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PubMed2004

Terminal syringomyelia: is it as innocent as it seems?--Case report.

A 5-day-old newborn girl presented with cutaneous signs of hypertrichosis, skin dimple, and subcutaneous lipoma in the lumbar region. Magnetic resonance (MR) imaging of the thoracolumbar spine demonstrated spinal dermal sinus, type II split cord malformation, and tethered cord. A small terminal syringeal cavity was also present. Five months after the diagnosis, weakness in dorsal flexion of the bilateral feet and toes was detected. Surgery to untether the cord was performed. Her deficits resolved completely. Nearly 4 years later, she was admitted with weakness in dorsal flexion of the right foot and toe. MR imaging of the spine showed holocord syringomyelia, postoperative changes, and tethered cord. No Chiari malformation was seen. Syringosubarachnoid shunt placement was performed. Terminal syringomyelia is common in cases of occult spinal dysraphism, but the natural history and management remain unclear. Progression, regression, or persistence of terminal syringeal cavity can occur whether untethering is performed or not. Progression of the terminal syrinx occurred after tethered cord release and the enlargement of the cavity involved the entire spinal cord in the present case. Terminal syringomyelia requires close follow up for a long time even in asymptomatic cases. Surgical treatment should be considered in symptomatic cases.

Female↗

PubMed2004

Tethered cord syndrome in childhood: diagnostic features and relationship to congenital anomalies.

Tethered Cord Syndrome (TCS) is a stretch-induced functional disorder of the spinal cord that often develops and presents in childhood in association with spinal dysraphism. While the subtlety with which TCS can present makes it challenging to diagnose, awareness of the common neurological, musculoskeletal and urologic symptoms are of great value to the clinician, and can aid timely referral for neurosurgical evaluation. This article reviews these symptoms, as well as the clinical and radiological findings of the most common dysraphic conditions associated with TCS.

Cauda Equina↗

PubMed2002

Ultrasound of the spinal cord in children: its role.

This article covers the indications for spinal ultrasound including its advantages and disadvantages in comparison with spinal MR. We describe the features of a normal spinal ultrasound and those features which may be found in infants with spinal dysraphism.

Child, Preschool↗

PubMedDate not supplied

[Diplomyelia and diastematomyelia. Case reports].

Diplomyelia and diastematomyelia. Case report. Three cases of diplomyelia [1] and diastematomyelia [2] were studied and treated between 1980 and 1990 years. All patients had symptomatic onset in adulthood. Pathological features were caused by localization on the lesion in cervical, thoracic and lumbar segment of the spinal cord. Plain X-rays showed a variety of osseous malformations associated with spinal dysraphism. The most usefull radiographic examination was computerized tomography (CT). Indications to surgical treatment are discussed.

Adolescent↗

PubMedDate not supplied

Diastematomyelia with terminal lipomyelocystocele arising from one hemicord: case report.

Diastematomyelia is an uncommon spinal dysraphism with midline dorsoventral clefting of the spinal cord. Formation of syrinx cavity within the hemicord, vertebral anomalies and lipomas is known to be associated with diastematomyelia. We present an unusual case of a 4-month-old girl, who presented with diastematomyelia and terminal myelocystocele, and dilatation of the terminal portion of the spinal canal with formation of a cystic structure. Findings were confirmed by subsequent surgery and histopathology. Ectopic right kidney, Chiari I malformation and changes of partial sacral agenesis were other findings noted in this case. No case report of formation of terminal myelocystocele arising from one hemicord was described in the literature we reviewed.

Abnormalities, Multiple↗

PubMed2003

An infant with an intradural lipoma of the cervical spine extending into the posterior fossa.

BACKGROUND: Intradural lipomas of the cervical spine are very rare. These tumours show no association with spinal dysraphism. We describe an infant with cervical spinal cord lipoma. Surgical decompression of the cord resolved all neurologic deficits. CASE DESCRIPTION: An 8 month old female infant presented with retarded development of motor function in the limbs, in addition to dysphagia. Computed tomography identified a tumour of fat density that extended from the medulla to C7. Magnetic resonance imaging showed hyperintensity on both T1- and T2-weighted images. A fat-suppression sequence demonstrated an area of signal enhancement in the dorsal portion of the tumour following administration of gadolinium. The adjacent spinal cord was normal. Partial removal of the tumour was performed together with decompressive laminoplasty. The enhancing region proved to be fibrous tissue. Motor development resumed in the week following operation. CONCLUSION: Retarded motor development was the main manifestation of this infant's rare spinal tumour. Neuroimaging was of considerable diagnostic value; in particular, fat-suppression magnetic resonance imaging demonstrated details of the tumour and surrounding structures. Decompressive laminoplasty and laminectomy with partial removal of the tumour was effective in reversing clinical deficits.

Cervical Vertebrae↗

PubMed1979

Transdiaphragmatic jejunal duplication: a report of five cases.

