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PubMed1992

Foot deformities and occult spinal abnormalities in children: a review of 16 cases.

A retrospective clinical and radiologic study was made of 16 children with foot deformities and associated occult spinal abnormalities in a 3-year period. Eleven children had bilateral foot deformities; the deformities were unilateral in five. Midline cutaneous lesions of the back were noted in 13 children; the most common dermal sign was a hairy patch. All children had radiologic features of spinal dysraphism on combined computed tomography (CT) scan and myelogram. Spinal dysraphism was not considered in the initial assessment of four children. Children with foot deformity should therefore have a careful assessment of the spine, including a neurologic evaluation.

Child↗

PubMed1996

The role of somatosensory evoked potentials in the evaluation of spinal cord retethering.

Early detection of clinically significant spinal cord retethering is of paramount importance for the preservation of neurologic function in patients with repaired spinal dysraphic lesions. We retrospectively analyzed 90 children who were followed with serial peroneal somatosensory evoked potentials (SEPs) after a repair of their spinal dysraphic lesions with the objective of evaluating whether SEPs were a useful way of monitoring these children to facilitate early detection of clinically significant retethering. Three hundred and nine studies were performed on these children yielding a mean of 3.4 studies per patient. The median time between SEP studies was 13 months. A clinical examination was performed at the time each SEP was done. Sixty-one patients (68%) had a myelomeningocele; 25 (28%) had a lipomyelomeningocele while other types of dysraphic lesions were found in the remaining 4%. Nineteen patients (21%) had no change in serial SEPs; 7 of these 19 (37%) had a deterioration in clinical status while the SEPs were stable. Twenty-six patients (29%) showed improvement in serial SEPs, however, 3 of the 26 had clinical worsening at the time SEPs improved. SEPs deteriorated in 45 patients (50%); 13 of the 45 had an associated clinical deterioration, and the remaining 32 were stable clinically. Twenty-three patients (26%) had a clinical deterioration; of these only 13 had a corresponding deterioration in SEPs, 7 patients had stable SEPs while 3 had improvement in SEPs at the time of clinical deterioration. This results in a false-positive rate of 71% and a false-negative rate of 43%. We conclude that serial SEPs do not correlate well with clinical status and are not a useful modality for monitoring patients at risk for retethering.

Adolescent↗

PubMed2006

Spectrum of nonterminal myelocystoceles.

OBJECTIVE: To present the magnetic resonance imaging features, clinical findings, and possible embryologic bases for nonterminal myelocystoceles, a distinct subset of closed spinal dysraphisms. METHODS: We retrospectively analyzed imaging series and clinical records from five newborns and one older child with skin-covered soft tissue masses along the posterior midline spine. Spinal (6 patients) and brain (5 patients) magnetic resonance imaging was performed before surgical repair and compared with clinical findings, observations at surgery, and final lesion histology. RESULTS: The lesions affected the cervical (n = 3), thoracic (n = 2), and lumbar (n = 1) regions. In each case, the dome of the mass was covered by thickened, dystrophic epithelium with no subcutaneous fat, whereas the base and lateral walls of the mass were covered by normal skin. All patients were neurologically intact at presentation. In three cases, a stalk emanated from the dorsal normal spinal cord, crossed a narrow posterior bony spina bifida, and coursed through a posterior meningocele to attach to the inner aspect of its dome. The other three cases showed dissection of a hydromyelic cavity into the stalk, converting it into a second "cyst" within the meningocele. Concurrent anomalies included focal hydromyelia immediately cranial to the origin of the posterior stalk (n = 2), mild Chiari II malformation (n = 3), triventricular hydrocephalus from aqueductal stenosis (n = 1), filar lipoma (n = 1), and presumed neurenteric cyst (n = 1). At surgery, the sac was resected in all cases, but intradural exploration and untethering was performed in only three children. Embryologic considerations indicate that the spectrum of these lesions likely arises from partial limited closure of the neural tube, failed disjunction of the cutaneous ectoderm, and variable degrees of hydromyelia. CONCLUSION: The nonterminal myelocystocele is a distinct form of closed spinal dysraphism characterized by a skin-covered meningocele, which is either crossed by a fibroneurovascular stalk that extends from the dorsal aspect of the spinal cord to attach to the dome of the meningocele (abortive form, or myelocystocele manqué) or contains a hydromyelic cavity that is continuous with the ependymal canal of the spinal cord (complete form).

Abnormalities, Multiple↗

PubMed1996

Spinal segmental maldevelopment with a dermal sinus.

