Lumbosacral Lipoma Research Articles

Placode rotation in transitional lumbosacral lipomas: are there implications for origin and mechanism of deterioration?

PurposeRotation of the lipoma-neural placode has been noted in transitional lumbosacral lipomas. The purpose of this study was to confirm this rotation; that this rotation occurs with a preference to the left, and correlates with clinical symptoms. In addition, this study tests the hypothesis that this rotation occurs through local mechanical forces rather than intrinsic congenital malformation.MethodsLipomas were classified as per the Chapman classification. Degree of rotation of the placode from the coronal plane was recorded along with the presence of herniation outside of the vertebral canal. Abnormalities on urodynamic testing were recorded, along with neuro-orthopaedic signs picked up on formal neuro-physiotherapy assessment.ResultsPlacode rotation occurs more frequently in the transitional group. Regardless of lipoma classification, rotation was much more common to the left. Furthermore, when lateralisation of symptoms was present, this strongly correlated with the direct of rotation. There was no difference in rotation of the placode whether it was within (lipomyelocoele) or without the vertebral canal (lipomyelomeningocoele).ConclusionsPlacode rotation is a feature of transitional lumbosacral lipomas and may account for the increase in symptoms amongst this subgroup. Herniation of the placode outside the vertebral canal does not increase the risk of rotation suggesting a congenital cause for this finding rather than a purely mechanical explanation.

The pathology of lumbosacral lipomas: macroscopic and microscopic disparity have implications for embryogenesis and mode of clinical deterioration.

AimsLumbosacral lipomas (LSL) are congenital disorders of the terminal spinal cord region that have the potential to cause significant spinal cord dysfunction in children. They are of unknown embryogenesis with variable clinical presentation and natural history. It is unclear whether the spinal cord dysfunction reflects a primary developmental dysplasia or whether it occurs secondarily to mechanical traction (spinal cord tethering) with growth. While different anatomical subtypes are recognised and classified according to radiological criteria, these subtypes correlate poorly with clinical prognosis. We have undertaken an analysis of surgical specimens in order to describe the spectrum of histological changes that occur and have correlated the histology with the anatomical type of LSL to determine if there are distinct histological subtypes.Methods and resultsThe histopathology was reviewed of 64 patients who had undergone surgical resection of LSL. The presence of additional tissues and cell types were recorded. LSLs were classified from pre‐operative magnetic resonance imaging (MRI) scans according to Chapman classification. Ninety‐five per cent of the specimens consisted predominantly of mature adipocytes with all containing thickened bands of connective tissue and peripheral nerve fibres, 91% of samples contained ectatic blood vessels with thickened walls, while 22% contained central nervous system (CNS) glial tissue. Additional tissue was identified of both mesodermal and neuroectodermal origin.ConclusionsOur analysis highlights the heterogeneity of tissue types within all samples, not reflected in the nomenclature. The diversity of tissue types, consistent across all subtypes, challenges currently held notions regarding the embryogenesis of LSLs and the assumption that clinical deterioration is due simply to tethering.

Aggressive Resection of Congenital Lumbosacral Lipomas in Adults: Indications, Techniques, and Outcomes in 122 Patients.

The authors reviewed the treatment of adult patients with congenital intraspinal lipomas with total/near-total resection and discussed their preoperative characteristics, prognostic factors, and surgical outcomes. Medical records of 122 adult patients with congenital lumbosacral lipomas undergoing total/near-total resection were systematically analyzed. The cohort was subdivided into 3 groups depending on symptom onset age: group 1 (≤5 years, n= 40), group 2 (>5 years but <18 years, n= 33), and group 3 (>18 years, n= 49). Preoperative and postoperative neurologic status were compared between groups and analyzed as a whole. The most common symptom was bladder dysfunction (82.0%), followed by constipation (76.2%). Atthe 3-month follow-up, improvement was noted in most patients presenting with pain (87.2%) and neuropathic ulcers (70.0%). Overall, neurologic status was improved in 73.0% of patients and stabilized in 19.7% of patients. Abinary logistic regression model identified shorter preoperative duration (P= 0.013) and preoperative pain (P=0.005) as independent predictors of postoperative improvement. Neurosurgical complications developed in 16 patients, and wound complications occurred in 2 patients. Two of 3 patients who had recurred symptoms underwent repeated detethering surgery during long-term follow-up. Despite longer preoperative duration than the pediatric population, adult patients with lumbosacral lipomas can still benefit from total/near-total resection especially regarding pain and foot ulcers, with low surgery-related morbidity. The long-term advantage of resecting additional lipoma in adults remains a point of discussion.

