“… 3 Lumboperitoneal shunting to divert CSF and the use of fibrin glue injection has also been used in some cases. 2 , 5 Needle guided aspiration should be avoided as it increases risks of meningitis. 1 Management of the ureteric obstruction should continue through ureteric stent exchange until the meningocele has been treated.…”
Section: Discussionmentioning
confidence: 99%
“…The size of this meningocele is noted to be one of the largest reported in the literature. 2 Fig. 1 Sagittal T2-weight fat supressed magnetic resonance imaging demonstrating a large meningocele extending from the right S1 nerve root, measuring 16.8 × 11.6 cm, causing significant displacement of pelvic organs.…”
Section: Case Presentationmentioning
confidence: 99%
“…Dural ectasia, present in over 90% of patients with Marfan's syndrome, refers to a widening of the thecal or dural sac as result of the defective elastic fibres. 2 In rare cases, the weakness of the dura results in a herniation of the cerebrospinal fluid out into retroperitoneum and pelvis, termed a meningocele. 1 Meningoceles can form both anteriorly and posteriorly to the spinal cord, and in their most severe form can present as large pelvic masses.…”
Sacral Meningoceles, ductal ectasia and pseudomeningoceles are all rare spinal defects that occur due to errors in collagen biosynthesis in the setting of Marfan's Syndrome. Meningoceles, which are extradural collections of cerebrospinal fluid, can form large pelvic collections which can compress local structures. In rare cases, this can lead to extrinsic ureteric obstruction, which can result in acute renal failure and urosepsis. We present a case of a 35-year old female with Marfan's syndrome, with one of the largest sacral meningoceles reported in the literature, causing acute ureteric obstruction, requiring urgent surgical intervention.
“… 3 Lumboperitoneal shunting to divert CSF and the use of fibrin glue injection has also been used in some cases. 2 , 5 Needle guided aspiration should be avoided as it increases risks of meningitis. 1 Management of the ureteric obstruction should continue through ureteric stent exchange until the meningocele has been treated.…”
Section: Discussionmentioning
confidence: 99%
“…The size of this meningocele is noted to be one of the largest reported in the literature. 2 Fig. 1 Sagittal T2-weight fat supressed magnetic resonance imaging demonstrating a large meningocele extending from the right S1 nerve root, measuring 16.8 × 11.6 cm, causing significant displacement of pelvic organs.…”
Section: Case Presentationmentioning
confidence: 99%
“…Dural ectasia, present in over 90% of patients with Marfan's syndrome, refers to a widening of the thecal or dural sac as result of the defective elastic fibres. 2 In rare cases, the weakness of the dura results in a herniation of the cerebrospinal fluid out into retroperitoneum and pelvis, termed a meningocele. 1 Meningoceles can form both anteriorly and posteriorly to the spinal cord, and in their most severe form can present as large pelvic masses.…”
Sacral Meningoceles, ductal ectasia and pseudomeningoceles are all rare spinal defects that occur due to errors in collagen biosynthesis in the setting of Marfan's Syndrome. Meningoceles, which are extradural collections of cerebrospinal fluid, can form large pelvic collections which can compress local structures. In rare cases, this can lead to extrinsic ureteric obstruction, which can result in acute renal failure and urosepsis. We present a case of a 35-year old female with Marfan's syndrome, with one of the largest sacral meningoceles reported in the literature, causing acute ureteric obstruction, requiring urgent surgical intervention.
“…La sintomatología secundaria al meningocele sacro anterior está directamente relacionada con las estructuras y raíces nerviosas que comprima abarcando desde dolor lumbar crónico (siendo el principal síntoma) hasta estreñimiento, incontinencia urinaria, sintomatología ginecológica, descritas en un 10% de los casos de acuerdo a Oren M et al (9) . De acuerdo a Hollenberg et al (10) , el hallazgo de una masa palpable puede acompañarse de los síntomas antes descritos.…”
“…Treatment options include clinical observation and surgical intervention for huge or symptomatic lesions. 10 Herein, we report on a patient with Marfan syndrome that presented as a giant ASM with iatrogenic CSF leakage, and we address diagnosis, imaging results, and surgical management.…”
BACKGROUND
Marfan syndrome is rarely accompanied by anterior sacral meningocele (ASM) resulting from erosion of the sacrum by dural ectasia. ASM may induce symptoms due to severe mass effects. ASM may also mimic ovarian cysts, and the risk of cerebrospinal fluid (CSF) leakage is high if spontaneous rupture of the cyst occurs. In this study, the authors presented a rare case of ASM with iatrogenic CSF leakage in a 34-year-old woman with suspected Marfan syndrome.
OBSERVATIONS
The patient initially presented with a giant ASM that was first misdiagnosed as an ovarian cyst. Previously, it had been partially resected, which was followed by iatrogenic CSF leakage. Symptoms of intracranial hypotension, including postural headache and dizziness, developed within 1 month. Brain magnetic resonance imaging (MRI) showed pituitary enlargement, bilateral subdural effusion, and tonsillar herniation. Preoperative computed tomography myelography provided three-dimensional (3D) examination of the deformed sacrum and CSF leakage site. Transabdominal approaches led to primary repair, and repair of the meningocele was achieved by intraoperative fluorescein fluorescence and 3D printed model–guided polymethyl methacrylate bone cement reconstruction. No CSF leakage or recurrent ASM was found at the 1.5-year follow-up visit.
LESSONS
Intraoperative intrathecal fluorescence and 3D-printed models are useful for ASM repair. Preoperative MRI is helpful for differentiating ASM from other causes of a huge pelvic mass, including ovarian cyst.
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