Content uploaded by Mark P Arts
Author content
All content in this area was uploaded by Mark P Arts on Jan 06, 2014
Content may be subject to copyright.
Thoracic Meningocele, Meningomyelocele or Myelocystocele? Diagnostic Difficulties, Consequent Implications and Treatment
Abstract
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.
... The neurological prognosis in cervicothoracic cystic dysraphism is often favorable. Usually no neurological deficits are present in the newborn [1,9]. However, neurological deterioration can result from associated anomalies such as hydrocephalus, Chiari II malformation, hydromelia or cord tethering, which can occur in three different situations: (1) preoperatively, if an unrecognized stalk is present and tethers the cord; (2) postoperatively, if the stalk is incompletely excised or adhesions develop, and (3) if an associated anomaly such as thickened filum tethers the cord [1][2][3]9]. ...
... Usually no neurological deficits are present in the newborn [1,9]. However, neurological deterioration can result from associated anomalies such as hydrocephalus, Chiari II malformation, hydromelia or cord tethering, which can occur in three different situations: (1) preoperatively, if an unrecognized stalk is present and tethers the cord; (2) postoperatively, if the stalk is incompletely excised or adhesions develop, and (3) if an associated anomaly such as thickened filum tethers the cord [1][2][3]9]. If mistaken for meningoceles due to a benign neurological exam and cystic nature, associated anomalies may be missed or meningocele with stalk and myelocystocele may be inappropriately ligated, potentially resulting in a poor neurological outcome [4,6]. ...
... The initial neurological exam in newborns is usually normal, likely due to the lack of functioning neurological tissue in the sac and/or the subtle nature of these neurological defects that do not become apparent until motor function has increased [4,9]. Some patients were initially treated for cosmetic reasons, with a limited excision of the protuberant portion, until it was realized that these patients often demonstrated progressive neurological decline [6]. ...
Cystic dysraphism of the cervical and upper thoracic spine is very rare. It differs from the much more common lumbosacral dysraphism in appearance and structure, and usually portends a better prognosis due to lack of functional neurological tissue in the dysraphic sac and absent or less severe intracranial anomalies. There is ambiguity in the literature regarding terminology because of the paucity of cases. We present cases of the most common type of cervicothoracic cystic dysraphism and emphasize differences from lumbosacral myelomeningocele. Patient outcome depends on the presence of associated anomalies and whether complete surgical resection is performed. Imaging plays a critical role in surgical planning, screening the central nervous system for additional anomalies, and in the postoperative setting for evaluation of retethering.
... Most commonly, the sac does not contain parts of the spinal cord, but the latter can be fixated by tissue adhesion to the column of MC [ 12 ]. Sometimes inside the sac can be found modified spinal roots and/or filum terminale, fused with its internal wall, and more rarely, ganglion cells or even a diverticulum of the central canal of the spinal cord [ 13 ]. ...
... The case we present does not confirm the findings of Kumar et al. [ 1 ] that in most patients with associated spina bifida cystica and split cord malformation, the sept is located either on the level of the first anomaly or one or two levels higher. In undiagnosed associated anomalies with meningocele (tethered cord syndrome, hydrocephaly, split cord malformation and others), a more or less pronounced neurological deficit is later possible [ 13 ]. ...
The association of dysraphic spinal anomalies arising from different periods of the embryogenesis is very rare. We describe the case of a 5-month-old child, without a neurological deficit, but with numerous lesions, including thoracic meningocele (Th4–Th6), diastematomyelia (type II split cord malformation on the level of L1–L3) and butterfly vertebra (L2and L3). In patients with spina bifida cystica in which there are no skin and significant orthopedic changes, as well as no neurological deficit, magnetic resonance imaging is able to confirm or omit other hidden associated anomalies.
... Meningomyelocele is a broad term representing herniation of extracranial contents through a congenital defect in the vertebral column. If only cerebrospinal fluid (CSF) and meninges herniate, it is termed as a meningocele [1]. Securing the airway with intubation becomes challenging in occipital meningocele due to difficulty in positioning the patient for conventional laryngoscopy. ...
... Meningomyelocele is hernial protrusion of part of meninges and neural elements in a sac [1]. Reported incidence is 1 in 5000 live births [4]. ...
