Anterior sacral meningocele in Marfan syndrome
Latest PubMed Anterior sacral meningocele in Marfan syndrome-related articles
Twenty-four Marfan and 2 Loeys-Dietz patients had anterior sacral meningocele at follow-up, compared with 21 and 1, respectively, at baseline. Three Marfan patients developed herniation of a nerve root sleeve during follow-up. This was not seen in other individuals. The dural sac ended significantly lower at follow-up, and the dural sac ratio at level L5 was significantly increased from baseline in the Marfan patients.
In Marfan and Loeys-Dietz syndrome, dural ectasia may present or worsen during adulthood. The cut-off value of dural sac ratio at level S1 is suggested elevated to 0.64. The results from the present study may help as guidance for appropriate follow-up of patients with dural ectasia 1).
Case reports
A 24-year-old woman with Marfan syndrome presented with polyuria, recurrent urinary tract infections, and renal injury for 3 months along with a globose abdomen, with a palpable mass in the middle and lower third of the abdomen that was massive on percussion. MRI showed an ASM consisting of two cystic lesions measuring 15.4 × 14.3 × 15.8 and 6.7 × 6.1 × 5.9 cm, respectively, compressing the distal third of the right ureter and causing a hydroureteronephrosis. Drainage and ligature of the cystic lesion were performed. The urinary outcome was excellent, with full recovery after surgery.
Lessons: ASM should be suspected in all abdominal masses with progressive symptoms in the setting of Marfan syndrome. Computed tomography and MRI are important to investigate genitourinary anomalies or other types of dysraphism to guide the best surgical approach 2)
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 3).
A case of 46-year-old woman who presented with urinary incontinence, early satiety, and back pain in the setting of a known anterior sacral meningocele. Before operative management, the anterior sacral meningocele ruptured with the patient presenting signs and symptoms of intracranial hypotension. Conservative management did not alleviate the pain. She was ultimately managed with posterior sacroplasty followed by anterior sacral meningocele resection and placement of a lumboperitoneal shunt. The patient did not have reaccumulation of the meningocele or recurrent symptoms at the latest follow-up.
The progression of dural ectasia in Marfan syndrome to an anterior sacral meningocele is uncommon. It is important to identify the characteristics associated with an expanding dural ectasia as this patient's symptoms progressed over time and the meningocele grew large. Given its rarity, there are no guidelines in place regarding size at which repair of an anterior sacral meningocele should occur prophylactically. It is important to review these cases in order continue to learn about progression, management, and outcomes of patients with an anterior sacral meningocele. 4).
A 43-year-old woman with a history of Marfan syndrome and a large ASM was referred for neurosurgical intervention. The ASM was filling the pelvic cavity and causing severe compression of the bladder. The patient underwent surgical decompression of the cyst through an anterior transabdominal approach and closure of the fistulous tract with a pedicled omental flap. This is the first reported case of successful closure of an ASM with an omental flap. At the 6-month follow-up, the ASM had not recurred on imaging and the patient's symptoms had resolved. Anterior sacral meningoceles are rare lesions that often require neurosurgical intervention. Although most can be treated posteriorly, large ASMs compressing the abdominal or pelvic organs may require a transabdominal approach. Moreover, ASMs with wide dural stalks may benefit from closure with an omental flap. 5).
A 43-year-old man diagnosed with Marfan syndrome suffered sacrococcygeal trauma. He was admitted to the emergency room due to symptoms of headache, nausea, and lower limb subjective weakness. CT and MRI showed a large retroperitoneal mass with hemorrhagic content close to the sacrum. Likewise, the MRI showed an image compatible with subarachnoid hemorrhage in the thoracic spinal area, cerebral convexity, and the basal cisterns. The patient went into surgery for an anterior abdominal approach in the midline to reduce the content of the lesion, and subsequently, in the same act, a posterior approach was done with an S1-S2 laminectomy and obliteration of the pedicle. Postoperative MRI 5 months later showed resolution of the ASM.
Anterior sacral meningocele is characterized by herniation of the dura mater and the arachnoid mater outside the spinal canal through a defect of the sacrum. We add the risk of bleeding after trauma-never seen in the literature-as one of the possible inherent complications of this lesion.
This report highlights a complication never seen in the literature of a relatively rare condition. In our case, the combined approach was effective for both clinical control and lesion regression. 6).
