|
1
|
Kurogi A, Murakami N, Mukae N, Shimogawa T, Shono T, Suzuki SO, Morioka T. Retained Medullary Cord Associated with Terminal Myelocystocele and Intramedullary Arachnoid Cyst. Pediatr Neurosurg 2022; 57:184-190. [PMID: 35259748 DOI: 10.1159/000523976] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/24/2021] [Accepted: 03/04/2022] [Indexed: 11/19/2022]
Abstract
INTRODUCTION The retained medullary cord (RMC) is a newly defined entity of closed spinal dysraphism that is thought to originate from regression failure of the medullary cord during the last phase of secondary neurulation. The terminal myelocystocele (TMC) is an unusual type of closed spinal dysraphism, characterized by localized cystic dilatation of the terminal part of the central canal that then herniates through a posterior spinal bifida. The co-occurrence of RMC and TMC is extremely rare. CASE PRESENTATION We treated a baby girl with a huge sacrococcygeal meningocele-like sac with two components. Untethering surgery and repair surgery for the sac revealed that RMC, associated with intramedullary arachnoid cyst (IMAC), was terminated at the bottom of the rostral cyst, forming the septum of the two cystic components, and the caudal cyst was TMC derived from the central canal-like ependymal lining lumen (CC-LELL) of the RMC at the septum. IMAC within the RMC communicated with TMC, and both contained xanthochromic fluid with the same properties. CONCLUSION We speculated that the mass effect of the coexistent IMAC impeded the flow of cerebrospinal fluid in the CC-LELL within the RMC and eventually formed a huge TMC. In surgical strategies for such complex pathologies, it is important to identify the electrophysiological border between the functional cord and nonfunctional RMC and the severe RMC to untether the cord, as with a typical or simple RMC.
Collapse
Affiliation(s)
- Ai Kurogi
- Department of Neurosurgery, Fukuoka Children's Hospital, Fukuoka, Japan
| | - Nobuya Murakami
- Department of Neurosurgery, Fukuoka Children's Hospital, Fukuoka, Japan
| | - Nobutaka Mukae
- Department of Neurosurgery, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
| | | | - Tadahisa Shono
- Department of Neurosurgery, Harasanshin Hospital, Fukuoka, Japan
| | | | - Takato Morioka
- Department of Neurosurgery, Harasanshin Hospital, Fukuoka, Japan
| |
Collapse
|
|
2
|
Rhodes RH. Congenital Spinal Lipomatous Malformations. Part 2. Differentiation from Selected Closed Spinal Malformations. Fetal Pediatr Pathol 2021; 40:32-68. [PMID: 31535937 DOI: 10.1080/15513815.2019.1651799] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/26/2022]
Abstract
BACKGROUND Congenital spinal lipomatous malformations (spinal lipomas, lipomyeloceles, and lipomyelomeningoceles) are closed neural tube defects over the lower back. Differentiation from some other closed neural tube defects in this region can be problematic for pathologists. MATERIALS AND METHODS This review is based on PubMed searches of the embryology, gross and histopathologic findings, and laboratory reporting requisites for retained medullary spinal cords, coccygeal medullary vestiges and cysts, myelocystoceles, true human vestigial tails, and pseudotails for comparison with congenital spinal lipomatous malformations. RESULTS Embryology, imaging, gross and histopathology of these closed neural tube lesions have different but overlapping features compared to congenital spinal lipomatous malformations, requiring context for diagnosis. CONCLUSION The lipomyelocele spectrum and to some degree all of the malformations discussed, even though they may not share gross appearance, anatomic site, surgical approach, or prognosis, require clinical and histopathologic correlation for final diagnosis.
Collapse
Affiliation(s)
- Roy H Rhodes
- Department of Pathology, Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey, USA
| |
Collapse
|
|
3
|
Gupta D, Varshney G. Giant terminal myelocystocele: A case report. J Pediatr Neurosci 2020; 15:286-289. [PMID: 33531948 PMCID: PMC7847119 DOI: 10.4103/jpn.jpn_157_19] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/25/2019] [Revised: 01/29/2020] [Accepted: 05/27/2020] [Indexed: 11/04/2022] Open
Abstract
Myelocystoceles, also known as syringoceles, syringomyeloceles, and lipomeningomyelocystoceles, represent 5% of all lumbosacral skin-covered masses. Terminal myelocystocele (TMC) is a rare variant of spinal dysraphism. At times, patients with TMCs can present with a huge lumbosacral mass (giant TMC). A 14-month-old female child presented with progressive increasing swelling in the lumbosacral region (35 cm × 35 cm × 30 cm) since birth with inability to move both lower limbs. Magnetic resonance imaging revealed a dilated terminal central canal herniating through a spina bifida defect (from L4 to S2) into a huge meningocele, suggestive of a giant TMC. The girl was operated on successfully and was doing well at 6 months follow-up, although neurologically unchanged. Giant TMC is a rare entity and only a few case reports are available in literature. Herein we present a case with TMC with the largest dimensions reported till date.
