Sacral Meningocele Research Articles

Surgical management of a rare giant sacral meningocele in a child

IntroductionAnterior sacral meningocele is a rare congenital disorder, occurring isolated or in syndromic disease. Case reportA 15-year-old patient who complained of abdominal pain and urinary dysfunction was managed surgically. Imaging diagnosed a giant presacral meningocele and agenesis of the coccyx. DiscussionThe presentation of sacral meningocele can be poorly symptomatic, which is why some patients are diagnosed late. Sometimes, diagnosis is suggested by non-specific abdominal symptoms or complications. Abdominal-pelvic radiological examination and lumbar spine MRI are essential, and treatment must be surgical. There are several surgical approaches, but currently no consensus. ConclusionAn unusual huge presacral cystic mass in a young patient may be isolated or part of a syndrome, and can be asymptomatic for a long time, leading to late diagnosis. The surgical approach should be based on multidisciplinary discussion. We operated on a giant anterior sacral meningocele in a child using a posterior approach, with a satisfactory result.

Surgical management of anterior sacral meningoceles: an illustrated case series and review of the literature

Background Anterior sacral meningocele (ASM) is an uncommon variant of spinal dysraphism. Surgical correction for this condition is challenging and optimal corrective approaches are uncertain. Objective To share our experience of managing this rare condition using the posterior trans-sacral approach and provide a contemporary review of the literature. Methods Retrospective review of case notes, operative records, and imaging of eligible patients treated via the posterior trans-sacral approach between 2006 and 2020 at our regional neurosciences centre. Results Three patients, two females and one male with a mean age of 30 years (range 16–38), were treated. Presenting symptoms included lower abdominal pain and recurrent miscarriages. Patients underwent corrective surgery using the posterior approach involving a sacral laminectomy, durotomy and closure of the communicating fistula. A single patient required reoperation due to early recurrence. Another patient proved challenging because of a very large sacral fistula and required two procedures due to the development of high-pressure headaches secondary to a recurrence. All patients improved symptomatically postoperatively and remained symptom free at the last clinic follow-up and have been discharged. Following review of the literature, only two other non-syndromic cases have been described. Conclusions ASM is an uncommon congenital abnormality, typically presenting with mass effect symptoms secondary to a presacral cystic mass. Surgical management using a posterior approach to close the meningeal sac is feasible and less invasive than an anterior approach. Long term clinical outcomes in our series were satisfactory.

Meningitis secondary to rectothecal fistula associated with congenital anterior meningocele

Meningitis secondary to rectothecal fistula is an exceptionally rare phenomena which has previously been reported exclusively in the setting of Currarino Syndrome for the pediatric population. We describe the case of a neonate presenting with polymicrobial meningitis due to rectothecal communication via congenital anterior sacral meningocele in the absence of an anorectal malformation. The authors opted for expeditious surgical resection of the fistulous tract and closure of the thecal sac using a posterior sagittal approach to the presacral space. The patient completed a course of intravenous antibiotics postoperatively. This case highlights the first reported neonatal case of rectothecal fistula in the absence of an anorectal anomaly. The surgical approach is described.

Six cases of occult sacral meningocele.

Background: Intrasacral occult sacral meningoceles (OSM) are uncommon congenital lesions that rarely become symptomatic, even over a patient’s life time.Methods: We operated on six patients with symptomatic OSM diagnosed on MR studies (all six) and/or CT examinations (four cases).Results: All six patients had uneventful postoperative recoveries. Nevertheless, despite the resolution of low back pain and radiculopathy, preoperative bladder dysfunction improved postoperatively in only one patient.Conclusion: Few cases of patients undergoing surgery for symptomatic OSM are reported in the literature. Here, we recount our experience with six patients with symptomatic OSM who demonstrated significant postoperative neurological recoveries except for rare improvement in bladder function.

Dermoid Cysts of the Conus Medullaris Associated with Sacral Meningocele. A Case Report

Spinal dermoid cysts are gradually progressive benign tumors that may be congenital or acquired. They account for 0.8% to 1.1% of all primary spinal tumors. MRI is the gold standard for the radiological assessment. Management is difficult for tumors involving highly eloquent areas such as the conus medullaris. In this report, we present a rare case of an adult woman with dermoid cysts in the conus medullaris associated with a sacral meningocele which was treated with a midline myelotomy that drained yellowish keratinous fluid and decompressed the cyst. No aggressive attempt at complete resection of the cyst wall was undertaken. The patient fully recovered, and her condition considerably improved after surgery and remains good at a 6-month follow-up.

Currarino triad.