Meningocele is a neural tube defect where meninges protrude through a skeletal defect. Occipital meningocele compounded with the problems of paediatric airway itself pose challenges to anaesthesiologist in securing and maintaining airway. We present a case report of a 3 month-old-child with occipital meningocele posted for ventriculo-peritoneal shunt for hydrocephalus, who was successfully intubated using a size 1 Airtraq optical laryngoscope. © 2012 Egyptian Society of Anesthesiologists. Production and hosting by Elsevier B.V.
... The meningocystocele is covered with normal skin. It is most frequently found in the lumbosacral region [17,18]. In the current case, because of the absence of CSF on aspiration, the lesion was excluded to be a meningocystocele, in spite of having the typical features of the consistency and location of the meningocystocele. ...
and the differential considerations to avoid misdiagnosis with other similar lesions and the surgical procedure for its excision and precautions should be taken. Case History A full-term five-day-old buffalo-calf, weighted 57 kg body weight (BW) was referred to the veterinary teaching hospital, department of surgery, anesthesiology, and radiology, faculty of veterinary medicine, Assiut University, Assiut, Egypt. The calf was born with a massive tumor-like lesion on its back. However, the owner confirmed normal suckling, standing, laying, and walking behavior of the calf. Also, the calf had no nervous manifestations since its birth when we asked the owner. Clinical examination and differential considerations Clinical examination of the calf revealed a dorsal thoracolumbar massive, but fluctuating mass on the back of the calf, along with its spine (Figure 1). The mass was covered by normal skin and revealed no pain on manipulation. For the first time, it was suspected to be a thoracic meningocystocele because of its location as well as the fluctuation. Under aseptic circumstances, exploratory puncture did not yield any aspirate. Ultrasonographic examination was not available at this time. The body vital parameters (rectal temperature, heart rate, and respiratory rate) of the calf were within the normal (37.8℃, 73beats/ min, and 17breaths/min, respectively). On neurological examination, the calf did not show any neurological deficit.
... Incidence is 0.4 to 1 out of 1000 live births, but incidence varies with environmental and genetic factors. 1,2,3 Failure of closure of neural tube in embryo result in sac like herniation of neural elements and meninges. Most common site is Lumbosacral. ...
Lumbosacral meningomyelocele and hydrocephalus are two major congenital neurological defects in paediatric patients.
METHODS
We have discussed management of two cases, one of large meningomyelocele and second of massive hydrocephalus. Both cases
were done successfully under general anaesthesia taking care of proper positioning. Child with meningomyelocele was positioned
supine on two sand bags, creating a doughnut shaped depression to accommodate the meningomyelocele. Patient with
hydrocephalus was placed on 25cms high pillow with head on a high head ring and an assistant’s hand supporting the head while
intubation.
RESULTS
Major challenges for anaesthesiologist in these patients include airway management with proper positioning, replacement of
blood and fluid losses and maintenance of body temperature.
Pulp capping materials are used in the cases of vital pulp therapy as liners and
bases. Calcium hydroxide has become a golden standard for the pulp capping materials.
First publications about Mineral trioxide aggregate (MTA) date from the nineties of the
previous century. It is classified as dental cement. Biodentine is presented in 2010 as a new
class synthetic dental material with excellent mechanical properties, biocompatibility and
bioactivity.
The three materials show biological activity to the pulp tissue in the cases of vital pulp
therapy because of the calcium, but they differ a lot about their components and clinical
protocol.
Laser is a device that produces a nearly parallel, nearly
monochromatic, and coherent beam of light by exciting atoms to a higher energy level and causing them to radiate their energy in phase. The first laser was created by Meiman in 1960. In 1997 the first laser approved by FDA for hard dental tissue treatment is Er:YAG
laser.
Nowadays dental laser systems with their ergonomic design give numerous
possibilities for applications in Dental Medicine. The two lasers with optimal parameters
for “optical drilling” of enamel and dentin are Er:YAG and Er, Cr:YSGG. There are also
many other lasers with different wavelengths which can be used for different procedures in Operative Dentistry and Endodontic.
Anterior myelocystocele has not been reported previously. Our case showed anterior herniation of myelocystocele sac. This challenges the established hypothesis of Steinbock and Cochrane which holds partial neural-epidermal adhesion responsible for drawing the neural tissue outwards towards the skin. This cannot explain occurrence of anterior myelocystocele as there is no neural-epidermal adhesion anteriorly at any stage of embryogenesis. Myelocystoceles need to be differentiated from meningoceles as early as possible (preferably prenatally) due to different surgical treatments and possibility of fetal intervention.
