Occult Spinal Dysraphism
January 2005 - March 2005
Occult spinal dysraphism should be suspected in any child who has unusual markings on the back, like tufts of hair, dimples or hyperpigmented patches. This article notes that not all children with these markers have spinal abnormalities. Early surgery to prevent neurological damage to the spinal cord is recommended in most instances. In order to avoid missing a case, radiologic investigation of children with these markers should be performed, even if some studies will be negative.
Robinson AJ, Russell S, Rimmer S. The value of ultrasonic examination of the lumbar spine in infants with specific reference to cutaneous markers of occult spinal dysraphism. Clin Radiol 2005 Jan;60(1):72-7.
Diagnosis of underlying problems in occult spinal dysraphism may be made using ultrasound or MRI scanning. Increasingly these diagnoses are being made prenatally. A child who has two or more markers on the back like tufts of hair, dimples or hyperpigmented patches is more likely to have an underlying spinal problem requiring treatment. In order to avoid missing a case, radiologic investigation of children with these markers should be performed, even if some studies will be negative.
Scatliff JH, Hayward R, Armao D, Kwon L. Pre- and post-operative hydromyelia in spinal dysraphism. Pediatr Radiol 2005 Mar;35(3):282-9
Hydromyelia is fluid in the center of the spinal cord that occurs commonly in meningomyelocele. This article notes that it often is found in babies after their backs are closed; it may be associated with symptoms and require surgical treatment.
April 2005 - June 2005
Occult spinal dysraphism (OSD), a condition present at birth, is often missed by clinicians until the child has developed irreversible neurological losses. This article reiterates the importance of evaluating babies who have marks on their back including hairy patches, and unusually colored spots.
Jaiswal AK, Mahapatra AK. Terminal myelocystocele. J Clin Neurosci 2005 Apr;12(3):249-52
This articles reviews myelocystocele, which is a type of spina bifida that is characterized by a localized, cystic dilatation of the terminal part of the central canal that then herniates through a posterior spina bifida. It can be associated with syringomyelia, Chiari-II malformation and hydrocephalus.
October 2005 - December 2005
A review of adult complex spinal dysraphism.
Kim K, Isu T, Matsumoto R, Miyamoto M, Isobe M. Unilateral spondylolysis with spina bifida occulta of lumbar spine--case report and review of the literature. No Shinkei Geka. 2005 Nov;33(11):1119-23. Japanese.
Unilateral spondylolysis and midline defects in the posterior spinal elements attributable to spina bifida occulta (SBO) result in the unilateral floating of the vertebral arch. We report a patient with unilateral spondylolysis associated with SBO in the lumbar spine and review the literature.
Takeyama J, Hayashi T, Saito M, Shimanuki Y, Watanabe M, Sasano H, Shirane R. Spinal hamartoma associated with spinal dysraphism. Childs Nerv Syst. 2005 Dec 3;:1-5
The aims of the study were (1) to review the pathological findings of spinal lipomatous masses associated with congenital spinal dysraphism and (2) to discuss the pathological diagnosis. It concluded that Spinal tumorous lesions associated with spinal dysraphism have been traditionally described as lipoma since they are composed mostly of fatty tissue.
James HE, Lubinsky G. Terminal myelocystocele. J Neurosurg. 2005 Nov;103(5 Suppl):443-5.
The authors describe the cases of eight patients with terminal myelocystoceles and report on their initial diagnoses, neuroimaging findings, surgical procedures, and clinical follow-up examinations. It concluded that patients with myelocystoceles without abdominal wall defects have a favorable neurological outcome when compared with those having ventral wall defects.
Gupta DK, Mahapatra AK. Terminal myelocystoceles: a series of 17 cases. J Neurosurg. 2005 Oct;103(4 Suppl):344-52.
Terminal myelocystocele is an uncommon form of occult spinal dysraphism. It is a skin-covered lesion in which the end of the spinal cord and the subarachnoid space are herniated through a posterior spina bifida. Sometimes they are associated with abnormalities in the abdominal wall. These three studies review several cases. As might be expected, children with isolated myelocystoceles do better than those who have associated abdominal wall defects.
January 2006 - March 2006
The authors report two unusual cases of extraspinal midline lumbosacral lesions that resembled the usual skin markers of occult spina bifida. Some lesions, as those we are reporting, although occurring at the lumbosacral midline, do not necessarily belong to occult spinal dysraphism, as was initially suspected. Plexiform neurofibroma and skin mastocytoma are very rare indeed in this spinal location. It concluded that the two cases represent an important addition to the differential diagnosis of a congenital dorsal midline mass.
Amirjamshidi A, Abbassioun K, Shirani Bidabadi M. Skin-covered midline spinal anomalies: a report of four rare cases with a discussion on their genesis and milestones in surgical management. Childs Nerv Syst. 2006 Feb 1;:1-6 [Epub ahead of print]
This presents (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. It concluded that 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.
