Cartilage within lipomyelomeningocele and ulnar longitudinal deficiency syndrome as VACTERL association, alliance in SHH/GLI3, and Wnt pathway: illustrative case.

BACKGROUND: Lipomyelomeningocele associated with an ulnar club hand in the spectrum of VACTERL association ([costo-]vertebral abnormalities; anal atresia; cardiac defects; tracheal-esophageal abnomalities, including atresia, stenosis, and fistula; renal and radial abnormalities; limb abnormalities; single umbilical artery) is a very rare and infrequently reported phenomenon. Within the fat mass of the lipoma, it is not common to find a well-defined cartilaginous mass with no attachments to the surrounding tissue. OBSERVATIONS: The authors present the case of a 3-month-old male with low-back swelling that was off-center to the left, accompanied by a left short forearm displaying outward bowing. Echocardiography showed an atrial septal defect. This rare VACTERL association comprises lipomyelomeningocele, atrial septal defect, and ulnar longitudinal deficiency syndrome. During surgical intervention for the lipoma, a well-defined cartilaginous mass was discovered within the adipose tissue. LESSONS: The manifestation of VACTERL association can be partially explained by the Shh/Gli and Wnt pathway defects. It is prudent to screen children with neural tube defects to be aware of any associated syndromes. This case is very rare, and the literature has contained no prior report on the VACTERL association of lipomyelomeningocele, atrial septal defect, and ulnar longitudinal deficiency.

Contiguous diastematomyelia with tethered cord, intradural extramedullary dermoid tumor, and lipomyelomeningocele: A unique case of spinal dysraphism.

Diastematomyelia, tethered cord, intradural extramedullary dermoid tumor and lipomyelomeningocele such disease entities themselves are rare in their own form and concurrent presentation of all those pathological states in a single individual can be considered one of the rarest forms of spinal dysraphism globally. Moreover for prompt management with optimal prognosis needs refined neurosurgical intervention guided by intraoperative neuromonitoring so as to bring about the best quality of life in the patient.

Use of a machine learning algorithm with a focus on spinopelvic parameters to predict development of symptomatic tethered cord after initial untethering surgery.

OBJECTIVE: Among patients with a history of prior lipomyelomeningocele repair, an association between increased lumbosacral angle (LSA) and cord retethering has been described. The authors sought to build a predictive algorithm to determine which complex tethered cord patients will develop the symptoms of spinal cord retethering after initial surgical repair with a focus on spinopelvic parameters. METHODS: An electronic medical record database was reviewed to identify patients with complex tethered cord (e.g., lipomyelomeningocele, lipomyeloschisis, myelocystocele) who underwent detethering before 12 months of age between January 1, 2008, and June 30, 2022. Descriptive statistics were used to characterize the patient population. The Caret package in R was used to develop a machine learning model that predicted symptom development by using spinopelvic parameters. RESULTS: A total of 72 patients were identified (28/72 [38.9%] were male). The most commonly observed dysraphism was lipomyelomeningocele (41/72 [56.9%]). The mean ± SD age at index MRI was 2.1 ± 2.2 months, at which time 87.5% of patients (63/72) were asymptomatic. The mean ± SD lumbar lordosis at the time of index MRI was 23.8° ± 11.1°, LSA was 36.5° ± 12.3°, sacral inclination was 30.4° ± 11.3°, and sacral slope was 23.0° ± 10.5°. Overall, 39.6% (25/63) of previously asymptomatic patients developed new symptoms during the mean ± SD follow-up period of 44.9 ± 47.2 months. In the recursive partitioning model, patients whose LSA increased at a rate ≥ 5.84°/year remained asymptomatic, whereas those with slower rates of LSA change experienced neurological decline (sensitivity 77.5%, specificity 84.9%, positive predictive value 88.9%, and negative predictive value 70.9%). CONCLUSIONS: This is the first study to build a machine learning algorithm to predict symptom development of spinal cord retethering after initial surgical repair. The authors found that, after initial surgery, patients who demonstrate a slower rate of LSA change per year may be at risk of developing neurological symptoms.