Segmental spinal dysgenesis is a rare form of spinal dysraphism. We report a case of segmental hypoplasia of the spinal cord extending from T6 to L1 vertebral levels, with a dorsal dermal sinus, diagnosed by spinal sonography and MRI and confirmed at surgery. The probable pathogenesis is suggested.

Diagnosis, Differential↗

PubMed1999

MR imaging of cerebrospinal fluid flow and spinal cord motion in neurologic disorders of the spine.

In summary, MR imaging of CSF and cord motion helps to evaluate diseases affecting cord and CSF motion and to identify the specific pathophysiology involved. A number of significant points have been made. First, MR imaging flow studies can be useful in evaluating CSF spaces and cystic diseases. Second, longitudinal and transverse motions occur in the spinal cord and CSF. Traveling wave motion occurs along the length of the spinal cord. Third, spinal cord tethering is associated with decreased cord velocity and loss of cord displacement at tethering site. Decreased transverse velocities occur with lateral cord tethering to the spinal canal. Fourth, in spinal dysraphism, longitudinal cord velocity is decreased by tethering, and is normal in asymptomatic patients with low conus. Normal cord motion helps to rule out possible tethering in symptomatic dysraphism with hydromyelia. Fifth, in acquired and nonmyelodysplastic symptomatic tethering, spinal cord motion is decreased. Sixth, in symptomatic cord compression, CSF flow and cord motion decrease, but recover after surgical decompression and after compensatory atrophy. Seventh, in asymptomatic spinal stenosis, cord motion is normal or increased. Diffuse spinal stenosis with cord atrophy leads to diffuse cord acceleration and prolonged cord caudal velocity, possibly related to the loss of the transverse mobility of the cord. Finally, focal spinal stenosis leads to focal dynamic cord deformation and can be associated with prominent intramedullary deformations. When compression is severe or symptomatic, cord motion is significantly decreased. Postoperative cases demonstrate good recovery of cord and CSF motion, unless compression or obstruction is still present.

Cerebrospinal Fluid↗

PubMed1993

Myelomeningocele and thick filum terminale with tethered cord appearing as a human tail. Case report.

Tail-like caudal appendages may be associated with spinal dysraphism, particularly with spinal lipomas or lipomyelomeningoceles. An unusual case is presented of a patient with a myelomeningocele and a thick filum terminale with tethered spinal cord, which presented with the external appearance of a human tail. A review of the literature reveals that human tails may be associated with dysraphic conditions. Extensive neuroradiological examinations should be performed for all such cases of apparent tails.

Abnormalities, Multiple↗

PubMed1998

[Congenital defects of the spinal cord and canal in neuroimaging].

Authors present the clinical classification of spinal dysraphisms and discuss all imaging modalities useful in the diagnosis: conventional X-rays, myelography, computed tomography, myelo-CT and magnetic resonance imaging. They precise the place of ultrasound in the prenatal and postnatal imaging of spinal dysraphisms.

Female↗

PubMed2006

Early start to therapy preserves kidney function in spina bifida patients.

OBJECTIVE: Renal scarring and renal failure remain life-threatening for children born with spinal dysraphism. We reviewed our data of spina bifida patients to evaluate whether optimal treatment of the neurogenic bladder from birth onwards can preserve kidney function. METHODS: We reviewed data on all newborns with spinal dysraphism who were referred to our hospital between January 1988 and June 2001. We looked at their situations at referral and at follow-up: the type of treatment, antimuscarinic agents, clean intermittent catheterisation (CIC), antibiotic prophylaxis, and operations (sling procedures, bladder augmentations, antireflux procedures). Renal function (ultrasound, DMSA scan, serum creatinin, creatinin clearance) and bladder function (urodynamic studies) were evaluated over time. RESULTS: Data of 144 children of 176 could be evaluated by the end of the study: 5 patients had pre-existing renal abnormalities, 69 had an overactive sphincter, 27 had reflux, and six had renal scarring. None are currently developing end-stage renal disease. All patients with spina bifida aperta started CIC and antimuscarinic therapy shortly after birth. Five of the six patients with renal scarring were started on therapy with intermittent catheterisation and antimuscarinic therapy several months after birth. Sixty-three of 82 children with spina bifida were dry at school age (age six), although 37 of these had not had an operation. CONCLUSION: We show that an early start to therapy helps to safeguard renal function for children born with spina bifida. Our data support other recent reports that children born with spina bifida can probably use their own kidneys for a lifetime, if they are given adequate urological treatment. To protect the upper urinary tract, we need to ensure low intravesical pressure by starting children early on CIC (the preferred treatment); antimuscarinic agents to counteract detrusor instability are indispensable in most cases. Proactive treatment of risks for upper tract deterioration results in a negligible loss of renal function, even when early urinary continence is included in the treatment protocol.