Perera GK, Atherton D. The value of MRI in a patient with occult spinal dysraphism. Pediatr Dermatol. 2006 Jan-Feb;23(1):24-6.
These three articles review the differential diagnosis of masses that are found in the middle of the backs of newborns. Not all of them are associated with 'spina bifida.' However, diagnostic studies such as MRI scans need to be performed whenever a suspicious swelling is found to insure that the spine and spinal cord under the lesions are normal.
Gupta DK, Ramdurg S, Mahapatra AK. Giant terminal lipomyelocystocele. Pediatr Neurosurg. 2006;42(1):49-53.
Terminal myelocystocele is a rare form of occult spinal dysraphism in which the hydromyelic caudal spinal cord and the subarachnoid space are herniated through a posterior spina bifida. A 1-year-old female child presented with a large lumbosacral mass (30 x 20 x 10 cm), flaccid paraplegia and urinary incontinence since birth. Magnetic resonance imaging revealed a low-lying conus (with associated conus lipoma) and a dilated central canal surrounded by a meningocele suggestive of terminal lipomyelocystocele and was operated on successfully. In our experience, this was a giant terminal lipomyelocystocele and such a large lesion has not been reported in the literature before. Terminal myelocystocele should be included in the differential diagnosis of congenital lesions presenting as a lumbosacral mass and operated early.
Schropp C, Sorensen N, Collmann H, Krauss J. Cutaneous lesions in occult spinal dysraphism-correlation with intraspinal findings. Childs Nerv Syst. 2006 Feb;22(2):125-131.
This review of a large series of children with occult spinal dysraphism (OSD) found correlations between cutaneous signs and underlying abnormalities in the spine. For instance, dimples and skin tags were associated with lipomas, while hairy patches were associated with split cord malformations. However, while these associations are useful, every child with a marker for OSD needs a careful individualized evaluation to determine the underlying abnormalities of the spine
July 2006 - September 2006
This study from a very prolific group of neurosurgeons in Alabama suggests that the angle made by the sacrum and lumbar spine (in the lower back) is linked with the occurrence of tethered spinal cord. Tethered spinal cord is often difficult to diagnose. This simple finding may help make this difficult diagnosis a little easier to make.
January 2007 - June 2007
Abstract: OBJECTIVES: To analyze the results after surgery for spinal cord lipomas. METHODS: The authors report their results of management of 63 non consecutive random children with spinal cord lipomas treated over a period from 2001 to 2005, at the All India Institute of Medical Sciences, New Delhi , India , a tertiary care neurosurgical centre. RESULTS: There were 63 cases consisting of 32 (52%) conus lipomas, 14 (22%) filum lipomas, 14 (22%) lipomeningomyelocele (lipoMMC), 2 (2.5%) cases of lipomyelocystocele and one (1.5%) case of mixed lipoma. None of the patients who were asymptomatic before surgery deteriorated neurologically, irrespective of the type of lipoma till the last follow up. In those patients with preexisting neurological deficits, the improvement in motor, sensory and bladder abnormalities was only to an extent of 15 %, 16 % and 21 % respectively. None of the children with preexisting neurological deficit regained overall normal function. Nevertheless, the improvement in symptoms after surgery made the patients lead a better social life. Only 6 % of patients developed deterioration in neurological function after surgery, all of them occurring in patients already having pre-operative deficits. CONCLUSIONS: The authors recommend prophylactic surgery which is safe and effective in preventing neurological deficits, irrespective of the type of lipoma. Most of the patients benefit only to some extent even after surgery, once they develop neurological dysfunction. A close long term follow up is recommended in order to detect neurological deterioration even in children operated prophylactically
[This study from India confirms that early surgery in asymptomatic children who have Occult Spinal Dysraphism (OSD) can prevent the development of neurological symptoms.]
Rajpal S, Tubbs RS, George T, Oakes WJ, Fuchs HE, Hadley MN, Iskandar BJ. Tethered cord due to spina bifida occulta presenting in adulthood: a tricenter review of 61 patients. J.Neurosurg.Spine 2007 Mar;6(3):210-5.
[Adults as well as children may present with neurological symptoms from a previously undiagnosed occult spinal dysraphism. This article recommends early surgery to prevent progression of the symptoms.]
Tubbs RS, Bui CJ, Rice WC, Loukas M, Naftel RP, Holcombe MP, Oakes WJ. Critical analysis of the Chiari malformation Type I found in children with lipomyelomeningocele. J.Neurosurg. 2007 Mar;106(3 Suppl):196-200.