Lumbosacral lipoma in childhood, how strong is the evidence base? A systematic review.

OBJECTIVE: Conus region lumbosacral lipomas (LSLs) are highly heterogeneous in their morphology, clinical presentation, and outcome, with an incompletely understood natural history and often treacherous surgical anatomy. This systematic review aims to critically evaluate and assess the strength of the current LSL evidence base to guide management strategies. METHODS: According to a systematic review following PRISMA guidelines, a search was conducted using the key term "lumbosacral lipoma" across MEDLINE (OVID), Embase, Cochrane Library, and PubMed databases from January 1951 to April 2021. All studies containing ten or more paediatric conus lipomas were included. Data heterogeneity and bias were assessed. RESULTS: A total of 13 studies were included, containing 913 LSLs (predominantly transitional type-58.5%). Two-thirds (67.5%) of all patients (treated and non-treated) remained clinically stable and 17.6% deteriorated. Neuropathic bladder was present in 8.6% at final follow-up. Of patients managed surgically, near-total resection vs. subtotal resection deterioration-free survival rates were 77.2-98.4% and 10-67% respectively. 4.5% (0.0-27.3%) required re-do untethering surgery. Outcomes varied according to lipoma type. Most publications contained heterogeneous populations and used variable terminology. There was a lack of consistency in reported outcomes. CONCLUSION: Amongst published series, there is wide variability in patient factors such as lipoma type, patient age, and methods of (particularly urological) assessment. Currently, there is insufficient evidence base upon which to make clear recommendations for the management of children with LSL. There is an imperative for neurosurgeons, neuroradiologists, and urologists to collaborate to better standardise the terminology, assessment tools, and surgical interventions for this challenging group of conditions.

Perioperative complications and secondary retethering after pediatric tethered cord release surgery.

OBJECTIVE: Tethered cord syndrome refers to a constellation of symptoms characterized by neurological, musculoskeletal, and urinary symptoms, caused by traction on the spinal cord, which can be secondary to various etiologies. Surgical management of simple tethered cord etiologies (e.g., fatty filum) typically consists of a single-level lumbar laminectomy, intradural exploration, and coagulation and sectioning of the filum. More complex etiologies such as lipomyelomeningoceles or scar formation after myelomeningocele repair involve complex dissection and dural reconstruction. The purpose of this study was to evaluate operative complications and long-term outcomes of secondary retethering related to pediatric tethered cord release (TCR) at a tertiary children's hospital. METHODS: Medical records of children who underwent surgery for TCR from July 2014 to March 2023 were retrospectively reviewed. Data collected included demographics, perioperative characteristics, surgical technique, and follow-up duration. Primary outcomes were 60-day postoperative complications and secondary retethering requiring repeat TCR surgery. Univariate and multivariate analyses were performed to identify risk factors associated with complications and secondary retethering. RESULTS: A total of 363 TCR surgeries (146 simple, 217 complex) in 340 patients were identified. The mean follow-up was 442.8 ± 662.2 days for simple TCRs and 733.9 ± 750.3 days for complex TCRs. The adjusted 60-day complication-free survival rate was 96.3% (95% CI 91.3%-98.4%) for simple TCRs and 88.7% (95% CI 82.3%-91.4%) for complex TCRs. Lower weight, shorter surgical times, and intensive care unit admission were associated with complications for simple TCRs. Soft-tissue drains increased complications for complex TCRs. The secondary retethering rates were 1.4% for simple TCRs and 11.9% for complex TCRs. The 1-, 3-, and 5-year progression-free survival rates in complex cases were 94.7% (95% CI 89.1%-97.4%), 77.7% (95% CI 67.3%-85.3%), and 62.6% (95% CI 46.5%-75.1%), respectively. Multivariate analysis revealed that prior detethering (OR 8.15, 95% CI 2.33-28.50; p = 0.001) and use of the operative laser (OR 10.43, 95% CI 1.36-80.26; p = 0.024) were independently associated with secondary retethering in complex cases. CONCLUSIONS: This is the largest series to date examining postoperative complications and long-term secondary retethering in TCR surgery. Simple TCR surgeries demonstrated safety, rare complications, and low secondary retethering rates. Complex TCR surgeries presented higher risks of complications and secondary retethering. Modifiable risk factors such as operative laser use influenced secondary retethering in complex cases.