Myelocystocele is a rare form of spinal dysraphism. Thoracic myelocystocele is still rarer. The occurrence of thoracic myelocystocele associated with type-1 split cord malformation, low lying tethered cord, dorsal syrinx and spina bifida is extremely rare. Clinical presentation of such a rare case and an early surgical management is discussed briefly.
Terminal myelocystocele (TMC) results from failure of embryonic CSF to drain outside the neural tube creating CSF reservoir within a dorsal meningocele. Association of Chiari II malformation with diastematomyelia and myelocystocele is extremely rare. Myelocystoceles do not have neural tissue so they have good prognosis after treatment, however when associated with hydromelia and Chiari malformation they present with neurological deficits. We present details of a 2 year old female who presented to us with this rare anomaly.
Between 4% and 8% of cases of spina bifida cystica occur in a cervical or cervicothoracic location. Despite a large body of literature concerning spinal dysraphism, there has been little written specifically about patients afflicted with this disorder in a cervical location. Eight children who presented at birth with posterior cervical or cervicothoracic lumps, all of which represented a dysraphic state, are discussed. Two types of abnormalities were noted. Three patients had hydromyelia with an associated myelocystocele herniating posteriorly into a meningocele sac. In these three patients there was an associated Chiari II malformation and hydrocephalus. The other five children had a meningocele in which a band of tissue extended from the posterior aspect of the spinal cord through a defect in the bone and fascia to the posterior part of the meningocele sac itself. No patient had a lesion that could be described as a meningomyelocele. The investigation and surgical management of these conditions are discussed and the need for intradural exploration to untether the spinal cord in the cervical region is stressed.
Simple meningoceles are infrequent forms of dysraphism and are often benign. They have been associated with other spinal anomalies. The uncommon cervical meningocele may have a higher propensity to be associated with other spinal anomalies. Four patients with cervical meningocele are presented with radiographic evaluation and clinical course. Multiple abnormalities were documented radiographically and operatively, including hydrocephalus, Chiari malformation, hydromyelia, lipomeningomyelocele, tethered cord, thickened filum terminale, diastematomyelia, Klippel-Feil syndrome, and thoracic hemivertebrae. Prior to the development of any late neurological abnormality from associated spinal anomalies, magnetic resonance imaging is recommended early in a child born with a simple meningocele.
Cervical spina bifida, either occult or overt, is relatively uncommon. These lesions tend to be associated with other abnormalities of the central nervous system and spine. For the occult dysraphic lesions, a Klippel-Feil syndrome is a particular association, especially the type I variety. For the spina bifida cystica lesions, the more common associated features include a Chiari II malformation and hydrocephalus. Spina bifida cystica in the cervical region is different from the myelomeningocele of the lower spine and is characterized by two types of abnormalities: the myelocystocele herniating posteriorly into a meningocele and a meningocele with or without an underlying split cord malformation. In both the occult dysraphic states and spina bifida cystica, there is underlying tethering of the cervical spinal cord, which can cause late neurologic deterioration, and surgical treatment is indicated both for cosmetic reasons and to untether the spinal cord. Children with spina bifida cystica may have neurologic dysfunction that may not be evident at birth or in the first few months of life but may be identifiable as the child grows older. One needs to be cautious about providing too optimistic a prognosis with respect to neurologic dysfunction based on a neonatal examination and to recognize that the appearance of neurologic deficits may not indicate deterioration.
Cervical myelomeningoceles are rare dysraphic lesions. Nine cases of cervical myelomeningoceles are reported. The external features of all nine myelomeningoceles were strikingly similar: They were sturdy, tubular protuberances from the back of the infants' necks, covered at the base by full-thickness skin and covered on the dome by thick squamous epithelium. Internally, these were tethered cord lesions in which fibroneural bands or sagittal midline fibrous septa were tightly tethering the cervical spinal cord to the adjacent dural or intrasaccular soft tissues. Six of our early cases (Group 1) were initially treated with simple subcutaneous resection of the sac and ligation of the dural fistula without release of the internal tethering structures. Five of these children subsequently deteriorated 13 months to 8 years later, all with worsening hand function and spastic legs. All five were reexplored, and the tethering bands and septa were excised; all showed improvement. The other three neonates (Group 2) treated in the last 4 years underwent initial intradural exploration of the lesions; in one case, the tethering fibrous elements were only partially eliminated and the patient deteriorated 4 years later, but improved after a second operation for resection of a missed ventral fibrous septum. The other two Group 2 infants had a thorough release of the fibroneural stalks initially, and both were neurologically stable 3 years later. We recommend that cervical myelomeningoceles should be studied preoperatively with magnetic resonance imaging and computed tomographic myelography to identify the internal structures. The minimum initial surgical treatment should be a two-level laminectomy, intradural exploration, and excision of all tethering bands and septa, in addition to resection of the sac. If a split cord is revealed by imaging studies, both the ventral and dorsal surfaces of the hemicords must be carefully inspected to locate the median septum.