Abstract: OBJECT: Occasional comments are found in the literature regarding patients with lipomyelomeningocele and concomitant Chiari malformation Type I (CM-I). The object of this study was to explore the association between these two conditions. METHODS: The authors performed a retrospective database analysis of lipomyelomeningocele cases to identify cases of concomitant CM-I. Analysis of posterior fossa volume (based on the Cavalieri principle) was performed in all identified cases in which appropriate neuroimages were available, and the results were compared with those obtained in age-matched controls. Seven (13%) of 54 patients with lipomyelomeningocele were found to also have CM-I. Two of these were symptomatic (cervicothoracic syrinx and occipital headaches) and required posterior fossa decompression. No correlation was found between the amount of hindbrain herniation and the level of the conus medullaris or the type of lipomyelome-ningocele (for example, caudal or transitional). Volumetric studies of the posterior fossa revealed normal age-matched volumes in all but one patient (who had asymptomatic CM-I). CONCLUSIONS: The incidence of CM-I in patients with lipomyelomeningocele appears to be significantly greater than that of the general population and the association rate is too high for the finding to be a chance occurrence. Decreases in the volume of the posterior cranial fossa were not found in the majority of patients in this small cohort; therefore, the cause of the concomitant occurrence of lipomyelomeningocele and CM-I remains undetermined. Clinicians should consider obtaining imaging studies of the entire neuraxis in patients with lipomyelomeningoceles and should investigate other causes for syringes found in association with lipomyelomeningoceles
[This study suggests that some children who have what appears to be an isolated occult spinal dysraphism (OSD) may have abnormalities in the brainstem. The authors suggest that children identified as having OSD should have imaging (e.g., MRI scan) of their brainstem and entire spinal cord to identify such abnormalities.]
June 2007 - January 2008
Abstract: OBJECTIVES: Diastematomyelia is a rare form of spinal dysraphism. We present eight cases of diastematomyelia diagnosed prenatally in our institution as well as a review of the literature in order to determine the prognosis of isolated cases of this very unusual condition. METHODS: Records of fetuses with diastematomyelia diagnosed in our institution between January 2000 and June 2005 were collected. All liveborn fetuses were examined by a pediatric neurologist. Pre- and postnatal data were analyzed. A search was then conducted using PubMed to review previously reported cases in the literature. RESULTS: Eight cases of diastematomyelia were diagnosed during the study interval. The mean (range) gestational age at diagnosis was 21 (13-25) weeks. The main sonographic findings were widening of the spinal canal in the coronal view and an additional echogenic focus in the posterior part of the spinal column in the axial view. The diagnosis of associated open spina bifida was made in one fetus with elevated levels of amniotic fluid alpha-fetoprotein (AF-AFP) and acetylcholinesterase (AF-AChE) and the pregnancy was terminated. The other seven cases of diastematomyelia had normal levels of AF-AFP and AF-AChE and were considered isolated. One pregnancy miscarried spontaneously 1 week following amniocentesis and the remaining six were delivered at term. Review of the literature revealed 14 reports involving 26 cases of prenatally diagnosed diastematomyelia. Twelve cases had normal biochemistry and/or no additional abnormalities and all had a favorable outcome. CONCLUSIONS: When diastematomyelia is not associated with other spinal anomalies, the prognosis is favorable. Prenatal diagnosis is generally made in the second trimester but sonographic signs may be recognized as early as the first trimester. Intrauterine diagnosis of diastematomyelia should facilitate appropriate management of affected cases.
[Diastematomyelia (which is the word with the greatest number of syllables that I know) is an abnormality in which the spinal cord is divided lengthwise into two columns by a cartilaginous or bony spur. It is one of the ‘split cord malformations' and occurs commonly in meningomyelocele. This article confirms that it can be diagnosed prenatally in some fetuses. Its prognosis depends on the occurrence of associated findings (like an open meningomyelocele). Prenatal diagnosis should improve care right after birth and improve neurological outcomes.]
Healy KA, Baumgarten DA, Lendvay TS, Fountain AJ, Galloway NT , Ogan K. Occult spinal dysraphism and urolithiasis: are patients at higher risk of stone disease? J.Endourol. 2007 Nov;21(11):1293-6.
Abstract: BACKGROUND AND PURPOSE: 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). PATIENTS AND METHODS: 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). RESULTS: 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. CONCLUSION: Unlike severe spinal dysraphism, OSD does not appear to confer an increased risk of stone disease.
[This study confirms that children with occult spinal dysraphism (OSD) are not at increased risk for having kidney stones. Only 83 children with OSD were included in the study, but clinical experience would confirm their findings. At least there is some good news to having OSD.]
Macejko AM, Cheng EY, Yerkes EB, Meyer T, Bowman RM, Kaplan WE. Clinical urological outcomes following primary tethered cord release in children younger than 3 years. J.Urol. 2007 Oct;178(4 Pt 2):1738-42.
