An anteroposterior pelvis radiogram demonstrated a sacral deformity and the scimitar sign. FOIA Together, these features have been defined as the Currarino syndrome. Linking to a non-federal website does not constitute an endorsement by CDC or any of its employees of the sponsors or the information and products presented on the website. Links with this icon indicate that you are leaving the CDC website.. Anterior-sacral meningocele; value of magnetic resonance imaging and abdominal sonography. Myelography, computed tomography, and magnetic resonance imaging are the best methods for identifying the precise anatomy of sacral meningocele and for proper planning of the operation. The combination of plain-film appearances and sonographic findings should be sufficient for diagnostic purposes. 1988; 31:806–807. Anterior Sacral Meningocele and the Scimitar Sign Report of a Case Paul J. Kovalcik, James B. Burke Ogilvie’S Syndrome Developing After Ethanol Ablation of Renal Cell Carcinoma Anterior sacral meningocele is a rare congenital disorder that usually occurs sporadically, but familial cases have been reported as part of the Currarino syndrome (, 15,, 28). In the cases of meningocele without ectopic spinal cord, whenever the defect in the bony spines was small there was no opportunity to inspect the inner side of the spinal canal and the meningocele was excised at its neck flush with the posterior spines. IntroductionAnterior sacral meningocele (ASM) is characterized by the herniation of the thecal sac into the presacral retro-peritoneal space [1]- [3]. Childs Nerv Syst. [ Links ] 10 Kovalcik PJ, Burke JB. Meningocele typically causes mild problems, with a sac of fluid present at the gap in the spine. Rectal examination and radiography of the pelvis with the sacral bone showing the "scimitar sign" are the main diagnostic methods. It is an occult form of spinal dysraphism resulting from a neural … 'Itsclinicaldiagnosisispar-ticularly difficult. Associations. Meningomyelocele is a type of spina bifida. Associated features include anorectal malformation, a presacral mass, and urogenital malformation. Prevention and treatment information (HHS). ... often referred to as the “scimitar sign”. Anterior sacral meningocele and the scimitar sign. A meningocele forms when the bones of the spine (vertebrae) do not close completely, and the meninges protrude in a sac through the hole that is formed. 11. 1980 Oct;53(4):548-52. doi: 10.3171/jns.1980.53.4.0548. Accessibility The sac often is visible from the outside of the back. Whenever a presacral mass is found, anterior sacral meningocele has to be a diagnostic consideration. Based on these developmental aberrations, CS has got a wide spectrum of clinical presentation. 2002 Dec;13(6):455-62; discussion 462. The sac includes spinal fluid, but does not contain neural tissue. Giant anterior sacral meningocele presenting as bacterial meningitis in a previously healthy adult. A year later, in a regional hospital a "cystic presacral tumor" was treated with biopsy and drainage. Other associations are neonatal bowel obstruction, chronic constipation, and tethered cord syndrome. Pathology. Another sign of spina bifida occulta is the presence of tufts of hair at the sacrum. Int J Colorectal Dis. Four years later, she developed constipation caused by perineal compression for which she was admitted to our department in which two anterior presacral cysts were excised. Bethesda, MD 20894, Copyright Dios Seoane J, Amaro S, Fantini MG, Blanco A, López L, Rodiño A, Rossi E, Vázquez A, Díaz J, Goyanes V, Dios García V. Neurocirugia (Astur). Rectal examination and radiography of the pelvis with the sacral bone showing the "scimitar sign" are the main diagnostic methods. … The recovery was complicated with meningitis, which was successfully treated with antibiotics. 2016 Feb;31(2):351-7. doi: 10.1007/s00384-015-2417-1. [Anterior sacral meningocele with Currarino's syndrome: report of two cases]. The presacral mass could be anterior sacral meningocele, teratoma, or enterogenous cyst. On plain films, 2 cases showed the classic scimitar sign, and 1 case showed a defect in the anterior sacrum. Careers. The case presented is a 37-year-old female in whom an asymptomatic presacral mass was discovered during her first delivery. Intrasacral meningocele. The meninges are three layers of protective tissues that cover the spinal cord. Spine (Phila Pa 1976). High index of suspicion with careful clinical examination is necessary to make early diagnosis. It makes up only 5% of all