The classification and embryogenesis of cystic cervical dysraphic lesions are discussed in the light of the authors' experience and review of the literature. It is felt that these lesions are best described as meningoceles or myelocystoceles, and the use of the term 'myelomeningocele' may be more confusing than clarifying. The authors hypothesize that the cervical meningocele and the myelocystocele are part of a spectrum of the same underlying developmental abnormality, namely limited dorsal myeloschisis, with the eventual abnormality depending on the presence or absence of associated hydromyelia.
Eight children born with occult spinal dysraphism were diagnosed in utero by ultrasonography. Post-natally, they were evaluated by MR scans. The ultrasound scans in all 8 fetuses revealed a spina bifida: the spinal cord was long in 5 and in 3 a meningocele was thought to be present, however, in 1, a post-natal MR scan revealed a lipomeningocele instead of a simple meningocele. In 3 fetuses an echogenic area was seen on the ultrasound scan which raised the possibility that an intradural lipoma was present, and was confirmed by post-natal MR scans. In 3 fetuses vertebral body anomalies and an additional ossification centre in a midline bony spur together with widening of the spinal canal were seen in the lower lumbar region. The lesions in all 8 fetuses were skin-covered. None had ventriculomegaly or an Arnold-Chiari malformation.
The prognosis for fetuses with spina bifida aperta is well documented in contrast to that for those with spina bifida occulta. The ability to identify a spinal dysraphic lesion pre-natally allows a more accurate assessment to be made of the likely outcome for an individual fetus.
Ultrasonography (US) of the spinal cord is performed in newborns with signs of spinal disease (cutaneous lesions of the back, deformities of the spinal column, neurologic disturbances, suspected spinal cord injury due to traumatic birth, and syndromes with associated spinal cord compression). The examination is performed with high-frequency linear- and curved-array transducers in the sagittal and axial planes from the craniocervical junction to the sacrum. Normal variants such as transient dilatation of the central canal and ventriculus terminalis can be demonstrated with US. US allows detection of congenital malformations, such as myelocele or myelomeningocele, spinal lipoma, dorsal dermal sinus, tight filum terminale syndrome, diastematomyelia, terminal myelocystocele, lateral meningocele, caudal regression syndrome, and hydromyelia or syringomyelia. Acquired intraspinal diseases following birth trauma and transient alterations after lumbar puncture can also be detected with US. US can demonstrate the entire spectrum of intraspinal anatomy and pathologic conditions with high geometric resolution. Therefore, US should be considered the initial imaging modality of choice for investigating the spinal cord in newborns.
We designed this study to elucidate the associated occult spinal lesions in patients with simple dorsal meningocele.
The study population was comprised of two groups. Group I comprised newly diagnosed patients with dorsal spinal meningocele, and group II comprised patients who had had surgery for meningocele and presented with progressive neurological deficits. Magnetic resonance imaging (MRI) scans of the whole spinal column were done. The associated spinal cord malformations were also treated at the same operation. There were 14 boys and 8 girls, with an age range from birth to 4 years (mean 3.9 months), in group I. Of 20 patients (90%) with associated spinal lesions, 6 had more than one lesion, excluding hydromyelia. Group II was made up of 6 patients who had been previously operated on for a meningocele and who presented with tethered cord syndrome. These were 4 boys and 2 girls, who ranged in age from 4 to 10 years (mean 6 years).
The level of the conus terminalis was lower than L3 in all patients. The other findings on MRI, besides low conus, were as follows: tight filum, split cord malformation, epidermoid, dorsal lipoma and hydromyelia.
