Occult Spinal Dysraphism Research Articles

Sonographic Findings in a Neonate With Diastematomyelia and a Tethered Spinal Cord

Congenital midline cutaneous paraspinal lesions, mostly located in the lumbosacral areas, are widely recognized as markers of occult spinal dysraphism. Diastematomyelia, or a split spinal cord, is an unusual congenital spinal abnormality in which the spinal cord is divided into 2 columns by a fibrous, cartilaginous, or bony septum. In most cases, lesions are located on the lower thoracic and upper lumbar parts of the spine. It often manifests in the neonatal period with midline paraspinal lesions. Sonography is a reliable tool for the study of the most severe spinal dysraphism. Diastematomyelia has been reported in neonates; however, to the best of our knowledge, the sonographic findings of diastematomyelia have rarely been described. We report a case of diastematomyelia and a tethered spinal cord in a neonate with lower back hemangioma at birth that was diagnosed via spinal sonography and was confirmed by spinal magnetic resonance imaging (MRI).

Pedicle Subtraction Osteotomy for Adult Tethered Cord Syndrome With Lumbar Canal Stenosis: Report of Two Cases

ABSTRACTTethered cord syndrome with spinal lipoma is the most common form of occult spinal dysraphism. For the symptomatic patients, surgical treatment is recommended; however, there are many patients who have not been encouraged to seek medical attention until adulthood, since their symptoms are not severe enough to interfere with their daily activities. We performed pedicle subtraction osteotomy (PSO) to achieve indirect untethering and neural decompression in two senior patients with tethered cord syndrome, who showed deteriorating neurological condition due to coexisting lumbar canal stenosis. Here we report two patients (aged 56 and 60 years) who underwent PSO of L3 or L4. The pain disappeared and the bladder dysfunction recovered significantly after surgery. Complete bone union and untethering were achieved in both patients. PSO is an alternative surgical technique for senior patients with tethered cord syndrome caused by lumbosacral spinal lipoma, when the syndrome occurs along with lumbar canal stenosis.

Intradural Neurenteric Cyst—Two Case Reports of Surgical Treatment

ABSTRACTIntradural neurenteric cysts are rare congenital lesions and arise from incomplete separation of the developing notochord and foregut in the embryo. Neurenteric cysts are often seen in conjunction with other forms of occult spinal dysraphism. The cases of a 48-year-old male with pain in the right shoulder and numbness in both hands and a 7-year-old girl with subacute muscle weakness of the lower extremities are presented. Both patients underwent surgery. One lesion was completely excised, while the other could be only partially removed because of negative monitoring potential during the operation. Histological examination, showing pseudostratified ciliated columnar epithelium, confirmed the diagnosis of neurenteric cyst. The symptoms in both patients nearly disappeared after surgery. Recurrence of cyst was observed in the girl, though without neurological symptoms. In conclusion, two cases of intradural extramedullary cysts are reported. Clinical presentations, intraoperative findings, and histological features are discussed with a review of the literature.