Neurosurgical management and pathology of lumbosacral lipomas with tethered cord.

Lumbosacral lipomas are the most common form of occult spinal dysraphism. The development of lumbosacral lipomas is from the premature disjunction of the neural tube from the surrounding ectoderm, leaving the neural plate open posteriorly and allowing for the infiltration of mesodermal tissue, including fatty tissue. Since lumbosacral lipomas are a common cause of spinal cord tethering that can lead to progressive neurological deficits, prophylactic neurosurgery for lumbosacral lipomas, including untethering of the spinal cord, is recommended. We briefly review the embryology, classification, clinical presentation, imaging evaluation, surgical indication, neurosurgical management and pathological examination that are involved in recognizing these complicated malformative pathologies.

Spinal column shortening for tethered cord syndrome associated with myelomeningocele, lumbosacral lipoma, and lipomyelomeningocele in children and young adults.

OBJECTIVE Tethered cord syndrome is the clinical manifestation of an abnormal stretch on the spinal cord, presumably causing mechanical injury, a compromised blood supply, and altered spinal cord metabolism. Tethered cord release is the standard treatment for tethered cord syndrome. However, direct untethering of the spinal cord carries potential risks, such as new neurological deficits from spinal cord injury, a CSF leak from opening the dura, and retethering of the spinal cord from normal scar formation after surgery. To avoid these risks, the authors applied spinal column shortening to children and transitional adults with primary and secondary tethered cord syndrome and report treatment outcomes. The authors' aim with this study was to determine the safety and efficacy of spinal column shortening for tethered cord syndrome by analyzing their experience with this surgical technique. METHODS The authors retrospectively reviewed the demographic and procedural data of children and young adults who had undergone spinal column shortening for primary or secondary tethered cord syndrome. RESULTS Seven patients with tethered cord syndrome caused by myelomeningocele, lipomyelomeningocele, and transitional spinal lipoma were treated with spinal column shortening. One patient with less than 24 months of follow-up was excluded from further analysis. There were 3 males and 4 females; the average age at the time was surgery was 16 years (range 8-30 years). Clinical presentations for our patients included pain (in 5 patients), weakness (in 4 patients), and bowel/bladder dysfunction (in 4 patients). Spinal column osteotomy was most commonly performed at the L-1 level, with fusion between T-12 and L-2 using a pedicle screw-rod construct. Pedicle subtraction osteotomy was performed in 6 patients, and vertebral column resection was performed in 1 patient. The average follow-up period was 31 months (range 26-37 months). Computed tomography-based radiographic outcomes showed solid fusion and no instrumentation failure in all cases by the most recent follow-up. Five of 7 patients (71%) reported improvement in preoperative symptoms during the follow-up period. The mean differences in initial and most recent Scoliosis Research Society Outcomes Questionnaire and Oswestry Disability Index scores were 0.26 and -13%, respectively; minimum clinically important difference in SRS-22 and ODI were assumed to be 0.4% and -12.8%, respectively. CONCLUSIONS Spinal column shortening seems to represent a safe and efficacious alternative to traditional untethering of the spinal cord for tethered cord syndrome.

Lumbosacral lipoma and low-lying cord with minimal deficits

Lumbosacral lipoma and low-lying cord with minimal deficits

Conservative and surgical treatment of pediatric asymptomatic lumbosacral lipoma: a meta-analysis.