Abstract: PURPOSE: Current practice at our institution is to recommend tethered cord release at diagnosis to prevent the onset or worsening of symptoms. Tethered cord release is frequently performed in children younger than 3 years who often have no urological manifestations. To our knowledge there are currently no long-term data on urological outcomes in this age group. MATERIALS AND METHODS: We completed a retrospective review of 475 cases of tethered cord release performed at a single institution between 1995 and 2002. Of these surgeries 173 were performed in children younger than 3 years. Clinical outcomes, and preoperative and postoperative urodynamic and radiographic studies were evaluated. RESULTS: A total of 79 patients met study criteria. Average age at surgery was 9.6 months and average followup was 5.2 years (range 6 months to 11.2 years). At followup 49 patients (62.1%) had no urological complaints and 30 (38%) had urological problems. A total of 20 children (25.3%) had minor problems (constipation, delayed toilet training or other) and 10 (12.7%) had major problems (need for clean intermittent catheterization, febrile urinary tract infection or reflux). Of 66 patients 30 (45.5%) had abnormal preoperative urodynamics. One of 31 patients (3.2%) had hydronephrosis on preoperative ultrasound. Statistical analysis revealed that abnormal preoperative urodynamics and ultrasound were not predictive of major urological problems. Lipomatous dysraphism and preoperative musculoskeletal symptoms positively correlated with major urological problems (p = 0.0076 and 0.0484, respectively). CONCLUSIONS: The majority of children did not experience urological problems following tethered cord release. Only a small set of children had major urological problems. Children with lipomatous dysraphism and musculoskeletal symptoms were more likely to experience poor urological outcomes.
[Early repair of the tethered spinal cord in asymptomatic infants is recommended to prevent the occurrence of permanent neurological damage. This study evaluated a group of children who had repair of tethered cord to determine urological outcomes. Approximately 13% of the children had major long-term urological complications. Complications were greater in children who had a lipoma and those who already had musculoskeletal symptoms. This relative low rate of major complications is encouraging and provides support to the current early treatment of tethered cord–even in those who are asymptomatic.]
Nakahara K, Shimizu S, Utsuki S, Suzuki S, Oka H, Fujii K. Lacunar skull deformity and hydrocephalus in infants with myelomeningocele: is lacunar skull deformity a predictor of hydrocephalus development? Childs Nerv.Syst. 2007 Aug;23(8):863-5.
Abstract: OBJECTS: We evaluated whether the presence of lacunar skull deformity (LSD) with myelomeningocele is a predictive factor for subsequent hydrocephalus development. MATERIALS AND METHODS: We reviewed the clinical and radiological records of 18 infants with myelomeningocele, divided the patients into groups with (group A, n=9) and without (group B, n=9) ventriculomegaly at birth and assessed whether the presence of LSD was predictive of the necessity for ventriculoperitoneal shunt (VPS) placement. RESULTS: LSD was present in five group A patients. All nine group A patients underwent VPS placement. Among the group B patients, five had LSD; they underwent VPS placement. A significantly higher proportion of those with ventricle enlargement or LSD at birth required VPS placement (p=0.0001). CONCLUSION: Adding to the ventriculomegaly at birth, the presence of LSD alerts to the necessity to monitor these infants closely to determine the necessity for VPS placement.
[A lacuna refers to a gap or defect (and comes from the word meaning lagoon). These authors found that children with meningomyelocele who had lacunar deformities of the skull on plain radiograms were more likely to require ventricular shunts.]
Rajpal S, Salamat MS, Tubbs RS, Kelly DR , Oakes WJ, Iskandar BJ. Tethering tracts in spina bifida occulta: revisiting an established nomenclature. J.Neurosurg.Spine 2007 Sep;7(3):315-22.
Abstract: OBJECT: The goal of the present study goal was to systematically confirm the previously recognized nomenclature for tethering tracts that are part of the spectrum of occult spinal dysraphic lesions. METHODS: The tethering tract in 20 patients with spina bifida occulta underwent histological examination with H & E staining and epithelial membrane antigen (EMA) immunolabeling, and additional selected specimens were stained with Masson trichrome. RESULTS: All tethering tracts contained fibrous connective tissue. Four tracts were lined with epithelial cells and either originated within a dermoid cyst, terminated at a skin dimple/sinus opening, or had both of these characteristics. No tethering tracts exhibited EMA positivity or meningeal elements. Although all tethering tracts originated in juxtaposition to the spinal cord, their termination sites were variable. CONCLUSIONS: Based on histological findings and presumed embryological origin, the authors broadly classified tethering tracts terminating within the dura mater, epidural space, or lamina as "short tethering tracts" (STTs). The STTs occurred mostly in conjunction with split cord malformations and had a purely fibrous composition. Tethering tracts terminating superficial to the overlying lamina were classified as "long tethering tracts" (LTTs), and the authors propose that these are embryologically distinct from STTs. The LTTs were of two varieties: epithelial and nonepithelial, the former being typically associated with a skin dimple or spinal cord (epi)dermoid cyst. In fact, analysis of the data suggested that not every tethering tract terminating in or on the skin should be classified as a dermal sinus tract without histological confirmation, and because no evidence of meningeal tissue-lined tracts was detected, the use of the term "meningocele manque" may not be appropriate.
[In this study the researchers examined the fibrous (or sinus) tracts associated with neural tube defects, including OSD. They fine tune the current system of classification of these tracts.]