Role of spinning top urethra in dysfunctional voiding

The role of spinning top urethra (STU) in children with dysfunctional voiding was evaluated retrospectively. From 1995 to 2002, the records of 154 children with dysfunctional voiding were reviewed retrospectively. Of the children 110 (71%) were girls and 44 (29%) were boys (mean age 8 years, range 4-14). All children were neurologically normal and no exhibited physical signs of occult spinal dysraphism. Patients were divided into two groups according to their width of proximal urethra: group I had STU and the group II had normal urethral width. The groups were compared with each other for gender, voiding symptoms, urinary tract infection (UTI), vesicoureteral reflux (VUR) and urodynamic observations. There were 84 children (mean age 8.3 +/- 2.2 years, range 4-14) in group I and 70 (mean age 8.0 +/- 2.1 years, range 4-14) in group II; no significant age difference was found between the two groups (p = 0.4674). Group I consisted of 66 (71%) girls and 18 (29%) boys and group II 44 (63%) girls and 26 (37%) boys. STU was observed more in girls than boys in group I (p = 0.0316). UTI was observed in 57 patients (68%) in group I and 34 (49%) in group II (p = 0.0154). Mean duration of symptoms was 42 +/- 24 months (range 6-118) and 39 +/- 23 (range 3-120) months in groups I and II, respectively (p = 0.6302). Postvoid residual urine (PVR) more than 10% of expected bladder capacity was detected in 15 patients (18%) in group I and seven (10%) in group II. No association was found between the meaningful PVR and STU (p = 0.1653). The presence of detrusor overactivity during filling was observed in 54 patients (64%) in group I and 42 (60%) in group II (p = 0.4676). Diminished bladder compliance (< 10 ml/cmH(2)O) was detected in 34 patients (40%) in group I and 17 (24%) in group II (p = 0.0335). The mean voiding pressure was measured as 56 +/- 29 cmH(2)O in group I, which was significantly higher than in group II (49 +/- 25 cmH(2)O) (p = 0.0373). The mean flow rate during the emptying phase of urodynamics was 16 +/- 8 and 15 +/- 6 ml/s in groups I and II, respectively (not significant, p = 0.2686). VUR was detected in 16 patients (19%) in group I and two (3%) in group II (p = 0.0018). STU was related to recurrent UTIs, VUR, poor bladder compliance and more serious functional urinary obstruction. Furthermore, STU may be a consequence of a neurogenic maturation defect in detrusor-sphincter coordination resembling that of urofacial syndrome, because development of this situation was found to be independent of the duration of symptoms.

Orthopaedic Deformities Associated With Lumbosacral Spinal Lipomas

Lipomeningocele is the most common cause of occult spinal dysraphism and spinal cord tethering. Children with this condition seem normal at birth except for cutaneous signs, and the initial complaints are usually musculoskeletal. We studied the orthopaedic deformities observed in this condition. We reviewed the medical charts of 159 patients with a diagnosis of lipoma of the lumbosacral spine that were examined in the Myelodysplasia Clinic over 25 years. Of these patients, 122 were treated by a single orthopaedic surgeon (L.D.) and were studied in detail. Of these 122 patients, 45 were over 15 years of age at the time of the final follow-up. Most patients had cutaneous stigmata. Foot deformities were the most common orthopaedic problems, followed by scoliosis. In patients over 15 years of age, the incidence of foot deformities was 44.2% (36 feet), with 20 feet requiring surgical treatment. The most common foot deformities were cavovarus, cavus, and equinocavovarus. In 70% of the surgical cases, good correction was achieved with only one procedure. Foot surgeries in patients under the age of 8 years were usually soft tissue procedures, and bony procedures were performed primarily in patients over the age of 11 years. Orthopaedic deformities are common at the initial presentation in patients with occult spinal dysraphism. A careful clinical examination with a high index of suspicion for spinal cord anomalies is indicated in all cases of spinal and lower extremity deformities. Foot deformities are very common and surgical treatment is usually successful. A thorough follow-up evaluation, including manual muscle strength testing, should be performed routinely to detect tethering of the cord in the early stages and to prevent worsening of the orthopaedic deformities. This was a retrospective case study. Level 4.

A Prospective Study of Cutaneous Manifestations of Spinal Dysraphism from India

Recognition of cutaneous markers of spinal dysraphism is important to prevent the morbidity associated with underlying spinal anomalies. To investigate the frequency and type of cutaneous stigmata in different forms of spinal dysraphism and to assess the role of ultrasonography and/or magnetic resonance imaging in diagnosing spinal dysraphism at two pediatric dermatology tertiary care centers. Over a 4-year period, all pediatric patients presenting to the dermatology clinic with dorsal midline cutaneous stigmata were evaluated clinically and with imaging studies (radiography, ultrasonographic examination and magnetic resonance imaging/Doppler). Surgical interventions were planned in conjunction with neurosurgery and orthopedic specialists. On examination, 245 (4.2%) had 285 cutaneous stigmata. Of the 180 patients evaluated with radiography, ultrasonographic examination and magnetic resonance imaging, 50 patients (28%) had spinal dysraphism (with 64 cutaneous stigmata). The most common stigmata associated with occult spinal dysraphism were lipoma (10) and dimples (12) and in open spinal dysraphism lipomeningomyelocoele (10) and meningomyelocoele (10). Statistically, lipomeningomyelocoele/myelomeningocoele, atypical dimples and port-wine stains were most associated with spinal dysraphism (p < 0.001). In 80 children less than 6 months of age, radiography with ultrasonographic examination revealed an SD in 16, while magnetic resonance imaging was diagnostic in four cases. Ultrasonographic examination performed fairly well in children less than 6 months and in cases of flat cutaneous stigmata it missed only 5% of cases, but in cases with bulky overlying masses (lipoma, hemangioma) it missed 15% of cases.