Currarino triad is an autosomal dominant hereditary condition, characterized by triad of sacral agenesis abnormalities, anorectal malformation and presacral mass consisting of teratoma, anterior sacral meningocele or both. It is caused by mutations in the MNX1 gene. We present a case of a 2-year-old male child, brought by his mother with complaints of abdominal distension, failure to pass urine/faeces for 6 days. On examination, swelling of size 4x3cm was seen in the sacrococcygeal region. Magnetic resonance imaging (MRI) of abdomen and pelvis showed large cystic multi lobulated mass in sacrococcygeal region with dural communication evident of an anterior sacral meningocele. One month later, biopsy was done, which showed malignant mixed germ cell tumor in presacral area. Child underwent sacral partial laminectomy, laminoplasty and transdural ligation in neck of meningocele.

Presacral epidermoid: A rare case and meningocele mimick

Epidermoid cysts of the presacral space are a rare congenital entity. They have mostly been reported among women. We report a case of a 28-year-old male presenting with chronic constipation wherein magnetic resonance imaging (MRI) was suggestive of an anterior sacral meningocele associated with an epidermoid. This patient underwent a laparotomy and total excision of the mass. The lesion turned out to be an epidermoid cyst without any intradural communication or associated meningocele. This report highlights the limitations of imaging while it accentuates the need to perform diffusion-weighted MRI sequences for all cystic tumors in this region and to choose the correct operative approach based on its exact location in the presacral space.

Anal Neoplasms, Presacral Tumors, and Rare Malignancies

An understanding of anal anatomy is essential for optimal management. Patients with anal lesions that appear suspicious or fail to respond to conventional therapy within a month should undergo a biopsy. Premalignant perianal lesions are managed with local excision. Epidermoid carcinoma of the anus is usually managed with chemoradiation. Presacral tumors are managed with excision. This review contains 11 figures, 13 tables, and 57 references. Keywords: anal cancer, anterior sacral meningocele, basal cell carcinoma, chordoma, epidermoid carcinoma, high-grade squamous intraepithelial lesions, retrorectal tumor

Intracranial hypotension as a unique complication of the rupture of an anterior sacral meningocele into a recurrent pilonidal cyst

Background: Intracranial hypotension (IH) is an underdiagnosed, but important cause of new-onset, daily persistent headache, especially among the young- and middle-aged population. It results from a cerebrospinal fluid (CSF) leak with subsequent lowered CSF pressure.Case Description: A 37-year-old female presented to the emergency department with sudden onset severe headaches. Two years earlier, she had undergone surgery for resection of a pilonidal cyst (PC). The night before admission, she had watery discharge from the recurrent PC and severe diffuse positional headaches associated with photophobia and neck pain. The head computed tomography showed pneumocephalus in the posterior fossa and a spine magnetic resonance imaging revealed an anterior sacral meningocele (ASM) in close contact with the recurrent PC. A final diagnosis was made of headaches due to IH. The leakage site was the rupture of the ASM in the PC. The surgical repair of the ASM was achieved suturing two overlapping dural flaps. There was no more CSF leakage from the PC and the headaches disappeared.Conclusion: This is a unique case of IH due to the rupture of an ASM into a recurrent PC. The association of an ASM and PC, at the best of our knowledge, is unique. Moreover, the fistulation of the ASM to the PC is exceptional. ASM can be successfully closed with a posterior approach, using two overlapping dural flaps.

Severe onset of inflammatory myositis in a child: think to paraneoplastic myositis