Tubbs RS, Winters RG, Naftel RP, Acharya VK, Conklin M, Shoja MM, Loukas M, Oakes WJ. Predicting orthopedic involvement in patients with lipomyelomeningoceles. Childs Nerv.Syst. 2007 Aug;23(8):835-8.
Abstract: INTRODUCTION: Lipomyelomeningoceles (LMM) occur in approximately 1 in every 4,000 live births in the United States . They are associated with a wide range of problems in affected patients, including skin abnormalities, sensory and motor deficits, pain, urinary bladder and anal sphincter dysfunction, and orthopedic deformities. MATERIALS AND METHODS: In an effort to better understand the orthopedic complications associated with LMM, the present study examined the long-term orthopedic deformities in 50 patients after surgical correction of their LMM and observed for correlation between these deformities and the type and level of LMM. RESULTS: Analysis of the collected data revealed a statistically significant relationship between of the type of LMM and the presence of orthopedic complications. However, no statistically significant relationship existed between the vertebral level of the LMM and the presence of orthopedic deformities. No correlation was identified between the level and type of LMM. CONCLUSION: In this study, caudal LMM were much more likely than either dorsal or transitional types to have orthopedic complications. These data may prove useful to clinicians in predicting outcome and in counseling patients and their parents.
[In this study the researchers examined the relationship between lipomyelomeningoceles and subsequent orthopaedic problems. They found that the type of lesion was more strongly linked to future problems than the location of the lesion.]
March 2009 - December 2009
† Muthukumar N. Congenital spinal lipomatous malformations: part II--Clinical presentation, operative findings, and outcome. Acta Neurochir (Wien ) 2009 March;151(3):189-97.
[This series of children with spinal cord lipomas emphasizes the heterogeneity of this condition. Overall the prognosis after surgery was quite good, especially if the surgery was done before the child was 2-years of age. Early diagnosis and early surgery can improve the outlook of this condition.]
† Oi S, Nomura S, Nagasaka M, Arai H, Shirane R, Yamanouchi Y, Nishimoto H, Date H. Embryopathogenetic surgicoanatomical classification of dysraphism and surgical outcome of spinal lipoma: a nationwide multicenter cooperative study in Japan. J Neurosurg Pediatr 2009 May;3(5):412-9.
[This study showed that surgery to repair children with spinal cord lipomas typically had good outcomes. However, surgery performed after symptoms developed did not have good outcomes. This supports early diagnosis and early surgery for occult spinal dysraphism.]
† Orakdogen M, Turk CC, Ersahin M, Biber N, Berkman Z. Spinal dysraphisms of the cervicothoracic region in childhood. Turk Neurosurg 2009 October;19(4):400-5.
[This study describes cervical and thoracic spinal dysraphisms. Compared to low thoracic, lumbar and sacral spinal dysraphisms, children with cervical and high thoracic lesions do much better in terms of neurologic and orthopaedic sequelae.]
† Radmanesh F, Nejat F, El KM. Dermal sinus tract of the spine. Childs Nerv Syst 2009 August 7.
[This study describes the complications like meningitis found in children who had dermal sinus tracts. The authors emphasize the importance of early diagnosis and treatment to prevent those complications.]
† Wakhlu A, Wakhlu G, Saxena S, Tandon RK. Single-stage treatment of spina bifida with hydrocephalus based on a prediction rule derived from preoperative cranial ultrasound. Pediatr Neurosurg 2009;45(4):271-5.
[This is another study that confirms that for some newborns closing the lesion on the back and inserting a ventricular shunt during the same anesthesia is a reasonable way to go.]
† Warf B, Ondoma S, Kulkarni A, Donnelly R, Ampeire M, Akona J, Kabachelor CR, Mulondo R, Nsubuga BK. Neurocognitive outcome and ventricular volume in children with myelomeningocele treated for hydrocephalus in Uganda. J Neurosurg Pediatr 2009 December;4(6):564-70.