Spinal sonography in term neonates with cutaneous markers of occult spinal dysraphism: Results of our experience

Occult spinal dysraphism (OSD) refers to skin-covered lesions, characterized by incomplete fusion of the midline mesenchymal, bony or neural elements of the spine. 3–8% of the infants with cutaneous markers in the lumbo-sacral region have an underlying OSD. We report the occurrence of OSD in neonates with congenital midline lumbosacral cutaneous markers. Over a 3 year period, 240 neonates (125 m/115 f) were scanned between the first day of life and the ninth week (m: 54 days) with a high resolution linear transducer (7.5 MHz). Data collected included: indication for ultrasound examination (US), date of birth, date of US, age at US, findings of antenatal US and other findings where relevant. Abnormalities had already been detected antenatally in two cases. In 10/240 examined neonates (4.1%) we observed: spinal lipoma (n° 3) lipomyelomeningocele (n° 2), lipomyelocele (n° 1), meningocele (n° 1) lumbar dermoid cyst (n° 2), and hydromelia (n° 1). In 6/10 newborns (60%) with OSD there was a combination of two or more different skin lesions. In all infants, it was confirmed by MRI imaging. Surgical correction was performed at the end of the first year of life (m: 5 m). MRI is the gold standard of screening for OSD, but it is time consuming, costly and invasive. US revealed an effective, non invasive, inexpensive screening method, useful to detect OSD as long as the vertebral arch region is not completely ossified. A combination of 2 or more congenital midline skin lesions constitutes the strongest marker of OSD.

Prioritizing neurosurgical education for pediatricians: results of a survey of pediatric neurosurgeons

Pediatricians play a vital role in the diagnosis and initial treatment of children with pediatric neurosurgical disease. Exposure of pediatrics residents to neurosurgical diseases during training is inconsistent and is usually quite limited. After residency, opportunities for pediatricians' education on neurosurgical topics are few and fall mainly on pediatric neurosurgeons. The American Association of Neurological Surgery/Congress of Neurological Surgeons Joint Section on Pediatric Neurological Surgery Committee on Education undertook a survey of practicing pediatric neurosurgeons to determine whether focused education of practicing pediatricians might lead to better patient outcomes for children with a sampling of common pediatric neurosurgical conditions. An Internet-based 40-item survey was administered to practicing pediatric neurosurgeons from the US and Canada identified from the roster of the American Association of Neurological Surgeons/Congress of Neurological Surgeons Joint Section of Pediatric Neurological Surgery. Survey topics included craniosynostosis and plagiocephaly, occult spinal dysraphism and tethered cord, hydrocephalus and endoscopic third ventriculostomy, Chiari malformation Type I, mild or minor head injury, spastic cerebral palsy, and brain tumors. Most questions pertained to diagnosis, initial medical treatment, and referral. One hundred three (38%) of the 273 practicing pediatric neurosurgeons completed the survey. Two-thirds of the respondents had completed a pediatric neurosurgery fellowship, and two-thirds were in academic practice. Eighty-two percent of the respondents agreed that the care of pediatric neurosurgical patients could be improved with further education of pediatricians. In the opinion of the respondents, the 3 disease topics in greatest need of educational effort were craniosynostosis and plagiocephaly, occult spinal dysraphism and tethered cord, and hydrocephalus. Head injury and spasticity were given the lowest priorities. This survey identified what practicing pediatric neurosurgeons perceive to be the most important knowledge deficits of their colleagues in pediatrics. These perceptions may not necessarily be congruent with the perceptions of practicing pediatricians themselves; nevertheless, the data from this survey may serve to inform conversations between neurosurgeons and planners of continuing medical education for pediatricians, pediatrics residency program directors, and medical school pediatrics faculty.