BackgroundJuvenile idiopathic inflammatory myopathies (JIIMs) are a group of heterogenous, acquired, autoimmune disorders that affect the muscle. While the association between IIMs and malignancy has been widely reported in adults, cancer-associated myositis (CAM) is rare in children, so that routine malignancy screening is not generally performed. This report shows a case of severe CAM in a child.Case presentationAn 11-years-old girl presented with worsening dyspnea after a 3-weeks history of progressive proximal weakness, myalgia, dysphagia, and weight loss. Her past history was remarkable for a type I Arnold-Chiari malformation associated with an anterior sacral meningocele. Physical examination showed severe hypotony and hypotrophy. Pulse oximetry and blood test showed a type II respiratory failure (SpO2 88%, pCO2 68 mmHg) and increased muscle enzyme levels (CPK 8479 U/L, AST 715 U/L, ALT 383 U/L, LDH 1795 U/L). The patient needed invasive mechanical ventilation. Inflammatory myositis was considered and treatment with intravenous methylprednisolone (30 mg/Kg/day for 3 days followed by 2 mg/Kg/day) and IVIG (1 g/kg/day for 2 days) was started. Muscle biopsy showed endomysial and perimysial necrosis and inflammation. The presence of serum anti-TIF1-γ antibody positivity led to a malignancy screening. Whole-body MRI showed a mature teratoma underneath sacral meningocele and both lesions were surgically removed.Given the histological and clinical severity of the myopathy, mycophenolate (500 mg twice a day) and rituximab (360 mg/m2, 4 weekly infusions) were added. Due to extreme muscular wasting, severe malnutrition and intolerance to enteral feeding the patient needed a transient tracheostomy and parenteral nutrition, followed by physiotherapy, speech therapy and nocturnal non-invasive ventilation. A complete remission was achieved 3 months after.ConclusionsAmong cancer-associated autoantibodies (CAAs) in adult patients, anti-TIF1-γ carries the highest risk of CAM, which recognizes with a high likelihood a paraneoplastic pathogenesis. In children, anti-TIF1-γ antibody has been associated with severe cutaneous disease, lipodystrophy, and chronic disease course, but not with CAM, which is overall rare in younger patients. Severe onset of a JIIM, especially if anti-TIF1-γ antibody positive, should prompt suspect of a CAM and lead to a screening for malignancy.

Giant anterior sacral meningocele and incomplete Currarino syndrome in a female adult patient: a case report

El meningocele sacro anterior es una protrusión del saco dural a través de un defecto óseo de la pared anterior del hueso sacro. Se presenta aislado o asociado a otras malformaciones, como en el síndrome de Currarino, en el que es parte de la triada clásica junto al hemisacro y a una malformación anorrectal, aunque posee gran variabilidad. Presentamos el caso de una mujer adulta con estreñimiento crónico, infección urinaria recurrente y distensión abdominal marcada, que tiene el diagnóstico de meningocele sacro anterior gigante y síndrome de Currarino incompleto. La paciente es sometida a una laminectomía sacra y ligadura del saco tecal a nivel del defecto óseo. Este trabajo describe este inusual tipo de disrafismo espinal oculto, así como la técnica quirúrgica empleada.

Giant dorsal sacral meningocele in a child

Sacral meningoceles are uncommon congenital lesions, usually described in the anterior or lateral position, and they typically are asymptomatic [1,7]. A nine-years male child presented with a progressively increasing swelling over the lumbosacral region since birth.

Anterior Sacral Meningocele: A New Classification and Treatment Using the Dorsal Transsacral Approach.

Retrospective case series. Anterior sacral meningocele (ASM) is a rare disorder. We reviewed 11 cases of congenital ASM and classified them into three types based on the anatomy and relationship between the cyst and sacral nerve roots. The cohort with ASM is relatively large; the classification is novel and has not been previously reported. Eleven consecutive patients with ASM who underwent surgery between February 2014 and January 2019 were retrospectively analyzed. They included four males and seven females. The dorsal transsacral approach was adopted in all cases. The follow-up time was at least 3 months. We attempted to classify ASM into three types. Of the 11 cases, six were caudal type, two were paraneural type, and three were nerve-root type. The meningocele was ligated after exploring no nerve involvement, in Type I and II. For Type III, the herniating sac and involved nerve roots were ligated when the nerve roots were indicated as nonfunctional on neurophysiological monitoring; otherwise, the sacral nerve roots were protected and imbricated on the residual sac like a hand-in-glove, and sutured to reconstruct the nerves sleeve. Eight cases were accompanied by tethered cord syndrome (TCS); spinal cord detethering was done with one-stage operation. Ten patients' presenting symptoms improved at 3 to 6 months' follow-up; notably, constipation significantly improved. Only one case accompanied by an epidermoid cyst had a second laparoscopic surgery by a general surgeon. Aim of surgical treatment is to obliterate the communication between the subarachnoid space and herniated sac, detether the spinal cord, and resect the congenital tumor. The new classification helps to recognize the relationship between the meningocele and sacral nerve roots, and subsequently adopt different surgical strategies. We consider the dorsal transsacral approach relatively feasible, safe, and with lower complication. 4.