Collapse
|
|
4
|
Kumar J, Afsal M, Garg A. Imaging spectrum of spinal dysraphism on magnetic resonance: A pictorial review. World J Radiol 2017; 9:178-190. [PMID: 28529681 PMCID: PMC5415887 DOI: 10.4329/wjr.v9.i4.178] [Citation(s) in RCA: 30] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/13/2016] [Revised: 02/15/2017] [Accepted: 03/02/2017] [Indexed: 02/06/2023] Open
Abstract
Congenital malformations of spine and spinal cord are collectively termed as spinal dysraphism. It includes a heterogeneous group of anomalies which result from faulty closure of midline structures during development. Magnetic resonance imaging (MRI) is now considered the imaging modality of choice for diagnosing these conditions. The purpose of this article is to review the normal development of spinal cord and spine and reviewing the MRI features of spinal dysraphism. Although imaging of spinal dysraphism is complicated, a systematic approach and correlation between neuro-radiological, clinical and developmental data helps in making the correct diagnosis.
Collapse
|
|
5
|
Allam ES, Shetty VS, Farmakis SG. Fetal and neonatal presentation of OEIS complex. J Pediatr Surg 2015; 50:2155-8. [PMID: 26520699 DOI: 10.1016/j.jpedsurg.2015.09.018] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/05/2015] [Accepted: 09/28/2015] [Indexed: 11/17/2022]
Abstract
OEIS complex is a rare entity comprising a combination of omphalocele, exstrophy of the cloaca, imperforate anus, and spinal defects. We present a case that demonstrates the imaging features of OEIS complex, which also has the rare diagnosis of a terminal myelocystocele, across multiple imaging modalities both prenatally and postnatally. A prenatal diagnosis of OEIS complex allowed for appropriate planned multidisciplinary management of this patient.
Collapse
Affiliation(s)
- Emad S Allam
- Saint Louis University School of Medicine Department of Radiology, 3635 Vista Avenue, St. Louis, MO 63110, USA.
| | - Vilaas S Shetty
- Saint Louis University School of Medicine Department of Radiology, 3635 Vista Avenue, St. Louis, MO 63110, USA
| | - Shannon G Farmakis
- Saint Louis University School of Medicine Department of Radiology, 3635 Vista Avenue, St. Louis, MO 63110, USA
| |
Collapse
|
|
6
|
Quong WL, Bulstrode NW, Thompson DNP. The use of de-epithelialized skin flap in the surgical repair of terminal myelocystoceles. Childs Nerv Syst 2015; 31:473-9. [PMID: 25480697 DOI: 10.1007/s00381-014-2598-x] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/08/2014] [Accepted: 10/29/2014] [Indexed: 11/25/2022]
Abstract
BACKGROUND Terminal myelocystocele is a severe form of spinal dysraphism characterized by cystic expansion of the terminal spinal cord that herniates through a deficiency of the posterior sacral spinal elements to fuse with the subcutaneous fat. Postnatal enlargement of the subcutaneous fluid-filled sac may result in progressive neurological deficit and threaten the viability of the overlying skin. Surgical repair entails spinal cord untethering, resection of nonfunctional neural elements and watertight reconstruction of the terminal thecal sac. Young age at the time of surgery, large dural defect, attenuated tissues and locally altered CSF dynamics frequently mean that wound complications including CSF leakage and pseudomeningocele formation are common. TECHNIQUE With consideration of these requirements, we describe our surgical technique in terminal myelocystocele repair, which combines a novel surgical incision and for the first time in a neurosurgical setting, the use of a de-epithelialized skin flap to augment the closure. We report successful operative outcomes in three infant patients with terminal myelocystocele.
Collapse
Affiliation(s)
- Whitney L Quong
- Department of Plastic and Reconstructive Surgery, The Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK
| | | | | |
Collapse
|
|
7
|
Guo A, Chitayat D, Blaser S, Keating S, Shannon P. Fetal syringomyelia. Acta Neuropathol Commun 2014; 2:91. [PMID: 25092126 PMCID: PMC4167126 DOI: 10.1186/s40478-014-0091-0] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/19/2014] [Accepted: 07/23/2014] [Indexed: 11/12/2022] Open
Abstract
We explored the prevalence of syringomyelia in a series of 113 cases of fetal dysraphism and hindbrain crowding, of gestational age ranging from 17.5 to 34 weeks with the vast majority less than 26 weeks gestational age. We found syringomyelia in 13 cases of Chiari II malformations, 5 cases of Omphalocele/Exostrophy/Imperforate anus/Spinal abnormality (OEIS), 2 cases of Meckel Gruber syndrome and in a single pair of pyopagus conjoined twins. Secondary injury was not uncommon, with vernicomyelia in Chiari malformations, infarct like histology, or old hemorrhage in 8 cases of syringomyelia. Vernicomyelia did not occur in the absence of syrinx formation. The syringes extended from the sites of dysraphism, in ascending or descending patterns. The syringes were usually in a major proportion anatomically distinct from a dilated or denuded central canal and tended to be dorsal and paramedian or median. We suggest that fetal syringomyelia in Chiari II malformation and other dysraphic states is often established prior to midgestation, has contributions from the primary malformation as well as from secondary in utero injury and is anatomically and pathophysiologically distinct from post natal syringomyelia secondary to hindbrain crowding.