Antibiotic Prophylaxis↗

PubMed1991

Developmental disorders of the pediatric spine.

Developmental disorders of the pediatric spine are a group of congenital malformations commonly referred to as spinal dysraphism. These malformations are characterized by incomplete or absent fusion of midline mesenchymal, bony, and neural structures. They can be classified according to the presence or absence of an associated back mass. A detailed analysis of the embryology, clinical findings, and radiologic characteristics of the common forms of spinal dysraphism is outlined.

Humans↗

PubMed1996

Cervico-thoracic neurenteric cyst: clinicoradiological correlation with embryogenesis.

Two cases of cervico-dorsal neurenteric cysts are presented, one located dorsal and the other ventral to the spinal cord, with associated Klippel-Feil anomaly, meningocele, spinal dysraphism and possibly spinal cord malformation. One patient was operated on as a neonate for a cervico-thoracic meningocele and presented during adolescence with spastic quadriparesis. Imaging revealed spina bifida, a dorsally located neurenteric cyst and a tendency towards split cord malformation. The other child presented with cervico-thoracic kyphoscoliosis and paraparesis. Imaging demonstrated persistence of a cervico-thoracic remnant of the primitive neurenteric canal through the upper thoracic spine.

Child↗

PubMed1999

Spinal cord tethering associated with amniotic band syndrome.

Amniotic band syndrome (ABS) comprises fetal morphological abnormalities that may be associated with fibrous amniotic bands that damage developing fetal parts resulting in cutaneous scars, erosions and ulcerations, digital constricting bands, craniofacial and visceral anomalies. Multiple asymmetric encephaloceles and anencephaly are neural-tube-like defects previously reported with ABS. This is the first report of spinal dysraphism with dorsal spinal cord tethering associated with ABS. We examine the pathogenetic theories of ABS in light of this report.

Amniotic Band Syndrome↗

PubMed1997

Spinal abnormalities in classic bladder exstrophy.

OBJECTIVE: To determine the frequency and clinical sequelae of significant spinal malformations in children born with classic bladder exstrophy. PATIENTS AND METHODS: All patients evaluated or treated for classic bladder exstrophy at this institution were reviewed retrospectively. Radiographs or reports pertinent to the spine were retrieved and reviewed with a paediatric radiologist and all vertebral abnormalities categorized. Clinical charts of those with spinal anomalies were reviewed to determine any clinical neurological disorders associated with the radiographic findings. RESULTS: Of 423 patients with classic bladder exstrophy who were identified, 299 had radiographs or reports available for adequate review. Of these, 34 (11%) normal variants, e.g. spina bifida occulta and lumbarization or sacralization of vertebrae, were identified. Abnormalities of spinal curvature were identified in eight patients (2.7%), all with uncomplicated scoliosis. Spinal dysraphism was diagnosed in 12 patients (4%) and included myelomeningocele, lipomeningocele, scimitar sacrum, posterior laminal defects in two or more vertebrae, vertebral fusion and hemivertebrae. The one patient with myelomeningocele had clinical neurological dysfunction, giving an overall incidence of 0.3%. CONCLUSIONS: Spinal anomalies, excluding normal variants, occur in children born with classic bladder exstrophy at a rate of about 6.7%. The incidence of this association is much less than that for cloacal exstrophy. Although rare, neurological dysfunction can occur in the case of spinal dysraphism. Paediatric urologists and neurologists should be aware of this significant difference between patients with classic bladder and cloacal exstrophy to properly diagnose, evaluate and treat the attendant neurological problems.

Adolescent↗

PubMed1994

Preexisting neurogenic voiding dysfunction in children with imperforate anus: problems in management.

