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.

Comparison of intraoperative neurophysiological monitoring methods for lumbosacral lipoma surgery in infants.

BACKGROUND: Stable intraoperative neurophysiology monitoring of infants, especially very young infants, is challenging. In this study, motor evoked potentials (MEPs), the bulbocavernosus reflex (BCR), and somatosensory evoked potentials (SEPs) were simultaneously monitored in infants with lumbosacral lipomas, and these methods were compared retrospectively. METHODS: A total of 21 surgeries performed for lumbosacral lipoma in patients less than 1 year old were studied. The mean age at surgery was 133.8 days (range 21-287 days; ≤ 120 days: 9 cases, > 120 days: 12 cases). Transcranial MEPs were measured in the anal sphincter and gastrocnemius, and tibialis anterior and other muscles were added as needed. The BCR was measured by the electromyogram of the anal sphincter muscle with stimulation of the pubic region, and SEPs were measured from the waveforms of stimulation of the posterior tibial nerves. RESULTS: For the BCR, stable potentials could be recorded for all 9 cases at ≤ 120 days of age. In contrast, for MEPs, stable potentials could be recorded in only 4 of 9 cases (p < 0.05). For all patients > 120 days of age, MEPs and the BCR were measurable. SEPs were undetectable in some patients regardless of age. CONCLUSION: The BCR could be more consistently measured than MEPs in infant patients with lumbosacral lipoma at ≤ 120 days of age.

Does conus morphology have implications for outcome in lumbosacral lipoma?

OBJECTIVE: Lumbosacral lipomas (LSL) of the 'dorsal' type have been associated with more favourable outcomes compared with other conus region lipomas. We hypothesised that integrity of the conus on MRI underpins the improved prognosis in this subgroup of LSL patients. METHODS: The definition of 'dorsal lipomas' included lipomas with attachment to the conus, but where the conus could be delineated on MRI (Morota type 1) as reported by Morota et al. (J Neurosurg Pediatr 19:428-439, 2017). Additional inclusion criteria included asymptomatic status at presentation, age >3 years at follow-up, and neurological and urological evaluation at presentation and at last follow-up. Lipoma extent and conus level were recorded. Outcome measures were the need for untethering surgery and neuro-urological status at last follow-up. Urological outcomes were defined by continence and efficacy of bladder emptying. RESULTS: Twenty-six children were included (median age 8.7 years). Conus level was low (at or below L2) in 92%. Nine required untethering surgery: 5 prophylactic, 4 because of clinical deterioration. Twenty-five children were continent at last follow-up, one had stress incontinence, and none required catheterisation. One had persisting ankle weakness after surgery requiring orthotic support. CONCLUSIONS: In LSL of the conus, visualisation of the conus on MRI is associated with good urological and motor outcomes. The integrity of the conus appears to be a more important prognostic factor than anatomical level. An observational approach to this group of LSL patients does not appear to compromise outcomes. These findings support a selective approach to untethering surgery.

Whole spine MRI is not required in investigating uncomplicated paediatric lumbosacral lipoma. A retrospective single-institution review.

PURPOSE: Lumbosacral lipoma (LSL) is a severe occult spinal dysraphism, frequently associated with neurological, urological and orthopaedic complications. Whole spine imaging is typically performed to identify concomitant, but spatially separate, congenital anomalies. Our hypothesis: the incidence of additional, clinically significant abnormalities of the neuraxis is low; thus, imaging should be optimised at the lumbosacral region. We aim to assess the prevalence and relevance of LSL-associated lesions. METHOD: A single-centre, retrospective, radiological review using a prospectively maintained operative database. INCLUSION CRITERIA: children (< 16 years) with confirmed diagnosis of LSL and received whole spine MRI. Fatty filum, syndromic cases and cutaneous stigmata above lesion level were excluded. Data was extracted from radiological imaging, reports and clinical correspondence. RESULTS: One hundred twelve patients (40:72, M:F) aged 0.5 years (0.2-2.7) (median ± IQR) with LSL had whole spine MRI between 2001 and 2017. Classification of LSL: transitional 48 (43%); dorsal 30 (27%); caudal 28 (25%) and chaotic 6 (5%). Additional anomalies included syringohydromyelia 44 (39%), subcutaneous tract 19 (17%), abnormal vertebral segmentation 18 (16%), dermoid cyst 1 and 1 Chiari I deformity. There were no Chiari II malformations. No child required surgery for an associated lesion. Binary logistic regression revealed no factors associated with predicting secondary lesions. CONCLUSIONS: In congenital LSL, additional anomalies of the neuraxis are typically loco-regional rather than pan-CNS and additional lesions are rarely clinically significant. The loco-regional distribution of anomalies suggests that only lumbosacral spinal imaging is required in the initial evaluation of LSL. Such a policy would lessen the anaesthetic/sedation time for children and reduce imaging cost per patient. MRI protocols could be refined to optimise imaging quality at the region of interest.

Radical excision of lumbosacral lipoma: an early experience of "followers".