Intérêt de l’échographie en ALR pédiatrique

The use of ultrasonographic guidance for regional anaesthesia has known recently a big interest in children in recent years. The linear ultrasound probes with a 25 mm active surface area (or probes with 38 mm active surface area in older children), with high sound frequencies in the range 8–14 MHz, allow a good compromise between excellent resolution for superficial structure and good penetration depths. In children, the easiest ultrasound guided blocks are axillar blocks, femoral blocks, fascia iliaca compartment blocks, ilio-inguinal blocks and para-umbilical blocks, caudal blocks. They permit a safe and easy learning curve of these techniques. The main advantage of ultrasound guided regional anaesthesia is the visualization of different anatomical structures and the approximate localization of the tip of needle. The other advantages for ultrasound guided peripheral nerve blocks in children are: faster onset time of sensory and motor block, longer duration of sensory blockade, increase of blockade quality and reduction of local anesthetic injection. The use of ultrasonographic guidance for central block allows to visualize different structures as well as spine and his content. Spinous process, ligament flavum, dura mater, conus medullaris and cerebrospinal fluid are identifiable, and give some information on spine, epidural space and the depth between epidural space and skin. At last, in caudal block, ultrasounds permit to evaluate the anatomy of caudal epidural space, especially the relation of the sacral hiatus to the dural sac and the search of occult spinal dysraphism. Benefit of this technique is the visualization of targeted nerves or spaces and the spread of injected local anaesthetic.

Occult spinal dysraphism: detection during ultrasound for epidural blockade in children

Occult spinal dysraphism is commonly asymptomatic and frequently associated with anorectal and genitourinary abnormalities. As neurological symptoms may develop due to distortion of the spinal cord and nerve roots as a baby grows, early detection is important to prevent neurological sequelae. There is a strong association between low spine anomalies and genitourinary and colorectal structures [1, 2]. Genitourinary, colorectal, and musculoskeletal systems are all of mesodermal origin and develop at the same time in the embryo, so that any insult inducing congenital vertebral malformations during organogenesis may also lead to congenital genitourinary or colorectal abnormalities [2]. If genitourinary anomalies are combined with VACTERL syndrome, there is a high incidence of tethered spinal cord [3]. In the series by Pierre-Kahn [4], 27% of patients with congenital spinal lipomas combined with visceral abnormalities, and 37.8% of these patients had genitourinary anomalies. Since the early 1980s ultrasound has been recognised as a screening test for occult spinal dysraphism in neonates and infants. Over the past 2 years, we have detected occult spinal dysraphism during ultrasound for epidural blockades in 4 of 500 children undergoing genitourinary surgery. The four children were aged 6–43 months and were undergoing hypospadias repair. Cases 1 and 2 had combined patent ductus arteriosus and atrial septal defect and case 3 had microtia and congenital thoracic scoliosis. A shallow hairy dimple or pit was seen in cases 1–3. Case 4 had no combined anomalies or cutaneous markers. On pre-puncture ultrasound using a high-frequency 8–13 MHz linear probe (LOGIQe™; GE Healthcare, Wauwatosa, WI, USA), low conus medullaris with thickened filum terminale were detected in four children. On MRI, a tethered spinal cord with lipoma of the filum terminale (cases 1, 3, and 4) and conus (case 2) were diagnosed (Fig. 1). Detethering of the spinal cord was performed 4–6 weeks postoperatively in cases 1–3. Low-lying conus medullaris (a) and thickened filum terminale (b) were showed on ultrasound. Low conus medullaris (c, left upper arrow) and lipoma of filum terminale (left, lower arrow; right) were showed on MRI. Our cases 1–3 showed shallow hairy dimples and pits. Studies have already shown that minor skin lesions, including shallow dimples, are possible normal variants in normal infants [5]. Nevertheless, occult spinal dysraphism should be considered if infants have cutaneous stigmata associated with congenital anomalies. While MRI is the most sensitive method for evaluating occult spinal dysraphism, spinal ultrasound is a simple and effective screening method for detecting abnormalities of the spine, including a low conus medullaris, a blunt conus medullaris, a thickened filum terminale, lipoma, fixed dorsal position of the cord in the thecal sac, and lack of pulsatile movement of the cord [6, 7]. Consequently, we recommend routine ultrasound prior to epidural blockade for postoperative analgesia in infants and children undergoing surgery with congenital anorectal and genitourinary anomalies.