Abstract: OBJECT: Despite lower failure and infection rates compared with shunt placement, it has not been known whether endoscopic third ventriculostomy/choroid plexus cauterization (ETV/CPC) might be inferior in regard to neurocognitive development. This study is the first to describe neurocognitive outcome and ventricle volume in infants with hydrocephalus due to myelomeningocele that was treated primarily by ETV/CPC. METHODS: The modified Bayley Scales of Infant Development (BSID-III) test was administered to 93 children with spina bifida who were 5-52 months of age. Fifty-five of these children had been treated by ETV/CPC, 19 received ventriculoperitoneal (VP) shunts, and 19 had required no treatment for hydrocephalus. Raw scores were converted to scaled scores for comparison with age-corrected norms. Ventricular volume was assessed by frontal/occipital horn ratio (FOR) calculated from late postoperative CT scans. The mean values between and among groups of patients were compared using independent samples t-test and ANOVA. The comparison of mean values to population normal means was performed using the single-sample t-test. Linear regression analyses were performed using BSID scores as the dependent variables, with treatment group and ventricular size (FOR) as the independent variables. Probability values < 0.05 were considered significant. RESULTS: There was no significant difference in mean age at assessment among groups (p = 0.8). The mean scale scores for untreated patients were no different from normal (all p > 0.27) in all portions of the BSID (excluding gross motor), and were generally significantly better than those for both VP shunt-treated and ETV/CPC groups. The ETV/ CPC-treated patients had nonsignificantly better mean scores than patients treated with VP shunts in all portions of the BSID (all p > 0.06), except receptive communication, which was significantly better for the ETV/CPC group (p = 0.02). The mean FOR was similar among groups, with no significant difference between the untreated group and either the VP shunt or ETV/CPC groups. The FOR did not correlate with performance. CONCLUSIONS: The ETV/CPC and VP shunt groups had similar neurocognitive outcomes. Neurocognitive outcomes for infants not requiring treatment for hydrocephalus were normal and significantly better than in those requiring treatment. The mean ventricular volume was similar among all 3 groups, and significantly larger than normal. There was no association between FOR and performance. Stable mild-to-moderate ventriculomegaly alone should not trigger intervention in asymptomatic infants with spina bifida.[Third ventriculostomy with cauterization of the choroid plexus (ETV/CPC) is a procedure to manage hydrocephalus that is used more commonly in developing countries where regular access to neurosurgical care (to manage shunt complications) is limited. In this study from Uganda, children who had the ETV/CPC procedure had developmental scores that were similar to those who had had ventricular shunts–in fact they did a little better with communication. This study adds to the growing body of literature suggesting that this procedure generally is a safe and effective alternative to ventricular shunting, at least for some children.]
January 2010 to January 2011
Ali MZ. Cystic spinal dysraphism of the cervical region: experience with eight cases including double cervical and lumbosacral meningoceles. Pediatr Neurosurg 2010;46(1):29-33.
Abstract: OBJECTIVE: Cervical meningoceles are rare. Clinical, radiological and surgical data of 8 cases were presented, including a unique case of double cervical and lumbosacral meningoceles. METHODS: This retrospective study included all children operated on for cervical meningocele from January 2004 to June 2009 at the Aburish Pediatric Hospital, Cairo University. RESULTS: Eight children (6 boys and 2 girls) were operated on. Their ages ranged between 2 months and 8 years. The clinical picture--apart form posterior cervical swelling--was almost normal. One case had paraplegia due to associated lumbosacral myelomeningocele (double meningocele). Four patients had associated hydrocephalus; 2 had Chiari malformation and 2 hydromyelia. CONCLUSION: The outcome after surgery for these lesions is excellent as the majority of the children have no or minimal neurological deficit and surgery does help in improving cosmesis and preventing the development of neurological deterioration.
[This study from Egypt reports surgical outcomes of 8 children born with cervical meningoceles, which are quite uncommon. Most of these children do not have peripheral nerve involvement (weakness or loss of sensation). They may, however, have conditions like hydrocephalus, Chiari malformation or hydromyelia (swelling of the spina cord’s central canal).]
Drolet BA, Chamlin SL, Garzon MC, Adams D, Baselga E, Haggstrom AN, Holland KE, Horii KA, Juern A, Lucky AW, Mancini AJ, McCuaig C, Metry DW, Morel KD, Newell BD, Nopper AJ, Powell J, Frieden IJ. Prospective study of spinal anomalies in children with infantile hemangiomas of the lumbosacral skin. J Pediatr 2010 November;157(5):789-94.
Abstract: OBJECTIVE: To prospectively evaluate a cohort of patients with infantile hemangioma in the midline lumbosacral region for spinal anomalies to determine the positive predictive value of infantile hemangioma for occult spinal anomalies and to make evidence-based recommendations for screening. STUDY DESIGN: A multicenter prospective cohort study was performed at 9 Hemangioma Investigator Group sites. RESULTS: Intraspinal abnormalities were detected in 21 of 41 study participants with a lumbosacral infantile hemangioma who underwent a magnetic resonance imaging evaluation. The relative risk for all patients with lumbosacral infantile hemangiomas for spinal anomalies was 640 (95% confidence interval [CI], 404-954), and the positive predictive value of infantile hemangioma for spinal dysraphism was 51.2%. Ulceration of the hemangioma was associated with a higher risk of having spinal anomalies. The presence of additional cutaneous anomalies also was associated with a higher likelihood of finding spinal anomalies; however, 35% of the infants with isolated lumbosacral infantile hemangiomas had spinal anomalies, with a relative risk of 438 (95% CI, 188-846). The sensitivity for ultrasound scanning to detect spinal anomalies in this high-risk group was poor at 50% (95% CI, 18.7%-81.3%), with a specificity rate of 77.8% (95% CI, 40%-97.2%). CONCLUSIONS: Infants and children with midline lumbosacral infantile hemangiomas are at increased risk for spinal anomalies. Screening magnetic resonance imaging is recommended for children with these lesions.