Retrorectal Cystic Hamartoma (Tail Gut Cyst): An Institutional Experience

Abstract Objectives Tailgut cyst (TGC) is a rare congenital benign cystic lesion arising from a remnant of the embryonic postnatal hindgut. TGCs are typically multinodular, uncapsulated, but usually well circumscribed. TGCs are generally rare in adults and the majority of the lesions are benign. Malignant transformation is rare and malignancies reported include adenocarcinomas and neuroendocrine tumors. Methods A retrospective database search was conducted and coded as “tailgut cyst or retrorectal cystic hamartoma” to identify cases of possible TGC between January 2005 and January 2019. The search utilized the pathology files of Yale-New Haven Hospital using CoPath laboratory information system. Clinical and surgical data were gathered from the surgical pathology report and surgical operative note when available. Results A total of 13 cases (10 women) were identified with a median age of 51 years. All the specimens were obtained by surgical excision. Most of the cases were excised from the presacreal area (11 cases), and two cases were excised from the retrorectal area. The two cases resected from the retrorectal area had a malignant component. The first case was a 33-year-old female who had a 1.5-cm tailgut cyst that showed a multifocal neuroendocrine tumor (carcinoid); the resection margin was also positive. The second case was a 59-year-old female who had a tailgut cyst that showed mucinous adenocarcinoma; the resection margin was also positive. Conclusion Diagnosis of tailgut is sometimes challenging. Possible differential diagnoses in this location include teratoma, dermoid cyst, anterior sacral meningocele, and presacral abscess. Imaging is an important component of the initial workup. Surgical resection is the treatment of choice when the cyst grows in size and if a solid component is present. Exclusion of other benign entities and malignant transformation/component on histological examination is crucial.

Dural ectasia in Marfan syndrome and other hereditary connective tissue disorders: a 10-year follow-up study

BACKGROUND CONTEXTDural ectasia is widening of the dural sac often seen in patients with Marfan syndrome and other hereditary connective tissue disorders. Dural ectasia can cause specific symptoms and is associated with surgical complications. The knowledge on how and at which age dural ectasia develops is incomplete. There is no established gold standard for diagnosing dural ectasia, making it difficult to compare results from different studies. PURPOSEOur primary aim was to explore whether the radiological findings of dural ectasia changed after 10 years in an adult cohort with suspected Marfan syndrome. Our secondary aim was to re-evaluate the radiological criteria of dural ectasia. STUDY DESIGNProspective cohort study. PATIENT SAMPLESixty-two persons from a cross-sectional study of 105 persons with suspected Marfan syndrome were included in a 10-year follow-up of dural ectasia. Forty-six were diagnosed with Marfan syndrome, 7 with Loeys-Dietz syndrome, and 5 with other hereditary connective tissue disorders. For comparison 64 matched hospital controls were evaluated. OUTCOME MEASURESPreviously used radiological criteria for dural ectasia based on quantitative measurements of the lumbosacral spine. METHODSMRI of the lumbosacral spine was performed if not contraindicated, and if so then CT was performed. Differences in the study group between baseline and follow-up were assessed with paired Student t test, Wilcoxon rank signed test, and McNemar test. Receiver operating characteristic curves were constructed to assess the ability of radiological measurement to differentiate between the study and control group. RESULTSFifty-two of 58 patients with hereditary connective tissue disorders and 11 controls had dural ectasia at follow-up. Forty-five Marfan patients had dural ectasia at follow-up vs. 41 at baseline. Five Loeys-Dietz patients had dural ectasia at follow-up vs. four at baseline. 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. CONCLUSIONSIn 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.

Magnetic resonance imaging of the lumbar spine in adult: Evaluation of spinal incidental findings in patients with low back pain

Aim: We aimed to incidental findings discovered in patients with suspected low back pain disease undergoing MRI of the lumbar spine and the clinical importance of those findings. Material and Methods: One thousand seven hundred fourteen patients included who were admitted to our hospital with a preliminary diagnosis of low back pain were examined. The duration of low back pain complaints of the patients was ranging from 1 week to 4 years. MRI examinations were interpreted by academic neuroradiologists who had 7 years of experience, at the time of the study. Results: Overall, 358 patients (20.9%) had incidental findings. Transitional vertebrae was the most common (134 cases, 7.8%) incidental finding, followed by vertebral hemangioma (65 cases, 3.8%) and Tarlov cyst (54 cases, 3.2%). The incidence of fatty filum terminale, compact bone island, spina bifida, synovial cyst and sacral meningocele was 2.1%, 1.5%, 1%, 0.8% and 0.7% respectively. Of these 1714 patients, 329 patients (92.06%) had one finding, 22 patients (5.95%) had two findings, and 7 patient (1.98%) had three findings. The conus medullaris was terminated in 1710 (99.7%) of the patients (L1-2) .This level were considered normal. In four cases, the spinal cord was determined to be below the required level. The prevalence of transitional vertebra was 7.8%. The prevalence of sacralization and lumbalization was 5.1% and 2.7%, respectively. Conclusion: In this study we showed that incidental findings are mostly benign but interestingly there were no malignant or potentially malignant incidental lesions.