Sevoflurane Provides Better Recovery as Compared With Isoflurane in Children Undergoing Spinal Surgery

Rapid recovery is desirable in pediatric neurosurgical patients to obtain an early neurologic assessment. We compared the recovery characteristics of 2 commonly used anesthetic agents, sevoflurane and isoflurane, under bispectral index-guided anesthesia in children undergoing spinal surgery. Eighty children who underwent surgery for occult spinal dysraphism at the lumbar and sacral level were randomized to anesthesia with sevoflurane or isoflurane in oxygen and nitrous oxide. Anesthesia depth was guided by a bispectral index monitor kept between 40 and 60. In addition to time at emergence, extubation and discharge, recovery (modified Aldrete) score, and hemodynamics were compared. The 2 groups did not differ significantly with respect to demographics, duration of surgery and anesthesia, and intraoperative hemodynamic changes. Extubation (6.4+/-3.3 vs. 10.7+/-4.6) and emergence (7.8+/-3.4 vs. 12.8+/-5.6) times (minutes) were significantly shorter with sevoflurane (P<0.001). Time (minutes) to achieve full Aldrete (modified) scores was less with sevoflurane (13.9+/-5.3 vs. 20.3+/-6.5) than isoflurane (P<0.001). However, the time (minutes) to achieve discharge criteria from postanesthesia care unit (140.7+/-49.3 vs. 146+/-43.3) and first dose of postoperative analgesic (60+/-24.1 vs. 72+/-33.4) in addition to incidence of postoperative agitation were similar in both groups (P>0.05). Sevoflurane results in an earlier recovery and assessment of modified Aldrete score when compared with isoflurane.

Effects of electrotherapy in treatment of neurogenic bladder in children with occult spinal dysraphism

Neurogenic bladder can develop as a result of various degrees of neurogenic lesion in spina bifida. The degree of bladder dysfunction depends on the level and type of spina bifida. Due to results upon complete diagnostic protocols, treatment options are applied. Comparison of therapy results of patients with occult spinal dysraphism with neurogenic bladder that underwent medicamentous therapy and medicamentous with electrotherapy treatment. We had 49 patients with neurogenic bladder that were treated at the University Children's Hospital in Belgrade in the period 2003-2008. The first group of children received medicamentous therapy and the second group received medicamentous therapy with transcutaneous electric nerve stimulation. In both groups we evaluated 4 symptoms: daily enuresis, enuresis nocturna, urgency and frequency and 4 urodynamic parameters: lower bladder capacity, unstable contractions and residual urine and detrusor sphincter dyssynergia. Follow-up urodynamic evaluation was done after 3, 6 and 12 months respectively. Our findings pointed out a high statistical significance of improvement in all evaluated urodynamic parameters of neurogenic bladder (predominantly in bladder capacity) in the group of children with combined therapy as well in resolution of symptoms (predominantly enuresis nocturna, urgency and frequency). Combined therapy is more efficient in treatment of children with neurogenic bladder. Electrotherapy is non-invasive, easily applicable and has had a significant place in treatment of children with dysfunctional voiding.

Gluteal pseudophallus in a male child: A rare cutaneous marker of occult spinal dysraphism

Congenital midline paraspinal cutaneous markers have been practically linked to the location and nature of neural-tissue lesions. One of the most interesting congenital midline paraspinal cutaneous markers has been the human tail in the lumbosacral region, with underlying spinal dysraphism. Human tails have many shapes and sizes and are usually localized to the lumbosacral region. After a complete neurological examination, the MRI is the most sensitive diagnostic modality to reveal the underlying occult spinal dysraphic state. Surgical excision is aimed at untethering of the spinal cord in symptomatic children and for aesthetic reasons in asymptomatic patients. Here we report an asymptomatic male child with normal external genitilia, whose tail is attached to the gluteal region like an adult phallus and investigations revealed an underlying spinal dysraphic state.

