Ultrasound Obstet Gynecol 2007; 30: 893896 Published online 5 September 2007 in Wiley InterScience (www.interscience.wiley.com). DOI: 10.1002/uog.

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Anterior sacral meningocele: management in gynecological practice

F. MANSON*, K. COMALLI-DILLON and A. MORIAUX
*21 Place Charles de Gaulle and 30 rue Jules Ferry, 76400 F camp, France and 1605 Novato Bl., Novato, CA 949473233, USA e

K E Y W O R D S: acquired ASM; anterior sacral meningocele; congenital ASM; gynecological management; obstetric management; sonography

ABSTRACT
We describe the case of a young woman with anterior sacral meningocele (ASM), initially identied during a routine ultrasound examination and subsequently diagnosed using magnetic resonance imaging (MRI). ASM is a rare disorder characterized by uni- or multilocular extensions of the meninges from the sacral spinal canal to the retroperitoneal presacral space. Common symptoms include lower back and pelvic pain, constipation, difculties in defecation, dysmenorrhea and dyspareunia, and urinary incontinence, retention or urgency. Perineal and lower-extremity paresthesias may present when nerve roots are affected. Despite its more posterior location, ASM can mimic an ovarian cyst or other adnexal cystic mass, and in the obstetric patient can present a mechanical obstacle to delivery with a risk of rupture and infection during labor and delivery. Although it is a rare condition, we feel that awareness of the etiology, presentation and imaging characteristics of ASM is of importance and have therefore carried out a review of the literature, taking into account case ndings and the obstetric and gynecological management of this disorder. Copyright 2007 ISUOG. Published by John Wiley & Sons, Ltd.

The uterus and both ovaries were well seen on ultrasound examination, appearing within normal limits (Figure 1). However, bilateral posterior cystic, multilobed, multilocular, thin-walled masses were noted. The anterior and posterior aspects of the masses were well dened (Figure 2). On magnetic resonance imaging (MRI), bilateral posterior, multilocular, cystic structures were seen to be directly in contact with the sacrum and posterior iliac bones; their appearance did not change after injection of gadolinium. No solid or calcied elements appeared within the masses. The structures communicated at multiple levels with the sacral dural sac, projecting into the retroperitoneal space through the anterior sacral foramina (Figures 3 and 4). A diagnosis of bilateral anterior sacral meningocele (ASM) was made. Medullary MRI was performed and was unremarkable. A neurosurgical consultation was requested; because the patient was asymptomatic, surgery was not advised.

DISCUSSION
ASM is a uni- or multilocular extension of the meninges from the sacral spinal canal to the retroperitoneal presacral space, through sacral bony defects or through the anterior sacral foramina1 . Its incidence is rare; since the rst description published in The Lancet (Bryant) in 1837, fewer than 300 cases have been reported1 3 . ASM is characterized by a communication with the subarachnoid sacral space4,5 . ASM contains only cerebrospinal uid and its wall consists of two layers, the inner arachnoid and the outer dura mater1,2 . Some authors report the variable coexistence of benign tumors such as teratomata4 . ASM is usually single and anterolateral bilateral ASM appears to be rare1 . ASM is described as a single entity in most articles. However, on reviewing the literature, we conclude that

CASE REPORT
We describe the case of a 34-year-old patient with no signicant medical history and two uneventful pregnancies with vaginal deliveries. She complained of no symptoms except recent intermittent vaginal bleeding, corrected with a prescription of different birth control pills. Bilateral pelvic masses were palpated upon vaginal examination which was otherwise unremarkable. The masses were posterior, xed, regular in contour and sensitive to light pressure.

Correspondence to: Dr F. Manson, 21 Place Charles de Gaulle, 76400 F camp, France (e-mail: frmanson@wanadoo.fr) e Accepted: 12 December 2006

Copyright 2007 ISUOG. Published by John Wiley & Sons, Ltd.

CASE REPORT & REVIEW

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Manson et al.

Figure 1 Ultrasound images of both ovaries, showing them to be normal.

Figure 2 Ultrasound images of bilateral posterior cystic, multilobed, multilocular masses.

Figure 3 T2-weighted axial magnetic resonance image showing bilateral posterior, multilocular, cystic structures ( ) seen to be directly in contact with the sacrum and posterior iliac bones.

two types of ASM exist congenital and acquired. Congenital ASM appears to be a true anterior dysraphism6 9

featuring a spectrum of bony anomalies, from minor bony defects to complete lower sacral agenesis. The defect is usually lateral with a pathognomonic sacral deformity known as scimitar sacrum seen on plain lm1,4 . Congenital ASM may be isolated, but is often associated with focal malformations, primarily cloacal, but which may be urinary or gynecological1,3 . Congenital ASM is usually sporadic in presentation, but predominantly autosomaldominant with variable penetrance2,10,11 ; X-linked cases have been reported9 . The embryonic etiology of congenital ASM seems to originate in a failure of the dorsoventral separation of the caudal eminence during late gastrulation2,8,12 . The neural tube begins at the origin of the spine, extending to S2 (somite 31); the caudal eminence originates from S1 and S2 in secondary neurulation. Since S1 and S2 are commonly spared in congenital ASM, the embryonic mechanism theory seems likely. Currarino syndrome consists of a triad of anomalies: (1) a sacral bony defect; (2) anorectal malformations; and (3) a presacral mass, which is an ASM in about two-thirds of cases, but which may also be a teratoma or enteric cysts.

Copyright 2007 ISUOG. Published by John Wiley & Sons, Ltd.

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Figure 4 T2-weighted parasagittal magnetic resonance images showing cystic structures communicating at multiple levels with the sacral dural sac and projecting into the retroperitoneal space through the enlarged anterior sacral foramina.