Collapse
|
|
8
|
Umamaheswara Reddy V, Agrawal A, Hegde KV, Sharma V, Malpani P, Manchikanti V. Terminal hemimyelocystocele associated with Chiari II malformation. EGYPTIAN PEDIATRIC ASSOCIATION GAZETTE 2014. [DOI: 10.1016/j.epag.2014.05.002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022] Open
|
|
9
|
Lee JY, Kim SP, Kim SW, Park SH, Choi JW, Phi JH, Kim SK, Pang D, Wang KC. Pathoembryogenesis of terminal myelocystocele: terminal balloon in secondary neurulation of the chick embryo. Childs Nerv Syst 2013; 29:1683-8. [PMID: 23780405 DOI: 10.1007/s00381-013-2196-3] [Citation(s) in RCA: 25] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/21/2013] [Accepted: 05/30/2013] [Indexed: 11/24/2022]
Abstract
PURPOSE Terminal myelocystocele (TMC) is thought to be caused by a misstep during secondary neurulation. However, due to the paucity of data on secondary neurulation and the rarity of TMC, proofs of this pathogenetic mechanism are unavailable. Based on a previous observation that TMC resembles a step of secondary neurulation in chick, a closer look was taken at secondary neurulation of chick embryos focusing on the cerebrospinal fluid-filled distal neural tube (terminal balloon). METHODS Chick embryos at Hamburger and Hamilton (H-H) stages of 28, 30, 33, 35, 37, and 40 were harvested. Hematoxying-eosin staining, additional immunohistochemistry (laminin, cytokeratin, nestin), and scanning electron microscopy were performed. RESULTS In H-H stages 28 to 30, after merging of the lumina of the primary and secondary neural tubes, the caudal end of the confluent tube dilates into a balloon-like structure (terminal balloon). As the proximal tube progressively becomes narrower, the terminal balloon dilates even further, and its wall fuses with the surface ectoderm (H-H stage 33). Later in H-H stages 35 to 40, the terminal balloon shrinks and becomes detached from the surface ectoderm and ultimately disappears, as the proximal lumen of the secondary neural tube continues to collapse. CONCLUSION A dilated balloon doubtlessly exists in the terminal secondary neural tube in chick embryos, and its subsequent disappearance occurs in a variable time course and sequence. Arrest of apoptosis resulting in failure of detachment of the terminal balloon from the surface ectoderm may well be the basis for human TMC.
Collapse
Affiliation(s)
- Ji Yeoun Lee
- Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul 110-769, Republic of Korea
| | | | | | | | | | | | | | | | | |
Collapse
|
|
10
|
Pang D, Zovickian J, Lee JY, Moes GS, Wang KC. Terminal myelocystocele: surgical observations and theory of embryogenesis. Neurosurgery 2012; 70:1383-404; discussion 1404-5. [PMID: 22270234 DOI: 10.1227/neu.0b013e31824c02c0] [Citation(s) in RCA: 57] [Impact Index Per Article: 4.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/19/2022] Open
Abstract
BACKGROUND The structural complexity of terminal myelocystocele (TMC) precludes a recognizable link to spinal cord development and therefore a plausible embryogenetic theory. OBJECTIVE To demystify TMC using clinical, imaging, surgical, electrophysiological, and histopathological data and to propose a theory of embryogenesis. METHODS Our series consisted of 4 newborns and 6 older children. All had preoperative magnetic resonance imagings and surgical repair of the myelocystocele with electrophysiological monitoring. RESULTS TMC can be deconstructed into essential and nonessential features. Essential features are present in all TMCs and constitute the core malformation, comprising an elongated spinal cord extending extraspinally into a cerebrospinal fluid--filled cyst that is broadly adherent to the subcutaneous fat. The functional conus resides in the proximal cyst or within the intraspinal cord, and the caudal myelocystocele wall is nonfunctional fibroneural tissue. Nonessential features include variable measures of hydromyelia, caudal meningocele, and fat, present in only some patients. The core structure of TMC strikingly resembles a transitory stage of late secondary neurulation in chicks in which the cerebrospinal fluid-filled bleblike distal neural tube bulges dorsally to fuse with the surface ectoderm, before focal apoptosis detaches it from the surface and undertakes its final dissolution. We theorize that TMC results from a time-specific paralysis of apoptosis just before the dehiscence of the cystic distal cord from the future skin, thereby preserving the embryonic state. CONCLUSION Besides tethering, the myelocystocele may show early rapid expansion causing precipitous deterioration. We recommend early repair with resection of the nonfunctional caudal cyst wall, reconstruction of the proximal neural placode, and duraplasty.