The loop-tail mouse model displays open and closed caudal neural tube defects.

Neural tube defects (NTDs) are the second most common cause of congenital malformations and are often studied in animal models. Loop-tail (Lp) mice carry a mutation in the Vangl2 gene, a member of the Wnt-planar cell polarity pathway. In Vangl2+/Lp embryos, the mutation induces a failure in the completion of caudal neural tube closure, but only a small percentage of embryos develop open spina bifida. Here, we show that the majority of Vangl2+/Lp embryos developed caudal closed NTDs and presented cellular aggregates that may facilitate the sealing of these defects. The cellular aggregates expressed neural crest cell markers and, using these as a readout, we describe a systematic method to assess the severity of the neural tube dorsal fusion failure. We observed that this defect worsened in combination with other NTD mutants, Daam1 and Grhl3. Besides, we found that in Vangl2+/Lp embryos, these NTDs were resistant to maternal folic acid and inositol supplementation. Loop-tail mice provide a useful model for research on the molecular interactions involved in the development of open and closed NTDs and for the design of prevention strategies for these diseases.

Nephrogenic rest vs immature teratoma associated with lumbosacral lipomyelomeningocele: a case report and review of the literature.

BACKGROUND: Lipomyelomeningoceles (LMMs) are subcutaneous lipomas with dural penetration that often present with spinal cord tethering and may lead to neurological deterioration if untreated. This report describes a rare case of an LMM associated with immature nephroblastic tissue, representing a nephrogenic rest (NR) or, less likely, an immature teratoma. CLINICAL PRESENTATION: An 8-day-old infant girl presented to the clinic with a sacral dimple. Imaging demonstrated a tethered spinal cord with low-lying conus medullaris and an LMM. A firm mass was noted in the subcutaneous lipoma. Detethering surgery and removal of the lipoma and mass were performed at the age of 6 months. Pathological examination identified the mass as cartilage, fat, and immature nephroblastic tissue consistent with NR tissue or, less likely, a teratoma with renal differentiation. CONCLUSION: This presentation of an LMM associated with an immature teratoma or NR poses a risk of malignant transformation in patients. As a result, careful surgical dissection, resection, and close clinical follow-up are recommended for these patients.

A rare case of thoracic lipomyelomeningocele in a young female: A case report.

Thoracic lipomyelomeningocele is a rare type of congenital occult spinal dysraphism. It is characterized by lipomatous tissue connected to the dorsal spinal cord that protrudes through a spinal defect together with the meninges or spinal cord to form a posterior mass beneath the skin. Closed spinal dysraphism can present diagnostic challenges when resources are scarce and advanced imaging techniques like magnetic resonance imaging are not readily available. Here, we describe a case of thoracic lipomyelomeningocele, a type of closed spinal dysraphism in a young female presenting with gradually progressive weakness and tingling sensation in bilateral lower limbs over the last 6 months. On physical examination, she had a soft tissue swelling with dimpling over the dorsal spine and paraparesis. Magnetic resonance imaging of the spine revealed dorsal lipomyelomeningocele corresponding to D4-D7 vertebral levels with tethered spinal cord.

Transition from partial to near-total/radical resection of spinal cord lipomas.