A patient with Marfan's syndrome and ASMs who was referred to gynaecology owing to dysmenorrhoea and left-sided pelvic pain radiating to the left leg. A transvaginal ultrasound scan (TVUS) detected a left pelvic cystic tubular structure, attributed to a hydrosalpinx, which, in retrospect, likely corresponded to the ASM. The patient went on to have TVUS-guided drainage of this cystic structure, resulting in an ASM abscess. It is difficult to distinguish ASM from the vastly more common hydrosalpinx using TVUS alone, and in patients with an atypical appearing posteriorly positioned cystic pelvic lesion or in the presence of underlying conditions known to be associated with ASMs, MRI should be considered before any interventional procedure to drain the suspected hydrosalpinx transvaginally. The patient was successfully treated using a minimally invasive CT-guided posterior trans-sacral drainage technique. 7).
A 20-year old female with Marfan syndrome who presented with abdominal distention that was misdiagnosed as an ovarian cyst on pelvic ultrasound. Pelvic magnetic resonance (MR) imaging showed large, well-defined multiloculated intrasacral and presacral cysts communicating via two separate broad necks and extending through defects in anterior aspect of sacral vertebrae. This case emphasizes that anterior sacral meningocele should be considered in the differential diagnosis of cases with pelvic cysts particularly in patients with underlying connective tissue disorders. Because severe neurologic complications or even death may occur without proper preoperative planning in such cases, MR imaging should always be performed for evaluation and characterization of pelvis cystic lesions. 8).
A young woman with Marfan syndrome who was being treated with anticoagulants for a prosthetic heart valve and who presented with a spontaneous retroperitoneal hemorrhage requiring surgical evacuation. No CSF leak was encountered at surgery, but she developed progressively more severe positional headaches over the following year. She then experienced the sudden onset of acute urinary obstruction, at which time CT revealed a 17 × 15 × 13-cm presacral pseudomeningocele communicating with the thecal sac through a sacral bone defect. An anterior surgical approach was used for drainage of the pseudomeningocele as well as for primary closure of the dural defect with a bovine pericardial patch and autologous subcutaneous fat graft. After a short period of lumbar subarachnoid drainage of the CSF, the patient was able to resume normal activity without recurrent symptoms. To the authors' knowledge, such a pseudomeningocele in a patient with Marfan syndrome has been reported only twice, and this case features the largest pseudomeningocele to date. They also review the pertinent literature regarding presentation, diagnosis, and management of these lesions. 9).
Relapse of Escherichia coli meningitidis due to sacral meningocele in Marfan syndrome, treated only with antibiotherapy 10).
Surgical Treatment of Anterior Sacral Meningoceles in Patients with Marfan Syndrome: A Report of Three Cases 11).
A case of bacterial meningitis secondary to fistulous communication between a sacral meningocele and sigmoid colon in the setting of diverticulitis 12).
Five women who underwent laparoscopic transperitoneal surgery were clinically, radiologically, and surgically evaluated.
Result: All 5 patients underwent laparoscopic transperitoneal surgery and showed satisfactory results. They had no major complications. Three patients had headaches as minor complications, but it was gone in at most 3 days. Decrease in operative time, blood loss, and length of hospitalization were the advantages of the procedure.
The laparoscopic approach to treating anterior sacral meningocele was feasible and safe, with only minor complications. 13).
A woman with Marfan's syndrome presenting with a clinical picture of acute gastroenteritis in whom severe bilateral hydroureteronephrosis associated to a neurogenic bladder and a giant anterior sacral meningocele was diagnosed incidentally. The importance of this case lies in the fact that the patient was asymptomatic despite the significant visceral repercussions already occurring that led to questioning of whether MRI follow-up would still be advisable even in the absence of symptoms. 14).
A 46-year-old woman with Marfan's syndrome exhibiting an incomplete Currarino triad and recurrent bacterial meningitis which recurred three times in about six months. An anterior sacral meningocele (ASM) was noted in a lumbar MRI, and multiple bone defects in the sacrum were noted in 3D-CT. Surgical approach to prevent the recurrence of infection was limited to plasty for the meningocele, but no meningitis has been observed for two years. Radical surgery should be considered in cases with ASM who recurred bacterial meningitis. 15).
A 52 year old woman with Marfan syndrome who presented with a significantly large anterior sacral meningocele without having associated symptoms. In light of this case, we recommend that asymptomatic Marfan patients with dural ectasia should be closely observed without need for immediate surgical intervention 16).