Collapse
Affiliation(s)
- Dachling Pang
- Department of Paediatric Neurosurgery, University of California, Davis, CA, USA.
| | | | | | | | | |
Collapse
|
|
11
|
Rufener S, Ibrahim M, Parmar HA. Imaging of congenital spine and spinal cord malformations. Neuroimaging Clin N Am 2012; 21:659-76, viii. [PMID: 21807317 DOI: 10.1016/j.nic.2011.05.011] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/22/2022]
Abstract
This article reviews normal embryologic development of the spine and spinal cord and the imaging features of congenital abnormalities of the spine and spinal cord, with particular focus on magnetic resonance imaging. The authors discuss spinal dysraphisms, a heterogeneous group of congenital abnormalities of the spine and spinal cord, and provide information to expand understanding of these complex entities.
Collapse
Affiliation(s)
- Stephanie Rufener
- Department of Radiology, University of Michigan Health System, Ann Arbor, MI 48109, USA
| | | | | |
Collapse
|
|
12
|
Joshua SP, Singh PK, Mahapatra AK. Vertebral choristoma in lipomyelomeningocele: a case report. Pediatr Neurosurg 2012; 48:59-61. [PMID: 22832189 DOI: 10.1159/000339314] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/11/2012] [Accepted: 05/07/2012] [Indexed: 11/19/2022]
Abstract
Lipomeningocele is a type of occult spinal dysraphism characterized by a subcutaneous lipomatous mass that protrudes through a midline bony defect. We report a rare presentation of this condition--a vertebral choristoma wherein the lipomatous mass displaced the normally formed posterior elements--lamina and spinous process of L4 vertebra dorsally to an abnormal location in the absence of a bony defect.
Collapse
Affiliation(s)
- S P Joshua
- Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India
| | | | | |
Collapse
|
|
13
|
Lee JY, Phi JH, Kim SK, Cho BK, Wang KC. Urgent surgery is needed when cyst enlarges in terminal myelocystoceles. Childs Nerv Syst 2011; 27:2149-53. [PMID: 21786178 DOI: 10.1007/s00381-011-1532-8] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/06/2011] [Accepted: 07/08/2011] [Indexed: 11/30/2022]
Abstract
PURPOSE The phenomenon of cyst enlargement in terminal myelocystocele (TMC) patients has been mentioned in the literature. However, its clinical significance has not been clarified in depth. We reviewed TMC patients who had experienced cyst enlargement during the preoperative period to determine the correlation with symptomatic progression. We sought to determine the optimal surgical strategy for such patients. METHODS Of eight patients who were operated on for TMC from 1991 to 2009, four patients that showed cyst enlargement before the operation were reviewed. A retrospective review of medical recordings, radiological imaging, and other evaluations for neurologic status was done, focusing on the temporal neurological changes that correlated with the cyst enlargement. RESULTS Each of the four patients with cyst enlargement showed concurrent progression of neurologic symptoms, two by motor weakness and two by neurogenic bladder. In only one case, early detection and prompt surgery were possible, and this patient showed dramatic improvement in muscle strength after the surgery. The progression of neurologic deterioration was arrested postoperatively for the other three patients. CONCLUSIONS Hence, cyst enlargement in a TMC patient should be considered as a warning for neurologic deterioration, and the operation should be performed expediently to prevent impairment in neurologic function.
Collapse
Affiliation(s)
- Ji Yeoun Lee
- Division of Pediatric Neurosurgery, Department of Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, 101 Daehakro, Jongno-gu, Seoul 110-769, Republic of Korea
| | | | | | | | | |
Collapse
|
|
14
|
|
|
15
|
Singh DK, Kumar B, Sinha VD, Bagaria HR. The human tail: rare lesion with occult spinal dysraphism--a case report. J Pediatr Surg 2008; 43:e41-3. [PMID: 18778987 DOI: 10.1016/j.jpedsurg.2008.04.030] [Citation(s) in RCA: 29] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/27/2008] [Revised: 04/18/2008] [Accepted: 04/18/2008] [Indexed: 12/18/2022]
Abstract
Human tail or tail-like caudal cutaneous appendage is a rare fingerlike, midline protrusion at the lumbosacrococcygeal region, often associated with occult spinal dysraphism. A 2-month-old male child presented here had a lumbosacral tail-like appendage with underlying spinal dysraphism without any appreciable neurological deficit. In contradiction to a previous report, true vestigial tails are not benign because they may be associated with underlying dysraphic state. About 50% of the cases were associated with either meningocele or spina bifida occulta. Management of such lesions must include complete neurological history and examination as well as magnetic resonance or computed tomographic imaging. After diagnosis, microsurgery should be performed if there is any intraspinal component to avoid any damage and neurological deficit.