PURPOSE: The objective of this retrospective study was to compare the outcomes and associated complication rates in the surgical management of spinal cord lipomas following a change of practice within our institution from partial resection (PR) to near-total/radical resection (NTR). METHODS: Twenty-four children underwent surgical treatment for symptomatic spinal cord lipomas between 2009 and 2020. The near-total/radical resection group included 20 patients with spinal cord lipomas and the comparison group included 6 patients with spinal cord lipomas who underwent partial resection. Filar lipomas were excluded. RESULTS: The mean age of the patients was 7 years (range 1-14 years). Post-operatively, a higher proportion of NTR patients (17/20, 85%) demonstrated improvement or stabilisation in Necker-Enfants Malades scores compared to PR patients (3/6, 50%) with a mean follow-up of 48 and 108 months respectively. Two patients underwent re-do untethering surgery, both of which initially underwent partial resection surgery. Complication rates did not significantly differ between the two groups. CONCLUSION: Our data supports the view that near-total/radical resection should be considered the technique of choice over the conventional method of partial resection for spinal cord lipomas with no significant increase in complication rates.

Analysis of factors affecting permanent clean intermittent catheterization and bladder function after primary neurosurgical repair of lipomyelomeningocele.

AIMS: This study was conducted to identify potential risk factors for permanent clean intermittent catheterization (CIC) and incontinence in patients with lipomyelomeningocele (LMMC) and evaluate how LMMC affects bladder function prognosis, measured by urodynamic (UD) score. METHODS: This retrospective study analyzed the electronic health records of patients who underwent primary neurosurgical repair for LMMC at a single tertiary referral center between January 2012 and December 2016 and were followed at least 3 years after surgery. Data regarding bladder function were obtained from medical records for multiple time points, including before surgery, after surgery but before hospital discharge, 3 months after surgery, and at outpatient visits during follow-up. RESULTS: This study enrolled 120 patients. At a mean follow-up of 62.6 ± 13.9 months after primary neurosurgical LMMC repair, 22 (18.3%) patients continued to require CIC for bladder emptying, only 7 (31.8%) of whom maintained bladder continence. A multivariate logistic regression model identified age at the time of surgery and the type of LMMC as significant presurgical prognostic risk factors for permanent CIC. In addition, postoperative urinary retention and a UD score greater than or equal to 5 measured 3 months after surgery were identified as significant postsurgical risk factors for permanent CIC and urinary incontinence. A linear mixed model adjusted for age at the time of surgery showed that patients with a transitional or chaotic LMMC type were more likely to experience gradual bladder function decline than patients with other LMMC types. CONCLUSIONS: This study identified both presurgical (age at the time of surgery, LMMC type) and postsurgical (postoperative urinary retention, UD score greater than or equal to 5 at 3 months postsurgery) risk factors for permanent CIC and urinary incontinence. In addition, LMMC type was identified as a prognostic risk factor for bladder function decline. These results will enhance the current understanding of bladder function outcomes in patients who undergo surgical treatment for LMMC.

Late diagnosis of dorsolumbar lipomyelomeningocele with tethered cord in a middle aged adult: A case report from Nepal.

Closed spinal dysraphism can present with diagnostic issues in settings with limited resources, when knowledge of the disorder and specialized radiological studies, such as magnetic resonance imaging (MRI), may not be readily available. Undiagnosed cases can develop serious neurological deficits. Here, we describe a case of dorsolumbar lipomyelomeningocele, a type of closed spinal dysraphism, presenting in a middle aged with paraplegia complicated by bed sores. A 38-year-old female with no significant past medical history experienced gradually progressive weakness of bilateral lower limbs over 9 years. On physical examination, patient had a soft swelling with hairy tuft over the lumbar spine, paraplegia, grade III bed sore over the gluteal region, and sensory loss below L1 sensory level. Her bowel and bladder sensation were decreased. The soft tissue swelling over her back was not evaluated appropriately before this presentation. MRI of the spine revealed dorsolumbar lipomyelomeningocele with tethered spinal cord.

Split Notochord Syndrome with Spinal Column Duplication and Spinal Cord Lipoma: A Case Report.