The aim of this study is to compare the outcomes of surgical and conservative treatments of pediatric asymptomatic lumbosacral lipomas, and to address whether the patients can benefit from prophylactic surgeries. The literature reports of surgical and conservative treatments of child asymptomatic lumbosacral lipomas were reviewed and collected, and a meta-analysis of the reports regarding the incidence of sphincter and lower limb dysfunctions was performed. A total of five literatures were collected, containing a total of 403 patients, among which 124 patients received conservative treatments with 32 (25.81%) cases developing neurological dysfunctions during follow-up, and 279 received prophylactic surgical treatments with 30 (10.75%) patients developing neurological dysfunctions in follow-up, the difference being statistically significant (P≤0.05). For pediatric asymptomatic lumbosacral lipomas of the three major subtypes, the limited source of literature so far suggests that prophylactic surgery is superior to conservative strategy in preventing the patients from neurological deterioration. Larger patient cohorts, randomized studies, and longer length of follow-ups are needed for further corroboration.

Spina Bifida Occulta in an Adult Presenting as Perilesional Hemorrhage

AbstractOccult spinal dysraphism with spinal lipoma, is a rare congenital spinal dysraphic anomaly which usually involves lumbosacral region and manifests in childhood. In the present article, we discuss a case of a 26-year-old man who developed sudden low back pain following intercourse. Magnetic resonance imaging of the lumbosacral spine was suggestive of spina bifida occulta with hemorrhage. The patient underwent a L4–5 laminectomy in the emergency. There was the presence of altered blood in the vicinity of the lesion suggestive of recent hemorrhage. Histopathological examination of the lesion was suggestive of the lipoma. In the present case, the patient had occult spina bifida due to lumbosacral lipoma leading to the tethering of the dura. Strenuous activity resulted in overstretching of the tethered structures and subsequent hemorrhage and intense low back pain which responded well to the surgical treatment.

Lumbosacral lipoma with macrodactyly deformities: report of a case

Lumbosacral lipoma with macrodactyly deformities: report of a case

Results of the prophylactic surgery of lumbosacral lipomas 20years of experience in the Paediatric Neurosurgery Department La Timone Enfants Hospital, Marseille, France.

We conducted a retrospective study of children with lumbosacral lipomas treated in the Department of Pediatric Neurosurgery hospital Timone Enfants in the last 20years. We selected patients with lipomas of the conus medullaris who underwent preventive surgery. 86 ,4% of the patients remained asymptomatic during the follow-up. Worse results were observed in children older than one year old at surgery with transitional lipoma, with the conus medullaris was below L5 and when the resection had been partial. In view of the results, the authors recommend the preventive surgery before the first year of life under neurophysiologic monitoring in order to perform a total near-total resection with a neural plate reconstitution.

Radiographic predictors of deterioration in patients with lumbosacral lipomas.

OBJECTIVE Although patients with lumbosacral lipomas may be asymptomatic at presentation, most develop neurological symptoms over time. Given the challenges in examining infants, the authors sought to determine whether MRI would be helpful in identifying patients who are more likely to deteriorate early in life and who would potentially benefit from early surgical intervention. METHODS A retrospective review of all patients with lumbosacral lipomas who were seen at the authors' institution between 1997 and 2013 and who were managed without prophylactic surgery was performed. The clinical history and imaging results for each patient were reviewed in detail and then correlated to the pattern of and age at clinical deterioration. RESULTS Twenty-four patients were identified. Nine worsened within the first 18 months of life (early deterioration), and 15 patients deteriorated or remained stable after 30 months (late deterioration/stable). No patients worsened between 18 and 30 months of age. Patients who deteriorated early were more likely to have large intradural lipomas that filled the canal, increased during the 1st year of life, and compressed neurological structures. Some had a syrinx extending above the neural-lipoma interface. Syrinxes in patients with early deterioration were large and expanded in infancy. Patients with early deterioration had motor deficits at the time of deterioration, whereas patients with late deterioration developed mixed urological and motor dysfunction. CONCLUSIONS Patients with large lipomas displacing the cord and an enlarging syrinx have a propensity for early clinical deterioration. Given this, their families may be counseled that 1) the risk of deterioration in infancy may be higher than in infants without these features, and 2) they require more diligent observation. Intervention before deterioration in these infants should also be considered. Patients without these features may be safely observed to a lesser extent.

The natural history and management of patients with congenital deficits associated with lumbosacral lipomas.