BACKGROUND: Indication, timing, and method for surgical treatment of lumbosacral lipoma are controversial. Radical resection of the lumbosacral lipoma and complete reconstruction of the placode are supported in that better long-term outcome can be achieved without increasing complication rate compared with traditional surgical techniques. OBJECTIVE: We analyzed the early surgical outcomes of lumbosacral lipoma treated with the untethering and radical excision of fat. METHODS: Retrospective analysis of surgically treated 81 fresh lumbosacral lipoma cases with dorsal, transitional, and chaotic types and true lipomyelomeningocele (LMMC) was performed. Caudal and filar types were excluded. RESULTS: Complete untethering was accomplished in 98%. Radical excision of the lipoma was attempted in all cases and achieved in 83%. Postoperative neurological complication was observed in 8 cases (10%). Group of lipoma types (dorsal + transitional vs. chaotic + true LMMC) and availability of radical lipoma excision turn out to be factors related to neurological outcomes in univariate analysis (p < 0.001 and p = 0.027, respectively). Group of lipoma types, availability of radical excision, and postoperative cord/dural sac (C/D) ratio are related factors in multivariate analysis (p = 0.025, p = 0.049, and p = 0.031). CONCLUSIONS: As a follower of untethering and radical excision of fat, careful consideration is required to plan the surgery of lumbosacral lipoma on account of the "underestimated" complication rate. Type of the lipoma is the important factor determining the surgical outcome. Availability of complete radical excision and postoperative C/D ratio are the operative factors related to the neurological outcomes.

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

AIMS: Lumbosacral 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 RESULTS: The 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. CONCLUSIONS: Our 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.

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.

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

INTRODUCTION: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

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

INTRODUCTION: We conducted a retrospective study of children with lumbosacral lipomas treated in the Department of Pediatric Neurosurgery hospital Timone Enfants in the last 20 years. 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.

Retethering : A Neurosurgical Viewpoint.

During the follow-up period after surgery for spinal dysraphism, a certain portion of patients show neurological deterioration and its secondary phenomena, such as motor, sensory or sphincter changes, foot and spinal deformities, pain, and spasticity. These clinical manifestations are caused by tethering effects on the neural structures at the site of previous operation. The widespread recognition of retethering drew the attention of medical professionals of various specialties because of its incidence, which is not low when surveillance is adequate, and its progressive nature. This article reviews the literature on the incidence and timing of deterioration, predisposing factors for retethering, clinical manifestations, diagnosis, surgical treatment and its complications, clinical outcomes, prognostic factors after retethering surgery and preventive measures of retethering. Current practice and opinions of Seoul National University Children's Hospital team were added in some parts. The literature shows a wide range of data regarding the incidence, rate and degree of surgical complications and long-term outcomes. The method of prevention is still one of the main topics of this entity. Although alternatives such as spinal column shortening were introduced, re-untethering by conventional surgical methods remains the current main management tool. Re-untethering surgery is a much more difficult task than primary untethering surgery. Updated publications include strong skepticism on re-untethering surgery in a certain group of patients, though it is from a minority of research groups. For all of the abovementioned reasons, new information and ideas on the early diagnosis, treatment and prevention of retethering are critically necessary in this era.

Rapid deterioration of an asymptomatic lumbosacral lipoma due to formation of an extracanalicular syrinx: case report.

The authors present the case of a 1-month-old girl with a lumbosacral lipoma who then developed an extracanalicular syrinx and experienced rapid deterioration. The patient's initial MRI study, obtained before she became symptomatic, revealed a spinal lipoma with a syrinx in contact with the lipoma-cord interface. She was initially asymptomatic but developed loss of motor function in the left leg 14 days after MRI. Emergency surgery was performed. Intraoperative findings revealed a swollen spinal cord. Lipomatous tissue on the caudal side of the conus was removed subtotally, and the central canal was opened. Expansion of the syrinx was observed intraoperatively. Postoperatively, the patient's left leg paresis remained. Postoperative MRI revealed rostral and extracanalicular expansion of the syrinx. This is the first report on the rapid deterioration of a conus lipoma due to extracanalicular expansion of a syrinx. Careful follow-up and repeat MRI should be considered for patients with spinal lipomas with syringomyelia, especially when the syrinx is attached to the lipoma-cord interface.

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

OBJECTIVE: 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. METHODS: 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. RESULTS: The most common symptom was bladder dysfunction (82.0%), followed by constipation (76.2%). At the 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. A binary 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. CONCLUSIONS: 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.

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.

Diagnóstico de lipoma lumbosacro y síndrome de médula anclada mediante resonancia magnética en un adolescente / Diagnosis of lumbosacral lipoma and anchored marrow syndrome by means of magnetic resonance in an adolescent

Se presenta el caso clínico de un paciente de 13 años de edad, quien había sido operado en el primer año de vida como consecuencia de un lipomeningocele, que en esta ocasión acudió al Hospital General Docente "Dr. Juan Bruno Zayas Alfonso" de Santiago de Cuba, donde se le diagnosticó, mediante resonancia magnética, una imagen hiperintensa en T1 y T2 en región lumbar, intrarraquídea, adosada al filum terminal, de posible origen graso. Se le realizó secuencia de supresión de grasa (STIR) y se definieron 2 malformaciones congénitas: lipoma lumbosacro y síndrome de médula anclada.

The case report of a 13 years patient who had been operated in the first year of life due to a lipomeningocele who in this occasion visited "Dr. Juan Bruno Zayas Alfonso" Teaching General Hospital in Santiago de Cuba, where he was diagnosed, by means of magnetic resonance, an hyperintense image in T1 and T2 in the intrarraquideal lumbar region, embedded to the phylum terminal, possibly of fatty origin, is presented. A sequence of fat suppression (SFS) was carried out and two congenital malformations were defined: lumbosacral lypoma and anchored marrow syndrome.