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

Author(s):  
Yumiko Komori ◽  
Masahiro Nonaka ◽  
Takamasa Kamei ◽  
Junichi Takeda ◽  
Tetsuo Hashiba ◽  
...  

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.

2021 ◽  
Vol 11 ◽  
Author(s):  
Quanquan Gu ◽  
Yajing Huang ◽  
Hao Zhang ◽  
Biao Jiang

BackgroundDiffuse midline glioma with H3K27-altered (DMG-H3K27a) is a novel tumor entity of the pediatric-type diffuse high-grade tumor in the latest WHO CNS 5. It mostly affects children and is only rarely found in adults. The tumor has a high level of aggressiveness, with a rapid progression and bad prognosis. In adults, the spinal cord is the most common site of DMG-H3K27a. Rare adult cases of primary DMG-H3K27a in the spinal cord were reported in this study, together with clinico-histopathologico-radiographic data.MethodsFrom January 2016 to December 2020, we conducted a retrospective study of five adults with primary DMG-H3K27a in the spinal cord, analyzing their clinical, pathohistological, and radiographic datasets from the first diagnosis to follow-up.ResultsAll five patients were diagnosed for the first time and were given full treatment. In three of the five patients, post-operative follow-up revealed tumor recurrence. The longest survival of the five patients was 45 months at the time of report submission, while the longest progression-free survival (PFS) following surgery was 20 months. Immunohistochemical studies showed the tumors featured aggressive behavior (grade 4) and were positive for the H3K27M mutation. The radiographic appearances were varied, but they were all initially mistaken as benign. DMG-H3K27a in the spinal cord was characterized by isointense/hyperintense on T1WI and isointense/hyperintense on T2WI, as well as cystic necrosis and peripheral spinal cord edema, as well as central canal enlargement and other types of enhancement.ConclusionThis is the first case report focusing on adult DMG-H3K27a of the pediatric-type diffuses high-grade gliomas in the spinal cord. In our cases, we discovered the following: 1) adults had a better prognosis with a longer PFS compared with prior pediatric reports; 2) despite aggressive behavior under the microscope, radiographic appearances of the tumors were less aggressive; and 3) adjuvant treatment, including TCM, may have played a role in the prognosis.


1998 ◽  
Vol 116 (6) ◽  
pp. 1879-1881 ◽  
Author(s):  
Silvana Fahel da Fonseca ◽  
Maria Stella Figueiredo ◽  
Rodolfo Delfini Cançado ◽  
Fernando Nakandakare ◽  
Roberto Segreto ◽  
...  

CONTEXT: Spinal cord compression due to extramedullary hematopoiesis is a well-described but rare syndrome encountered in several clinical hematologic disorders, including <FONT FACE="Symbol">b</font>-thalassemia. CASE REPORT: We report the case of a patient with intermediate <FONT FACE="Symbol">b</font>-thalassemia and crural paraparesis due to spinal cord compression by a paravertebral extramedullary mass. She was successfully treated with low-dose radiotherapy and transfusions. After splenectomy, she was regularly followed up for over four years without transfusion or recurrence of spinal cord compression. DISCUSSION: Extramedullary hematopoiesis should be investigated in patients with hematologic disorders and spinal cord symptoms. The rapid recognition and treatment with radiotherapy can dramatically alleviate symptoms.


2004 ◽  
Vol 62 (3b) ◽  
pp. 885-888
Author(s):  
Donizeti Honorato ◽  
Wilson Borges ◽  
Antonio Augusto Roth Vargas ◽  
Ricardo Ramina

Syringohydromyelia is defined as a longitudinal dilatation of the central canal of the spinal cord with accumulated cerebrospinal fluid. This condition may cause neurologic deficits when the cavity enlarges and compresses the spinal cord. We present the case of a 33 years-old female with progressive paraparesis caused by syringohydromyelia. This patient underwent previously multiple clinical and surgical treatments for severe form of neurocysticercosis. Surgical decompression of the posterior fossa and syringostomy resolved the neurologic symptoms. The possibility of syringohydromyelia should be considered in the case of patients who have previously undergone surgical and clinical treatment for severe form of neurocysticercosis.


PM&R ◽  
2010 ◽  
Vol 2 ◽  
pp. S127-S128
Author(s):  
Walter C. Damper ◽  
Steve M. Gnatz ◽  
Robbie Logan ◽  
Mohammed N. Siddiqui

2017 ◽  
Vol 2017 ◽  
pp. 1-5 ◽  
Author(s):  
Mahmoud Almasri ◽  
Waleed Kishta ◽  
Fahad H. Abduljabbar ◽  
Vincent Arlet ◽  
Neil Saran ◽  
...  

Ischiospinal Dysostosis (ISD) is a complex and very rare medical entity. It is associated with kyphoscoliosis, dysplasia or aplasia of the ischial rami, segmental anomalies of the bony vertebrae, and peculiar facial morphologies. In this case report, we present a child with Ischiospinal Dysostosis and Pierre-Robin Syndrome. This case report is unique as we followed the patient for 13 years in which he had multiple spinal procedures to treat his kyphoscoliosis. In this paper, we elucidated the number of case reports with documented follow-up regarding spinal cord injury or other complications of ISD and its management.


