging studies to exclude any communication to the central nervous system should precede any invasive evaluation or intervention.- - - - - - - - - - ranking = 7keywords = tube (Clic here for more details about this article) |
4/20. spinal dysraphism associated with congenital heart disorder in a girl with MELAS syndrome and point mutation at mitochondrial dna nucleotide 3271.We describe a case of mitochondrial encephalopathy, lactacidosis, and stroke-like episode (melas syndrome) associated with ventricular septal defect and meningocele at the L3 level in a 5-year-old girl. Mitochondrial dna analysis showed point mutation at nucleotide 3271--> TC. The occurrence of heart and neural tube defects in association with usual features of the melas syndrome might be explained by either defective high-energy metabolism during early embryogenesis or a common genetic cause.- - - - - - - - - - ranking = 1keywords = tube (Clic here for more details about this article) |
5/20. Spontaneous CSF rhinorrhoea from separate defects of the anterior and middle cranial fossa.Multiple congenital dehiscence of the skull base is rare and can give rise to spontaneous CSF rhinorrhoea. A search of the world literature revealed only five reports of CSF leak with more than one concomitant skull base defect. When treating a patient with spontaneous CSF rhinorrhoea the possibility of its originating from the middle ear and eustachian tube should be considered. An intrathecal injection of fluorescein is useful in establishing the site of a CSF leak especially when a computed tomography scan (CT) or magnetic resonance image (MRI) has not localized the site. We discuss a case of a 72-year-old lady presenting with CSF rhinorrhoea, who had an anterior skull base defect localized with the help of intrathecal fluorescein and repaired surgically. Subsequent to this she had a further episode of CSF rhinorrhoea that originated from a middle-ear meningocele that was then repaired.- - - - - - - - - - ranking = 1keywords = tube (Clic here for more details about this article) |
6/20. klippel-feil syndrome plus atretic meningocele in one identical twin and anencephaly in the other.klippel-feil syndrome (KFS) is a heterogeneous entity, characterized by specific congenital anomalies of segmentation of the cervical spine. We report a case of KFS plus atretic meningocele in one identical twin and anencephaly in the other. These identical twins were both male with no chromosome defect. One of them had anencephaly diagnosed at the gestational age of 17 weeks and died soon after birth, whereas the co-twin had KFS with multiple congenital anomalies including short neck with low hairline, occipital atretic meningocele, butterfly vertebrae, hemivertebrae, cervico-thoracic scoliosis, left thumb preaxial polydactyly, and right undescended testis. He received rehabilitation during infancy and could walk at the age of 15 months. Both of these infants had preaxial polydactyly, which is an uncommon anomaly associated with KFS. In conclusion, iniencephaly and anencephaly are probably the extreme manifestations of KFS rather than different dysraphisms. Prompt clinical suspicion with early diagnostic imaging for spine lesions and occult opening of neural tube is mandatory for further intervention and rehabilitation.- - - - - - - - - - ranking = 1keywords = tube (Clic here for more details about this article) |
7/20. Diagnosis and management of spontaneous cerebrospinal fluid-middle ear effusion and otorrhea.OBJECTIVES/HYPOTHESIS: Spontaneous leak of cerebrospinal fluid (CSF) into the middle ear can occur in adults without a history of temporal bone trauma or fracture, meningitis, or any obvious cause. Therefore, clues may be lacking that would alert the otolaryngologist that fluid medial to an intact eardrum, or fluid emanating from an eardrum perforation, is likely to be CSF fluid. A review of relevant medical literature reveals that herniation of the arachnoid membrane through a tegmen defect may be congenital, or CSF leak may occur when dynamic factors (i.e., brain pulsations or increases in intracranial pressure) produce a rent in the arachnoid membrane. Because tegmen defects may be multiple rather than single, identifying only one defect may not be sufficient for achieving definitive repair. Data on nine cases of spontaneous CSF leak to the ear in adult patients from four medical centers are presented and analyzed to provide collective information