Double Lumbosacral Lipomas of the Dorsal and Filar Types Associated With OEIS Complex -Case Report-

A female baby was born at 37 weeks and 6 days gestation by vaginal delivery with omphalocele, exstrophy of the cloaca, and imperforate anus, indicating the presence of OEIS complex, a rare combination of defects consisting of omphalocele (O), exstrophy of the cloaca (E), imperforate anus (I), and spinal deformity (S), associated with lumbosacral lipoma. The most common associated spinal deformity is terminal myelocystocele, and spinal lipoma is rare. Constructive interference in steady-state magnetic resonance imaging clearly revealed double lipomas, a dorsal-type lipoma, located dorsal to the low-lying conus medullaris, and a filar-type lipoma, revealed by a thickened and fatty filum terminale. After recovery from abdominogenital repairs, debulking of the dorsal-type lipoma and untethering of the spinal cord by sectioning of the filar-type lipoma were performed at the age of 14 months. Neurosurgical treatment for occult spinal dysraphism should be undertaken after recovery from the initial series of major abdominogenital procedures.

Intraspinal Neurenteric Cysts in Children

Neurenteric cysts are rare congenital epithelium-lined cysts of the central nervous system. They are found predominantly in the spinal cord, with lower incidence in the intracranial compartment, and may be associated with various other congenital spinal anomalies. Seven patients with symptomatic intraspinal neurenteric cysts are presented. Seven patients with intraspinal neurenteric cysts aged from nine months to ten years treated at this hospital from May 2000 to July 2006 were reviewed. The clinical manifestations, imaging and surgical findings of patients were analyzed retrospectively. All patients underwent operation. One patient's cervical neurenteric cyst was resected using the lateral cervical approach, and the other six resections were performed with posterior approach. All seven patients presented with neurological involvement. One patient had an intramedullary cyst, while the other six cysts were situated ventrally. Three patients' cysts occurred in the cervical region, two in the cervicothoracic region, one in the thoracic region and one in the lumbar region. One patient had bony anomalies, and one had a lumbar posterior occult spinal dysraphism. Five patients' symptoms improved rapidly after surgery. Intraspinal neurenteric cysts in children are rare and most occur ventral to the spinal cord. Magnetic resonance imaging (MRI) is the most effective imaging modality. Earlier diagnosis and surgical resection of spinal neurenteric cysts improves prognosis.

The human tail: rare lesion with occult spinal dysraphism—a case report

Human tail or tail-like caudal cutaneous appendage is a rare fingerlike, midline protrusion at the lumbosacrococcygeal region, often associated with occult spinal dysraphism. A 2-month-old male child presented here had a lumbosacral tail-like appendage with underlying spinal dysraphism without any appreciable neurological deficit. In contradiction to a previous report, true vestigial tails are not benign because they may be associated with underlying dysraphic state. About 50% of the cases were associated with either meningocele or spina bifida occulta. Management of such lesions must include complete neurological history and examination as well as magnetic resonance or computed tomographic imaging. After diagnosis, microsurgery should be performed if there is any intraspinal component to avoid any damage and neurological deficit.

A rare cutaneous sign of occult spinal dysraphism with tethered spinal cord

©2008, World J Pediatr. All rights reserved. A female, born after normal pregnancy and labor, was referred for consultation for a 1-cm long fi brous appendage in the midline of her lumbosacral region one day after birth (Fig. 1). Although she was neurologically intact, computed tomography at 3 months revealed L4-L5 spina bifi da (Fig. 2A), and magnetic resonance imaging (MRI) showed a low conus medullaris level (L3), and a tethered dorsal spinal cord that seemed to connect with the site of the skin lesion (Fig. 2B). Spina bifi da occulta with tethered spinal cord due to a dermal sinus tract was diagnosed and the patient was treated surgically. At operation, the sinus tract extending from the skin to the spinal cord was observed, but the thickening of the fi lum terminale was not evident. Occult spinal dysraphism with tethered spinal cord, if not recognized, may result in permanent neurologic defi cits. Moreover, the neurological disturbances can be diffi cult to demonstrate in a young baby and clinical evaluation of bladder function is equally diffi cult at this age. To prevent serious sequelae, early screening of high-risk infants with cutaneous markers that present in 50%-90% of patients, radiologic diagnosis using X-rays, either MRI or sonography, and urodynamic testing, and the appropriate timing of surgical untethering should be performed. However, dorsal cutaneous lesions may be ignored unless they are common lesions such as midline hemangiomas, hairy patches, dimples or sinuses, or pigmented macules. In order to screen effectively, the physician must have a high degree of suspicion for such a rare skin abnormality as this case. References 1 Guggisberg D, Hadj-Rabia S, Viney C, Bodemer C, Brunelle F, Zerah M, et al. Skin markers of occult spinal dysraphism in children: a review of 54 cases. Arch Dermatol 2004;140:1109-1115. 2 Cornette L, Verpoorten C, Lagae L, Van Calenbergh F, Plets C, Vereecken R, et al. Tethered cord syndrome in occult spinal dysraphism: timing and outcome of surgical release. Neurology 1998;50:1761-1765. 3 Yamada S, Won DJ, Siddiqi J, Yamada SM. Tethered cord syndrome: overview of diagnosis and treatment. Neurol Res 2004;26:719-721. Received March 31, 2008 Accepted after revision May 8, 2008 A rare cutaneous sign of occult spinal dysraphism with tethered spinal cord