Collapse
Affiliation(s)
- Deepak Kumar Singh
- Department of Neurosurgery, S.M.S. Medical College, Jaipur, Rajasthan 302004, India
| | | | | | | |
Collapse
|
|
16
|
Abstract
Tethered cord syndrome is a clinical phenomenon resulting from anatomic restriction of the normal movement of the spinal cord or vascular compromise leading to hypoxia of its distal structures. Tethering can be acquired (secondary) or congenital (primary). This article presents the relevant embryology, primary and secondary causes of tethering, clinical presentations, the treatment of specific entities, indications and options for Tethered cord syndrome is a clinical phenomenon resulting from anatomic restriction of the normal movement of the spinal cord or vascular compromise leading to hypoxia of its distal structures. Causes of tethering can be acquired (secondary) or congenital (primary). This article presents the relevant embryology, primary and secondary causes of tethering, clinical presentations, the treatment of specific entities, indications and options for surgical treatment, and surgical complications.
Collapse
Affiliation(s)
- Pankaj K Agarwalla
- Department of Neurosurgery, Children's Hospital of Boston, Boston, MA 02115, USA
| | | | | | | |
Collapse
|
|
17
|
Rossi A, Gandolfo C, Cama A, Tortori-Donati P. Congenital Malformations of the Spine, Spinal Cord, and Cranio-Cervical Junction. ACTA ACUST UNITED AC 2007. [DOI: 10.1007/978-3-540-68483-1_1] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
|
|
18
|
Rossi A, Gandolfo C, Morana G, Piatelli G, Ravegnani M, Consales A, Pavanello M, Cama A, Tortori-Donati P. Current Classification and Imaging of Congenital Spinal Abnormalities. Semin Roentgenol 2006; 41:250-73. [PMID: 17010690 DOI: 10.1053/j.ro.2006.07.001] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Affiliation(s)
- Andrea Rossi
- Department of Neuroradiology, G. Gaslini Children's Research Hospital, Genova, Italy.
| | | | | | | | | | | | | | | | | |
Collapse
|
|
19
|
Jaiswal AK, Mahapatra AK. Terminal myelocystocele. J Clin Neurosci 2005; 12:249-52. [PMID: 15851075 DOI: 10.1016/j.jocn.2004.03.038] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/03/2004] [Accepted: 03/23/2004] [Indexed: 11/26/2022]
Abstract
Myelocystocele is a variety of occult spinal dysraphism that is characterized by a localized, cystic dilatation of the terminal part of the central canal that then herniates through a posterior spina bifida. We describe a series of eight cases of myelocystocele. The clinical and radiological features and treatment are discussed and the relevant literature reviewed.
Collapse
Affiliation(s)
- Awadhesh Kumar Jaiswal
- Department of Neurosurgery, Neurosciences Centre, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India
| | | |
Collapse
|
|
20
|
Rossi A, Biancheri R, Cama A, Piatelli G, Ravegnani M, Tortori-Donati P. Imaging in spine and spinal cord malformations. Eur J Radiol 2004; 50:177-200. [PMID: 15081131 DOI: 10.1016/j.ejrad.2003.10.015] [Citation(s) in RCA: 82] [Impact Index Per Article: 3.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/03/2003] [Revised: 10/09/2003] [Accepted: 10/13/2003] [Indexed: 01/13/2023]
Abstract
Spinal and spinal cord malformations are collectively named spinal dysraphisms. They arise from defects occurring in the early embryological stages of gastrulation (weeks 2-3), primary neurulation (weeks 3-4), and secondary neurulation (weeks 5-6). Spinal dysraphisms are categorized into open spinal dysraphisms (OSDs), in which there is exposure of abnormal nervous tissues through a skin defect, and closed spinal dysraphisms (CSD), in which there is a continuous skin coverage to the underlying malformation. Open spinal dysraphisms basically include myelomeningocele and other rare abnormalities such as myelocele and hemimyelo(meningo)cele. Closed spinal dysraphisms are further categorized based on the association with low-back subcutaneous masses. Closed spinal dysraphisms with mass are represented by lipomyelocele, lipomyelomeningocele, meningocele, and myelocystocele. Closed spinal dysraphisms without mass comprise simple dysraphic states (tight filum terminale, filar and intradural lipomas, persistent terminal ventricle, and dermal sinuses) and complex dysraphic states. The latter category further comprises defects of midline notochordal integration (basically represented by diastematomyelia) and defects of segmental notochordal formation (represented by caudal agenesis and spinal segmental dysgenesis). Magnetic resonance imaging (MRI) is the preferred modality for imaging these complex abnormalities. The use of the aforementioned classification scheme is greatly helpful to make the diagnosis.