Many patients with lumbosacral lipoma are asymptomatic; however, a significant proportion will have neurological deficits present at birth. Implication of these deficits with respect to natural history and management are not well understood. A retrospective review of all infants with lumbosacral lipoma seen at BCCH between 1997 and 2013 was carried out. The study population was stratified on the presence of a congenital, non-progressive deficit and subdivided on treatment approach. The subsequent developments of deficits resulting in untethering procedures were recorded. Of the 44 infants in this study, 24 patients had no neurologic deficit while 20 patients had a fixed, non-progressive deficit evident at birth. Ten of 24 patients without a neurological deficit at birth underwent a prophylactic untethering with 3 eventually requiring repeat untethering after, on average, 62.7 months. Eleven of 14 asymptomatic, monitored patients required untethering for clinical deterioration. Two required a second untethering procedure after 48.7 months. Ten of 20 infants with congenital deficits present at birth underwent prophylactic untethering, and 4 required further surgery after 124 months. Ten patients underwent observation with 8 eventually requiring surgery. Two required repeat untethered after 154 months. The complication rates and operative burden for patients are similar whether prophylactic or delayed surgery is performed. The presence of congenital neurologic deficit does not affect the likelihood of deterioration in patients managed expectantly; prophylactic detethering of these patients did not prevent delayed neurologic deterioration. Comparing the need for repeat surgery in prophylactically untethered patients with initial untethering of patients operated upon at the time of deterioration, prophylactic untethering may confer a benefit with respect to subsequent symptomatic tethering if complication rates are low. However, in a setting with multidisciplinary follow-up, a period of observation for patients and intervention when patients become symptomatic is an acceptable approach for patients with or without congenital deficits.

Surgical Outcome of Children and Adolescents with Tethered Cord Syndrome

Study DesignRetrospective cohort study.PurposeTo compare long-term results of surgery with the outcomes of symptomatic and asymptomatic tethered cord syndrome (TCS) in children and adolescents and to assess the surgery duration for those with TCS.Overview of LiteraturePediatric patients with TCS continue to pose significant diagnostic and management challenges.MethodsWe retrospectively analyzed the outcomes of 31 patients (16 males, 15 females) with TCS, including 21 with lumbosacral lipoma. All were surgically treated between 1989 and 2015. They were divided into symptomatic and asymptomatic TCS groups. The results of the treatment were summarized and analyzed using a non-parametric Mann–Whitney U test.ResultsNineteen patients had symptomatic TCS and 12 had asymptomatic TCS. Patients had a median age of 34 months (range, 0–201 months). The median follow-up period was 116 months (range, 7–223 months). Of the 19 symptomatic TCS patients, preoperative deficits improved after surgery in two (10.5%) and remained stable in 17 (89.5%) patients. One of the 12 asymptomatic TCS (8.3%) patients showed an exacerbated illness after surgery, and one in 11 patients remained stable (11.7%). There were significant differences in monthly age at surgery, preoperative bowel and bladder dysfunction, neurological function, presence/absence of clean intermittent catheterization introduction, and presence/absence of motor disorder at final follow-up (all p<0.05). In the univariate analysis, the presence/absence of preoperative bowel and bladder dysfunction, and symptoms were strongly associated with the risk of children and adolescents with TCS (p<0.05).ConclusionsEarly accurate diagnosis and adequate surgical release might lead to successful outcomes in children and adolescents with TCS. Surgical untethering is a safe and effective method for treatment of children with TCS.

Cloacal reconstruction after a complex treatment of perineal haemangioma in a variant of PELVIS syndrome.