retrorectal masses [4]- [6]. Developmental cysts constitute approximately two-thirds of congenital presacral masses. Acta Neurochir (Wien). Case report and review of the literature. It is possible that while there is no opening, vertebrae are missing and there is damage to nerve tissue. On magnetic resonance imaging (MRI), the meningocele sac contains only fluid without any visible tissue. The aberrant vein is located posteriorly in lateral chest radiographs. It typically occurs along the spinal column and can range in size. An intrathoracic meningocele is a relatively rare disease, and it commonly accompanies neurofibromatosis type 1. Affected children generally have no neurologic deficits. 2011 Aug;29(7):528-31. doi: 10.1007/s11604-011-0582-x. Dis Colon Rectum. Sonography demonstrated the anterior sacral cysts in all cases. Spinal meningocele is defined as a protrusion of the spinal meninges through a defect in th e vertebral column or foramina usually is association with a congenitally dysraphic vertebrae, with the spinal cord remaining entirely confined to the vertebral canal The symptoms are usually related to the compression on rectum, bladder, and sacral nervous plexus. Whenever a presacral mass is found, anterior sacral meningocele has to be a diagnostic consideration. 1990 Dec;27(6):981-6. doi: 10.1097/00006123-199012000-00020. Am J Dis Child. Koksal A, Canyigit M, Kara T, Ulus A, Gokbayir H, Sarisahin M. Jpn J Radiol. 2 The majority of these lesions are benign, including developmental cysts (tailgut, rectal duplication, dermoid and epidermoid cysts) or anterior sacral meningoceles. National Library of Medicine Careers. A meningocele is a sac protruding from the spinal column, which contains some of the spinal fluid and meninges. Int J Obstet Anesth. Transvaginal or transrectal aspiration and drainage are not advised, because they may result in a lethal outcome caused by sepsis. J Neurosurg. Chronic fistulae can be evaluated with a fistulogram, which documents anato my and rules out communication with the rectum . Unable to load your collection due to an error, Unable to load your delegates due to an error. Sonography is a noninvasive method of monitoring progress or contraction of a lesion, and it can be used to demonstrate any associated pathology. 2013;22:64-7. 2005 Jun;17(6):423-5. doi: 10.1080/14767050500123616. The other lesion appeared radiologically to be a purely intrasacral meningocele. Naidich TP, Fernbach SK, McLone DG, et al. [ Links ] 9 Mohta A, Das S, Jindal R. Anterior sacral meningocele presenting as constipation. Patients tend to have no symptom but if its size is too large and compresses a lung and neighboring organs, it needs shunt drainage or surgical resection. Anterior sacral meningocele. Epub 2015 Nov 7. Congenital cystic lesions are commonly encountered presacral masses with a female predilection. 1980 Apr;134(4):385-6. doi: 10.1001/archpedi.1980.04490010039013. Unusual presentation of an anterior sacral meningocele: magnetic resonance imaging, multidetector computed tomography, and fistulography findings of bacterial meningitis secondary to a rectothecal fistula. Meningocele. Clipboard, Search History, and several other advanced features are temporarily unavailable. The symptoms are usually related to the compression on rectum, bladder, and sacral nervous plexus. 8 McGregor C, Katz S, Harpham M. Management of a parturient with an anterior sacral meningocele. Anterior sacral meningocele. Maternal anterior sacral meningocele in pregnancy. It may be covered with skin or with meninges (the membranes that cover the central nervous system). Unable to load your collection due to an error, Unable to load your delegates due to an error. 1989 Dec;14(12):1418-20. Occulta has no or only mild signs, which may include a hairy patch, dimple, dark spot or swelling on the back at the site of the gap in the spine. Usually asymptomatic, it can present as constipation, urinary problems or rarely neurological symptoms. Please enable it to take advantage of the complete set of features! Scimitar Deformity A deformity of the sacrum which has been fancifully likened to a scimitar, a finding highly characteristic of Currarino syndrome or triad, which is accompanied by a presacral mass, anterior sacral meningocele or teratoma, and anorectal malformation Autosomal dominant sacral agenesis is characterised by a partial agenesis of the sacrum typically involving sacral vertebrae S2-S5 only. Myelography outlined part of the ASM in two cases. Clipboard, Search History, and several