Collapse
Affiliation(s)
- Andrea Rossi
- Department of Neuroradiology, G. Gaslini Children's Research Hospital, Largo G. Gaslini 5, I-16147 Genova, Italy.
| | | | | | | | | | | |
Collapse
|
|
21
|
Rossi A, Cama A, Piatelli G, Ravegnani M, Biancheri R, Tortori-Donati P. Spinal dysraphism: MR imaging rationale. J Neuroradiol 2004; 31:3-24. [PMID: 15026728 DOI: 10.1016/s0150-9861(04)96875-7] [Citation(s) in RCA: 48] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/13/2023]
Abstract
Spinal cord development occurs through the three consecutive periods of gastrulation (weeks 2-3), primary neurulation (weeks 3-4), and secondary neurulation (weeks 5-6). Spinal cord malformations derive from defects in these early embryonic stages, and are collectively called spinal dysraphisms. Spinal dysraphisms may be categorized clinically into open and closed, based on whether the abnormal nervous tissue is exposed to the environment or covered by skin. Open spinal dysraphisms include myelomeningocele and other rare abnormalities such as myelocele, hemimyelomeningocele, and hemimyelocele, and are always associated with a Chiari II malformation. Closed spinal dysraphisms are further divided into two subsets based on whether a subcutaneous mass is present in the low back. Closed spinal dysraphisms with mass comprise lipomyelocele, lipomyelomeningocele, meningocele, and myelocystocele. Closed spinal dysraphisms without mass comprise simple dysraphic states (tight filum terminale, filar and intradural lipomas, persistent terminal ventricle, and dermal sinuses) and complex dysraphic states. The latter category involves abnormal notochordal development, either in the form of failed midline integration (ranging from complete dorsal enteric fistula to neurenteric cysts and diastematomyelia) or of segmental agenesis (caudal agenesis and spinal segmental dysgenesis). Magnetic resonance imaging is the imaging modality of choice for evaluation of this complex group of disorders.
Collapse
Affiliation(s)
- A Rossi
- Department of Pediatric Neuroradiology, G Gaslini Children's Research Hospital, Genoa, Italy.
| | | | | | | | | | | |
Collapse
|
|
22
|
Abstract
Defects of development of the neural tube can result in a number of seemingly different malformations. Understanding the abnormal embryology helps one understand the malformations and their surgical treatments. The clinical presentations and the follow-up of these patients require attention to various end organs besides the nervous system. For most of these conditions, long-term follow-up is necessary regardless of initial treatment. A decline in function is not a part of the natural history of these malformations and requires prompt evaluation and treatment.
Collapse
Affiliation(s)
- Bruce A Kaufman
- Department of Neurosurgery, Medical College of Wisconsin, Milwaukee, WI 53201, USA.
| |
Collapse
|
|
23
|
Parmar H, Patkar D, Shah J, Maheshwari M. Diastematomyelia with terminal lipomyelocystocele arising from one hemicord: case report. Clin Imaging 2003; 27:41-3. [PMID: 12504320 DOI: 10.1016/s0899-7071(02)00522-3] [Citation(s) in RCA: 20] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
Diastematomyelia is an uncommon spinal dysraphism with midline dorsoventral clefting of the spinal cord. Formation of syrinx cavity within the hemicord, vertebral anomalies and lipomas is known to be associated with diastematomyelia. We present an unusual case of a 4-month-old girl, who presented with diastematomyelia and terminal myelocystocele, and dilatation of the terminal portion of the spinal canal with formation of a cystic structure. Findings were confirmed by subsequent surgery and histopathology. Ectopic right kidney, Chiari I malformation and changes of partial sacral agenesis were other findings noted in this case. No case report of formation of terminal myelocystocele arising from one hemicord was described in the literature we reviewed.
Collapse
Affiliation(s)
- Hemant Parmar
- Department of Radiology, King Edward VII Memorial Hospital, Mumbai, India.
| | | | | | | |
Collapse
|
|
24
|
Abstract
Terminal myelocystocele is an unusual form of occult spinal dysraphism. It consists of a cystic dilatation of a low-lying terminal cord herniated posteriorly through a skin covered lumbosacral spina bifida. This condition is often associated with OEIS complex i.e. opmphalocele, exstrophy of the bladder, imperforate anus and spinal abnormality. We studied 4 cases of terminal myelocystocele. They revealed no preoperative neurological deficit. None of these had associated OEIS complex. One of the cyst was unique due to presence of copious amount of pus in its cavity. All four cases underwent successful repair and surgery remained uneventful in all of them. No child showed neurological deterioration during the follow-up.
Collapse
Affiliation(s)
- Raj Kumar
- King George's Medical College, Lucknow, India.
| | | |
Collapse
|
|
25
|
Evans A, Stoodley N, Halpin S. Magnetic resonance imaging of intraspinal cystic lesions: a pictorial review. Curr Probl Diagn Radiol 2002; 31:79-94. [PMID: 12140512 DOI: 10.1067/cdr.2002.125402] [Citation(s) in RCA: 29] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022]
Abstract
Magnetic resonance imaging is the imaging modality of choice for the evaluation of diseases of the spinal cord and its coverings primarily because of improved lesion detection and characterization. The differentiation of intramedullary pathologic conditions (arising within the spinal cord) from extramedullary lesions (lying either within the thecal sac (intradural) or outside it (extradural) is usually straightforward. Although many lesions may occur in more than one compartment or may extend to involve more than one compartment simultaneously, accurate positioning of a detected lesion within the spinal canal and the assessment of its signal characteristics may help differentiate many of the more commonly encountered lesions and aid preoperative diagnosis. This pictorial review discusses the imaging findings and distinguishing features of a variety of intramedullary and extramedullary intraspinal cystic lesions with the use of magnetic resonance imaging. The imaging appearances are correlated with histologic findings where available.