We evaluated 22 boys and 2 girls 1 month to 8 years old with imperforate anus to determine the relationship between neurogenic voiding dysfunction and bony sacral or spinal cord anomalies. Lower urinary tract function before anorectoplasty was normal in 12 children (group 1), abnormal (detrusor-sphincter dyssynergia) in 9 (group 2) and not evaluated in 3 (group 3). High lesions of imperforate anus were present in 22% of the patients in group 1 and in 100% of those in group 2. Plain radiography revealed partial sacral agenesis in 1 child in group 1 and 4 in group 2. Magnetic resonance imaging detected occult spinal dysraphism in 1 patient in group 1 and 2 in group 2. (Occult spinal dysraphism included sacral lipoma, tethered cord, syringomyelia and thick filum terminale.) Seven children in group 2 had vesicoureteral reflux before anorectoplasty. All children in group 2 were placed on clean intermittent catheterization for the management of neurogenic voiding dysfunction. Normal upper urinary tract function was maintained in all patients in group 1 and 6 in group 2. The remaining 3 children in group 2 had high grade reflux at presentation associated with severe renal damage or noncompliance with clean intermittent catheterization. These findings indicate that in children with imperforate anus lower urinary tract function should be evaluated before anorectoplasty because of the high incidence of associated congenital neurogenic voiding dysfunction and the potential risk for renal deterioration. When possible, neurogenic voiding dysfunction should be managed with clean intermittent catheterization to prevent renal damage.

Anus, Imperforate↗

PubMedDate not supplied

Prenatal spinal evaluation and functional outcome of patients born with myelomeningocele: information for improved prenatal counselling and outcome prediction.

OBJECTIVE: Prenatal ultrasonography can localize the level of the spinal cord malformation, allowing prediction of the potential postnatal neurological deficit and functional prognosis. METHODS: This study has two evaluations: (a) a retrospective prenatal review of 26 fetuses with spinal dysraphism (1987-1991), and (b) a follow-up descriptive study of patients (1971-1981) who underwent closure of the spinal lesion and ventricular shunting in the neonatal period. RESULTS: Prenatal ultrasound evaluation enabled the accurate definition of the last intact vertebral level which allows separation of fetuses into three functional groups (last intact level L2, L3-4, L5-sacral). Patterns of ambulation, urinary and bowel continence, and school performance vary according to level of spinal lesion and the neurological deficit. The need for ventricular shunts, the incidence of other spinal malformations and surgical interventions did not vary with the level of the spinal lesion. CONCLUSIONS: The functional outcome for patients with myelomeningocele is variable; however, distinct patterns emerge based on the level of spinal dysraphism and the resultant neurological deficit. By relating the level of the fetal spinal lesion to outcome data, more precise functional prognoses can be given to families.

Counseling↗

PubMed1993

[Overgrowth and DNA synthesis of neuroepithelium in embryonic stages of induced Long-Evans rat myeloschisis].

Overgrowth of the myeloschisis, namely the excessive amount of the neural plate tissue, has been reported in the human myeloschisis. However, it is still debatable how the overgrowth develops and whether the overgrowth is the cause, or the secondary effect of spinal dysraphism. The author induced myeloschisis in the fetuses of Long-Evans rats by the administration of ethylenethiourea (ETU) to pregnant rats on day 10 of gestation. The fetuses were removed 1 hour after the treatment with bromodeoxyuridine (BrdU) to the dams on day 14 and 21. The fetuses were fixed in alcohol and embedded in paraffin. H-E staining and the immunohistologic examination were performed on the staining patterns to anti-neurofilament (NFP), anti-glial fibrillary acidic protein (GFAP) and anti-BrdU antibody by ABC method. On day 14, the lateral portion of everted neural plate showed a loose arrangement of cells and there was rosette formation in the mesoderm. On day 21, cell necrosis was observed at the dorsolateral portion of myeloschisis, although the ventral portion showed almost normal cytoarchitecture and was positive to NFP and GFAP. The cause of myeloschisis in this model is supposed to be the local and direct cytotoxic effect of ETU to neuro-ectodermal junction. On day 14, control animals contained few BrdU-incorporated cells at the basal plate of neural tube. In contrast, everted neural plate showed an active uptake of BrdU diffusely in the subependymal matrix layer cells. Overgrowth was not yet identified. On day 21, overgrowth of myeloschisis was found in spite of a few positive cells to BrdU which was identical to the control animals. These findings seem to suggest that cells in the myeloschisis retain their ability of DNA synthesis for longer periods of development and overgrowth found on day 21 is possibly a secondary effect of spinal dysraphism in this model.

Animals↗

PubMed2006

Intradural extramedullary mature cystic teratoma: not only a childhood disease.