about a disorder that can be difficult to diagnose and manage. STUDY DESIGN: Retrospective review of nine cases of spontaneous CSF middle ear effusion/otorrhea. RESULTS: The majority of patients presented with symptoms of aural fullness and middle ear effusion. Many developed suspicious clear otorrhea only after insertion of a tympanostomy tube. Two patients had multiple defects in the tegmen and dura, and five patients had meningoencephaloceles confirmed intraoperatively. Five patients underwent combined middle cranial fossa/transmastoid repair. Materials used in repair included temporalis fascia, free muscle graft, Oxycel cotton, calvarial bone, pericranium, bone wax, and fibrin glue. CONCLUSIONS: CSF middle ear effusion/otorrhea can develop in adults without a prior history of meningitis or head trauma or any apparent proximate cause. Although presenting symptoms can be subtle, early suspicion and confirmatory imaging aid in establishing the diagnosis. Because surgical repair by way of a mastoid approach alone can be inadequate if there are multiple tegmen defects, a middle fossa approach alone, or in combination with a transmastoid approach, should be considered in most cases.- - - - - - - - - - ranking = 1keywords = tube (Clic here for more details about this article) |
8/20. Thoracic meningocele, meningomyelocele or myelocystocele? Diagnostic difficulties, consequent implications and treatment.spina bifida cystica is a closing disorder of the neural tube which infrequently occurs in the thoracic region. A rare lesion called myelocystocele is a variant of spina bifida cystica and is associated with syringomyelia, Chiari type 2 malformation and hydrocephalus. Usually the patient has no neurological deficit, but future deterioration can occur due to posterior tethering of the spinal cord by adhesions. The prenatal diagnosis by ultrasound study can be misleading and in order to attain the correct diagnosis, especially if abortion is considered, a prenatal MRI scan should be done before the parents are counselled, and should be repeated prior to operative treatment. Surgical correction of myelocystocele is not only for cosmetic reasons, but also to untether the spinal cord prophylactically to prevent future neurological deterioration. In this case report, we present a child born with a thoracic myelocystocele, the diagnostic difficulties, consequent implications and surgical treatment.- - - - - - - - - - ranking = 1keywords = tube (Clic here for more details about this article) |
9/20. Spinal congenital dermal sinus associated with upper thoracic meningocele. Case Report.The congenital dermal sinus is an abnormal epithelium-lined sinus tract between the skin surface and deeper tissues. It occurs during neurulation when the neural groove closes to form the neural tube on Day 26 of gestation and results from a failure of neuroectoderm to separate from the cutaneous ectoderm. The most frequent location is the lumbosacral area; an upper thoracic location is quite rare. This 37-year-old man presented with headache and numbness in both arms. No specific neurological findings were observed. physical examination revealed a dimple at T-2. radiography and magnetic resonance imaging of the thoracic spine revealed spina bifida at T1-3, a meningocele, and a dermal sinus tract complex. The treatment approach and outcome in this unusual case are presented.- - - - - - - - - - ranking = 1keywords = tube (Clic here for more details about this article) |
10/20. Cephalocele involving the oral cavity.A cephalocele is an uncommon neural tube defect that rarely involves the oral cavity. We present a case of an oral cephalocele associated with polyhydramnios discovered on antenatal ultrasound. The differential diagnosis includes epignathus and epulis. The prognosis is dependent not only on the extent of brain involvement and associated anomalies, but also on the exact location of the herniated mass. If the oral cavity is extensively involved, airway management at birth may be difficult. Therefore, preparations for delivery may include maternal tertiary site referral, antenatal neurosurgical and pediatric consultation, and anticipation of complications such as rupture of the sac, dystocia, or the need for immediate tracheostomy if intubation is not possible.- - - - - - - - - - ranking = 1keywords = tube (Clic here for more details about this article) |
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