Meningocele frequently camouflages a second, occult, spinal lesion. MRI of the whole spinal column should be performed. An intradural exploration performed with a microneurosurgical technique is needed to detect the fibrous bands that may lead to spinal cord tethering and to release the entrapped nerve roots. The other associated spinal anomalies should be operated on during the same operation.
We present a case of a fetal lumbar myelocystocele, a rare congenital malformation, characterized by herniation of the central canal through a bony spina bifida. Routine ultrasound examination at 11 weeks' gestation by the primary obstetrician showed a suspicious cyst on the fetal back. Initially, the suspected diagnosis was a meningocele. After sonographic detection of newly developed fetal brain anomalies at 22 weeks' gestation the patient was referred to us. The enlarged cyst, which floated freely in the amniotic fluid, had a funnel-like appearance and was covered by a very thin layer of skin. With the help of ultrafast fetal magnetic resonance imaging the diagnosis of a fetal myelocystocele was made.
To evaluate the clinical profiles, image findings and surgical outcome of 155 cases of spinal dysraphism.
155 patients were studied prospectively (143) or retrospectively (12). The male to female ratio was 1.5:1. Mean age at presentation was 5.7 years. Out of 155 cases of spinal dysraphism, 119 had open spina bifida [meningomyelocele (MMC) in 113 (72%), meningocele in 3 (2%) and myelocystocele in 3 (2%)] and 36 had occult spina bifida [split cord malformation (SCM) without overt MMC sac (pure SCM) in 29 (19%) and midline dermal sinus in 7 (4.5%)]. Lipomeningomyelocele constituted 73 of the 113 cases of MMC (65%). Twenty cases of MMC (18%) had associated SCM (complex spina bifida). The total number of cases with SCM was 49 (32%). Twenty-four children with MMC presented with an operative scar from previous surgery, performed at the periphery. After a detailed clinical evaluation of all patients, craniospinal MRI was the preferable mode of investigation. All patients underwent surgery and were clinically assessed over a mean follow-up period of 3.6 years.
MMC sac was the commonest skin manifestation seen in 89/155 cases (57%). Hypertrichosis and previous operative scar were noticed in 24 cases each (15%). Cutaneous hemangioma, skin tag and multiple neurofibroma were the other common superficial skin manifestations. Clinically, 103 patients (66.5%) had variable weakness of the lower limbs, and muscle atrophy was noticed in 56 cases (41%). Graded sensory loss, sphincteric dysfunction, trophic ulcer and backache were present in 89 (57%), 64 (36%), 17 (11%) and 9 cases (6%), respectively. Significant scoliosis in 56 cases (36%) and congenital talipes equinovarus in 51 cases (33%) were the most frequent neuroorthopedic deformities apart from high-arched foot, leg length discrepancy and flat foot. Common image findings were low-lying cord in 101 (65%), neural placode in 76/113 (49%), hydrosyrinx in 42 (27%), hydrocephalus in 71 (46%) and Chiari malformation in 62 cases (45%). The lumbosacral region in 56 cases (38%), followed by the lumbar region in 47 (30%), were the most common sites of occurrence of spina bifida. Of 71 hydrocephalic patients, 39 (55%) needed shunting before definitive surgery and 21 (30%) after the surgery. Eleven patients (15%) did not require shunting at all. CSF leak in 51 patients (33%), pseudomeningocele in 26 (17%), wound infection in 22 (14%) and meningitis in 13 (8%) were the most common postoperative complications. Two patients died in the postoperative period. During an average follow-up of 3.6 years (range 1.5-8 years), motor weakness improved in 47 children (45%) and remained static in 53 (52%), and 3 children showed deterioration in motor power. Sensory dysfunction improved in 43 (48%), remained static in 42 (47%) and deteriorated in 4 patients (4%). Sphincteric function clinically improved in 26 patients (41%) and was static in 38 (59%), and 6 patients (4%) had deterioration. Backache was dramatically relieved in all 9 patients with this complaint (100%). In 13 children with trophic ulcer (76%), it completely healed.
A significant proportion of patients with open spinal dysraphism harbor an underlying SCM, an entity coined complex spina bifida (in our previous study). The incidence of hydrocephalus in spinal dysraphism is low in India in comparison to the Western world. Some patients with mild hydrocephalus on scan may not require a shunt operation. Surgical intervention should be early after entire neural axis screening by MRI.