Multiple neurosurgical treatments for different members of the same family with Currarino syndrome

IntroductionCurrarino's syndrome (CS) is an autosomal dominant disorder of embryonic development causing a rare malformating syndrome characterized by a triad of an anorectal malformations, presacral mass (most commonly an anterior sacral meningocele) and sacral bony defects. Mutations of the HLXB9 gene have been identified in most CS cases, but a precise genotype-phenotype correlation has not been described so far. Family screening is obligatory. The diagnosis is usually made during childhood and rarely in adulthood. In this context, imaging, and especially MRI plays a major role in the diagnosis of this syndrome. Surgical management is provided by pediatric surgeons or neurosurgeons. Familial case reportHere, we present a family case report with CS requiring different neurosurgical management. The son, a 3-year-old boy, developed a tethered spinal cord syndrome associated to a lipoma of the filum terminale, a sacro-coccygeal teratoma and an anal adhesion. A combined surgical approach permitted a good evolution on the urinary and digestive functions despite a persistent fecal incontinence. The 2-year-old daughter presented with a cyst of the thyreoglossal tract infected and fistulized to the skin. She was also followed for a very small lipoma of the filum terminale that required a neurosurgical approach. The father, 44-year-old, manifested functional digestive and urinary disorders caused by a giant anterior sacral meningocele. The ligation of the neck of the cyst and aspiration of the liquid inside in full through a posterior partial approach permit a complete collapse of the cyst with an instantly satisfactory clinical outcome. ConclusionIn these cases, cooperation between pediatric surgeons and neurosurgeons was crucial. The follow-up of these patients should be done in a spina bifida clinic. A geneticist evaluation must be offered to the patient in the case of a CS as well as a clinical evaluation of the relatives (parents, siblings).

Anterior sacral meningocele mimicking an adnexal mass in an infertile woman: a report of an extremely unusual case

Anterior sacral meningocele mimicking an adnexal mass in an infertile woman: a report of an extremely unusual case

Intraoperative diagnosis of an anterior sacral meningocele mimicking a giant ovarian cyst in an adult

Introduction. Anterior sacral meningocele (ASM) is a very rare condition that is the herniation of the meningeal sac into the pelvic cavity through a developmental bone defect of the anterior wall of the sacrum. Most of the ASM are diagnosed in childhood but the reported cases that are diagnosed in adults exhibit a gamut of complications. Case report. We presented a case of ASM excision that was misdiagnosed as a giant ovarian cyst. A 28-year-old woman was admitted to the General Surgery Clinic and than to the Gynecology Department with suspect of ovarian cyst depending on ultrasonography (US) scans solely. Adnexial torsion was suspected and surgery to remove the cyst and adnexial exploration was planned. When the lesion was found out to be ASM, neurosurgery team tied the neck and excised the whole meningocele. Histopathologic evaluations confirmed dural sac. Neurological examinations right after the operation revealed 20% weakness in knee extension. It totally recovered in 6 months time. Her MR imaging studies and computed tomography (CT) scans revealed multiple ASM sacs and defects of the anterior wall of the sacrum. Conclusion. This case emphasizes the importance of utilizing available screening tools including CT andmagnetic resonance imaging (MRI) studies as the gold standard in addition to US scans in the preoperative period in order to accurately evaluate and characterize any pelvic lesion.

Anterior sacral meningocele infected with Fusobacterium in a patient with recently diagnosed colorectal carcinoma \u2013 a case report

BackgroundAnterior sacral meningoceles are rare, and usually occur with other malformations of the posterior lower spine. While these are more frequently reported in pediatric cohorts, we report a case in an elderly woman.Case presentationWe report on a 71 year-old woman with a recently diagnosed colorectal adenocarcinoma who presented with a severe bacterial meningitis. The cerebrospinal fluid cell count revealed a pleocytosis of 80,000 cells/μl and a severe disturbance of the blood-brain-barrier. Fusobacterium nucleatum was cultured as the causing pathogen. A lumbar MRI showed, in addition to contrast-enhancing meninges as sign of inflammation, a presacral mass. In the next step, the mass was diagnosed as an anterior sacral meningocele connected to the gut. An adequate antibiotic was used to treat the leptomeningitis. The connection between gut and meningocele was closed surgically and the patient recovered well and underwent further treatment of her colorectal adenocarcinoma.ConclusionWe report on a case of meningitis with an anterior sacral meningocele that was connected to the gut in a patient with a infiltrative colorectal adenocarcinoma. Anatomic variants have to be considered as rare causes of meningitis with typical intestinal germs.