It has autosomal-dominant inheritance1 4,13,14 , and may include congenital ASM. Congenital ASM can be diagnosed in early childhood14,15 or even prenatally, as reported by Kivilevitch2 . Tethered cord is the most frequent earlychildhood neurological malformation seen in ASM4 . Acquired ASM features abnormal structure of the dura mater, and is related to connective tissue disorders2 . It may be seen in Marfans and Marfanoid syndromes, in neurobromatosis, in EhlersDanlos syndrome, in ankylosing spondylitis and in osteogenesis imperfecta1,7 9 . Acquired ASM may also be present without these pathologies16 . The etiology of the meningocele seems to be progressive distension of the dura mater and the arachnoid by hydrostatic pressure from cerebrospinal uid. The apparent absence of ASM in young people is explained by its progressive course; over time, the meninges herniate through the anterior sacral foramina17 . Localized symptoms of both types of ASM present in two-thirds of all cases1,2,12,13 . These symptoms are produced by pressure from the ASM on the sacral nerve roots, rectum, bladder, or genitalia. The most common symptoms are lower back and pelvic pain, constipation, difculties in defecation, dysmenorrhea and dyspareunia, and urinary incontinence, retention or urgency. Perineal and lower-extremity paresthesias may present when nerve roots are affected. The clinician observing these symptoms must include ASM in the differential diagnosis1 3,11 . Two systemic features may also indicate ASM: headaches and meningitis. Intermittent

headaches are produced by alternating intracranial hyper- and hypotension. Intracranial hypertension is due to the ASM emptying into the subarachnoid space during increased intra-abdominal pressure (such as during defecation). Intracranial hypotension occurs secondary to ASM lling. The ASM must have a large communication in order for these symptoms to present2,9,11,13 . Meningitis associated with ASM can be recurrent6 and is usually polymicrobial18 , and it can be spontaneous or iatrogenic9,12,15 . Clinical examination may be unrevealing. ASM can be felt during rectal or vaginal digital examination where the mass yields to gentle palpation. It is uni- or, rarely, bilateral, and posterior1,12 . Physical examination allows a search for associated malformations and neurological problems such as anal sphincter hypotonia and perineal hypoesthesia11 . Several radiological modalities are essential for the investigation and diagnosis of ASM. Because of pelvic symptomatology, ultrasonography is usually the rst examination that is performed. Although ultrasound imaging clearly demonstrates cystic masses, it can fail to diagnose ASM owing to an inability to conclusively demonstrate the communication between the lesion and the dural sacral canal2 . However, elements such as the very posterior location of the mass and visualization of both normal ovaries might arouse suspicion of a nongynecological pathology. Ultrasound examination has been suggested by some authors for post-surgical follow up4 . MRI is the gold standard for the diagnosis of ASM2,4,7,12,19 . It yields a positive diagnosis when the communication between the presacral cystic lesion and

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Manson et al. MD, PhD, and to Jim Baun, BS, RDMS, RVT, FSDMS, for their support.

the sacral subarachnoid space is shown18 . MRI allows for the most accurate preoperative imaging to determine any surgical course20 . Myelography is a historic exam for the diagnosis of ASM. It is performed under uoroscopy, after which a computed tomography (CT) scan is carried out and a correlation made. Myelography is particularly useful should MRI fail to demonstrate the communication between the lesion and the subdural space (owing to narrowing of the communication of the ASM). Myelography permits distinction between ASM and other presacral cystic masses with no communication between the lesion and the dural sac2,12 such as type IV cystic teratomata, neuroectodermic cysts, and Tarlov cysts2,10,12 . Anterior sacral meningocele does not regress2 . Therefore, symptomatic ASM requires surgical intervention for the patients comfort and safety. Opinions on management diverge according to different authors in cases of asymptomatic ASM. Some authors recommend surgery in all cases to avoid infectious complications1,6,7 , and different surgical techniques have been described to this end3,18 . Others recommend conservative management, suggesting surgical intervention only in the case of symptoms or increase in size of the lesion due to heightened hydrostatic pressure1,6 8,13 . From a gynecological perspective, on palpation ASM can mimic an ovarian cyst or other adnexal cystic mass, but the ASM will be located more posteriorly10 . The gynecologist must treat discovery of these masses with great suspicion to reach the correct diagnosis before surgical exploration3,5,7,14 . Diagnosis may be made at the time of surgery because of the retroperitoneal location of the ASM3,5,7,14 . From the obstetric viewpoint, ASM can present a mechanical obstacle to delivery5,11,12,16 . The most critical risk is rupture of the ASM during labor and delivery, which could lead to infection with a high likelihood of materno-fetal mortality2,3,5 . A prophylactic Cesarean section should be scheduled, since few cases of uncomplicated vaginal delivery have been reported in cases of ASM2,3,5,11 . Four other obstetric elements not mentioned in the literature should be considered: 1) MRI should be performed for pregnant women with known Marfans syndrome, neurobromatosis, or familial history of ASM in order to plan the method of delivery; 2) treatment with folic acid 5 mg q.d. before conception is suggested in congenital ASM; 3) genetic counseling for parents must be provided, with particular attention to the risk of complete or partial Currarino syndrome or associated malformations; and 4) targeted fetal sonographic examination should be used for screening major anomalies, but in cases with a family history of ASM, MRI should be used to search for ASM, and a multidetector CT scan is helpful for studying the anatomy of the fetal bones, in particular the spine.

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ACKNOWLEDGMENT
The authors extend their appreciation to Philippe Jeanty,

Copyright 2007 ISUOG. Published by John Wiley & Sons, Ltd.

Ultrasound Obstet Gynecol 2007; 30: 893896.