BACKGROUND AND IMPORTANCE: Split notochord syndrome (SNS) is an exceedingly rare type of spinal dysraphism. SNS is sometimes associated with other congenital dysraphic defects but, as in our case, the association with spinal cord lipoma, tethered cord, and spinal deformity in the form of spinal column duplication would be exceedingly rare. Herein, the authors report a three-year-old child presented with SNS associated with complex spinal deformity and other associated congenital anomalies. The patient underwent microsurgical release of the tethering element with excellent short- and long-term outcomes. CLINICAL PRESENTATION: A male newborn with healthy nonconsanguineous parents was born with multiple gastrointestinal and genitourinary anomalies, and duplicated vertebral columns at the lumbosacral area consistent with split notochord syndrome. The patient was initially managed for the gastrointestinal and genitourinary anomalies. As there was no obvious neurological deficit initially, the neurosurgical intervention was postponed till the child reached 30 months of age, when he underwent uneventful release of both spinal cords at their spit point. CONCLUSIONS: SNS is an exceedingly rare developmental anomaly that is usually associated with varying degrees of complex congenital dysraphic defects. Early clinical diagnosis, understanding of the pathophysiology of spinal cord tethering, and microsurgical cord untethering are the important steps in optimal management.

Recurrent Tethering in Conus Lipomas: A Late Complication Not to Be Ignored.

BACKGROUND: Recurrent symptomatic tethered cord (RTC) is a long-term complication of spinal cord lipomas, responsible for progressive motor deficits, urologic dysfunction, and aggravation of spinal deformities. We retrospectively analyzed all cases of recurrent tethering after spinal cord lipoma surgery, the clinical and radiologic features that led to the diagnosis, the surgical management, and the neuro-orthopedic outcome at the last follow-up. METHODS: The study was carried out over 20 years on 209 pediatric patients from a single institution, initially treated for a conus lipoma. RESULTS: Nine patients (4.8%) were surgically treated for an RTC. The age at retethering ranged from 2 to 12 years (median, 7.4 years). The time until the first and the second surgical procedure ranged from 19 to 140 months (median, 92 months [7.5 years]). The follow-up period after the second surgery ranged from 3 months to 13 years (median, 50 months). Regarding symptoms, pain responded well to surgery. Gait disturbances improved in 50% of patients after surgery. One patient with bladder dysfunctions also improved. The remaining patients maintained their presurgical status. CONCLUSIONS: When RTC is confirmed, the child should be referred to surgery as soon as possible, because we show that the postoperative clinical outcome improved and surgery did not worsen patients' condition. Follow-up should be as long as possible for these patients.

High prevalence of gram-negative and multiorganism surgical site infections after pediatric complex tethered spinal cord surgery: a multicenter study.

OBJECTIVE: Complex tethered spinal cord (cTSC) release in children is often complicated by surgical site infection (SSI). Children undergoing this surgery share many similarities with patients undergoing correction for neuromuscular scoliosis, where high rates of gram-negative and polymicrobial infections have been reported. Similar organisms isolated from SSIs after cTSC release were recently demonstrated in a single-center pilot study. The purpose of this investigation was to determine if these findings are reproducible across a larger, multicenter study. METHODS: A multicenter, retrospective chart review including 7 centers was conducted to identify all cases of SSI following cTSC release during a 10-year study period from 2007 to 2017. Demographic information along with specific microbial culture data and antibiotic sensitivities for each cultured organism were collected. RESULTS: A total of 44 SSIs were identified from a total of 655 cases, with 78 individual organisms isolated. There was an overall SSI rate of 6.7%, with 43% polymicrobial and 66% containing at least one gram-negative organism. Half of SSIs included an organism that was resistant to cefazolin, whereas only 32% of SSIs were completely susceptible to cefazolin. CONCLUSIONS: In this study, gram-negative and polymicrobial infections were responsible for the majority of SSIs following cTSC surgery, with approximately half resistant to cefazolin. Broader gram-negative antibiotic prophylaxis should be considered for this patient population.

Characterizing syringomyelia and its clinical significance in 140 patients with lipomyelomeningocele.