Five infants with intrathoracic transdiaphragmatic duplication cysts are presented. Diagnosis was possible from the combination of several features on plain chest radiographs: (a) right-sided cystic areas lying posteriorly in the chest, (b) lower cervical or upper dorsal spinal dysraphism, and (c) unusual gas collection beneath the right hemidiaphragm. Barium studies, positive-contrast myelography, and computed tomography were helpful in preoperative assessment.

Cysts↗

PubMed1999

Segmental spinal dysgenesis: neuroradiologic findings with clinical and embryologic correlation.

BACKGROUND AND PURPOSE: Segmental spinal dysgenesis (SSD) is a rare congenital abnormality in which a segment of the spine and spinal cord fails to develop properly. Our goal was to investigate the neuroradiologic features of this condition in order to correlate our findings with the degree of residual spinal cord function, and to provide insight into the embryologic origin of this disorder. We also aimed to clarify the relationship between SSD and other entities, such as multiple vertebral segmentation defects, congenital vertebral displacement, and caudal regression syndrome (CRS). METHODS: The records of patients treated at our institutions for congenital spinal anomalies were reviewed, and 10 cases were found to satisfy the inclusion criteria for SSD. Plain radiographs were available for review in all cases. MR imaging was performed in eight patients, one of whom also underwent conventional myelography. Two other patients underwent only conventional myelography. RESULTS: Segmental vertebral anomalies involved the thoracolumbar, lumbar, or lumbosacral spine. The spinal cord at the level of the abnormality was thinned or even indiscernible, and a bulky, low-lying cord segment was present caudad to the focal abnormality in most cases. Closed spinal dysraphisms were associated in five cases, and partial sacrococcygeal agenesis in three. Renal anomalies were detected in four cases, and dextrocardia in one; all patients had a neurogenic bladder. CONCLUSION: SSD is an autonomous entity with characteristic clinical and neuroradiologic features; however, SSD and CRS probably represent two faces of a single spectrum of segmental malformations of the spine and spinal cord. The neuroradiologic picture depends on the severity of the malformation and on its segmental level along the longitudinal embryonic axis. The severity of the morphologic derangement correlates with residual spinal cord function and with severity of the clinical deficit.

Coccyx↗

PubMed1998

Pediatric neurosurgical disease.

The most common, primary referrals to a pediatric neurosurgeon's office are the evaluation and management of the child with a large head (to rule out hydrocephalus and other space occupying lesions) a mishappen head (to rule out various forms of craniosynostosis), or some form of congenital spinal abnormality (spinal dysraphism). The authors discuss the pathogenesis and clinical features of these disorders, provide a framework for diagnostic evaluation and referral, and discuss the various treatment options available for each.

Child, Preschool↗

PubMed1995

MR of terminal myelocystoceles.

PURPOSE: To delineate the clinical and MR findings in children with an unusual type of spinal dysraphism, the terminal myelocystocele. Infants with a terminal myelocystocele carry a favorable neurologic prognosis if the entity is diagnosed early. Understanding the MR characteristics of this entity will allow for earlier and more accurate diagnosis. METHOD: Analysis of the medical charts and MR studies in 15 children with surgically and histologically proven myelocystocele. RESULTS: In all 15 children, MR demonstrated the primary findings of a terminal cyst of the central canal of the spinal cord which is tethered and herniated with arachnoid and cerebrospinal fluid through an area of spinal dysraphia onto the back as a mass. Of these children, 10 had additional findings (one or more) on MR of Chiari I (five cases), Chiari II (one case), cervicothoracic hydromyelia (two cases), lumbar hydromyelia (two cases), hydrocephalus (2 cases) segmentation anomalies of vertebrae (3 cases) and partial agenesis of sacrum (six cases). Of the clinical findings, all 15 children had a back mass, 10 also had cloacal exstrophy. One had imperforate anus, 10 were girls, five had ambiguous genitalia and all were neurologically intact. CONCLUSION: Children with a terminal myelocystocele present with a back mass and there is a high association with cloacal exstrophy. MR is the best noninvasive modality to diagnose all of the components of a terminal myelocystocele and the associated central nervous system findings.

Anus, Imperforate↗

PubMed2003

Neurological presentation and long-term outcome following operative intervention in patients with meningocele manqué.

We review and describe the neurological presentation and long-term outcome of patients with meningocele manqué, and describe the dysraphic features associated with this entity. Our series of patients was collected over a 25-year period with a mean follow-up of 11.5 years. The mean age of presentation was 10.4 years and 72% of the patients presented with an abnormal neurological examination. At most recent follow-up, symptoms were stable, improved, and progressed in 47, 37 and 16%, respectively. Meningocele manqué was associated with focal hirsutism in 37% of the cases. These bands were primarily found in the lumbar spine and involved two sequential vertebral levels in 42% of cases. Sectioning of meningocele manqué has good long-term results in the majority of patients. These bands should be sought in the evaluation of patients with spinal dysraphism and surgically transected.