other advanced features are temporarily unavailable. Would you like email updates of new search results? Epub 2012 Sep 1. On plain films, 2 cases showed the classic scimitar sign, and 1 case showed a defect in the anterior sacrum. John Caffey Award. Diagnostic steps and staged operative approach in Currarino's triad: a case report and review of the literature. ( si'mi-tahr sīn) A curvilinear structure seen in the lung roentgenographically, associated with anomalous pulmonary venous drainage, suggesting the sickle shape of a Turkish saber; also used to refer to the scalloped shape of the sacrum in spinal dysraphism with anterior meningocele. Anterior sacral meningocele and the scimitar sign. Cases J. Occult, bilateral anterior sacral and intrasacral meningeal and perineurial cysts: case report and review of the literature. Anterior sacral meningocele (ASM) is a rare form of spinal dysraphism in children. Would you like email updates of new search results? The diagnosis was confirmed by computed tomography with myelographic enhancement. The vein may not be visible if it is small or obscured by the heart shadow. Bal S, Kurtulmuş S, Koçyiğit H, Gürgan A. Spine (Phila Pa 1976). Upon these imaging findings, the patient got the diagnosis of an anterior sacral meningocele. Privacy, Help Bethesda, MD 20894, Copyright This site needs JavaScript to work properly. Neurosurgery. Anterior sacral meningocele is a rare anomaly most frequently presenting as a presacral mass. The prevalence of ASM is 1 in 1000 live births [7]. If signs and symptoms also suggest a meningocele, special studies such as ... Anterior sacral meningocele is a rare entity,withonlyabout100cases reported. Since the first description in 1837, approximately 150 cases have been reported. Ultrasonography revealed a large cystic structure. Scimitar sacrum is pathognomonic for anterior sacral The radiographic appearance of the pulmonary vein descending along the right cardiac border is characteristic (scimitar sign) (, 1,, 45) (,,,,, Fig 11b). Contrast enhancing CT or MRI of the pelvis is widely used and can differentiate solid from cystic lesions, vascular, adjacent organ involvement and bony destruction. Sonography of the caudal spine and back: congenital anomalies in children. Four cases of anterior sacral meningocele (ASM) are described, with comparisons between radiological and sonographic findings. Four cases of anterior sacral meningocele (ASM) are described, with comparisons between radiological and sonographic findings. 2011;6:40-3. Case report. Kontopoulos EV, Oyelese Y, Nath C, Schwebel M, Smulian JC, Vintzileos AM. Currarino's syndrome misinterpreted as Hirschsprung's disease for 17 years: a case report. the characteristic scimitar sacrum is observed on a pelvic roentgenogram. Identification of Report of a case. Scimitar sacrum, also known as sickle-shaped sacrum or hemisacral agenesis, is a unilateral, well-marginated, crescent-shaped defect in the lateral sacrum. 8600 Rockville Pike Radiography of the pelvis demonstrated a sacral deformity or “scimitar sign” that is pathognomonic for anterior sacral meningocele. The pathognomonic scimitar sign caused by a unilateral sacral defect indicates presence of anterior sacral meningocele . Plain X-ray may demonstrate sacral destruction (chordoma), calcification in the mass (teratoma) or pathognomonic signs such as ‘scimitar sign’ (anterior sacral meningocele). doi: 10.1097/01.brs.0000131212.36990.0a. Anterior sacral meningocele is defined as a meningeal cyst that occurs in the presacral space secondary to agenesis of a portion of the anterior … Spina bifida is a birth defect in which the spinal canal and the backbone don't close before birth. Sumner TE, Crowe JE, Phelps CR 2nd, Park RW. Lateral meningocele syndrome is caused by mutations in the NOTCH3 gene. Sacral malformations: use of imaging to optimise sacral nerve stimulation. J Pediatr Neurosci. The expansion of a meningocele causes scalloping and lateral displacement of the sacral plate. Accessibility cra A triangular bone made up of five fused vertebrae and forming the posterior section of the pelvis. Povo A, Arantes M, Matzel KE, Barbosa J, Ferreira MA. A case report. Dural primordial anomalies give rise to anterior sacral meningocele. 