[This study confirms that infants who have hemangiomas (red birth marks comprised of blood vessels) of the back are at increased risk for having abnormalities of the spine and spinal cord. They all should be evaluated by MRI scan (or ultrasonography) to evaluate their spinal cords and surrounding tissues.]
Kim SM, Chang HK, Lee MJ, Shim KW, Oh JT, Kim DS, Kim MJ, Han SJ. Spinal dysraphism with anorectal malformation: lumbosacral magnetic resonance imaging evaluation of 120 patients. J Pediatr Surg 2010 April;45(4):769-76.
Abstract: PURPOSE: We evaluated the prevalence of spinal dysraphism (SD) in patients with anorectal malformation (ARM) by magnetic resonance imaging (MRI). METHODS: From January 2002 to March 2009, 120 patients with ARM who underwent anorectal reconstruction were evaluated for SD with sacral plain film, spinal ultrasonography (US), and lumbosacral MRI. We adopted Krickenbeck international classification of ARM. RESULTS: Spinal dysraphism was present in 41 (34.2%) of 120 patients with ARM, 3 (13.0%) of 23 patients with perineal fistula, 7 (29.2%) of 24 patients with vestibular fistula, 4 (36.4%) of 11 patients with rectovesical fistula, 18 (40.9%) of 44 patients with rectourethral fistula, and 9 (60.0%) of 15 patients with cloacal anomaly (P = .04). Among 41 patients having SD detected by MR, 26 patients (26/41; 63.4%) underwent detethering surgery for tethered spinal cord. The mean sacral ratio (SR) in patients who underwent detethering surgery (0.54 +/- 0.19) was significantly lower than in patients who did not undergo detethering surgery (0.69 +/- 0.13; P < .001). The optimal cutoff for the SR value predicting SD requiring detethering surgery was 0.605, with sensitivity of 65.4% and specificity of 77.7%. CONCLUSIONS: Spinal dysraphism is common in patients with ARM, and its prevalence is higher in patients with complex ARM. Spinal anomalies can occur even with benign types of ARM and, therefore, that all patients should be screened. Magnetic resonance imaging is useful in detecting occult SD that may be missed by conventional radiologic evaluation, physical examination, and spinal US. We further recommend a lumbosacral MRI examination in those whose SR is lower than 0.6.
[In this study from 13% to 63% of children who had some sort of anorectal malformation had evidence of spinal dysraphism, and many required surgical detethering of the spinal cord. Thus, any child with a malformation fitting their criteria (including a sacral ratio [a radiologic measure] of greater than 0.6) should have evaluation of the lower spine with MRI..]
Lee YC, Solomon LB, Ruhli FJ, Schiess R, Ohrstrom L, Sullivan T, Alkadhi H, Henneberg M. Confirmation of microevolutionary increase in spina bifida occulta among Swiss birth cohorts. Eur Spine J 2010 July 15.
Abstract: Previous studies on the prevalence of spina bifida occulta have indicated a microevolutionary increase in its frequency and possible population differences in the prevalence of the condition. We studied the frequencies of closed and open sacral canals at each sacral level among two birth cohorts in Switzerland. Transverse CT scans and multiplanar reconstruction images of sacra of 95 males and 96 females born in 1940-1950 and 99 males and 94 females born in 1970-1980 in Switzerland were reviewed. We found that individuals born later have significantly more open sacral arches at all sacral levels compared to those born 30-40 years earlier. When results were related to previously published data on Australian cohorts, the trend was the same, but Swiss in both cohorts were less likely to have an open section than Australians at all locations apart from S2. This study confirmed a microevolutionary trend in the opening of sacral canal among two different generations in Switzerland and demonstrated a population difference in the prevalence of spina bifida occulta.
[Although the birth prevalence of neural tube defects has been decreasing in most developed countries, this study found a higher prevalence of spina bifida occulta [which is usually asymptomatic] among the current cohort of adults when compared with those born 30–40 years earlier. They also found that people from Australia had a higher prevalence than people from Switzerland. Neither the authors nor I are certain what this means.]
Radmanesh F, Nejat F, El KM. Dermal sinus tract of the spine. Childs Nerv Syst 2010 March;26(3):349-57.
Abstract: OBJECTIVE: Spinal dermal sinus tract (DST) is a rare entity which usually comes to clinical attention by cutaneous abnormalities, neurologic deficit, and/or infection. We performed this study to assess presentation patterns, associated anomalies, radiologic, urologic, and surgical findings in children afflicted with spinal DST. METHODS: Medical records of 35 children treated for spinal DST in Children's Medical Center from January 2001 to December 2008 were reviewed. RESULTS: Patients' age ranged from 3 days to 8.44 years. Dermal sinuses were located most frequently in the lumbar and lumbosacral regions. The most common causes for referral were abnormal skin findings (57.1%) and infection (31.4%). Notably eight patients presented with meningitis. The overall rate of neurologic abnormalities was 37.1%, four of whom presented acutely with rapidly progressive paraplegia and meningitis. The most common magnetic resonance imaging finding was tethered cord (63%). At least one urological evaluation was performed in 30 patients, which revealed abnormal results in 77% of them. All patients underwent complete resection of the tract and repair of associated abnormalities. Most tracts terminated within the intradural space. Fifteen patients (42.8%) had inclusion tumor with the dermoid tumor being the most common. None of the patients demonstrated neurological deterioration postoperatively. CONCLUSION: Dermal sinus tract is an innocuous-appearing spinal dysraphism that may contribute to devastating morbidities if not timely addressed. In order to prevent complications, timely surgical intervention including complete resection of sinus tract with intradural exploration and correction of associated abnormalities is of utmost importance.