Adolescent↗

PubMed1975

[Caudal vertebro-medullary dysrhaphia. Intra-sacral forms].

The mean theories of the sacral spinal dysraphisms are reviewed and 4 cases of "meningeal intrasacral diverticulum" are reported. The authors prefer this term to the more common "intrasacral occult meningocele". The proeminent symptom in their cases was late vesical incontinence, which disappeared after surgery. In the pertinent literature 24 similar cases were described till now.

Adolescent↗

PubMed2005

Floating-Harbor syndrome complicated by tethered cord: a new association and potential contribution from growth hormone therapy.

Floating-Harbor syndrome is a rare syndrome with short stature, severely delayed bone age, typical facies and delay in expressive speech. Structural malformations are uncommon, and tethered cord or other forms of spinal dysraphism have not previously been reported. We report on a case of Floating-Harbor syndrome, complicated by tethered cord and discuss the possibility that growth hormone therapy may contribute to the development of symptoms of this malformation.

Abnormalities, Multiple↗

PubMed1984

Computed tomography of sacral and perisacral lesions.

The sacrum and perisacral soft tissues are not readily amenable to investigation by conventional radiological techniques. Computed tomography (CT) has provided an excellent imaging modality for the evaluation of these areas. This article describes the normal CT anatomy of the sacrum, the CT technique for evaluating the sacrum and perisacral soft tissues and the contribution of CT to the assessment of the many disorders that may involve the sacrum. Conditions discussed include osseous tumors of the sacrum, spinal dysraphism, cauda equina tumors and cysts, lesions arising primarily in the presacral space and sacral trauma. The use of CT scanning of the sacrum following intrathecal administration of Metrizamide is described.

Adolescent↗

PubMed2005

Terminal myelocystocele.

Myelocystocele is a variety of occult spinal dysraphism that is characterized by a localized, cystic dilatation of the terminal part of the central canal that then herniates through a posterior spina bifida. We describe a series of eight cases of myelocystocele. The clinical and radiological features and treatment are discussed and the relevant literature reviewed.

Child, Preschool↗

PubMedDate not supplied

Magnetic resonance imaging of the pediatric spine.

Magnetic resonance is an excellent modality for imaging the pediatric spine. Its successful use requires understanding both the basic physics and the sedation protocols necessary for acquiring high-resolution images. Interpreting the images accurately depends on appreciating the differences between the normal anatomy of the pediatric and the adult spine. Evaluating the images requires familiarity with the differential diagnosis of pediatric spine disease, including the most common processes (infections, neoplasms, and trauma) as well as spinal dysraphism. Despite the acknowledged usefulness of magnetic resonance imaging of the pediatric spine, controversies remain related to its safety in this age group and its limitations in diagnosing and evaluating scoliosis and tethered cord syndrome.

Adolescent↗

PubMedDate not supplied

Spinal neurenteric cyst. Report of 4 cases and review of the literature.

Neurenteric cysts are rare congenital lesions of the spine that are lined with entodermal epithelium. We describe 4 unusual cases of neurenteric cysts in patients aged between 3 and 16 years. The cyst was situated anteriorly in 3 patients and posteriorly in 1. All the patients had varied clinical and radiological features. The child with dorsal neurenteric cyst had stigmata of spinal dysraphism along with other vertebral anomalies. Another child had an associated syringomyelia along with evidence of severe arachnoiditis. All 4 patients underwent laminectomy and total microsurgical excision of the cysts through a posterior approach. The patient with diastematomyelia developed early recurrence of the cyst and underwent reoperation. Three patients showed an excellent recovery after surgery. The child with arachnoiditis failed to recover neurologically.

Adolescent↗

PubMedDate not supplied

Neurogenic bladder: urodynamic and surgical aspects.

Children with spina bifida often have vesico-urethral dysfunction manifesting either as upper urinary tract deterioration or voiding dysfunction, chiefly incontinence. Surgery of the back and presence of or increase in hydrocephalus may contribute to neuro-urologic worsening; secondary cord tethering and syrinx or hydromyelia may be additional factors coming into play later in life. Urodynamic assessment using simple modalities like uroflowmetry, external sphincter EMG, residual urine volume and cystometry provide data useful to classify patients according to detrusor and sphincter activity. Besides diagnosis, urodynamic studies are useful in guiding therapy of children with vesico-urethral dysfunction, and for their follow-up to detect sub-clinical deterioration. They also help to prognosticate risk of upper tract deterioration and the possible success of measures to contain incontinence. Urodynamic data in thirty one patients with spinal dysraphism who presented to us with urologic symptoms were analysed. Twenty-three children had hyper-reflexic bladders while in the other 8 the bladder was areflexic. 13 children showed upper tract dilatation. The leak point volume was significantly lower in this group of patients compared to those who did not show upper tract dilatation. Our results are comparable to earlier similar studies.

Adolescent↗