1992 ◽  
Vol 76 (1) ◽  
pp. 23-31 ◽  
Author(s):  
Michael B. Bracken ◽  
Mary Jo Shepard ◽  
William F. Collins ◽  
Theodore R. Holford ◽  
David S. Baskin ◽  
...  

✓ The 1-year follow-up data of a multicenter randomized controlled trial of methylprednisolone (30 mg/kg bolus and 5.4 mg/kg/hr for 23 hours) or naloxone (5.4 mg/kg bolus and 4.0 mg/kg/hr for 23 hours) treatment for acute spinal cord injury are reported and compared with placebo results. In patients treated with methylprednisolone within 8 hours of injury, increased recovery of neurological function was seen at 6 weeks and at 6 months and continued to be observed 1 year after injury. For motor function, this difference was statistically significant (p = 0.030), and was found in patients with total sensory and motor loss in the emergency room (p = 0.019) and in those with some preservation of motor and sensory function (p = 0.024). Naloxone-treated patients did not show significantly greater recovery. Patients treated after 8 hours of injury recovered less motor function if receiving methylprednisolone (p = 0.08) or naloxone (p = 0.10) as compared with those given placebo. Complication and mortality rates were similar in either group of treated patients as compared with the placebo group. The authors conclude that treatment with the study dose of methylprednisolone is indicated for acute spinal cord trauma, but only if it can be started within 8 hours of injury.


2014 ◽  
Vol 21 (2) ◽  
pp. 223-226
Author(s):  
R.S. Mittal ◽  
Pankaj Gupta ◽  
Ashok Gandhi ◽  
Achal Sharma ◽  
Vinod Sharma ◽  
...  

Abstract Myelocystocele is a rare variety of spinal dysraphism that presents as a skin covered, midline, lumbosacral mass. Many a time it is associated with other congenital anomalies but isolated myelocystocele is rarely associated with neurological deficit. MRI is the modality of choice for preoperative diagnosis. A 3 years old female child presented with skin covered lumbosacral mass since birth. There was no associated neurological deficit. MRI revealed single cyst, which was continuous with central canal of spinal cord. Peroperatively, myelocystocele was found with tethering of cord. Untethering of cord and repair of myelocystocele was performed with uneventful recovery.


Neurosurgery ◽  
1986 ◽  
Vol 18 (5) ◽  
pp. 611-615 ◽  
Author(s):  
David G. McLone ◽  
Thomas P. Naidich

Abstract The value of laser resection was assessed in the last 50 of 128 consecutive cases of pediatric spinal lipoma. In this series, there was no mortality. No patient suffered increase in neurological or urological deficit. Postoperatively, 8 of 20 patients with prior motor deficit (40%) had substantially improved motor function. Two of 17 previously incontinent patients became continent of urine (12%). Use of the CO2 laser reduced the length of operation, the intraoperative blood loss, and the degree of manipulation of the spinal cord and nerve roots. Most of the fat could be removed successfully from the liponeural junction, permitting more nearly anatomical removal of the intramedullary component of the lesion and greater ease in replacing the cord into a reconstructed arachnoid-dural canal. In the authors' opinions, proper management of the patient with spinal lipoma now requires early prophylactic resection of the lipoma and untethering of the spinal cord.


2018 ◽  
Vol 21 (5) ◽  
pp. 498-503 ◽  
Author(s):  
Stephen J. Johans ◽  
Kevin N. Swong ◽  
Daniel J. Burkett ◽  
Michael P. Wemhoff ◽  
Sean M. Lew ◽  
...  

Superficial siderosis (SS) of the CNS is a rare and often unrecognized condition. Caused by hemosiderin deposition from chronic, repetitive hemorrhage in the subarachnoid space, it results in parenchymal damage in the subpial layers of the brain and spinal cord. T2-weighted MRI shows the characteristic hypointensity of hemosiderin deposition, classically occurring around the cerebellum, brainstem, and spinal cord. Patients present with progressive gait ataxia and sensorineural hearing impairment. Although there have been several studies, case reports, and review articles over the years, the clear pathophysiology of subarachnoid space hemorrhage remains to be elucidated. The proposed causes include prior intradural surgery, prior trauma, tumors, vascular abnormalities, nerve root avulsion, and dural abnormalities.Surgical repair of a dural defect associated with SS has been shown to be efficacious at preventing symptomatic progression. There have been several reports of dural defects within the spinal canal treated with surgery. Here, the authors present the first known case of a dural defect of the ventral skull base, namely a clival meningocele, presumed to be causing SS. In this case report, a 10-year-old girl with a history of head trauma at the age of 3 years was found to have a clival meningocele 3 years after her original trauma. On follow-up imaging, the patient was found to have radiographic growth of the meningocele along with evidence of SS of the CNS. The patient was treated conservatively until she began to have progressive hearing loss. It was presumed that the growing meningocele was the source of her SS. An endoscopic endonasal transclival approach with a multilayer dural reconstruction was performed to fix the dural defect and repair the meningocele in hopes of mitigating the progression of her symptoms. At her 12-month postoperative follow-up, she was doing well, with audiometry showing a slightly decreased hearing threshold in the left ear but improved speech discrimination bilaterally. Postoperative MRI showed a stable level of hemosiderin deposition and meningocele repair. Long-term follow-up will be necessary to evaluate for continued clinical stabilization or possible improvement.


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