BackgroundPELVIS is an acronym defining the association of perineal hemangioma, malformations of external genitalia, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus and skin tag. Eleven cases have been reported according to the Orphanet data. Acronyms of LUMBAR and SACRAL syndrome have been used and most probably represent a spectrum of the same entity. Very little is known about the success and timing of cloacal reconstruction after the treatment of hemangioma. We present a variant of PELVIS syndrome and discuss the possibilities and optimal timing of surgical reconstruction.Case presentationFemale infant was born with persistent cloaca and multiple hemangiomas of genitals, perineal area and left thigh. Colostomy was performed after birth. In order to treat hemangioma and to make the reconstruction of cloaca possible, corticosteroid treatment orally and multiple laser treatments were performed alternating Nd:YAG laser and pulsed dye laser therapy. Cystoscopy confirmed hemangiomatosis in the mucosa of the common channel, bladder neck and septate vagina. Oral propranolol treatment was started at the age of 18 months and continued for 1 year. It induced rapid improvement of hemangiomas. Two more pulsed dye laser treatments were performed to remove residuals of hemangiomas from the perineum and genital area. Posterior sagital reconstruction by separation of the rectum, mobilization of urogenital sinus and vaginal reconstruction was performed with no major bleeding at the age of 4 years. Postoperatively, after a period of progressive rectal dilatation colostomy was closed. Girl is now 6 years old, dry day and night without residual urine and normal upper tracts. Rectal calibration is normal, fecal continence is still to be evaluated but constipation is easily manageable. CT of the spine and the perineum showed sacral dysplasia and spina bifida with lumbo-sacral lipoma and tethering of terminal filum without neurological deterioration at the moment but requiring close neurological monitoring.ConclusionsLarge perineal hemangiomas are commonly associated with extracutaneous abnormalities. Successful reconstructive surgery is possible after significant reduction of hemangioma by complex treatment.

178 A Comparison of Prophylactic and Symptomatic Detethering of Patients With Lumbosacral Lipomas in a Modern Era

178 A Comparison of Prophylactic and Symptomatic Detethering of Patients With Lumbosacral Lipomas in a Modern Era

Usefulness of three-dimensional T1-weighted spoiled gradient-recalled echo and three-dimensional heavily T2-weighted images in preoperative evaluation of spinal dysraphism

The aim of this study was to evaluate the usefulness of three-dimensional T1-weighted spoiled gradient-recalled echo (3D T1-GRE) images for the preoperative anatomical evaluation of lumbosacral lipoma, thick filum terminale, and myelomeningocele as a means of compensating for the drawbacks of 3D heavily T2-weighted (3D hT2-W) images. Nine patients with lumbosacral lipomas, one patient with tight filum terminale, and five patients with myelomeningoceles were included in this study. 3D T1-GRE images were compared with 3D hT2-W images or conventional magnetic resonance images in terms of delineation of lipomas and other structures in the patients with lipomas and tight filum terminale. For patients with myelomeningoceles, 3D T1-GRE images were compared with 3D hT2-W images in terms of artifacts in the cerebrospinal fluid (CSF) space. The 3D T1-GRE images demonstrated lipomas with good contrast to the spinal cord and CSF space and more clearly delineated the anatomical relationship between lipomas and these structures than did the 3D hT2-W images. The 3D T1-GRE images delineated dural defects through which extradural lipomas penetrated into the intradural space. The 3D T1-GRE images also demonstrated the presence or absence of lipomas in the filum terminale and the absence of artifact in the myelomeningoceles. Furthermore, they were useful for differentiating artifacts observed on the 3D hT2-W images from nerve elements. The complementary use of 3D T1-GRE and 3D hT2-W images may compensate for the drawbacks of 3D hT2-W images and may eventually improve lesion visualization and surgical decision making.

Alteration in radiological subtype of spinal lipoma: case report

We experienced a rare case of lipomyelomeningocele diagnosed at birth by magnetic resonance imaging which transformed the radiological appearance after 2 months into the transitional-type spinal lipoma with rapid increase in size of lipoma. Intraoperative findings revealed the presence of the dural sac extended dorsally outside the vertebral canal, which was characteristic of lipomyelomeningocele. Although there have been several reports showing that the size of spinal lipoma changed during development, there have been no reports which demonstrated alteration of radiological subtype with rapid increase of lipoma. Herein, we describe the first case of lumbosacral lipoma which changed radiological subtype from lipomyelomeningocele into transitional-type spinal lipoma.