Collapse
Affiliation(s)
- A Evans
- Department of Radiology, University Hospital of Wales, Heath Park, Cardiff, Wales.
| | | | | |
Collapse
|
|
26
|
Tortori-Donati P, Rossi A, Biancheri R, Cama A. Magnetic resonance imaging of spinal dysraphism. Top Magn Reson Imaging 2001; 12:375-409. [PMID: 11744877 DOI: 10.1097/00002142-200112000-00003] [Citation(s) in RCA: 79] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/13/2023]
Abstract
Spinal cord development occurs through three consecutive periods. Gastrulation (weeks 2-3) is characterized by conversion of the embryonic disk from a bilaminar to a trilaminar arrangement and establishment of a notochord. Primary neurulation (weeks 3-4) produces the uppermost nine tenths of the spinal cord. Secondary neurulation and retrogressive differentiation (weeks 5-6) result in formation of the conus tip and filum terminale. Defects in these early embryonic stages produce spinal dysraphisms, which are characterized by anomalous differentiation and fusion of dorsal midline structures. Spinal dysraphisms may be categorized clinically into two subsets. In open spinal dysraphisms, the placode (non-neurulated neural tissue) is exposed to the environment. These disorders include myelomeningocele, myeloschisis, hemimyelomeningocele, and hemimyelocele, and are always associated with a Chiari II malformation. Closed spinal dysraphisms are covered by intact skin, although cutaneous stigmata usually indicate their presence. Two subsets may be identified based on whether a subcutaneous mass is present in the low back. Closed spinal dysraphisms with mass comprise lipomyeloschisis, lipomyelomeningocele, meningocele, and myelocystocele. Closed spinal dysraphisms without mass comprise complex dysraphic states (ranging from complete dorsal enteric fistula to neurenteric cysts, split cord malformations, dermal sinuses, caudal regression, and spinal segmental dysgenesis), bony spina bifida, tight filum terminale, filar and intradural lipomas, and persistent terminal ventricle. Magnetic resonance imaging is the imaging method of choice for investigation of this complex group of disorders.
Collapse
Affiliation(s)
- P Tortori-Donati
- Department of Pediatric Neuroradiology, G. Gaslini Children's Research Hospital, Genova, Italy.
| | | | | | | |
Collapse
|
|
27
|
Abstract
The most common, primary referrals to a pediatric neurosurgeon's office are the evaluation and management of the child with a large head (to rule out hydrocephalus and other space occupying lesions) a mishappen head (to rule out various forms of craniosynostosis), or some form of congenital spinal abnormality (spinal dysraphism). The authors discuss the pathogenesis and clinical features of these disorders, provide a framework for diagnostic evaluation and referral, and discuss the various treatment options available for each.
Collapse
Affiliation(s)
- M S Dias
- Department of Pediatric Neurosurgery, Children's Hospital of Buffalo, State University of New York at Buffalo, USA
| | | |
Collapse
|
|
28
|
Nishino A, Shirane R, So K, Arai H, Suzuki H, Sakurai Y. Cervical myelocystocele with Chiari II malformation: magnetic resonance imaging and surgical treatment. SURGICAL NEUROLOGY 1998; 49:269-73. [PMID: 9508113 DOI: 10.1016/s0090-3019(97)00180-8] [Citation(s) in RCA: 23] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
BACKGROUND The myelocystocele is a rare clinical entity, and there have only been six case reports concerning the cervical myelocystocele, including ours. METHODS The case of a female neonate with cervical myelocystocele, who presented with respiratory distress beginning at birth is reported. RESULTS Magnetic resonance (MR) imaging disclosed a cystic lesion surrounded by two layers of membrane, which were recognized to be the dural and arachnoid layer and the ependymal lining layer, respectively, during operation and on histopathologic examination. Hydromyelia at C2-C7 with Chiari II malformation was also observed. After a thorough repair and untethering surgery, the hydromyelia was diminished on a follow-up MR examination. The patient's respiratory status normalized several days postoperatively, and her growth and development were within normal limits at the 2-year follow-up. CONCLUSIONS In cases of cervical myelocystocele with Chiari II malformation, untethering through intradural exploration to treat the tethered cord and outer decompression of the foramen magnum are needed. MR imaging is the best modality for preoperative determination of anatomic relationships.