OBJECTIVE: The aims of this study were to characterize syringomyelia in patients with lipomyelomeningocele (LMMC) and investigate the association between the presence of a syrinx and patient neurological deficits. METHODS: Patients with LMMC who had been admitted between 2007 and 2020 were included in the study. Syringomyelia on magnetic resonance imaging (MRI) was classified into three groups: true syrinx, early syrinx, and presyrinx. The correlation of clinical deficits (at birth, new onset, and progressive) and type of lipoma with the presence and type of syrinx was studied. RESULTS: Among a total of 140 patients, the median age was 2.5 (IQR 1.3-9) years. Neurological deficits were present at birth in 75 (53.6%) patients, but 67 (47.9%) had new-onset deficits or progression of their birth deficits. The most common type of spinal lipoma was the transitional type (75.7%). Thirty-nine (27.9%) patients had a syrinx (37 with a true syrinx, 2 with an early syrinx), and 25 (17.9%) patients had a presyrinx. There was no significant correlation between the presence of a syrinx and patient neurological deficits. However, patients with a syrinx that was not adjacent to the lipoma (≥ 1 vertebral level away) had a higher incidence of deficits at birth (p = 0.045), whereas those with a syrinx spanning ≥ 5 vertebral levels had a higher incidence of progressive neurological deficits (p = 0.04). Six (75%) of 8 patients in whom serial MRI had shown syrinx progression had clinical worsening. CONCLUSIONS: Syrinx location with respect to the spinal lipoma, syrinx length, and progressive syringomyelia may have clinical significance in patients with LMMC.

Sacral myolipoma with involuntary contraction causing tethered cord syndrome: illustrative case.

BACKGROUND: Spinal lipomas sometimes involve various ectopic tissues originating from the ectoderm, mesoderm, and endoderm in the process of morphological development. OBSERVATIONS: A 29-year-old male patient with myolipoma of the conus medullaris at the S2 and S3 levels was described. The unusual finding, involuntary muscle contraction, was presented in an operative video and a literature review. In the present case, sacral myolipoma with involuntary contraction caused tethered cord syndrome in adulthood, and untethering surgery resolved continuous buttock and leg pain. LESSONS: This rare finding is considered a surgical indication for adult patients with myolipoma.

Repair of refractory postoperative cerebrospinal fluid leakage using a reversed dermis flap in a pediatric lipomyelomeningocele patient.

INTRODUCTION: Cerebrospinal fluid (CSF) leak and pseudomeningocele are common complications after surgery for spinal dysraphism. CASE REPORT AND TECHNIQUE: We report a 6-month-old girl with a lumbosacral lipomyelomeningocele and accessory lower limb who developed a refractory cerebrospinal fluid leak and pseudomeningocele after lipomyelomeningocele repair and removal of the accessory limb. The pseudomeningocele was successfully repaired using a reversed dermis flap made from excess skin that covered the meningocele. CONCLUSION: This technique can be performed without using synthetic material or an additional surgical incision.

Spinal column shortening for tethered cord syndrome: a systematic review and individual patient data meta-analysis.

OBJECTIVE: Tethered cord release (TCR) is the gold standard treatment for tethered cord syndrome (TCS); however, there are significant shortcomings including high rates of retethering, especially in complex and recurrent cases. Spinal column shortening (SCS) is an alternative treatment for TCS intended to avoid these shortcomings. Early studies were limited to case reports and smaller case series; however, in recent years, larger case series and small cohort studies have been conducted. Given the increase in available data, a repeat systematic review and meta-analysis is warranted to assess the safety and efficacy of SCS for TCS. METHODS: The authors conducted a systematic review using MEDLINE (OVID), Embase (Elsevier), and Web of Science records dating from 1944 to July 2021 to identify all articles investigating SCS for TCS. They performed standard and individual patient data (IPD) meta-analyses, with 2 independent reviewers using PRISMA-IPD guidelines. Primary outcomes were improvement of preoperative clinical symptoms of pain, motor weakness, and bladder and bowel dysfunction, and also surgical complication rate. Secondary outcomes included urodynamic improvement and health-related quality-of-life outcomes determined using patient-reported outcome tools. Individual study quality assessment was performed using a standardized assessment tool for case reports/series, and publication bias was assessed using funnel plot analyses. RESULTS: The review yielded 15 studies with 191 cases of TCS treated with SCS. IPD were available in 11 studies with 89 cases. The average age at time of surgery was 28.0 years (range 5-76 years). The average follow-up time was 33.2 months (range 7-132 months). Improvement was observed at last follow-up in 60 of 70 (85.7%) patients with preoperative pain, in 38 of 60 (60.3%) patients with preoperative weakness, and in 36 of 76 (47.4%) patients with preoperative bladder or bowel dysfunction. Complications of CSF leak, new neurological deficit, wound infection, or reoperation occurred in 4 of 89 (4.5%) patients. CONCLUSIONS: SCS may be considered a safe and efficacious treatment option for TCS in children and adults (level C evidence; class IIb recommendation), especially for recurrent and complex cases. Current evidence is likely to be affected by selection and publication bias. Prospective comparative studies of SCS and TCR for TCS are recommended to determine long-term duration of outcomes, long-term safety in skeletally immature children, and exact indications of SCS versus traditional TCR.