1991;109(3-4):154-7. doi: 10.1007/BF01403013. Miletic D, Poljak I, Eskinja N, Valkovic P, Sestan B, Troselj-Vukic B. Orthopedics. tar sign. A case with cauda equina syndrome due to bacterial meningitis of anterior sacral meningocele. The other lesion appeared … It is a rare entity and constitutes a very small portion of the cystic masses of the pelvis. A meningocele is a birth defect where there is a sac protruding from the spinal column. This is a preview of subscription content, log in to check access. Abdominal and spinal sonography should be the first diagnostic investigation and can diagnose anterior sacral meningocele and differentiate it from other cytic lesions in the Hogan AM, Mannion M, Ryan RS, Khan W, Waldron R, Barry K. Int J Surg Case Rep. 2013;4(3):299-301. doi: 10.1016/j.ijscr.2012.08.005. 8600 Rockville Pike 2009 Feb 3;2(1):118. doi: 10.1186/1757-1626-2-118. FOIA National Library of Medicine This site needs JavaScript to work properly. A meningocele (Fig 4) is an isolated full skin thickness sac, filled with CSF and lacking central nervous issue. Myelomeningocele, also known as … Epub 2011 Sep 1. This gene provides instructions for making a protein with one end (the intracellular end) that remains inside the cell, a middle (transmembrane) section that spans the cell membrane, and another end (the extracellular end) that projects from the outer surface of the cell. Privacy, Help Alerts and Notices Synopsis A meningocele is a protrusion of the meninges without any accompanying spinal cord or nervous tissue. 1986;28(5-6):512-27. doi: 10.1007/BF00344104. Please enable it to take advantage of the complete set of features! It may be uncommonly associated with epidermoid cysts (, 2). 2008 Feb;31(2):182. doi: 10.3928/01477447-20080201-21. Scimitar is a two year old Arabian colt who suffered major injuries when he mistakenly galloped into a tree in his pasture, driving a three inch stick into his head. Magnetic resonance imaging is the investigation of choice. Because normal delivery was impossible, a cesarean section was performed. 14 On MRI, the signal intensity of the content within the meningocele should parallel the CSF signal on all sequences . The Centers for Disease Control and Prevention (CDC) cannot attest to the accuracy of a non-federal website. If an operative procedure is contemplated, then myelography may be performed in an attempt to outline the stalk. Neuroradiology. J Matern Fetal Neonatal Med. Real-time sonographic display of caudal spinal anomalies. The frequency of meningoceles is one-tenth that of MMCs. 2004 Jul 15;29(14):E298-9. 2000 Aug;16(8):522-4. doi: 10.1007/PL00007296. The possibility of an anterior sacral meningocele must always be kept in mind … This sac is usually well covered with skin, and typically causes no problems. ‘Scimitar’ sign a smooth curved unilateral sacral defect simulating shape of Arabic sabre on plain X-ray is considered to be pathognomic of anterior sacral meningocele. Prevention and treatment information (HHS). Anterior Sacral Meningocele . Later, in a previously healthy adult scimitar sign meningocele is 1 in 1000 live [... Anterior sacral meningocele with Currarino 's syndrome: report of two cases ] 1000 live [! 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Smulian JC, Vintzileos AM bone made up of five fused vertebrae and forming the section. The Centers for disease Control and Prevention ( CDC ) can not attest to the compression on rectum bladder! In which the spinal cord ( 3-4 ):154-7. doi: 10.3928/01477447-20080201-21 M. Management a., these features have been defined as the Currarino syndrome sacral nerve stimulation and symptoms also suggest a (! Syndrome: report of two cases, Kara T, Ulus a, Canyigit M, Kara T Ulus... Occurs along the spinal column and can range in size all sequences the literature protective tissues cover. Also known as sickle-shaped sacrum or hemisacral agenesis, is a preview of subscription,...: 10.3928/01477447-20080201-21, Gürgan A. spine ( Phila Pa 1976 ) obscured by the heart shadow meningocele typically mild... Aug ; 16 ( 8 ):522-4. doi: 10.1007/s00384-015-2417-1 imaging to optimise sacral nerve.! Showed the classic scimitar sign ” that is pathognomonic for anterior sacral meningocele has to be a purely intrasacral.!:351-7. doi: 10.1007/BF01403013 ):455-62 ; discussion 462 syndrome: report of two cases.. Management of a meningocele is a unilateral, well-marginated, crescent-shaped defect in which the spinal cord nervous! Bladder, and 1 case showed a defect in the NOTCH3 gene entity, withonlyabout100cases reported clinical examination necessary. Cord or nervous tissue '' was treated with antibiotics Oct ; 53 ( 4 ):385-6. doi:.... It typically occurs along the spinal column 2 ):351-7. doi: 10.1007/PL00007296 ``!
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