Collapse
Affiliation(s)
- A Nishino
- Department of Neurosurgery, Sendai National Hospital, Tohoku University, School of Medicine, Japan
| | | | | | | | | | | |
Collapse
|
|
29
|
Raftopoulos C, Balériaux D, Hancq S, Closset J, David P, Brotchi J. Evaluation of endoscopy in the treatment of rare meningoceles: preliminary results. SURGICAL NEUROLOGY 1995; 44:308-17; discussion 317-8. [PMID: 8553249 DOI: 10.1016/0090-3019(95)00199-9] [Citation(s) in RCA: 18] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/31/2023]
Abstract
BACKGROUND Endoscopy is used on different occasions-for instance, to open the floor of the third ventricule in triventricular hydrocephalus, to open a cyst into the cerebrospinal fluid circulation, for biopsy or for partial resection of some tumors, or to insert a shunt in hydrocephalus or syringohydromyelia. However, the use of endoscopes for evaluating and treating meningoceles remains to be assessed. METHODS Five different kinds of rare meningoceles are presented. In each, neuroendoscopy was used as the main tool for exploration and treatment. RESULTS Two sacral meningoceles and one oral cephalocele were cured through a keyhole opening under endoscopic control. One posterior sacral meningocele was explored and no communication with normal subarachnoid spaces was observed, allowing a simple suture of the posterior to the anterior walls. And, last, a complex case with three intrasacral meningocles was explored and partially treated. CONCLUSIONS Meningocles with very small communication within the normal subarachnoid spaces appeared the most suitable to be cured by an endoscopic procedure. In case of a larger communication, the meningocele could be treated, or at least the morphology can be better understood, by using a keyhole procedure under endoscopic control. In all cases the surgery was of short duration (less than 1 hour) and very well tolerated.
Collapse
Affiliation(s)
- C Raftopoulos
- Department of Neurosurgery, Erasme Hospital, Université Libre de Bruxelles, Belgium
| | | | | | | | | | | |
Collapse
|
|
30
|
Abstract
PURPOSE To delineate the clinical and MR findings in children with an unusual type of spinal dysraphism, the terminal myelocystocele. Infants with a terminal myelocystocele carry a favorable neurologic prognosis if the entity is diagnosed early. Understanding the MR characteristics of this entity will allow for earlier and more accurate diagnosis. METHOD Analysis of the medical charts and MR studies in 15 children with surgically and histologically proven myelocystocele. RESULTS In all 15 children, MR demonstrated the primary findings of a terminal cyst of the central canal of the spinal cord which is tethered and herniated with arachnoid and cerebrospinal fluid through an area of spinal dysraphia onto the back as a mass. Of these children, 10 had additional findings (one or more) on MR of Chiari I (five cases), Chiari II (one case), cervicothoracic hydromyelia (two cases), lumbar hydromyelia (two cases), hydrocephalus (2 cases) segmentation anomalies of vertebrae (3 cases) and partial agenesis of sacrum (six cases). Of the clinical findings, all 15 children had a back mass, 10 also had cloacal exstrophy. One had imperforate anus, 10 were girls, five had ambiguous genitalia and all were neurologically intact. CONCLUSION Children with a terminal myelocystocele present with a back mass and there is a high association with cloacal exstrophy. MR is the best noninvasive modality to diagnose all of the components of a terminal myelocystocele and the associated central nervous system findings.
Collapse
Affiliation(s)
- S E Byrd
- Department of Radiology, Children's Memorial Hospital, Chicago, IL 60614, USA
| | | | | |
Collapse
|
|
31
|
McLaughlin KP, Rink RC, Kalsbeck JE, Keating MA, Adams MC, King SJ, Luerssen TG. Cloacal Exstrophy: The Neurological Implications. J Urol 1995. [DOI: 10.1016/s0022-5347(01)67162-3] [Citation(s) in RCA: 21] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/25/2022]
Affiliation(s)
- Kevin P. McLaughlin
- James Whitcomb Riley Hospital for Children, Indiana University Medical Center, Indianapolis, Indiana
| | - Richard C. Rink
- James Whitcomb Riley Hospital for Children, Indiana University Medical Center, Indianapolis, Indiana
| | - John E. Kalsbeck
- James Whitcomb Riley Hospital for Children, Indiana University Medical Center, Indianapolis, Indiana
| | - Michael A. Keating
- James Whitcomb Riley Hospital for Children, Indiana University Medical Center, Indianapolis, Indiana
| | - Mark C. Adams
- James Whitcomb Riley Hospital for Children, Indiana University Medical Center, Indianapolis, Indiana
| | - Shelly J. King
- James Whitcomb Riley Hospital for Children, Indiana University Medical Center, Indianapolis, Indiana
| | - Thomas G. Luerssen
- James Whitcomb Riley Hospital for Children, Indiana University Medical Center, Indianapolis, Indiana
| |
Collapse
|
|
32
|
Abstract
Sonography of the infant spine can rapidly and safely exclude or confirm the presence of a congenital malformation. This article reviews the embryology of the spinal cord, abnormal and normal sonographic findings, and findings associated with occult dysraphic lesions. Patient selection for sonographic assessment is also discussed. Illustrations of normal anatomy and the more common dysraphic lesions are provided.
Collapse
Affiliation(s)
- H E Korsvik
- Department of Diagnostic Radiology, Yale University, School of Medicine, New Haven, CT 06520-8042
| |
Collapse
|
|
33
|
|