Tethered Cord Syndrome Associated With Lumbar Lipomyelomeningocele: A Case Report.

The objective is to describe a rare case of lumbar lipomyelomeningocele presenting as progressive urinary incontinence. Lipomyelomeningocele is a type of closed spinal dysraphism typically presenting as a lipomatous mass contiguous with a neural defect above the gluteal crease. Tethered cord syndrome is defined as symptoms and signs caused by excessive spinal cord tension from an abnormally low conus medullaris, with an abnormally thick filum terminale attached to the lower sacral region. A 19-year-old male with no remarkable medical history presented with low back pain and urinary incontinence for the past one year. On physical exam patient had normal motor strength, sensory testing to all modalities was intact. The rectal tone was normal, and no saddle anesthesia was noted. MRI lumbar spine revealed lumbar lipomyelomeningocele with associated tethered cord syndrome. The patient underwent tethered cord release surgery with lipoma excision. Pathology of the soft tissue showed fibrovascular tissue and mature adipose tissue consistent with lipoma. The majority of cases of tethered cord syndrome are related to spinal dysraphism, a rare pediatric syndrome. It is potentially treatable if caught early, and MRI can help with an accurate diagnosis of the condition. Older adults are more likely to present with urological and neurological complaints. Surgical un-tethering is indicated in patients with progressive symptoms. In our case, the only presenting symptom was urinary incontinence, and the neurological exam was normal other than lower lumbar paraspinal tenderness.

Risk factors for pre-operative functional deterioration in children with lipomyelomeningocele.

PURPOSE: To characterize the temporal profile of pre-operative deterioration in children with lipomyelomeningocele (LMMC) including those with congenital deficits and identify risk factors for clinical worsening. METHODS: Records of 87 children who underwent surgery for LMMC were retrospectively reviewed to study the temporal profile of pre-operative deterioration, defined by the onset of new neurological dysfunction or progression of a pre-existing deficit. Preoperative magnetic resonance imaging (MRI) studies were examined to identify radiological features associated with deterioration. In children with extra-spinal placodes, the angle subtended by the terminal placode with the cord at the level of the laminar-fascial defect ("J sign") was assessed. RESULTS: Pre-operative deterioration in function was seen in 37 children (43%), occurring at a median age of 36 months and was more frequent in children without congenital deficits (54% versus 27%; p = 0.016). On Cox regression analysis, extra-spinal location of the placode (p = 0.003) and presence of a congenital deficit (p = 0.009) were positively and negatively associated with deterioration respectively. On Kaplan-Meier analysis, the median deterioration-free survival time was 72 months and was positively associated with presence of congenital deficit (p = 0.026) and negatively associated with presence of an extra-spinal placode (p < 0.001) or a meningocele sac (p = 0.001). CONCLUSION: Extra-spinal location of the neural placode was associated with higher risk of clinical deterioration in children with LMMC, whereas the presence of a congenital deficit conferred a decreased risk. Risk stratification based on clinical and radiological features can be used to guide decisions regarding early prophylactic surgery in children with LMMC.