Martinez-Lage JF, Almagro MJ, Ferri-Niguez B, Izura A, V, Serrano C, Domenech E. Spinal dermal sinus and pseudo-dermal sinus tracts: two different entities. Childs Nerv Syst 2010 October 27.
Abstract: BACKGROUND: Occult spinal dysraphism (OSD) encompasses various conditions. A dermal sinus tract (DST) consists of a duct communicating to the skin with deep structures that carries an important risk of infection. A different lesion consisting of a translucent skin opening and a fibrous tract that lacks a lumen can also be found in OSD. We termed this lesion pseudo-dermal sinus tract. PATIENTS AND METHODS: We reviewed clinical features of 20 patients with spinal skin orifices. The patients were classified into two categories: group 1 comprised children with true DST and group 2 included patients with skin dimples resembling a DST. AIMS: The aim of this study was to analyze differential features of patients in these two groups as they behaved dissimilarly in regard to clinical significance and outcomes, especially those concerning infectious risk. RESULTS: Children of group 1 (n = 8) presented with tiny skin orifices and with superficial or deep infection. In contrast, group 2 (n = 12) mostly manifested with neurological symptoms. No patient in group 2 developed an infection preoperatively. In both groups, magnetic resonance showed tracts that ended in different structures. Histopathology of the lesions were dissimilar, those of group 1 being hollow tubular structures lined by epithelium while those of group 2 being solid tracts of fibrous connective tissue. CONCLUSIONS: DST constitutes a clinicopathological diagnosis. Although sharing some common cutaneous and neuroimaging findings, both groups behaved differently. Patients of group 1 tended to show up with infection requiring urgent surgery. Patients of group 2 often presented with neurological manifestations and skin lesions, but needed no immediate operation.
[A dermal sinus tract is a tunnel that leads from the skin to an internal site. On the lower back, these tracts may lead into the area of the spinal cord and be associated with infections, including meningitis. These two studies review their experience with dermal sinus tracts. The general rule is that if the opening of a dimple on the back is unusual (high up or not in the midline), or if the bottom of it cannot be seen (meaning it is more likely a dermal sinus tract than a dimple), then follow-up radiological images of the underlying spine need to be obtained (usually an MRI scan). If abnormalities are found, early definitive surgery is recommended.]
Tarcan T, Tinay I, Temiz Y, Alpay H, Ozek M, Simsek F. The value of sacral skin lesions in predicting occult spinal dysraphism in children with voiding dysfunction and normal neurological examination. J Pediatr Urol 2010 November 26.
Abstract: OBJECTIVE: The role of magnetic resonance imaging (MRI) in detecting occult spinal dysraphism (OSD) in children with voiding dysfunction and a normal neurological examination is still under debate. The aim of this study was to assess the correlation of sacral skin lesions with OSD detected on MRI, in a population of children with resistant lower urinary tract symptoms (LUTS). PATIENTS AND METHODS: A total of 114 children over 5 years of age with urinary tract infection (UTI) and/or LUTS and normal neurological examination were enrolled. All children underwent sacral neurological examination, urine analysis and cultures, renal/bladder ultrasound, voiding cystourethrogram and urodynamic examination. After a treatment period of 6 months, the patients were re-evaluated and spinal MRI was performed in 61 with ongoing LUTS or UTI. RESULTS: Nineteen of 61 children (31%) had cutaneous stigmas. MRI detected spinal abnormality in 2/42 children with a normal sacral examination in comparison to 7/19 children with an abnormal sacral finding (Chi-squared test, P < 0.005). The sensitivity and specificity of an abnormal sacral finding in predicting MRI abnormality were 0.76 and 0.77, respectively. Urodynamic parameters did not predict an abnormal spinal MRI. CONCLUSIONS: Abnormal sacral findings, but not urodynamic studies, are strong predictors of OSD. A normal sacral examination does not rule out OSD.
[This study evaluated children with resistant lower urinary tract symptoms, such as inability to toilet train, who had normal neurological examinations. 35% of those who had abnormal signs on their back (e.g., hairy patch) had an abnormal MRI scan showing occult spinal dysraphism. Interestingly, 5% of those without any visible marks had OSD. Urinary tract symptoms like incontinence in children may be due to spinal cord abnormalities.]