Tethered spinal cord syndrome detected during ultrasound for caudal block in a child with single urological anomaly

With the increasing use of ultrasound for assuring safe and accurate caudal or epidural block for postoperative analgesia in children, hidden spinal dysraphism may be found incidentally. Children with congenital anomalies such as VATER or VACTERL frequently present with accompanying occult spinal dysraphisms (OSD), which is characterized with skin-covered lesions such as tethered spinal cord (TSC), meningocele, or lipomyelomeningocele. OSDs are usually found incidentally during the evaluation for other diseases or after neurological symptoms manifest. However, in children with a simple urogenital anomaly, OSD may not be found until the symptoms of OSD manifest because ultrasound screening is not routinely performed in neonates. We report a case of TSC that was discovered during routine ultrasound for caudal block in an asymptomatic child undergoing urological surgery. A 12-month-old, 9.8 kg, otherwise healthy female infant was scheduled to receive a pyeloureterostomy for hydronephrosis. When the patient was placed in lateral decubitus position for caudal block after general anesthesia, an intergluteal simple dimple (1.2 cm in length) was noted. Upon ultrasound (LOGIQe™; GE Healthcare, Wauwatosa, WI, USA) for the spinal structure, we found a thickened filum terminale (FT) 2.2 mm in diameter with low-lying conus medullaris (CM) (L3 level) (Fig. 1A and 1B). We did not perform a caudal block and recommended magnetic resonance imaging (MRI) study for further evaluation. In the postoperative MRI, TSC with FT lipoma was confirmed (Fig. 1C) and detethering surgery was performed after one month. Fig. 1 Ultrasound (A, B) and MRI (C) findings. CM: conus medullaris, FT: filum terminale. In (C), the solid arrow and dotted arrow indicate the low-lying CM and thickened FT. The prognosis of TSC depends mostly on early diagnosis and treatment. While the exact incidence of TSC is not known, greater clinical awareness and MRI diagnostics have lead to a higher rate of incidental detection. Although several studies have reported that children with lumbosacral lipoma have other congenital malformations combined with urogenital anomalies, the association between TSC with a single urogenital anomaly has not been clearly established [1,2]. There have been arguments that minor skin markers are not a stigmata of OSD. One study found OSD to be present in children with minor skin markers and in children without skin markers [3]. Compared to normal infants, in which the CM terminates above L1/2 and the thickness of the FT is < 2 mm at the L5/S1 level, the CM in this patient was located at the L3 level and the FT thickness was 2.2 mm upon ultrasound. Therefore, a pediatric patient presenting with a single urogenital anomaly together with a minor skin marker may require greater attention with further investigation into a possible underlying OSD. Ultrasound is a simple and effective screening method for detecting spinal abnormalities. In clinical practice, if the ultrasound is available for a caudal block, lumbosacral spinal structures should be evaluated thoroughly even in children with a single urogenital anomaly.

Microsurgical treatment of lumbosacral lipoma with tethered cord syndrome

Objective To investigate the classification and microsurgical treatment effect of the combined lumbosacral lipoma with tethered cord syndrome.Methods A retrospective analysis of 39 children patients suffered from lumbosacral lipoma and tethered spinal cord disease in neurosurgery department of the First Affiliated Hospital of Harbin Medical University from May 2008 to July 2011 was performed.According to the preoperative MRI features and intraoperative anatomy characteristics,the patients were divided into the dorsal,transitional,and chaotic lipomas.Under general anesthesia,the microscope lipoma resection and spinal neurolysis cut wire surgery were performed.The children Hoffman functional classification were carried out at one week before surgery and three months after surgery to compare the treatment effects.Results All 39 patients were followed up,with an average of fifteen months,no deaths,12 cases of dorsal lipomas and 18 transitional lipomas were improved or stable after surgery,no neurological deficit increase,the results were satisfactory.9 chatic lipomas were improved or stable after surgery compared with preoperative symptoms,but 2 patients′ incontinence got worse.Two cases occurred cerebrospinal fluid leakage postoperative,one transitional case and one chaotic case,were subject to stringent anti-inflammatory treatment to be healed.Conclusions For the lumbosacral dosal and transitional lipoma either with or without neurological deficit early,as early as possible under the microscope have advocate resection of spinal cord lipoma release the final wire off the surgery;but for the chaotic lipomas,surgery are difficult to fully identify the cauda equina and filum terminale,the symptoms impoved after surgery is not ideal,more compilications,early operation should be carefully considered.

Commentary to the article: asymptomatic lumbosacral lipomas—a natural history study, by Wykes V, Desai D, and Thompson DNP

Commentary to the article: asymptomatic lumbosacral lipomas—a natural history study, by Wykes V, Desai D, and Thompson DNP