OBJECTIVE: The authors report on the first case of a spinal intradural extramedullary cystic teratoma in an aged patient. These lesions have been reported in adolescents and young adults often with a history of spinal dysraphism. They are believed to be congenital lesions; however, they have also been reported in patients with a history of posterior spinal surgery or lumbar puncture. METHOD: An 85-year-old man was evaluated for persistent and progressive lower extremity paresis. His symptoms began after relatively minor trauma. He had no prior lumbar surgeries. Neuroimaging studies revealed an L1-L2 intradural mass. A putative diagnosis of a cystic intradural extramedullary mass was made. A lumbar laminectomy and durotomy were performed. RESULTS: A cystic lesion containing hair follicles, cartilage, adipose, and neural tissue was encountered. Pathologic review corroborated the diagnosis of cystic teratoma. CONCLUSIONS: The occurrence of cystic teratomas in the absence of previous surgery or lumbar puncture is uncommon. Even rarer are reports of these lesions in aged patients. Of particular interest in this case is the fact that this patient had not undergone any previous lumbar procedures nor did he have a history of spinal dysraphism. Though rare, this entity should be included in the differential of cystic intradural spinal cord lesions.

Aged, 80 and over↗

PubMed2004

The "human tail": a rare cause of tethered cord: a case report.

STUDY DESIGN: Observational case report. OBJECTIVES: To report a case of dorsal cutaneous appendage or the so-called human tail that was the cause of tethered cord syndrome. SUMMARY OF BACKGROUND DATA: The dorsal cutaneous appendage, or so-called human tail, is often considered to be a cutaneous marker of underlying occult spinal dysraphism. In such cases, there is usually a separate underlying tethering lesion. There have been only three case reports in the literature where this appendage itself was the tethering lesion. The fourth such case is being reported. METHODS: An 11-month-old male child was brought for consultation for a "tail-like" structure in the low back since birth. Examination revealed a subtle thinning of the right lower extremity and a caudal appendage in the lower lumbar region. Plain radiographs revealed spina bifida at S1. MRI revealed a transitional lipoma at L5-S1 with a terminal syrinx. RESULTS: During surgery, a fibrous tract was seen extending from the base of the appendage through the defect in the bone and dura. The tract ended in the transitional lipoma of the cord at L5-S1. Sectioning of the tract and debulking of the transitional lipoma was done. After surgery, there was no change in the neurologic status of the patient. CONCLUSIONS: This case illustrates that the so-called "human tail" or the dorsal cutaneous appendage is not just a marker of underlying occult spinal dysraphism. In rare cases, the appendage itself can be the tethering lesion. In every case of dorsal cutaneous appendage, the surgeon should diligently search for the intraspinal extension of the lesion even if such an extension is not revealed by the MRI.

Fibrosis↗

PubMed2006

Skin-covered midline spinal anomalies: a report of four rare cases with a discussion on their genesis and milestones in surgical management.

BACKGROUND: The differential diagnosis for a dorsal midline mass presenting in a newborn encompasses a wide range of pathological conditions, including spinal dysraphisms, tumoral overgrowths including teratoma and hamartoma, disturbances in regression of fetal tail, and pseudotail formation. OBJECTIVES: To present (a) three rare cases of human tails and one case of tethered cord due to a midline anomaly resembling part of a human limb, (b) to discuss different kinds of clinical and pathological conditions which may be encountered in such newborns, (c) to hypothesize a theory about genesis of these congenital lesions, and (d) to remind drawbacks in preoperative evaluations and surgical management of these newborns. MATERIALS AND METHODS: Four newborns with dorsal midline malformations initially diagnosed as heterotopias are presented. Appropriate preoperative investigations and proper surgical interventions leading to resection of the lesions were performed. All four were skin-covered lesions containing well-differentiated cellular elements of fat, vascular, muscular, bony, and cartilaginous origin. Midline spinal dysraphism was detectable both pre- and intraoperatively only in one case in which cord untethering was also performed along with the first surgical intervention. In the other three "tailed cases," midline bone defect could not be detected in the available X-ray films; consequently, simple excision of the tail-like lesions was done. The first patient had to be explored for repeat untethering of the cord after 4 years, but the others have not yet developed any sign of tethering during an average period of 12 years follow-up. CONCLUSION: Morphological diagnosis of these lesions is not easy, and the attending pediatrician and neurosurgeon should be familiar with the differential diagnosis of such lesions and be prepared for possible time-consuming operation mandatory to achieve total resection of the lesion in a newborn under general anesthesia. Reviewing the possible theories regarding the genesis of such anomalies, it is hypothesized and suggested that all similar cases could have been of hamartomatous origin rather than defects of embryogenesis.

Dermatologic Surgical Procedures↗