18-year-old man with Marfan syndrome, diagnosed upon MRI morphological evaluation which showed a huge cystic mass in the pelvic space. Surgical excision even if curative was not performed in consideration of a stationary picture after one year since diagnosis. 17).
Two cases presenting as nongynaecological pelvic masses are described highlighting the clinical difficulty in diagnosis and the classical radiological findings 18).
Anterior sacral meningocele presenting as a pelvic/abdominal mass in a patient with Marfan syndrome 19).
The first case of a giant anterior sacral meeningocele rapidly cured by a simple endoscopic procedure 20). The postoperative course of the patient was characterized by immediate disappearance of the preoperative clinical signs. Computed tomographic scans demonstrated progressive complete collapse of the anterior sacral meningocele and partial resolution of the intrasacral component 21).
A marfanoid patient underwent celiotomy for a pelvic mass and was found to have an anterior sacral meningocele. Nine years prior to that time she had undergone surgery because of a “sacrococcygeal cyst,” which in retrospect seemed to be a posterior meningocele 22).
Purulent meningitis from surgical inoculation of an anterior sacral meningocele 23).
Anterior sacral meningocele in association with Marfan's syndrome 24).
HGUA
A 32-year-old female was referred by a giant presacral anterior meningocele with symptoms of abdominal distension and frequent urination. Past medical history of hypertension, Marfan syndrome with bilateral (blindness) and aorta dilation. Hashimoto's thyroiditis without treatment. Cataracts. Retinal detachment. Surgically intervened scoliosis. Tympanoplasty for chronic otitis media. Mandibular fracture
Enlargement of the lumbosacral canal and dural sac were consistent with dural ectasia, with herniations of cystic morphology located anteriorly and posteriorly through several lower right lumbar and bilateral sacral foramina. The largest cystic lesion measures approximately 15 cm in maximum diameter and communicates with the spinal canal through the left S1-S2 sacral foramen, occupying a significant portion of the pelvis with anterior displacement of the intestinal loops and bladder, findings are consistent with a large anterior sacral meningocele. Signs of left-sided thoracolumbar scoliosis and generalized morphological alterations of the vertebral bodies with a decrease in their anteroposterior diameter. Stabilizing rods for thoracolumbar scoliosis with transpedicular fusion screws at L1-L3.
General anesthesia and prone position on Wilson's frame. Intraoperative monitoring of lumbosacral nerve roots. Midline skin incision at the lumbosacral level, from the spinous process of L5 to S4. Desperiostization of sacral laminae and bilateral laminectomy from S1 to S3. Several dural tears occur during the opening with a motor due to significant adhesion. Midline dural opening and visualization of a large sac and a large left S1 foramen, with anterior meningocele extending to the retroperitoneum. Dissection of the meningocele capsule over the peritoneum is impossible. Two sacral nerves are identified entering through the foramen, preventing primary dural closure at that level. Dissection of the foramen while preserving the left S1 and S3 nerves and placement of a titanium plate at the entry, screwed into the S1 body medially. Multiple Tachosil sheets are placed, sealing the entry. Subsequently, a semi-hermetic dural closure is performed and sealed with Tachosil. Replacement of sacral laminae with four titanium plates. Closure of muscles and layers with Vicryl, as well as subcutaneous closure. Skin closure with non-absorbable monofilament. An external lumbar drainage is left in place, connected to a bag, positioned 4-5 cm above the external auditory canal.
3 months later the patient comes to the Emergency Department complaining of severe, non-radiating left-sided low back pain that has been present for one day. They also report two days of constipation and two episodes of vomiting.
The anterior transperitoneal abdominal approach in Lloyd Davies position. Supra and infra umbilical midline laparotomy. Retroperitoneal opening over the meningocele and release of adhesions to the uterus and both ovaries, sigmoid colon, and presacral space until visualization of the meningocele neck. Hemostasis confirmation with fibrillar Surgicel and Floseal.
Once the meningocele is dissected, intraquistic catheter localization is performed up to the S1 foramen. The cyst is opened 2 cm around the foramen and the intraquistic catheter is posteriorly removed. Primary closure with 1/0 silk sutures in multiple layers until a watertight seal is achieved. Sealed with Tachoseal.
Placement of a Blake drain in the Douglas pouch and closure of the laparotomy with Stratafix.