Syringo-subarachnoidal shunting to correct unilateral leg deformity in a child with terminal syringomyelia: case report

Among cases with terminal syringomyelia, 25% are associated with tethered cord syndrome. As it can be difficult to determine whether the neurological deficits are attributable to a syrinx or to a coexisting occult spinal dysraphism, it is not easy to determine the correct surgical strategy. We report a 19-month-old girl with an underdeveloped right leg and pes varus detected when she was 1 month old; lumbosacral magnetic resonance imaging (MRI) revealed syringomyelia. She developed recurrent urinary tract infections and consulted our department with a diagnosis of congenital neurogenic bladder. She presented with clubbed equine position, wore a short brace on her underdeveloped right leg, and exhibited limping gait due to shortening of the right leg. There was no anal reflex. The skin on her lower back was normal. MRI study showed that the lower end of the conus medullaris existed at the L3/4 level; central-type syringomyelia was recognized in the conus medullaris at the T12/L1-L2/L3 level. Computed tomography myelography detected no trabeculae causing tethering effects or influx of contrast medium into the syrinx. There was no comorbid disease like hydrocephalus or Chiari malformation. We performed syringo-subarachnoidal shunt by L1-L2 hemilaminectomy. Postoperative MRI confirmed shrinkage of the syrinx. One year later, although her perianal sensory loss and bowel and bladder dysfunction remained, her right leg had caught up with the left and at 1 year and 9 months after the procedure there is no discrepancy in her legs and she is able to run without limping.

The tale of a tail

Human tail refers to a congenital cutaneous appendage protruding at the lumbosacral region. This interesting anomaly is often associated with occult spinal dysraphism and presents with treatment dilemma. Management of such lesions must include complete neurological examination and magnetic resonance imaging. Appropriate management including early diagnosis and microsurgical intervention can prevent development or progression of severe neurological deficits in later life. In this article, three cases of human tails are reported along with their successful treatment and review of the literature.

Occult Spinal Dysraphism and Urolithiasis: Are Patients at Higher Risk of Stone Disease?

Spinal dysraphism is associated with urinary-tract dysfunction in severe cases such as meningomyelocele, in part because of incomplete innervation of the lower urinary tract. Patients with meningomyelocele are at higher risk for stone formation, possibly secondary to stasis of urine from aberrant storage and emptying. However, minimal data exist on the risk of stone formation in patients with milder forms of spinal dysraphism. The purpose of this study was to examine whether an association exists between urolithiasis and occult spinal dysraphism (OSD). All patients who underwent a non-contrast CT scan of the abdomen and pelvis during a 4-month period were included. The final dataset consisted of 374 consecutive patients (195 men, 179 women) with a mean age of 54.2 years (range 18-95 years). Scans were reviewed for evidence of urolithiasis and, independently, for skeletal abnormalities. Patients with urolithiasis included those with a kidney, ureteral, or bladder stone(s). Patients with OSD included those with bifid lumbosacral bony elements, posterior arch defects, or incomplete fusion or non-fusion of S1, S2, or S3. The association between urolithiasis and OSD was examined by calculating series of crude and adjusted odds ratios (ORs) with corresponding 95% confidence intervals (CIs). Of the 374 patients, 135 (36.1%) had urinary calculi, and 83 (22.2%) had OSD. No relation was found between the prevalence of OSD and stone disease (OR 1.22; 95% CI 0.72, 2.08), even after adjusting for potential confounders. Unlike severe spinal dysraphism, OSD does not appear to confer an increased risk of stone disease.